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Long-term outcomes in adults with repaired tetralogy of Fallot and pulmonary atresia

Published online by Cambridge University Press:  10 July 2019

Alexander C. Egbe Open the ORCID record for Alexander C. Egbe [Opens in a new window] ,
Juan Crestanello ,
Joseph A. Dearani ,
Karim Osman ,
Keerthana Banala ,
Mounika Angirekula ,
Maria Najam  and
Naser M. Ammash
Alexander C. Egbe*
Affiliation:
Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN 55905, USA
Juan Crestanello
Affiliation:
Department of Cardiovascular Surgery, Mayo Clinic, Rochester, MN 55905, USA
Joseph A. Dearani
Affiliation:
Department of Cardiovascular Surgery, Mayo Clinic, Rochester, MN 55905, USA
Karim Osman
Affiliation:
Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN 55905, USA
Keerthana Banala
Affiliation:
Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN 55905, USA
Mounika Angirekula
Affiliation:
Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN 55905, USA
Maria Najam
Affiliation:
Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN 55905, USA
Naser M. Ammash
Affiliation:
Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN 55905, USA
*
Author for correspondence: A. C. Egbe, MD MPH, FACC Mayo Clinic and Foundation, 200 First Street SW Rochester, MN 55905, USA. Tel: +507-284-2520; Fax: +507-266-0103; E-mail: [email protected]
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Abstract

Background:

There are limited outcome data in adults with tetralogy of Fallot and pulmonary atresia. The purpose of this study was to describe re-operations and all-cause mortality in adults with tetralogy of Fallot and pulmonary atresia.

Methods:

Retrospective review of adults with repaired tetralogy of Fallot and pulmonary atresia who received care at the Mayo Adult Congenital Heart Disease Clinic, 1990–2016. All-cause mortality was calculated as events per 100 patient-years from the time of first presentation to the Adult Congenital Heart Disease Clinic.

Results:

Of the 221 patients, the age at initial tetralogy of Fallot repair was 6 (5–13) years, and the age at first presentation to the clinic was 27 – 8 years. All patients had at least one right ventricular to pulmonary artery conduit re-operation. There were 31 deaths (14%) at mean age of 41 – 14 years. The causes of death were end-stage heart failure (n = 17), sudden cardiac death (n=9), post-operative death after cardiac surgery (n = 2), sepsis with multi-system organ failure (n = 2), and unknown (n = 1). All-cause mortality rate was 1.7 per 100 patient-years. The risk factors for all-cause mortality were older age (>12 years) at the time of repair (hazard ratio 1.41, 95 confidence interval 1.06–2.02, p = 0.033), non-sustained ventricular tachycardia (hazard ratio 1.36, 95 confidence interval 1.17–2.47, p = 0.015), and left ventricular ejection fraction <50% (hazard ratio 1.39, 95 confidence interval 1.08–2.31, p = 0.031).

Conclusion:

Based on a review of 221 adults with repaired tetralogy of Fallot and pulmonary atresia, all patients had re-operations and all-cause mortality rate was 1.7 events per 100 patient-years. The current study provides important outcomes data for risk stratification in adults with tetralogy of Fallot and pulmonary atresia.

Keywords

Tetralogy of Fallotpulmonary atresiamortalityre-operation
Type
Original Article
Information
Cardiology in the Young , Volume 29 , Issue 8 , August 2019 , pp. 1078 - 1081
Copyright
© Cambridge University Press 2019 

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