In congenitally corrected transposition of the great arteries, the morphological right ventricle supports the systemic circulation. This chronic exposure to pressure overload ultimately leads to... Show moreIn congenitally corrected transposition of the great arteries, the morphological right ventricle supports the systemic circulation. This chronic exposure to pressure overload ultimately leads to systemic right ventricular (sRV) dysfunction and heart failure. Pharmacological options for the treatment of sRV failure are poorly defined and no solid recommendations are made in the most recent guidelines. Sodium-glucose cotransporter 2 (SGLT-2) inhibitors are a new class of antihyperglycaemic drugs that have been demonstrated to significantly reduce the risk of worsening heart failure and death from cardiovascular causes in patients with chronic heart failure with reduced left ventricular ejection fraction, yet no data are available in sRV patients. We report on the treatment and clinical follow-up of a patient with advanced heart failure and poor sRV function in the context of congenitally corrected transposition of the great arteries, who did not tolerate sacubitril/valsartan and had a high burden of heart-failure-related hospitalizations. Treatment with dapagliflozin was well tolerated and resulted in (small) subjective and objective functional and echocardiographic improvement and a reduction in heart-failure-related hospitalizations. Show less
Nederend, M.; Zeppenfeld, K.; Jongbloed, M.R.M.; Egorova, A.D. 2022
Background Patients with a systemic right ventricle (sRV) in the context of transposition of the great arteries (TGA) late after atrial switch are prone to heart failure. Complications like... Show moreBackground Patients with a systemic right ventricle (sRV) in the context of transposition of the great arteries (TGA) late after atrial switch are prone to heart failure. Complications like tricuspid valve regurgitation (TR) can further aggravate sRV dysfunction. Tricuspid valve regurgitation is usually secondary to annular dilatation and restriction. Criteria for cardiac resynchronization therapy (CRT) in this patient group are not well defined and should be considered on a case-by-case basis. Case summary We present a case of a 42-year-old male with sRV failure and TR in the context of TGA after atrial switch. Patient had progressive reduction in exercise capacity. Electrocardiogram showed a wide QRS complex (right bundle branch block configuration). Echocardiography showed significant TR and signs of electromechanical dyssynchrony of the failing sRV with severely reduced systolic function. He underwent heart catheterization and invasive haemodynamic evaluation to assess the potential benefit of CRT. During sequential atrial-sRV pacing, 20% increase in Dp/Dt was measured, suggesting that he would be a CRT responder. Concomitant angiography showed no baffle leakage nor obstructive coronary artery disease. Hybrid CRT-defibrillator implantation resulted in successful resynchronization and improved sRV function, reduced TR and better exercise capacity. Discussion Invasive haemodynamic contractility evaluation can help assess the potential benefit of CRT in patients with systemic right ventricular failure in the context of transposition of TGA after atrial switch. Successful CRT can result in improved sRV function, reduced TR and improved exercise capacity. Show less
Nederend, M.; Erven, L. van; Zeppenfeld, K.; Vliegen, H.W.; Egorova, A.D. 2021
Background Patients with congenitally corrected transposition of the great arteries (ccTGA) are prone to the development of advanced atrio-ventricular block requiring chronic ventricular pacing.... Show moreBackground Patients with congenitally corrected transposition of the great arteries (ccTGA) are prone to the development of advanced atrio-ventricular block requiring chronic ventricular pacing. The morphological right ventricle (RV) often develops systolic dysfunction as it is unable to withstand the chronic pressure overload it is exposed to when supporting the systemic circulation.Case summary A 56-year-old woman with dextrocardia and complex ccTGA with a history of dual-chamber implantable cardioverter-defibrillator (DDD-ICD, high degree atrio-ventricular-block and syncopal ventricular tachycardia), presented with progressive heart failure and symptomatic atrial arrhythmias. She underwent a successful ablation and concomitant invasive haemodynamic evaluation of potential alternative/biventricular pacing modalities. During biventricular pacing, the QRS narrowed and the systemic RV intraventricular pressure (Dp/Dt) increased with 30%. She underwent a successful transvenous upgrade to cardiac resynchronization therapy (CRT). The electrocardiogram post-implantation showed biventricular capture and patient showed subjective and objective clinical improvement.Discussion Systemic RV dysfunction in ccTGA can be aggravated by chronic pacing-induced dyssynchrony, contributing to progression of heart failure in this patient group. Transvenous CRT is feasible in ccTGA anatomy and may be pursued in order to improve or preserve the functional status of pacing-dependent ccTGA patients. Invasive haemodynamic contractility evaluation can help assess the potential benefit of CRT in patients with complex anatomy. Show less
Dissel, A.C. van; Winter, M.M.; Bom, T. van der; Vliegen, H.W.; Dijk, A.P.J. van; Pieper, P.G.; ... ; Bouma, B.J. 2019
Objectives: In the VAL-SERVE (Valsartan in Systemic Right Ventricle) trial, three-year valsartan treatment improved systemic ventricular function only in symptomatic patients with congenitally or... Show moreObjectives: In the VAL-SERVE (Valsartan in Systemic Right Ventricle) trial, three-year valsartan treatment improved systemic ventricular function only in symptomatic patients with congenitally or with an atrial switch corrected transposition of the great arteries. The aim of the current study was to investigate the longer-term clinical outcomes after valsartan treatment.Methods: From 2006 to 2009, 88 adults were randomly allocated 1:1 to either valsartan or placebo for three consecutive years. Endpoints were defined as overall survival and freedom from clinical events (arrhythmia, heart failure, tricuspid valve surgery, death).Results: Cardiac drug use andmedian follow-up after trial close-out (8.3 years) was similar between the randomization groups. Six patients (valsartan n = 3, placebo n = 3) died in 364 and 365 person-years (P = 0.999). No difference in the composite or separate clinical endpoints was found between the randomization groups, with corresponding long-term event-free survival rates of 50% and 34%. Nevertheless, in symptomatic patients valsartan significantly reduced the risk for events compared to placebo (HR 0.37, 95% CI 0.17-0.92). Analysis for repeated events and on-treatment analysis with any renin-angiotensin-aldosterone-system-inhibitor did not alter these results.Conclusions: Valsartan treatment in systemic RV patients did not result in improved survival at longer-term follow-up, but was associated with decreased risk of events in symptomatic patients. (c) 2018 Elsevier B.V. All rights reserved. Show less
