Best of JACC: Case Reports From ACC.23.

Heart Failure and Cardiomyopathies

A CASE OF TOO MANY ANTIBODIES: NOVEL USE OF DARATUMUMAB IN A PRE-HEART TRANSPLANT PATIENT

Soniya V. Rabadia, Ashley Fan, Darko Vucicevic, Ali Nsair, Megan Y. Kamath, University of California-Los Angeles, Los Angeles, CA, USA

Background

Highly sensitized pre-heart transplant patients wait longer for transplant due limited suitable donors and prolonged desensitization protocols. Daratumumab has rarely been used to desensitize pre-heart transplant patients.

Case

A 36 year old female with history of end stage non-ischemic cardiomyopathy, kidney transplant with graft failure on hemodialysis, and prior opportunistic infections presented for combined heart and kidney transplant evaluation. Upon anti-HLA antibody screening, she was found to have 100% calculated panel reactive antibodies (CPRA). Despite treatment with intravenous immunoglobulin and plasmapheresis, CPRA level continued to remain high.

Decision-making

Due to persistent sensitization, daratumumab was added to her treatment regimen. After 23 daratumumab infusions over the course of 1 year, CPRA level was undetectable (Figure 1). Complications were limited to mild neutropenia. Current recommended treatment for pre-heart transplant desensitization focuses on antibody removal. Daratumumab is an IgG CD38-targeting monoclonal antibody that directly inhibits antibody development by targeting plasma B-cells. It is only FDA approved for multiple myeloma treatment and has not been studied in desensitizing pre-heart transplant patients.

Conclusion

Here, we demonstrate the safe and effective use of daratumumab for desensitization. Randomized control trial data is needed to standardize its use in pre-heart transplant patients.

Multimodality Imaging

42-YEAR-OLD MAN WITH CHEST PAIN

Bria J. Rice, Samantha M. Espinosa, Pragnesh Parikh, Peter Pollak, Mayo Clinic, Jacksonville, FL, USA

Background

Partial pericardial agenesis can cause life-threatening complications. We present an ST Elevation Myocardial Infarction (STEMI) caused by herniation of the left ventricle (LV) through a pericardial defect.

Case

A healthy 42-year-old man awoke with chest pain and presented to an outside ER. Electrocardiogram showed lateral ST elevation. Coronary angiography revealed dynamic occlusion of the first diagonal (D1), ramus intermedius, and obtuse marginal arteries in a linear pattern thought to be myocardial bridging. A drug-eluting stent was placed in D1 - the assumed culprit lesion. The patient’s chest pain persisted.

Decision-making

Subsequent review of the coronary angiography revealed filling abnormalities in diastole that resolved in systole, concerning for external compression. Echocardiogram, Gated Cardiac Computed Tomography (CT), and Cardiac Magnetic Resonance Imaging together confirmed ring-shaped constriction around a thickened and hypokinetic, lateral LV wall with herniation of inflamed and edematous myocardium through a deformed pericardium. The D1 stent was compressed on CT. Pericardiectomy was performed, demonstrating strangulation of the lateral LV wall and coronary vessels. The anterior pericardium was removed, vessels decompressed, and defect closed. Balloon angioplasty of the D1 stent was subsequently performed.

Conclusion

Symptomatic partial pericardial defects require surgery to prevent adverse sequelae. Multimodality imaging can aid diagnosis.

Heart Failure and Cardiomyopathies

LATE PRESENTATION Of HIGH-OUTPUT HEART FAILURE RESULTING FROM AN UNDIAGNOSED TRAUMATIC ARTERIOVENOUS FISTULA

Pollakrit Chiewvit, Nattapat Lilitsuwan, Porntera Sethalao, Jitladda Wasinrat, Wiwun Tungsubutra, Siriraj hospital, Bangkok, Thailand

Background

Traumatic arteriovenous fistula (AVF) is a rare cause of heart failure (HF).

Case

A 60-year-old man with HTN presented with dyspnea and orthopnea for 3 months. 20 years ago he sustained a gunshot injury to his right thigh and was eventually diagnosed with lymphedema. BP was 165/60 mmHg with rales in both lungs. His right lower extremity (LE) had marked swelling with hyperpigmentation and a bruit (fig A). CXR showed pulmonary edema.

Decision-making

High-output HF from the LE AVF was diagnosed. Echo showed a dilated LV, dynamic LV function and an elevated cardiac output. CTA showed an AVF between the proximal superficial femoral artery and vein (SFA, SFV) (fig B). After consultation with vascular surgery, the AVF anatomy was deemed feasible for endovascular closure with a low procedural risk. Angiography showed early contrast filling from the SFA to SFV (fig C) and a stent graft was successfully placed in the proximal SFA. One-month post-procedure, his symptoms improved and CTA showed patency of the stent graft. The early enhancement in the accompanying vein was no longer seen. The dilated tortuous superficial veins along the right leg, the degree of skin thickening and subcutaneous fat stranding decreased substantially (fig D).

Conclusion

Traumatic AVF is a treatable cause of HF. Patients who present with high-output HF should undergo a thorough search for an AVF. To make the diagnosis, careful history-taking and physical exam are as vital as advanced imaging tools. AVF closure may lead to full recovery.

Multimodality Imaging

A UNIQUE CASE OF PERICARDIAL EFFUSION AND TAMPONADE

Jarmanjeet Singh, Femina Patel, Bhavdeep Singh, Prabhdeep S. Sethi, Ramdas Pai, Sami B. Nazzal, University of California, Riverside, Riverside, CA, USA

Background

We present a unique case of a female who developed a large pericardial effusion (PEff) from a rare cause.

Case

A 36-year-old female with recent COVID-19 infection presented with acute dyspnea. She had undergone liposuction, rectus plication and breast augmentation two months ago. Heart rate was 90/min and blood pressure 86/57mmHg. CT angiogram of the chest revealed a massive PEff with tamponade. She had large right-sided pleural effusion also. She underwent ultrasound-guided pericardiocentesis with the removal of 950 milliliters of serosanguineous fluid. Follow-up echocardiogram showed re-accumulation of fluid. Due to the rapid onset of PEff, she underwent a pericardial window and bilateral chest tube placement.

Decision-making

Pleural and pericardial fluid analysis showed silicone-gel particles (Figure. 1). Pericardial biopsy showed nonspecific chronic inflammation. Autoimmune workup was unremarkable. Elevated ESR and CRP in the presence of embolized gel particles indicated foreign body reaction from silicone embolism. Plastic surgery advised implant removal. Silicone embolism is known to cause silicone thorax, pleural effusions, and anaplastic large cell lymphoma. To our knowledge, this is the first reported case of PEff due to silicone embolism from breast implants.

Conclusion

Cardiologists should be aware of this rare but serious complication. Silicone embolism should be considered in the differential of PEff in patients with breast implants.

Interventional and Structural

BIPOLAR RADIOFREQUENCY SEPTAL ABLATION FOR RESISTANT LEFT VENTRICULAR OUTFLOW TRACT OBSTRUCTION FOLLOWING TRANSCATHETER MITRAL VALVE REPLACEMENT IN MITRAL ANNULAR CALCIFICATION

Zekun Feng, David Daniels, Elliott Groves, Christian Spies, Christopher Woods, California Pacific Medical Center, San Francisco, CA, USA

Background

Left ventricular outflow tract obstruction (LVOTO) after transcatheter mitral valve replacement (TMVR) is a common complication and carries risk for significant morbidity and mortality.

Case

84F with hypertrophic cardiomyopathy (HCM) and severe mitral stenosis (MS) presented with progressive dyspnea. TTE showed LVEF 75%, LVOT gradient 91 mmHg and mean transmitral gradient 11 mmHg. TEE showed heavy MAC with SAM of the anterior leaflet. Given advanced age and frailty, she underwent percutaneous alcohol septal ablation (ASA) with improvement of left ventricular to aortic (LV-Ao) gradient from 91 to 7 mmHg. But she remained dyspneic. Repeat TTE showed persistent severe MS.

Decision-making

She enrolled in the Mitral II trial and underwent successful TMVR (29 mm Edwards Sapien S3) with post-op mean transmitral gradient of 4 mmHg. Despite laceration of anterior mitral leaflet (LAMPOON) procedure, post-op TTE now showed LVOT gradient 69 mmHg, which persisted after repeat ASA. She remained dyspneic and elected for salvage electroanatomic mapping guided septal radiofrequency ablation (RFA), using Tacticath bipolar system utilizing half normal saline, set at 20-30 Watts for 90-120 seconds. Post-op LV-Ao gradient was 5 mmHg, with TTE showing LVOT gradient of 36 mmHg. Her dyspnea improved drastically and was discharged the next day.

Conclusion

This is the first reported case of successful bipolar septal RFA for treatment of acute persistent LVOTO post-TMVR after failing empiric LAMPOON and ASA.

Heart Failure and Cardiomyopathies

RECURRENT CARDIAC INTIMAL SARCOMA EXTENDING FROM RIGHT VENTRICLE INTO PULMONARY ARTERY

Ohad Oren, David D'Alessandro, Aferdita Spahillari, Deepak L. Bhatt, Massachusetts General Hospital, Boston, MA, USA, and Brigham and Women's Hospital, Boston, MA, USA

Background

Cardiac intimal sarcoma is a rare type of primary cardiac tumor. Complete surgical excision is challenging.

Case

A 43 year-old woman was diagnosed with cardiac intimal sarcoma originating from the RA and effacing the RV requiring surgical excision and tissue TV replacement. Surgical margins were positive, and she completed adjuvant doxorubicin, ifosfamide, and mesna followed by proton radiotherapy to the RV. Four years later, patient develops petechia and labs are consistent with consumptive coagulopathy. CT shows a large mass originating from the RV and extending into the main PA. Patient undergoes surgical resection, bovine pericardial patch reconstruction of the RV free wall and cryoablation. Biopsy shows recurrent intimal sarcoma with positive margins.

Decision-making

An expedited transplant evaluation was conducted. However, due to high sensitization and concern about effects of immunosuppression on cancer, the patient was not listed for transplant. Patient was initiated on ifosfomide and etoposide and underwent proton beam radiation.

Conclusion

Outcomes of patients with high-grade cardiac sarcomas are generally poor. Although adjuvant chemoradiation is associated with improved median overall survival (15.5 vs 2.6 months), the disease remains incurable. Cardiac transplantation may be an option for curative treatment in patients without metastatic disease; however, survival at 1 year is inferior to transplant survival for other indications and is estimated at 61% ± 7%.

Heart Failure and Cardiomyopathies

DIFFUSE LARGE B-CELL LYMPHOMA WITH CARDIAC INVOLVEMENT

Logan Disney, Benjamin Stoner, Adrian W. Messerli, University of Kentucky, Lexington, KY, USA

Background

Diffuse Large B-Cell Lymphoma (DLBCL) is an aggressive, highly treatment-responsive malignancy that rarely affects the heart.

