Tricuspid atresia natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor-In-Chief:Sara Zand, M.D.[2] Keri Shafer, M.D. [3] Priyamvada Singh, MBBS [4] Assistant Editor-In-Chief: Kristin Feeney, B.S. [5]
Natural History, Complications and Prognosis
- Early clinical features in infants include cyanosis of lips and tongue, difficulty in breathing, tiring easily during feeding.
- The severity of cyanosis ininfants with pulmonary stenosis is dependent on the amount of pulmonary blood flow passing throughpatent ductus arteriosus.
- After physiologic closure of patent ductus arteriosus (PDA), the cyanosis will be aggravated.
- In patients with normal pulmonary blood flow, complications of heart failure may occur.
- Prognosis is generally poor without surgery and 90% of patients will die before 10 years old.
- The 15-year survival of patients with Fontan procedure is approximately %92 according to Merry et al.
- Complications of Fontan procedure include:
- Exercise intolerance due to ventricular failure
- Pericardial effusion andpleural effusion, chylothorax and protein-losing enteropathy due to lymphatic dysfunction
- Pulmonary embolism, blood clot formation in shunt
- Liver failure and portal hypertension as a result of increased pressure in the shunt
- leakage of theanastomosis
- Pulmonary hypertension
- Right atrium dilation
- Arrhythmia