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Total Anomalous Pulmonary Venous Connection Medication

  • Author: Allen D Wilson, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
 
Updated: May 02, 2015
 

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.

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Pulmonary Vasodilators

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)

 

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

 

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)

 

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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Contributor Information and Disclosures
Author

Allen D Wilson, MD Professor, Department of Pediatrics, University of Wisconsin School of Medicine and Public Health; Department of Pediatrics, University of Wisconsin Hospital and Clinics

Allen D Wilson, MD is a member of the following medical societies: American College of Cardiology, American Heart Association, American Society of Echocardiography, Society of Pediatric Echocardiography

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

John W Moore, MD, MPH Professor of Clinical Pediatrics, Section of Pediatic Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital

John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Chief Editor

Howard S Weber, MD, FSCAI Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children's Hospital

Howard S Weber, MD, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Received income in an amount equal to or greater than $250 from: St. Jude Medical.

Additional Contributors

Juan Carlos Alejos, MD Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California, Los Angeles, David Geffen School of Medicine

Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, International Society for Heart and Lung Transplantation

Disclosure: Received honoraria from Actelion for speaking and teaching.

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