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PubMed2004

Micro-venous valves in the superficial veins of the human lower limb.

It is commonly believed that venous valves are not present in veins smaller than 2 mm in diameter. Venous valves, however, have been identified recently in small veins in several regions of the body. This study was undertaken to determine the size distribution of venous valves in the human lower limb micro-venous circulation. Vascular casts were made from six adult lower limbs and the sampled areas were viewed by scanning electron microscopy. In total, 2,376 valves were identified from 410 cm(3) of subcutaneous tissue. The vast majority (94%) of the valves were in veins less than 300 microm in luminal diameter, with 65% of the valves present in venous channels less than 100 microm in luminal diameter. The smallest valves identified were present in venous channels 18 microm in diameter. All valves were bicuspid and often associated with a tributary. Endothelial cells on the vein wall not associated with a valve were fusiform and arranged parallel to the long axis of the vessel, however, the endothelial cells on the luminal and valve sinus surfaces of the cusp were more polyhedral in shape and showed no obvious pattern of alignment. This study provides direct evidence to show that small superficial veins of the human lower limb do contain abundant venous valves and, for the first time, shows that the majority of these valves are present within venous channels less than 100 microm in luminal diameter.

Aged↗

PubMed1982

Origin of both coronary arteries from the same sinus of Valsalva: report of two cases.

Origin of both coronary arteries from the same sinus of Valsalva was found in two patients. In one patient the right coronary artery opened from the left sinus of Valsalva, coursed between the pulmonary artery and aorta, disclosed a 95% obstruction in its proximal third with good runoff. The portion of the artery between the aorta and pulmonary artery showed cyclic narrowing during systole. In the second patient the left coronary opened from the right sinus of Valsalva, the left main trunk crossed anteriorily the pulmonary artery conus, and both the circumflex and the left anterior descending branches showed significant narrowing. In both patients the arteries shared a common sinus with two separate orifices, both had bicuspid aortic valve, and in both, nonaberrant coronary vessels also had stigmata of atherosclerosis.

Aged↗

PubMed1985

Pulmonary valve morphology after balloon dilatation of pulmonary valve stenosis.

Percutaneous balloon valvuloplasty (BV) for pulmonic valve stenosis (PS) is increasingly becoming a nonsurgical alternative in patient management. However, the mechanism by which BV dilates the obstructive lesion has not been firmly established. We have had the opportunity to examine the effects of BV in the setting of PS and present two illustrative cases documenting the morphology after BV of the stenotic bicuspid and tricuspid pulmonary valve.

Adolescent↗

PubMed1989

Fatal aortic rupture: an unusual complication of percutaneous balloon valvuloplasty for acquired valvular aortic stenosis.

A case report complicated by a perforation of the ascending aorta during percutaneous balloon valvuloplasty for acquired aortic stenosis in a bicuspid aortic valve is presented. The clinical and pathological findings are discussed. It is suggested that in patients with hard calcified valve cusps and calcifications of the aortic annulus balloon sizes exceeding the aortic diameter should be avoided.

Aged↗

PubMed1995

Effectiveness of balloon valvuloplasty in the young adult with congenital aortic stenosis.

The objective of this study was to assess the effectiveness of balloon valvuloplasty in the young adult with congenital aortic stenosis and to compare its effectiveness with children. Percutaneous balloon valvuloplasty is effective in children with congenital aortic stenosis, but not in adults with acquired calcific aortic stenosis. Because effectiveness of balloon valvuloplasty in young adults with congenital aortic stenosis is not well defined, we evaluated the outcome in 15 patients aged 16-24 years (18 +/- 0.6; mean +/- SEM) who underwent balloon valvuloplasty from 1985 to 1993. The aortic valve annulus diameter ranged from 18.5 to 30 mm (24 +/- 0.9). The aortic valve was bicuspid in 12 and tricuspid in 3 patients, and calcification was present in one patient. Balloon valvuloplasty was performed using a double balloon technique in 12 patients and a single balloon technique in three patients. Three patients had inadequate relief of gradient with a residual peak systolic gradient > or = 70 mm Hg. Three patients required valve replacement-two patients for a residual gradient > or = 70 mg Hg, and one patient 4 years later for severe aortic valve regurgitation. Eight of the remaining 12 have undergone elective follow-up catheterization 1.2-2.5 years (1.5 +/- 0.1) later. The peak systolic aortic valve gradient decreased by 55% from 73 +/- 5.8 mm Hg to 35 +/- 5.4 mm Hg immediately postvalvuloplasty, and was 30 +/- 4.4 mm Hg at follow-up (P < 0.001). The left ventricular systolic pressure decreased from 179 +/- 7.5 to 147 +/- 6.5 mm Hg immediately postvalvuloplasty and was 147 +/- 4 mm Hg at follow-up. Aortic insufficiency was unchanged after valvuloplasty in 9, increased by 1+ in 4, and by 2+ in 2 patients. Balloon valvuloplasty was as effective in these young adults as in 70 children (age 6 +/- 0.7 years) with congenital aortic stenosis (peak systolic gradient pre- 79 +/- 3 mm Hg versus post- 34 +/- 2 mg Hg; at 1-2 years follow-up 34 +/- 4 mm Hg). Balloon valvuloplasty provides effective treatment in most young adults with congenital aortic stenosis, without early restenosis. Balloon valvuloplasty is as effective in young adults as in children, where it is currently the treatment of choice.

