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Int J Cardiol. 2015 Oct 1;196:1-6. doi: 10.1016/j.ijcard.2015.05.142. Epub 2015 May 28.

Pulmonary outflow obstruction protects against heart failure in adults with congenitally corrected transposition of the great arteries.

Author information

1
Division of Cardiology, Department of Cardiovascular Sciences, KU Leuven - University of Leuven, B-3000 Leuven, Belgium; Department of Cardiology, University Hospitals Leuven, B-3000 Leuven, Belgium.
2
Department of Cardiology, University Hospitals Leuven, B-3000 Leuven, Belgium.
3
Division of Experimental Cardiac Surgery, Department of Cardiovascular Sciences, KU Leuven - University of Leuven, B-3000 Leuven, Belgium; Department of Cardiac Surgery, University Hospitals Leuven, B-3000 Leuven, Belgium.
4
Department of Cardiac Surgery, University Hospitals Leuven, B-3000 Leuven, Belgium; Division of Clinical Cardiac Surgery, Department of Cardiovascular Sciences, KU Leuven - University of Leuven, B-3000 Leuven, Belgium.
5
Division of Cardiovascular Developmental Biology, Department of Cardiovascular Sciences, KU Leuven - University of Leuven, B-3000 Leuven, Belgium; Department of Paediatrics, University Hospitals Leuven, B-3000 Leuven, Belgium.
6
Division of Cardiology, Department of Cardiovascular Sciences, KU Leuven - University of Leuven, B-3000 Leuven, Belgium; Department of Cardiology, University Hospitals Leuven, B-3000 Leuven, Belgium. Electronic address: werner.budts@uzleuven.be.

Abstract

BACKGROUND:

Pulmonary outflow tract obstruction (POTO) reduces systemic atrioventricular valve (SAVV) regurgitation severity in congenitally corrected transposition of the great arteries (ccTGA). Therefore, pulmonary artery banding is proposed as a palliative intervention. We aimed to investigate the effect of native or surgically induced POTO on event-free survival, defined as the composite of all-cause mortality, heart transplantation, or congestive heart failure (CHF).

METHODS AND RESULTS:

Patients with ccTGA (n=62; median age 27.5 (IQR 18.4-39.4) years; 39% with POTO) were selected from the Adult Congenital Heart Disease database of a tertiary hospital. At first visit, SAVV regurgitation ≥ 3/4, systemic RV dysfunction ≥ moderate, and CHF were present in 26%, 26%, and 15% of patients, respectively. Over a mean follow-up time of 10.1 ± 6.1 years, all-cause mortality, rate of heart transplantation, and CHF were 18%, 8% and 40%, respectively. SAVV regurgitation (HR: 1.99; 95% CI: 1.01-3.92; P=0.048) and systemic RV dysfunction severity (HR: 1.89; 95% CI: 1.05-3.37; P=0.033) were associated with the composite endpoint, independently of age at baseline, POTO, Ebstein-like malformation, and systemic RV dilatation. Patients with POTO had lower risk for developing SAVV regurgitation ≥ 3/4 (HR: 0.18; 95% CI: 0.05-0.58; P=0.004) and moderate systemic RV dysfunction (HR: 0.34; 95% CI: 0.15-0.78; P=0.011). When POTO was present, the mean progression-free interval for the composite endpoint increased from 11.2 to 18.1 years (P=0.035).

CONCLUSIONS:

POTO is associated with an improved event-free survival in adults with ccTGA.

KEYWORDS:

Congenital heart defects; Heart failure; Survival; Transposition of great vessels; Tricuspid valve insufficiency

PMID:
26070176
DOI:
10.1016/j.ijcard.2015.05.142
[Indexed for MEDLINE]

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