Right aortic arch with retroesophageal left aberrant innominate artery.

Right aortic arch with a left retroesophageal innominate artery (type D double aortic arch) is rare. The diagnosis is made by aortography. The present case is the first known patient to undergo a magnetic resonance imaging study that outlined the anomaly clearly.

Left innominate vein compression by a brachiocephalic artery anomaly.

An unique case of left innominate vein compression by a leftward origin of a brachiocephalic artery in conjunction with an aberrant right subclavian artery anomaly occurred in a young patient. Aortography and magnetic resonance imaging were invaluable in arriving at a diagnosis.

Pulmonary blood supply by a branch from the distal ascending aorta in pulmonary atresia with ventricular septal defect: differential diagnosis of fifth aortic arch.

A patient with pulmonary atresia and a ventricular septal defect is described in whom an arterial branch from the distal ascending aorta supplied segments of both lungs. The branch is considered to represent a persistent fifth aortic arch. The possible morphogenesis and differential diagnosis of a communication between the ascending aorta and the pulmonary artery in pulmonary atresia with ventricular septal defect are discussed.

Retrobronchial vertical vein in totally anomalous pulmonary venous connection to the innominate vein and its specific occurrence in right isomerism.

Recently, a case of right isomerism with totally anomalous pulmonary venous connection (TAPVC) to the innominate vein through a vertical vein that ascended in the mediastinum posterior to the left-sided main bronchus was encountered. This retrobronchial vertical vein that has not been described previously in patients with TAPVC to the innominate vein prompted the performance of a retrospective analysis of 44 cases with TAPVC to the innominate vein in which angiography had been performed. The 44 cases consisted of 36 patients with situs solitus, 7 with right isomerism, and 1 with left isomerism. The spatial relation of the vertical vein to the adjacent pulmonary artery and main bronchus could be defined in 41 cases. The vertical vein was retrobronchial in 4 of 5 cases with right isomerism in which the location of the vertical vein could be defined with certainty. No case with situs solitus or left isomerism was associated with a retrobronchial vertical vein. In all 4 patients with a retrobronchial vertical vein, angiographic evidence of obstruction of pulmonary venous drainage was present, and was due to an extrinsic bronchial compression in 2 and to an intrinsic narrowing of the vertical vein in 2.

A case report of double-orifice tricuspid valve.

A heart specimen with double-orifice tricuspid valve is described. The tricuspid valve was divided into the anterior and posterior orifices by a bridge of leaflet tissue. The valve was stenotic because of the arcade deformity of the anterior papillary muscle to which the bridging leaflet tissue had short chordal insertions. The anterior orifice was regurgitant as a result of a deficient septal leaflet. Clinical correlation was obtained by magnetic resonance imaging of the specimen. The functional significance of the malformed valve was assessed by three-dimensional reconstruction of the two-dimensional magnetic resonance images.

Rare types of aortic arch anomalies.

Rare types of aortic arch malformations include isolation of the right or left subclavian arteries in conjunction with a left or right aortic arch respectively. Isolation of the innominate or carotid arteries and an aberrant left innominate artery may exist in association with a right aortic arch. Other anomalies are persistent fifth aortic arch, subclavian artery as the first branch of the aortic arch, pulmonary artery, and ductus arteriosus sling. The angiocardiographic features and embryology of each of these malformations are discussed.

Results of the Fontan procedure for patients with univentricular heart.

