Sang C. Park

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.

Complications of endomyocardial biopsy in children

OBJECTIVESnTo evaluate the incidence of, and risk factors for, complications of endomyocardial biopsy in children.nnnBACKGROUNDnEndomyocardial biopsy (EMB) is a low risk procedure in adults, but there is a paucity of data with regard to performing this procedure in children.nnnMETHODSnRetrospective review of the morbidity and mortality of 1,000 consecutive EMB procedures.nnnRESULTSnOne thousand EMB procedures (right ventricle 986, left ventricle 14) were performed on 194 patients from July 1987 through March 1996. Indications for EMB included heart transplant rejection surveillance (846) and the evaluation of cardiomyopathy or arrhythmia for possible myocarditis (154). Thirty-seven (4%) procedures were performed on patients receiving intravenous inotropic support. There was one biopsy related death, secondary to cardiac perforation, in a two-week-old infant with dilated cardiomyopathy. There were nine perforations of the right ventricle, eight occurring in patients with dilated cardiomyopathy and one in a transplant recipient. The transplant patient did not require immediate intervention; two patients required pericardiocentesis alone, and six underwent pericardiocentesis and surgical intervention. All nine perforations were from the femoral venous approach (p < 0.01). Multivariate analysis demonstrated that the greatest risk of perforation occurred in children being evaluated for possible myocarditis (p = 0.01) and in those requiring inotropic support (p < 0.01). Other complications included arrhythmia (5) and single cases of coronary-cardiac fistula, flail tricuspid leaflet, pneumothorax, hemothorax, endocardial stripping and seizure.nnnCONCLUSIONSnRisk of endomyocardial biopsy is highest in sick children with suspected myocarditis on inotropic support. However, EMB can be performed safely with very low morbidity in pediatric heart transplant recipients.

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.

Carotid artery approach for balloon dilation of aortic valve stenosis in the neonate: A preliminary report

Balloon valvuloplasty in neonates with severe aortic valve stenosis is limited by difficulties in catheter manipulation around the arch and across the valve and by the risk of femoral artery complications. A right common carotid artery cutdown was utilized for balloon aortic valvuloplasty in five neonates 1 to 20 days of age, weighing 3.1 to 3.9 kg. Standard balloon valvuloplasty was performed through a 6F sheath inserted in the right carotid artery. The arteriotomy was repaired at the end of the procedure. Mean left ventricular systolic pressure was reduced from 142 to 97 mm Hg, with a decrease in mean peak systolic pressure gradient from 76 to 33 mm Hg. Only one patient developed mild aortic regurgitation. One patient with a hypoplastic left ventricle died, and one patient required open valvotomy. All four survivors have a normal carotid pulse and no neurologic sequelae. Two of these patients required repeat balloon dilation to treat residual aortic valve stenosis at 8 and 10 months of age, respectively. Balloon valvuloplasty using a carotid artery approach is feasible and was safe in five neonates with severe aortic valve stenosis.

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.

Rest and exercise right and left ventricular function late after the mustard operation: Assessment by radionuclide ventriculography☆

To evaluate ventricular function late after atrial repair of transposition of the great arteries (TGA), 26 asymptomatic patients had rest and exercise radionuclide ventriculography performed a mean of 9 years (range 5 to 15) after undergoing the Mustard operation. The mean resting right (systemic) ventricular (RV) ejection fraction (EF) was 0.50 +/- 0.10 (+/- 1 standard deviation); the RVEF was less than 0.45 in 8 patients. With exercise the RVEF increased in 9 patients and either failed to increase or decreased in 15 (including all 8 patients with resting values less than 0.45). The weight-adjusted work load performed was a first predictor of RV exercise response (sensitivity 87%, specificity 92%); patients whose RVEF increased did more work. The mean resting left (pulmonary) ventricular (LV) EF was 0.58 +/- 0.09; the LVEF was less than 0.50 in 3 patients. With exercise the LVEF increased in 14 patients and did not increase in 10 (including all 3 with resting values less than 0.50). The presence of complex ventricular arrhythmia documented on Holter monitoring was a first predictor of failure of the LVEF to increase with exercise (sensitivity 84%, specificity 71%). The patients age, operative age, postoperative interval, residual arterial desaturation, preoperative large ventricular septal defect or pulmonary stenosis, postoperative pulmonary stenosis or superior vena caval obstruction, or performance of a second open-heart procedure was predictive of the rest or exercise EF of either ventricle.

Anomalous origin of one pulmonary artery from the ascending aorta: a review of echocardiographic, catheter, and morphological features.

Six patients with anomalous origin of one pulmonary artery from the ascending aorta were reviewed. Four had anomalous origin of the right pulmonary artery and two had anomalous origin of the left pulmonary artery from the ascending aorta. Two of these six patients had tetralogy of Fallot. Two patients died in the first month of life. No changes in the pulmonary vasculature were seen at necropsy. Corrective surgery was attempted in two patients with associated tetralogy of Fallot when they were two years old but both died. At necropsy there was severe pulmonary vascular disease in the lung supplied by the anomalous pulmonary artery but no pulmonary vascular hypertensive changes in the lung supplied by the pulmonary artery from the right ventricle. Two recent patients underwent successful anastomosis of the anomalous pulmonary artery to the main pulmonary artery at three months and one month and three weeks of age respectively. Intraoperative lung biopsy in the latter patient showed early changes in both lungs. Both echocardiography and cardiac catheterisation were used in the diagnoses. Systemic or suprasystemic pressures were found in the pulmonary artery arising from the right ventricle as well as the anomalous pulmonary artery in the three patients without tetralogy of Fallot. Anomalous origin of a pulmonary artery from the ascending aorta is a distinct entity and differs from other aorto-pulmonary arterial connections. Early surgical intervention is recommended in all patients (including those patients with associated tetralogy of Fallot) because of the risk of rapid development of irreversible pulmonary vascular disease.

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)