Pediatric Cardiology

, Volume 37, Issue 2, pp 304–312 | Cite as

Use of Pulmonary Hypertension Medications in Patients with Tetralogy of Fallot with Pulmonary Atresia and Multiple Aortopulmonary Collaterals

  • Elena K. GrantEmail author
  • John T. Berger
Original Article


Tetralogy of Fallot (TOF) with pulmonary atresia (PA) and multiple aortopulmonary collaterals (MAPCAs) is a rare and severe form of congenital heart disease with poor prognosis. Aortopulmonary collaterals expose pulmonary arterioles to systemic pressure resulting in pulmonary hypertension (PH). To date, reports regarding the role of PH medications in this population are sparse. The objective of this study was to assess the effect of PH medications in patients with TOF, PA and MAPCAs or similar anatomy, with emphasis on symptoms, echocardiography and invasive hemodynamics. A retrospective review was performed for patients at a single tertiary care pediatric center. Twelve of 66 patients were treated with PH medications (18 %), and eight of these patients had adequate follow-up for further analysis. Median age at last follow-up was 6 years (range 1.4–21 years). Median length of therapy with PH medication was 4 years (range 0.3–17 years). PH medications included sildenafil, bosentan, ambrisentan, inhaled treprostinil and prostacyclin infusion. PH therapy was associated with improvement in symptoms in all patients and improvement in PH by hemodynamic measures in the majority of patients. All patients underwent at least one cardiac intervention by catheterization or surgery while taking PH medication. Two patients died from non-PH-related causes. The remaining six patients are alive and remain on PH medication. This review indicates that PH medications are well tolerated by this patient group and provide symptomatic improvement. Further studies are required to determine whether PH medications provide long-term survival benefit for patients with complex congenital heart disease.


Pulmonary hypertension Tetralogy of Fallot Pulmonary atresia Aortopulmonary collaterals 



Extracorporeal membrane oxygenation


Femoral artery


Left pulmonary artery


Multiple aortopulmonary collaterals


Main pulmonary artery


Pulmonary atresia


Pulmonary hypertension


Pulmonary vascular resistance


Right pulmonary artery


Right ventricle


Right ventricle to femoral artery


Right ventricle to the pulmonary arteries


Tetralogy of Fallot


Ventricular septal defect


Compliance with ethical standards

Conflicts of interest



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Copyright information

© Springer Science+Business Media New York 2015

Authors and Affiliations

  1. 1.Division of CardiologyChildren’s National Health SystemWashingtonUSA
  2. 2.Division of Critical Care MedicineChildren’s National Health SystemWashingtonUSA

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