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.     

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 diagnosis of supravalvular aortic stenosis

The echocardiographic features of supravalvular aortic stenosis seen in a patient with valvular aortic and multiple pulmonary branch stenosis are described. Since patterns of valvular, discrete subvalvular, and supravalvular aortic stenosis can be recognized by echocardiography, it is now possible to screen close relatives of patients having supravalvular aortic stenosis and pulmonary branch stenosis by this non-invasive technic.     

Single-stage repair of aortic arch obstruction and associated intracardiac defects with pulmonary homograft patch aortoplasty

PURPOSE: To investigate the involvement of the cornea during endotoxin-induced uveitis (EIU) in the rat and the effect of Ngamma-nitro-L-arginine methyl ester (L-NAME) as nitric oxide synthase (NOS) inhibitor, administered by iontophoresis. METHODS: EIU was induced in Lewis rats that were killed at 8 and 16 hours after lipopolysaccharide (LPS) injection. The severity of uveitis was evaluated clinically at 16 hours, and nitrite levels were evaluated in the aqueous humor at 8 hours. Corneal thickness was measured, 16 hours after LPS injection, on histologic sections using an image analyzer. Transmission electron microscopy (TEM) was used for fine analysis of the cornea. Transcorneoscleral iontophoresis of L-NAME (100 mM) was performed either at LPS injection or at 1 and 2 hours after LPS injection. RESULTS: At 16 hours after LPS injection, mean corneal thickness was 153.7+/-5.58 microm in the group of rats injected with LPS (n=8) compared with 126.89+/-11.11 microm in the saline-injected rats (n=8) (P < 0.01). TEM showed stromal edema and signs of damage in the endothelial and epithelial layers. In the group of rats treated by three successive iontophoreses of L-NAME (n=8), corneal thickness was 125.24+/-10.36 microm compared with 146.76+/-7.52 microm in the group of rats treated with iontophoresis of saline (n=8), (P=0.015). TEM observation showed a reduction of stromal edema and a normal endothelium. Nitrite levels in the aqueous humor were significantly reduced at 8 hours by L-NAME treatment (P=0.03). No effect on corneal edema was observed after a single iontophoresis of L-NAME at LPS injection (P=0.19). Iontophoresis of saline by itself induced no change in corneal thickness nor in TEM structure analysis compared with normal rats. CONCLUSIONS: Corneal edema is observed during EIU. This edema is significantly reduced by three successive iontophoreses of L-NAME, which partially inhibited the inflammation. A role of nitric oxide in the corneal endothelium functions may explain the antiedematous effect of L-NAME.     

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."     

Should a bicuspid aortic valve be replaced in the presence of subvalvar or supravalvar aortic stenosis?

BACKGROUND: A bicuspid aortic valve is commonly associated with other levels of left ventricular outflow tract obstruction. Providing the bicuspid aortic valve is competent and nonobstructive, repair of subvalvar or supravalvar stenosis usually focuses on the obstructive lesions, leaving the valve in situ. The aim of this report was to examine the impact of a bicuspid aortic valve on the risk of reoperation for patients undergoing operation for subvalvar or supravalvar aortic stenosis. METHODS: Since 1976, 47 patients with supravalvar or subvalvar aortic stenosis have undergone repair. The median follow-up is 5.1 years (range, 2 months to 20.1 years). Sixteen patients (34%) had a bicuspid aortic valve that was competent and nonobstructive, and 31 (66%) had a tricuspid aortic valve. RESULTS: Reoperation was required in 9 patients (56%) with a bicuspid aortic valve, in each involving aortic valve replacement with an autograft (3), homograft (2), or prosthesis (4). Six patients (19%) with a tricuspid aortic valve required reoperation, yet only 1 required aortic valve replacement. The freedom from valve replacement was 43% (70% confidence interval, 31% to 55%) in the bicuspid aortic valve group versus 100% (70% confidence interval, 94% to 99.5%) in the tricuspid group at 5 years (p = 0.0001). The freedom from any reoperation at 5 years was 43% (70% confidence interval, 31% to 55%) in patients with a bicuspid aortic valve versus 86% (70% confidence interval, 80% to 93%) in the tricuspid group (p = 0.02). CONCLUSIONS: The data suggest that patients with subvalvar or supravalvar aortic stenosis and a bicuspid valve may be better palliated with a more definitive operation such as the Ross or Ross-Konno procedure.     

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.     

Surgical commissurotomy of the aortic valve: outcome of open valvotomy in neonates with critical aortic stenosis

The prevalence of the soil-transmitted helminthiases is extremely high in some rural areas in China. The endemic foci of these human parasites constitute one of the most neglected public health problems. A large scale survey on human parasites (1988-1992) was conducted in China. The prevalences of the soil-transmitted helminthiases in the 30 provinces, autonomous regions and municipalities were reported and correlated with ecological factors (Yu et al, 1994; Xu et al, 1995). In this paper, we re-examined and analyzed the reported results with a spatial pattern test statistic, the D statistic. The values of the D statistic indicated that the spatial correlation of the prevalence of the soil-transmitted helminthiases in China was significant. We also found that the spatial correlation of the prevalence could largely be explained by the paddy field area per capita. The correlation of the prevalence of the soil-transmitted helminthiases and the paddy field area per capita was higher than the correlation of the prevalence with the factors found previously by Xu et al (1995).     

