Publications by authors named "Marieke Nederend"

Background: The Fontan operation resulted in improved survival of patients with congenital heart defects not equipped to sustain biventricular circulation. Long-term complications are common, such as veno-venous collaterals (VVC). The aim of this study was to evaluate patient characteristics, percutaneous treatment strategy and (short-term) outcomes in adult Fontan patients with VVC, and review literature to date.

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Catheter interventions can offer patient tailored solutions in high-risk congenital heart disease patients. A 21-year-old male with a Fontan circulation in the setting of unbalanced atrioventricular septal defect with a hypoplastic left ventricle and an aortic homograft connecting the right ventricular outflow tract to the ascending aorta, developed failure of the heavily calcified homograft with severe regurgitation and stenosis. He underwent three sequential transcatheter aortic valve-in-valve implantations to address the homograft failure and the subsequent paravalvular regurgitation, with satisfactory result and improved hemodynamics.

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Aims: Given the compelling evidence on the effectiveness of sodium-glucose cotransporter 2 inhibitors (SGLT2i) in the conventional heart failure population, SGLT2i deserve exploration in systemic right ventricular (sRV) failure. The initial experience with dapagliflozin in sRV failure patients is described, with a focus on tolerability and short-term effects on clinical outcomes.

Methods And Results: Ten patients (70% female, median age 50 years [46.

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Objective: Patients with a systemic right ventricle (sRV) in the context of transposition of the great arteries (TGA) after atrial switch or congenitally corrected TGA (ccTGA) are prone to sRV dysfunction. Pharmacological options for sRV failure remain poorly defined. This study aims to investigate the tolerability and effects of sacubitril/valsartan on sRV failure in adult patients with sRV.

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Article Synopsis
  • Patients with univentricular hearts represent a severe subset of congenital heart disease, with ongoing research into genetic links to these conditions and limited gene identification to date.
  • A case of an adult who underwent Fontan palliation for complex congenital heart issues, including tricuspid atresia and pulmonary stenosis, showed late complications involving multiple organ systems, highlighting the need for genetic evaluation.
  • Genetic screening revealed a significant variant linked to Noonan spectrum disorders, emphasizing the importance of understanding genetic factors in adult patients with congenital heart disease and their associated complications.
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  • * An observational study followed 28 patients between 1989 and 2020, finding that 41% developed a need for chronic ventricular pacing after their surgery, with many experiencing complications within two years post-op.
  • * Patients with chronic pacing showed worse heart function as indicated by higher QRS duration and NT-pro-BNP levels, and 75% faced progressive heart failure outcomes, contrasting with a much lower percentage among those with
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  • Patients with systemic right ventricle (sRV) failure, particularly after atrial switch for transposition of the great arteries (TGA), often experience heart complications like tricuspid valve regurgitation (TR), which can worsen their condition.
  • A case study of a 42-year-old male with sRV failure and significant TR showed a 20% improvement in heart function during a pacing test, which indicated he could benefit from cardiac resynchronization therapy (CRT).
  • After receiving a hybrid CRT-defibrillator, the patient experienced improved sRV function, reduced TR, and enhanced exercise capacity, highlighting the effectiveness of CRT in such cases.
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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 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.

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Article Synopsis
  • * The Fontan procedure connects the inferior vena cava to the pulmonary circulation, allowing passive blood flow without a functional second ventricle, which leads to long-term health challenges for patients.
  • * A multidisciplinary team approach is essential for monitoring and managing these patients post-surgery, in order to detect complications early and enhance their quality of life.
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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. 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.

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Aims: Patients with a systemic right ventricle (sRV) in the context of transposition of the great arteries (TGA) after atrial switch or congenitally corrected TGA are prone to heart failure and arrhythmias. This study evaluated feasibility, patient adherence, and satisfaction of a smart technology-based care pathway for heart failure treatment optimization in these patients.

Methods And Results: Patients with symptomatic sRV failure eligible for initiation of sacubitril/valsartan were provided with four smartphone compatible devices (blood pressure monitor, weight scale, step counter, and rhythm monitor) and were managed according to a smart technology-based care pathway.

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Objective: Pharmacological options for patients with a failing systemic right ventricle (RV) in the context of transposition of the great arteries (TGA) after atrial switch or congenitally corrected TGA (ccTGA) are not well defined. This study aims to investigate the feasibility and effects of sacubitril/valsartan treatment in a single-centre cohort of patients.

Methods: Data on all consecutive adult patients (n=20, mean age 46 years, 50% women) with a failing systemic RV in a biventricular circulation treated with sacubitril/valsartan in our centre are reported.

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