History of Presentation
A 45-year-old man presented with fatigue, exertional dyspnea consistent with NYHA functional class II status, and generalized edema. Physical examination showed pedal edema, periorbital swelling, and anasarca. Vital signs were within the normal range. Take-Home Messages • In cardiac AL amyloidosis, congestion assessment may be unreliable when nephrotic syndrome, anorexia, and treatment-related fluid shifts coexist. • Pulmonary artery pressure–guided management may help tailor diuretic therapy during stem cell transplantation in selected cardio-oncology patients with restrictive physiology and unstable volume status.
Past Medical History
The patient had no significant prior medical history. He was obese (body mass index: 40.9 kg/m 2 ) and had a past smoking history, with smoking cessation at age 40 years. He was not taking chronic medication at presentation.
Differential Diagnosis
The differential diagnosis of new-onset heart failure (HF) with increased ventricular wall thickness and nephrotic syndrome included cardiac amyloidosis, hypertensive heart disease, hypertrophic cardiomyopathy, myocarditis, and other infiltrative cardiomyopathies.
Investigations
Transthoracic echocardiography showed a reduced left ventricular ejection fraction of 39%, severe concentric left ventricular hypertrophy with a maximal wall thickness of 22 mm, a small pericardial effusion, restrictive diastolic filling, and elevated pulmonary artery systolic pressure (48 mm Hg plus an estimated central venous pressure of 7 mm Hg). Electrocardiography showed low voltages and diffuse repolarization abnormalities. Given the signs of HF and abnormalities detected during cycloergometry testing, coronary angiography was performed and excluded obstructive coronary artery disease.
Laboratory evaluation revealed elevated N-terminal pro–B-type natriuretic peptide of 2,486 ng/L, serum creatinine of 1.67 mg/dL, nephrotic-range proteinuria of 11.73 g/g creatinine, and severe hypoalbuminemia of 17.6 g/L. Serum and urine testing showed excess lambda free light chains. Bone marrow aspiration and biopsy demonstrated 62% plasmacytosis with t(11; 14) and del(13q), establishing the diagnosis of multiple myeloma. Bone scintigraphy using technetium-99m showed no cardiac tracer uptake (Perugini grade 0), arguing against transthyretin amyloidosis. Cardiac magnetic resonance (CMR) showed myocardial fibrosis and edema with diffuse subendocardial late gadolinium enhancement. Taken together, these findings supported the diagnosis of cardiac amyloid light chain (AL) amyloidosis secondary to multiple myeloma.
Management
Guideline-directed HF therapy was initiated with spironolactone 25 mg once daily, empagliflozin 10 mg once daily, bisoprolol 2.5 mg once daily, sacubitril/valsartan 24/26 mg twice daily, and bumetanide 1 mg once daily. After hematologic diagnosis, induction treatment with daratumumab, bortezomib, cyclophosphamide, and dexamethasone was started. During induction therapy, repeated changes in the diuretic regimen were required because of chemotherapy-related fluid shifts, nephrotic syndrome, and variable oral intake. Body weight did not reliably reflect intravascular volume status.
Because euvolemia proved difficult to maintain and even more pronounced hemodynamic instability was anticipated during high-dose chemotherapy and autologous stem cell transplantation (SCT), an implantable pulmonary artery pressure (PAP) sensor was placed in a posterior branch of the left pulmonary artery before transplantation. The rationale for implantation was 3-fold: 1) recurrent episodes of both congestion and dehydration during induction therapy; 2) marked extravascular overload from nephrotic syndrome, limiting the value of physical examination; and 3) anorexia and fluctuating body weight in the oncologic setting, reducing the usefulness of weight trends.
The sensor was used for regular remote PAP assessment, with a predefined target diastolic PAP of 22 mm Hg. Sensor readings were used to guide diuretic adjustments throughout the peritransplant phase ( Figure 1 ). Figure 1 Pulmonary Artery Pressure Trends Over Time Guiding Diuretic Adjustments Administration of additional intravenous loop diuretics are indicated by the yellow markers. PAP = pulmonary artery pressure.
Before sensor implantation, the patient had 1 hospitalization for volume overload. After implantation, no further HF hospitalizations were required. During the sensor-guided period, additional intravenous loop diuretics were administered a total of 4 times (as indicated by the yellow markers in Figure 1 ) based on PAP trends and concurrent clinical events, while oral loop diuretics were also intermittently dose-adjusted and temporarily withheld in response to the PAP measurements. The peritransplant hospitalization lasted 36 days.
