1/22. A strange complex of diffuse congenital cardiovascular disease and cardiomyopathy, with localised myocardial calcification.This unique case is of a man, followed clinically since infancy, who had a ventricular septal defect which closed spontaneously, a small arterial duct, and a minor degree of aortic coarctation, all without obvious symptoms. He later developed progressive cardiac failure which was attributed to some obscure form of congenital cardiomyopathy. He died at the age of 45 years. Necropsy showed a grossly abnormal arrangement of ventricular myocardial fascicles and bands, with absence of the papillary muscles causing tricuspid and mitral regurgitation. The various malformations are considered to be a gross example of a diffuse congenital cardiovascular disease complex, to the best of our knowledge previously undescribed.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/22. pseudoxanthoma elasticum and calcinosis cutis.A 42-year-old white woman presented with clinical and histologic manifestations of both calcinosis cutis and pseudoxanthoma elasticum: discrete milia-like calcifications at the anterior aspect of the neck, a funduscopic examination with classic eye findings, peripheral vascular disease, and a mottled appearance of the skin at the axillae, groin, and lateral aspects of the neck. A younger sibling had similar skin lesions and deteriorating visual acuity. The patient was normocalcemic and normophosphatemic. This case may represent the coincidental occurrence of two rare entities in the same person or may be suggestive of a pattern of dystrophic calcification associated with pseudoxanthoma elasticum.- - - - - - - - - - ranking = 0.2keywords = vascular disease (Clic here for more details about this article) |
3/22. Detection of cardiac calcinosis in hemodialysis patients by whole-body scintigraphy with 99m-technetium methylene diphosphonate.A noninvasive method for the diagnosis of cardiac calcinosis, a life-threatening complication in hemodialysis patients with end-stage renal disease (ESRD), has not, as yet, been firmly established. We tested whether whole body scanning with 99m-technetium methylene diphosphonate (MDP) might visualize cardiac calcinosis. In 19 consecutive chronic hemodialysis ESRD patients (13 males and 6 females, aged 40-81, mean 63 /- 8 years) with cardiovascular disease [mitral annular calcinosis and/or calcified aortic valve (n = 4), hemodialysis cardiomyopathy (n = 1), coronary artery disease (n = 9) and peripheral artery atherosclerotic disease (n = 6)], MDP uptake in the heart was compared to that in 7 non-ESRD controls with hyperparathyroidism due to adenoma. Cardiac and lung field MDP uptake was confirmed in only 3 (16%) and 5 (26%) of the 19 ESRD subjects, respectively, but was absent in controls. Positive cardiac uptake was related to cardiac calcified complications (mobile intracardiac calcinosis, myocardial calcinosis and mitral annular calcification) and the duration of hemodialysis (p = 0.015). While it was statistically insignificant, subjects showing MDP uptake were elder and had higher serum Ca or Ca x P product and lower intact parathyroid hormone levels. These results suggest that cardiac calcinosis in ESRD patients can be detected noninvasively by myocardial scintigraphy with 99m-technetium MDP.- - - - - - - - - - ranking = 0.2keywords = vascular disease (Clic here for more details about this article) |
4/22. Dramatic worsening of vascular calcifications after kidney transplantation in spite of early parathyroidectomy.vascular calcification is a common feature in chronic dialysis patients, but their clinical significance is debated and the role of kidney transplantation (TP) in the natural history of their development has received scanty attention. We will describe a case of dramatic worsening of vascular calcifications during TP in a young patient in spite of early and successful parathyroidectomy (PTX), and will discuss other causes which might be putatively linked to vascular damage during the time of TP. A 37-year-old man on regular dialytic treatment (RDT) for 11 years, received his first cadaveric transplantation in January 1993. He underwent PTX 6 months after TP because of the lack of decreasing in parathyroid hormone values despite normal graft function. Although PTX was effective, a dramatic worsening was evident in large as well as in medium and small-sized arteries during the following three years of TP. In February 1997, few months after starting dialysis again because of the recurrence of his primary membranoproliferative glomerulonephritis (MPGN), the patient experienced myocardial infarction followed by aorto-coronary bypass (right coronary artery and anterior descending coronary artery) and leg "claudicatio". Though a role for parathyroid hormone in vascular disease has been commonly accepted, the case here reported clearly shows that blunting parathyroid gland activity may be unable to avoid the worsening of a process of vascular disease during the time of TP. Many other factors--linked to the time of TP--may be involved in vascular diseases, such as nephrotic syndrome, dyslipidemia, hypertension and drugs. In the case of our patient, a clear cut risk factor for his progressive atherosclerosis can be designated hyperlipidema and other disturbancies secondary to a nephrotic syndrome due to relapse of MPGN, together with persistent hypertension. This is the first case report in the English literature which clearly demonstrates that TP may add fuel to the fire of vascular disease also in young people and even in the absence of parathyroid hyperactivity, perhaps on the basis of a favorable genetic background. Furthermore, the history of our patient demonstrates that vascular calcifcation heralds major cardiovascular diseases.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
5/22. Idiopathic calcinosis cutis of the vulva in an elderly woman. A case report.BACKGROUND: Idiopathic calcinosis cutis of the vulva is a rare condition of unknown etiology. Only seven cases have been reported to date, and all of them were in children. We report the first case in an elderly woman. CASE: A 68-year-old woman presented with a labial lesion of unknown etiology. Excisional biopsy was performed, and histopathologic evaluation showed subepidermal calcification. Follow-up biochemical and hormonal analysis and screening tests for collagen vascular diseases revealed normal results. CONCLUSION: After diagnosis of calcinosis cutis, a laboratory workup to rule out abnormalities of calcium and phosphorus metabolism, malignant processes and collagen vascular diseases must be carried out. This approach in the evaluation of calcinosis cutis could lead to diagnosis of the underlying disease at an early stage.- - - - - - - - - - ranking = 0.4keywords = vascular disease (Clic here for more details about this article) |
