Cora C. Lenox

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery A New Method of Surgical Repair

Anomalous origin of the left coronary artery from the pulmonary artery has been surgically corrected by means of a technique that avoids direct suturing of the coronary artery itself. The anomalous left coronary artery is removed from the pulmonary artery with a cuff of pulmonary artery tissue about its orifice. In a five-and-one-half-year-old patient the anomalous coronary artery was anastomosed directly to the aorta using this technique. In another patient, seven months of age, the anomalous coronary artery could not be anastomosed directly to the aorta and a segment of the patients subclavian artery was interposed between the left coronary artery and the aorta to complete the anastomosis. In the latter patient, profound hypothermia and circulatory arrest were used during construction of the anastomosis in an attempt to minimize further myocardial damage. The establishment of a two coronary artery system has been demonstrated to be feasible in the infant or young child with this anomaly.

Blade atrial septostomy: collaborative study.

During the past 4 years, five institutions have collaborated in evaluating the efficacy of blade atrial septostomy. The procedure was performed in 52 patients, including 31 with transposition of the great arteries, 10 with mitral atresia, five with tricuspid atresia and six with miscellaneous anomalies. The patients ages ranged from 1 day to 12 years (mean 13 months). Improvement occurred in 41 of 52 patients (79%). Four patients had an intact interatrial septum, and blade atrial septostomy was successfully performed by a transseptal technique. One patient died from a lacerated left atrial wall; other complications occurred in four patients. Blade atrial septostomy is an effective palliative procedure, even when the interatrial septum is thickened or intact.

Absent right superior vena cava with persistent left superior vena cava: implications and management.

Seven cases of absent right superior vena cava with persistent left superior vena cava and normal situs were diagnosed at Childrens Hospital of Pittsburgh. All patients had associated cardiac defects. In two cases the diagnosis was made at autopsy, the first in 1957 and in a 26 day old infant with multiple cogenital defects and the second in 1965 in a 22 day old infant who had pulmonary atresia with ventricular septal defect and patent ductus arteriosus. Since 1966 absent right superior vena cava has been diagnosed at cardiac catheterization in five children. Three of these children have had surgery, two for subaortic stenosis and one for an atrial septal defect. One has an insignificant atrial septal defect and the fifth has a ventricular septal defect. The electrocardiogram of four reveals s short P-R interval and a leftward frontal plane axis of the P wave, suggesting a low atrial focus. None has had any significant conduction problem. All five children are living and well, the oldest has survived 13 years postoperatively. Certain precautions are necessary should corrective cardiac surgery or transvenous pacemaker insertion be necessary.

Mechanisms of closure of perimembranous ventricular septal defect

Although more defects in the muscular ventricular septum close spontaneously than do defects abutting on the membranous septum (perimembranous defects), a good proportion of the latter show a tendency to diminish in size and to close. The mechanism responsible for this closure is usually described as an aneurysm of the membranous septum, although some doubt has been cast as to whether this is the precise mechanism. In this study, 69 unoperated hearts with ventricular septal defect were examined; 26 (38%) showed some evidence of partial closure and none extended exclusively into the outlet part of the septum. Two thirds of the trabecular perimembranous defects, almost half of the inlet defects, and one third of the confluent defects showed signs of partial occlusion. The anatomic structures most frequently responsible were reduplication of tricuspid valve tissue (16 cases), adhesion of tricuspid valve leaflets (6 cases), and prolapse of an aortic valve leaflet (3 cases). Only in 2 cases were subaortic tissue tags observed which might have originated from the remnant of the membranous septum. The present study shows that although anatomic evidence of partial closure was present in two fifths of the hearts studied, only rarely was it due to so-called aneurysm of the membranous septum.

Clinical use of blade atrial septostomy.

SUMMARY A cardiac catheter enclosing an extensible blade was used to enlarge the interatrial opening in seven patients. Two patients with transposition of the great arteries who had balloon atrial septostomy as newborns subsequently presented with clinical evidence of a restrictive interatrial opening at I and 4 months of age. Cardiac catheterization confirmed restenosis of the interatrial opening and inadequate intracardiac mixing. After blade atrial septostomy the systemic arterial oxygen saturation increased by 20% and 30%, respectively. Five patients with mitral atresia complex, ages 2 months-91/2 years, had a restrictive interatrial communication and severe pulmonary venous hypertension (mean left atrial pressures ranged from 20-38 mm Hg). Following blade atrial septostomy, the pressure gradient between the atria was almost completely abolished and prompt clinical improvement was observed in each patient. All patients tolerated the procedure without complications.Blade atrial septostomy was a safe, effective procedure for enlarging the interatrial communication in this limited series of patients with an interatrial septum too thick to permit adequate rupture by conventional balloon atrial septostomy.

Tetralogy of Fallot with absent pulmonic valve: analysis of 17 patients.

