Standards for exercise testing in the pediatric age group. American Heart Association Council on Cardiovascular Disease in the Young. Ad hoc committee on exercise testing.
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Biomedical subjects
Publications and source records attributed to J H Moller.
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Two cases are presented of persistent common atrioventricular (A-V) canal with interventricular communication in which associated membranous subaortic stenosis became apparent only after correction of the basic malformation and replacement of the mitral valve. The features of the membranous subaortic stenosis present only after operation were a left ventricular outflow pressure gradient and angiocardiographic demonstration of the membrane. In one case, additional echocardiograms demonstrated systolic flutter of the aortic cusps after the first operation (no echocardiograms were taken preoperatively). The flutter disappeared after a second operation in which the subaortic membranous tissue was resected. The appearance of hemodynamic signs of subaortic stenosis after the corrective operation for the persistent common A-V canal is explained by the fact that preoperatively the interventricular communication allowed decompression of the left ventricle so as to preclude a left ventricular outflow gradient. It is presumed that the use of additional angiocardiographic projections might have demonstrated the anatomic state preoperatively.
A structured interview was tested as a means of constructing patient management problems (PMPs). Three paediatric cardiologists and three medical students were each interviewed separately to collect information to construct PMPs. Four PMPs--two devised by cardiologists and two by students--were constructed. The PMPs were administered to other doctors and medical students. No difference in performance or user reaction were found between the PMPs authored by doctors or those by students. We believe PMPs can be developed by a time-saving technique of a structured interview, and that senior medical students can provide a reservoir for realistic PMPs.
Criss-cross heart is an angiographically confusing malformation where apparent crossing but not mixing of contrast medium is seen at atrioventricular level. The malformation can be understood by a clockwise or counter-clockwise ventricular rotation. In the normal heart or one with complete transposition ventricular rotation is clockwise. With corrected transposition rotation occurs in a counter-clockwise fashion. One case of criss-cross heart with associated corrected transposition and two criss-cross hearts occurring with complete transposition are reported.
Restrictive cardiomyopathy characterized by evidence of left ventricular inflow obstruction was studied in four cases--one child and three adults. In three of the cases, including the child, there was pathologic evidence of hypertrophic cardiomyopathy with asymmetric septal hypertrophy. In the fourth case, no cardiac disease was identified as the anatomic basis for the inflow restriction. This latter case broadens the concept of restrictive cardiomyopathies.
Echocardiographic left atrial dimension has been reported to correlate with left-to-right shunt (Qp/Qs) in children with isolated ventricular septal defect (VSD) or patent ductus arteriosus (PDA). In 17 children with isolated VSD, we prospectively compared Qp/Qs with the ratio of echocardiographic left atrial dimension to aortic root diameter (LAD/Ao) and with the left atrial dimension corrected for body surface area (LAD/M2). The correlation between LAD/Ao and Qp/Qs was not statistically significant (r = 0.49, p > 0.05). The LAD/M2 correlated significantly with Qp/Qs (r = 0.66, p < 0.01); however, prediction intervals were excessively wide. Our findings indicate that it is inappropriate to predict Qp/Qs in these patients from echocardiographic LAD.
