Mycotic aneurysm of the innominate artery with supravalvular aortic stenosis

A case of mycotic innominate artery aneurysm in association with supravalvular aortic stenosis has been presented with successful resection as the first such case documented. Blood flow was restored with low cervical autogenous internal jugular vein, as an end-to-side vein graft from the left common carotid and end-to-end to the right subclavian and right common carotid arteries. The future repair of the supravalvular aortic stenosis could then be more easily accomplished.     

Surgical repair for supravalvular aortic stenosis: intermediate to long-term follow-up

The prevalence of different malocclusion features was investigated in 396 6-year-old Icelandic children, using the epidemiologic registration method described by Bj?rk et al. in 1964. Girls were ahead of boys with regard to dental stage (P < 0.01). One or more permanent teeth were congenitally missing in 5% of the children. Postnormal occlusion was found in 27% of the boys and in 31% of the girls, and prenormal occlusion was found in 6% and 5%, respectively. Straight terminal plane at the second deciduous molars was found in individuals with either normal or postnormal occlusion. Thus, it can be misleading to use the relation of the terminal planes as a measurement of the sagittal relation between the jaws. The prevalence of hypodontia was much lower than has been reported previously for Icelandic children.     

Magnetic resonance features of supravalvular aortic stenosis in William''s syndrome. Report of 2 cases

A wandering spleen and splenic cysts are uncommon conditions in children. A combination of both entities has only rarely been reported in the literature. Another case of this complex pathology in 12-year-old girl is presented. She was initially referred for evaluation of a large abdominal mass. Diagnostic studies raised the suspicion of a hematoma in an abnormally located spleen, but the definitive diagnosis was established at laparotomy. Successful surgical management consisted of total cystectomy and splenopexy.     

Familial supravalvular aortic stenosis. Report of a large family and review of the literature

Familial supravalvular aortic stenosis has been recognized as a distinct syndrome. A large family with five proven cases and a review of the literature on familial supravalvular aortic stenosis are presented. The diagnosis was substantiated in all 63 cases by cardiac catheterization, surgery, or postmortem examination.     

Concurrence of supravalvular aortic stenosis and peripheral pulmonary stenosis in three generations of a family: a form of arterial dysplasia

Isolated supravalvular aortic stenosis (SVAS) commonly is an autosomal dominant trait; it may also occur in the Williams syndrome (WS). While peripheral pulmonary stenosis (PPS) can occur in the same individual with familial isolated SVAS, concurrence of these lesions in different relatives of a family is uncommon. We describe five affected individuals in one family; three had isolated SVAS, one had isolated PPS, and one had SVAS and PPS. Based on this family and review of literature, we suggest that SVAS is a form of arterial dysplasia encompassing PPS in its spectrum. It is developmentally distinct from other left heart obstructive lesions that are hypothesized to be related to blood flow abnormalities in the developing embryo. We also conclude that the clinical disorder in this family represents one that is distinct from WS.     

Echocardiographic hemodynamic and morphometric predictors of survival after two-ventricle repair in infants with critical aortic stenosis

OBJECTIVES: The purpose of this study was to identify echocardiographic hemodynamic and morphometric factors that would predict which infants with critical aortic stenosis could undergo relief of left ventricular outflow obstruction as opposed to the Norwood procedure. BACKGROUND: Echocardiographic predictors of survival in infants with critical aortic stenosis after two-ventricle repair have been mainly limited to morphometric factors, which have limitations. Echocardiographic hemodynamic predictors of survival in these patients have not previously been studied. METHODS: Doppler color flow mapping and pulsed Doppler techniques were used to obtain hemodynamic measurements of flow in the ascending, transverse and descending aorta, the ductus arteriosus, and across the aortic and mitral valves in infants with critical aortic stenosis. Morphometric measurements of the left heart structures were obtained, and comparisons were made between survivors and nonsurvivors for the hemodynamic and morphometric factors. RESULTS: Twenty-eight infants (mean age 1 +/- .6 days, mean weight 3.6 +/- .6 kg) with critical aortic stenosis were evaluated. Nineteen had a two-ventricle repair initially attempted, and nine had a Norwood operation. Among the patients with a two-ventricle repair, the hemodynamic factors associated with survival after two-ventricle repair included predominant or total antegrade flow in the ascending (p < 0.01) and transverse aorta (p < 0.05). Aortic valve gradient, mitral valve inflow and direction of flow in the ductus arteriosus and descending aorta were unrelated to outcome. The morphometric factors associated with survival after two-ventricle repair included the indexed aortic annulus (p < 0.0002), aortic root (p < 0.003), ascending aorta (p < 0.008) and left ventricular long-axis length (p < 0.01). Left ventricular volume, mass, ejection fraction and mitral valve area were not related to outcome after two-ventricle repair. CONCLUSIONS: In infants with critical aortic stenosis, predominant or total antegrade flow in the ascending and transverse aorta was associated with survival after two-ventricle repair. Determination of a one- versus two-ventricle repair remains a complex issue in infants with critical aortic stenosis. In addition to established morphometric predictors, hemodynamic information on the direction of flow in the aorta may help to define candidates for the Norwood operation.     

