Surgical treatment of pulmonary stenosis with intact interventricular septum.
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We describe six patients with situs solitus of viscera and atria, dextrocardia, atrioventricular discordance, ventricular septal defect, double outlet from the morphological right ventricle, pulmonic stenosis and levomalposition of the aorta. Four patients were male and two female; their age ranged from 3.5 to 31 years (mean 13.8 years). All had various degrees of disability, and presented with cyanosis, clubbing and high hematocrit levels. One patient had an atrio-ventricular block that varied from first to third degree; another patient showed intermittent junctional rhythm. At angiography the ventricular septum appeared to be almost perpendicular to the frontal plane in most cases, so that the anteroposterior projection resulted in a true axial view. One overriding left atrioventricular valve and one straddling right atrio-ventricular valve were demonstrated; no significant incompetence of either valve was observed. The ventricular septal defect was always single and related to the subpulmonary outflow. Pulmonic stenosis was valvular in every patient; an additional infundibular obstruction was present in one case. In two cases an additional stenosis was discovered at the supravalvular level. The left pulmonary branch was stenotic in one case; it was hypoplastic, with controlateral dilatation, in two cases; both pulmonary arteries were dilated in one case. The aorta was always to the left of the pulmonary artery, either anterior or side by side. Three patients were operated on in different Institutions: one had a pulmonic valvotomy at the age of six years; one, aged twenty, had a right Blalock-Taussig shunt; the third, with overriding left atrioventricular valve, underwent a modified Fontan operation at the age of thirty years with success.(ABSTRACT TRUNCATED AT 250 WORDS)
The clinical, hemodynamic, angiocardiographic and pathologic findings are presented in an infrequent but surgically correctable type of double outlet right ventricle. This study is based on six cases, one with autopsy confirmation. In all, the viscera and atria were in situs solitus (S). A ventricular d-loop was present (D). There was I-malposition of the great arteries, the aorta being to the left of, and anterior to, the pulmonary artery (L). Hence, this anomaly may conveniently be represented as double outlet right ventricle (S,D,L,). The ventricular septal defect to the ventricular septum. A bilateral conus was present beneath both the aortic and pulmonary valves, preventing any semilunar-atrioventricular fibrous continuity. The subpulmonary conus was poorly expanded, resulting in pulmonary infundibular and valvular (annular) stenosis. The clinical features were those of cyanosis, clubbing and accentuation of the second heart sound in the pulmonary area (related to aortic valve closure). There was a systolic ejection murmur along the upper left sternal border, related to pulmonary outflow tract stenosis. Selective right and left ventricular angiocardiography was diagnostic. Relatively early surgical correction is suggested to minimize the progression of pulmonary infundibular stenosis and to avoid acquired atresia. In this malformation, pulmonary outflow tract reconstruction is more difficult than in tetralogy of Fallot because of the rather posterior location of the pulmonary outflow tract, and because the right coronary artery crosses the stenotic pulmonary outflow tract in front of the pulmonary valve.
Case histories of four infants with single ventricle and left atrioventricular (AV) valve atresia, with interatrial obstruction but without pulmonic stenosis, are presented. Relief of interatrial obstruction by surgical or balloon atrial septostomy resulted in a marked fall in the left atrial pressure and the pulmonary artery mean pressure and resistance as well as a marked increase in the left-to-right interatrial shunt, pulmonary blood flow, and systemic arterial oxygen saturation. Because of this predictable fall in pulmonary vascular resistance, concomitant pulmonary artery banding was performed in each case with good results. There are only a few previously reported cases of single ventricle with left AV valve atresia. Interatrial obstruction is quite common in this lesion. The concept that relief of interatrial obstruction produces a rapid and predictable fall in the pulmonary vascular resistance and that concomitant pulmonary artery banding is necessary in these infants with complex congenital heart disease is emphasized.
1. Right ventricular hypertrophy was produced in dogs by banding of the pulmonary artery for 28 days (n = 7) and results were compared with those in sham-operated dogs (n = 5). 2. Myocardial noradrenaline and adrenaline levels were depressed in both the hypertrophied right ventricle and the non-hypertrophied left ventricle and plasma levels were increased compared to the control dogs. 3. Myocardial beta-adrenoceptors were assessed in membrane preparations of both ventricles by 3H-dihydroalprenolol binding. No change in binding site concentration (pmol/mg membrane protein) or in binding affinity was found in either the hypertrophied right ventricle or in the left ventricle. 4. Divergent results of studies on beta-adrenoceptors in ventricular hypertrophy are difficult to reconcile.
Pulmonary balloon valvuloplasty was performed in 35 children aged 3 days to 18 years (mean 4.5 years). Balloon/annulus ratio was 1.21 +/- 0.12. There were 6 infants less than 1 year of age (including 1 newborn), 4 with dysplastic valves and 4 with postoperative restenosis. Systolic transvalvular gradient showed a decrease of 69 +/- 14% from 68 +/- 15 mm Hg before to 21 +/- 11 mm Hg after dilatation (p less than 0.0001), and right ventricular pressure/systemic pressure ratio decreased from 0.8 +/- 0.29 to 0.4 +/- 0.15 (p less than 0.0001). Follow-up studies were performed in all patients up to 5 years (2.5 +/- 1.3 years) after dilatation. In 63% (22/35) follow-up period was more than 2 years (3.5 +/- 0.95 years). Doppler derived gradient had further decreased to 16 +/- 11 mm Hg (n.s.). Results in the 6 infants showed an immediate gradient relief of 71 +/- 11% from 85 +/- 4 mm Hg to 25 +/- 10 mm Hg (p less than 0.0001) with a further decrease to 20 +/- 10 mm Hg at follow-up. There were two early complications which had to be treated (tachycardia in WPW-syndrome, femoral vein dissection), no late complications were seen. The good immediate results and high persistent long-term success rate confirm balloon pulmonary valvuloplasty being the treatment of first choice for all patients in childhood.
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The case of a 59-year-old man with corrected transposition of the great arteries (CTGA) is reported. This anomaly is rare in adult patients, uncommon in patients over 50. Most of the cases with CTGA are diagnosed in childhood because of associated cardiac abnormalities. When isolated or complicated by other mild anomalies, CTGA can cause no symptoms until adult life or can even go unrecognized. However, most of these patients present with congestive heart failure when they are 40 or 50 years old. Our patient was admitted to the hospital because of heart failure. Cardiac catheterization and selective angiography demonstrated a CTGA associated with severe pulmonary valve stenosis. The case here reported shows that CTGA even when associated with other cardiac abnormalities, can be a relatively benign lesion, and can be revealed in adult life by signs of cardiac failure.
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