Case

A 42-year-old female presented with chest tightness, acute right heart failure, and 2:1 atrial flutter. TTE revealed a large right heart mass with associated RV wall motion abnormality. Cardiac MRI revealed a 4.2 cm mass in the right AV groove invading the RA, RV, and TV. Additional disease was identified in the liver, kidneys, mediastinal lymph nodes, and spine on CT scans. Mediastinal lymph node biopsy revealed DLBCL.

Decision-making

There is risk of sudden death in patients with cardiac DLBCL due to myocardial rupture resulting from rapid tumor response to treatment. There are reports of successful management with low-dose chemotherapy with dose increases guided by imaging. Due to this patient's advanced disease, high-dose chemotherapy with inpatient monitoring was chosen. After one month, there was substantial reduction in cardiac tumor burden and the patient continued treatment as an outpatient. The cardiac mass resolved on follow-up imaging seven months after diagnosis.

Conclusion

Cardiac DLBCL is rare and may initially be treated with low-dose chemotherapy to reduce risk of myocardial rupture. Patients with advanced disseminated disease may require high-dose chemotherapy and should be monitored closely until cardiac tumor burden is reduced.

Ischemic Heart Disease

EOSINOPHILIC MYOCARDITIS COMPLICATED BY OBSTRUCTIVE CORONARY ARTERY DISEASE TREATED WITH MEPOLIZUMAB

Kevin Pink, Manuel A. Garcia-Smester, Amanda Warren, Rahil Dabhad, Gurinder Sunner, Michael Malekan, New York Presbyterian Brooklyn Methodist Hospital, Brooklyn, NY, USA

Background

Eosinophilic myocarditis is a severe complication of hypereosinophilic syndrome or eosinophilic granulomatosis with polyangitis (EGPA) typically treated with aggressive immunosuppressive intervention, corticosteroids and cyclophosphamide. We present a case of eosinophilic myocarditis treated with Mepolizumab, a monoclonal antibody targeting IL-5, recently approved for the treatment of EGPA.

Case

A 63 year old female with a past medical history of eosinophilic esophagitis presented for nausea and epigastric pain. On admission, chest computed tomography showed diffuse thickening and inflammation of the esophagus and she was started on intravenous pantoprazole. She developed worsened epigastric pain and was subsequently found to have marked elevation in serum troponin. The troponin rose from 311 to 3,299 ng/L. Further laboratory findings included an ESR of 50 mm/hr, CRP of 16.8 mg/L, IgE of 978 kU/L, 4,810 eosinophils/microliter and a negative strongyloides antibody. Electrocardiogram showed new ST segment depressions in the anterior leads and the patient was taken for left heart catheterization showing 80% stenosis of the proximal left anterior descending artery. No stents were placed due to bleeding risk of dual antiplatelet therapy in the setting of active severe gastritis. The patient was started on aspirin and high dose corticosteroids for EGPA with cardiac involvement. Cardiac magnetic resonance imaging confirmed active myocarditis. The patient was discharged on a prednisone taper with plans for staged percutaneous coronary intervention later.

Decision-making

As an outpatient, she was started on Mepolizumab at a dose of 300 mg subcutaneously every 4 weeks. At 6-months follow-up, the patient had complete resolution of symptoms, normalized IgE, ESR, CRP and troponin levels, as well as an improved degree of obstructive coronary artery disease seen on angiogram.

Conclusion

Approximately 10% of patients with eosinophilic myocarditis die during hospitalization and 30% will not live beyond 3 years. In our patient, Mepolizumab was an effective alternative treatment for severe myocarditis, and it’s role in this disease should be confirmed by randomized trials.

Valvular Heart Disease

EOSINOPHIL MEDIATED MYOCARDITIS RESULTING IN MITRAL VALVE PAPILLARY MUSCLE AVULSION

Zurain Niaz, Mohammed Alomar, University of South Florida Morsani College of Medicine, Tampa, FL, USA

Background

Hypereosinophilic syndrome (HES) is a rare condition characterized by overproduction of eosinophils. Cardiac involvement has been reported and may include myocarditis, heart failure, intracardiac thrombi, and valvular dysfunction. However, this usually occurs in the later stage by chronic valvular regurgitation and fibrosis. We present an unusual case of hypereosinophilic myocarditis resulting in mitral valve (MV) papillary muscle avulsion.

Case

18 year old female presented for abdominal pain suspicious for intra-abdominal fluid collection. While being evaluated, a transthoracic echocardiogram was performed which showed an LVEF of 56%, severe mitral regurgitation (MR), a possible vegetation on the anterior leaflet versus a ruptured chord. A repeat limited echo showed the MV appearing echogenic with a large vegetation 1.59 x 0.94 cm on anterior leaflet. The anterior leaflet appeared flail prolapsing into the left atrium. She was emergently taken for ex-lap with bowel resection for necrotic bowel due to HES, placed on ECMO, and then underwent mitral valve replacement (MVR) with a 29mm Magna-Ease pericardial valve.

Decision-making

Bone marrow biopsy confirmed idiopathic HES. Biopsy of the native valve was indicative of eosinophilic infiltration. This established development of cardiogenic shock secondary to eosinophil-mediated myocarditis resulting in MV papillary muscle avulsion. She was placed intermittently on steroid tapers for symptomatic flares and coumadin for anticoagulation. Five months later she had recurrence of symptoms and was emergently taken again for MVR using #29 Medtronic porcine mosaic bioprosthesis for severe mitral stenosis thought to be secondary to hypereosinophilic flare. She was then placed on hydroxyurea for idiopathic HES and continued coumadin therapy.

Conclusion

Severe MR due to eosinophil-mediated destruction can be safely managed with surgical repair, anticoagulation and adequate eosinophil suppression. Additional studies need to be performed to help further guide therapy and surgical interventions due to the higher risk of thrombosis in idiopathic HES and prosthetic valves despite anticoagulation.

Heart Failure and Cardiomyopathies

BALLOON ATRIAL SEPTOSTOMY FOR LEFT VENTRICULAR UNLOADING IN AN ADULT SUPPORTED WITH VA-ECMO: A CASE OF FULMINANT MYOCARDITIS

Rebecca Smoller, Daniel E. Asemota, Craig Basman, Zachary N. Kon, Miguel Alvarez Villela, Lenox Hill Hospital, New York, NY, USA

Background

Use of left ventricular (LV) venting during peripheral veno-arterial (VA) extracorporeal membrane oxygenation (ECMO) is an area of active research. Balloon atrial septostomy (BAS) is a common venting method in pediatrics, less well described in adults.

Case

A 31-year-old man presented with fulminant myocarditis and refractory cardiogenic shock (CS) despite intra-aortic balloon pump (IABP). Support was upgraded to peripheral VA-ECMO (23Fr venous and 19Fr arterial cannulae) without intubation. 12hrs later, IABP augmentation was poor with ECMO flow of 3.6 LPM. CXR revealed pulmonary congestion. TTE showed LV distension with end-diastolic dimension (LVEDD) of 6.5cm, increased from 5.6cm.

Decision-making

LV distension is a complication of VA-ECMO that can lead to impaired LV recovery. Mechanical venting with IABP or Impella is well described but can lead to bleeding and hemolysis. BAS was selected for passive LV unloading to avoid additional devices and lower the risk of adverse events. Septostomy was performed with a 20mm compliant balloon under intracardiac echocardiography. PCWP was reduced (30 mmHg to 17 mmHg) and equalized with RAP. The procedure was uncomplicated with improvement of pulmonary congestion and reduction of LVEDD to 5.7cm within 48 hrs. ECMO was de-cannulated with LVEF recovery and no adverse events.

Conclusion

BAS may effectively treat LV distension in adults with CS on VA-ECMO. Further research is warranted to compare BAS with mechanical LV venting strategies.

Pediatric and Congenital Heart Disease

WHEN THE APPLE FALLS FARTHER FROM THE TREE: A MOTHER WITH LONG QT SYNDROME WITH A MORE SEVERELY AFFECTED FETUS

Jessica Raviv, Lauren Ferrara, Courtney Juliano, Miwa Geiger, Raghav Murthy, Veronica Fettig, Barry A. Love, Mount Sinai Medical Center, New York, NY, USA

Background

Long QT syndrome (LQTS) is an autosomal dominant condition. Patients are at-risk for Torsades de Pointes (TdP) and sudden death. More severely affected infants can have 2:1 AV block and/or ventricular tachycardia (VT). Doctors and patients often rely on the severity of disease in the affected parent to predict the course of affected offspring.

Case

A 29 year old G1P0 mother presented at 21 weeks gestation. The fetus was found to have 2:1 AV block. The mother has LQT II with a known mutation (KCNH2 C1882 G>A) and herself had 2:1 heart block in infancy requiring an epicardial pacemaker. She had a dual chamber defibrillator at age 21 for syncope and TdP.

Decision-making

Subsequent fetal echocardiograms showed episodes of VT. Obstetrics, Electrophysiology, Fetal cardiology, Genetics, Neonatology and CT surgery collaborated. The mother was treated with IV magnesium, high dose beta blocker and mexilitine, but the fetus developed incessant VT and hydrops. The fetus was delivered at 28 5/7 weeks with 2:1 heart block and nearly incessant TdP responding minimally to IV lidocaine. Temporary epicardial ventricular wires were placed at 2 hours of life and pacing suppressed VT but the infant developed intractable hypotension and died at 8 days of age. Genetic testing confirmed the same mutation as the mother with no additional LQT mutations.

Conclusion

LQTS may affect offspring more severely than the affected parent. This family will consider in-vitro fertilization and preimplant testing for future pregnancies.

Ischemic Heart Disease

SHOCK TO THE HEART: A ONE IN A MILLION ETIOLOGY OF PERSISTENT ST ELEVATION

Max Bourdillon, Francisco Fuentes, University of Texas Health Science Center Houston, Houston, TX, USA

Background

A 24 year old previously healthy male who presented following witnessed direct lightning strike complicated by prolonged cardiac arrest.

Case

On return of spontaneous circulation in the emergency department, a 12-lead electrocardiogram (ECG) demonstrated inferior ST elevation (STE) with reciprocal changes. He was hemodynamically stable and initial labs demonstrated elevated cardiac biomarkers (19,423 ng/L), mild acute kidney injury, and resolving lactic acidosis.

Decision-making

ECG changes were attributed to electrical injury associated depolarization changes and the patient was initially admitted to the burn unit. Serial cardiac enzymes were consistent with acute myocardial injury (peak troponin of 38,692 ng/L). Serial ECGs demonstrated persistent inferior lead STE. The decision was made to pursue diagnostic coronary angiography, which did not reveal obstructive coronary artery disease (CAD) (Fig).