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.     

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.     

Midterm results of balloon dilation of congenital aortic stenosis: predictors of success

OBJECTIVES: We evaluated patient and procedural characteristics that influence the midterm success of balloon dilation of congenital aortic stenosis. BACKGROUND: Balloon dilation is a new treatment for congenital aortic stenosis. Factors that influence midterm success are unknown. METHODS: We performed a retrospective review of 148 children >1 month old who underwent balloon dilation for aortic stenosis. RESULTS: Balloon dilation was successful in 87% of patients, with a procedural mortality rate of 0.7%. The average immediate peak to peak gradient reduction was 56.4 +/- 19.9% (mean +/- SD). Prior valvotomy was the only factor that significantly reduced the immediate gradient reduction after dilation (47.1 +/- 21.8% vs. 57.8 +/- 19.6%, p < 0.01). Survival after dilation was 95% at 8 years. Seventy-five percent of patients were free of repeat intervention 4 years after dilation, whereas 50% remained free of repeat intervention at 8 years. Asymmetrically thick valve leaflets (risk ratio [RR] 0.17, p < 0.01) and prior aortic valvotomy (RR 0.35, p = 0.02) decreased the risk of repeat intervention. Aortic regurgitation grade > or = 3 (RR 4.27, p = 0.04) and residual gradient after dilation (RR 1.63 for 10 mm Hg, p < 0.01) increased the risk. CONCLUSIONS: The 8-year survival rate after dilation was 95% with 50% of patients free of repeat intervention. Factors that increased the risk for repeat intervention included symmetrically thin or thick aortic valve leaflets, regurgitation grade > or = 3 after dilation and a high residual gradient after dilation. The incidence of repeat intervention after dilation was high owing to its palliative nature.     

Three-year outcome after balloon aortic valvuloplasty. Insights into prognosis of valvular aortic stenosis

BACKGROUND: To identify predictors of long-term outcome after balloon aortic valvuloplasty, we analyzed data on 674 adults (mean age, 78 +/- 9 years; 56% were women) undergoing this procedure at 24 clinical centers who had a mean initial increase in aortic valve area of 0.3 cm2. METHODS AND RESULTS: Baseline data included clinical, echocardiographic, and catheterization variables. Follow-up data included mortality, cause of death, rehospitalization, 6-month echocardiography, and functional status. Kaplan-Meier curves and log-rank tests were used to evaluate survival in subgroups. Multivariate Cox regression models were used to identify independent predictors of survival. Overall survival was 55% at 1 year, 35% at 2 years, and 23% at 3 years, with the majority of deaths (70%) classified as cardiac by an independent review committee. Rehospitalization was common (64%), although 61% of survivors at 2 years reported improved symptoms. Echocardiography at 6 months (n = 115) showed restenosis from the postprocedural valve area of 0.78 +/- 0.31 cm2 to 0.65 +/- 0.25 cm2 (P < .0001). With stepwise multivariate analysis, sequentially adding clinical, echocardiographic, and catheterization variables, the overall model identified independent predictors of survival as baseline functional status, baseline cardiac output, renal function, cachexia, female gender, left ventricular systolic function, and mitral regurgitation. Baseline and postprocedural variables were examined to identify which subgroup of patients has the best outcome after aortic valvuloplasty. A "lower-risk" subgroup (28% of the study population), defined by normal left ventricular systolic function and mild clinical functional limitation, had a 3-year survival of 36% compared with 17% in the remainder of the study group. CONCLUSIONS: Long-term survival after balloon aortic valvuloplasty is poor with 1- and 3-year survival rates of 55% and 23%, respectively. Although survivors report fewer symptoms, early restenosis and recurrent hospitalization are common.     

Consequences of intramyocardial arterial lesions in aortic valvular stenosis

Proliferative lesions, which included collagen deposition, developed with age in intramyocardial arteries of 27 patients with aortic stenosis and matched controls. Those with the most extensive intramyocardial artery lesions developed massive subendocardial infarcts during surgery. Using histologic quantitation, the percent of intramyocardial arteries with lesions in a patient was correlated with decreases in the amount of muscle in arterioles between the subepicardial and subendocardial zones of the left ventricle. The mean decrease in arteriolar muscle was 43% in patients with aortic stenosis and 19% in controls. Blood pressures correlate with the amount of muscle in arterioles, so subendocardial perfusing pressures were presumably low in those with aortic stenosis. Patients with the greatest decrease in arteriolar muscle across the myocardium had the most impaired left ventricular function, i.e., highest end diastolic pressures, lowest ejection fractions, and lowest mean fiber shortening rates.