1/27. A novel form of familial congenital muscular dystrophy in two adolescents.We report on two brothers (the product of first-degree consanguineous marriage; aged 15 and 12 years) who presented with severe hypotonia at birth, proximal muscle weakness associated with delayed motor milestones but normal cognitive function. Investigations (at 4 years of age) revealed mildly elevated serum creatine kinase (CK) levels (300 and 824 IU/l; N < or = 210). Muscle biopsies showed minimal change myopathy, no neurogenic atrophy but remarkable type-1 fibre predominance (up to 85.5%) without fibre-type disproportion. Clinical examination at 12 and 9 years, respectively, showed mild facial weakness and high-arched palate in both patients. The younger sibling also had ptosis but otherwise normal external ocular muscles. They showed symmetric proximal muscle weakness and wasting associated with calf-muscle hypertrophy. They could walk independently. A repeat muscle biopsy showed advanced dystrophic changes in the younger patient at the age of 10 years. Virtually all the remaining fibres were type 1. immunohistochemistry revealed normal expression of the dystrophin-glycoprotein complex (DGC), including dystrophin, beta-dystroglycan, alpha-(adhalin), beta-, gamma-, and delta-sarcoglycan, laminin-alpha2 chain (merosin) and syntrophin. Mild dystrophic features and type-1 fibre predominance (92.5%) were seen in the biopsy of the older patient, whereas immunohistochemistry showed normal expression of the DGC. Both cases also showed clear expression of integrin alpha7 at the muscle fibre surface and in the blood vessels. Three years later, they could still walk, but with difficulty, and the older brother showed enlargement of the tongue and echocardiographic features of left ventricular dilated cardiomyopathy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/27. Anesthetic management of high-risk cardiac patients undergoing noncardiac surgery under the support of intraaortic balloon pump.patients with severely impaired left ventricular function, an uncorrectable coronary artery disease, and a recent myocardial infarction are at high risk of cardiac complications after major noncardiac surgery. We present two patients with extensive three-vessel coronary artery disease who underwent intraperitoneal surgery under the support of intraaortic balloon pump (IABP). In one patient, the IABP was inserted urgently because of the development of chest pain with significant ST depression on arrival in the operating room, and the other patient was managed with prophylactic IABP. There were no intraoperative or postoperative cardiac events in either patient. Thus, IABP should be considered in the perioperative management of patients with severe cardiac diseases.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/27. Diffuse and severe left ventricular dysfunction induced by epicardial coronary artery spasm.Endothelial dysfunction and effectiveness of treatment of calcium antagonists are suggestive of coronary artery spasm as an underlying disorder in dilated cardiomyopathy (DCM). The aim of this study is to determine whether or not the epicardial coronary artery spasm can induce severe cardiac dysfunction like DCM. Thirty-four consecutive patients with angiographically normal coronary arteries and diffuse left ventricular hypokinesis whose causes had been unknown underwent acetylcholine provocation test and left ventricular biopsy. Eight patients were excluded according to the clinical and laboratory data and biopsy findings suggesting myocarditis or other systemic diseases. According to the results of the acetylcholine provocation test, 17 patients were finally diagnosed as having DCM, and nine patients (35% of the study patients), who had acetylcholine-induced diffuse and multivessel coronary spasm, were diagnosed as having DCM-like vasospastic angina pectoris (VSA). Clinical and cardiac catheterization data including hemodynamics and biopsy findings were similar between the two groups except that left ventricular end-systolic volume was significantly greater in DCM than in DCM-like VSA. After the acetylcholine provocation test, DCM patients received both a beta blocker and an angiotensin-converting enzyme inhibitor, and DCM-like VSA patients received antianginal drugs. In echocardiographic findings at predischarge and those after 6-month drug treatment, both DCM-lke VSA and DCM showed significant reduction in end-diastolic and end-systolic diameters and significant increase in fractional shortening and ejection fraction, whereas changes in ejection fraction and fractional shortening were significantly greater in DCM-like VSA than those in DCM. Epicardial coronary artery spasm can induce diffuse and severe left ventricular dysfunction like DCM in VSA. Although antianginal drugs markedly improve left ventricular function of these patients, only the acetylcholine provocation test can identify DCM-like VSA.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/27. Evidence for early vessel involvement in the dysfunctional myocardium of Takayasu's arteritis.A 15-year-old girl presented with persistent fevers, night sweats, leukocytosis, an elevated erythrocyte sedimentation rate, and a 13-pound weight loss over 2 months. Duplex Doppler scans, computed tomographic scan, and magnetic resonance imaging studies were suggestive of Takayasu's arteritis. Left ventricular dysfunction occurred during the episode of active disease, and an endomyocardial biopsy demonstrated increased HLA-DR (human leukocyte antigen-DR) on the endothelium and evidence of immune complex deposition in the walls of small vessels. One year later, after treatment with corticosteroids and resolution of clinical symptoms, repeat endomyocardial biopsy revealed focal interstitial fibrosis and persistent immune complex deposition. These results indicate that the inflammatory, vasculitic process affecting the large vessels in Takayasu's arteritis may also involve the endomyocardium and its small vessels resulting in ventricular dysfunction.- - - - - - - - - - ranking = 7keywords = vessel (Clic here for more details about this article) |
