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Biomedical subjects

C A Moes

Publications and source records attributed to C A Moes.

At least 19 recordsLinked to original sources

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.

Abnormalities, Multiple↗

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.

Adolescent↗

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.

Angiocardiography↗

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.

Humans↗

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.

Analysis of Variance↗

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)

Abnormalities, Multiple↗

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.

Adolescent↗

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.

Child↗

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.

Child↗

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)

Anastomosis, Surgical↗

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)

Angiocardiography↗

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.

Catheterization↗

The dysplastic pulmonary valve: echocardiographic features and results of balloon dilatation.

The feasibility of using balloon dilatation to relieve stenosis caused by dysplasia of the pulmonary valve was assessed in seven patients (five female, mean age two years) with angiographically confirmed dysplasia who were identified among 38 patients with pulmonary valve stenosis selected for balloon dilatation over a two year period. The clinical features in three patients were consistent with Noonan's syndrome. In all patients the gradient across the valve was assessed by cross sectional echocardiography and Doppler echocardiography before cardiac catheterisation. Balloon dilatation was performed by conventional techniques. In one patient, who had balloon dilatation in the operating room before surgical valvectomy, the diameter of the valve orifice increased from 3 mm to 10 mm. Inspection showed a tear along the anterior commissure. The mean (SD) pressure gradients between the right ventricle and pulmonary artery before and immediately after dilatation in five patients were not significantly different (58 (28) and 47 (12) mm Hg) respectively. There was no overall significant change in the degree of stenosis when four of these patients were examined by Doppler echocardiography six months after operation (44 (17) mm Hg), although one patient (case 5) did show a significant reduction in gradient. This patient had angiographic and echocardiographic features of dysplasia and commissural fusion. Several echographic features were common to all patients and distinguished them from cases of typical pulmonary valve stenosis. These were: pronounced thickening of leaflets; leaflet immobility in diastole and systole; no dilatation of the sinuses of Valsalva in diastole, and supra-annular narrowing. These poor results of balloon dilatation suggest that commissural fusion is not an important mechanism for causing stenosis in the dysplastic pulmonary valve. When dysplasia of the pulmonary valve is identified clinically and echocardiographically, balloon dilatation is unlikely to improve haemodynamic function; it should be attempted if commissural fusion is present.

Angioplasty, Balloon↗

The morphology of the right ventricular outflow tract after percutaneous pulmonary valvotomy: long term follow up.

Twenty nine patients (19 male, mean (SD) age 6.25 (0.5) years (range 0.16-15 years] with typical pulmonary valve stenosis were treated by balloon dilatation of the pulmonary valve. They were studied by echocardiography before the procedure, immediately after it, and at follow up (mean (SD) 10.2 (5.6) months, n = 18). The morphology of the pulmonary valve, the right ventricular-pulmonary artery gradient, and ratio of the systolic to diastolic endocardial dimensions (infundibular ratio) were examined. No patient had pulmonary regurgitation before the study. The valve gradient was significantly reduced (47%) from a mean (SD) of 72 (31) to 37 (23) mm Hg with no short term change in cardiac index after dilatation with a balloon with a mean (SD) diameter that was 118 (10.8)% of the valve annulus. The infundibular ratio was unchanged by the procedure (0.49 (0.11) (n = 21) before dilatation and 0.47 (0.14) (n = 16) after dilatation). In twenty seven patients the commissure of the pulmonary valve was seen to be torn after dilatation. Two patients with bicuspid valves had flail leaflets. Doppler examination at follow up showed mild pulmonary insufficiency in all 29 patients; the mean (SD) valve gradient (31 (+/- 21) mm Hg) at follow up was no different from the gradient found immediately after the procedure and infundibular ratio (0.58 (0.15) was not abnormal. These data indicate that commissural tears are the primary mechanism of valve disruption and demonstrate that the dynamic right ventricular outflow tract obstruction relaxes and gradient reduction persists at follow up.

Adolescent↗

"Horseshoe" lung: report of five new cases.

A "horseshoe" lung is a rare congenital anomaly in which the posterobasal segments of the right and left lungs are fused behind the pericardial reflection at the cardiac apex. Most patients with such pulmonary fusion share many of those cardiovascular anomalies typical of the "scimitar" or hypogenetic right lung syndrome. The clinical diagnosis of horseshoe lung can be made by pulmonary angiography, which shows a branch of the right pulmonary artery originating from its proximal but inferior aspect and coursing into the left hemithorax. Thus, there are two conditions in which an artery from the right lung supplies part or all of the left lung: errant right pulmonary artery in the horseshoe lung deformity and so-called left pulmonary artery sling.

Angiography↗