Asymmetric septal hypertrophy in infants of diabetic mothers.
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Biomedical subjects
Publications and source records attributed to J G Stevenson.
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The patient we have described had coronary artery aneurysms pathognomonic for Kawasaki disease after a nonspecific febrile illness not meeting the classic clinical criteria. The child had significant thrombocytopenia, which in the past has been thought to militate against the diagnosis of Kawasaki disease. Echocardiography should be considered in febrile infants with thrombocytopenia of uncertain etiology.
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Forty-four youngsters with precordial murmurs and carotid bruits were evaluated clinically and independently, using pulsed Doppler ultrasound. The precordial murmur was evaluated with M-mode echocardiography combined with Doppler flow evaluation, and the carotid bruit was evaluated with peripheral vascular sector scan with Doppler flow evaluation. These ultrasonic techniques can identify abnormal blood flow at anatomic sites such as the aortic valve and in the carotid arteries. The patients had no symptoms and their condition, except for six, was mild enough that catheterization was not indicated. The clinical diagnosis of aortic stenosis was made in 30 children, and nine were thought to have no heart disease. On the basis of the ultrasonic examinations, 28 patients were diagnosed as having aortic stenosis and seven subjects had no intracardiac turbulence. However, there was disagreement in 14 instances; four of the six clinical "normals" were found to have aortic stenosis by pulsed Doppler echocardiography; six patients diagnosed as having mild aortic stenosis on a clinical basis were found to have no aortic abnormality. The results confirm that aortic stenosis usually presents as a murmur maximal in the aortic area, which is associated with a carotid bruit. Unfortunately, in at least one-fourth of the cases the murmur was not maximal at the aortic area, and a carotid bruit was found in several normal subjects. Since the consequences of over- or under-diagnosis of aortic stenosis are substantial, careful thought should be given to the differential diagnosis, and if possible, pulsed Doppler echocardiography should be utilized for a definitive statement of aortic valve-induced turbulence.
During precatheterization M mode echocardiographic examination, 33 infants were identified as having findings including right ventricular enlargement and presence of a line within the left atrial portion of the M mode tracing suggesting the possibility of cor triatiatum or total anomalous pulmonary venous return. Pulsed Doppler echocardiography was used to sample blood characteristics on either side of the left atrial line to determine which line was artifactual and which was indicative of an important structure. Five patients whose blood flow characteristics were different on either side of the left atrial line were subsequently proved to have total anomalous pulmonary venous return at cardiac catheterization. In the remaining infants blood flow characteristics were identical on either side of the line, and catheterization excluded total anomalous pulmonary venous return and cor triatriatum. In all patients who had total anomalous pulmonary venous return, drainage involved a persistent left superior vena cava, and this vascular structure was identified by pulsed Doppler examination from the suprasternal notch. Pulsed Doppler echocardiography appears to be a useful technique for resolution of left atrial lines found on M mode echocardiography.
Forty-one infants and children with the combination of patent ductus arteriosus (PDA) and ventricular septal defect (VSD) were encountered over 20 years. Twenty-four presented in infancy with congestive cardiac failure. Pulmonary hypertension was present in 32, the cause in 19 being increased pulmonary blood flow. Increased pulmonary vascular resistance (PVR) was detected in 13 (indicated by a pulmonary to systemic resistance ratio (Rp : RS) greater than 0.24:1 and PVR greater than 4 units). Thus 22% had a pulmonary artery systolic pressure less than 30 mmHg and 68% had a pulmonary vascular resistance below four units, indicating an unusually mild form of the combined condition in these patients. Surgical management is discussed, and in particular the question of simultaneous closure of the defects during infancy. Cardiac failure, resistant to drug treatment, and increased PVR are indications of operation. The PDA should be closed and only if there is no substantial fall in pulmonary artery pressure is the VSD repaired.
