Medication Summary

Newborns or patients in early infancy with obstructed total anomalous pulmonary venous connection (TAPVC) frequently have pulmonary edema with varying degrees of increase in pulmonary arterial and venous resistance. Pulmonary edema is probably treated best with surgical relief of the pulmonary venous obstruction, but diuretics and assisted ventilation with high fraction of inspired oxygen (FIO₂) and end-expiratory pressure are often helpful preoperatively and postoperatively.

Class Summary

When sustained severe cyanosis or severe hypercyanotic episodes occur in patients with obstructed total anomalous pulmonary venous connection postoperatively, treatment with one or more pulmonary vasodilators may be helpful. The pulmonary vascular bed may be somewhat reactive in the postoperative period, resulting in episodic pulmonary hypertension and low cardiac output. Although this should improve over time, interim therapy with pulmonary vasodilator agents may be useful in this setting. Vasodilators that are specific for the pulmonary vasculature are rare; however, inhaled nitric oxide may be a good agent in this setting. Therefore, the following 3 vasodilators can be used to treat elevated pulmonary vascular resistance in the postoperative period. Note that this therapy is controversial in the preoperative patient with known pulmonary venous obstruction.

Nitric oxide, inhaled (INOmax)

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Stimulates guanylate cyclase to form cyclic GMP, which causes relaxation of vascular smooth muscle.

Because it can be delivered by inhalation directly to alveolar units and is rapidly inactivated by hemoglobin, it is the most selective of currently available pulmonary vascular dilators (except for oxygen). Requires an inhalation delivery system (not available everywhere); approved for use in children in December 1999.

Magnesium sulfate

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Reportedly useful in patients with obstructed TAPVC who have hypercyanotic episodes to decrease pulmonary vascular resistance and decrease pulmonary vascular reactivity. Mechanism of action is believed to be direct action on vascular muscle cells but may also increase formation or release of NO. MgSO₄ has systemic and pulmonary vascular dilating effects, and use of a slow infusion of lower-dose MgSO₄ is wise to avoid systemic hypotension.

Alprostadil IV (Prostin VR)

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Prostaglandin E₁ (PGE₁) that causes dilation of vascular smooth muscle in the ductus arteriosus, systemic arteries, and pulmonary vascular muscles. In obstructed TAPVC, PGE₁ is usually used as a pulmonary vascular dilator, but its effects on the ductus arteriosus and ductus venosus can be very important (eg, in subdiaphragmatic connection, PGE₁ can help dilate the ductus venosus and improve pulmonary venous flow. In other types of connection with obstruction, PGE₁ can dilate pulmonary arteries and increase pulmonary flow or dilate the ductus arteriosus and systemic arteries and increase right-to-left shunting and worsen cyanosis). PGE₁ is readily available and easily administered, preferably via a large vessel. Care must be taken to observe its effects in the complex circulation of TAPVC.

Each 1-mL ampule contains 500 mcg/mL.

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