1/47. 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 = fibrillation, atrial fibrillation (Clic here for more details about this article) |
2/47. Acute, symptomatic atrial fibrillation after sildenafil citrate therapy in a patient with hypertrophic obstructive cardiomyopathy.This case report describes a patient with hypertrophic cardiomyopathy who developed symptomatic atrial fibrillation on two occasions after ingesting sildenafil citrate (Viagra). Sildenafil citrate should be withheld or used with extreme caution in persons with hypertrophic obstructive cardiomyopathy.- - - - - - - - - - ranking = 0.625keywords = fibrillation, atrial fibrillation (Clic here for more details about this article) |
3/47. Apical hypertrophic cardiomyopathy due to a de novo mutation Arg719Trp of the beta-myosin heavy chain gene and cardiac arrest in childhood. A case report and family study.Hypertrophic cardiomyopathy (HCM) is a myocardial disease with variable phenotpye and genotype. To demonstrate that the mutation Arg719Trp in the cardiac beta-myosin heavy chain (beta MHC) gene is a high risk factor for sudden death and can be associated with an unusual apical non-obstructive HCM, we report the case of a 6 1/2 year old boy, who suffered cardiac arrest. The proband had a de novo mutation of the beta MHC gene (Arg719Trp) on the paternal beta MHC allele and a second maternally transmitted mutation (Met349Thr), as was shown previously (Jeschke et al. 1998 (11)). Here we report the clinical phenotype of the proband and of his relatives in detail. The proband had a marked apical and midventricular hypertrophy of the left and right ventricle without obstruction. There was an abnormal relaxation of both ventricles. Holter monitoring detected no arrhythmia. ventricular fibrillation was inducible only by aggressive programmed stimulation. The boy died 3 1/2 years later after another cardiac arrest due to arrhythmia. Five carriers of the Met349Thr mutation in the family were asymptomatic and had no echocardiographic changes in the heart, suggesting a neutral inherited polymorphism or a recessive mutation. It is concluded that there is an association of the mutation Arg719Trp in the beta-myosin heavy chain with sudden cardiac death in a young child. disease history in conjunction with the genetic analysis suggests that the implantation of a defibrillator converter would have been a beneficial and probably life saving measure.- - - - - - - - - - ranking = 0.07960771442579keywords = fibrillation (Clic here for more details about this article) |
4/47. ventricular fibrillation induced by rapid atrial rates in patients with hypertrophic cardiomyopathy.AIMS: To describe the mechanisms of induction of ventricular fibrillation (VF) by rapid atrial rates in patients with hypertrophic cardiomyopathy (HCM). methods: Electrophysiological studies, management and follow-up in three patients with HCM with VF induced by atrial pacing. RESULTS: In one patient, spontaneous sinus tachycardia triggered VF. In another patient, VF occurred after verapamil infusion during rapid atrial fibrillation, and in the remaining patient there was no clinical VF. In all three patients, short runs of atrial pacing (cycle length 272-380 ms) induced VF, and QRS widening preceded fibrillation in all patients. Marked ventricular electrogram fragmentation was documented in one patient during atrial pacing and in another patient during late ventricular extra-stimuli. hypotension was associated with sinus tachycardia in one patient. The two patients developing clinical VF underwent atrioventricular (AV) junctional ablation; a ventricular defibrillator was implanted in one, and a mode-switching dual-chamber pacemaker in the other. No arrhythmic events occurred during 34- and 35-month follow-up, respectively. In the other patient, postatrial fibrillation pauses caused syncope, and he is asymptomatic 52 months after implantation of a dual-chamber pacemaker. CONCLUSIONS: Rapid atrial rates can trigger VF in some patients with HCM, probably through a combination of electrophysiological and ischaemic mechanisms. AV junctional ablation may prevent VF in selected cases.- - - - - - - - - - ranking = 0.72764628655474keywords = fibrillation, atrial fibrillation (Clic here for more details about this article) |
5/47. Serious arrhythmias in patients with apical hypertrophic cardiomyopathy.We report cases of serious arrhythmias associated with apical hypertrophic cardiomyopathy (AHCM). Thirty-one patients were referred to our institute to undergo further assessment of their AHCM from 1988 to 1999. Three patients with nonsustained ventricular tachycardia demonstrated an 123I-MIBG regional reduction in the tracer uptake. In two patients with ventricular fibrillation (VF), the findings from 123I-MIBG imaging revealed regional sympathetic denervation in the inferior and lateral regions. Electrophysiologic study demonstrated reproducible induction of VF in aborted sudden death and presyncopal patients, resulting in the need for an implantable defibrillator device and amiodarone in each patient. patients with refractory atrial fibrillation with a rapid ventricular response suffered from serious congestive heart failure. A prudent assessment and strategy in patients with this disease would be indispensable in avoiding a disastrous outcome.- - - - - - - - - - ranking = 0.20460771442579keywords = fibrillation, atrial fibrillation (Clic here for more details about this article) |
