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

Abstract

Background: Pulmonary growth in the survivors of congenital diaphragmatic hernia (CDH) still remains an intriguing issue. In the literature there are conflicting reports on pulmonary vascular growth in CDH. Objective: In a cohort of CDH patients treated without extracorporeal membrane oxygenation (ECMO), serial perfusion studies were conducted prospectively to follow the growth of pulmonary vasculature. Material and Methods: Survivors of CDH repair between January 2000 and January 2003 were studied prospectively. Patient demography, time of presentation, arterioalveolar oxygen gradient (A-aDO₂), time to complete lung expansion, and respiratory symptoms were noted. Lung perfusion studies were done at 3 months, 9 months, and 6 years of follow-up. Results: 24 neonates completed the study. Mean presentation was 6.9 h, and preoperative A-aDO₂ was 288.8. 16/24 were managed by conventional ventilation and 8/24 required high-frequency ventilation and nitric oxide therapy (none were treated with ECMO). Mean duration of preoperative stabilization was 26.5 h, time to full lung expansion was 2.8 days, and postoperative ventilation was 3.7 days. Mean perfusion (%) of ipsilateral lung was 33.7% at 3 months, 43.2% at 9 months, and 46.8% at 6 years. Perfusion of ipsilateral lung improved by 9.4% from 3 to 9 months (p < 0.001), 13.1% from 3 months to 6 years (p < 0.001), and 3.7% from 9 months to 6 years postoperatively (p < 0.001). Respiratory symptoms were seen in 45% (11/24) of survivors at 3 months, 14% (4/24) at 9 months, and 12.5% (3/24) at 6 years postoperatively. Conclusions: There was reduced perfusion of affected lung at 3 months which improved significantly at 9 months and at 6 years. Recovery of pulmonary vascular function correlated with amelioration of respiratory symptoms. The majority of survivors showing a significant vascular growth lead a symptom-free life.

Pulmonary support on day of life 30 is a strong predictor of increased 1 and 5-year morbidity in survivors of congenital diaphragmatic hernia

“…(29) The need for ECMO and other predictors of disease severity such as the need for patch repair, have also been associated with ventilation/perfusion mismatches years later. (12, 18–20) The presence of a V/Q mismatch itself has also been demonstrated to be a sensitive predictor of future pulmonary morbidities such as obstructive pulmonary disease. (12, 19) We found that PS on DOL-30 may be at least as good of a predictor of outcome as the presence of V/Q mismatch.…”

“…(11–17) Ventilation and perfusion scans, which are used by some multidisciplinary clinics to measure pulmonary function in CDH patients, have also been shown to be predictive of long-term outcome. Although some studies have shown that early ipsilateral V/Q mismatch can be a sensitive predictor of subsequent pulmonary function in survivors of CDH, (12, 18–20) others suggest that it may lack specificity and conclude that it does not warrant the costs of repeated nuclear medicine evaluation. (21) Given the significant long-term morbidity associated with the disease, many authors have promoted the use of extended multidisciplinary clinics for survivors of CDH.…”

Lung function in young adults with congenital diaphragmatic hernia; a longitudinal evaluation

“…Neonates with CDH are at risk of developing bronchopulmonary dysplasia (BPD) due to ventilator induced injury and high concentrations of oxygen 4, 5. Respiratory morbidity through different stages of life of CDH patients was reported in several cross‐sectional studies 6–12…”

Survival in Congenital Diaphragmatic Hernia: Use of Predictive Equations in the ECMO Population

“…Clinical variables such as hypercapnia and hypoxemia have been described to be predictive of mortality in several series [8][9][10][11][12][13][14][15][16][17][18] . Such parameters that reflect ventilation and oxygenation capacity are key, since the degree of pulmonary hypoplasia is a major determinant of survival as well as long-term pulmonary outcome in patients with CDH [19] . Predictive equations have previously been proposed by the Wilford Hall/Santa Rosa (WHSR) and CDH Study Group (CDHSG) for predicting survival in patients with CDH [20,21] .…”