Pulmonary atresia with dilated right ventricle: A case with congenital atrial flutter☆

Abstract A case is reported of pulmonary atresia with intact ventricular septum and enlarged right ventricle in a newborn infant who had associated congenital atrial flutter. Immediately after birth this infant exhibited profound right heart failure and frank tricuspid insufficiency with a vigorously pulsating liver. This clinical picture has not been previously reported in humans with this congenital malformation and is thought to be due to the combined hemodynamics of both problems. A review of 22 patients whose only problem was a similar cardiac malformation reveals that early surgical intervention affords the only opportunity for survival beyond infancy. This fact re-emphasizes the necessity for prompt cardiac evaluation. Reports of 17 patients with congenital atrial flutter were reviewed. It was common to find some evidence of cardiac decompensation at birth; symptoms gradually became more severe if the arrhythmia persisted. Spontaneous reversion of the flutter to another rhythm rarely occurred. Digitalis was the most effective drug used to achieve a normal sinus mechanism. Once reverted to normal sinus rhythm, the flutter did not recur. The association of this arrhythmia with a congenital cardiac malformation has been attended by a severe morbidity and a high mortality.