We report a successful neonatal balloon aortic valvoplasty after prenatal diagnosis of critical aortic stenosis with severe left ventricular dysfunction. The infant was delivered by cesarean section at 34 weeks of gestation and had balloon valvoplasty via the right common carotid artery 67 minutes after birth. Left ventricular contraction improved immediately after the procedure. Scheduled balloon aortic valvoplasty immediately after birth subsequent to prenatal diagnosis can improve the outcome of critical aortic stenosis.

A case of a complete atrioventricular septal defect was detected in a 20-week gestation fetus, in whom the ventricular component of the defect was subsequently noted to close spontaneously with advancing gestational age. The antenatal and postnatal findings are discussed.

Morphometric methods were used to study hearts with perimembranous ventricular septal defects in 22 human fetuses and neonates weighing from one to 3.8 kg (2.221±0.852 kg). All specimens displayed concordant atrioventricular and ventriculoarterial connections, and all had perimembranous ventricular septal defects. A total of 17 interventricular parameters (nine in the right ventricle and eight in the left) were measured in each specimen. The values were compared with patterns of normality established by Rowlatt and her colleagues in hearts from 83 children ranging in age from birth to 15 years. These patterns were found to be less reliable than our own data from fetal and perinatal hearts from children ranging in age from birth to one month. Our morphological data, when appropriately interpreted, have immediate clinical and surgical applications in the treatment of fetal and perinatal cardiovascular disorders.

Combined antiarrhythmic drug therapy is an occasionally necessary but problematic approach to the child with recalcitrant supraventricular tachycardia. There is little experience with the combined use of amiodarone and class 1-C agents (flecainide, propafenone and encainide) in children. To judge the efficacy and safety of this combination, we reviewed the case notes and results of investigation in all nine children with supraventricular tachycardia who received such therapy between 1984 and 1993. These nine children received combined therapy on 12 occasions. Five were infants with either atrioventricular reentrant tachycardia (n=3) or atrial ectopic tachycardia (n=2), and four were older children with atrial flutter seen after a Fontan procedure. Amiodarone was combined with flecainide on eight occasions, with propafenone on three occasions, and with encainide on one occasion. Both infants with atrial ectopic tachycardia were successfully controlled, but only one of three infants with atrioventricular reentrant tachycardia had successful control on combination therapy. In three of the four patients with atrial flutter, the combination was useful in reducing the number of arrhythmic episodes. Three infants suffered side effects. At electrophysiologic study to judge efficacy, ventricular tachycardia was induced in two patients (one infant and one Fontan patient), necessitating a change in the 1-C agent. One patient had skin rash due to flecainide and was placed on propafenone with success. One Fontan patient died of complications after an elective surgical procedure. No deaths occurred attributable to proarrhythmia. Thus, combined therapy with amiodarone and 1-C agents was found to be safe and fairly effective in children with certain types of intractable supraventricular tachycardia.

We report a case of interruption of the aortic arch associated with patency of the arterial duct, restrictive ventricular septal defect, obstruction of the left ventricular outflow due to a discrete subaortic shelf and severe pulmonary arterial hypertension. The patient survived to adulthood, when a complete angiographic diagnosis was made. The subaortic obstruction was treated by percutaneous balloon dilation performed through a right brachial arteriotomy. Correction of the interrupted arch was carried out surgically using a woven Dacron graft from the aortic arch to the descending thoracic aorta associated with ligation of the duct. Hemodynamic studies after one year revealed a marked decrease in pulmonary vascular resistance and a sustained reduction in the gradient across the left ventricular outflow tract. The case is of interest in the light of the unusual survival and the success of staged management of this complex situation.

A congenital defect of the right coronary leaflet (fenestration) is an unusually rare cause for aortic incompetence in childhood. We report an eight-year-old child with such an anomaly who presented with infective endocarditis of the left ventricle. The importance of excision of the vegetation prior to the occurrence of life-threatening embolic complications is discussed.

We describe a two-year-old boy suffering from pulmonary hypertension due to pulmonary venous stenosis. Previous reports suggest that it is not easy to diagnose this entity during life, and most cases are diagnosed at operation or autopsy. Our patient was diagnosed relatively early in life and treated successfully by pericardial patch venoplasty. Histologic examination of the lung biopsy performed at operation was considered to be useftil prognostically. The minimal pulmonary vascular changes noted were consistent with a good outcome after the surgical repair.

Isolated noncompaction of ventricular myocardium is a rare cardiomyopathy with persistence of a myocardial architecture such as seen early in fetal development with numerous prominent trabeculations and deep intertrabecular spaces. Symptoms are related to cardiac failure, arterial embolism, and severe arrhythmias. Familial recurrence has been described. We describe a case where isolated noncompaction of ventricular myocardium could be recognized on an ultrasound study at 23 weeks gestation, thus supporting the developmental hypothesis.

Aneurysms of the aortic sinus of Valsalva are rare and generally do not produce symptoms unless the aneurysm ruptures. When rupture occurs, the clinical findings depend to an extent on the site of the aneurysm and the cardiac chamber into which the aneurysm ruptures. We report a case of acute rupture of an aneurysm of the right aortic sinus, which was misdiagnosed as a ventricular septal defect. The clinical condition of the patient deteriorated rapidly, and transcatheter closure of the defect with an umbrella was associated with a fatal outcome.

We present a patient with primary pulmonary hypertension who had unusually high pulmonary arterial pressure prior to double-lung transplantation. Obstruction of the right ventricular outflow tract developed after transplantation and progressed over the subsequent two years.

A 33-year-old woman known to have tetralogy of Fallot and a straddling tricuspid valve had bilateral systemic-to-pulmonary arterial shunts constructed at the age of six months. She subsequently developed infundibular atresia. Antegrade flow of blood from the right ventricle to pulmonary trunk was re-established by percutaneous laser perforation of the outflow tract, later followed by implantation of a stent.

We describe a neonate who presented with aortic atresia, a hypoplastic left ventricle and aorto-left ventricular tunnel. These findings, recognized by transthoracic and transesophageal cross-sectional echocardiography complemented by Doppler color flow imaging, were confirmed at surgery by direct inspection. This complex of associations has not been described before and suggests a primary abnormality of the left ventricle because diastolic filling from the aorto-left venticular tunnel and mitral flow should tend to ensure fetal left ventricular growth.

We report an unusual form of aortic atresia with a normal sized left ventricle, intact ventricular septum and isolated cleft of the left atrioventricular valve, with abnormal attachments creating both subaortic atresia and massive regurgitation across the left atrioventricular valve. This combination, never described before as far as we are aware, can be considered as an extreme form of subaortic stenosis complicating a very particular type of atrioventricular septal defect.

We report a patient with a mediastinal cyst compressing the left atrium that was diagnosed prenatally by fetal echocardiography. Postnatally, attempted aspiration guided by computerized tomography was unsuccessful. Because of respiratory distress, the cyst was removed surgically. Pathological examination revealed a bronchogenic cyst.

We describe a male adolescent with co-existence of quadricuspid aortic and pulmonary valves and rheumatic heart disease. As far as we know, the presence of quadricuspid aortic and pulmonary valves in the same patient has not previously been described. Furthermore, the presence of rheumatic heart disease in this case makes it a true rarity.

A seven-year-old patient with severe valvar pulmonary stenosis developed a mycotic aneurysm of the pulmonary trunk, which was followed closely with echocardiography. The aneurysm increased rapidly in size over a period of three weeks and required surgical removal.