Fluorescent in situ hybridisation (FISH) for hemizygous deletion at the elastin locus in patients with isolated supravalvular aortic stenosis

We describe two cases of left-side pulmonary vein obstruction observed after the arterial switch operation (Jatene) for D-transposition of the great arteries. This appears to be related to left-sided pulmonary vein obstruction occurring coincidently with D-transposition of the great arteries, rather than a consequence of arterial switch operation.     

Supravalvular aortic stenosis in aortic dissection

Supravalvular aortic stenosis is a rare complication of aortic dissection. We report on echocardiographic and magnetic resonance observations in 2 cases of aortic dissection with false lumen thrombosis of the ascending aorta and severe narrowing of the true lumen.     

Echocardiographic oscillating flap in aortic root dissecting aneurysm

A patient had a history, physical findings, and chest x-ray film suggesting type I aortic dissection. This diagnosis could not be confirmed angiographically. Echocardiographic studies predicted both the presence of dissection and the anatomic findings at surgery. A regularly oscillating echo corresponding to the intimal flap was found in the false lumen. This is suggested as a new echocardiographic finding in dissecting aneurysm.     

Peripartum cardiomyopathy complicating severe aortic stenosis

A 30-year-old woman presented with cardiac failure caused by severe aortic stenosis and biopsy proven myocarditis 4 weeks after an uneventful pregnancy. The course of the myocarditis was followed and predicted by serial endomyocardial biopsies. Our experience suggests that peripartum cardiomyopathy should never be ignored as a cause of cardiac disease in the puerperium even when there is another, more obvious, pathology and we report the efficacy of serial transvenous cardiac biopsies in timing aortic valve replacement in this patient.     

Diagnosis and treatment of aortic valve stenosis

The present review has attempted to summarize the classic symptoms and signs of aortic valve stenosis, especially in an adult. It is emphasized that all the classic signs rarely are present and their absence may mislead an unwary clinician. The diagnostic help provided by noninvasive tests, including echocardiography and phonocardiography, has been emphasized. A need for cardiac catheterization and angiography in most patients prior to corrective surgery is stressed. The natural history of the disease without operative intervention is dim and a significant risk of sudden death exists. The current surgical approach with immediate and long-term results is summarized. Finally, attention has been drawn to the special clinical circumstances when the aortic valve stenosis provides a strinkingly different clinical picture. We cannot find a better way to end this review than by quoting a warning note given by Thomas Lewis in 1920: "It is the faint cry of an anguished and fast failing muscle, which, when it comes, all should strain to hear, for it is not long repeated. A few months, a few years at most, and the end comes."     

Valve replacement in congenital aortic stenosis]

The internal organs of 155 cats originating from 3 different territories in the eastern part of Brandenburg state were examined for parasites between 1993 and 1995. Parasites were found in 85% of cats. Parasites with the highest prevalence were Toxocara mystax (45%), Hydatigera taeniaeformis (22%), Ancylostoma tubaeforme (17%) and Opisthorchis felineus (16%). Metorchis bilis, Mesocestoides spp., Taenia pisiformis, Capillaria plica, Capillaria sp., Ollulanus tricuspis, Aelurostrongylus abstrusus, Cystoisospora spp. and Sarcocystis spp. were diagnosed with a lower frequency. The occurrence of H. taeniaeforme, A. tubaeformis und O. felineus was depending on the origin of the cats.     

Familial bilateral blepharoptosis and subvalvular aortic stenosis

PURPOSE: To select ideal radiologic projections for mapping and ablation of tachycardias of right ventricular outflow tract (RVOT). METHODS: Ten hearts from human corpses were studied utilizing radiopaque material to identify the pulmonary valve and three distinct sites on this valve: septal anterior (A), septal posterior (P) and free-wall (L). Next, the hearts were filmed in the frontal plane and in oblique projections with 15 degrees increments to the right and to the left. The projections in which the sites were lateralized on the valve, eased radiologic interpretation and were considered ideal for mapping and ablation. Depending on the proximity of the sites to the lateral extremes of the pulmonary valve, the projections were considered ideal ( ), intermediary (++) and inadequate (+). RESULTS: Projections [table: see text] CONCLUSION: The A site of RVOT was best indicated in the 60 and 45 degrees left anterior oblique projections; the 0 degree postero anterior projection was best for mapping the P site; the L region was best explored in the 60 degrees right anterior oblique projection.     

Echocardiographic diagnosis of the dissection of the thoracic aorta

Nine patients with aortic root dilatation without dissection and four patients with a dissecting aneurysm of the ascending aorta were studied with single-plane echocardiography. Previously reported echocardiographic findings, which have been shown to be similar in these two groups of patients, are confirmed. In addition to (a) aortic root dilatation, (b) thickening of the anterior and/or the posterior aortic wall, and (c) the multiple parallel echoes within the widened aortic walls, which are all considered to be not specific signs of dissection, the authors outline the presence of some specific echocardiographic patterns in patients with a dissecting hematoma of the ascending aorta. These include: 1) the loss of contiguity between the inner borders of the anterior aortic wall and the interventricular septum when the dissection is confined to the anterior wall of the aorta; 2) the loss of contiguity between the inner and/or outer borders of the posterior aortic wall and the mitral anulus if the hematoma extends to the posterior wall of the aorta.