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M A Engle

Publications and source records attributed to M A Engle.

At least 37 records · Page 2Linked to original sources
PubMed1989

Cardiac malformations in trisomy-18: a study of 41 postmortem cases.

The cardiac malformations in 41 karyotyped and autopsy cases of trisomy-18 are presented in detail. The salient findings were a ventricular septal defect in all cases; tricuspid valve anomalies in 33 cases (80%); pulmonary valve anomalies in 30 (70%); aortic valve malformations in 28 (68%); mitral valve anomalies in 27 (66%); polyvalvular disease (that is, malformations of more than one valve) in 38 (93%); a subpulmonary infundibulum (conus) in 40 (98%); a bilateral conus with a short subaortic infundibulum in 1 case with double outlet right ventricle (this being the only documented case of bilateral infundibulum in trisomy-18); double outlet right ventricle in 4 cases (10%), three having a subpulmonary infundibulum only and all 4 having mitral atresia; tetralogy of Fallot in 6 cases (15%), 2 having pulmonary atresia; and a striking absence of transposition of the great arteries and inversion at any level (visceral or cardiac), findings that appear to be characteristic of all trisomies. These data suggest that excessive chromosomal material (as in trisomies) may result in situs solitus at all levels. The malformations of the atrioventricular and semilunar valves were characterized by redundant or thick myxomatous leaflets, long chordae tendineae and hypoplastic or absent papillary muscles. The ventricular septal defect was associated with anterosuperior conal septal malalignment in 25 cases (61%). On the basis of the characteristic valvular lesions, the type of ventricular septal defect and the absence of transposition or inversions, two-dimensional echocardiographic diagnosis of trisomy-18 in the fetus may become possible.

Chromosomes, Human, Pair 18↗
PubMed1989

Rug shampoo and Kawasaki disease.

Following personal interviews with 63 families of children with Kawasaki disease and with 63 control families with children paired for race, sex, and age, no epidemiologic differences were seen except for use of rug shampoo within 1 month of onset in 16 episodes in 15 children with Kawasaki disease in 14 families (24% of children, 22% of families) compared with two families of control children (3%, P less than .005). Explanations for this strong association of recent rug shampoo and Kawasaki disease include the question of recall bias as well as the possibility that an agent in the shampooing process does cause or does contribute to illness.

Allergens↗
PubMed1989

Epidemiologic aspects of Kawasaki disease in a Manhattan hospital.

Epidemiologic and clinical features of Kawasaki disease in 106 patients seen between 1980 and 1986 at The New York Hospital in midtown Manhattan were compared with those in large series from the United States, Canada, and Japan. Dissimilarities in our Kawasaki disease experience included ethnic heterogeneity of our patients (50% white, 18% black, 16% Hispanic, and 16% Oriental) and, in comparison with the Japanese experience, an older mean age (3 1/2 vs 1 1/2 years) with fewer children less than 2 years of age (32% vs 50% to 60%). In comparison with the general population of the geographic urban and suburban referral area for our hospital and in comparison with our general pediatric population, Oriental children with Kawasaki disease were overrepresented (16% vs 2%). More families of children with Kawasaki disease were members of the upper and middle class (73%) than were the population seen in general pediatrics (31.7%) at our hospital. Personal interviews with 63 families of children with Kawasaki disease and 63 control families with children paired for ethnic group, sex, and age revealed no epidemiologic differences except for use of rug shampoo within 1 month of onset in 16 episodes in 15 children with Kawasaki disease in 14 families (22% of families) compared with two families of control children (3%) (P less than .001).

Age Factors↗
PubMed1988

Correlation of electrocardiographic and echocardiographic changes in Kawasaki syndrome.

To determine whether changes in ECGs correlated with abnormalities in echocardiograms in children with acute and convalescent Kawasaki syndrome, we undertook a retrospective analysis of 44 patients examined during the first 2 weeks of illness and followed for at least 6 months, 31 for longer than 1 year. We analyzed 360 ECGs, 282 echocardiograms, and clinical status. Results of echocardiography showed 18 children with myocardial dysfunction, pericardial effusion, and/or coronary arterial abnormalities (41%); 26 children had no abnormalities. All 18 of the former and all but 10 of the latter had abnormalities on serial ECGs that normalized on recovery. Thirty-four of 44 patients (77%) had ECG abnormalities. These abnormalities were most prevalent in the first month: 68% appeared in the first week, about 50% in the second to fourth weeks, 16% at 2 months, and 10% at 3 months. The type of ECG change did not predict the type of echocardiographic abnormality, except that low voltage of QRS was found only with pericardial effusion. The most frequent early changes were in T waves, which were flattened in 28 (64%). Later 12 patients had high, peaked, abnormal T waves. Prolonged PR interval occurred in 13 patients, seven of whom had echocardiographic abnormalities. Of six with prolonged QT interval, four had abnormal echocardiograms. The possibility of having an abnormal echocardiogram increased with the number of ECG changes: from 0 risk with no change to 37% for one, 47% for two, 80% for three, and 100% for four changes in serial ECGs. Both ECGs and echocardiograms are important in early and subsequent evaluation of these patients. Inasmuch as the ECGs showed abnormalities in 77% whereas echocardiograms showed changes in 41%, we believe that serial ECGs in comparison with prior tracings offer the more sensitive noninvasive indicator of pancarditis in young children during acute and convalescent Kawasaki syndrome.

Child↗
PubMed1987

Coronary artery involvement in Kawasaki syndrome in Manhattan, New York: risk factors and role of aspirin.

