Follow up of TCPC patients

Fontan start to do this operation from 1971 for a patient with tricuspid atresia and after that it was done for patients with univentricular heart. Nowadays modification of this operation is routinely done in many centers in a single or two stage operations. The surgical techniques are: atriopulmonary Fontan, cavopulmonary connection was first described by de Leval in 1988, Fenestration and since the year 2000, the majority of patients undergoing a Fontan procedure have an extracardiac conduit placed. After Fontan procedure, patients face substantial morbidity and mortality risk and require lifelong follow-up with a cardiologist experienced in the care of patients with complex congenital heart disease. Postoperative 15- to 20 year survival rates after Fontan procedure range from 60 to 85 percent. Predictors of all-cause mortality or transplantation included: a history of hypoplastic left heart syndrome, elevated right atrial/central venous pressures, and a history of protein-losing enteropathy. Survival for patients with hypoplastic left heart syndrome who undergo a Fontan operation ranges from 72 to 85 percent at 10 years. During long-term follow-up, Fontan survivors commonly require surgical or catheter-based reintervention. In a series of 773 patients who underwent Fontan procedure, freedom from reoperation was 69 percent at 15 years and 63 percent at 20 years; the most common operations were permanent pacemaker placement and Fontan revision. Freedom from catheter-based intervention was 53 percent at 15 years and 50 percent at 20 years; the most common catheter-based procedures were fenestration closure and pulmonary artery intervention. Annual clinical evaluation by a specialist is recommended at a minimum. Patients with complications following Fontan operation require more frequent follow-up; the frequency of follow-up and monitoring is individualized. New symptoms that warrant investigation after Fontan operation include: palpitations, dyspnea, fatigue, diarrhea, and symptoms of edema or ascites. Such symptoms may be related to arrhythmias, ventricular dysfunction, valve dysfunction, protein-losing enteropathy, liver disease, or more complex conditions. The signs are: Edema, cyanosis, DVT, ascites, chest collaterals, hepatomegaly, and etc. Annual laboratory testing including hematology, renal function (including urine for protein content), electrolyte assessment, and serum albumin and total protein are recommended. Starting five years after Fontan operation, annual laboratory testing should also include assessment of liver structure and function. Annual ECG, CXR and echocardiography is generally suggested. If echocardiography results are inadequate or inconclusive they may need cath., CT and MRI. Cardiopulmonary exercise testing is performed regularly and consider overnight oximetry to screen for obstructive sleep apnea.