Challenges in the Treatment of Congenital Heart Disease in Preterm and Low-Birth-Weight Neonates: Results of the European Association Brain in Congenital Heart Disease (EUR-ABC)

Background: Treatment of congenital heart disease (CHD) in preterm and low birthweight (LBW) neonates is challenging due to the need for early intervention. This study aimed to investigate cardiac and neurodevelopmental outcomes of this population.

Method: From 2016 until 2020, preterm and LBW term neonates (<2,500 grams) with CHD requiring therapeutic cardiac intervention within the first year of life from 3 European centers (Munich, Zurich, and Utrecht) were included. Solitary closure of a persistent ductus arteriosus was excluded. Clinical data and neurodevelopmental (ND) outcome were collected.

Results: A total of 308 neonates (51% male) were included; 237 (77%) patients were born preterm; 71 (23%) were LBW term newborns; 41% were additionally small for gestational age. The median (IQR) gestational age was 35.4 weeks (33.3–36.9) and the mean birth weight was 2,016 g (SD: 580). Type of CHD was classified as mild in 51 (16%), moderate in 184 (60%), and severe in 73 (24%) patients. Cardiac diagnosis included biventricular CHD (n = 276) and univentricular CHD (n = 32). A total of 119 (39%) had cyanotic CHD. Rate of associated anomalies was high (n = 128, 42%); 256 (90%) patients underwent surgery; 30 (10%) received interventional catheterizations performed at a median (IQR) chronological age of 68 days (12–155) and postmenstrual age of 43.1 weeks (38–57) at 3,275 g (2,383–5,143). Fifty-eight (19%) patients did not survive the first year of life. Twenty-two (7%) patients died before intervention and 36 of 286 (13%) patients died postintervention. Neonatal mortality was 8%. Univariate analysis revealed that APGAR at 5 minutes and head circumference at birth were significantly lower in patients who died. Those had more frequent diagnosis of bronchopulmonary dysplasia, more severe, more often cyanotic, duct dependent CHD, and more frequent genetic anomalies (all p < 0.05). In patients who died, weight at intervention was lower, head circumference was smaller, and they were younger at time of intervention. Perioperative cranial ultrasound was standard of care (76% pre- and 47% post-intervention), while MRI was rarely performed (12% pre- and 13% post-intervention). ND outcome data acquired during the first year of life was available in 48 patients (19% of the 250 survivors).

Conclusion: The high mortality confirms the high risk of preterm and LBW neonates with CHD. Intervention was preferably performed at term with bodyweight >3 kg. The low availability of ND outcome data in this high-risk population justifies the prospective assessment of structural neuroimaging, neuromonitoring, and functional ND outcomes.

Publication History

Article published online:
28 January 2023