Neonatal Pulmonology 1: Lung Development, Control of Breathing
247 - Congenital diaphragmatic Hernia-Qatar (CDH-Q): Five-Year Outcomes of a New Program with a Multinational Population and a High Incidence of Associated Anomalies
Sunday, April 30, 2023
3:30 PM – 6:00 PM ET
Poster Number: 247 Publication Number: 247.339
Moath Alhamad, Sidra Medicine / Weill Cornell Medical College - Qatar, Doha, Ad Dawhah, Qatar; Dhullipala Anand, Sidra Medicine, Doha, Ar Rayyan, Qatar
Attending Neonatologist Sidra Medicine / Weill Cornell Medical College - Qatar Doha, Ad Dawhah, Qatar
Background: Congenital diaphragmatic hernia (CDH) is a complex congenital anomaly with significant morbidity and mortality, which are further increased when it is associated with other congenital and genetic anomalies. Survival varies between centers but is mostly around 70%. Termination of pregnancy (TOP) is common in cases of CDH, especially when other anomalies are present. CDH – Qatar (CDH-Q) program was established in 2018 and receives prenatal and postnatal referrals for all cases of CDH in Qatar. In this study, we report the 5-year outcomes of CDH-Q program, which is characterized by a multinational population with a high incidence of associated anomalies and a low rate of termination of pregnancy (TOP). We also compare these outcomes to the published literature.
Objective: To review and define the characteristics and outcomes of CDH patients in Qatar, and compare these characteristics and outcomes to other centers. Design/Methods: The data from CDH-Q registry between 2018 and 2022 was analyzed and compared to the published data by the CDH study group (CDHSG), the European Surveillance of congenital anomalies (EUROCAT), Florida Birth Defects Registry, and other centers.
Results: 53 cases of CDH from 16 nationalities were referred to CDH-Q, with only one resulting in TOP. For the 52 infants born alive, the median birth gestational age was38 weeks (IQR: 36-39) and the median birth weight was2.8 kg (IQR: 2.5-3.2). 39 infants (75%) were inborn, and 42 infants (81%) were diagnosed antenatally. A unique aspect of CDH-Q is the high rate of associated congenital heart disease (10 infants, 19%), genetic abnormalities (14 infants, 27%), and major non-cardiac congenital anomalies (22 infants, 42%). Despite that, CDH-Q survival rates are comparable to those reported in literature: Excluding 3 infants who are still admitted, overall survival is 69% (34 out of 49 infants), with higher survival among infants who were actively resuscitated at birth (34 out of 46, 74%), infants who underwent surgical repair (34 out of 41 infants, 83%), and infants with isolated CDH (24 out of 31 infants, 77%).
Conclusion(s): The outcomes of CDH-Q program are similar to other centres despite low incidence of TOP and high incidence of associated anomalies. These findings show that CDH outcomes can be improved when patients are referred to a dedicated CDH program, even for the most complicated cases with associated anomalies. Our findings also suggest that the current criteria for termination of CDH pregnancies with associated anomalies may need to be reconsidered.