Conclusion

Massive electrical injury can result in myocardial injury without obstructive CAD. Sudden cardiac death from direct lightning strike from ventricular parasystole is common in the setting of simultaneous depolarization of the entire myocardium. Proposed mechanisms of myocardial injury in the setting of direct lightning strike include coronary vasospasm, myocardial contusion owing to CPR or thermal injury, catecholamine release, and global hypoperfusion.

Valvular Heart Disease

AVULSED AORTIC VALVE: UNUSUAL CAUSE OF PULMONARY EDEMA POST BLUNT CHEST TRAUMA

Nikky Bardia, Mariam A. Riad, Usman Sarwar, Mustafeez Ur Rahman, Maulik Patel, Christopher Michael Malozzi, Bassam A. Omar, Department of Cardiology, University of South Alabama, Mobile, AL, USA

Background

Blunt chest trauma can present with variable intrathoracic organ involvement, including valvular dysfunction. Mitral valve chordal rupture is a more common cause of heart failure post chest trauma, while aortic valve abnormalities are rare.

Case

59-year-old male presented with a traumatic fall from a ladder. He sustained left clavicular and spinal fractures. He was hemodynamically stable and had no prior known cardiac disease. He underwent clavicle fracture repair and spinal fusion, with post-op pulmonary edema. Transthoracic echo revealed severe aortic regurgitation (AR). Transesophageal echo (TEE) revealed an avulsed right coronary cusp (Fig. 1), with resultant severe aortic insufficiency by color Doppler (Fig. 2) and by pressure half-time (Fig. 3).

Decision-making

Despite the pulmonary edema, which responded to IV diuretics, the patient remained hemodynamically stable. Given the acuity of the AR, which was anticipated to be poorly tolerated, the decision was to perform aortic valve replacement. The patient wanted to be physically active and elected to have a bioprosthetic valve to avoid long term anticoagulation. He underwent successful aortic valve replacement with a tissue prosthesis (Fig. 4).

Conclusion

Blunt chest trauma, including falling from heights, can result in valvular rupture. In the setting of new pulmonary edema, this should be suspected, and proper studies performed so that prompt medical and/or surgical therapy can be undertaken.

Electrophysiology

INADVERTENT VENTRICULAR SEPTAL DEFECT DURING CONDUCTION SYSTEM PACING DEVICE IMPLANT: A CASE REPORT

Jurgen Shtembari, Dhan Bahadur Shrestha, Pablo Salazar, Dipayon Roy, Gaurav A. Upadhyay, University of Chicago Medicine, Chicago, IL, USA, and Mount Sinai Hospital, Chicago, IL, USA

Background

Conduction system pacing (CSP) is growing in popularity as an option to provide physiological pacing for conventional pacing, and a potential alternative for CRT. There is a paucity of data regarding complications related to the intraventricular septum (IVS) associated with CSP implant.

Case

A 77-year-old male with cardiac history of nonischemic cardiomyopathy and severely reduced EF presented for an elective left-septal electroanatomic mapping and CRT implantation. After uncomplicated RV lead placement, a Medtronic 3830 Select Secure lead was used to target the left bundle branch area. After 4-6 turns, the lead was noted to penetrate to the left ventricle (see Figure, Panel A). Contrast injection confirmed interventricular communication (Panel B). The lead was retracted and subsequently fixated at the His location with an ultimate pacing threshold of 1.0 V at 0.4 ms and an impedance of 513 ohms and nonselective QRS correction.

Decision-making

The patient was monitored in the CCU with supportive treatment. TTE showed no evidence of VSD and pacing parameters were unchanged. During 2 year follow-up TTE has shown improvement of LVEF with no residual ventricular septal defect (VSD). The most recent His lead threshold was 1.75 V at 0.4 ms and impedance 285 ohms with QRS correction.

Conclusion

Iatrogenic VSD is a rare complication of CSP. In our patient this was a benign complication with no hemodynamic impact, although future data are needed to characterize overall severity and impact.

Valvular Heart Disease

PERCUTANEOUS PULMONARY VALVE IMPLANTATION IN A PATIENT WITH DILATED PULMONARY VALVE ANNULUS COMPLICATING HIGH CORONARY ARTERY COMPRESSION RISK

Yin-Jian Yang, Yun-Peng Wei, Jing Yang, Ming Yang, Fan Guo, Zhi-Cheng Jing, Peking Union Medical College Hospital, Beijing, People's Republic of China

Background

Percutaneous pulmonary valve implantation (PPVI) is challenging in patients with dilated pulmonary valve annulus complicating high coronary artery compression risk.

Case

A 51-year-old man experienced exertional dizziness in past 1 year. He had a history of patch repair of tetralogy of Fallot 30 years ago. Transthoracic Doppler echocardiography demonstrated severe pulmonary regurgitation. The multiplanar reconstruction of cardiac computed tomography angiography revealed a remarkable dilated and deformed pulmonary valve annulus with the short diameter of 27 mm and the long axis of 45 mm. The minimum distance between left main coronary artery and pulmonary trunk was only 1 mm, indicating a high coronary compression risk in PPVI.

Decision-making

A novel symmetrical dumbbell-shaped self-expandable transcatheter pulmonary valve was selected. Both the inflow and outflow portions of the device can land. A 40 mm long device with a 26 mm diameter porcine pericardium valve was landed at 1 cm inferior to pulmonary valve annulus with its inflow portion. The inflow and the valvular portions of the device did not compress surrounding structures. No residual pulmonary regurgitation nor paravalvular leak was detected. Exertional dizziness did not occur during the 1-year follow-up.

Conclusion

In patients with dilated pulmonary valve annulus complicating high coronary artery compression risk, a symmetrical dumbbell-shaped self-expandable transcatheter pulmonary valve can be used safely.

Prevention and Health Promotion

SITOSTEROLEMIA: A RARE CAUSE OF PREMATURE CORONARY ARTERY DISEASE

Juliette Logan, Todd C. Villines, University of Virginia, Charlottesville, VA, USA

Background

Sitosterolemia is an autosomal recessive disorder of lipid metabolism marked by increased plant sterol absorption via decreased biliary excretion. Clinical presentation is similar to familial hyperlipidemia (FH) but is much rarer with significant differences in management.

Case

29-year-old vegan male presented with exertional chest pain. Initial EKG displayed an anteroseptal infarct and Troponin I 2.4 ng/mL (normal <0.02 ng/mL). Exam showed xanthelasmas and a tendon xanthoma. Echocardiography demonstrated low normal systolic function with septal hypokinesis. Labs notable for LDL 230 mg/dL, TChol 290 mg/dL, TG 75 mg/dL, and HDL 39 mg/dL. Coronary angiography revealed multi vessel disease (90% LAD stenosis, 70% D1 stenosis, and severe diffuse RCA disease). He underwent three vessel coronary artery bypass. Genetic testing for congenital lipid abnormalities identified two pathogenic mutations in ABCG8, diagnostic of sitosterolemia. Sitosterol level was 365.4mg/L (normal <15mg/L). He was initiated on rosuvastatin and ezetimibe with significant improvement in his lipid profile with plans to start a bile-acid sequestrant. His sister later underwent genetic testing, also diagnostic of sitosterolemia.

Decision-making

Sitosterolemia is a genetic disorder of lipid metabolism that presents with premature coronary atherosclerosis and xanthomas. Diagnostic criteria is comprised of clinical manifestations, serum sitosterol levels, pathogenic mutations and exclusion of FH. Definitive diagnosis must include pathogenic mutations in ABCG8 or ABCG5. Once diagnosis is established, first line management is dietary restriction of sterol rich foods, traditionally considered beneficial in FH. Recommended medications are ezetimibe and bile-acid sequestrants. Statin therapy may be beneficial although response is blunted due to significant down-regulation of LDL biosynthesis.

Conclusion

Sitosterolemia is a rare cause of premature atherosclerosis that must be accurately diagnosed through genetic testing and cascade family testing. Accurate and timely diagnosis is crucial given its unique management considerations, which focus on major diet and lifestyle modifications.

Pediatric and Congenital Heart Disease

ACUTE CORONARY SYNDROME FOLLOWING SPONTANEOUS THROMBUS FORMATION IN NATIVE AORTA IN A PATIENT WITH HYPOPLASTIC LEFT HEART SYDNDROME

Esiemoghie J. Akhigbe, Ebubechukwu Ezeh, Jeffrey Harris, Jack R. Stines, Marshall University, Huntington, WV, USA

Background

Thrombus formation in the native aortic root is a rare but potentially life-threatening complication of patients with Hypoplastic left heart syndrome.

Case

A 4-year-old M with a history of hypoplastic left heart syndrome (MS/AS) and aortic coarctation status post-Norwood. He presented to the ED on account of new-onset chest pain.

Decision-making

At the ED patient was tachycardic (120bpm) with Initial EKG showing new onset PR prolongation, QRS widening, and ST segment depression in the precordial leads (Figure 1). Labs showed an elevated Troponin (4.950ng/ml). Echo showed HLHS (MS/AS) s/p Norwood operation, a large new thrombus in the neo-aortic root not seen in previous study. The thrombus measures about 8 x 11 mm. Heparin drip was immediately started and transferred to a surgery-enabled center where an emergent native aorta and left coronary thrombectomy was performed. The patient was transitioned to warfarin with thrombotic labs ordered for close outpatient follow-up.

Conclusion

After Norwood procedure, the native aortic root becomes the channel for coronary blood flow. Low flow through this conduit serves as a catalyst for thrombus formation. Since retrograde flow into the native aorta serves as the solitary source for blood flow to the coronary arteries, its blockage can lead to myocardial infarction and sudden cardiac death. Routine Echocardiograms are important in early identification of this complication, while aggressive thrombolysis, and anticoagulation are mainstay therapy.

Heart Failure and Cardiomyopathies

ARE WE CREATING A NEW POTENTIAL PROBLEM WITH PROLONGED TEMPORARY MECHANICAL SUPPORT? DEVELOPMENT OF DE NOVO ANTI-HUMAN LEUKOCYTE ANTIGEN ANTIBODIES PRODUCTION IN A PATIENT BRIDGED BY IMPELLA 5.5 DEVICE TO ORTHOTOPIC HEART TRANSPLANTATION

Hira Shakoor, Neville Maliakkal, Staci M. McKean, Johanna van Zyl, Raksha Patel, Caroline Harrison, Shelley A. Hall, Amit Alam, Baylor Scott and White Research Institute, Dallas, TX, USA

Background

Little is known regarding the development of anti-human leukocyte antigen antibodies (HLAs) with the new Impella 5.5 as a bridge to transplantation.

Case

A 52-year-old African American male with history of nonischemic dilated cardiomyopathy underwent Impella 5.5 placement as a bridge to transplantation for worsening cardiogenic shock despite dual inotropic support. Prior to the Impella insertion, the patient's -HLAs Calculated Panel Reactive Antibody (CPRA) was 0% (1A). HLAs were monitored weekly post Impella placement and patient was noted to have de novo class I and II antibodies with a CPRA of 56% at day 14. Despite not receiving any blood products the patient developed further de novo HLA antibodies with CPRA rising to 88% (1B).