During the peritransplant period, the patient developed several complications associated with large-volume changes and shifts, including intravenous fluid administration, poor oral intake due to reduced appetite and oral mucositis, nausea, vomiting, diarrhea, anemia, and thrombocytopenia requiring transfusions, neutropenic fever, and transient delirium. PAP-guided management reduced the need for repeated echocardiographic volume assessments and helped preserve protective isolation during the hematologic nadir.
Outcome and Follow-Up
Body weight fluctuated markedly during the course of illness and treatment, from 140 to 82 kg, underscoring the limited value of weight-based assessment in this setting ( Figure 2 ). Despite these hemodynamic challenges, the clinical course remained free of overt HF decompensation during the peritransplant period. Figure 2 Fluctuations in Body Weight During Treatment Course
After autologous SCT, the patient achieved complete hematologic remission. At follow-up several months later, he was asymptomatic, with no signs of disease activity or fluid overload, while receiving monthly daratumumab. Repeat CMR 1 year after transplantation showed stable left ventricular ejection fraction and a reduction in myocardial edema, consistent with cardiac stabilization.
Discussion
This case illustrates the importance of a multidisciplinary approach in patients with multiple myeloma complicated by cardiac AL amyloidosis. The key clinical problem was that conventional markers of congestion were unreliable: Nephrotic syndrome caused major extravascular edema, oncologic anorexia caused weight variability, and peritransplant complications created abrupt shifts between fluid overload and depletion. In that setting, direct hemodynamic monitoring offered a pragmatic way to titrate diuretics.
Current guidelines on heart failure support the use of remote monitoring technologies, including PAP sensors, in selected patients with recurrent congestion. 1 Evidence supporting implantable PAP monitoring comes primarily from broader HF populations. In the CHAMPION trial, wireless PAP-guided management reduced HF hospitalizations in symptomatic chronic HF. 2 Follow-up analyses and later trials, including GUIDE-HF and MONITOR-HF, further supported the concept that hemodynamic congestion precedes overt clinical decompensation and that earlier treatment adjustment can improve outcomes, especially HF hospitalization burden and quality of life. 3 , 4 , 5
Data specific to infiltrative cardiomyopathies and an immunosuppressed patient population remain very limited. To our knowledge, PAP sensor monitoring has previously been applied successfully in a patient with cardiac AL amyloidosis undergoing heart transplantation. 6 This supports the feasibility of PAP-guided management in this specific patient population. Moreover, cardiac amyloidosis is conceptually well suited to PAP-guided management because restrictive physiology produces a narrow therapeutic window: Relatively small intravascular changes may provoke congestion, hypotension, or renal dysfunction. In addition, physical signs can be misleading when nephrotic syndrome or marked interstitial edema coexist. For that reason, PAP monitoring may be especially useful in selected amyloidosis patients with recurrent or rapidly changing congestion, although this remains an extrapolation from general HF evidence rather than a population specifically tested in randomized trials. Amyloidosis diagnosis and management guidelines also emphasize the complexity of volume control in these patients.
The same rationale applies to the cardio-oncology setting. Patients receiving intensive chemotherapy or SCT often undergo repeated intravenous hydration, transfusions, gastrointestinal losses, reduced intake, and infection-related hemodynamic stress. These factors make bedside volume assessment particularly difficult. In this case, the PAP sensor permitted serial, treatment-linked diuretic decisions during a highly unstable period and reduced the need for repeated echocardiographic reassessment while the patient was in protective isolation. That may be particularly attractive in selected cardio-oncology patients with concomitant HF, infiltrative cardiomyopathy, or a strong mismatch between symptoms, weight, and true filling status. This remains a niche indication at present, but the physiology is compelling.
Potential risks and limitations should also be acknowledged more explicitly. Implantation-related and postmarketing adverse events reported with PAP sensors include pulmonary artery injury, hemoptysis, device failure/malposition, and procedure-related complications; in postmarketing surveillance, adverse events were uncommon but not negligible. 7 Infection risk is generally lower than with systems that require a subcutaneous generator or transvenous leads, yet any implanted foreign body deserves caution in an immunocompromised patient. In a post-SCT population, concerns include bacteremia during neutropenia, the difficulty of evaluating fever in the presence of an implanted device, and the possibility that thrombocytopenia or coagulopathy might increase procedural bleeding risk. These considerations argue for careful patient selection and close multidisciplinary follow-up rather than routine use.
