Pediatric Tricuspid Atresia Medication

Updated: Jan 17, 2017
  • Author: Syamasundar Rao Patnana, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
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Medication Summary

Congenital structural malformations such as tricuspid atresia cannot be corrected with drug therapy. However, some of the pathophysiologic abnormalities are amenable to treatment, and some functions can improve.

Neonates with arterial hypoxemia, pulmonary oligemia, and ductal-dependent pulmonary flow can experience improvement with intravenous administration of alprostadil (PGE1). PGE1 helps to dilate the ductus arteriosus with resultant augmentation of pulmonary blood flow and improvement in systemic arterial oxygen saturation. This effect allows for stabilization of the neonate's condition so that a detailed workup can be performed and important issues regarding management can be defined. PGE1 is effective only in the neonatal period; therefore, a more permanent solution involving a Blalock-Taussig type of shunt should be offered to resolve pulmonary oligemia.

In neonates with severe aortic coarctation, PGE1 may bypass the aortic obstruction by opening the ductus. After the patient's condition is stabilized, surgical repair of coarctation may be undertaken. An alternative approach is balloon angioplasty, especially if the coarctation is discrete. If balloon angioplasty is contemplated on an urgent basis, PGE1 should not be administered.

In the presence of congestive heart failure, decongestive medications such as digoxin, furosemide, and afterload-reducing agents may be administered. Angiotensin-converting enzyme inhibitors reduce afterload and are generally thought to be useful in augmenting left ventricular function, especially after a bidirectional Glenn procedure and Fontan operation. Anticoagulants and platelet-inhibiting drugs are thought to be useful in preventing thrombus formation after a Fontan operation.

The American Heart Association recommends that prophylaxis with antibiotics be given before any bacteremia-producing procedures are performed. For more information, see Antibiotic Prophylactic Regimens for Endocarditis.



Class Summary

These agents are used to dilatate the ductus. Alprostadil (PGE1) is used for treatment of ductal-dependent cyanotic congenital heart disease, which is due to decreased pulmonary blood flow.

Alprostadil IV (Prostin VR)

Used to open ductus in young neonates with ductal-dependent pulmonary flow or severe aortic coarctation. First-line palliative therapy to temporarily maintain patency of ductus arteriosus prior to surgery. Produces vasodilation and increases cardiac output. Inhibits platelet aggregation and stimulates intestinal and uterine smooth muscle. Each 1-mL ampule contains 500 mcg/mL.