Right and left isomerism: the cardiac surgeon's view

Area of Science:

• Cardiovascular Surgery
• Pediatric Cardiology
• Congenital Heart Disease

Background:

• Complex cardiovascular anomalies are frequently associated with right isomerism (asplenia syndrome) and left isomerism (polysplenia syndrome).
• These developmental syndromes present unique anatomical challenges for surgical intervention.

Purpose of the Study:

• To evaluate surgical options and outcomes for patients with complex cardiovascular anomalies and isomerism.
• To identify technical challenges in managing these complex intracardiac morphologies.

Main Methods:

• Retrospective analysis of 12 patients with right or left isomerism undergoing palliative or corrective surgery.
• Surgical procedures included systemic-pulmonary artery shunts, anatomical intracardiac repair, and modified Fontan procedures.
• One patient with pulmonary arteriovenous fistulas underwent exploratory sternotomy but was not operated on.

Main Results:

• Two early deaths occurred following systemic-pulmonary artery shunts in 4 patients.
• Anatomical intracardiac repair was successful in 2 patients.
• The modified Fontan procedure in 5 patients resulted in only 1 survivor, indicating significant mortality.

Conclusions:

• Surgical treatment for complex cardiovascular anomalies in isomerism syndromes carries substantial risk.
• The modified Fontan procedure demonstrated a particularly high mortality rate in this cohort.
• Complex intracardiac morphology in these syndromes presents significant surgical hurdles.