Decision-making

Forty-eight days post-Impella the patient was successfully transplanted. Due to the rise in CPRA levels the team decided to use anti-thymocyte globulin 4.5mg/kg for induction. At 3 months post transplantation, the patient remains on routine immunosuppression. He has had no episodes of rejection, no hospitalizations and has not developed any donor specific antibodies (1C).

Conclusion

Similar to experience with durable left ventricular assist devices, the temporary mechanical support (tMCS) device, Impella 5.5, may result in the development of new HLAs. Further studies measuring HLAs in protracted tMCS are needed to determine the incidence and clinical significance of these antibodies and outcomes following heart transplantation.

Heart Failure and Cardiomyopathies

MIMICKING THE GREAT MIMICKER: LMNA CARDIOMYOPATHY PRESENTING AS ISOLATED CARDIAC SARCOIDOSIS

Karen Flores, Ravi Karra, Albert Y. Sun, Han W. Kim, Duke University Hospital, Durham, NC, USA

Background

Arrhythmogenic cardiomyopathies (ACM) remain a diagnostic dilemma and this case illustrates the diagnostic value of cMRI and PET. We present a case of LMNA cardiomyopathy mimicking isolated cardiac sarcoidosis (iCS).

Case

A 44-year-old man with no prior medical history presented with exertional dyspnea and bradycardia in the setting of high-degree atrioventricular block. An echocardiogram showed an ejection fraction of 60% and normal left ventricular size and wall thickness. A Dobutamine stress test showed no wall motion abnormalities. He underwent dual-chamber pacemaker placement. Years later, he presented with monomorphic ventricular tachycardia (VT) and received a CRT-D. A cMRI showed multiple areas of non-CAD scar and a cardiac PET suggested active inflammation localized to similar regions as the LGE on cMRI. An endomyocardial biopsy was not performed due to scar location. Given the history and imaging findings, he was treated for presumed iCS with Prednisone and Methotrexate. He had VT recurrence requiring antiarrhythmics, VT ablation, and transition to Infliximab. Given the persistence of arrhythmias, an alternative diagnosis was pursued. A follow-up cMRI suggested a pattern not entirely consistent with iCS and rather consistent with LMNA cardiomyopathy. Genetic testing was positive for a likely pathogenic LMNA variant (c.639+1G>T). Immunosuppression was discontinued after shared decision making.

Decision-making

A multidisciplinary approach, sequential imaging, and genetic testing were used to diagnose LMNA cardiomyopathy. LMNA is one of the genes associated with ACM and the presence of a likely pathogenic variant in this case prompted cascade screening. Inflammation is thought to be part of ACM’s pathogenesis, but it is unclear if immunosuppression in addition to concurrent antiarrhythmics and VT ablation is beneficial.

Conclusion

This case highlights the importance of keeping a high index of suspicion for ACM among patients with early conduction disease, arrhythmias, LGE on cMRI, and inflammation on PET. Patterns of LGE on cMRI can be used to identify ACM and guide genetic testing. Further research is needed to understand the role of immunosuppression in ACM.

Heart Failure and Cardiomyopathies

CARDIAC LYMPHOMA MANAGED WITH CAR-T THERAPY

Hilda M Gonzalez Bonilla, Choon-Ta Ng, Jennifer Gile, Ian Chang, Grace Lin, Joerg Herrmann, Mayo Clinic, Rochester, MN, USA

Background

CAR-T therapy in the setting of cardiac lymphoma is challenging due to concerns of grave complications such as ventricular rupture and cardiomyopathy. Herein we report on the safety and feasibility of CAR T in a patient with cardiac lymphoma and multifocal cardiac involvement

Case

A 38-year-old man with refractory diffuse large B cell lymphoma despite chemotherapy and stem cell transplant presented for further therapy options. His staging FDG-PET raised concerns for cardiac involvement, confirmed by cardiac MRI. Biomarker profile showed cardiac troponin T elevation and ECG diffuse T wave inversions. CT chest showed normal coronaries

Decision-making

CAR-T therapy for cardiac lymphoma carries risk of cardiac perforation. Treatment associated cytokine release syndrome (CRS) can lead to cardiomyopathy and arrhythmias. Our patient underwent CAR-T and developed mild grade 1 CRS requiring intravenous fluids for hypotension. LVEF was unchanged on repeat TTE. Repeat FDG-PET scan 1 month later showed near complete resolution of FDG avidity in the heart. ECG showed resolution of T wave inversions

Conclusion

Lymphomatous involvement of the heart is rare, but should be looked for in case of cardiac troponin elevation and ECG abnormalities with cardiac MRI as the imaging technique of choice. Carefully selected patients with cardiac lymphoma can undergo CAR T therapy without life-threatening cardiac complications and compromise of cancer therapy outcomes

Heart Failure and Cardiomyopathies

SAVING THE KIDNEYS—SUCCESSFUL USE OF THE AORTIX DEVICE IN A PATIENT WITH LOW CARDIAC OUTPUT STATE AND SEVERE CARDIORENAL SYNDROME AWAITING HEART-KIDNEY TRANSPLANTATION: A CASE REPORT

Antonio Lewis, Dipan Uppal, Alejandro Sanchez, Mileydis Alonso, Malcolm L. McDonald, Yelenis Seijo De Armas, Radhika Neicheril, Camila De Carvalho Teixeira, Emad Hakemi, Nicolas Ariel Brozzi, Steven Minear, Jerry D. Estep, David A. Baran, Mauricio Velez, David Snipelisky, Cleveland Clinic Florida, Weston, FL, USA

Background

Cardiorenal syndrome (CRS) increases morbidity in Heart Failure (HF)

Case

56 yr old male with history of radiation-induced restrictive cardiomyopathy presented in decompensated HF. Unfortunately, despite inotropes the patient responded poorly to high doses of intravenous diuretics plus metolazone. He developed acute kidney injury (Creatinine (Cr) 2,9g/dL). His cardiac filling pressures and azotemia continued to increase and decision was made to consider listing for combined heart-kidney transplantation (CHKT)

Decision-making

Due to worsening CRS, the novel Aortix® device, a percutaneously inserted microaxial pump positioned in the descending aorta above the renal arteries with the goal to increase its perfusion, was implanted as part of the ongoing pivotal trial (Fig 1a). Inotropes & diuretics were continued at same dose and the patient demonstrated adequate response with increased urine output and improved Cr back to baseline. There was near-normalization of filling pressures & the device was removed at day 5 (Fig 1b). Considering the marked response, decision was made to pursue single organ heart transplantation rather than CHKT

Conclusion

We describe the first report of successful use of the Aortix® device in a patient with severe CRS in which CHKT was initially considered. This case demonstrates marked improvement in renal function, presumably secondary to improved renal artery flow from the Aortix® device, with the ability to de-escalate to a single organ transplant option

Heart Failure and Cardiomyopathies

MONKEYPOX MYOCARDITIS

Jared Krainin, Chad Harris, William J. Bugni, Stephen W. Mester, Doniya Milani, HCA Florida Brandon Hospital, Tampa, FL, USA

Background

Human Monkeypox is a zoonotic orthopoxvirus previously endemic to Western and Central Africa. An increased incidence of the virus worldwide has led to identification of novel clinical manifestations. This orthopoxvirus is known to cause viral prodrome with ulcerative maculopapular eruptions on the epithelium of the epidermis and mucosa. Cardiac sequelae of the virus has yet to be firmly established, however a case of acute myocarditis post infection has now been reported in Portugal. We present another case of myocarditis after confirmed Monkeypox infection.

Case

A thirty-one year old man with a previous medical history of HIV on antiretroviral therapy presented with chest discomfort and progressive dyspnea on exertion. Two weeks prior to symptom onset he was diagnosed with Monkeypox virus confirmed by PCR of the cutaneous lesions. Laboratory workup was remarkable for undetectable HIV viral load and high-sensitivity troponin I assay of 405 ng per liter. ECG showed normal sinus rhythm. There was preserved left and right ventricular systolic function on transthoracic echocardiogram. Cardiac MRI demonstrated abnormal fast spin-echo T2 signal of the myocardium located in the apex and infero-apical wall consistent with focal myocarditis.

Decision-making

The patient was treated with tecovirimat and supportive care with eventual resolution of clinical symptoms. Tecovirimat is now being used first line as treatment of Monkeypox virus for adults with severe disease or underlying medical conditions. There is evidence that tecovirimat decreases mortality after infection with orthopoxviruses in animal studies.

Conclusion

This case report demonstrates further evidence that Monkeypox infection, like other viral illnesses, may result in acute myocarditis.

Interventional and Structural

PERCUTANEOUS RETRIEVAL OF AN EMBOLIZED MITRACLIP DEVICE CAUSING ACUTE ST-SEGMENT ELEVATION MI

Charlie Lin, Leon Zhou, Shravan Rao, Anilkumar O. Mehra, Aron Bender, University of California, Los Angeles, Los Angeles, CA, USA, Keck Medicine of USC, Los Angeles, CA, USA

Background

MitraClip (Abbott, Chicago, IL, USA) device embolization is a rare phenomenon, typically resulting in surgical retrieval or need for vascular repair.

Case

A 76-year-old male with a history of severe, symptomatic mitral regurgitation and normal coronary anatomy underwent percutaneous repair via deployment of two MitraClip devices, after declining surgery. Two weeks later, he presented to the emergency department with acute, severe chest pain and was found to have an inferior STEMI on ECG, complicated by development of cardiogenic shock.

Decision-making

Emergent coronary angiography revealed that one of the two MitraClip devices had embolized and occluded the ostium of the right coronary artery. Percutaneous retrieval was attempted due to concurrent cardiogenic shock, with surgical services remaining on standby. Femoral arterial access allowed for snaring and relocation of the embolized device into the abdominal aorta, using a 12 - 20 mm EN Snare Endovascular Snare System (Merit Medical, South Jordan, UT). There was immediate improvement in his hemodynamic status and resolution of the ST-segment elevation. Quantitative coronary angiography was then used to approximate the diameter of the device and to appropriately choose the size of the femoral arterial sheath intended for device externalization. As this diameter was estimated to be 5.5 mm, a 20-French GORE DrySeal Flex Introducer Sheath (Gore Medical, Flagstaff, AZ) was secured in the contralateral femoral artery. Following device handoff to an Amplatz Goose Neck Snare (Medtronic, Minneapolis, MN) and re-introduction of the EN Snare through the 20-French sheath, the embolized device was successfully retrieved and externalized.

Conclusion

While embolization of MitraClip devices is a rare complication, delayed presentation and embolization occurring after the immediate implantation in the interventional laboratory is rarer still. Of the published cases available for review, delayed embolization invariably resulted in the need for surgical retrieval. We present the first case of an embolized MitraClip retrieved via percutaneous access.

