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Pulmonary vasodilator therapy in tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals: case series and review of literature

Published online by Cambridge University Press:  08 August 2017

Sotiria C. Apostolopoulou ,
George Vagenakis  and
Spyridon Rammos
Sotiria C. Apostolopoulou*
Affiliation:
Department of Pediatric Cardiology, Onassis Cardiac Surgery Center, Athens, Greece
George Vagenakis
Affiliation:
Department of Pediatric Cardiology, Onassis Cardiac Surgery Center, Athens, Greece
Spyridon Rammos
Affiliation:
Department of Pediatric Cardiology, Onassis Cardiac Surgery Center, Athens, Greece
*
Correspondence to: S. C. Apostolopoulou, MD, PhD, Department of Pediatric Cardiology, Onassis Cardiac Surgery Center, 356 Syngrou Ave, Athens, GR 176 74, Greece. Tel: +30-210-949-3865; Fax: +30-210-949-3853; E-mail: riapos@hol.gr
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Abstract

We present the use of pulmonary vasodilators in three adult patients with unrepaired tetralogy of Fallot, pulmonary atresia, aortopulmonary collaterals, and segmental pulmonary arterial hypertension. Patients improved by 1–2 NYHA classes with modest exercise-tolerance increase, and remained stable without side effects during 2.5, 10, and 14 years. Literature review revealed five studies with pulmonary vasodilators in heterogeneous, mostly repaired patient populations.

Keywords

Bosentanendothelin receptor antagonistsegmental pulmonary arterial hypertension
Type
Brief Report
Information
Cardiology in the Young , Volume 27 , Issue 9 , November 2017 , pp. 1861 - 1864
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Copyright
© Cambridge University Press 2017 

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