6/22. Cartilaginous metaplasia in calcified diabetic peripheral vascular disease: morphologic evidence of enchondral ossification.The mechanism of arterial calcification is not clear. We examined histological sections of major arteries from lower extremities of two patients with longstanding type II (or non-insulin-dependent) diabetes mellitus, and found morphological evidence of cartilaginous metaplasia and ectopic ossification with associated severe medial arterial calcification and atherosclerosis. hematoxylin and eosin, alcian blue, and toluidine blue stains were applied for the demonstration of cartilage cells and their specific matrix proteins, and immunohistochemical studies for type II collagen. To our knowledge, cartilaginous metaplasia has not previously been described in medium-sized human muscular arteries. This observation supports the hypothesis that active enchondral ossification may be a pathway leading to arterial calcification in diabetic obstructive peripheral vascular disease.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
7/22. Cardiovascular calcifications in pediatric patients receiving maintenance dialysis.Cardiovascular disease is a major cause of morbidity and mortality in adult patients with end-stage renal disease receiving maintenance dialysis. Coronary artery calcifications (CAC) contribute to the high prevalence of cardiac disease and are associated with hyperphosphatemia, an elevated calcium-phosphorus product (CaxP), and prolonged time on dialysis. Chronic inflammation and malnutrition are also associated with an increased risk for development of cardiac calcifications. Young adults receiving maintenance dialysis develop cardiac calcifications at a degree out of proportion to healthy adults of the same age and gender. Many of these young adults initiated dialysis as children or teenagers. risk factors associated with the development of CAC are also seen in the pediatric dialysis population. To date, reports of cardiac calcifications in pediatric patients receiving maintenance dialysis are limited to post-mortem studies. We present two pediatric patients with ANCA-positive vasculitis diagnosed with cardiac calcifications while receiving maintenance dialysis. hyperphosphatemia and an elevated CaxP product were seen in both patients and probably contributed to the development of extraskeletal calcifications. In addition, both patients had an underlying systemic inflammatory disease and significant weight loss/malnutrition that may have contributed to the early and rapid onset of cardiac calcifications.- - - - - - - - - - ranking = 0.2keywords = vascular disease (Clic here for more details about this article) |
8/22. Off-pump coronary artery revascularization: ideal indication for patients with porcelain aorta and calcification of great vessels.patients with porcelain aorta and severe calcification of the great vessels are a challenging dilemma for the cardiovascular surgeon regarding bypass technique, choice of conduit, and selection of proximal anastomotic sites due to the high incidence of devastating thromboembolization and aortic injury. No currently proposed surgical approach avoids manipulation of the heavily calcified ascending aorta. Three patients presented with unstable angina and decreased ventricular function secondary to significant left main coronary artery stenosis and 3-vessel coronary artery disease. In addition to the coronary artery disease, severely calcified ascending aorta and great vessels were discovered. One patient presented with near total distal abdominal aortic occlusion, severe peripheral vascular disease, history of stroke, and carotid endarterectomy. Surgical coronary revascularization was indicated. coronary artery bypass grafting using internal thoracic artery and greater saphenous vein composite arterial inflow grafts in combination with off-pump beating heart surgery was successfully used. cardiopulmonary bypass and clamping of the aorta was avoided. No new neurologic deficit was observed. Coronary revascularization with internal thoracic artery composite grafts and avoiding cardiopulmonary bypass and clamping the calcified aorta is an effective method to prevent clamp injury and thromboembolization. Off-pump coronary artery bypass grafting seems to be an ideal indication in patients with porcelain aorta because the surgical techniques of "no-touch" and "no-cannulation" can be applied.- - - - - - - - - - ranking = 0.2keywords = vascular disease (Clic here for more details about this article) |
9/22. inflammation and coronary calcification in renal patients--factors that may explain increased cardiovascular risk, and poorer results of coronary interventions?Cardiovascular disease (CVD) is one of the major causes of mortality in patients with renal diseases, with increased odds ratio of mortality with risk factors as diverse as cholesterol, vascular stiffness, chronic inflammation and hyperhomocysteinemia. Several factors have been incriminated to explain the increase in coronary vascular calcification (CVC) in this particular population. Increased duration of dialysis, dyslipidemia, altered calcium-phosphorus metabolism, and chronic inflammation have all been associated with increased CVC. We present here four case reports illustrating the differences in the pathophysiology, therapy and prognosis of calcific coronary heart disease seen in uremic patients.- - - - - - - - - - ranking = 0.2keywords = vascular disease (Clic here for more details about this article) |
10/22. Hutchinson-Gilford progeria syndrome with severe calcific aortic valve stenosis and calcific mitral valve.The case of a 12-year-old girl with clinical features of progeria with severe calcific valvar aortic stenosis is presented. The mitral valve showed the presence of calcium, and peripheral vascular disease was also present, though there was no family history of this. Aortic valve replacement was deferred because of insufficient data relating to this condition. The genetics and phenotypic mechanisms of the disease are reviewed. In view of the association of progeria with valve disease, all patients should undergo electrocardiography and echocardiography as part of their routine work-up.- - - - - - - - - - ranking = 0.2keywords = vascular disease (Clic here for more details about this article) |
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