Heart Failure and Cardiomyopathies

GIANT CELL MYOCARDITIS AFTER MOGAMULIZUMAB

Dylan P. Marshall, Irina Sobol, Steven Horwitz, Udhay Krishnan, Jeffrey Abrams, Chen Zhang, Syed S. Mahmood, Division of Cardiology, Department of Medicine, Weill Cornell Medicine, New York, NY, USA, and Lymphoma Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY, USA

Background

Mogamulizumab is a monoclonal antibody against C-C chemokine receptor 4 (CCR4). We present the first case of biopsy-proven giant cell myocarditis (GCM) after mogamulizumab.

Case

An 81-year-old female treated with mogamulizumab for Sezary syndrome, initiated six months prior, came to our emergency department with dyspnea. She was cool to touch with elevated lactate, creatinine and liver enzymes. High sensitivity troponin I was 14 ng/dL (normal ≤40 ng/dL). Swan-Ganz catheter revealed CI of 1.2 L/min/m 2 with elevated filling pressures. Twelve days prior, she was at another hospital with ventricular tachycardia (VT). Coronary artery angiogram showed mild disease. Cardiac MRI revealed a newly reduced LVEF of 32%, inferior hypokinesis and subendocardial scar.

Decision-making

Intra-arterial balloon pump (IABP) was placed after minimal response to diuresis. VT burden improved but CI remained low. Upon failing IABP wean, endomyocardial biopsy was performed, showing multinucleated giant cells (Figure 1), consistent with GCM; immunostaining revealed histiocytic predominance. Unfortunately, despite pulse-dose steroids her CI remained low and IABP wean continued to be unsuccessful. As she was not a candidate for advanced therapies given her cancer prognosis, she requested IABP removal and passed soon after.

Conclusion

This is the first published case of GCM after CCR4 inhibitor therapy. It highlights the need for investigation of the association between CCR4 inhibitors and myocarditis.

Heart Failure and Cardiomyopathies

NOT YOUR COMMON CAUSE OF PALPITATIONS: CARDIAC PARAGANGLIOMA IN YOUNG ADULT

Erica G. Otero-Cardenas, William Redondo, Andres Cordova Toro, Hector Nunez Medina, Maria Alejandra Rodriguez-Santiago, Gisela D. Puig-Carrion, Hector Luis Banchs Pieretti, University of Puerto Rico, San Juan, Puerto Rico, USA

Background

Myxomas are the most common primary cardiac neoplasm arising from the left atrium (LA). However, other etiologies should be considered.

Case

A 20 years old man presented with palpitations. He was diagnosed with supraventricular tachycardia and managed with beta blockers and amiodarone. He denied chest pain, shortness of breath, edema, night sweats, or weight loss. His physical exam was remarkable for a systolic murmur. Echocardiogram (Fig 1-2) showed a severely enlarged LA with a large intracavitary mass, near complete LA obliteration, moderate mitral regurgitation, and low normal LV systolic function. CT Angiogram showed a large, vascular and avidly enhancing LA mass with central necrosis (10.5x11.7x10.9 cm), mass effect on pulmonary arterial trunk bifurcation, patent pulmonary veins. 24 hours Metanephrines urine collection was negative.

Decision-making

Cardiothoracic surgery attempted to remove mass unsuccessfully. Intraoperative biopsy consistent with cardiac paraganglioma. PET/CT scan confirmed absence of metastatic disease. He underwent orthotopic heart transplantation.

Conclusion

Cardiac paragangliomas are exceptionally rare extra-adrenal neuroendocrine tumors arising from chromaffin cells of the autonomic paraganglia. They may present with palpitations, hemoptysis or heart failure symptoms. CT and/or MRI can provide further characterization for diagnosis. Cardiac transplantation should be available as a therapeutic option when resection is high risk.

Pediatric and Congenital Heart Disease

EXCEEDINGLY RARE LARGE CARDIAC PARAGANGLIOMA IN A PEDIATRIC PATIENT

Natalia Betancourt-Guzman, Gabriela L. Navarro Gallardo, Joao M. Cantellops-Cueli, Benjamin J. Smith, Natasha Gonzalez, University of Puerto Rico, San Juan, Puerto Rico, USA, and University of Minnesota, Minneapolis, MN, USA

Background

Paragangliomas (PGLs) are extremely rare neoplasms in the pediatric population. Cardiac PGLs present a unique management challenge. It is essential to evaluate the precise location of the tumor and its extension since compression of adjacent structures may result in ischemia, arrhythmias, cardiac dysfunction or compromised cardiac output. Surgical resection is the preferred approach for most PGLs, though caution is needed if catecholamine-releasing.

Case

A 19-year-old male with no past medical history presented with 6 days of intermittent palpitations. He denied fever, chills, chest pain, shortness of breath, dizziness, exercise intolerance, diaphoresis, and weight loss. No known toxic exposure, drug, or alcohol use. He was normotensive with adequate peripheral saturations. Clinically, well appearing and in no acute distress. He had a hyperdynamic precordium, 3/6 systolic murmur along the left sternal border and 2/6 holosystolic murmur at the apex. No adventitious lung sounds or hepatomegaly. Telemetry showed supraventricular tachycardia at a rate of 170 bpm which was successfully treated with amiodarone. Echocardiogram showed a left atrial mass measuring 10 x 8 cm with moderate mitral regurgitation, moderate pericardial effusion, and preserved function.

Decision-making

Given the size of the mass, tachycardia, and risk of compromise to cardiac output he was referred for surgical resection. Surgery was complicated by high vascularity and extension of the mass beyond the left atrium and into the mediastinum, making it unresectable. Biopsy confirmed the diagnosis of a Cardiac PGL. After ruling out metastasis, he was listed for and received a heart transplant.

Conclusion

This is an exceedingly rare case of a cardiac PGL in a pediatric patient. It highlights that presentation is variable and symptoms can be subtle which adds to the diagnostic challenge. A multidisciplinary team was key to his diagnosis and management.

Heart Failure and Cardiomyopathies

CHAGAS REACTIVATION DURING DESENSITIZATION FOR ORTHOTOPIC HEART TRANSPLANTATION

Sthuthi David, Negeen Shahandeh, Darko Vucicevic, Megan Y. Kamath, UCLA Health, Los Angeles, CA, USA

Background

Patients with Chagas cardiomyopathy who undergo orthotopic heart transplantation (OHT) carry a risk of Chagas reactivation post operatively due to the heavy immunosuppression. However, there is a paucity of data on the risk of reactivation in those undergoing desensitization prior to OHT. We describe a case in which a patient with a left ventricular assist device (LVAD) experienced Chagas reactivation during desensitization.

Case

A 50-year-old woman with a Heartware LVAD for Chagas cardiomyopathy presented with fevers and diarrhea. One week prior, she had received daratumumab for desensitization. She was highly sensitized despite prior treatments with plasmapheresis, intravenous immunoglobulin, rituximab, bortezomib, and obinutuzumab. Empiric antibiotics were started, but a Karius test detected Trypanosoma cruzi (T. cruzi), confirming Chagas reactivation.

Decision-making

She was treated with benznidazole. However, one month into treatment, she developed aphthous ulcers and a palmar rash which were attributed to benznidazole hypersensitivity. Fortunately, repeat T. cruzi polymerase chain reaction (PCR) was negative, therefore treatment was stopped and the T. cruzi PCR was monitored every two weeks. Due to high risk for recurrent Chagas reactivation, further desensitization has been deferred, and she is no longer an acceptable transplant candidate. Interestingly, she had previously reported several months of intermittent left eye swelling which was initially attributed to an allergic reaction. Although unilateral periorbital swelling is associated with initial Chagas inoculation, it is a rare finding in Chagas reactivation, which is why suspicion for this diagnosis did not arise until she developed systemic signs.

Conclusion

Chagas reactivation is not a phenomenon contained to the post OHT period and the diagnosis should be considered in patients undergoing desensitization prior to OHT. Reactivation has significant consequences and may prohibit transplant candidacy. Further research is needed to better understand which desensitization regimens carry the greatest risk for Chagas reactivation.

Multimodality Imaging

TIGHT SQUEEZE: A CASE OF CARDIAC EXTENSIVE LIPOMATOSIS WITH ATRIAL INFILTRATION AND SUPERIOR VENA CAVA COMPRESSION

Fadi Azar, Kristin Boulier, Cameron Hassani, Negeen Shahandeh, Daniel Cruz, Jamil A. Aboulhosn, Michael C. Fishbein, Murray H. Kwon, Eric H. Yang, Kirk Kerkorian School of Medicine at UNLV, Las Vegas, NV, USA, and David Geffen School of Medicine at UCLA, Los Angeles, CA, USA

Background

Primary cardiac tumors are rare and can cause diverse symptoms depending on location. We present a case of a patient presenting with dyspnea secondary to superior vena cava (SVC) syndrome caused by cardiac lipomatosis.

Case

A 66-year-old female with a history of asthma, obesity and obstructive sleep apnea presented with a 1 year history of progressive dyspnea. Her symptoms did not improve with pulmonary treatments. A computed tomography scan of the chest revealed a large intracardiac mass centered in the interatrial septum and extending to occlude the SVC.

Decision-making

Cardiac magnetic resonance imaging suggested lipomatous cardiac hypertrophy, but liposarcoma could not be excluded. After multidisciplinary discussions, intracardiac echo guided biopsy was performed demonstrating myocyte hypertrophy and fibrosis. With malignancy being less likely, surgical resection was attempted. Intraoperatively, a large infiltrative mass with involvement of the right atrium, interatrial septum and SVC was revealed. Resection was performed with reconstruction of both atria and the SVC. Pathologic exam showed mature adipose tissue infiltrating the myocardium, consistent with lipomatosis (Figure).

Conclusion

Extensive cardiac lipomatosis, a separate entity from lipoma due to infiltration of the myocardium, is a rare phenomenon in the current literature. Despite multiple cardiac imaging modalities, tissue diagnosis was essential for the proper diagnosis and treatment of this pathology.

Heart Failure and Cardiomyopathies

A CASE OF DISAPPEARING AMYLOID

Uzair Mahmood, Diwakar Jain, Stephen Pan, Westchester Medical Center, Valhalla, NY, USA

Background

Tc-PYP scanning is highly sensitive and specific for diagnosing Transthyretin cardiac amyloidosis (TTR-CA). However, the effect of Tafamidis on Tc-PYP scans is unknown.

Case

A 79-year-old female with a history of heart failure with preserved ejection fraction, type 2 diabetes, and paroxysmal atrial fibrillation was referred to us in March 2022. In 2019, she had new-onset atrial fibrillation; its workup included a Tc-PYP scan consistent with TTR-CA (fig. 1A). Since then, she had been on Tafamidis but continued to have heart failure exacerbations. Genetic testing at our center revealed a pathogenic variant in MYH7 consistent with hypertrophic cardiomyopathy. No variant in the TTR gene was identified.

