Hour-glass deformity of the pulmonary valve: a third type of pulmonary valve stenosis.

In the 12 years from 1975 to 1987, 55 patients had open pulmonary valve surgery for isolated congenital stenosis of the pulmonary valve. Three types of pulmonary stenosis were seen: (a) dome-shaped pulmonary stenosis (34 patients); (b) dysplastic pulmonary valves with thick cauliflower-like cusps (12 patients), and (c) hour-glass deformity of the pulmonary valve, with "bottle-shaped" sinuses (nine patients). This third type has not been described before.

Coarctation of the aorta associated with pulmonary valve stenosis.

A 7-year-old patient had coarctation of the aorta associated with moderate and dysplastic pulmonary valve stenosis. The child underwent corrective surgery of the coarctation. This unusual association cannot be satisfactorily explained by the theory of fetal flow patterns.

Assessment of intestinal and cardiorespiratory function in children with congenital heart disease on high-caloric formulas.

Fourteen infants with congenital heart disease were investigated for failure to thrive. Assessment of intestinal function revealed minor absorptive abnormalities (mild steatorrhea in three patients, bile salt loss in four patients), delayed gastric emptying, and abnormal triglyceride loading tests. Low caloric intake (88.

Permanent cardiac pacing in congenital heart disease: a follow-up study of 20 patients.

Sinus nodal dysfunction (SND) and complete heart block (CHB) in congenital heart disease (CHD) are commonly associated with congestive heart failure, syncopal attacks, and sudden death. Permanent cardiac pacing (PCP) is required to avoid these manifestations which are frequently associated with a high rate of complications, particularly in the younger age group. Twenty patients with CHD aged 4 months to 46 years underwent pacemaker implantation.

Prosthetic needs and demands for services of a group of elderly people in Israel.

Prosthetic needs and demands for care were investigated in a group of the elderly, residing in Romema, Jerusalem, Israel. Of the population aged 60 and above residing in the neighborhood, 34% (n = 116) were selected at random. These were examined under standardized conditions and prosthetic status and denture requirements were recorded.

The obstructive subaortic conus.

Eleven children are reported who had stenosis under a malposed aorta with gradients of 20 to 76 mm Hg between the right ventricle and aorta. The subaortic obstruction was caused by hypertrophy of the foreshortened infundibulum and malalignment of the infundibular septum relative to the remainder of the ventricular septum. Of these 11 patients, nine had a ventricular septal defect and seven had coarctation of the aorta.

Retroesophageal right aortic arch.

Right aortic arch with left descending aorta and a retroesophageal aortic segment is an uncommon congenital vascular malformation which may cause symptoms of compression from a vascular "ring." The presence of this malformation may be suspected by the findings on barium swallow and the diagnosis confirmed by angiocardiography. Surgical intervention may be necessary in patients who have severe symptoms.

Two-dimensional echocardiography in discrete subaortic stenosis.

Thirty-seven patients with discrete subaortic stenosis (DSS) underwent 2-dimensional echocardiography (2-D echo) and cardiac catheterization. The peak systolic pressure gradients ranged from 0 to 150 mm Hg. Thirty-two patients had membranous DSS and 5 had fibromuscular DSS.

The renal effects of radiocontrast administration during cardioangiography in two different groups with congenital heart disease.

Renal effects of the administration of contrast media during cardiac catheterisation were compared in two groups of patients with congenital heart diseases. Group A consisted of 21 patients with cardiac malformations, characterised primarily by left ventricular volume overload and known to be associated occasionally with left heart failure, such as: left to right shunts and left ventricular valvular defects. Group B consisted of 23 patients with lesions affecting the right ventricle which are rarely associated with left heart failure, such as: Tetralogy of Fallot and Pulmonic stenosis.

Diagnosis of pulmonic regurgitation by contrast echocardiography.

In order to determine whether pulmonic regurgitation (PR) can be reliably diagnosed using contrast echocardiography, we studied 24 subjects using echocardiography during intravenous injections of 5% dextrose solution. Twelve were without PR, and twelve had PR (10 after intracardiac repair of tetralogy of Fallot, one after a Brock procedure for pulmonic stenosis, and one after insertion of a right ventricle-pulmonary artery conduit for pseudo truncus arteriosus). Two blinded independent observers correctly diagnosed PR in all patients when it was present, and correctly excluded it in 11 of 12 of the patients without PR.

Persistent left ventricular disease in clinically "cured" primary endocardial fibroelastosis.

We studied by serial cardiac catheterisation eight patients with the dilated form of primary endocardial fibroelastosis in whom congestive heart failure disappeared with treatment. All remained without symptoms for at least three years before recatheterization. Four patients showed regression of the abnormal electrocardiographic findings, three showed persistence, and one showed progression of electrocardiographic left ventricular overload pattern.

Discrete subaortic stenosis associated with congenital valvular aortic stenosis--a diagnostic challenge.

Discrete subaortic stenosis has only rarely been reported in association with congenital valvular aortic stenosis. The valvular lesion may obscure the subaortic stenosis or may be obscured by it. Our experience with seven such cases (10% of our 71 patients with discrete subaortic stenosis) is reported and the diagnostic problems are discussed.

Severe congestive heart failure in mild pulmonic stenosis due to dysplastic pulmonary valve associated with cardiomyopathy.

Congestive heart failure has never been described in patients with dysplastic stenotic pulmonary valve without associated shunt lesions. We describe two patients with mild pulmonic stenosis due to valvular dysplasia associated with cardiomyopathy who developed severe congestive heart failure. Since the small pressure gradients across the pulmonary valve cannot cause this complication, we suggest that it resulted from the associated hypertrophic non-obstructive cardiomyopathy.

Pre-excitation syndrome in infants and children. Effect of digoxin, verapamil, and amiodarone.

Clinical and electrocardiographic findings for 30 patients with the pre-excitation syndrome are described together with details of treatment. Nineteen (63%) were younger than 2 years, 14 of whom were under 2 months. Sixteen infants and 7 children (77%) presented with paroxysmal supraventricular tachycardia, 14 (61%) of whom had the electrocardiographic pattern of type A Wolff-Parkinson-White (WPW) syndrome.