1/99. Myopathy in very-long-chain acyl-coa dehydrogenase deficiency: clinical and biochemical differences with the fatal cardiac phenotype.A 30-year-old man suffered since the age of 13 years from exercise induced episodes of intense generalised muscle pain, weakness and myoglobinuria. fasting ketogenesis was low, while blood glucose remained normal. Muscle mitochondria failed to oxidise palmitoylcarnitine. Palmitoyl-CoA dehydrogenase was deficient in muscle and fibroblasts, consistent with deficiency of very-long-chain acyl-coa dehydrogenase (VLCAD). The gene of this enzyme had a homozygous deletion of three base pairs in exon 9, skipping lysine residue 238. fibroblasts oxidised myristate, palmitate and oleate at a rate of 129, 62 and 38% of controls. In contrast to patients with cardiac VLCAD deficiency, our patient had no lipid storage, a normal heart function, a higher rate of oleate oxidation in fibroblasts and normal free carnitine in plasma and fibroblasts. 31P-nuclear magnetic resonance spectroscopy of muscle showed a normal oxidative phosphorylation as assessed by phosphocreatine recovery, but a significant increase in pH and in Pi/ATP ratio.- - - - - - - - - - ranking = 1keywords = pain (Clic here for more details about this article) |
2/99. Intraprocedural myocardial contrast echocardiography as a routine procedure in percutaneous transluminal septal myocardial ablation: detection of threatening myocardial necrosis distant from the septal target area.Percutaneous transluminal septal myocardial ablation (PTSMA) has been introduced as an alternative to surgery for symptomatic hypertrophic obstructive cardiomyopathy (HOCM). Visualization of the ablation area prior to induction of the chemical necrosis is possible by intraprocedural myocardial contrast echocardiography (MCE). We report on two patients in whom MCE showed opacification of the medial papillary muscle or the left ventricular posterolateral free wall. In both patients the correct ablation area could be identified by MCE after a change of the target vessel, thus avoiding potentially fatal complications due to induction of a necrosis of myocardium distant from the septal target area.- - - - - - - - - - ranking = 3.7653522504204keywords = area (Clic here for more details about this article) |
3/99. Hypertrophic cardiomyopathy with mid-ventricular obstruction and splenic infarction associated with paroxysmal atrial fibrillation: a case report.A 54-year-old woman had been treated for hypertrophic cardiomyopathy and paroxysmal atrial fibrillation since 1992. She was admitted with paroxysmal atrial fibrillation which was resolved by medical treatment. However, on the next day, left lateral chest pain appeared. Computed tomography disclosed a low density area in the spleen. She received anticoagulant therapy under a diagnosis of splenic infarction, and the pain disappeared. echocardiography showed hypertrophic cardiomyopathy with mid-ventricular obstruction. She was treated with cibenzoline to prevent paroxysmal atrial fibrillation attack and attenuate the hemodynamic load. After treatment, the pressure gradient decreased from 41 to 7 mmHg. This patient with hypertrophic cardiomyopathy suffered a rare isolated splenic infarction associated with paroxysmal atrial fibrillation.- - - - - - - - - - ranking = 434.27976345433keywords = chest pain, chest, pain, area (Clic here for more details about this article) |
4/99. Fatal myocardial embolus after myectomy.Coronary embolism is an infrequent phenomenon. A 56-year-old man with hypertrophic obstructive cardiomyopathy and severe mitral regurgitation who underwent left ventricular septal myectomy and mitral valve annular repair is presented. The patient had a cardiac arrest 36 h after surgery. Cardiac standstill, tamponade and a left ventricular rupture were noted when the chest was opened during attempted resuscitation. autopsy revealed an occlusive embolus of myocardium in the proximal left anterior descending coronary artery. It showed pathological features of hypertrophic cardiomyopathy. There was an extensive acute transmural anteroseptal left ventricular myocardial infarction with rupture of the anterior free wall. Embolism of myocardium - to the coronary arteries, the systemic circulation or the pulmonary circulation - is a rare event, with only nine other cases reported in the literature in the past 30 years. This is the first reported case of myocardial embolus to a coronary artery in a patient with hypertrophic obstructive cardiomyopathy following septal myectomy.- - - - - - - - - - ranking = 10.403619247304keywords = chest (Clic here for more details about this article) |
5/99. An unusual case of ST elevation in a 39-year-old man.A 39-year-old man presented to a university hospital emergency department with anginal chest pain, ventricular tachycardia and ST elevation in the anterolateral leads (V3 to V6, I and aVL). Due to discrepancies in the history and physical examination, thrombolysis was withheld until a past electrocardiogram could be obtained, which was unchanged. Subsequent investigations revealed no evidence of myocardial necrosis, and the patient was diagnosed with hypertrophic cardiomyopathy. This is the first reported case of hypertrophic cardiomyopathy with ST elevation as the predominant electrocardiographic abnormality. In patients with discrepancies in the clinical presentation, it is essential to obtain past elecrocardiograms to ensure appropriate utility of thrombolysis.- - - - - - - - - - ranking = 432.74185598998keywords = chest pain, chest, pain (Clic here for more details about this article) |