Adolescent↗

PubMed1983

Isolated tricuspid stenosis: report of a case submitted to valvular commissurotomy.

In this report, a rare case of tricuspid stenosis uncomplicated by other valve lesions is presented, with clinical, hemodynamic, echocardiographic, and angiographic studies. The patient was markedly incapacitated, mostly as a result of a restricted cardiac output. Tricuspid commissurotomy was performed, with a stenotic deformity of a bicuspid atrioventricular valve, probably of congenital origin, found at surgery. Considerable improvement was observed, notwithstanding the persistence of some signs of residual tricuspid obstruction as a result of an incomplete commissurotomy, to avoid increasing the slight degree of preexistent valvular regurgitation.

Cardiac Catheterization↗

PubMed1984

Aortic dilatation resulting in chronic aortic regurgitation and complicated by aortic dissection in a patient with Turner's syndrome.

A patient with XO Turner's syndrome with a 12-year history of progressive aortic root dilatation resulting in chronic aortic regurgitation is presented. Her case is unique in that it occurred in the absence of coarctation of the aorta, bicuspid aortic valve, or hypertension. Idiopathic dilatation of the aorta may be an additional risk factor to the development of aortic dissection in the setting of Turner's syndrome.

Adult↗

PubMed1984

Pseudocoarctation of the aortic arch in a patient with Turner's syndrome.

Pseudocoarctation of the aortic arch is a rare congenital anomaly which resembles true coarctation by the presence of a narrowing in the descending thoracic aorta immediately distal to the origin of the left subclavian artery. However, it is differentiated from that entity by the absence of a significant hemodynamic obstruction and by the presence of aneurysmal dilatation proximal and distal to the area of narrowing. As a result, the commonly observed clinical findings of coarctation such as a prominent collateral circulation and decreased blood pressure in the lower extremities are not observed. "Kinking" or "buckling" are common adjectives used to describe the roentgenographic appearance of the aortic arch in this condition. The association of various congenital cardiac abnormalities with pseudocoarctation has been reported, and are similar to those commonly seen with true coarctation. We now present the first reported case of pseudocoarctation associated with a probable bicuspid aortic valve in a patient with Turner's syndrome.

Aortic Coarctation↗

PubMed1985

The "straight back" syndrome: current perspective more often associated with valvular heart disease than pseudoheart disease: a prospective clinical, electrocardiographic, roentgenographic, and echocardiographic study of 50 patients.

Fifty consecutive patients (36 male, 14 female, mean age 28 years) who had heart murmurs and clinical and radiographic evidence of straight upper dorsal spine (straight back syndrome, SBS) underwent detailed clinical, electrocardiographic, roentgenographic, and echocardiographic evaluation. Palpable systolic thrill noted in one (2%) and widened S2 with persistent splitting in 2 (4%) patients were uncommon. Murmurs were invariably systolic in nature. Those located at the base of the heart in 19 (38%) patients were ejection in type and best heard during expiration. Those located at the apex in 26 (52%) patients were either mid-, late-, or pansystolic, and often associated with midsystolic click. Five (10%) patients had both types of murmurs. Diastolic murmurs were not heard in any patient. EKGs were normal in the majority. Cardiomegaly (C:T greater than 55%) was present in only 5 (10%) and dilatation of the main pulmonary artery in 2 (4%) patients. Thus the incidence of pseudoheart disease (PsHD) was small (14%). Echocardiograms were normal in 18 (36%) and abnormal in 32 (64%) patients. There was evidence of mitral valve prolapse (MVP) in 29 (58%) patients and 3 (6%) had evidence of bicuspid aortic valve (BAV). In a control group of 40 age- and sex-matched patients (26 male, 14 female, mean age 29.5 years), who also had heart murmurs but lacked straight upper dorsal spine, only 7 (17.5%) had MVP and none had BAV. The difference is both clinically and statistically significant (p less than 0.001). It is concluded that SBS is more often associated with valvular heart disease (MVP and BAV) than PsHD. Therefore, the diagnosis of SBS should remain presumptive until echocardiography has been performed to exclude MVP and BAV. SBS patients who have valvular heart disease should receive infective endocarditis prophylaxis.

