An increased incidence of total anomalous pulmonary venous drainage among aboriginal Canadians.

OBJECTIVE: To determine whether Aboriginal Canadians from Manitoba and Ontario have an increased incidence of isolated total anomalous pulmonary venous drainage (TAPVD) and to compare results obtained from two different data sources and time periods. DESIGN: A nonconcurrent cohort study was undertaken. Incidence rates and relative risk from 'traditional' data sources (cases from medical records data; births from Census, Vital Statistics and Native Registry data for Manitoba and Ontario) from 1972-84 were derived and compared with those from computerized hospital abstract data from Manitoba for 1987-91. RESULTS: Using traditional data sources an incidence of 0.282/1000 live births was noted in Aboriginals versus 0.062 in non-Aboriginals for a relative risk of 4.6 (95% CI = 2.7-7.7). For Manitoba only the relative risk was 5.8 (95% CI = 2.6-12.8). Using computerized administrative data from Manitoba the relative risk was 5.8 (95% CI = 1.3-25.8). CONCLUSION: There is an increased incidence of isolated TAPVD in Aboriginal peoples from Manitoba and Ontario. Further epidemiological investigation is necessary to determine the nature of this association.

A comparison of iohexol and diatrizoate-meglumine in children undergoing cardiac catheterization.

Iohexol (Omnipaque) and meglumine and sodium diatrizoate (Renografin-76) were compared in a double-blind, randomized study for their efficacy, safety, and hemodynamic effects as angiographic contrast agents in children. Forty-four children were randomly allocated to receive either iohexol or diatrizoate as a component of their routine or emergency cardiovascular evaluation. Following age stratification, baseline physiologic parameters were not significantly different between patients receiving either iohexol or diatrizoate. After systemic ventricular injection, iohexol produced significantly less hemodynamic alteration in systemic systolic blood pressure, systemic ventricular end-diastolic pressure, and dP/dt. Less alteration in heart rate and significantly less effect on the QT interval were seen with iohexol. Image quality was comparable, although significantly more patient mobility was associated with diatrizoate-meglumine. This study shows that iohexol, a nonionic contrast medium, causes less hemodynamic disturbance than diatrizoate-meglumine in children. Therefore, its use to be preferred in these potentially high-risk patients.

Interventricular septal motion and left ventricular function in patients with atrial septal defect.

In order to assess whether the paradoxical motion of the interventricular septum seen in patients with atrial septal defect (ASD) is due to a true abnormality in septal contraction, eight patients with ASD (age, 1.6-17 years) and eight age-matched control patients were studied using qualitative and quantitative two-dimensional (2D) and M-mode echocardiography. 2-D-echocardiographic images recorded from the parasternal short-axis projection at the level of the papillary muscles and 2D-directed M-mode tracings at this level were obtained. Comprehensive wall motion analysis of the left ventricular (LV) endocardial and epicardial borders was performed using both fixed reference and center of mass (floating reference) models. Our results indicate that interventricular septal wall motion and function are normal in patients with ASD. The apparent "paradoxical" motion is due to excessive anterior motion of the entire left ventricle, and is present only when a fixed reference system is used to assess myocardial motion, but is not present when a center of mass (floating reference system) is employed. Left ventricular function assessed by % area and perimeter change, mean radial shortening fraction, and mean radial wall thickening (2D) as well as LV shortening fraction and septal and posterior wall thickening (M-mode) was not significantly different between the two groups. Standard M-mode tracings can therefore be used to assess LV function despite this apparent abnormal septal motion.

Cardiac embryogenesis: a three-dimensional approach.

In order to aid students in the understanding of cardiac embryogenesis, seven three-dimensional fiberglass models depicting stages eleven to eighteen of cardiac embryogenesis, and one model of a mature heart were constructed. The details of these models, and a review of cardiac embryogenesis is presented. The results we have had with these models in the teaching of cardiac embryogenesis to medical students, cardiac technologists, and nurses have been encouraging, and this approach to teaching is highly recommended.

Evaluation of pulmonary vascular resistance in the presence of a large pulmonary arteriovenous malformation.

A 1 day old infant presented with severe cyanosis and congestive heart failure. Cardiac catheterization confirmed the clinical suspicion of a pulmonary arteriovenous malformation of the right lower lobe. Catheter occlusion of the right lower pulmonary artery allowed evaluation of the integrity of the remaining pulmonary vascular bed as well as improvement of the patient's hemodynamic condition pending more definitive therapy.

Severe mitral insufficiency associated with Kawasaki's valvulitis.

A two-month-old male infant with Kawasaki's disease, severe mitral insufficiency, and normal coronary arteries is described. We postulate the mitral insufficiency was secondary to Kawasaki's valvulitis, and that this occurred in the absence of other forms of cardiac involvement characteristic of Kawasaki's disease.

Adjustment following surgical implantation of a penile prosthesis: a critical overview.

The literature on postsurgical adjustment and/or satisfaction of patients and/or their partners following implantation of a penile prosthesis is reviewed. Only those studies that have systematically assessed psychological variables independent of surgical or functional outcome are included. It is reported that while transitory emotional problems were not uncommon, 64-100% of the implant recipients reported being at least fairly satisfied. Similar levels of satisfaction were reported for their partners when they were interviewed together or when patients responded for their partners. However, lower levels were reported when partners were interviewed separately. Satisfaction was reported to be associated with several variables, and high-risk populations were identified. It is important to interpret these conclusions with extreme caution because of the many serious methodological weaknesses contained in these studies. These methodological problems in the literature are reviewed, and recommendations for future research are presented.

Congenital valvular aortic stenosis: surgical management and long-term results.

Case histories of 50 consecutive infants and children ith congenital valvular aortic stenosis treated at The Montreal Children's Hospital during the past 16 years were reviewed in order to determine the efficacy of the initial treatment as well as the long-term results. The operative technique employed was to incise fused commissures out almost to the anulus without causing aortic insufficiency, but often the accomplishment was limited by valve anatomy or cusp dysplasia. Six of the seven operative deaths occurred in infants. Eight to 16 year follow-up on the first 25 survivors revealed one late death, four "good" results, five aortic valve replacements, and three second valvotomies. Ten patients have recurrent aortic stenosis and two have moderate aortic insufficiency. Aortic valvotomy is a palliative operation, and about a third of the children operated upon will require a second operation within 10 years.

DiGeorge syndrome presenting as severe congenital heart disease in the newborn.

In seven infants with DiGeorge syndrome the major clinical manifestation was cardiac failure in the 1st week of life. All had severe congenital heart disease: five had interruption of the aortic arch and associated lesions, one had a ventricular septal defect and a cervical aorta, and one had truncus arteriosus. All but one died by 2 weeks of age. Necropsy data lent support to the hypothesis of a relation between the cardiovascular anomalies and defective development of structures derived from the third and fourth pharyngeal pouches.