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Tetralogy of Fallot: Multimodality Imaging and Key Historical Contributions to Diagnosis and Treatment.

Tetralogy of Fallot (TOF) is one of the most common cyanotic congenital heart malformations, characterized by four pathological features: right ventricular outflow tract obstruction/pulmonic stenosis, a ventricular septal defect, an overriding aorta, and right ventricular hypertrophy. It was initially partially defined by Nicholas Steno in the 17th century and completely described by Étienne-Louis Arthur Fallot and Maude Abbott in the 19th and 20th centuries. The advances in multimodality imaging and innovative surgical and transcatheter techniques have led to advances in the management of TOF

Complete atrioventricular septal defect, Australian repair: Technique and long-term results.

BackgroundSurgical repair of complete atrioventricular septal defect (CAVSD) has evolved substantially, with early mortality markedly reduced over the past decades. However, achieving durable left atrioventricular valve (LAVV) competence and preserving left ventricular outflow tract (LVOT) geometry remain critical determinants of long-term success.MethodsThis review summarizes the anatomical principles, operative details, and clinical outcomes of the Nunn-modified single-patch ("Australian") technique for CAVSD repair.ResultsThe Australian technique-characterized by direct approximation of bri

Mitral valve disease--morphology and mechanisms.

Mitral valve disease is a frequent cause of heart failure and death. Emerging evidence indicates that the mitral valve is not a passive structure, but--even in adult life--remains dynamic and accessible for treatment. This concept motivates efforts to reduce the clinical progression of mitral valve disease through early detection and modification of underlying mechanisms. Discoveries of genetic mutations causing mitral valve elongation and prolapse have revealed that growth factor signalling and cell migration pathways are regulated by structural molecules in ways that can be modified to limit

Mitral Valve Prolapse in Obstructive Hypertrophic Cardiomyopathy.

Obstructive hypertrophic cardiomyopathy (oHCM) and mitral valve (MV) prolapse (MVP) are the 2 conditions which could cause symptomatic heart failure and sudden cardiac death. The clinical characteristics and surgical outcomes of patients with oHCM and MVP have not been well reported. From April 2012 to February 2018, 84 patients with oHCM (28 patients with MVP and 56 gender- and age-matched patients without MVP) who underwent septal myectomy at our institution were enrolled in this study. Information on clinical characteristics and outcomes was obtained from electronic medical records and foll

[Clinical characteristics and hypertrophic segments distribution of patients with hypertrophic cardiomyopathy].

OBJECTIVE: to analyze the clinical data and hypertrophic segments distribution of patients with hypertrophic cardiomyopathy (HCM). METHODS: … atients. Systolic murmurs were present in 126 patients. Echocardiography examination evidenced left ventricular outflow obstruction in 95 patients, mitral insufficiency in 32 patients, 32.1% segments were hypertrophied, asymmetrical hypertrophy presented in 222 patients and symmetrical hypertrophy in 3 patients. The left atrial dimension was (39.4 ± 8.3) mm, and left ventricular …

Comparison of Alcohol Septal Ablation With Mavacamten in Obstructive Hypertrophic Cardiomyopathy.

Obstructive hypertrophic cardiomyopathy (HCM) is associated with significant morbidity attributed to left ventricular outflow tract (LVOT) obstruction. Although alcohol septal ablation (ASA) is an established interventional treatment, mavacamten, a novel cardiac myosin inhibitor, has emerged as a noninvasive pharmacologic alternative. Understanding the comparative efficacy of these 2 treatments is important for optimizing patient care. This single-center retrospective study assessed the hemodynamic and functional changes in adult patients with obstructive HCM treated with ASA (n = 58) or mavac

The Central Role of Echocardiography in Mavacamten Therapy for Obstructive Hypertrophic Cardiomyopathy: A Comprehensive Review.

Mavacamten is a cardiac myosin inhibitor used in adults with symptomatic obstructive hypertrophic cardiomyopathy (HCM). Echocardiography is central to selecting suitable patients, confirming left ventricular outflow tract (LVOT) obstruction, excluding inappropriate use when left ventricular ejection fraction (LVEF) is < 55% and guiding dose titration during treatment. This narrative review summarises the practical echocardiographic approach to mavacamten therapy, with emphasis on reproducible LVOT gradient assessment, goal-directed Valsalva manoeuvres, LVEF monitoring, mitral valve and systoli

ANMCO position paper 'Hypertrophic cardiomyopathy: from diagnosis to treatment'.