Fabry's disease results from deficient activity of the enzyme alpha-galactosidase A. Cardiac abnormalities result from glycosphingolipid deposition in the myocardium, valvular tissue, and vessel walls. A noninvasive method to examine these abnormalities would be useful in the evaluation of patients. We examined the echocardiograms of 32 patients, 25 hemizygous and seven heterozygous, for Fabry's disease. The aortic root diameter was measured in each hemizygote. In nine patients under 26 years it was 33.2 +/- 1.2 mm. and in two it was dilated. In 16 patients over 26 years it was 38.8 +/- 1.2 mm. (p < 0.01), and in 12 it was dilated. The left ventricular posterior wall was measured in the echocardiogram of 21 normotensive hemizygotes. The difference in thickness between nine patients under 26 years (9.6 +/- 1.3 mm.) and 12 patients over 26 years (12.4 +/- .07 mm.) was not statistically significant. Only two of the nine younger patients had left ventricular wall thickness greater than normal compared to eight of the 12 older patients. The mean left ventricular shortening fraction of 22 hemizygous patients was normal. One hemizygote had echocardiographic evidence of mitral valve prolapse. Four of the seven heterozygotes had normal echocardiograms. Among the other three, one had increased left ventricular wall thickness, a second had disproportionate ventricular septal thickness, and a third had both abnormalities. The echocardiographic aortic root size was normal in each heterozygote. Abnormal echocardiographic findings were more common in older hemizygous patients, a distribution similar to that of the age of onset of cardiac dysfunction. Increased left ventricular wall thickness probably reflects glycosphingolipid deposition in the myocardium. Dilatation of the aortic root may result from degenerative changes of the aortic media. Abnormalities of mitral valve echoes were uncommon.
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Anomalies and disease processes of the coronary arteries in children are uncommon. However, with the recent advances in cardiac surgical techniques, many of these lesions are amenable to surgery that requires accurate anatomic delineation by angiography. Ascending aortography is the angiographic procedure commonly used to image the coronary arterial anatomy in children. In some instances this procedure fails to show the extent or nature of the pathologic process. In some cases, selective coronary arteriography is essential for successful management. Twelve patients are presented in whom selective coronary arteriography was indicated, and its value is compared with that of nonselective angiography. Indications for selective coronary arteriography in children are given, and its complications are also discussed.
Recent reports suggest that peak left ventricular systolic pressure can be estimated from the echocardiogram. This study compares the accuracy of echocardiographic measurements with clinical estimates of severity of aortic stenosis derived from information of the Joint Natural History Study. Twenty-seven children with isolated aortic valve stensosis were evaluated clinically and with echocardiography and cardiac catheterization. From the echocardiograms, the ratio of systolic left ventricular wall thickness to internal dimension (Ws/Ds) showed a better correlation (r = 0.82) with peak left ventricular systolic pressure than did the diastolic ratio. However, there was significant variation so that a precise estimate of peak left ventricular systolic pressure could not be made from the echocardiogram. Eight of the 27 patients had a normal Ws/Ds ratio (0.55 or less), and their cardiac catheterization data revealed mild aortic stenosis not requiring operation. In contrast, clinical criteria based on the Natural History Study identified only three of the patients with mild aortic stenosis. Prospective studies are needed to confirm the usefulness of echocardiography in noninvasive evaluation of children with aortic valve stenosis.
Pulmonary valve replacement is an uncommon surgical procedure, yet lifesaving when performed under the appropriate circumstances. The patient reported on here had the classic indications for valve replacement: increased pulmonary vascular resistance and associated pulmonary valve incompetence following repair of a congenital heart defect resulting in right heart failure and secondary tricuspid insufficiency. A review of the literature provides the basis for the decision to replace the pulmonary valve with a glutaraldehyde-preserved porcine heterograft.
Sixty-three patients who underwent banding of the pulmonary artery between 1968 and 1975 were studied. Mortality among patients who underwent the banding procedure was 22%, and among those with ventricular septal defect it was 7%. Thirty-eight of the 49 survivors of the banding procedure were investigated for abnormalities of the pulmonary outflow tract caused by the band. Seventy-one percent of these 38 patients had an identifiable abnormality of the pulmonary valve or artery. These complications occurred more frequently in patients banded at a very young age (less than 2 months old) and in patients in whom the band was in place more than two years. Corrective operations have been performed in 24 of the 49 patients who survived banding. Seventy-six percent of the patients with ventricular septal defect survived corrective operation, while only 29% of the patients with more complex lesions survived.
