Spontaneous regression of severe aortic coarctation in trisomy 18

Abstract

Spontaneous regression of severe aortic coarctation with ductus dependency has not been reported. We experienced a case of trisomy 18 with spontaneous regression of severe aortic coarctation complicated by ventricular septal defect and patent ductus arteriosus. The aortic isthmus diameter was 1.2 mm at birth. After 5 months, it increased to 4.5 mm, and the shape of the isthmus was fully normalised.

Case Details

Disease Location

Heart (great vessels)

Personal Characteristics

Female infant

Clinical Characteristics

She was born, via vaginal delivery, at gestational age of 35 weeks and 1 day, weighing 1592g, apgar 4,5,6. Fetal ultrasound indicated trisomy 18 and detected persistent left superior cava vein, aortic coarctation, and ventricular septal defect. Trisomy 18, was diagnosed based on postnatal chromosome analysis. Echocardiogram revealed persistent left superior caval vein, a large perimembranous ventricular septal defect (5.2 mm), patent ductus arteriosus (4.7 mm) with dominant right-to-left shunt, and severe aortic coarctation (1.2 mm). Left aortic arch with the left common carotid artery originating from the brachiocephalic trunk. Cardiopulmonary status stabilized, and extubation was performed on day 4. Hospital discharge was on day 142

Remission Characteristics

The aortic isthmus spontaneously dilated (2.5 mm at 4 weeks, and 4.5mm at 5 months) and aortic coarctation had almost fully regressed. Patent ductus arteriosus flow was bidirectional, with a dominant left-to-right shunt

Treatment & Mechanisms

Proposed Remission Mechanisms

Natural tendency for a patent ductus arteriosus in trisomy 18 spontaneous regression of severe aortic coarctation can occur

Clinical Treatment

Pulmonary artery banding and patent ductus arteriosus ligation at 6 months (after remission)