6/47. 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 = 0.25keywords = fibrillation, atrial fibrillation (Clic here for more details about this article) |
7/47. Left atrial free floating ball thrombus in hypertrophic cardiomyopathy: a case report.In this article we reported a 33-year-old female patient who was referred to our emergency department with atrial fibrillation, congestive heart failure, and cardiogenic shock. In her transthoracic echocardiography, a free-floating ball-type mass was seen in the left atrial cavity with a hypertrophic cardiomyopathy. An emergency open-heart surgery was performed. The mass was removed, and pathologic examination confirmed the thrombotic material.- - - - - - - - - - ranking = 0.125keywords = fibrillation, atrial fibrillation (Clic here for more details about this article) |
8/47. A novel TPM1 mutation in a family with hypertrophic cardiomyopathy and sudden cardiac death in childhood.We sought to define the pathogenic mutation in a family with hypertrophic cardiomyopathy (HC) and a markedly arrhythmogenic phenotype. The proband was an 8-year-old female with a sentinel event of sudden death. Screening echocardiograms revealed HC in 2 of her 3 siblings and her father. Her youngest male sibling was diagnosed with HC at age 2 years and died suddenly at age 6 years from ventricular fibrillation despite an implanted cardioverter defibrillator. Using dna extracted from peripheral lymphocytes, linkage exclusion was performed by haplotype analysis of polymorphic markers for the HC genes. genes not excluded by linkage were analyzed for mutations using denaturing high-performance liquid chromatography (DHPLC) and direct dna sequencing. Using this strategy, a 610 T>G nucleotide substitution in the alpha-tropomyosin gene (TPM1) was identified resulting in a novel L185R (leucine [L] to arginine [R]) missense mutation. This mutation was a spontaneous germ-line mutation originating in the proband's father. L185R-TPM1 cosegregated with family members having clinical evidence of HC, including the proband as confirmed by molecular autopsy. The mutation was not present in 400 reference alleles. Thus, a novel missense mutation in TPM1 was discovered in a family with HC and sudden death in childhood. Unlike previously defined mutations that may disrupt the interactions between alpha-tropomyosin monomers, the L185R mutation may affect troponin-T binding. Defining the pathogenic mutation enabled definitive molecular diagnosis of 2 surviving children.- - - - - - - - - - ranking = 0.07960771442579keywords = fibrillation (Clic here for more details about this article) |
9/47. Pulmonary vein firing triggering atrial fibrillation after open heart surgery.We report the case of a 44-year-old woman with obstructive hypertrophic cardiomyopathy without history of prior arrhythmias who underwent surgical myectomy. She developed symptomatic postoperative atrial fibrillation (AF) that was refractory to antiarrhythmic therapy and could not be adequately rate controlled. AF always was preceded by short and fast runs of atrial tachycardia. In the electrophysiology laboratory, this arrhythmia appeared to originate from the right superior pulmonary vein and conducted with variable degrees of exit block. Pulmonary vein isolation guided by circular mapping was performed, and no further episodes of either atrial tachycardia or AF were noted. This case highlights the potential role of the pulmonary veins in the pathophysiology of postoperative AF.- - - - - - - - - - ranking = 0.625keywords = fibrillation, atrial fibrillation (Clic here for more details about this article) |
10/47. Sudden death due to atrial fibrillation in hypertrophic cardiomyopathy: a predictable event in a young patient.This case refers to a 39-year-old woman with hypertrophic cardiomyopathy (HCM) and family history of sudden death (SD). In 1985, high rate atrial stimulation induced VF. In 1996 an ICD was implanted and she remained without arrhythmic events until November 2000 when the device reported one episode of atrial fibrillation degenerating into VF and terminated by the ICD. The VF induction mechanism recorded by the ICD was similar to that observed in 1985. The high incidence of atrial tachyarrhythmias in HCM renders cases like this at higher risk of SD. The predictive role of incremental atrial stimulation merits highlighting in future studies.- - - - - - - - - - ranking = 0.625keywords = fibrillation, atrial fibrillation (Clic here for more details about this article) |
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