Overall, this case does not establish routine PAP sensor use in cardiac amyloidosis or cardio-oncology. It does, however, suggest that such monitoring can be clinically valuable when the central management challenge is not diagnosis but repeated uncertainty about intravascular filling status during a high-risk treatment phase.
Conclusions
In a patient with multiple myeloma, nephrotic syndrome, and cardiac AL amyloidosis undergoing autologous SCT, PAP-guided management provided actionable hemodynamic information when body weight and clinical examination were unreliable. This strategy may be considered in highly selected patients with infiltrative heart disease or complex cardio-oncology treatment courses, particularly when recurrent congestion and hypovolemia both threaten clinical stability.
Funding Support and Author Disclosures
The authors have reported that they have no relationships relevant to the contents of this paper to disclose. Visual Summary Timeline of Case Presentation Time Events First presentation A 45-year-old man presented with symptoms of progressive fatigue, exertional dyspnea (NYHA functional class II), and generalized edema. 3 mo First cardiology assessment: TTE showed reduced LVEF of 39%, severe concentric left ventricular hypertrophy and pericardial effusion. Coronary angiography demonstrated no obstructive coronary disease. 3.5 mo Referral to heart failure clinic: Identification of light chain lambda excess in both serum and urine, along with a negative bone scintigraphy (Perugini grade 0). 4 mo Bone marrow biopsy: Diagnosis of multiple myeloma with cardiac and renal AL amyloidosis. 4.5 mo Start of chemotherapy (daratumumab, bortezomib, cyclophosphamide, and dexamethasone). 8 mo Implantation of pulmonary artery pressure sensor. 8.5 mo CMR demonstrated myocardial fibrosis and edema, LVEF of 48% with increased end-systolic and end-diastolic volumes. 9 mo Peripheral stem cell collection. 10 mo High-dose melphalan and autologous SCT. PAP-guided diuretic management of associated fluid changes and shifts. 1 y and 10 mo Repeat CMR demonstrated largely unchanged findings with a stable LVEF, reduced myocardial edema. 2 y Follow-up with no evidence of disease activity, monthly daratumumab therapy. AL = amyloid light chain; CMR = cardiac magnetic resonance; LVEF = left ventricular ejection fraction; PAP = pulmonary artery pressure; SCT = stem cell transplantation; TTE = transthoracic echocardiography.
Visual Summary
Timeline of Case Presentation
AL = amyloid light chain; CMR = cardiac magnetic resonance; LVEF = left ventricular ejection fraction; PAP = pulmonary artery pressure; SCT = stem cell transplantation; TTE = transthoracic echocardiography.
| Time | Events |
| First presentation | A 45-year-old man presented with symptoms of progressive fatigue, exertional dyspnea (NYHA functional class II), and generalized edema. |
| 3 mo | First cardiology assessment: TTE showed reduced LVEF of 39%, severe concentric left ventricular hypertrophy and pericardial effusion. Coronary angiography demonstrated no obstructive coronary disease. |
| 3.5 mo | Referral to heart failure clinic: Identification of light chain lambda excess in both serum and urine, along with a negative bone scintigraphy (Perugini grade 0). |
| 4 mo | Bone marrow biopsy: Diagnosis of multiple myeloma with cardiac and renal AL amyloidosis. |
| 4.5 mo | Start of chemotherapy (daratumumab, bortezomib, cyclophosphamide, and dexamethasone). |
| 8 mo | Implantation of pulmonary artery pressure sensor. |
| 8.5 mo | CMR demonstrated myocardial fibrosis and edema, LVEF of 48% with increased end-systolic and end-diastolic volumes. |
| 9 mo | Peripheral stem cell collection. |
| 10 mo | High-dose melphalan and autologous SCT. PAP-guided diuretic management of associated fluid changes and shifts. |
| 1 y and 10 mo | Repeat CMR demonstrated largely unchanged findings with a stable LVEF, reduced myocardial edema. |
| 2 y | Follow-up with no evidence of disease activity, monthly daratumumab therapy. |
References
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