Pulmonary Morbidity in Congenital Diaphragmatic Hernia Survivors Treated at a Non-ECMO Center From 1998 to 2015: A Cross-Sectional Study

Abstract

Introduction: A main feature of CDH is lung hypoplasia and the related presentation of pulmonary hypertension and cardiac dysfunction. Multiple factors influence pulmonary status after CDH: degree of hypoplasia, ventilator-induced injury, altered growth and development of pulmonary structures, reduced diaphragm function and chest wall abnormalities. The evolution of pulmonary sequela in this population is still unclear. We aimed to describe the pulmonary status of our population of CDH-survivors and evaluated on risk factors.

Methods: CDH-survivors (1998-2015) were included and performed lung function tests and chest X-rays.

Results: Fifty-one (51/71, 71.8%) participated. Median age was 12.2 (5.5-21.4) years, 28 (54.9%) male, 42 (82.4%) had left-sided hernias, 10 (19.6%) needed patch-repair and median length of stay in hospital was 28.0 (IQR 18.5-61.6) days in Table 1. Spirometry including bronchodilator response (BDR)-test, body plethysmography, and diffusion capacity, were available for 48, 42, and 40 participants. The mean (SD) z-score for FEV1 and FVC was -0.26 (1.70) and -0.28 (1.70). Twenty-one (43.8%) had obstructive patterns and six had positive BDR. TLC mean (SD) z-score was -0.18 (1.10). Four showed restricted/mixed patterns and 13 showed signs of hyperinflation. Increased RV/TLC-ratio and reduced FEV1 was associated with longer time on mechanical ventilation. Diffusion capacity was decreased in three cases. Chest X-ray revealed hernia recurrence (13.9%) and scoliosis (38.9%).

Conclusion: Mild obstructive impairment and hyperinflation was frequent in our CDH cohort and only a small subset of restrictive disorders were identified. We advocate follow-up by a specialized multidisciplinary team through childhood and into adulthood.

Figure 1

Flowchart of study inclusion.

Figure 2

Classification of ventilatory impairment according to the ERS/ATS technical standards on interpretive strategies for lung function tests [16]. FEV1, forced expiratory volume in first second; FVC, forced vital capacity; LLN, lower limit of normal; TLC, total lung capacity.