Research: Cardiopulmonary Morbidity in Adults Born With Congenital Diaphragmatic Hernia


. 2023 Oct 1;152(4):e2023062341.

 doi: 10.1542/peds.2023-062341.

Cardiopulmonary Morbidity in Adults Born With Congenital Diaphragmatic Hernia

Ulrike S Kraemer 1 2Lieke S Kamphuis 3Pierluigi Ciet 4Lidewij Visser 3Dick Tibboel 1Beatrijs Bartelds 5Suzan C M Cochius-den Otter 2Ivo de Blaauw 6Joost van Rosmalen 7Saskia J Gischler 1J Marco Schnater 1Hanneke IJsselstijn 1

Affiliations expand


Objectives: Studies concerning cardiopulmonary outcomes of adults born with congenital diaphragmatic hernia (CDH) are sparse. Moreover, they don’t include participants who have been treated with extracorporeal membrane oxygenation (ECMO) during the neonatal period. This study evaluated the cardiopulmonary morbidities in young adults born with CDH.

Methods: We assessed 68 participants between the ages of 18 and 30 years. The assessment included auxology assessment, lung function tests, pulmonary imaging, cardiopulmonary exercise testing, and echocardiography.

Results: Lung function parameters in the overall group were significantly worse than normal values. Mean (SD) scores postbronchodilator forced expiratory volume in 1 second were -2.91 (1.38) in the ECMO-treated and -1.20 (1.53) in the non-ECMO-treated participants. Chest computed tomography scans showed mild to moderate abnormal lung structure in all ECMO-treated participants, and to a lesser extent in non-ECMO treated participants. A recurrent diaphragmatic defect was observed in 77% of the ECMO-treated group and in 43% of the non-ECMO-treated group. Except for 2 cases with acute symptoms, no clinical problems were noted in cases of recurrence. Cardiopulmonary exercise testing revealed mean (SD) percentage predicted peak oxygen consumption per kilogram of 73 (14)% and 88 (16)% in ECMO-treated and non-ECMO-treated participants, respectively. The mean (SD) workload was normal in the non-ECMO-treated group (111 [25]% predicted); in the ECMO-treated group, it was 89 (23)%. Cardiac evaluation at rest revealed no signs of pulmonary hypertension.

Conclusions: In young adults who survived treatment of CDH, significant pulmonary morbidity, reduced exercise capacity, and frequent hernia recurrence should be anticipated. Lifelong follow-up care, with the emphasis on prevention of further decline, is to be recommended.

Recommended Articles

Translate »