6/99. Familial hypertrophic cardiomyopathy associated with prolongation of the QT interval.In a 61 year old female patient who suffered from atypical chest pain we diagnosed long qt syndrome by QTc duration of 467 ms, macroscopic T wave alternans and notched T waves in three leads and hypertrophic cardiomyopathy with asymmetric thickening of basal parts of the septum (2.0 cm) without relevant outflow tract obstruction by echocardiography. coronary angiography could exclude coronary artery disease. In a systematic family screening two sons of the patient could also be diagnosed as having long qt syndrome with QTc durations of 472 and 496 ms and asymmetric septal thickening (1.8 and 2.1 cm, respectively). One of these two sons suffered from pre-syncope, the other was asymptomatic despite maximum sports activity. In the third son, LQTS and hypertrophic cardiomyopathy could be excluded.- - - - - - - - - - ranking = 432.74185598998keywords = chest pain, chest, pain (Clic here for more details about this article) |
7/99. Acardiac twin pregnancy with neonatal resolution of donor twin cardiomyopathy.BACKGROUND: Twin reverse arterial perfusion, as with acardiac fetuses, is a rare complication of multifetal pregnancy. prognosis of the donor twin is guarded because of the high risk of cardiac failure, polyhydramnios, and preterm delivery. CASE: A 40-year-old woman, gravida 4, para 3, was diagnosed at 19 weeks' gestation with a twin pregnancy complicated by an acardiac, acephalic fetus. Serial amniocenteses were done to decompress the hydramnios between 27 and 33 weeks. ultrasonography showed ventricular septal hypertrophy in the donor twin at 27 weeks, and cesarean delivery was done at 33 weeks. The neonatal course was complicated by hypertrophic cardiomyopathy, which resolved without sequelae by 1 year of age. CONCLUSION: Antenatal sonographic monitoring of interventricular septal thickness can be a useful marker to predict neonatal cardiac sequelae.- - - - - - - - - - ranking = 0.53790746434577keywords = area (Clic here for more details about this article) |
8/99. Anteroapical stunning and left ventricular outflow tract obstruction.Dynamic left ventricular outflow tract (LVOT) obstruction is typically observed in the setting of hypertrophic cardiomyopathy. It has also been reported with concentric LV hypertrophy, excessive sympathetic stimulation, and acute myocardial infarction. We describe 3 patients with chest discomfort after emotional stress, who had pronounced abnormalities on electrocardiograms, insignificant obstructive coronary disease and hemodynamic instability with LVOT obstruction, and regional wall motion abnormalities. Suppression of contractility with beta-blockers resulted in resolution of the gradient and in clinical improvement. On follow-up, functional recovery was excellent, and ventricular function had normalized. The conditions and mechanisms that may produce this sequence of events are discussed. The most probable scenario is that an acute ischemic insult secondary to vasospasm, LV stunning, and acute geometric remodeling produced a substrate for LVOT obstruction that was exacerbated by basal LV hypercontractility. The importance of this observation is that routine treatment of cardiogenic shock cannot be used and that conservative management results in excellent prognosis.- - - - - - - - - - ranking = 10.403619247304keywords = chest (Clic here for more details about this article) |
9/99. Myectomy for hypertrophic obstructive cardiomyopathy after failed alcohol septal ablation: clinicopathological correlations.Hypertrophic cardiomyopathy - a genetically transmitted cardiac disorder - has diverse clinical, pathological and molecular manifestations. echocardiography is the most reliable tool for clinical diagnosis of hypertrophic cardiomyopathy. Reduction of the intraventricular pressure gradient and improvement of symptoms are major objectives of all therapeutic strategies. The recently introduced concept of catheter-based interventional treatment involves selective coronary perforator branch injection of 96% ethanol to reduce septal thickness, left ventricular outflow obstruction, left ventricular filling pressure and symptoms. The long term morphological features after medical ablation are presented for the first time and compared with both the echocardiographic findings and the findings reported in the English-language literature. The findings show that injection of ethanol into the perforator branch is associated with a fairly localized area of myocardial scarring.- - - - - - - - - - ranking = 0.53790746434577keywords = area (Clic here for more details about this article) |
10/99. Pulmonary hypertension secondary to left-sided heart disease: a cause for ventilation-perfusion mismatch mimicking pulmonary embolism.ventilation-perfusion (V/Q) scans are commonly performed in patients with suspected pulmonary thromboembolism (PE). V/Q mismatch is typically attributed to PE. We describe a case in which a V/Q scan performed on a patient with advanced hypertrophic obstructive cardiomyopathy showed large areas of V/Q mismatch not due to PE. The mismatch was due to pulmonary hypertension secondary to left-sided heart disease. The pathophysiology is briefly reviewed.- - - - - - - - - - ranking = 0.53790746434577keywords = area (Clic here for more details about this article) |
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