Progressive pulmonary arterial stenosis.
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Biomedical subjects
Publications and source records attributed to G M Folger.
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A detailed clinical analysis of 16 patients with various forms of congenital cardiac malformations with the potential for large left-to-right shunts who developed extreme pulmonary hypertension in infancy and early childhood has been carried out. The study plan was to include only patients with sufficiently long follow up to allow analysis of their course in both the prepubescent and postpubescent periods. In early life these patients were all characterized by lack of evidence for significant development of the expected left-to-right shunt indicating inappropriate maturation of their pulmonary vascular circulation. Throughout all phases of their life, they were significantly polycythemic in comparison with a control group but this polycythemia, representative of peripheral systemic hypoxemia, increased markedly with the acquisition of puberty in all patients. These two findings indicate both an early abnormality, suggestively primary, of the pulmonary vasculature and a late progression of the disease associated with pubertal maturation.
Three newborn infants were observed to have murmurs restricted to diastole. None was considered ill, and none has exhibited symptoms or findings of cardiopulmonary disease throughout follow-up. Localization of the diastolic timing and absence of a significant systolic component of the murmur were confirmed by phonocardiography. The transient nature of the finding was established by serial phonocardiography. Essentially normal cardiac anatomy was determined by routine chest radiograms, electrocardiograms, and echocardiography in both M and two-dimensional modes. Suggestive evidence for left-to-right shunting was present in each case. Invasive studies were not performed. An anatomic cause for the diastolic murmur was not discovered, suggesting it could be related to the ductus arteriosus or mild pulmonary insufficiency.
The M-mode echocardiogram is considered a satisfactory diagnostic tool for the determination of the presence of a bicuspid aortic valve. M-mode echocardiographic failure to determine this condition is occasionally encountered and has been suggested to be due to the anatomic configuration wherein the two aortic valve leaflets are nearly equal in size. By employing a specific angiographic study (orifice-view aortography), it has been shown that accurate identification of aortic valve anatomy is possible. Ten patients having orifice-view aortographic findings of an aortic valve having two essentially equally sized leaflets have been studied both by this technique and M-mode echocardiography. Five of these had M-mode echographic evidence of a bicuspid aortic valve. However, 3 exhibited no criteria diagnostic of a bicuspid valve and 2 had only suggestive criteria. The results confirm that M-mode echocardiography alone is insufficient to determine reproducibly the presence of an aortic valvular malformation with or without stenosis when the anatomy is that of an equally bicuspid aortic valve.
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A child with a bronchogenic cyst is described who manifested clinical findings indicative of pulmonary arterial stenosis. Compression of the right pulmonary artery by the cyst with a significant pressure gradient between the proximal and distal segments of the right pulmonary artery was verified at surgery. The post-operative follow-up findings have confirmed relief of the stenosis with absence of the murmur originating from it.
Orifice-view aortography is a contrast cineaortographic technique that allows en face viewing of the aortic valve. In this projection the anatomic configuration of the aortic valve is identified, and it is possible to planimeterize the visualized aortic valve opening and accurately determine the orifice area. Fifteen individuals with congenitally deformed aortic valves with gradients ranging from trivial (< 10 mm Hg) to surgically significant (6E 60 mm Hg) were subjected to aortic orifice area measurement by orifice-view aortography, and the area was compared with the peak systolic gradient and valve area. The results yielded a cloe correlation with the aortic valve area index as measured hydraulically or, as in three instances, directly from the surgically excised specimen (r = 0.94). A satisfactory correlation was also shown with the valve area index and the peak aortic valve gradient (r = 0.85). These observations indicate that orifice-view aortography is an accurate method for the measurement of the aortic valve orifice and may be particularly useful when hydraulic measurements are unattainable or invalid.
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Infants with the hypoplastic left heart syndrome present a reproducible appearance that terminates in irreversible shock during the neonatal period. There is disagreement over the exact cause of this appearance. This case involves a newborn infant with tricuspid atresia and transposition of the great arteries, and with the clinical appearance and course usually reserved for neonates with the hypoplastic left heart syndrome. Accurate diagnosis is crucial in all such infants to correctly distinguish those with a hopeless outcome from those amenable to palliative cardiovascular surgery. This case supports the contention that ductal closure alone is not the principal cause of death in infants with hypoplastic left heart syndrome.
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M-mode echocardiographic features are presented in four patients with membranous ventricular septal defect. The most consistent echo findings in all the patients were the presence of fuzzy, fluttering echoes of the tricuspid valve during systole. More careful echocardiographic evaluation of the tricuspid valve in ventricular septal defect is therefore recommended.
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The purpose of this investigation is to demonstrate the potential diagnostic value of intracardiac sound recordings in patients with subaortic stenosis. Intracardiac pressure and sound were measured in 10 patients with various types of subaortic obstructions using a catheter-tip micromanometer. Seven patients had idiopathic hypertrophic subaortic stenosis (IHSS), 2 had a subvalvular membrane, and 1 had a subvalvular tunnel. Within the left ventricular cavity, at the site of maximal systolic left ventricular pressure, either there was no systolic murmur, or the murmur was of low intensity. However, within the outflow tract of the left ventricle, distal to the site of intraventricular obstruction, a prominent systolic murmur was detected in all patients. This murmur was of higher intensity than the one measured distal to the aortic valve. In one patient, in whom no subvalvular obstruction was present, but in whom entrapment of the tip of the catheter occurred, no murmur was detected in the left ventricle even though a subvalvular pressure gradient was observed. Therefore it appears that a systolic murmur recorded with maximal intensity in the outflow tract of the left ventricle may be of substantial help in distinguishing between an artifactual intraventricular pressure gradient, and one that results from intraventricular obstruction.
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The identification of absence of continuity of the inferior vena cava (IVC) with the right atrium assumes major importance at the time of right heart catheterization. The appearance of the IVC in the lateral chest radiogram is diagnostically different from the normal when discontinuity is present and 2 radiographic patterns have been recognized: 1) total absence of the IVC shadow; 2) straightened and posteriorly directed IVC shadow contrasting sharply with the anteriorly directed normally continuous IVC. This latter sign is considered reproducibly accurate in the identification of the IVC which fails to have continuity with the right atrium.