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Best management in isolated right ventricular hypoplasia with septal defects in adults

Zahra Khajali1, Maedeh Arabian1, Maryam Aliramezany2

  • 1Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran.

Journal of Cardiovascular and Thoracic Research
|October 30, 2020
PubMed

Insights

Isolated right ventricular hypoplasia, a rare congenital heart defect, presents variably. This study details the management of six adult cases, highlighting diverse clinical courses and treatment strategies for this uncommon anomaly.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Congenital Heart Disease

Background:

  • Isolated right ventricular hypoplasia is a rare congenital anomaly.
  • It is distinct from forms associated with pulmonary or tricuspid atresia.
  • Often presents with an atrial septal defect causing right-to-left shunting.

Observation:

  • This condition exhibits highly variable clinical presentations.
  • Some cases are diagnosed neonatally requiring immediate intervention.
  • Other individuals remain asymptomatic into old age.

Findings:

  • This report presents 6 adult cases of isolated right ventricular hypoplasia.
  • Management strategies included medical and surgical interventions.
  • Treatment was tailored to individual disease severity and symptoms.

Implications:

  • Understanding the variable course of isolated right ventricular hypoplasia is crucial for timely diagnosis and management.
  • This case series contributes to the limited literature on adult presentations.
  • Further research may elucidate long-term outcomes and optimal therapeutic approaches.

Keywords:
Adult Congenital Heart DiseaseAtrial Septal DefectCyanosisRight Ventricular Hypoplasia

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