Seventeen patients with tetralogy of Fallot (TF) and absent pulmonic valve were seen at Childrens Hospital of Pittsburgh from 1958 to 1981. In 7 patients, severe respiratory symptoms developed due to bronchial compression by an aneurysmal pulmonary artery, and 6 died. In 4 patients, moderate respiratory symptoms developed. Six patients remained free of respiratory distress. Medical and surgical management of patients with respiratory distress in infancy has been frustrating and frequently unsuccessful. A 4-month-old infant with respiratory distress underwent complete intracardiac repair with valve insertion and has subsequently survived with relief of symptoms. Considering the high mortality rate in this group of patients, further attempts at aggressive surgical repair in infancy for patients with severe respiratory symptoms is warranted. An anatomic review of pathologic specimens with this anomaly confirmed previous reports of the frequent association of absence of the ductus arteriosus in patients with tetralogy of Fallot and absent pulmonary valve. The exception is noted in patients with discontinuity between the right and left pulmonary arteries in which a ductus arteriosus may be present supplying the isolated left pulmonary artery.

Absent proximal coronary arteries associated with pulmonic atresia

Abstract A 2 month old infant had coronary arteries that were not connected to either the aorta or pulmonary artery but arose from the right ventricle and several associated anomalies: atresia of the pulmonic valve with intact ventricular septum, a small right ventricle and an Ebstein-like anomaly of the tricuspid valve. Cardiac catheterization and autopsy data are employed to discuss the abnormal perfusion of the myocardium which led to subendocardial infarction.

Ventricular septal defect associated with aneurysm of the membranous septum

The most common variety of ventricular septal defect, a perimembranous defect, is frequently associated with a so-called aneurysm of the membranous septum. Previous studies have suggested that ventricular septal defects associated with an aneurysm of the membranous septum tend to spontaneously decrease in size or close more than defects without such an aneurysm. To better define the natural history of this entity, clinical and catheterization data from 87 patients with ventricular septal defect and aneurysm of the membranous septum were reviewed. The initial evaluation was made at a median age of 0.3 years (range 0.1 to 11), with the final evaluation at a median age of 10 years (range 1.5 to 20) and a median duration of follow-up of 8.6 years (range 1.2 to 18.8). Approximately 75% of the ventricular septal defects had a small or no left to right shunt at last evaluation. Overall, 48 patients (55%) had no significant change in the size of the defect, and 39 (45%) showed improvement during the period of observation. Only four patients (5%) had spontaneous closure of the defect. Of the 49 patients who presented with a large left to right shunt, with or without congestive heart failure, 23 (47%) had persistence of a shunt large enough to warrant surgery. When spontaneous improvement occurred, it did so by 6 years of age in all but one patient. Therefore, a continued tendency for a ventricular septal defect associated with an aneurysm of the membranous septum to spontaneously decrease in size or close after this age may be less likely than previously suggested.(ABSTRACT TRUNCATED AT 250 WORDS)

Hemodynamic function after the mustard operation for transposition of the great arteries

Pre- and postoperative cardiac catheterization data and cinenangiocardiograms of 82 patients who survived the Mustard operation for transposition of the great arteries (TGA) were reviewed. The post-operative catheterizations were performed 20 days to 10 years after operation (mean 2.5 years). Forty-six patients (56%) had no or insignificant associated cardiac lesions, whereas 36 (44%) had ventricular septal defect, pulmonary stenosis, or both, and required surgical intervention at the time of the Mustard operation. Postoperatively, 11 patients (13%) had significant systemic venous obstruction. Of the 11 patients, 6 required reoperation, and 2 patients had evidence of restenosis or complete obstruction in the superior vena cava after reoperation. In most patients, superior vena caval obstruction was well tolerated even in the presence of high pressure in the superior vena cava. Pulmonary venous obstruction occurred in 5 patients (6%), 3 of whom had no clinical symptoms despite severe pulmonary venous obstruction, although all had radiographic evidence of pulmonary venous congestion. The incidence of obstruction was drastically reduced after the Mustard operation was modified to include routine enlargement of the pulmonary venous atrium. Tricuspid regurgitation was uncommon (10%), but did occur in patients who had transatrial closure of a ventricular septal defect. Preoperatively, left ventricular outflow obstruction occurred in 38%. In 12 patients an attempt was made to relieve the obstruction at surgery. The 6 patients who had localized obstruction had a good result, but patients with more diffuse narrowing of left ventricular outflow had little or no relief of obstruction. Mild to moderate left ventricular outflow gradients regressed spontaneously in most patients after the Mustard operation.

Left ventricular anomalies associated with Ebstein's malformation of the tricuspid valve.

Seventeen patients with Ebsteins malformation of the tricuspid valve have been evaluated. The majority of patients (11/17) presented in the newborn period with cyanosis and cardiomegaly. The remainder (6/17) were referred because of cardiomegaly on chest X-ray and/or a heart murmur heard on routine examination. Three patients have died; one in the newborn period and two suddenly at the age of 11 and 15 years. Thirteen patients have un-dergone right and left heart cardiac catheterization within the last seven years. Twelve of these 13 patients (92%) had angiocardiographic left ventricular contraction abnormalities. Five patients also had mitral valve prolapse associated with left ventricular dysfunction. Since many patients with Ebsteins anomaly may have significant left ventricular abnormalities, careful and systematic evaluation of the left ventricle is warrante