Hypertrophic cardiomyopathy (HCM) is a non-rare genetic cardiomyopathy, with an estimated prevalence of 1:500, characterized by an increase in the left ventricular wall thickness in the absence of increased loading conditions. The hypertrophy is mostly asymmetric and predominantly affects the basal septum and anterior wall. Left ventricular outflow tract obstruction, at rest or after provocative tests, is detected in many patients and represents the primary cause of reduced functional capacity, as well as an independent predictor of sudden cardiac death and advanced heart failure. Until ∼1 yea

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… Multiparametric assessment of left ventricular outflow tract obstruction Left ventricular outflow obstruction is an important pathophysiological mechanism in HCM and a major cause of symptoms such as dyspnoea, chest pain, pre-syncope, and syncope. 30 The presence of LVOTO is also associated with poor …

Cardiac myosin inhibition in hypertrophic cardiomyopathy: review of the evolving evidence base.

INTRODUCTION: There is an unmet need for effective medical therapies in the treatment of obstructive hypertrophic cardiomyopathy (HCM). This is changing with emergence of cardiac myosin inhibitors (CMI), which reduce cardiac myocyte hypercontractility, normalize left ventricular function, and reduce left ventricular outflow tract obstruction. Mavacamten and aficamten are the first 2 drugs in this class with high-quality phase III randomized clinical trial data (Based on PUBMED search, last query April 2025). AREAS COVERED: In the current review, we perform a detailed analysis of the backgroun

Mavacamten for Obstructive Hypertrophic Cardiomyopathy: Targeting Sarcomeric Hypercontractility with Demonstrated Long-Term Safety and Efficacy-A Narrative Review.

Hypertrophic cardiomyopathy (HCM) is the most prevalent hereditary cardiovascular disorder characterized by unexplained left ventricular hypertrophy, sarcomeric hypercontractility, and dynamic left ventricular outflow tract (LVOT) obstruction in approximately 70% of patients. Current therapies predominantly offer symptomatic relief through indirect modulation of cardiac function, leaving the underlying molecular pathophysiology unaddressed. Mavacamten, a first-in-class, selective allosteric inhibitor of β-cardiac myosin ATPase, exemplifies a precision pharmacological approach by directly targe

Use of Peripheral Nerve Blocks in Perioperative Management of Cases with Hypertrophic Cardiomyopathy Undergoing Lower Limb Orthopedic Surgeries.

Hypertrophic obstructive cardiomyopathy is a type of hypertrophic cardiomyopathy (HCM) that involves the left ventricular outflow tract obstruction. Most important parameters are preload, afterload, and ventricular contractility that are prone to fluctuations in HOCM patients in the perioperative period due to the surgical procedure, anesthetic agents and changes in intravascular volume. These lead to increased chances of arrhythmias and myocardial ischemia and can pose significant morbidity and mortality in HCM patients perioperatively. Here, we report three challenging cases of HCM with como

[Quantitative evaluation of right heart obstructions in children using Doppler echocardiography].

To assess the accuracy of pulsed and continuous-wave Doppler echocardiography (DE) for estimating pressure drops across right ventricular ou … DE provides an accurate noninvasive method of estimating pressure gradients in patients with right ventricular outflow obstructions and is helpful in determining the subsequent clinical management.

[Ventricular-infundibular morphology in visceral heterotaxia with left isomerism. An echocardiographic-angiocardiographic study].

We report 2D-echocardiographic and angiocardiographic assessment of 28 cases of visceral heterotaxia with left atrial isomerism, with particular emphasis on infundibular ventricular morphology. Ventricular D-loop was present in 15 cases (52.6%), and ventricular L-loop in 13 (46.4%); 25/48 patients had concordant ventricular loop and cardiac position (89.3%). In 18 patients (64.3%) there were 2 balanced ventricles; of the other 10 patients, 9 (32.1%) presented right and 1 (3.6%) left ventricular dominance. Ventricular septal defect was present in 12 cases (42.8%). Ventriculo-arterial connection

Controversies in cardiovascular medicine. Benefits of surgery in obstructive hypertrophic cardiomyopathy: bring septal myectomy back for European patients.