Twenty-five patients with proven patent ductus arteriosus were examined by pulsed Doppler echocardiography (PDE) before invasive assessment. Ten patients had normal pulmonary artery pressures, and by PDE, pandiastolic ductal flow. Fifteen patients had elevation of mean pulmonary artery pressure, and by PDE, all had abbreviations of diastolic ductal flow. PDE correctly distinguished between patients with normal pressure and those with evidence of pulmonary hypertension; the ECG did not allow such differentiation. Detection by PDE of pulmonary hypertension complicating patent ductus arteriosus appears to be clinically useful.
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Of 129 patients with either mitral or aortic valve disease angina was present in 55 (42%). It was more frequent in aortic (60%) than in mitral valve disease (33%). The standard 12-lead electrocardiogram was not helpful in distinguishing underlying occlusive coronary artery disease. Coronary arteriography demonstrated coronary artery disease in 26 patients (20%), only 2 of whom had no angina. The incidence of coronary artery disease was almost identical in both the mitral and aortic groups (22% and 17%, respectively), but the percentage of those with demonstrable coronary artery disease accompanying angina was much higher in the mitral group (67% as against 29%). Angina in mitral valve disorders is thus much more likely to be the result of disease of the coronary arteries. Coronary arteriography is mandatory in all patients in both groups who have angina. Otherwise it seems unnecessary as coronary artery disease was found in only 2 patients who did not have angina.
Twelve infants with valvular aortic stenosis manifested symptoms of heart failure during the first 2 months of life. Nine became symptomatic and demonstrated cardiomegaly and pulmonary vascular congestion during the first week of life. The other 3 infants became symptomatic at 3-8 weeks. Echocardiograms revealed normal or enlarged left atrial and ventricular dimensions excluding hypoplastic left heart. Left ventriculography demonstrated segmental dysfunction in 7 infants and generalized hypokinesis in 2 others. Trabecular effacement corresponded with the sites of left ventricular dysfunction. Postoperative studies revealed a decrease in cardiac size and improvement in segmental dysfunction in most infants.
The M-mode echocardiographic findings of ventricular septal defect (VSD) are nonspecific. A specific pulsed Doppler echocardiographic (PDE) diagnosis of VSD can be made by following the turbulent VSD jet through the septum. To assess the sensitivity, specificity and limitations of PDE diagnosis of VSD, 105 children undergoing cardiac catheterization were examined by PDE. These children had a variety of cardiac defects, and a PDE diagnosis of VSD was made in 46/51 (90%) who had VSD proven at catheterization. There was one false positive PDE diagnosis of VSD, for a specificity of 98%. Factors influencing the ability to diagnose VSD by PDE include the location of the defect, level of pulmonary vascular resistance and direction of blood flow through the VSD. The presence of additional defects did not interfere with PDE diagnosis of VSD. The PDE detection of additional defects may identify situations where M-mode echocardiographic estimation of dimensions may not be indicative of the size of VSD shunt.
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Daily weights and mean daily fluid volumes administered to 62 infants with birth weights of less than 2,000 gm, who required respiratory support for respiratory distress syndrome, were reviewed. In 31 infants signs of patent ductus arteriosus developed. In a comparison group of 31 infants, the mean daily fluid volume was 144 ml/kg/24 hours, and the mean body weight was 102% of expected, differing significantly from the 189 nl/kg/24 hours, and 114% of expected weight in those infants who developed PDA. Those infants who developed PDA had not differed significantly from the comparison group in either mean daily fluid volumes or expected weights prior to a period two days before clinical evidence of PDA. Seven infants developed PDA in association with increased fluid administration on more than one occasion during nursery stay. Diuresis after excessive fluid administration was associated with improvement in, or resolution of, signs of PDA in many infants. The results suggest that excessive fluid administration to premature infants with RDS may be one factor associated with the developed of PDA complicating RDS (PDA/RDS).
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109 children who survived surgical treatment for isolated pulmonary valve stenosis were followed for up to 17 years. In all the postoperative status was assessed as satisfactory. Cardiac catheterization repeated in 43 gave a resting valve gradient below 40 mmHg. The 22 children whose pulmonary valves had been excised were as healthy as the 87 who had undergone pulmonary valvotomy. Consideration was given to the desirable length of postoperative review. Except for the few children with symptoms before operation, a postoperative increase in exercise tolerance was not a feature.