Hypoplastic Right Heart Syndrome: Pathophysiology, Surgical Management, and Long-Term Outcomes

Faculty Mentor

Dr. Myka Bussey-Campbell

Location

Savannah Ballroom

Type of Research

Proposed

Session Format

Poster Presentation

College

Waters College of Health Professions

Department

Clinical Sciences

Abstract

Abstract

This case study entails a fetus at 21 weeks with Hypoplastic Right Heart Syndrome (HRHS) , which is a congenital heart defect that involves the underdevelopment of the right ventricle, tricuspid valve, and pulmonary artery, or most commonly presenting as Tricuspid Atresia or Pulmonary Atresia with Intact Ventricular Septum (PAIVS). HRHS results in significant obstruction of pulmonary blood flow and neonatal cyanosis. This causes restriction of the lungs receiving oxygenated blood. This review examines the anatomical variations of the fetus’s HRHS and the evolution of supportive strategies. HRHS has to rely on alternate pathways of delivering oxygenated blood to the lungs like ductus arteriosus stent. However, long-term management typically requires a staged single-ventricle surgical pathway, progressing from neonatal shunting or a surgery known as the Glenn. This surgery involves ductal stenting to the bidirectional and eventually the Fontan procedure, which is the final palliative heart surgery.  Although advances in surgical techniques and perioperative care have dramatically improved survival rates into adulthood, patients can still face  significant long-term conditions, including arrhythmias, ventricular dysfunction, and protein-losing enteropathy. This paper summarizes current diagnostic modalities used to diagnose HRHS, compares surgical versus hybrid catheterization interventions, and examines the move from survival as the primary goal to improved quality of life in the adult congenital heart disease population.

Keywords: Hypoplastic right heart syndrome, Pulmonary atresia with Intact ventricular septum, pulmonary blood flow, ductus arteriosus stent, palliative, congenital heart

Program Description

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Start Date

4-21-2026 1:30 PM

End Date

4-21-2026 3:30 PM

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Apr 21st, 1:30 PM Apr 21st, 3:30 PM

Hypoplastic Right Heart Syndrome: Pathophysiology, Surgical Management, and Long-Term Outcomes

Savannah Ballroom

Abstract

This case study entails a fetus at 21 weeks with Hypoplastic Right Heart Syndrome (HRHS) , which is a congenital heart defect that involves the underdevelopment of the right ventricle, tricuspid valve, and pulmonary artery, or most commonly presenting as Tricuspid Atresia or Pulmonary Atresia with Intact Ventricular Septum (PAIVS). HRHS results in significant obstruction of pulmonary blood flow and neonatal cyanosis. This causes restriction of the lungs receiving oxygenated blood. This review examines the anatomical variations of the fetus’s HRHS and the evolution of supportive strategies. HRHS has to rely on alternate pathways of delivering oxygenated blood to the lungs like ductus arteriosus stent. However, long-term management typically requires a staged single-ventricle surgical pathway, progressing from neonatal shunting or a surgery known as the Glenn. This surgery involves ductal stenting to the bidirectional and eventually the Fontan procedure, which is the final palliative heart surgery.  Although advances in surgical techniques and perioperative care have dramatically improved survival rates into adulthood, patients can still face  significant long-term conditions, including arrhythmias, ventricular dysfunction, and protein-losing enteropathy. This paper summarizes current diagnostic modalities used to diagnose HRHS, compares surgical versus hybrid catheterization interventions, and examines the move from survival as the primary goal to improved quality of life in the adult congenital heart disease population.

Keywords: Hypoplastic right heart syndrome, Pulmonary atresia with Intact ventricular septum, pulmonary blood flow, ductus arteriosus stent, palliative, congenital heart