Decision-making

A repeat Tc-PYP scan was negative for TTR-CA 2 hours after injection of radiotracer (fig. 1B). Review of prior PYP images confirmed intense myocardial enhancement. We performed an endomyocardial biopsy. Congo red staining was positive, and mass spectrometry identified it as wild-type TTR amyloid. Myocyte hypertrophy and sarcomeric disarray were also present suggestive of co-existing hypertrophic cardiomyopathy.

Conclusion

This is the first report of a negative Tc-PYP scan after initial strong positivity following Tafamidis therapy for approximately 2.5 years. Whether this suggests a potential interaction between Tafamidis and Tc-PYP resulting in a false negative scan, or a quantitative reduction in TTR-CA disease burden with Tafamidis therapy remains to be elucidated.

Heart Failure and Cardiomyopathies

FALSE POSITIVE PYP SCINTIGRAPHY IN SYSTEMIC SCLEROSIS AND MEDIASTINAL RADIATION

George O. Ajene, Danny Diab, Christian A. Inchaustegui, Ajith Nair, Baylor College of Medicine, Houston, TX, USA

Background

Technetium-99m pyrophosphate (PYP) scintigraphy is critical in diagnosing ATTR cardiomyopathy (ATTR-CA); however, specificity for ATTR-CA declines in the setting of confounding infiltrative processes. We present two novel instances of false-positive PYP scans in the background of cardiac fibrosis due to Systemic Sclerosis (SS) and mediastinal radiation therapy (MRT).

Case

A 59-year-old female with bilateral carpal tunnel syndrome and SS presented with chronic dyspnea and lower extremity edema. Echocardiography revealed pulmonary peak systolic pressure of 75 mmHg with a preserved ejection fraction. Ischemic workup was negative and PYP scintigraphy was consistent with ATTR-CA. Genetic testing for hereditary transthyretin (hATTR) was negative, and subsequent endomyocardial biopsy showed only myocardial fibrosis on trichrome stain without amyloid deposition on Congo Red stain. Case 2: A 61-year-old female with Hodgkin’s lymphoma treated with mediastinal radiation and severe aortic stenosis treated with transcatheter aortic valve replacement presented with exertional dyspnea and lower extremity edema. Echocardiography revealed an ejection fraction of 45%, LV wall thickness of 15 mm, normal valve function and abnormal global longitudinal strain. PYP scintigraphy revealed grade 2 myocardial tracer uptake concerning for ATTR-CA. Genetic testing for hATTR was negative. Endomyocardial biopsy showed mild myocardial fibrosis on trichrome stain, and Congo Red staining was negative for amyloid.

Decision-making

PYP scintigraphy has proven highly sensitive and specific for ATTR-CA in the absence of light-chain amyloidosis. However, other infiltrative processes such as myocardial fibrosis, resulting from SS or MRT, may also produce false-positive PYP scintigraphy results. Further study is required to determine the extent to which fibrosis deposition reduces the specificity of PYP scans, but cardiac biopsy can be useful in confirming or ruling out amyloid deposition.

Conclusion

Although cardiac scintigraphy has emerged as a cornerstone of the noninvasive diagnosis of ATTR-CM, false positives can occur in patients with SS and MRT.

Heart Failure and Cardiomyopathies

TAKOTSUBO CARDIOMYOPATHY IN A RECENTLY TRANSPLANTED HEART

Brian Mitchell, Nimesh Kirit Patel, Jayanthi N. Koneru, Virginia Commonwealth University Medical Center, Richmond, VA, USA

Background

The pathophysiologic mechanism of Takotsubo syndrome (TTS) is incompletely understood but is most likely due to activation of pre-synaptic post-ganglionic noradrenergic neurons and stress-related neuropeptide release leading to myocardial dysfunction. Heart transplantation (OHT) results in extrinsic cardiac denervation due to post-ganglionic parasympathetic and sympathetic nerve fiber disruption, then to myocardial afferent denervation and blunting of vasoregulatory response. Reinnervation occurs in a time-dependent fashion.

Case

A 62-year-old female with a history of transthyretin amyloidosis and heart transplantation 4 years earlier presented with a one-day history of worsening dyspnea and peripheral edema and was found with elevated cardiac biomarkers. She reported full compliance with her immunosuppressive regimen. Echocardiography (TTE) revealed a left ventricular ejection fraction (LVEF) of 20%, along with akinesis of the entire apex of the left ventricle. IV steroids were initiated for possible rejection. Urgent coronary angiography was negative for significant coronary artery stenosis. Right heart catheterization with endomyocardial biopsy was completed.

Decision-making

Pathology from endomyocardial biopsy and HLA laboratory results were negative for rejection. After obtaining a more detailed history, the patient reported multiple recent intense life stressors. Given lack of obstructive coronary artery disease or rejection in the setting of classic TTE findings, the patient was diagnosed with TTS. Steroids were discontinued and her immunosuppressive medications were resumed. Repeat TTE one week later showed improved LVEF to 45-50%.

Conclusion

We report an exceedingly rare case of TTS following OHT, and the earliest known presentation of emotionally triggered TTS following OHT. While the mechanism is unclear, we speculate that sympathetic, but not sensory afferent, re-innervation may have occurred in this patient. This case demonstrates the need to recognize TTS as a potential cause of acute onset heart failure in patients post OHT, and the importance of utilizing a comprehensive diagnostic algorithm in this population.

Heart Failure and Cardiomyopathies

INTENTIONAL TAXUS BACCATA INGESTION PRESENTING WITH CARDIOGENIC SHOCK AND VENTRICULAR TACHYCARDIA

Ramy Mando, Olivia Allen, Matthew Hanna, Richard Bloomingdale, Ilana B. Kutinsky, Michael J. Gallagher, David E. Haines, Beaumont Health System, Royal Oak, MI, USA

Background

The prevalence of anxiety and depression has increased following the COVID pandemic. Young women are disproportionally at risk of suicidal behaviors. Ingestion of Taxus baccata (English Yew) may lead to cardiotoxicity and death. Intoxication is known to be resistant to standard treatments with no effective antidotes.

Case

A 20-year-old female with a history of major depressive disorder presented to our emergency department unresponsive in pulseless ventricular tachycardia (VT) (figure 1A). She underwent CPR and achieved ROSC, however, was persistently hypotensive despite multiple pressors and was subsequently placed on VA ECMO. Review of a home journal revealed a plan to ingest 50 grams of Yew with suicidal intent.

Decision-making

Taxoids have been reported to have similar properties to digoxin and therefore digoxin-specific FAB antibodies were administered. She was also started on amiodarone and lidocaine for management of VT. After 3 hours she converted into sinus rhythm and had no further episodes of VT. Her clinical course was complicated by severe LV dysfunction and dilation while on VA-ECMO necessitating placement of an LV Impella. By day three, all mechanical support was weaned off and she had normalization of her EF. Patient was discharged to an inpatient psychiatry facility on day 7 of hospitalization.

Conclusion

Ingestion of English Yew with suicidal intent is rare but may cause cardiogenic shock and VT requiring aggressive hemodynamic support until the toxin is metabolized.

Heart Failure and Cardiomyopathies

PREGNANT, ARE YOU SURE? BROADENING THE DIFFERENTIAL FOR CHANGES IN ALLOSURE VALUES

Marwah Shahid, Megan Y. Kamath, University of California Los Angeles, Los Angeles, CA, USA

Background

Use of AlloSure levels is contraindicated during pregnancy due to the confounding measurement of cell-free DNA from two genomes, maternal and fetal. However, a sudden rise in AlloSure levels in a woman of child-bearing age should consist of a broad differential, including unplanned pregnancy.

Case

A thirty-one-year-old-female status-post orthotopic heart transplant nine years prior for nonischemic cardiomyopathy presented with elevated AlloSure levels and missed menstrual periods. The patient was found to be pregnant at eight weeks gestation. Her Allosure level at this time was noted to be 0.14%. Her level remained elevated throughout pregnancy and peaked at 1.3%. Two days after delivery, her level decreased to less than the threshold for detection.

Decision-making

While AlloSure has not been validated for use in pregnant transplant patients, trending lab values may be clinically useful. A sudden rise in Allosure values in a woman of childbearing age should raise the suspicion of a possible pregnancy. However, determining trends may help determine the possibility of and complications of pregnancy.

Conclusion

An increase in donor derived cell free DNA is suggestive of active rejection. However, other factors such as unwashed blood transfusions, recent biopsies, time since transplant and pregnancy should all be considered as possible causes of an elevated AlloSure level. In the latter case, an elevated Allosure level could be the first sign of an unplanned pregnancy.

Heart Failure and Cardiomyopathies

TRANSCATHETER TRICUSPID VALVE REPLACEMENT IN A HEART TRANSPLANT RECIPIENT

Aleesha Shaik, Negeen Shahandeh, Jamil A. Aboulhosn, Megan Y. Kamath, University of California-Los Angeles, Los Angeles, CA, USA

Background

Valve disease poses unique surgical challenges following heart transplantation. We present a complex case of a heart transplant recipient with a bioprosthetic tricuspid valve (TV) who was found to have severe prosthetic TV stenosis.

Case

A 59-year-old man with a history of heart transplantation in 2007 for rheumatic heart disease presented with recurrent abdominal pain and dyspnea. He had previously undergone minimally invasive bioprosthetic TV replacement with a 27 mm Carpentier-Edwards Perimount TV 5 months post-transplant for severe TV regurgitation due to a flail TV leaflet following an endomyocardial biopsy. His physical exam and echocardiogram were consistent with right heart failure due to severe prosthetic TV stenosis. Endomyocardial biopsy was negative for rejection.

Decision-making

Due to multiple prior sternotomies and concern that he may soon need another heart transplant, surgical TV replacement was deemed high risk. He underwent transcatheter TV replacement (TTVR) with a 29 mm Sapien S3 valve using intracardiac echocardiographic guidance. Following successful TTVR, his cardiac output doubled and the mean diastolic gradient decreased from 9.4 to 1.7 mmHg (Figure 1). His symptoms have now resolved.

Conclusion

Valve disease in heart transplant recipients requires an individualized approach. Transcatheter valve replacement and repair are alternatives to high risk open heart surgery and can result in resolution of heart failure, as in this case.

Heart Failure and Cardiomyopathies

ACCELERATED MITRAL ANNULAR CALCIFICATION (MAC) IN PATIENTS WITH FABRY’S DISEASE (FD)

Simrat Kaur, Nicholas G. Smedira, Wael A. Jaber, Angelika Erwin, Amy Shealy, E. Rene Rodriguez, Milind Y. Desai, Cleveland Clinic Foundation, Cleveland, OH, USA

Background

We present two male patients with FD presenting with left ventricular outflow tract obstruction (LVOTO) as hypertrophic cardiomyopathy in sixth decade of life.