Adolescent↗

PubMed1992

Cardiac pathology in 2007 consecutive forensic autopsies.

The incidence of various types of cardiovascular disease was evaluated in 2007 consecutive forensic patients. Cardiovascular deaths accounted for 22.8% of the study patients and atherosclerotic coronary heart disease was the most common type of cardiac disease (18%). Among subjects dying of atherosclerotic coronary disease, sudden death was three times more frequent than acute myocardial infarction. Expected cardiac findings included the incidence of severe coronary atherosclerosis (21%), floppy mitral valves (5%), and congenital bicuspid aortic valves (1%). Major cardiac findings occurred in 32% and minor cardiac findings were found in 40%. Only 17% of hearts were anatomically normal. An unexpected cardiac necropsy finding included the high frequency of myocardial bridges (23%). Unexpected cardiac findings included the low incidence of acute myocarditis (0.6%) and common finding of tunneled epicardial coronary arteries ("myocardial bridges") (23%).

Adult↗

PubMed1995

Pathology of pulmonic valve stenosis and pure regurgitation.

Little morphologic information is available on operatively excised pulmonic valves. The causes of pulmonic stenosis are limited to a few conditions: (1) rheumatic and (2) nonrheumatic (congenital, carcinoid, infective endocarditis). Congenital causes of pulmonic stenosis constitute well over 95% of these conditions. Congenital types of pulmonic stenosis include acommissural dome-shaped, dysplastic, and bicuspid. Rare acquired causes of pulmonic stenosis include carcinoid, rheumatic, and infective endocarditis. Of the acquired causes of pulmonic stenosis, carcinoid is the most common condition. In contrast, causes of pure pulmonic regurgitation are multiple. Two major categories of pure pulmonic regurgitation include (1) conditions associated with anatomically abnormal valve cusps (congenital, rheumatic, carcinoid, trauma, and infective endocarditis) and (2) conditions associated with anatomically normal cusps (elevated pulmonary artery systolic pressures, idiopathic dilated pulmonary trunk, and Marfan's syndrome).

Humans↗

PubMed2001

Association between calcific aortic stenosis and hypercholesterolemia: is there a need for a randomized controlled trial of cholesterol-lowering therapy?

BACKGROUND: Calcific aortic stenosis may have common etiological factors with atherosclerosis. HYPOTHESIS: In this retrospective, case-control study, we aimed to determine whether there is an association between hypercholesterolemia and calcific aortic valve stenosis. METHODS: Consecutive patients undergoing single aortic or mitral valve replacement in a regional cardiothoracic surgical center were reviewed and preoperative patient characteristics were recorded: demographics, comorbidity (including coronary artery disease and associated risk factors), serum total cholesterol, lipid-lowering therapy, and serum creatinine. RESULTS: Serum total cholesterol concentrations were significantly higher in patients with calcific aortic stenosis than in controls (6.2+/-1.1 vs. 5.3+/-1.1 mmol/l; p < 0.001). The significant difference in serum cholesterol concentrations remained following correction for gender and body mass index (p = 0.02) and when patients with coronary artery disease were excluded (6.3+/-1.1 vs. 5.3+/-1.4 mmol/l; p<0.001). Subgroup analysis demonstrated that the association between elevated serum cholesterol concentrations and calcific aortic stenosis was particularly strong in patients with tricuspid aortic valves (6.4+/-1.2 vs. 5.3+/-1.1 mmol/l; p < 0.001) compared with those with bicuspid valves (5.9+/-1.1 vs. 5.3+/-1.1 mmol/l; p = 0.06). CONCLUSIONS: We conclude that hypercholesterolemia is associated with calcific aortic stenosis and may be implicated in its pathogenesis and progression. We believe that there is now a need for a randomized, controlled trial of cholesterol-lowering therapy in patients with calcific aortic stenosis.

Aged↗

PubMedDate not supplied

Well-functioning double-orifice mitral valve in a young adult.

