1/60. 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 = 1keywords = chest pain, chest (Clic here for more details about this article) |
2/60. 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 = 0.033276359581999keywords = chest (Clic here for more details about this article) |
3/60. 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 = 1keywords = chest pain, chest (Clic here for more details about this article) |
4/60. 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 = 1keywords = chest pain, chest (Clic here for more details about this article) |
5/60. 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 = 0.033276359581999keywords = chest (Clic here for more details about this article) |
6/60. Apical hypertrophic cardiomyopathy: bedside diagnosis by intravenous contrast echocardiography.Apical cardiomyopathy is rare in the West. The characteristic appearance on left ventriculography has been used to confirm the diagnosis of this condition; transthoracic echocardiography can also be useful in this regard. However, apical artifacts may obscure the typical appearance during echocardiography, and although the advent of tissue harmonic echocardiography has resulted in improved image quality, the technique still may be inadequate in the establishment of a diagnosis. We hypothesized that contrast echocardiography, which improves endocardial border delineation, may be the technique of choice for the diagnosis of apical hypertrophic cardiomyopathy. We report the case of a 40-year-old woman with down syndrome who had chest pain. The electrocardiogram showed T-wave changes in the lateral precordial leads, but cardiac enzymes were normal. Tissue harmonic echocardiography showed apical akinesia. Intravenous contrast echocardiography, however, revealed typical features of hypertrophic apical cardiomyopathy. Thus contrast echocardiography may be used to establish the diagnosis of this condition.- - - - - - - - - - ranking = 1keywords = chest pain, chest (Clic here for more details about this article) |
7/60. A malignant phenotype of hypertrophic cardiomyopathy caused by Arg719Gln cardiac beta-myosin heavy-chain mutation in a Chinese family.Mutations of the cardiac beta-myosin heavy-chain (beta-MHC) gene cause hypertrophic cardiomyopathy (HCM). Recent genotype-phenotype correlation studies have shown that mutations carry prognostic significance. We studied five unrelated Chinese families with hypertrophic cardiomyopathy. exons 3-27 and 40 of the beta-MHC gene were screened with both the polymerase chain reaction-single-strand conformation polymorphism (PCR-SSCP) method and the cycle sequencing of the PCR products. A previously reported heterozygous mutation Arg719Gln (arginine-->glutamine in codon 719) in exon 19 was found in one family. The proband is a 30-year-old female diagnosed at age of 25 years when she presented with symptoms of chest pain, palpitations, and frequent incidents of dizziness and syncope. A two-dimensional echocardiogram showed moderate asymmetrical septal hypertrophy with left atrial enlargement. There was no obstruction of the left ventricular outflow tract (LVOT). The patient also developed atrial fibrillation. The proband's mother and one of her sisters had similar clinical manifestations and both died suddenly at the age of 38 years. In addition, two silent nucleotide substitutions (ACT63ACC, TTT244TTC) in the cardiac beta-MHC gene were identified in the other four families. These synonymous mutations did not cosegregate with the disease in the families and they were also present in the 60 healthy and age-matched control subjects. Of the five families studied, we did not find any missense mutation in the remaining four families. The missense mutation Arg719Gln found in the Chinese family is associated with a malignant phenotype of severe clinical symptoms and poor survival prognosis. This mutation also causes atrial enlargement and atrial fibrillation. Our study provides further evidence that the mutation, which alters the charge of the myosin heavy chain, is associated with a serious clinical outcome.- - - - - - - - - - ranking = 1keywords = chest pain, chest (Clic here for more details about this article) |
8/60. Successful surgical resection of a muscular bridge in a patient with nonobstructive hypertrophic cardiomyopathy--a case report.The authors report a case of myocardial bridging associated with nonobstructive hypertrophic cardiomyopathy and severe intractable chest pain that was relieved by surgical resection of the muscular bridge. Surgical resection of a myocardial bridge may be considered in patients with refractory symptoms that can clearly be attributed to muscular bridge.- - - - - - - - - - ranking = 1keywords = chest pain, chest (Clic here for more details about this article) |
9/60. Fractional flow reserve in a patient with intermediate coronary stenosis and hypertrophic cardiomyopathy.We discuss the case of a 61-year-old male patient with hypertrophic cardiomyopathy and chest pain on exertion. coronary angiography and intravascular ultrasound revealed an intermediate stenosis in the proximal site of the left anterior descending artery, while Tc-99m myocardial scintigraphy revealed exercise-induced myocardial ischemia in the anteroseptal wall and apical portion. Flow velocity-derived coronary flow reserve (CFR) and pressure-derived fractional flow reserve (FFRmyo) were both low (1.1 and 0.59), suggesting that the stenosis was functionally significant. Directional coronary atherectomy greatly improved the FFRmyo (0.99), the scintigraphic findings, and anginal pain but did not improve the CFR (1.2). FFRmyo was useful in assessing the functional significance of an equivocal coronary stenosis and its interventional resolution.- - - - - - - - - - ranking = 1keywords = chest pain, chest (Clic here for more details about this article) |
10/60. prevalence and age-dependence of malignant mutations in the beta-myosin heavy chain and troponin t genes in hypertrophic cardiomyopathy: a comprehensive outpatient perspective.OBJECTIVES: The goal of this study was to determine the prevalence of "malignant" mutations in hypertrophic cardiomyopathy (HCM). BACKGROUND: Previous genotype-phenotype studies have implicated four mutations (R403Q, R453C, G716R and R719W) as highly malignant defects in the beta-myosin heavy chain (MYH7). In the cardiac troponin t gene (TNNT2), a specific mutation (R92W) has been associated with high risk of sudden death. Routine clinical screening for these malignant mutations has been suggested to identify high-risk individuals. methods: We screened 293 unrelated individuals with HCM seen at the Mayo Clinic in Rochester, minnesota, between April 1997 and October 2000. Deoxyribonucleic acid (dna) was obtained after informed consent; amplification of MYH7 exons 13 (R403Q), 14 (R453C) and 19 (G716R and R719W), and TNNT2 exon 9 (R92W) was performed by polymerase chain reaction. The mutations were detected using denaturing high-performance liquid chromatography and automated dna sequencing. RESULTS: The mean age at diagnosis was 42 years with 53 patients diagnosed before age 25. The mean maximal left ventricular wall thickness was 21 mm. Nearly one-third of cases were familial and one-fourth had a family history of sudden cardiac death. Only 3 of the 293 patients possessed one of the five "malignant" mutations, and all 3 patients were <25 years of age at presentation (p < 0.006). CONCLUSIONS: This finding underscores the profound genetic heterogeneity in HCM. Only 1% of unrelated individuals seen at a tertiary referral center for HCM possessed one of the five "malignant" mutations that were examined. Routine clinical testing for these specific mutations is of low yield.- - - - - - - - - - ranking = 0.033276359581999keywords = chest (Clic here for more details about this article) |
| | Next -> |