Hostname: page-component-78c5997874-ndw9j Total loading time: 0 Render date: 2024-11-02T20:00:47.176Z Has data issue: false hasContentIssue false
  • English
  • Français

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]
Get access Rights & Permissions [Opens in a new window]

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 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Hoffman, JI, Kaplan, S. The incidence of congenital heart disease. J Am Coll Cardiol 2002; 39: 1890–1900.CrossRefGoogle ScholarPubMed
Egbe, A, Uppu, S, Stroustrup, A, Lee, S, Ho, D, Srivastava, S. Incidences and sociodemographics of specific congenital heart diseases in the United States of America: an evaluation of hospital discharge diagnoses. Pediatr Cardiol 2014; 35: 975–982.CrossRefGoogle ScholarPubMed
Egbe, A, Uppu, S, Lee, S, Ho, D, Srivastava, S. Changing prevalence of severe congenital heart disease: a population-based study. Pediatr Cardiol 2014; 35: 1232–1238.CrossRefGoogle ScholarPubMed
Reddy, VM, McElhinney, DB, Amin, Z, et al. Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 85 patients. Circulation 2000; 101: 1826–1832.CrossRefGoogle ScholarPubMed
Watanabe, N, Mainwaring, RD, Reddy, VM, Palmon, M, Hanley, FL. Early complete repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. Ann Thorac Surg 2014; 97: 909–915; discussion 914–915.CrossRefGoogle ScholarPubMed
Patrick, WL, Mainwaring, RD, Reinhartz, O, Punn, R, Tacy, T, Hanley, FL. Major aortopulmonary collateral arteries with anatomy other than Pulmonary Atresia/Ventricular Septal Defect. Ann Thorac Surg 2017; 104: 907–916.CrossRefGoogle ScholarPubMed
Malhotra, SP, Hanley, FL. Surgical management of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a protocol-based approach. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2009; 1: 145–151.CrossRefGoogle Scholar
Mainwaring, RD, Patrick, WL, Ma, M, Hanley, FL. An analysis of patients requiring unifocalization revision following midline unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. Eur J Cardiothorac Surg 2018; 54: 63–70.CrossRefGoogle ScholarPubMed
Mainwaring, RD, Reddy, VM, Peng, L, Kuan, C, Palmon, M, Hanley, FL. Hemodynamic assessment after complete repair of pulmonary atresia with major aortopulmonary collaterals. Ann Thorac Surg 2013; 95: 1397–1402.CrossRefGoogle ScholarPubMed
Mainwaring, RD, Reddy, VM, Perry, SB, Peng, L, Hanley, FL. Late outcomes in patients undergoing aortopulmonary window for pulmonary atresia/stenosis and major aortopulmonary collaterals. Ann Thorac Surg 2012; 94: 842–848.CrossRefGoogle ScholarPubMed
Carrillo, SA, Mainwaring, RD, Patrick, WL, et al. Surgical Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals With Absent Intrapericardial Pulmonary Arteries. Ann Thorac Surg 2015;100:606–614.CrossRefGoogle ScholarPubMed
Khairy, P, Harris, L, Landzberg, MJ, et al. Implantable cardioverter-defibrillators in tetralogy of Fallot. Circulation 2008; 117: 363–370.CrossRefGoogle ScholarPubMed
Khairy, P, Aboulhosn, J, Gurvitz, MZ , et al. Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study. Circulation 2010; 122: 868–875.CrossRefGoogle ScholarPubMed
Valente, AM, Gauvreau, K, Assenza, GE, et al. Contemporary predictors of death and sustained ventricular tachycardia in patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort. Heart 2014; 100: 247–253.CrossRefGoogle ScholarPubMed
Bokma, JP, Geva, T, Sleeper, LA, et al. A propensity score-adjusted analysis of clinical outcomes after pulmonary valve replacement in tetralogy of Fallot. Heart 2018; 104: 738–744.CrossRefGoogle ScholarPubMed
Bokma, JP, Winter, MM, Vehmeijer, JT, et al. QRS fragmentation is superior to QRS duration in predicting mortality in adults with tetralogy of Fallot. Heart 2017; 103: 666–671.CrossRefGoogle ScholarPubMed
Bokma, JP, Winter, MM, Oosterhof, T, et al. Preoperative thresholds for mid-to-late haemodynamic and clinical outcomes after pulmonary valve replacement in tetralogy of Fallot. Eur Heart J 2016; 37: 829–835.CrossRefGoogle ScholarPubMed
Bokma, JP, Winter, MM, Oosterhof, T, et al. Pulmonary Valve Replacement After Repair of Pulmonary Stenosis Compared With Tetralogy of Fallot. J Am Coll Cardiol 2016; 67: 1123–1124.CrossRefGoogle ScholarPubMed