Subaortic stenosis: an unrecognized problem in transposition of the great arteries.

Right ventricular (subaortic) obstruction has only rarely been described in complete transposition of the great arteries. five patients with complete transposition of the great arteries in whom subaortic stenosis was angiocardiographically demonstrated were studied. All had a pressure gradient of 30 to 55 mm Hg across the aortic outflow tract. Two of the patients manifested mild tricuspid insufficiency, and another two had coarctation of the aorta. The etiologic, anatomic, hemodynamic and prognostic aspects of this unusual anomaly are discussed. An angiocardiographically demonstrated series of this anomaly has not been reported on before.

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. Of 37 patients with DSS, 2-D echo diagnosed the presence and type in 35; in 2, a membrane was demonstrated by angiography. Of the 35 patients accurately diagnosed by 2-D echo, angiography corroborated the diagnosis in 33, but failed to show the membrane in 2. Subsequent cardiac surgery confirmed the accuracy of the echocardiographic diagnosis in these 2 patients. In all patients with membranous DSS, the anterior insertion of the membrane was demonstrated. In 9 of them the posterior insertion was demonstrated by tilt of the transducer but the anterior insertion disappeared. In 4 patients both insertions were demonstrated simultaneously and in 3 patients the membrane was demonstrated as a continuous line. In 4 of the 5 patients with fibromuscular DSS, both insertions of the lesion were demonstrated simultaneously. However, 2-D echo was unsuccessful in assessing the severity of obstruction. In only 1 patient did demonstration of the whole subaortic membrane as a continuous line below the aortic valve correlate with severe obstruction. Thus, the presence and type of DSS, but not the degree and severity, can be accurately and reliably diagnosed by means of 2-D echo.

Correction of tetrad of Fallot with reduced incidence of right bundle branch block.

In 20 patients who underwent a modified surgical repair of tetrad of Fallot complete right bundle branch block developed in only 8 (40 percent). Standard and intraoperative conduction studies indicated that in these patients the right bundle branch block was due to injury of the right bundle branch near the ventricular septal defect (proximal right bundle branch block). The modified operative technique is aimed at minimizing the injury to the right ventricle and it includes a significantly shorter than usual ventriculotomy incision and avoidance of the septal (moderator) band during infundibulectomy. Intra- and postoperative hemodynamic studies of these patients revealed that relief of the right ventricular outflow obstruction was optimal.

Congenital atrial flutter.

Two cases of congenital atrial flutter, one of which was documented electrocardiographically before birth, are reported. In both patients sinus rhythm was restored with digoxin treatment; in one patient the transition was preceded by various arrhythmias. No cardiac malformation was found in either case, and no materal disease occurred during pregnancy. Both mothers had received medication during pregnancy, but its role as a causative factor is questionable.

Impeded coronary flow in anatomic correction of transposition of the great arteries: prevention, detection, and management.

Six infants and a 3-year-old boy underwent primary anatomic repair (arterial switch) of their dextro-transposition of the great arteries (d-TGA). Three died: one on the operating table because of an irreducible kink in the left main coronary artery, one because of postoperative bleeding and tamponade, and one because of a postoperative management error. In the four survivors the preoperative left ventricular-to-right ventricular systolic pressure interrelationships were 35 to 115, 54 to 73, 30 to 80, and 70 to 90 mm Hg. While left ventricular inability to take over the systemic pressure did not appear to be a problem in any of our cases, reduced coronary perfusion seemed to be the rule rather than the exception in this operation. One approach to the problem of obstructed coronary flow is first to construct continuity of the "new" aorta, unclamp it, and mark the appropriate places for coronary anastomoses on the fully distended aorta. It is our conviction that further improvement in the operative technique and better understanding of the risk factors will eventually make the primary anatomic repair of d-TGA the operation of choice for most dextro-transpositions.

Coarctation of the aorta with congenital hemangioma of the face and neck and aneurysm or dilatation of a subclavian or innominate artery. A new syndrome?

Congenital cutaneous hemangioma associated with congenital heart disease is extremely rare. We report four infants with congenital cavernous hemangioma of the face and neck and coarctation of the aorta. Three also had congenital aneurysm of a subclavian or innominate artery. One patient also had mild congenital valvular aortic stenosis. The unusual combination of lesions may represent a new syndrome.