One hundred twenty-four consecutive patients with univentricular heart undergoing the Fontan operation were reviewed. Patients with tricuspid atresia or biventricular heart with hypoplasia of one ventricle were excluded. Eighty-four patients had left ventricular morphology. Atrioventricular connection was double-inlet (n = 76), common (n = 29), absent left atrioventricular connection (n = 14), and absent right atrioventricular connection (n = 5). Actuarial survival was 77% (70% confidence limits, 73% to 81%) at 1 year, 66% (70% confidence limits, 60% to 72%) at 5 years, and 49% (70% confidence limits, 36% to 61%) at 10 years, indicating a continuing risk for premature death. Multivariate analysis identified preoperative ventricular function and hypertrophy as risk factors for survival. High postrepair right atrial pressure (greater than 15 mm Hg) emerged as a strong intraoperative predictor of survival. Logistic regression analysis of these factors predicts high probability of death for certain subgroups of patients after the Fontan operation. Forty-four percent (n = 53) of these original 124 patients are alive and in New York Heart Association class I at follow-up. Thirty-eight percent (n = 33) of survivors have worse ventricular function than preoperative. Long-term survival is disappointing. Certain identifiable subgroups of patients with univentricular heart have unacceptable risks for the Fontan operation and should have alternate management. High postrepair right atrial pressure is an ominous sign, and if it persists the Fontan should be fenestrated or taken down.

Spectrum of cardiovascular anomalies in Williams-Beuren syndrome.

This study is presented to identify and characterize the spectrum of the cardiovascular anomalies in children presenting with Williams-Beuren syndrome and cardiovascular anomalies at The Hospital for Sick Children, Toronto from 1966 to 1988. Forty-nine children were diagnosed and followed. The female to male ratio was 1.2:1. The age ranged from 1 month to 14 years at the time of diagnosis (mean 39 months), and follow-up periods were from 9 months to 20 years (mean 10 years). All patients having the typical features were also evaluated by geneticists. Based on cardiovascular findings four groups were identified. Group 1 had isolated supravalvular aortic stenosis (SVAS) (28 patients). There was follow-up in 24 of these children. Six had worsening of supravalvular narrowing and underwent surgery. One showed an increased gradient from 10-40 mmHg during 7 years. Seventeen had mild narrowing and showed no progression over a period of 75 months. Group 2 had isolated pulmonary artery branch stenosis (8 patients). Seven had mild narrowing which remained unchanged over a mean period of 16 months and one underwent surgery. Group 3 had combined lesions (11 patients). Six showed increased left-side narrowing, while right-side obstruction remained static or improved. Five showed improvement in narrowing in both outflow tracts. Five underwent surgery. Additional cardiovascular anomalies included peripheral artery stenosis in two patients, coronary artery abnormalities in three, mitral valve prolapse in three, and coarctation of the aorta in two. Group 4 had isolated lesions. One patient had isolated coarctation of the aorta and one isolated mitral prolapse.(ABSTRACT TRUNCATED AT 250 WORDS)

Repair of coarctation of the aorta in children: postoperative morphology.

To determine the morphologic sequelae after surgical repair of coarctation of the aorta, the authors retrospectively reviewed angiograms and hemodynamic and clinical data on 215 patients who underwent cardiac catheterization after surgical repair of coarctation of the aorta during a 13-year period. Ninety-seven patients (45%) underwent coarctation resection with end-to-end anastomosis; 92 (43%), subclavian-flap angioplasties; and 26 (12%), synthetic-patch repairs. Sixty-four patients (30%) had an "aneurysm," defined as a measurement ratio of repair site to diaphragmatic aorta greater than 1.5. Transverse-arch or isthmic hypoplasia or recoarctation was detected in 86 patients (40%) and was most commonly associated with septal defects or obstruction of the left ventricular outflow tract. Pullback systolic pressure gradients at catheterization were significantly higher (P = .0001) in the patients with transverse-arch hypoplasia and recoarctation than in those with ratios of transverse arch to diaphragmatic aorta greater than 0.9. Significant postoperative arch obstructions can be predicted with measurement ratios on the basis of the diameter of the abdominal aorta.

Analysis of survival in patients with pulmonic valve atresia and ventricular septal defect.

