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Background: The 'univentricular' heart encompasses a variety of congenital cardiac defects characterized by a single functional ventricle and an underdeveloped ventricular chamber. Surgical intervention, typically in infancy or childhood, aims to regulate pulmonary blood flow volume. In adulthood, untreated patients may experience limitations in physical activity and elevated morbidity due to persistent cyanosis and arrhythmias, notably after the Fontan procedure.

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Background: With an increasing number of Fontan patients surviving into adulthood, the burden of end-stage heart failure is increasing. Prior studies have reported suboptimal heart transplantation (HTx) outcomes. Therefore, the authors describe their institutional experience of HTx in patients with Fontan circulation failure, including heart-liver transplantation (HLTx) and pretransplant systemic ventricular assist device (SVAD) therapies.

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As the Fontan population grows, understanding successful strategies for ventricular assist device (VAD) support of the failing Fontan circulation is needed. We performed a retrospective analysis of patients with Fontan circulation and systemic VAD support in the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry. Competing outcomes and Kaplan-Meier estimated survival methods were used.

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Computer-generated Clinical Decision-making in the Treatment of Pulmonary Atresia with Intact Ventricular Septum.

Cardiovasc Eng Technol

December 2024

Department of Mechanical Engineering, Koc University, Rumeli Feneri Campus, Sarıyer, Istanbul, 34450, Turkey.

Purpose: Pulmonary atresia with intact ventricular septum is a multifactorial disease requiring complex surgeries. The treatment route is determined based on the right ventricle (RV) size, tricuspid annulus size and coronary circulation dependency of RV. Since multiple parameters influence the post-operative success, a personalized decision-making based on computed hemodynamics is hypothesized to improve the treatment efficacy.

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Management of Tricuspid Atresia With Normally Related Great Arteries and Left-Sided Obstruction.

World J Pediatr Congenit Heart Surg

November 2024

Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.

Article Synopsis
  • Tricuspid atresia (TA) is a heart condition affecting infants and is the second most common type of functionally univentricular heart, with rare cases of left ventricular outflow tract obstruction (LVOTO) in patients who have normally related great arteries.
  • A study involving 445 patients with Type I TA showed that 3% of infants needed interventions for LVOTO, and most of those who underwent surgical procedures had promising outcomes.
  • The long-term survival rate for these infants was estimated at 79% over 20 years, indicating that timely surgical management can lead to successful interventions and good heart function later in life.
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