The drop of residual right ventricular pressure 24 hours after conservative infundibulectomy in repair of tetralogy of Fallot.

Because of late complications such as right ventricular (RV) dysfunction and sudden death after tetralogy repair, a modification in the technique of total repair of tetralogy of Fallot is sought. In this study a shorter than usual conal incision and limited division of only the parietal muscle bands were utilized in 17 patients. The mean RV pressure at the end of the operation was 84 mm Hg; 24 hours later it had dropped to 55 mm Hg. The mean RV/LV pressure ratio at the end of the operation was 0.85, and 24 hours later it was 0.48. Pressure measurements were also taken at the end of the operation, at four predetermined points along the RV outflow tract. It was found that 40% of the average residual obstruction was located at the level of the infundibular ostium, 50% at the level of the conus, and 10% at the pulmonary valve. It is concluded that a 40% drop in the RV/LV pressure ratio can be anticipated to occur in less than 24 hours in cases in which (1) the contractility of the infundibular ostium and conus is preserved by conservative infundibulectomy and (2) there is a residual obstruction at a muscular level. This is on condition that the passage between the crista and the anterior conal wall is of adequate size.

Uninterrupted inferior vena cava with azygos continuation.

Azygos continuation of the inferior vena cava is diagnosed in infants in the presence of obstruction to flow in the inferior vena cava due to infrahepatic interruption. Recently we studied in an infant complex congenital heart disease in which there was azygos continuation of the inferior vena cava without infrahepatic interruption or any other obstructive lesion of this vessel. The blood from the lower part of the body drained into the right atrium by two wide patent veins: the inferior vena cava and the azygos system. The angiocardiographic observations of this condition in an infant are reported for the first time to our knowledge, and the embryologic development is briefly reviewed.

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.

Normal mean manifest electrical qRS in dysplastic pulmonary valve associated with hypertrophic non-obstructive left ventricular cardiomyopathy.

Dysplastic pulmonary valve is usually associated with extreme right axis deviation. A mean manifest electrical QRS axis of + 160 degrees or less was present in 11 of 30 cases of DPV studied by us. In 9 of these cases associated left ventricular hypertrophic non-obstructive cardiomyopathy was found. We suggest that a normal mean manifest electrical QRS axis in the presence of dysplastic stenotic pulmonary valve indicates the presence of associated cardiomyopathy.

Wide vascular pedicle on thoracic roentgenogram in complete transposition of the great arteries.

A narrow vascular pedicle on the thoracic roentgenogram is an important sign in the clinical diagnosis of complete transposition of the great arteries (Shaher, 1973a). Rarely a wide vascular pedicle is present in this condition, mainly due to associated lesions. The differential diagnosis in these cases is discussed, based on our experience with 14 cases.

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 findings indicate that the prosthetic status was poor and the treatment needs extensive. In all, over 83% of the study population were edentulous, the majority in both arches. Although 86.3% of the edentulous subjects possessed dentures, over 86% of these subjects needed some form of prosthetic treatment. About 60% needed new full or partial dentures and over 25% had dentures whose fit and retention could be improved by rebasing, relining or minor adjustments.

Application of the periodontal treatment need system (PTNS) in a group of young Israeli adults.

The Periodontal Treatment Need System (PTNS) was applied to a group of young Israeli adults, 18-25 years of age, to test the system in the field. A total of 830 persons were examined. Their periodontal treatment needs were assessed. One of the aspects investigated was that of treatment needs according to distribution by sex. Only 2.7% of young adults studied were free of plaque, calculus and gingival inflammation. All the rest (97.3%) needed some kind of periodontal treatment. The time required for treatment needed was also calculated. It was found that on the average 2.4 h per person would be required to deliver the periodontal treatment needs of the group. A close association between sex and PTNS was established in this study.

Interruption of the aortic arch with complete transposition of the great arteries. Clinical and angiocardiographic diagnosis at the age of one day.

The association of complete transportation of the great arteries and complete interruption of the aortic arch is very rare. This combined lesion was diagnosed clinically in a 1-day-old infant in whom it caused cyanosis of the upper half of the body. The diagnosis was confirmed angiocardiographically. We believe this is the youngest patient in whom this diagnosis was made in vivo.