G23(P) A 10 year experience of neonates with truncus arteriosus

Abstract

Background Congenital heart defect affects around 10 in a 1000 live born babies. Truncus arteriosus is a rare conotruncal defect in which a common arterial trunk supplies systemic, pulmonary and coronary circulation. The incidence of truncus arteriosus is quoted between 1–2% of all congenital heart malformations. Aim To evaluate the initial neonatal management and outcomes of newborns with truncus arteriosus. Method We conducted a retrospective review of all newborns with truncus arteriosus admitted to our neonatal unit between 01/04/2009 and 31/03/2019. Data were collected from Badger and Cardiology databases to evaluate the initial neonatal management and long-term outcomes of this cohort of patients. Results Sixteen newborns with truncus arteriosus were admitted to our neonatal unit in the last 10 years. Their mean gestational age was 36 weeks with a mean birth weight of 2407 grams. Cardiac defects were detected prenatally in 13 patients (82%). Three babies were diagnosed postnatally. One following failed pulse oximetry screening requiring non-invasive respiratory support and 2 diagnosed following on-going respiratory support on echocardiography. Respiratory support was initiated within the first 24 hours of life in 8 patients; these included all patients with a postnatal diagnosis of truncus arteriosus. Four patients required mechanical ventilation. Whilst in the neonatal intensive care unit, 3 babies developed heart failure and were treated with diuretics and 2 developed necrotising enterocolitis which was managed conservatively. Median length of stay before transfer to our local surgical cardiology centre was 3 days. Fourteen patients (87%) underwent common arterial trunk repair. 11 (78%) babies survived to date. Two patients died in the initial post-operative period and one patient at 14 months of age. The remaining two patients did not receive any surgical intervention and subsequently died (4 and 11 days old). Conclusion Truncus arterisus is a rare conotruncal defect with the majority being detected antenatally in our cohort. They are likely to require respiratory support in the neonatal period. Despite the complex nature of their congenital heart disease, their surgical outcomes are good in our cohort of babies. The following information can be used when counselling parents antenatally and managing their expectations.