5/27. Pacing-induced left ventricular dysfunction. Relationship with coronary perfusion.In a patient admitted with symptomatic complete heart block, a DDD pacemaker was implanted. Prior to implantation, echocardiography showed normal left ventricular function. Shortly after implantation, acute congestive heart failure developed with extensive regional hypo- and akinetic segments in the anteroseptal, anterolateral and apical region. Subsequent perfusion imaging with methoxyisobuticeisonitrite (MIBI) at rest demonstrated hypoperfusion in the same regions while coronary angiography showed normal epicardial coronary vessels. Thus, impaired regional coronary flow can be associated with cardiac stimulation, inducing marked deterioration of left ventricular function.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/27. Conversion of atrial fibrillation into a sinus rhythm by coronary angioplasty in a patient with acute myocardial infarction.Atrial tachyarrhythmias are important complications occurring in more than 8% of acute myocardial infarctions (AMI). atrial fibrillation (AFi) during the early phase of AMI is caused by atrial ischaemia, atrial distension due to the left ventricular failure or significant diastolic left ventricular dysfunction. AFi in patients with inferior and posterior AMI indicates at least two vessel coronary diseases, a circumflex coronary artery (CX) occlusion before taking off of the left atrial branches as well as significant stenosis or occlusion of the right coronary artery (RCA). In this article the case of a 67-year-old woman with an acute infero-posterior AMI is described. AMI was complicated with a left heart failure, acute AFi with tachyarrhythmia, transient arterial hypotension and ischaemic mitral regurgitation. Emergency coronary angiography disclosed occlusion of the CX, myocardial infarct related artery, and significant stenoses of the RCA. After opening the occluded CX during the PTCA, AFi with a tachyarrhythmia of 160 beats per minute (bpm) immediately converted into a sinus rhythm with 80 bpm, followed by a normalization of blood pressure and cardiac recompensation. Our case report supports the opinion that AFi in patients with inferior and posterior AMI indicates at least a two-vessel coronary disease. Reopening of the occluded atrial coronary branches during urgent medical treatment was casual and effective treatment of both ischaemic heart disease and consequent AFi.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
7/27. Aortic valve replacement combined with endoventricular circulatory patch plasty (Dor operation) in a patient with aortic valve stenosis and severe ischemic cardiomyopathy.A 58-year-old woman with ischemic cardiomyopathy and aortic valve stenosis, underwent aortic valve replacement and simultaneous endoventricular circulatory patch plasty (Dor operation). She underwent coronary artery bypass grafting for severe triple vessel disease 10 years ago. Recently she started to show severe congestive heart failure. aortic valve stenosis with pressure gradient of 85-mmHg was also found. Coronary bypasses were all patent, but the left ventricle (LV) was severely dilated (LVDd/Ds=71/61 mm) and the ischemic cardiomyopathy was considered as the cause. She successfully underwent aortic valve replacement and endoventricular circulatory patch plasty. The initial postoperative course was complicated with intractable ventricular arrhythmia, but subsequent course was smooth and the patient was discharged with improved symptoms (NYHA Class II). Postoperative catheterization showed decreased left ventricular volume and improved contractility. This case implies the role of LV remodeling procedure in the ischemic cardiomyopathy combined with aortic valve lesion- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/27. Iterative atrial tachycardia originating from the coronary sinus musculature.A case of iterative atrial tachycardia leading to dilated cardiomyopathy is reported. During electrophysiologic study, the tachycardia showed a markedly irregular cycle length associated with changes in atrial activation breakthrough as demonstrated by coronary sinus (CS) recordings and frequently degenerated into self-terminating atrial fibrillation. Left atrial transseptal mapping demonstrated the earliest endocardial atrial activation close to the posterolateral mitral annulus, but this was invariably later than that recorded within the CS, where low-energy radiofrequency applications eliminated the tachycardia. No acute vessel damage was observed at postablation CS angiography. In accordance with previously published experimental data, we hypothesized that the muscular sleeves surrounding the CS might be involved in the genesis of this tachycardia. During 6-month follow-up, the patient remained asymptomatic without tachycardia recurrences and with complete recovery of left ventricular function, confirming the reversible nature of the tachycardia-induced cardiomyopathy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/27. Normal coronary arteries and isolated, regional, left ventricular dysfunction in myotonic dystrophy: a case report.Development of cardiomyopathy in myotonic dystrophy is rare. Regional ventricular abnormality in the presence of normal coronary vessels has not been reported in this condition. We describe a case of isolated, regional, left ventricular dysfunction with corresponding defects on nuclear perfusion imaging in a patient with classical myotonic dystrophy and normal coronary arteries.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/27. Atypical presentation of an anomalous origin of the right coronary artery with severe compression between the great vessels.Anomalous aortic origin of the right coronary artery is a rare coronary anomaly which, in a minority of cases, can cause clinical manifestations such as ischemic chest pain or arrhythmic syncope. We describe a case of anomalous aortic origin of the right coronary artery characterized by signs of left heart failure associated with ventricular tachycardia.- - - - - - - - - - ranking = 4keywords = vessel (Clic here for more details about this article) |
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