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]

Overview

Early clinical features in infants include cyanosis of lips and tongue, difficulty in breathing, tiring easily during feeding. The severity of cyanosis in infants with pulmonary stenosis is dependent on the amount of pulmonary blood flow passing through patent 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.

Early clinical features in infants include cyanosis of lips and tongue, difficulty in breathing, tiring easily during feeding.
The severity of cyanosis in infants with pulmonary stenosis is dependent on the amount of pulmonary blood flow passing through patent 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.^[1]
Complications of Fontan procedure include:^[2]
- 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

↑ Mery, Carlos M.; De León, Luis E.; Trujillo-Diaz, Daniel; Ocampo, Elena C.; Dickerson, Heather A.; Zhu, Huirong; Adachi, Iki; Heinle, Jeffrey S.; Fraser, Charles D.; Ermis, Peter R. (2019). "Contemporary Outcomes of the Fontan Operation: A Large Single-Institution Cohort". The Annals of Thoracic Surgery. 108 (5): 1439–1446. doi:10.1016/j.athoracsur.2019.05.039. ISSN 0003-4975.
↑ Fredenburg, Tyler B.; Johnson, Tiffanie R.; Cohen, Mervyn D. (2011). "The Fontan Procedure: Anatomy, Complications, and Manifestations of Failure". RadioGraphics. 31 (2): 453–463. doi:10.1148/rg.312105027. ISSN 0271-5333.