Conversion to total cavopulmonary connection after failed one and one-half ventricular repair

Area of Science:

• Cardiovascular Surgery
• Pediatric Cardiology
• Congenital Heart Disease

Background:

• One and one-half ventricular repair is a complex procedure for certain congenital heart defects.
• Long-term outcomes, particularly right ventricular function, require ongoing evaluation.
• Progression of right ventricular dysfunction can occur years after initial repair.

Observation:

• Three patients with prior one and one-half ventricular repair developed progressive right ventricular dysfunction over 10 years.
• Clinical presentation mimicked the atriopulmonary Fontan procedure, prompting reoperation.
• Reoperation involved conversion to total cavopulmonary connection.

Findings:

• Hemodynamic parameters improved significantly after conversion to total cavopulmonary connection.
• Two patients exhibited abnormal right atrial wall histopathology and developed persistent atrial arrhythmia.
• These two patients subsequently required pacemaker implantation due to sinus nodal dysfunction.

Implications:

• Conversion to total cavopulmonary connection can be a viable strategy for managing late right ventricular dysfunction after one and one-half ventricular repair.
• Histopathological abnormalities in the right atrial wall may predict arrhythmias and conduction disturbances.
• Further research is needed to understand the long-term implications of atrial pathology in this patient cohort.