Our study was undertaken to determine the type, incidence, and functional significance of residual anomalies in patients who have undergone corrective repair for tetralogy of Fallot. We reviewed data from cardiac catheterisations performed on 132 survivors. A significant residual ventricular septal defect was present in only 12 patients. Resting right ventricular systolic pressure was less than 80 mmHg in 100 patients and ranged from 80 to 150 mmHg in the other 32 patients. Thirty-five patients were studied both at rest and during supine exercise. In most patients, the relation between oxygen consumption and cardiac output was normal during exercise. The stroke index and right ventricular end-diastolic pressure at rest and on exercise were compared in 34 patients. Seventeen showed a normal response to exercise. In the other 17 patients, right ventricular end-diastolic pressure rose on exercise; in 5 of these the stroke index fell during exercise, indicating abnormal myocardial response. Our studies indicate the frequent occurrence of residual abnormalities, even in patients who appear asymptomatic, after total correction of tetralogy of Fallot.
Necropsy studies were performed in 125 infants, 2 years old or less, with a variety of congenital heart malformations. Disproportionate ventricular septal thickening (septal-to-left ventricular free wall thickness ratio greater than or equal to 1.3) was present in 31 (25%) of the 125 patients. In the majority of patients, an abnormal septal-to-free wall ratio did not appear to be a manifestation of genetically transmitted hypertrophic cardiomyopathy, since: 1) marked disorganization of septal myocardium (involving greater than or equal to 5% of the tissue section) was present in only five of the 31 patients with disproportionate septal thickening; and 2) echocardiographic studies in first degree relatives of four other patients with disproportionate septal thickening and normal septal architecture did not show asymmetric septal hypertrophy. The relatively high prevalence of septal-to-free wall ratios of 1.3 or greater in this population of infants with congenital heart disease appeared to be due in part to minor differerences in ventricular wall thicknesses (in patients with relatively thin absolute wall thicknesses) which may produce particularly large deviations from unity in septal-to-free wall ratio. Hence, 1) nongenetically transmitted disproportionate septal thickening is relatively common in infants with congenital heart diseases studied at necropsy: and 2) a septal-to-free wall ratio of greater than or equal to 1.3 cannot be used as the sole criterion for identifying associated hypertrophic cardiomyopathy in infants with other congenital heart diseases, particularly if marked absolute septal thickening is absent.
Management of symptomatic atrioventricular canal (AVC) in infancy may be difficult. Between July, 1969, and September, 1977, 31 infants with complete AVC presented in congestive heart failure (CHF) to the University of Minnesota Hospitals. Fifteen of these patients have responded to medical management and have been followed as outpatients. The other 16 patients remained in CHF. Six of them died of persistent heart failure within 4 months. The other 10 infants, aged 3 weeks to 1 year (mean 4 months), underwent pulmonary artery banding and seven survived operation. One of the survivors died with apparent pneumonia 1 month postoperatively. Each of the remaining six patients, who have been followed for 9 months to 9 years, had minimal mitral insufficiency and a large ventricular shunt. The three patients dying after banding had significant mitral insufficiency. We believe that pulmonary artery banding is an effective palliative procedure for infants with complete AVC and CHF who have large ventricular shunts and minimal mitral insufficiency.
Six infants with anomalous connection of the pulmonary veins to the coronary sinus were studied with single crystal echocardiography. The posterior left atrial wall echo was identified by its continuity with the left ventricular posterior wall echo during a sweep from the left atrium to the left ventricle. In five of the six infants an echo-free space measuring 7 to 9 mm was identified behind the posterior left atrial wall; this space was thought to represent the common pulmonary vein. In four of these five, and also in the remaining infant, a highly mobile linear echo with a double wave form per cardiac cycle was recorded behind the posterior aortic wall and anterior mitral leaflet echoes. Echocardiographic contrast studies after the injection of saline solution into the coronary sinus disclosed that this additional echo was produced by the anterior wall of the coronary sinus. The anatomic connection of the pulmonary veins were established in each patient with pulmonary cineangiography. In four of the six patients all four pulmonary veins were connected to the coronary sinus by way of a common pulmonary vein; in the remaining two patients three of four pulmonary veins were connected to the coronary sinus, while the left upper lobe pulmonary vein was connected to the left innominate vein. The coronary sinus was greatly enlarged in each patient.
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