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PubMedDate not supplied

Congenital absence of the pulmonary valve associated with pulmonary stenosis, large ductus arteriosus and intact ventricular septum. Case report.

A six-day-old neonate was diagnosed with a severe form of the syndrome of absent pulmonary valve associated with pulmonary stenosis, aneurysmal dilatation of the pulmonary artery and rare findings including an intact ventricular septum and large ductus arteriosus. The patient underwent surgical repair by closed technique. Cardiac catheterization data, hemodynamic and clinical findings, and surgical technique are reported. Congenital absence of the pulmonary valve is a rare cardiac anomaly. An especially severe form occurs with ventricular septal defect and pulmonary stenosis. The usual findings are respiratory distress, aneurysmal dilatation of the pulmonary arteries and pulmonary stenosis.

Ductus Arteriosus, Patent↗

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Bicuspid aortic valve and coarctation of aorta.

Bicuspid aortic valve (BAV) and coarctation of aorta (COA) are frequently seen together. It is believed that these malformations result from a single developmental diathesis. A case is presented of COA, aortic stenosis and aneurysm of the ascending aorta corrected by patch aortoplasty and commisurotomy. An aneurysm at the site of the coarctation repair can develop as late as 20 to 25 years after surgery. Almost all of the aneurysms described have occurred in patients undergoing patch aortoplasty. We do not recommend this technique except in special cases.

Aortic Aneurysm↗

PubMedDate not supplied

Incidence and severity of arrhythmias and conduction disturbance after repair of tetralogy of Fallot.

Ventricular and supraventricular arrhythmias and conduction disturbances were evaluated by routine electrocardiography and 24-hour ambulatory monitoring in 31 patients who underwent correction of tetralogy of Fallot. The interval from operation to the study was 1 month to 14 years (mean 4.8 +/- 2.8). Complete right bundle branch block occurred in 22 (71%) patients and incomplete right bundle branch block in 9 (29%) patients. Bifascicular block with right bundle branch block and left axis deviation (LAD) occurred in one patient. Two patients had second degree type II atrioventricular block. Twenty-four-hour ambulatory electrocardiographic monitoring was performed in all patients and they were divided in two groups according to the frequency of ventricular arrhythmias (Lown classification). Group 1 included the 23 patients who had no arrhythmia or rare ventricular arrhythmias (Lown grade 0-1). Group 2 was comprised of eight patients (26%) with significant ventricular arrhythmias (Lown grade 2-5). Twelve patients (39%) had supraventricular arrhythmias, three patients rare supraventricular tachycardia attacks, and seven patients occasional supraventricular ectopies. One patient had bradycardia-tachycardia attacks and one patient had junctional tachycardia. There was no correlation between age at the time of surgery and ventricular arrhythmias. Of the patients who had ventricular and supraventricular arrhythmias of various degrees on ambulatory monitoring, two had significant arrhythmias on routine electrocardiogram. Symptoms were rare in these patients. In conclusion, both supraventricular and ventricular arrhythmias were found in considerable frequency in our patients. As ventricular arrhythmias may be the cause of sudden death and supraventricular arrhythmias are a main cause of morbidity, it is important to evaluate ventricular and supraventricular arrhythmias by ambulatory monitoring in patients who have undergone correction of tetralogy of Fallot.

Adolescent↗