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Prediction of heart failure and death in an adult population of Fontan patients

Published online by Cambridge University Press:  30 April 2019

Jan Thorben Sieweke Open the ORCID record for Jan Thorben Sieweke [Opens in a new window] ,
Arash Haghikia ,
Christian Riehle ,
Carolin Klages ,
Muharrem Akin ,
Tobias König ,
Carolin Zwadlo ,
Jens Treptau ,
Andreas Schäfer  and
Johann Bauersachs
...Show all authors
Jan Thorben Sieweke
Affiliation:
Department of Cardiology and Angiology, Hannover Medical School, Hannover, Germany
Arash Haghikia
Affiliation:
Department of Cardiology, Charité Universitätsmedizin Berlin, Campus Benjamin Franklin, Berlin, Germany
Christian Riehle
Affiliation:
Department of Cardiology and Angiology, Hannover Medical School, Hannover, Germany
Carolin Klages
Affiliation:
Department of Cardiology and Angiology, Hannover Medical School, Hannover, Germany
Muharrem Akin
Affiliation:
Department of Cardiology and Angiology, Hannover Medical School, Hannover, Germany
Tobias König
Affiliation:
Department of Cardiology and Angiology, Hannover Medical School, Hannover, Germany
Carolin Zwadlo
Affiliation:
Department of Cardiology and Angiology, Hannover Medical School, Hannover, Germany
Jens Treptau
Affiliation:
Department of Cardiology and Angiology, Hannover Medical School, Hannover, Germany
Andreas Schäfer
Affiliation:
Department of Cardiology and Angiology, Hannover Medical School, Hannover, Germany
Johann Bauersachs
Affiliation:
Department of Cardiology and Angiology, Hannover Medical School, Hannover, Germany
Mechthild Westhoff-Bleck*
Affiliation:
Department of Cardiology and Angiology, Hannover Medical School, Hannover, Germany
*
Author for correspondence: Mechthild Westhoff-Bleck, MD, Department of Cardiology and Angiology, Hannover Medical School, Carl-Neuberg-Straße 1, 30625 Hannover, Germany. Tel: +49 511 532 2532; Fax: +49 511 532 9435; E-mail: [email protected]
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Abstract

Background:

Late Fontan survivors are at high risk to experience heart failure and death. Therefore, the current study sought to investigate the role of non-invasive diagnostics as prognostic markers for failure of the systemic ventricle following Fontan procedure.

Methods:

This monocentric, longitudinal observational study included 60 patients with a median age of 24.5 (19–29) years, who were subjected to cardiac magnetic resonance imaging, echocardiography, cardiopulmonary exercise testing, and blood analysis. The primary endpoint of this study was decompensated heart failure with symptoms at rest, peripheral and/or pulmonary edema, and/or death.

Results:

During a follow-up of 24 months, 5 patients died and 5 patients suffered from decompensated heart failure. Clinical (NYHA class, initial surgery), functional (VO2 peak, ejection fraction, cardiac index), circulating biomarkers (N-terminal pro brain natriuretic peptide), and imaging parameters (end diastolic volume index, end systolic volume index, mass-index, contractility, afterload) were significantly related to the primary endpoint. Multi-variate regression analysis identified afterload as assessed by cardiac magnetic resonance imaging as an independent predictor of the primary endpoint (hazard ratio 1.98, 95% confidence interval 1.19–3.29, p = 0.009).

Conclusion:

We identified distinct parameters of cardiopulmonary exercise testing, cardiac magnetic resonance imaging, and blood testing as markers for future decompensated heart failure and death in patients with Fontan circulation. Importantly, our data also identify increased afterload as an independent predictor for increased morbidity and mortality. This parameter is easy to assess by non-invasive cardiac magnetic resonance imaging. Its modulation may represent a potential therapeutic approach target in these high-risk patients.

Keywords

Fontanuniventricular heartcongenital heart diseasecardiac magnetic resonance imagingheart failure
Type
Original Article
Information
Cardiology in the Young , Volume 29 , Issue 5 , May 2019 , pp. 602 - 609
Copyright
© Cambridge University Press 2019 

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