Case

Both patients were noted to have significant LVOTO, aortic and mitral valvular disease and severe MAC. Patient A also had evidence of aortic root dilatation and coronary artery pruning due to intimal hyperplasia. Significant renal disease was absent in both patients. Both patients underwent septal myectomy, debridement of MAC, AVR with #25 CE valve and left atrial appendage closure. In addition Patient A underwent chordal sparing MVR with # 29 Biocor valve and ascending aortic aneurysm replacement. Histopathological assessment showed disarray with focal vacuolization.

Decision-making

Diagnosis of FD was confirmed by enzymatic testing in both patients. Five years post-surgery patient B developed recurrent MAC resulting in moderately severe mitral stenosis and regurgitation with a mean gradient of 10 mmHg, area of 1.90 cm2 by planimetry and pressure half time of 131 seconds requiring redo sternotomy and MVR with #27 St. Jude Mechanical valve.

Conclusion

Accelerated MAC in FD can be attributed to vacuolization of cells from glycolipid deposition and increased deposition of extracellular matrix leading to early interstitial fibrosis followed by late cell replacement fibrosis. Renal involvement in Fabry disease affecting calcium phosphorus metabolism also contributes to mitral annular calcification in these patients.

Pediatric and Congenital Heart Disease

ACUTE MASSIVE THROMBUS FOLLOWING FONTAN PROCEDURE

Alexandria Barrett, Amar Ingravera, Joshua Wong, Waseem J. Cossor, Tarek S. Husayni, Sunthorn Muangmingsuk, Eleanor Tripp Ross, Melissa Nater, Gina M. Golem, Aran Lim, Kimberly Radowicz, Sandra McIntyre, Diane Ward, Narutoshi Hibino, Luca Vricella, Chawki El-Zein, Kristen Nelson McMillan, Advocate Children's Hospital, Oak Lawn, IL, USA, and University of Chicago, Chicago, IL, USA

Background

Patients with hypoplastic left heart syndrome (HLHS) undergo a Fontan procedure as part of single ventricle surgical palliation. Post-Fontan, sluggish blood flow and an imbalance in coagulant factor proteins may predispose to thrombus formation. Other risk factors may include chylothorax as well as acute and chronic inflammation. Currently, there is no standardized surveillance strategy to detect thrombus in Fontan patients.

Case

A 34-month old male with HLHS underwent an extracardiac non-fenestrated Fontan complicated by chylothorax treated with 5 days of IV steroids and diuretics. He was on therapeutic aspirin. After progressive worsening of right pleural effusion, a chest tube was placed three weeks post-Fontan with continued chylous output. Stool alpha 1 antitrypsin was negative.

Decision-making

Given persistent chylothorax, a repeat echocardiogram was performed revealing a large mass in the Fontan circuit less than one month post-op. Cardiac CT showed occlusive thrombus filling the entirety of the Fontan conduit extending into hepatic veins and bilateral pulmonary arteries. He underwent extensive surgical thrombectomy and Fontan conduit revision. Hypercoagulable work-up revealed elevated factor 8 and von Willebrand factor activity which persisted more than one month post-op. Patient’s history was also significant for COVID-19 infection 6 months prior. He was initially anticoagulated with bivalirudin with tirofiban initiated for antiplatelet therapy. He was ultimately transitioned to rivaroxaban, pentoxifylline and aspirin with chylothorax resolution over one month without thrombus recurrence.

Conclusion

Development of risk stratification tools to identify patients at higher risk for thrombi formation post-Fontan may facilitate patient selection for more aggressive anticoagulation. Consideration of elevated factor 8 as well as persistent or recurrent chylothorax may be beneficial, as increased thrombosis risk has been reported for both conditions in Fontan patients.

Interventional and Structural

ANTIBODIES AGAINST THE ANTIDOTE: MANAGEMENT OF AN ACUTE STEMI IN A PATIENT WITH HEPARIN-INDUCED THROMBOCYTOPENIA (HIT)

Krina Patel, Akshee Batra, Tyler Wark, Joshua D. Zimmer, Carrie Mahurin, Magdalena A. Zeglin-Sawczuk, University of Vermont Medical School, Burlington, VT, USA

Background

Acute stent thrombosis is reported in 0.5-0.7% of cases within the first 24 hours after PCI. While typical factors increasing the risk of acute thrombosis include stent under-expansion, patient factors such as autoimmune HIT also need to be considered.

Case

We present a 57-year-old-male who presented with acute chest pain; ECG showed 1-2 mm ST segment elevation in leads I, aVL, V4 and V5. He was hypoxic and hypotensive requiring mechanical ventilation and vasopressors. He was given 324 mg aspirin and started on an unfractionated heparin infusion. He underwent an Impella-assisted PCI with three stents from the mid-LM artery to mid-LAD artery. Post-PCI intravascular ultrasound (IVUS) images confirmed adequate stent expansion. He was discharged on aspirin and clopidogrel. 6 days after his initial PCI, he presented with ST segment elevations in leads V3-V4. Angiography showed acute thrombosis at the proximal edge of the mid-LAD stent that was treated with balloon angioplasty. Labs revealed platelets of 50,000 and troponin-I of 0.270. HIT was suspected and the patient was started on a bivalirudin infusion. HIT was confirmed with a positive antibody against platelet factor 4. Despite remaining on a bivalirudin infusion, 3 days from his second PCI, patient developed acute MI and angiography showed 100% thrombosis of his mid-LAD requiring stenting.

Decision-making

Due to multiple MIs with appropriate PCI and treatment for HIT, therapy was switched to an argatroban infusion. His HIT remained refractory complicated by a left ventricular thrombus, multiple segmental pulmonary emboli and disseminated intravascular coagulation. Per hematology recommendations, he was started on methylprednisolone 250 mg every 6 hours for 48 hours, intravenous immunoglobulin 1g/kg infusion over 24 hours and rituximab. His platelets improved to 112,000 and he was discharged home.

Conclusion

This case demonstrates recurrent MIs from autoimmune HIT and highlights the importance of high clinical suspicion for HIT in the setting of acute stent thrombosis when IVUS images demonstrate appropriate stent expansion. It also reveals the importance of effective interdisciplinary management of complex patients.

Pediatric and Congenital Heart Disease

TRANSCATHETER AORTIC VALVE REPLACEMENT FOR SYMPTOMATIC SEVERE AORTIC VALVE STENOSIS DURING PREGNANCY IN AN ADULT WITH CONGENITAL HEART DISEASE

Michael Clark, Caroline Rouse, Anjan K. Sinha, Jeffrey Everett, Stephen C. Cook, Indiana University School of Medicine, Indianapolis, IN, USA

Background

Cardiovascular disease is the most common cause of pregnancy-related mortality. Pregnant women with symptomatic, severe aortic stenosis (AS) carry the greatest complication risk. We present a case of transcatheter aortic valve replacement (TAVR) during pregnancy.

Case

A 34-year-old G8P1243 female with bicuspid aortic valve (BAV), hypertension, history of IV drug use, aortic valve endocarditis, recurrent fetal loss, and gestational diabetes presented to Adult Congenital Heart Disease (ACHD) clinic at 15-weeks gestation. TTE revealed a calcified BAV with severe AS (Fig 1A-B). At 17 weeks, she was hospitalized for symptomatic hypertension (>160/100). With abdominal shielding and Sentinel cerebral protection device, a 34mm Medtronic Evolut R Corevalve was deployed (Fig 1C-E). Procedural fluoroscopy time: 21.5 mins. Post-TAVR TTE revealed reduction in valve gradient (Fig 1F). She was discharged on post-procedure day 2 and underwent C-section at 36 weeks.

Decision-making

During initial counseling, patient affirmed desire to continue pregnancy. Multidisciplinary meetings suggested a higher risk of fetal loss with SAVR. TAVR was pursued with shared decision making.

Conclusion

Symptomatic, severe AS during pregnancy is associated with heart failure and hospitalization. Expansion of TAVR use in the pregnant ACHD patient provided a safer option for both mother and fetus. Multidisciplinary collaboration was key to formulating an individualized plan of care and successful outcome.

Vascular Medicine

AORTIC INVOLVEMENT OF GRANULOMATOSIS WITH POLYANGIITIS

Katlin T. Schmitz, Kathleen Young, Mayo Clinic, Rochester, MN, USA

Background

Granulomatosis with polyangiitis (GPA) is a necrotizing vasculitis that typically affects small- or medium- sized vessels of the upper respiratory tract and kidneys. Involvement of the aorta by GPA is rare, but life threatening.

Case

A 65-year-old male was hospitalized due to memory impairment and abnormal gait. Extensive evaluation included a dural biopsy, which showed pathology consistent with granulomatosis with polyangiitis. Subsequent PET-CT demonstrated FDG-avidity representing vasculitis in the root of the ascending aorta. This was complicated by severe aortic regurgitation and enlarged sinus of Valsalva, with a diameter of 48 mm.

Decision-making

The patient was diagnosed with GPA with pachymeningitis and aortitis involvement. Aortic involvement of GPA is rare, therefore there are no established guidelines to direct management. He was started on pulse dose steroids, and following multidisciplinary discussion, was transitioned to Rituximab with subsequent plan to undergo aortic valve replacement following 2-3 months of treatment. His cognition and gait improved clinically, and he was discharged home on Rituximab to wait for his aortic valve replacement.

Conclusion

We present a case of aortitis secondary to granulomatosis with polyangiitis. Although rare, periaortic inflammation can be life threatening, and GPA should remain on the differential for large vessel vasculitides.

Heart Failure and Cardiomyopathies

ACUTE HEART FAILURE DUE TO MOLAR PREGNANCY-INDUCED THYROID STORM

Nattapat Lilitsuwan, Pollakrit Chiewvit, Chaninart Phongsri, Chalitpol Wanitphisitphan, Wiwun Tungsubutra, Siriraj hospital, Bangkok, Thailand

Background

Thyroid storm (TS) is a life-threatening condition that can present with heart failure (HF). Molar pregnancy (MP) is a rare condition that can cause hyperthyroid (HP) due to the thyrotropic activity of human chorionic gonadotropin (hCG). We present a rare case of MP-induced TS that presented with acute HF.

Case

A 32-year-old woman, presented with a 2-week history of dyspnea, edema and scant vaginal bleeding. She was diagnosed with HP 1 month ago when she presented with a one-month history of palpitations that was treated with methimazole and propranolol. Her temperature was 100.5 o F, heart rate 120 bpm and BP 184/109 mmHg. Chest x-ray showed pulmonary edema, and echo revealed preserved LV systolic function. Lab tests confirmed HP, urine pregnancy test was positive and serum β-hCG was 2,145,191 mIU/ml (normal value 0-3).

