C.A.F. Moes

Spectrum of cardiovascular anomalies in Williams-Beuren syndrome

SummaryThis 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 geneticsts. 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. In conclusion, supravalvular aortic stenosis was the most common lesion, whereas pulmonary artery stenosis improved in most patients. The role of coronary artery abnormalities has yet to be defined.

Bilateral ductus arteriosus (or remnant): An analysis of 27 patients

Bilateral ductus arteriosus (DA) was clinically recognized in 27 patients studied angiographically from 1963 through May 1983. Distal bilateral DA origin of non-confluent pulmonary arteries was identified in 15 patients, ectopic or distal ductal origin of 1 pulmonary artery in 9 patients (5 without evidence of intracardiac disease) and isolation of the left subclavian artery in 3 (all 3 of whom had a right aortic arch). Other conditions reported to be associated with bilateral DA include interruption of the aortic arch with isolation of a subclavian artery, aortic atresia with interruption of the aortic arch in which bilateral DA supports the entire systemic circulation, bilateral DA complicating forms of congenitally malformed hearts other than those just stated, and, rarely, bilateral DA in isolation. Understanding the symmetric or paired nature of the primitive aortic arch system in the developing human heart facilitates recognition of the patterns of fourth and sixth arch anomalies seen with bilateral DA.

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.

Echocardiographic evaluation of obstructive mechanism of tetralogy of Fallot with restrictive ventricular septal defect

Abstract Despite the trend toward earlier total surgical correction of tetralogy of Fallot, a number of complicating factors, most often related to unfavorable pulmonary arterial anatomy, 1 and, rarely, reduced left ventricular size, 2 may make palliation with a systemic to pulmonary arterial anastomosis necessary before corrective surgery. A restrictive ventricular septal defect (VSD) complicating tetralogy of Fallot is one of the less well-recognized features associated with a high mortality when surgical repair is delayed beyond infancy. 3–11 The echocardiographic recognition of this anomaly should be of importance because of the trend toward use of noninvasive diagnostic modalities in infants with various forms of congenital heart disease.12 This study reviews echocardiographic features of tetralogy of Fallot in patients with a documented restrictive VSD, and examines the contribution of clinical findings to the diagnosis of this entity. The progression over time of VSD restriction and its impact on surgical management are also assessed.

The atrioventricular junction in the univentricular heart: A two-dimensional echocardiographic analysis

SummaryThe atrioventricular junction of 40 patients with univentricular heart was evaluated by two-dimensional echocardiography. The apical 4 chamber view optimally imaged the atrioventricular junction, and allowed determination of the type of atrioventricular connection: double inlet, common atrioventricular orifice, and absent right or left atrioventricular connection. When double inlet to 1 ventricle was demonstrated, the 4 chamber view allowed immediate comparison of the form and function of the right and left atrioventricular valves. Because anomalies of the atrioventricular valves frequently complicate the univentricular heart, two-dimensional echocardiographic assessment is a most important adjunct to the preoperative investigation of these patients.

Anomalous pulmonary venous connections

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