Pulmonary function and exercise testing in adult survivors of congenital diaphragmatic hernia

Abstract

Congenital diaphragmatic hernia (CDH) is accompanied by pulmonary hypoplasia and structural abnormalities of the pulmonary vascular bed. It is unknown whether pulmonary function, exercise capacity, and gas exchange during exercise are impaired in adult CDH survivors. The objective of this study was to assess the long-term pulmonary function, exercise capacity, and gas exchange during exercise and relate these findings with quality of life. Of the 23 patients eligible for this study, 12 adult CDH survivors (mean age, 24.3 +/- 4.1 years) with high-risk CDH agreed to participate. Pulmonary function tests, diffusion capacity, and a cardiopulmonary exercise test (CPET) were performed. The FEV1 (mean z-score +/- SD; -1.30 +/- 1.37), FEF25-75% (-1.49 +/- 1.14), and the KCO (-1.03 +/- 1.24) were found to be lower in CDH survivors. The RV/TLC ratio (28.2% +/- 5.0%) was found to be higher. Despite these abnormalities, percent predicted work load (102% +/- 17.2%) and percent predicted maximal oxygen uptake (90.8% +/- 18.9%) were normal in most of the patients. The quality of life of CDH survivors, assessed with the SF-36 questionnaire, is comparable to the general population. Comparison of participants to non-participants did not reveal significant differences in clinical characteristics. In this first study assessing pulmonary function in adult survivors of CDH, mild airway obstruction was observed in most of the patients together with a slightly reduced diffusion capacity for CO. Exercise capacity and gas exchange parameters were normal in this group, indicating that patients do not have a physical impairment, as reflected by a normal quality of life of CDH patients.

Serial Perfusion Study Depicts Pulmonary Vascular Growth in the Survivors of Non-Extracorporeal Membrane Oxygenation-Treated Congenital Diaphragmatic Hernia

“…It is largely believed that alveolar function shows rapid improvement whereas vascular growth lags behind significantly [4,[7][8][9] . However, marked clinical improvement in respiratory symptoms, absence of significant ventilation-perfusion mismatch and its sequel in the form of pulmonary hypertension in the majority of survivors contradicts this finding [5,10,11] . Only a few studies have shown improved vascular remodeling parallel with the alveolar growth of hypoplastic lung in CDH survivors [12][13][14] .…”

The long-term follow-up of patients with a congenital diaphragmatic hernia: a broad spectrum of morbidity

“…So far there is only one study describing potential consequences in adulthood. Exercise capacity and gas exchange parameters were normal in all 12 adult CDH survivors, indicating these patients have a normal pulmonary vascular bed [90].…”

Fusionless procedures for the management of early-onset spine deformities in 2011: What do we know?

“…Similarly, fixed airway obstruction, such as subglottic stenosis, can produce a secondary pectus excavatum deformity, presumably as a result of long-term increased intrathoracic pressures during inspiration [47]. Up to 27% of children with congenital diaphragmatic hernia and unilateral lung hypoplasia develop scoliosis concave to the hypoplastic side by adulthood [42,48]. Whether this is related to the thoracotomy performed to correct the diaphragm defect early in life or the degree of unilateral lung hypoplasia or both is unclear.…”