Double-outlet right ventricle associated with persistent common atriventricular canal

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Cardiac Surgery

Background:

• Double-outlet right ventricle (DORV) is a complex congenital heart defect.
• Common atrioventricular (A-V) canal is another significant cardiac anomaly.
• The co-occurrence of DORV and common A-V canal presents a unique diagnostic and therapeutic challenge.

Purpose of the Study:

• To describe a specific variant of double-outlet right ventricle (DORV) associated with common atrioventricular (A-V) canal.
• To analyze the clinical presentation, diagnostic features, and associated anomalies in patients with this syndrome.
• To underscore the current unrepaired status of this complex cardiac malformation.

Main Methods:

• Retrospective analysis of 16 patients diagnosed with DORV and common A-V canal.
• Classification of patients into groups based on cardiac apex position (levocardia vs. dextrocardia).
• Review of diagnostic data including electrocardiography, right and left ventricular angiocardiography, and postmortem/exploratory cardiotomy findings.

Main Results:

• The syndrome involves DORV with common A-V canal (Rastelli type C) in 14 patients.
• Clinical presentation varied based on the presence of pulmonary stenosis, mimicking Tetralogy of Fallot or VSD with bidirectional shunting.
• Common associated anomalies included splenic anomalies (asplenia/polysplenia), persistent left superior vena cava, and anomalous pulmonary venous connection.

Conclusions:

• This DORV and common A-V canal variant represents a severe congenital heart defect with significant associated anomalies.
• Diagnostic features on ECG and angiocardiography are characteristic but show variations based on cardiac position.
• The current lack of successful surgical repair emphasizes the complexity and poor prognosis of this specific cardiac syndrome.