CARDIAC SURGERY IN THE ADULT CONGENITAL HEART DISEASE PATIENT: A SPECTRUM OF LESIONS


Bassem N. Mora, M.D., Sheikh Khalifa Medical City, Cleveland Clinic, Abu Dhabi, United Arab Emirates

Objectives: To discuss the spectrum of anomalies which require cardiac surgery in the adult congenital heart disease (ACHD) patient.

Background: There are >1,000,000 ACHD patients in North America. Cardiac surgery in those is divided into three categories:
1. Great complexity: cyanotic lesions, single ventricle, double-outlet ventricle
2. Moderate complexity: anomalous pulmonary venous drainage, atrioventricular septal defects, coarctation, Ebstein’s, right ventricular (RV) or left ventricular (LV) outflow tract obstruction, non-secundum atrial septal defect (ASD), pulmonary regurgitation (PR) or stenosis, tetralogy of Fallot, complicated ventricular septal defect (VSD)
3. Simple complexity: isolated congenital valve stenosis or insufficiency, simple VSD, secundum ASD

Results: Treatment decisions should be individualized:
• ASD: should undergo closure if RV volume overload exists, irrespective of symptoms, with concomitant maze procedures for atrial fibrillation.
• VSD: should undergo surgical closure if pulmonary-to-systemic blood flow ratio is >1.5 and pulmonary vascular resistance is <2/3 systemic.
• Valve repair/replacement: Worsening CHD valvar stenosis or regurgitation should undergo valve therapy as in patients with non-congenital valve disease. Most are reoperative.
• Subaortic stenosis: Should undergo resection if peak LV outflow tract gradients exceed 50mmHg • RV outflow tract obstruction: Severe valvar obstruction requires surgery if percutaneous therapy is contraindicated. Subvalvar stenosis, supravalvar stenosis and double-chambered RV require surgery. • Anomalous aortic origin of coronary arteries (AAOCA): Surgery for patients with left AAOCA irrespective of symptoms, and with right AAOCA with ischemia.
• Pulmonary valve replacement: For severe PR with symptoms, RV dysfunction/enlargement, arrhythmias, or significant tricuspid insufficiency.
• Ebstein’s: Surgery if symptoms, cyanosis, embolism or progressive RV dilatation/dysfunction.
• Single ventricle: Some will present in adulthood; they should undergo staged palliation if otherwise suitable.

Conclusions: Cardiac surgery for ACHD patients involves a wide spectrum of lesions with established indications and reasonable morbidity and mortality. As more CHD patients survive into adulthood, the variety of cardiac surgical repairs will increase in complexity.