Congenital complete atrioventricular block is a known lethal condition. Although antenatal diagnosis and the technical advances of pacemaker treatment have reduced its mortality, treatment of premature babies with significant myocardial damage remains a challenge. In this paper, we report the case of a premature low-birth-weight infant with congenital complete atrioventricular block and extremely low ventricular rate, fetal hydrops, and myocarditis who was successfully treated with staged permanent pacemaker implantation.

Although cardiac tumours are rare, such tumours are increasingly being diagnosed with increasing frequency and great accuracy by antenatal ultrasound. Cardiac haemangiomas account for less than one-twentieth of all primary cardiac tumours, with most being diagnosed in the neonatal period. We report 3 instances of successful neonatal resection of cardiac haemangioma subsequent to prenatal diagnosis. Such diagnosis is important in perinatal management, since early surgical intervention provides a good prognosis.

A hydropic neonate, born at 28 weeks gestation, died 45 minutes after birth. Autopsy showed an abnormally long and tortuous arterial duct which wasoccluded by laminated thrombus. The right heart chambers and the left ventricle were dilated, but there was no other structural cardiac abnormality. Apart from the abnormal ductal morphology, there was no evident cause for the intrauterine thrombosis of the arterial duct, which appeared to be the sole cause of fatal heart failure.

We discovered supraventricular tachycardia with advanced hydrops in the setting of normal cardiac anatomy at 26 weeks of gestation which resolved successfully following administration of digoxin and flecainide to the mother. A female baby was born after a premature rupture of the membranes at 30.6 weeks. The neonate was in sinus rhythm, showed a progressive regression of right ventricular insufficiency, but developed signs of acquired pulmonary valvar and subvalvar stenosis at 2 months of age.

An hydropic fetus seen at 28-weeks gestation had a saccular aortic aneurysm in the descending thoracic aorta. Histology disclosed marked fibrointimal hyperplasia, thrombus, and attenuation of the tunica media. The remainder of the descending thoracic aorta showed fibrointimal hyperplasia. We speculate that the narrowed lumen and rigid aortic wall resulting from this vasculopathy provided an increased afterload leading to cardiac failure.

At 32 weeks, a fetus was found to be hydropic with congenitally complete heart block in an asymptomatic mother who was positive to anti-Ro antibodies. Maternal therapy with oral salbutamol was successful in prolonging gestation for a period of 3 weeks so that preterm delivery was delayed until the 35th week of gestation. Following treatment with inotropes and diuretics, a permanent transvenous pacemaker was implanted at 8 weeks of age. The child is now thriving at 20 months of age.