Since January 1980, 110 children having 113 attacks of Kawasaki syndrome were studied. Age at onset was 7 weeks to 12 years (mean 3 6/12 years, median 2 9/12 years); 77% were younger than 5 years of age; the male to female ratio was 1.8; racial distribution was 52% white, 19% black, 14% Hispanic, and 16% Asian. Protocol of management consisted of high-dose aspirin (100 mg/kg/d) until afebrile, and then 81 mg every day until free of coronary aneurysm. Two-dimensional echocardiograms were done weekly during the acute stage, at 2 and 6 months after onset, and yearly if a coronary abnormality was detected. At 1 month, 51 coronary arterial abnormalities were present in 25 patients. Risk factors for a coronary abnormality were duration of fever greater than or equal to 2 weeks, level of platelet count, marked elevation of ESR, and age younger than 5 years. No statistically significant difference in incidence of aneurysms was detected between patients on high-dose aspirin and those on medium-or low-dose aspirin.

Aspirin↗
PubMed1984

Sympathetic nervous system and exercise tolerance response in normotensive and hypertensive adolescents.

Comparative evaluation of isometric and dynamic exercise performance in normotensive, borderline hypertensive and hypertensive adolescents was made. Hemodynamic changes were correlated with level of adrenergic sympathetic nervous system activity as measured by plasma epinephrine and norepinephrine values. No significant intergroup differences were found with respect to isometric exercise with the exception of high peak isometric exercise heart rates and plasma epinephrine levels in the patients with significant hypertension. During dynamic treadmill testing, patients with the most marked hypertension demonstrated a statistically significantly greater tachycardia response to exercise that correlated with highest peak exercise epinephrine levels. The most hypertensive systolic dynamic exercise response was evidenced in the borderline hypertensive group in which peak exercise norepinephrine values were significantly higher than in other groups. Maximal exercise serum lactate levels were higher in hypertensive patient groups than in normotensive subjects. Altered hemodynamic response to peak dynamic exercise appears to exist in adolescents with borderline and significant hypertension and is in part mediated by altered activity of the sympathetic nervous system.

Adolescent↗
PubMed1984

Determinants and importance of atrial pressure morphology in atrial septal defect.

A prominent "v" wave relative to the "a" wave in the jugular vein and right atrial pressure tracing is considered to be a common haemodynamic sign of atrial septal defect. Since the prevalence, age relation, and haemodynamic determinants of the "v" greater than or equal to "a" wave configuration have not been studied the pressure recordings from 15 adults and 80 children with an isolated secundum atrial septal defect in sinus rhythm and from 40 adults and 55 children in sinus rhythm without structural cardiac abnormalities or with coronary and valvular heart disease were studied to assess the sensitivity and specificity of the "v" greater than or equal to "a" wave configuration in atrial septal defect. Only 20% of adults with an atrial septal defect had prominent right atrial "v" waves compared with 63% of children, although the specificity was quite high for each group. In adults "left atrialisation " of the right atrium ("v" greater than or equal to "a" wave) occurred in younger patients with higher right atrial and right ventricular end diastolic pressures. In contrast, in children no age related or haemodynamic determinants for the "v" greater than or equal to "a" pattern were found. In addition, most adults but few children with an atrial septal defect had "right atrialisation " of the left atrial wave configuration ("a" greater than "v"). This was found in older adults with lower right atrial and right ventricular end diastolic pressures and in older children with larger left to right shunts. Thus in contrast to children adults with an atrial septal defect rarely show a prominent "v" wave in the right atrium. The presence of a prominent right atrial "v" wave in adults with an atrial septal defect is associated with relatively higher left atrial and right heart pressures than is the absence of this sign and may be related to relatively higher systolic transatrial flow in these patients. The relative paucity of prominent right atrial "v" waves in older adults suggest that the systolic phase flow may diminish with age, possibly from progressive alteration in compliance of the chronically dilated right ventricle.

Adolescent↗
PubMed1983

Noninvasive diagnosis of persistent fetal circulation versus congenital cardiovascular defects.

Congenital cardiovascular anomalies associated with right-left atrial or ductal shunts must be excluded before a diagnosis of persistent fetal circulation (PFC) can be made. Despite the advent of 2-dimensional echocardiography (2-D echo), this differentiation can be difficult and may require cardiac catheterization with selective angiography. Fifteen consecutive cases were analyzed in which difficulty was encountered with this differential diagnosis, and experience with the use of cardiac auscultation, the 12-lead electrocardiogram (ECG), arterial blood gas determinations and 2-D echo, both alone and with injection of venous contrast material, is reviewed. Electrocardiographic abnormalities of ventricular axis, hypertrophy or dominance (p = 0.002) and suspicion of cardiovascular disease on 2-D echo (p = 0.011) were the most useful findings in differentiating patients with PFC from those with congenital cardiovascular abnormalities. The ECG was the most sensitive test (100% sensitivity, 90% specificity), while 2-D echo was the most specific (100% specificity, 75% sensitivity). Evidence of right-left shunting at atrial or ductal levels or both did not differentiate between the groups; both groups had evidence of such shunts. A decision tree was developed to facilitate this differential diagnosis, which uses the ECG and 2-D echo. If the ECG reveals no abnormalities of ventricular axis, dominance or hypertrophy, the 2-D echo shows no structural abnormalities, and total anomalous pulmonary venous return and coarctation/interruption of the aorta are specifically excluded, a congenital cardiovascular anomaly is effectively eliminated. We suggest that this approach can optimize the management of the cyanotic newborn with suspected PFC by eliminating the risks of cardiac catheterization and angiography without missing the diagnosis of a major structural cardiovascular anomaly.

Diagnosis, Differential↗