European Heart Journal Advance Access published February 23, 2016
CARDIOVASCULAR FLASHLIGHT
doi:10.1093/eurheartj/ehw032
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Drowned and saved: native pulmonary vasculature protection in an unoperated adult with tetralogy of Fallot and pulmonary atresia Marzia Rigolli1 and Boris S. Lowe2* 1 Division of Cardiology, Department of Medicine, University of Verona, Verona, Italy; and 2Green Lane Cardiovascular Service, Auckland City Hospital, 2 Park Road, Grafton, Private Bag 92024, Auckland 1142, New Zealand
* Corresponding author. Tel: +64 93670000, Fax: +64 93074950, Email: [email protected]
Supplementary material is available at European Heart Journal online. Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2016. For permissions please email: [email protected].
Downloaded from http://eurheartj.oxfordjournals.org/ by guest on March 10, 2016
A 33-year-old woman arrived from Fiji presenting with dyspnoea, orthopnoea, and peripheral oedema for 2 months. The patient self-reported congenital heart disease diagnosed at infancy but was not under medical surveillance. She had a sedentary lifestyle with long-standing intolerance of vigorous exercise. An echocardiogram suggested tetralogy of Fallot (TOF), although surprisingly there was no cyanosis and mild digital clubbing with oxygen saturation 89%. Cardiovascular magnetic resonance (CMR) imaging confirmed an anterior malaligned ventricular septal defect, overriding aorta, and infundibular pulmonary atresia rather than stenosis (Panels A and B). The left and right ventricular ejection fractions were 51 and 41%, respectively. Furthermore, CMR revealed multiple tortuous vessels arising predominantly from the descending aorta to hypoplastic pulmonary arteries bilaterally (Panels C and D). The major aorto-pulmonary collateral arteries (MAPCAs) had stenoses or were diminutive caliber (Supplementary material online, video), protecting the pulmonary circulation from high systemic pressures and subsequent pulmonary vascular occlusive disease. Infants born with TOF, pulmonary atresia, and MAPCAs have unpredictable presentations owing to anatomic variability of the lesion. Minimal collateralization is associated with early severe cyanosis. Patients with extensive collateralization can be minimally symptomatic but may develop heart failure due to pulmonary overcirculation or progressive cyanosis due to pulmonary flow paucity. This patient’s ‘balanced’ circulation with restrictive but sufficient MAPCAs likely explain the milder clinical course than would have been expected in unoperated patients with the most extreme variant of TOF. A conservative approach was adopted because of uncertainty if the native pulmonary artery vasculature could be maximized further at this late stage with complex multiple operations.
CARDIOVASCULAR FLASHLIGHT
doi:10.1093/eurheartj/ehw032
.............................................................................................................................................................................
Drowned and saved: native pulmonary vasculature protection in an unoperated adult with tetralogy of Fallot and pulmonary atresia Marzia Rigolli1 and Boris S. Lowe2* 1 Division of Cardiology, Department of Medicine, University of Verona, Verona, Italy; and 2Green Lane Cardiovascular Service, Auckland City Hospital, 2 Park Road, Grafton, Private Bag 92024, Auckland 1142, New Zealand
* Corresponding author. Tel: +64 93670000, Fax: +64 93074950, Email: [email protected]
Supplementary material is available at European Heart Journal online. Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2016. For permissions please email: [email protected].
Downloaded from http://eurheartj.oxfordjournals.org/ by guest on March 10, 2016
A 33-year-old woman arrived from Fiji presenting with dyspnoea, orthopnoea, and peripheral oedema for 2 months. The patient self-reported congenital heart disease diagnosed at infancy but was not under medical surveillance. She had a sedentary lifestyle with long-standing intolerance of vigorous exercise. An echocardiogram suggested tetralogy of Fallot (TOF), although surprisingly there was no cyanosis and mild digital clubbing with oxygen saturation 89%. Cardiovascular magnetic resonance (CMR) imaging confirmed an anterior malaligned ventricular septal defect, overriding aorta, and infundibular pulmonary atresia rather than stenosis (Panels A and B). The left and right ventricular ejection fractions were 51 and 41%, respectively. Furthermore, CMR revealed multiple tortuous vessels arising predominantly from the descending aorta to hypoplastic pulmonary arteries bilaterally (Panels C and D). The major aorto-pulmonary collateral arteries (MAPCAs) had stenoses or were diminutive caliber (Supplementary material online, video), protecting the pulmonary circulation from high systemic pressures and subsequent pulmonary vascular occlusive disease. Infants born with TOF, pulmonary atresia, and MAPCAs have unpredictable presentations owing to anatomic variability of the lesion. Minimal collateralization is associated with early severe cyanosis. Patients with extensive collateralization can be minimally symptomatic but may develop heart failure due to pulmonary overcirculation or progressive cyanosis due to pulmonary flow paucity. This patient’s ‘balanced’ circulation with restrictive but sufficient MAPCAs likely explain the milder clinical course than would have been expected in unoperated patients with the most extreme variant of TOF. A conservative approach was adopted because of uncertainty if the native pulmonary artery vasculature could be maximized further at this late stage with complex multiple operations.