We report the case of a rare congenital anomaly, a double-orifice mitral valve, in a 23-year-old woman who was asymptomatic and had no history of heart disease. Transthoracic and multiplane transesophageal echocardiography revealed 2 functionally normal orifices of equal size, the least frequent anatomic presentation of this anomaly. We prescribed antibiotic prophylaxis because of the concomitant presence of a mildly stenotic bicuspid aortic valve and recommended annual follow-up examinations to monitor both lesions for possible progression.

Adult↗

PubMed2001

Histochemical analysis of lymphatic endothelial cells in lymphostasis.

The ultrastructure of endothelial cells of intestinal lymphatics and the thoracic duct (TD) and the relation to lymphostasis were examined in rats and monkeys. Localization of 5'-nucleotidase (5'-Nase) and endothelial nitric oxide synthase (eNOS) was studied. In normal lymphatic endothelial cells, 5'-Nase reaction product was evenly deposited on the cell surface in vivo and on cultured TD endothelial cells (TDECs), whereas eNOS was evenly distributed throughout the nucleus and cytoplasm. TDECs had a long filamentous process extending towards the subendothelial extracellular matrix but became flat and regular within 30-40 minutes after gastric perfusion with olive oil. According to their electron-density, two types of cells were found in the TD endothelial layer. The cells with low electron-density exhibited stronger 5'-Nase activity. Valves were bicuspid formations and the valvular endothelial surface of the convex side showed weaker 5'-Nase activity than the concave side. During TD blockage-induced lymphostasis in rats, the 5'-Nase product was almost not discernible in the TDECs within 2 weeks. Larger vesicles were found in the endothelial cytoplasm of the ligated TD. Their number decreased after 6-12 weeks. The small intestinal lymphatics in the mucosa and submucosa were dilated, with numerous open intercellular junctions. The endothelial lining appeared to have reduced activities for 5'-Nase and eNOS in 9 of 11 experimental animals. The results indicated that the inability of the open intercellular junctions, normally working as one-way endothelial flap valves, may be a key morphological feature after TD blockage. Reduced eNOS and 5'-Nase may functionally influence contractile activity and transport capability of the lymphatic vessels in the lymphostasis.

5'-Nucleotidase↗

PubMed1990

Microvasculature of the nasal salt gland of the duckling, Anas platyrhynchos: quantitative responses to osmotic adaptation and deadaptation studied with vascular corrosion casting.

The three-dimensional microvasculature of the nasal salt gland of the duckling was studied by vascular corrosion casting and scanning electron microscopy. Changes in the vascular volume of the gland in response to osmotic stress were also determined using cast weights and densities. The richly vascularized gland is supplied on its medial surface by large branches of the supraorbital and ethmoidal arteries. Numerous arterial branches enter the gland and distribute to lobes via the interlobar connective tissue. Lobar arterioles penetrate to the periductal areas of the lobes before dividing into capillaries supplying the ductal epithelium and secretory tubules. Capillaries envelope the secretory tubules and run radially from the ducts toward the lobe periphery, so that blood flows counter to the tubular secretion. Blood is collected via venous plexuses seen as distinct drainage units on the periphery of each lobe. Veins exhibit large numbers of bicuspid valves. Following 1 day and 4 days of osmotic loading (feeding 1% NaCl), vascular volume of the gland increased fivefold and ninefold, respectively, a response that precedes and exceeds that of the gland weight or Na,K-ATPase activity. When salt water-adapted ducklings were fed fresh water for only 24 hr (deadaptation), vascular volume fell to 2.8 times the control level. Changes in blood flow to the gland during osmotic adaptation and deadaptation are rapid and dramatic and may represent the initial steps in the control of gland secretion.

Adaptation, Physiological↗

PubMed1983

Structural teratogenicity evaluation of methyl chloride in rats and mice after inhalation exposure.

