Biomedical subjects
Publications and source records attributed to D W Sapire.
Two patients with cervical aortic arch are described, both with deletions in chromosome 22q11 region, and thymic hypofunction. This suggests that cervical aortic arch is part of the spectrum of the CATCH 22 group of defects.
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Interatrial septal aneurysm (IASA) has been detected with increasing frequency since the advent of two-dimensional echocardiography (2DE). In this study, we investigated the prevalence, characteristics, and predisposing factors in IASA formation in 724 infants, children, and adolescents undergoing 2DE. Among this group, 12 patients had an IASA (prevalence = 1.7%). Eleven of the 12 patients had abnormal atrial hemodynamics as a result of either structural heart disease or significant supraventricular dysrhythmias. Follow-up 2DE performed in 10 of 12 patients, 1 to 48 months (mean 16.5 months) after the initial diagnosis, demonstrated diminution or resolution of the IASAs in those patients who had normal hemodynamics following surgical or spontaneous closure of their intracardiac defect, or medical control of their dysrhythmias. In contrast, IASAs became more prominent in those patients with persistent hemodynamic abnormalities. The high prevalence rate and subsequent involution of many IASAs in infants suggest that in the presence of abnormal atrial hemodynamics, the neonatal atrial septum may be prone to aneurysm formation because of inherent structural weaknesses of the neonatal septal tissue. In the absence of continued hemodynamic stress., the majority of these IASAs in infants resolve with maturational changes in the septum and normal cardiac growth.
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Fifty-one patients at the Brompton Hospital were identified as having either right or left atrial isomerism by reviewing the records and catheterization, echocardiographic and, in 17 cases, autopsy data. Every study patient had 1 or more of the following conditions: isomeric bronchial anatomy; angiographic or necropsy evidence of atrial anatomy; echocardiographic diagnosis of isomerism; and anomalies of systemic or pulmonary venous connection. Patients with anomalous venous connections all had additional signs of isomerism. Neither right nor left type was predominant in either sex. The heart was in the left chest slightly more often than the right. Abdominal visceral heterotaxy was the most frequent radiographic finding. An ambiguous and biventricular connection was the commonest type of atrioventricular (AV) connection in left isomerism. A common AV valve was the most frequent mode of connection in both forms. Univentricular AV connection, double-outlet right ventricle, pulmonary atresia and discordant ventriculoarterial connection were commoner in right isomerism. Left isomerism is associated with much longer survival, its constellation of associated malformations frequently being less severe and more amenable to corrective surgery. A precise diagnosis must be made during life in order to select the most appropriate treatment.
This study evaluates and compares the effects of right and left atrial stimulation on atrioventricular conduction in ten children without electrocardiographic evidence of dysrhythmias. Atrioventricular nodal conduction (AH) shortened from left atrial stimulation but not from right atrial stimulation. No statistical difference was demonstrated between right and left atrial refractory periods. Similar findings have been shown in adults who were evaluated for dysrhythmias or conduction defects during coronary sinus pacing. Therefore, the internodal pathway through which left atrial activation reaches the atrioventricular node retains its properties of fast conduction throughout the maturational process.
Verapamil has become a popular antiarrhythmic drug for the acute management of supraventricular tachycardia in infants and children. A full-term, 1-hour-old infant presented with supraventricular tachycardia and hypotension that did not respond to vagal maneuvers and direct current cardioversion. After intravenous verapamil, the heart rate slowed and the underlying rhythm was atrial flutter.
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In this report, a 12-hr-old male infant was demonstrated to have the absent connexion variant of right atrioventricular valve atresia with the left atrium connected to the morphologically right ventricle with a right-sided rudimentary left ventricle. The aorta arose from the right ventricle and there was pulmonary atresia, the pulmonary circulation being duct-dependent. In addition there was right juxtaposition of the atrial appendages. The definitive cross-sectional echocardiographic and angiographic findings are presented.
Twenty-four patients with various manifestations of increased vagal tone were seen between 1975 and 1981. Three distinct groups could be identified by clinical characteristics and to some extent by age. The first group showed evidence of prolonged atrioventricular conduction on their electrocardiograms but were asymptomatic. The others presented with syncope, the etiology of which was determined to be vasovagal. Some patients were older and more athletic. They suffered from syncope associated with exercise or heavy exertion. The youngest patients, however, experienced frequent prolonged episodes of syncope often mistaken for seizures. Treatment for the older patients with syncope consisted mainly of behavior modification, while 5 out of 6 younger patients required the implantation of a ventricular demand pacemaker to prevent repeated and problematic recurrences of syncope.
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Hemoptysis was the presenting symptom in a 4-year, 11-month-old male who had had a Mustard operation for hemodynamic correction of transposition of the great vessels at the age of five months. Chest roentgenography demonstrated hyperlucency of the left lung and tomography showed compression and narrowing of left main stem bronchus. Angiography documented the absence of antegrade flow in the left pulmonary artery and no pulmonary venous drainage on the left. The left lung was supplied by bronchial collateral arteries which drained by retrograde filling of the left pulmonary artery. It is surmised that pulmonary venous drainage on the left was compromised at surgery and that the dilated main pulmonary artery compressed the left main stem bronchus. This combination promoted bronchial collateral ingrowth. Hemoptysis is a complication of enlargement of bronchial collateral vessels.
Two patients with polytetrafluoroethylene (PTFE) grafts developed mycotic aneurysms at the suture lines. Both patients had chronic illnesses and recurrent infections. Attempts to surgically treat these aneurysms were unsuccessful. Infections involving PTFE grafts used in the management of children with congenital heart disease have not been reported. Problems related to diagnosis and management are discussed.
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Four children with systolic thrills and harsh pansystolic murmurs in the second left and right intercostal spaces, radiating to the base of the neck, and suspected of having a subaortic ventricular septal defect and possible aortic prolapse underwent angiocardiography using axial projections. All demonstrated an unusual "tunnel-like" track into the right ventricle and no subaortic ventricular septal defect or prolapse. In our opinion, these physical findings suggest this abnormality and that axial angiography clearly delineates the lesion.
Four children presented with episodic loss of consciousness. Two of the children were siblings. Neurologic causes were initially suspected in all but extensive evaluations and EEGs excluded seizures. ECGs in one patient demonstrated first- and second-degree AV block and first-degree AV block in another. The QT and QTc intervals were normal in all. Eyeball pressure in all patients produced profound bradycardia. All patients became asymptomatic after the implantation of pacemakers, although one died 15 months afterward from another cause.
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