Double outlet left ventricle. A new type of presentation

Area of Science:

• Cardiology
• Congenital Heart Disease
• Pediatric Cardiology

Background:

• Double outlet left ventricle (DOLV) is a rare congenital heart defect.
• Accurate classification and understanding of associated anomalies are vital for patient management.

Observation:

• A post-mortem examination revealed a unique presentation of DOLV.
• Key findings included an enlarged right atrium with a large Eustachian valve and an atrial septal defect (ostium secundum).

Findings:

• The heart exhibited tricuspid valve hypoplasia with fused intercordal spaces, resembling tricuspid atresia, and a hypoplastic right ventricle.
• Both the aorta and pulmonary artery originated from the left ventricle, with a sub-aortic ventricular septal defect due to septal malalignment.
• Pulmonary stenosis (subvalvular and valvular) and a hypoplastic main pulmonary artery were noted, with atresia of the left pulmonary artery supplied by a patent ductus arteriosus.

Implications:

• This specific DOLV variant presents unique anatomical challenges.
• Accurate prenatal or early postnatal diagnosis is essential for planning potential surgical interventions.
• Understanding such complex variations aids in improving surgical strategies for congenital heart defects.