This study reviews the clinical course of 104 consecutive patients with pulmonic valve atresia and ventricular septal (VSD) defect who were diagnosed in the first year of life and followed for a mean period of 4.95 years (range 2 days to 13.75 years). Specific attention was paid to the nature of the pulmonary blood supply and to its influence on patient outcome. Confluent pulmonary arteries supplied by a single ductus arteriosus were present in 72 patients (69%, group I), whereas 32 patients (31%, group II) had a pulmonary blood supply that was partially or exclusively dependent on systemic collateral arteries. An estimate of the probability of survival for 10 years was 69% in the entire cohort, with no different between patients in group I and group II. Definitive surgical repair was performed in 33 of 72 group I patients (46%), compared with 5 of 32 group II patients (16%). Arborization and distribution abnormalities of the pulmonary arteries as well as intrapulmonary stenoses that were exclusively present in patients with systemic collateral arteries (p less than 0.00001) accounted for the significantly lower probability of undergoing corrective surgery in group II patients.

Thromboexclusion of the right ventricle in children with pulmonary atresia and intact ventricular septum.

Twelve children with pulmonary atresia and intact ventricular septum underwent closure of the tricuspid valve as a part of a new surgical procedure. In two cases a concomitant Fontan operation was performed. In each patient the right ventricle was very small and right ventricular pressure was higher than systemic pressure. Ventricle-coronary connections provided flow of desaturated blood from the right ventricle into the coronary arteries in 11 of 12 cases. Five of the 12 children did not survive operation and postmortem examination of each revealed severe acute and chronic myocardial ischemic damage and high-grade obstruction or interruption of the proximal left anterior descending coronary artery. Preoperative angiography demonstrated occlusive changes in the coronary arteries, resulting in right ventricular dependent circulation, in all five children who died and in one child who survived operation. Seven children who survived operation are well 4 months to 3.5 years later. Two have undergone subsequent successful Fontan operation and two others are considered suitable candidates for this operation. Tricuspid valve closure is recommended for a carefully selected group of infants with pulmonary atresia and intact ventricular septum provided a right ventricular-dependent coronary circulation can be excluded on the basis of preoperative coronary cineangiography.

William Glenn lecture. The cavopulmonary shunt. Evolution of a concept.

A bold and imaginative development, the cavopulmonary anastomosis, appeared to originate in several centers almost simultaneously. After extensive research on right heart bypass, Glenn was the first in North America to perform a successful experimental cavopulmonary shunt, and it became known by his name. In properly selected patients, palliation success was excellent, and mortality rates were low. From 1961 through 1988, we used a cavopulmonary anastomosis for palliation in 139 infants and children. There were eight hospital deaths, and most occurred early in the series. Palliation generally lasted 6-8 years-until the child outgrew the blood supply to the contralateral lung. Palliation could be restored by increased flow to that lung with another shunt. Six otherwise inoperable patients received benefit from the addition of an axillary arteriovenous fistula. Late pulmonary arteriovenous fistulas were identified in 11% of our patients by angiography, but with more sensitive testing, the incidence rate may be as high as 21%. The occurrence of pulmonary arteriovenous fistulas caused general concern and less frequent use of the shunt. Recent application of an end-to-side anastomosis, creating a bidirectional shunt, has restored interest. A major legacy of the cavopulmonary anastomosis was demonstration of the feasibility of partial right heart bypass, which paved the way for the Fontan operation, and it is frequently constructed as part of that operation. Currently, the Glenn shunt is most often used as a temporary or permanent alternative to a Fontan repair if there appears to be significant risk. The risk factors usually encountered include small pulmonary arteries, young age, poor ventricular function, atrioventricular valve incompetence, and myocardial hypertrophy-sometimes alone but often in combination.(ABSTRACT TRUNCATED AT 250 WORDS)

Coronary angiography of pulmonary atresia, hypoplastic right ventricle, and ventriculocoronary communications.

