HRCR Rare Diseases Article Collection
- Ebstein’s anomaly, a rare and highly variable congenital heart defect,1 still presents a treatment challenge. The currently used cone repair of the tricuspid valve has carried favorable results in suitable patients.2 Arrhythmogenic substrates including accessory pathways3,4 and right bundle branch block5 associated with electromechanical ventricular dyssynchrony present additional therapeutic targets. We present a patient with Ebstein’s anomaly of tricuspid valve and Wolff-Parkinson-White syndrome in whom joint electrophysiological and surgical interventions were used to address all major disease components.
- The population of individuals with adult congenital heart disease is expanding as advances in surgical and medical management allow patients to live into adulthood. Consequently, these patients are developing other cardiovascular diseases, such as arrhythmias and heart failure, later in life. Procedures involving congenital anatomic variations are becoming more commonplace, and operators must become familiar with them. One such congenital abnormality, dextrocardia, involves the reversal of the base–apex axis of the heart caudally and to the right.
- Cor triatriatum sinister (CTS) accounts for <0.1% of all congenital heart diseases. It is a condition in which the fibromuscular membrane divides the left atrium (LA) into 2 chambers.1 The superior and posterior chambers receive the pulmonary veins, and the inferior and anterior chambers are connected to the left atrial appendage and mitral orifice.2 Pathophysiologically, CTS is similar to mitral stenosis,3 and the symptoms are correlated with pulmonary venous congestion and pressure loading at the right side of the heart.
- Entirely subcutaneous implantable cardioverter-defibrillators (S-ICD™ system; Boston Scientific Corp, Marlborough, MA) provide effective defibrillation and reduce the risk of infection or lead-related problems.1–3 S-ICD systems may be a valid alternative to transvenous implantable cardioverter-defibrillator (ICD) in patients in whom bradycardia pacing or cardiac resynchronization therapy (CRT) is not required.4,5 The Micra™ AV Transcatheter Pacing System (Medtronic Inc, Minneapolis, MN) is a recently approved leadless pacemaker enabling atrioventricular (AV) synchronized pacing, which may, in theory, compensate the S-ICD’s inability to pace.
- Eisenmenger syndrome is at the extreme end of the phenotypic spectrum of pulmonary arterial hypertension associated with congenital heart disease. Over time, a large nonrestrictive left-to-right shunt provokes progressive pulmonary vascular disease that results in the shunt becoming bidirectional or reversing direction (ie, right-to-left). When the need for pacing arises, transvenous leads are generally considered contraindicated owing to a prohibitively high risk of paradoxical thromboembolism.