Hypertrophic cardiomyopathy (HCM), a heterogeneous genetic heart disease with global distribution, is an important cause of heart failure disability at any age. For 50 years, surgical septal myectomy has been the preferred and primary treatment strategy for most HCM patients with progressive, drug refractory functional limitation due to left ventricular (LV) outflow tract obstruction. With very low surgical mortality at experienced centres, septal myectomy reliably abolishes impedance to LV outflow and heart failure-related symptoms, restores quality of life, and importantly is associated with

Outcomes of reparative and transplantation strategies for multilevel left heart obstructions with mitral stenosis.

BACKGROUND: Conventional management for multilevel left heart obstructions and mitral stenosis (Shone's complex) involves multiple operations that carry additive risks. This study reviews our experience with reconstructive and transplantation approaches for Shone's complex. METHODS: Between 1987 and 2007, 43 patients with mitral stenosis and one or more left-sided obstructions were identified: supramitral ring (n = 13), subaortic stenosis (n = 25), aortic stenosis (n = 24), hypoplastic arch (n = 20), and coarctation (n = 38). Thirty patients underwent a staged reparative approach, including 2

Clinical Outcomes and Echocardiographic Predictors of Reintervention After Interrupted Aortic Arch Repair.

Left ventricular outflow tract obstruction (LVOTO) remains a significant complication after primary repair of interrupted aortic arch with ventricular septal defect (IAA-VSD). Clinical and echocardiographic predictors for LVOTO reoperation are controversial and procedures to prophylactically prevent future LVOTO are not reliable. However, it is important to identify the patients at risk for future LVOTO intervention after repair of IAA-VSD. Patients who underwent single-stage IAA-VSD repair at our center 2006-2021 were retrospectively reviewed, excluding patients with associated cardiac lesion

Prognosis Analysis of Children with Interrupted Aortic Arch Complicated with Ventricular Septal Defect and Other Associated Intracardiac Defects after One-Stage Radical Surgery.

BACKGROUND: High rates of mortality and aortic arch stenosis have been reported for one-stage radical surgery of interruption of aortic arch (IAA) with ventricular septal defect (VSD) and other associated intracardiac defects, but the sample size of the study is relatively small, and the credibility of the study is not high. The risk factors of death and aortic arch stenosis will be analyzed in a large sample size of infants with IAA, VSD and other associated intracardiac defects after one-stage radical resection. METHODS: A retrospective analysis was performed on 152 children with IAA, VSD a

Initial Clinical Trial of a Novel Pulmonary Valved Conduit.

Valved allografts and xenografts for reconstruction of the right ventricular outflow tract (RVOT) lack durability and do not grow. We report the first clinical use of a completely bioabsorbable valved conduit (Xeltis pulmonary valve - XPV) in children. Twelve children (six male), median age five (two to twelve) years and median weight 17 (10 to 43) kg, underwent RVOT reconstruction with the XPV. Diagnoses were: pulmonary atresia with ventricular septal defect (VSD) (n = 4), tetralogy of Fallot (n = 4), common arterial trunk (n = 3), and transposition of the great arteries with VSD and pulmonar

PTFE monocusp valve reconstruction of the right ventricular outflow tract.

BACKGROUND: Transannular patching of right ventricular outflow tract obstructive (RVOTO) defects results in pulmonary insufficiency (PI). Biologic monocusp valves (MO) can prevent acute PI but are prone to early degeneration and progressive regurgitation. Polytetrafluoroethylene (PTFE, 0.1 mm) MO leaflets demonstrated favorable characteristics in animal studies, and the technique was applied to a variety of RVOTO anomalies. METHODS: From June 1990 through June 1999, 158 patients underwent either PTFE MO RVOT reconstruction (n = 115 patients; 120 implants) or nonvalved transannular repair (TA)

[Non-surgical ventricular training before arterial switch. Focus on an animal model in lambs].

One of the conditions for successful anatomical correction of transposition of the great arteries (arterial switch) or of double discordance (double switch) is the ability of the subpulmonary left ventricle to adapt to the systemic circulation when restored to its subaortic position. A balloon catheter designed for training the subpulmonary ventricle by progressive occlusion of the main pulmonary artery (NuMed) was implanted in six lambs aged 45 days and inflated progressively to obtain the maximum tolerated right ventricular pressure. Six controls were instrumented without balloon inflation.