Tricuspid atresia medical therapy

Tricuspid atresia Microchapters
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Keri Shafer, M.D. [2] Priyamvada Singh, MBBS [3]; Assistant Editor-In-Chief: Kristin Feeney, B.S. [4]

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Medical Therapy

Treatment Medical Therapy

• The mainstay of therapy for the cyanotic neonate with severe PS and small VSD is using prostaglandin E1 (PGE1) for keeping patency of ductus arteriosis.
• The mainstay of therapy for heart failure symptoms is using diuretic for reloading and then starting ACEI.

Surgery Surgery is the mainstay of therapy for tricuspid atresia. · In the first 8 weeks of life if there are severe cyanosis and pulmonary obstruction and normal positioning aorta and pulmonary artery, making a shunt between systemic subclavian artery to pulmonary artery is necessary which is called Blalock -Taussig(BT shunt). · If the pulmonary artery comes from the left ventricle and is overflowed, PA banding is useful for lowering the pulmonary blood flow. · In older children, bi-direction Glenn shunt which is the connection between superior vena cava to the pulmonary artery will be done for transferring the blood to the pulmonary system. Fontan procedure is a conduit between the inferior vena cava and the pulmonary artery and transfers the systemic venous blood to pulmonary circulation in the age of 2-3 years old. Endocarditis prophylaxis before every procedure is recommended.

Class I

"1. Warfarin should be given for patients who have a documented atrial shunt, atrial thrombus, atrial arrhythmias, or a thromboembolic event. (Level of Evidence: C)"

Class IIa

"1. It is reasonable to treat systemic ventricular dysfunction with angiotensin-converting enzyme (ACE) inhibitors and diuretics. (Level of Evidence: C)"