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

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 quality 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 connection). Resting spirometry, cardiopulmonary exercise tests, and quality-of-life assessment (SF-36 questionnaire) were performed between 2003 and 2015.Results: Overall, mean forced expiratory volume in one second (FEV 1 ) was 74.7 ± 17.8%predicted (%pred). In 59.5% of the patients, FEV 1 was <80%pred., and all of these patients had FEV 1 /forced vital capacity (FVC) > 80%, suggestive of a restrictive ventilatory pattern. Reduced FEV 1 was associated with a reduced peakVO 2 of 67.0 ± 17.6%pred. (r = 0.43, P < .0001), even if analyzed together with possible confounding factors (sex, BMI, age, years after palliation, number of interventions, scoliosis, diaphragmatic paralysis). Synergistically to exercise capacity, FEV 1 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 FEV 1 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. K E Y W O R D SCPET, Fontan, quality of life, spirometry, ventilatory function

Better lung function with increased handgrip strength, as well as maximum oxygen uptake, in congenital heart disease across the lifespan

“…UVH and Fallot) was relatively small, and these are the defects most reliably associated with respiratory limitations such as a restrictive ventilatory pattern. 39…”

“…Furthermore, the effect of exercise on respiratory function in CHD patients may differ from that in healthy populations. Surgical and congenital modifications of the chest musculature are associated with a restrictive ventilatory pattern 39 and also often with inability to exercise at the levels which are most beneficial. Thus patients with these defects may be limited by inadequate respiratory function and thus benefit from targeted strength training in the same way as do other populations with lung disease.…”

The “Super-Fontan” Phenotype: Characterizing Factors Associated With High Physical Performance

“…Lung function is commonly impaired in people with a Fontan circulation and is associated with prognosis and exercise capacity ( 58 – 60 ). The majority of our Fontan cohort with baseline spirometry results recorded had evidence of ventilatory restriction or mixed defect.…”

Resting respiratory lung volumes are “healthier” than exercise respiratory volumes in different types of palliated or corrected congenital heart disease

“…Recently, a restrictive ventilator pattern was shown to be associated with reduced exercise tolerance and QoL in patients after Fontan palliation. 10 However, there is a lack of studies focusing on the role of resting respiratory performance in reducing exercise tolerance in patients with CHD.…”