Open Access

ARTICLE

A restrictive ventilatory pattern is common in patients with univentricular heart after Fontan palliation and associated with a reduced exercise capacity and quality of life

Alessia Callegari^1,2,4, Rhoia Neidenbach², Ornella Milanesi¹, Biagio Castaldi¹, Martin Christmann⁴, Masamichi Ono³, Jan Müller², Peter Ewert², Alfred Hager²

1 Department of Women’s and Children’s Health, Pediatric Cardiology, University of Padua, Padua, Italy
2 Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technical University of Munich, München, Germany
3 Department of Cardiovascular Surgery, Deutsches Herzzentrum München, Technical University of Munich, München, Germany
4 Pediatric Cardiology, Pediatric Heart Center, Department of Surgery, University Children’s Hospital Zurich, Zurich, Switzerland

* Corresponding Author: Alessia Callegari, Pediatric Heart Center, Department of Surgery, University Children’s Hospital, Steinwiesstrasse 75, 8032 Zurich, Switzerland. Email:

Congenital Heart Disease 2019, 14(2), 147-155. https://doi.org/10.1111/chd.12694

Abstract

Aim: The Fontan circulation is highly dependent on ventilation, improving pulmonary blood flow and cardiac output. A reduced ventilatory function is reported in these patients. The extent of this impairment and its relation to exercise capacity and qual‐ ity of life is unknown and objective of this study.
Methods: This multicenter retrospective/cross‐sectional study included 232 patients (140 females, age 25.6 ± 10.8 years) after Fontan palliation (19.8% atrioventricular connection; 20.3% atriopulmonary connection; 59.9% total cavopulmonary connec‐ tion). Resting spirometry, cardiopulmonary exercise tests, and quality‐of‐life assess‐ ment (SF‐36 questionnaire) were performed between 2003 and 2015.
Results: Overall, mean forced expiratory volume in one second (FEV₁) was 74.7 ± 17.8%predicted (%pred). In 59.5% of the patients, FEV₁ was <80%pred., and all of these patients had FEV₁/forced vital capacity (FVC) > 80%, suggestive of a restric‐ tive ventilatory pattern. Reduced FEV₁ was associated with a reduced peakVO₂ of 67.0 ± 17.6%pred. (r = 0.43, P < .0001), even if analyzed together with possible con‐ founding factors (sex, BMI, age, years after palliation, number of interventions, sco‐ liosis, diaphragmatic paralysis). Synergistically to exercise capacity, FEV₁ was associated to quality of life in terms of physical component summary (r = 0.30, P = .002), physical functioning (r = 0.25, P = .008), bodily pain (r = 0.22, P = .02), and general health (r = 0.16, P = .024). Lower FEV₁ was associated with diaphragmatic paralysis (P = .001), scoliosis (P = .001), higher number of interventions (P = .002), and lower BMI (P = .01). No correlation was found to ventricular morphology, type of surgeries, or other perioperative/long‐term complications.
Conclusions: This study shows that the common restrictive ventilatory pattern in Fontan patients is associated with lower exercise capacity and quality of life. Risk factors are diaphragmatic paralysis, scoliosis, a high total number of interventions and low BMI.

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