One hundred bred Fischer-344 female rats were exposed daily for 6 hours to atmospheres containing 0, 100, 500, or 1,500 ppm methyl chloride, 25 females per exposure concentration, from gestation day (gd) 7 through gd 19. On gd 20, the females were sacrificed for evaluation of maternal reproductive and fetal parameters. Maternal and fetal toxicity was apparent at the highest exposure concentration. There were no methyl chloride-induced external, skeletal, or visceral abnormalities seen in the fetuses. One hundred thirty-two C57BL/6 female mice bred to C3H males to produce B6C3F1 offspring were exposed daily for 6 hours to atmospheres containing 0, 100, 500, or 1,500 ppm methyl chloride, 33 females per exposure concentration, from gd 6 through gd 17. Exposure to the entire 1,500-ppm group was terminated on gd 10-14, with the animals killed in extremis. Selective necrosis of neurons in the internal granular layer of the cerebellum, ranging from individual cell involvement to focal areas comprising large numbers of neurons, was found in all females. On gd 18, the females from the other treatment groups, all of which survived, were killed for evaluation of maternal reproductive and fetal parameters. No evidence was seen of maternal or fetal toxicity in these exposure groups. There were no significant alterations in external appearance in fetuses from any of the exposure groups. Visceral examination of mouse fetuses revealed a small, but statistically significant, incidence of heart defects in litters of the 500-ppm group. The anomaly, a reduction or absence of the atrioventricular valve, chordae tendineae, and papillary muscle, was observed on the left side (bicuspid valve) in three fetuses and the right side (tricuspid valve) in six fetuses: three males and six females. It is concluded that methyl chloride inhalation exposure in pregnant rats, during critical periods of embryo and fetal development, is not teratogenic at concentrations which elicit maternal and fetal toxicity. In pregnant mice, methyl chloride was severely toxic to dams following 4 days or more of exposure to 1,500 ppm in air. Methyl chloride, at 500, but not 100 ppm, was teratogenic in mice, leading to a malformation in the heart. No embryo-fetal toxicity or teratogenicity was associated with exposure of mice, during critical periods of embryo and fetal development, to 100 ppm of ethyl chloride.

Air↗

PubMed2003

Turner's syndrome in fetal life.

OBJECTIVE: To compare the incidence and type of heart disease found in association with 45X karyotype in fetal life with postnatal life and to examine the outcome after fetal diagnosis. METHODS: Fifty-three fetuses with a 45X karyotype were examined echocardiographically over a 4-year period between 1999 and 2002. Of these, 47 were referred because of increased nuchal translucency (NT). RESULTS: A cardiac abnormality was detected in 33/53 (62.2%) fetuses. The most common diagnosis was coarctation of the aorta in 24/53 (45.3%) fetuses, followed by the hypoplastic left heart syndrome (HLHS) in 7/53 (13.2%) fetuses. The mean NT was significantly higher in fetuses with a heart defect than in those with normal echocardiography. Termination of pregnancy was carried out in 45/53 (84.9%) fetuses and intrauterine death occurred in six cases. Two of four fetuses with a mosaic karyotype are currently alive. CONCLUSION: Turner's syndrome is associated with a higher incidence of heart defects detected prenatally when compared to postnatal reports. The commonest associated heart defects detected prenatally are HLHS and coarctation of the aorta, in contrast to postnatal life where a bicuspid aortic valve is the most common diagnosis. The typical intrauterine presentation of Turner's syndrome with a markedly increased NT or with hydrops and with a typical 45X karyotype has an extremely poor prognosis for intrauterine survival.

Aortic Coarctation↗

PubMed2004

Prenatal diagnosis of Shone's syndrome: parental counseling and clinical outcome.

OBJECTIVE: To describe a series of fetuses diagnosed as having Shone's syndrome, which includes four cardiac defects and for which there is a wide variety of clinical presentations, surgical treatments and outcomes, and to discuss the counseling strategy. METHODS: We reviewed retrospectively the records of four babies who were suspected prenatally to have Shone's syndrome. RESULTS: The mean age at diagnosis was 27.5 (range, 22-35) weeks. A small left ventricle, mitral and aortic annulus and ascending aorta were detected in three cases. In three fetuses there was an abnormal mitral valve apparatus and in two fetuses a sub-aortic membrane was detected. Coarctation of the aorta was an impending diagnosis in three babies. Following counseling, all parents decided to continue the pregnancy to term. Echocardiographic evaluation was performed soon after birth. The mean mitral valve annulus diameter was 8.2 (range, 7-10) mm, and that of the aortic valve annulus was 6 (range, 5-7) mm. The aortic valve was bicuspid in all babies with an additional sub-aortic membrane in two babies. Doppler ultrasound examination revealed high-velocity flow through the mitral valve in three babies; two of them had a parachute mitral valve. Coarctation repair was performed in two babies during the first week of life. One patient underwent mitral valvuloplasty followed by later mitral valve replacement. All patients were alive after a mean of 7.8 (range, 3.3-10.5) years' follow-up. CONCLUSION: When counseling families regarding fetal Shone's syndrome, it is important to emphasize the wide variety of clinical presentations and possible outcomes. Differentiation between hypoplastic left ventricle and Shone's complex is crucial and may influence the physician's attitude, the presentation of the case to the family and the family's final decision.

Counseling↗