Decision-making

Pelvic ultrasound showed multiple intrauterine cystic vesicles. After consultation with Obstetrics and Endocrinology, HF, MP-induced TS and preeclampsia were diagnosed. She was given labetalol, furosemide, propylthiouracil, iodine solution and steroids. Emergency uterine suctional curettage was performed and pathology confirmed hydatiform mole. She had a dramatic response within a day and was discharged on hospital day 6.

Conclusion

We report a rare case of reversible HFpEF due to TS from MP. All women of childbearing age with de novo HF should have a work-up for HP and pregnancy. Early recognition and multidisciplinary team approach are paramount.

Interventional and Structural

HARD TO STOMACH: SEVERE MITRAL STENOSIS FROM GASTRIC COMPRESSION

Swati Chand, Qiu Tong, Sangharsha Thapa, Omofolarin Babayale, Harry McCrea, III, Gaurav Sharma, Rochester General Hospital, Rochester, NY, USA

Background

We present a novel case of functional mitral stenosis (MS) due to gastric compression of the heart from an acutely obstructed and distended hiatal hernia.

Case

81-year-old female with history of complete heart block status post pacemaker, chronic hiatal hernia presented with abdominal pain and vomiting. On exam, she had decreased breath sounds, laterally displaced apical impulse and loud systolic and diastolic murmurs. She was in sinus tachycardia with stable blood pressure. CT imaging revealed a large hiatal hernia with organo-axial gastric volvulus and outlet obstruction. The distended gastric body was mostly supradiaphragmatic with mass effect in the thorax (Figure 1). Echocardiogram (TTE) revealed a hyperdynamic, under-filled left ventricle (LV) with discordant motion from external compression (Figure 2a). Mitral annular calcification was noted with mean inflow gradient of 13 mmHg, suggesting severe MS.

Decision-making

Low-dose beta blocker was cautiously given to improve LV filling. Endoscopic decompression was done for gastric obstruction. After multidisciplinary discussion, she underwent robotic assisted hernia repair. Post-decompression TTE showed near-normal gradients (Figure 2b).

Conclusion

Large hiatal hernias can be managed conservatively, but acute gastric obstruction causing distension can lead to mass effect on other thoracic organs. Compression on the heart can lead to complications, including functional MS, requiring emergent gastric decompression.

Valvular Heart Disease

SUCCESSFUL USE OF ANGIOVAC FOR DEBULKING OF MITRAL VALVE ENDOCARDITIS

Tori Brown, Benjamin Stoner, Amartya Kundu, University of Kentucky, Lexington, KY, USA

Background

Infective endocarditis (IE) has increasing prevalence and high mortality. Early surgery is recommended for patients with large vegetations, septic emboli or persistent bacteremia. However, there are few options for non-surgical patients with left-sided vegetations.

Case

A 76-year-old male was transferred for IE. He had MRSA bacteremia and a >1cm MV vegetation with mild regurgitation on TEE. CT head showed an acute embolic infarct with a small area of hemorrhagic conversion.

Decision-making

The patient was deemed a non-surgical candidate by CT surgery. Given the large vegetation size, ongoing septic embolization and persistent bacteremia the decision was made to attempt percutaneous debulking. An aspiration catheter was constructed from a modified 25F Biomedicus venous cannula with distal side holes removed and a 180-degree distal bend. Under TEE guidance, the cannula was inserted across the inter-atrial septum, directed to the affected leaflet and foreign material was aspirated. Repeat TEE showed decreased vegetation size and mild regurgitation. No subsequent evidence of peripheral emboli and blood cultures cleared.

Conclusion

The AngioVac system has increasing use in the management of right-sided endocarditis. This case demonstrates successful AngioVac for left-sided endocarditis without resulting peripheral embolization or worsening valvular disease. Implementation of this novel technique can allow for safe rehabilitation prior to definitive surgical treatment.

Heart Failure and Cardiomyopathies

TACROLIMUS-INDUCED CARDIOTOXICITY: A RARE CASE OF REVERSIBLE ACUTE CARDIAC FAILURE

Gianluca Di Pietro, Antonella Ciuffreda, Lucia Ilaria Birtolo, Riccardo Improta, Andrea Ascione, Nicola Galea, Massimo Mancone, Carmine Dario Vizza, Francesco Fedele, Viviana Maestrini, La Sapienza, University of Rome, Roma, Italy

Background

Tacrolimus may cause cardiac failure. We present a rare case of acute heart failure in a transplanted patient on tacrolimus immunosuppressive therapy with reverse remodeling at follow-up.

Case

A 68-years-old female underwent to liver transplant for recurrent hepatocarcinoma. After forty days, she presented with dyspnea and peripheral edema. Electrocardiography revealed new diffuse inverted T waves (A). Echocardiogram showed severe biventricular dysfunction. Troponins, inflammatory indices and blood tacrolimus levels were normal.

Decision-making

Coronary angiography was negative (B). Cardiac Magnetic Resonance (CMR) confirmed severe biventricular dilatation and dysfunction and myocardial edema without late gadolinium enhancement (C,D). Endomyocardial biopsy showed cytoplasmic vacuolation of cardio-myocytes, interstitial fibrosis and vascular endothelial swelling, suggestive of cardiotoxicity (E). Cardiotropic viruses were absent in myocytes. Based on the suspicion of cardiac toxicity, tacrolimus was switched to everolimus. Medical therapy was optimized by angiotensin receptor-neprilysin inhibitor (ARNI), betablocker and diuretics. At six months, CMR showed reverse remodeling and preserved systolic function (F).

Conclusion

Tacrolimus-induced reversible heart failure is a rare but serious event. A strict cardiological follow-up should be envisaged for these patients. Further studies are needed to establish the beneficial effect of everolimus and ARNI.

Prevention and Health Promotion

DOUBLE-HETEROZYGOUS APOB/PCSK9 VARIANTS AND UNIQUE ATTENUATED RESPONSES TO DIFFERENT THERAPIES

Rahul Rege, Bani Mekisha Azari, Jessica A. Schillow, Eugenia Gianos, Loukas S. Boutis, Sean M. Donahoe, John N. Makaryus, Danni Fu, Guy L. Mintz, Benjamin J. Hirsh, North Shore University Hospital Northwell Health, Manhasset, NY, USA

Background

Familial hypercholesterolemia (FH) is a genetic condition characterized by elevated LDLc and early onset ASCVD. We present a patient with a rare double-heterozygous FH genotype, CAD, and insufficient response to lipid-lowering therapies.

Case

A 61-year-old female with history of hypercholesterolemia and CAD presented for management of elevated cholesterol. Family history is notable for high prevalence of ASCVD (Figure 1a). Lipid-lowering therapies and response noted in Figure 1b. Patient showed complete lack of response to pro-protein subtilisin kexin-9 (PCSK9) inhibitors.

Decision-making

Given family history of ASCVD and non-response to PCSK9 inhibitor, patient was referred to genetics. Genetic testing revealed variants of unknown significance of the apolipoprotein B (APOB) gene and PCSK9 gene. There was a p.(Arg3558Cys) (CGC>TGC): c.10672 C>T mutation in exon 26 of APOB gene. Patient also had p.(Arg496Trp) (CGG>TGG): c.1486 C>T mutation in exon 9 of PCSK9 gene. There is currently no literature on this unique double-heterozygous genotype. The mutation in the PCSK9 gene is within the catalytic C-terminal domain of the gene and illustrates the mechanism of lack of response to PCSK9 therapy. Patient was started on evinacumab, a medication only FDA-approved for homozygous FH.

Conclusion

This case highlights the rising importance of utilizing a pharmacogenomic approach in the treatment of elevated cholesterol. Based on genomic testing, the patient was approved for evinacumab.

Electrophysiology

NOVEL TRANS-TRACHEAL DEEP CARDIAC PLEXUS BLOCKADE IN THE MANAGEMENT OF INAPPROPRIATE SINUS TACHYCARDIA

Bharat Narasimhan, Fabrizio Assis, Rushil Nilesh Shah, Harikrishna S. Tandri, Johns Hopkins, Baltimore, MD, USA

Background

Management of Inappropriate Sinus Tachycardia (IST) is extremely challenging. Currently available therapeutic options are of limited clinical efficacy. We describe a novel method of sympathetic neuromodulation in the management of refractory IST.

Case

37y/o female with a history of Ehlers-Danlos syndrome presents with recurrent syncope and reduced exercise tolerance over the past 2 years. Holter revealed persistent sinus tachycardia (100-125bpm). TTE and cMRI were normal. Pharmacotherapy (metoprolol, midodrine, diltiazem and ivabradine) had proven ineffective.

Decision-making

Following extensive discussions and ethics board approval, a novel transtracheal, ultrasound-guided deep cardiac plexus blockade was performed using liposomal Bupivacaine. The procedure was well tolerated with no complications. A significant decline in mean HR from 107 to 89bpm was noted with significant symptom relief. Based on this positive response, VATS-assisted cervicothoracic sympathectomy was performed. During a follow-up of ∼2 years, the patient had no further syncopal events. Mean heart rate, a year later remains ∼80 bpm on Holter monitoring.

Conclusion

Though preliminary, transtracheal bupivacaine block could provide a comprehensive albeit transient sympathetic blockade. This could potentially aid in patient selection by predicting responses to the more invasive and definitive VATS procedure while also serving as a temporizing measure in other conditions such as VT storm.

Pediatric and Congenital Heart Disease

TRANSCATHETER REPAIR OF SINUS VENOSUS ATRIAL SEPTAL DEFECT

Raj Patel, Muhammad Adil Sheikh, Manjari R. Regmi, Shruti Hegde, Southern Illinois University, Springfield, IL, USA

Background

Sinus venosus atrial septal defect (SVASD) is a rare adult congenital anomaly. Diagnosis and management of SVASD in adults with co-morbidities can pose multiple challenges.

Case

57 year female presented with dyspnea on exertion. ECG showed sinus rhythm with incomplete right bundle branch block. She had history of morbid obesity, mild COPD, sleep apnea. TTE revealed severe right heart dilatation (RHD) with interventricular septal motion indicative of RV volume overload, mild tricuspid regurgitation, and no obvious intracardiac shunt on color Doppler. Estimated pulmonary artery pressure was 56.2 mmHg indicating moderate pulmonary hypertension (PH).

Decision-making

PH and RHD were out of proportion to her pulmonary pathology and hence TEE was performed. It showed moderate-large communication in the posterior part of interatrial septum, indicating SVASD. Subsequent contrast CT did not show any anomalous pulmonary venous return. Cardiac catheterization showed saturation in IVC of 79%, SVC of 66% and RA of 71% with QP/QS of 0.57% suggesting hemodynamically significant shunt and no significant CAD. Patient was deemed unfit for surgery and successfully underwent percutaneous closure of SVASD with stent placement in SVC. Post-procedure patient was started on clopidogrel. 6 months later, the patient reported significant improvement in her symptoms.

Conclusion

Transcatheter closure of SVASD may be considered as an alternative to surgery in select patients where surgery is not an option.

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