A retrospective study was undertaken to determine the spectrum of angiographic abnormalities of the coronary arteries in infants and children with pulmonary atresia, hypoplastic right ventricle, and right ventriculocoronary arterial communications. Twenty-nine patients with 67 angiographic examinations were reviewed; findings in seven patients were compared with those at autopsy. Twenty-seven (93%) of 29 patients had caliber abnormalities of the involved coronary arteries, including obstructive lesions in 20 (69%) of 29 and segmental dilatation in 16 (55%) of 29. Nine patients had interruption of the anterior descending artery and one had absent connection between the coronary arteries and the aorta. A single coronary artery was found in four of 29 patients. There was excellent correlation between clinical angiography and autopsy findings in seven patients. This study confirms the ability of high-quality clinical angiography to show significant abnormalities of the coronary arteries in infants and children with pulmonary atresia and ventriculocoronary communications.

Pulmonary artery tears following balloon valvotomy for pulmonary stenosis.

Tears involving the anterior supravalvar annulus developed in 2 children and an infant following percutaneous balloon pulmonary valvotomy using oversized balloons. The 3 patients had angiographic features of three different types of stenotic valves: usual pulmonary valve stenosis in 1, a form of dysplastic pulmonary valve with supravalvar narrowing in a second, and a doming valve in a neonate. All had a successful reduction in right ventricular outflow tract gradient following the procedure. The pulmonary arterial tears were not associated with balloon rupture or clinical symptoms. It is postulated that the relative deficiency of elastic fibers in the supravalvar commissure makes this site relatively vulnerable to intimal tearing.

Surgical experience with left ventricular outflow tract obstruction in patients with complete transposition of the great arteries and essentially intact ventricular septum undergoing the Mustard operation.

During a 24-year period (1963-1987), 46 infants and children with complete transposition of the great arteries (CTGA) and an essentially intact ventricular septum (IVS) have undergone surgical relief of left ventricular outflow tract obstruction (LVOTO) concurrently with the Mustard operation (MO). The obstruction was valvar in 2 patients, subvalvar in 43 and combined valvar and subvalvar in 1; the ventricular septum was intact in 42, and 4 patients had a small VSD at the time of repair. Neonatal and pre-Mustard cardiac catheterization (CC) data were analyzed to obtain LV to right ventricular peak systolic pressure ratio (LV/RVPSP) and LVOT peak systolic gradient (PSG). The mean neonatal LV/RVPSP (n = 31) was 0.79 +/- 0.04; pre-Mustard LV/RVPSP (n = 44) was 0.75 +/- 0.05; pre-Mustard LVOT PSG (n = 34) was 46.5 +/- 3.5 mmHg. Neonatal (n = 29) and pre-Mustard (n = 30) cardiac angiograms were retrospectively reviewed to analyse morphologic substrates of LVOTO. In the neonatal period, LVOTO was present in 7 patients (anatomic, n = 4; dynamic, n = 2; combined, n = 1). Immediately prior to the MO, LVOTO was present in 29 (anatomic, n = 20; dynamic n = 2; combined, n = 7). Techniques to correct LVOTO at the initial MO included pulmonary valvotomy (n = 3), ventriculomyectomy (n = 41), resection of an windsock aneurysm (n = 1), LV to pulmonary artery valved conduit (VC) (n = 1). Two patients underwent VC early postoperatively (PO) for severe residual LVOTO (both died).(ABSTRACT TRUNCATED AT 250 WORDS)

Angiographic features associated with percutaneous balloon valvotomy for pulmonary valve stenosis.

Changes in the mobility of the pulmonary valve were determined by a retrospective review of right ventricular cineangiograms from 25 balloon pulmonary valvotomy (BV) procedures in 23 infants and children. The angiographic changes were compared with the post-BV catheter and Doppler pressure gradients across the right ventricular outflow tract. Angiographic features felt to indicate valve tearing were present following 17 of 25 procedures and included increased excursion or straightening of leaflets, localized change in leaflet motion (flail leaflet), and the presence of an additional contrast jet through the valve. There was no statistically significant relationship between any of the angiographic parameters and the pressure data. Most patients with marked increase in angiographic valve mobility had low residual right ventricular to pulmonary artery gradients. However, the absence of angiographic change was not always associated with a high residual gradient.