This is a case of a 3 year old male diagnosed with DORV who underwent Hemifontan procedure with systemic venous hypertension and the role of interventional cardiology in hemodynamically significant post-operative surgical lesions.
The patient came in with a chief complaint of engorgement of vein on the anterior chest and abdomen. He presented with recurrent severe pneumonia and then developed cyanosis, interrupted feeding and diaphoresis. He was diagnosed with DORV without pulmonary stenosis and underwent pulmonary artery banding. Hemifontan was eventually done however without ligating the pulmonary artery thus maintaining the pulmonary blood flow. He then developed signs and symptoms suggestive of systemic venous hypertension. Hemodynamic study showed elevated RV systolic pressure which was carried over to RPA and SVC.
Trial occlusion was done along the RVOT. This showed significant decrease in RV pressure and pulmonary pressure hence RVOT device closure was performed. After closure, there was note of significant reduction of visible vessel on the anterior chest walls and abdomen. There are varieties of extracardiac and intracardiac communication that require closure for the relief of the hemodynamic burden.
Today, the procedure is accomplished less invasively with transcatheterization. Primary consideration should be whether their clinical utility, morbidity and mortality justify a nonsurgical approach for the patient.