Modified hemi-Fontan operation: An alternative definitive palliation for high-risk patients

Background We conceptualized that by adding small amounts of prograde pulmonary blood flow to the hemi-Fontan operation, or bidirectional Glenn procedure, this modified hemi-Fontan operation could be safely done at an early age, with better oxygenation, and with less potential for pulmonary arteriovenous fistulae. Methods Since April 1992 the hemi-Fontan operation was modified by adding some prograde flow through the native pulmonary artery in 10 high-risk infants, either by leaving the critical subpulmonary stenosis untreated (n = 6) or by tightening a previously placed pulmonary artery band (n = 4). All other sources of pulmonary blood flow were interrupted. Patients were 4 to 23 months old (3.8 to 10.3 kg). Diagnoses included isolated dextrocardia with single ventricle (3) and polysplenia syndrome (2). Cardiopulmonary bypass was needed in 5 patients. Results There were no hospital deaths. Mean postoperative intensive care unit stay was 2 days, and 9 of 10 patients were discharged within 7 days of operation. One 4-month-old infant with Downʹs syndrome survived postoperative takedown of the hemi-Fontan repair after pneumonia and caval thrombosis developed. Eight patients are currently asymptomatic receiving minimal medication, and oxygen saturations range from 84% to 93%. Conclusions Adding small volumes of prograde pulmonary blood flow to the hemi-Fontan operation is safe, provides improved oxygenation, may encourage growth of central pulmonary arteries, and represent an alternative definitive palliation for high-risk Fontan candidates.