Cases reported "Ventricular Dysfunction"

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1/4. Perioperative management of biventricular failure after closure of a long-standing massive arteriovenous fistula.

    PURPOSE: To report the perioperative management of arteriovenous fistula (AVF) closure in a patient with high-output heart failure and pulmonary hypertension. CLINICAL FEATURES: In a 71-yr-old man, closure of a long-standing massive AVF between the right femoral artery and vein was performed. After closure of the AVF, his pulmonary artery pressure (PAP) increased from 52/21 mmHg to 68/26 mmHg, his cardiac index decreased from 5.27 L.min(-1).m(-2) to 3.18 L.min(-1).m(-2), and his pulmonary wedge pressure increased from 15 mmHg to 32 mmHg due to an acute increase in afterload. Co-administration of prostaglandin E and a phosphodiesterase III inhibitor improved the cardiac index and the PAP. CONCLUSIONS: Surgical closure of the fistula may not always lead to resolution of the high output cardiac failure. In this case, afterload management using arterial dilators (prostaglandin E1, phosphodiesterase III inhibitor), use of inotropic drugs (phosphodiesterase III inhibitor), and close attention to volume status was crucial for a successful outcome after surgical AVF closure.
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2/4. Novel KCNJ2 mutation in familial periodic paralysis with ventricular dysrhythmia.

    BACKGROUND: Mutations in the KCNJ2 gene, which codes cardiac and skeletal inward rectifying K channels (Kir2.1), produce Andersen's syndrome, which is characterized by periodic paralysis, cardiac arrhythmia, and dysmorphic features. methods AND RESULTS: In 3 Japanese family members with periodic paralysis, ventricular arrhythmias, and marked QT prolongation, polymerase chain reaction/single-strand conformation polymorphism/dna sequencing identified a novel, heterozygous, missense mutation in KCNJ2, Thr192Ala (T192A), which was located in the putative cytoplasmic chain after the second transmembrane region M2. Using the xenopus oocyte expression system, we found that the T192A mutant was nonfunctional in the homomeric condition. Coinjection with the wild-type gene reduced the current amplitude, showing a weak dominant-negative effect. CONCLUSIONS: T192, which is located in the phosphatidylinositol-4,5-bisphosphate binding site and also the region necessary for Kir2.1 multimerization, is a highly conserved amino acid residue among inward-rectifier channels. We suggest that the T192A mutation resulted in the observed electrical phenotype.
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3/4. Torsade de pointes and T-wave alternans in a patient with brainstem hemorrhage.

    Torsade de pointes, a form of life-threatening ventricular arrhythmia associated with QT prolongation, is rarely reported in intracranial hemorrhage. T-wave alternans is recently considered a risk marker for ventricular arrhythmias. To our knowledge, we report the first case of brainstem hemorrhage in which the patient presented with torsade de pointes and T-wave alternans. The implication of this association is discussed.
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4/4. Beta2-agonist induced ventricular dysrhythmias secondary to hyperexcitable conduction system in the absence of a long qt syndrome.

    BACKGROUND: The use of inhaled beta 2-agonists for bronchodilation in the treatment of lower airway obstruction is accepted worldwide. These agents are used for symptomatic relief of lower airway obstruction and, as well, can be employed prophylactically in exercise-induced bronchospasm. Cardiac dysrhythmias, specifically the long qt syndrome, have been associated with cardiac events precipitated by sympathomimetics. There are reports of documented long qt syndrome in association with syncope in children; however, there are no reports of beta 2-agonist-induced syncope in the absence of long qt syndrome. OBJECTIVE: To determine predisposing cardiac factors resulting in syncope associated with inhaled beta 2-agonist use. METHOD: Case report. The index case was evaluated for cardiac pathology through non-invasive techniques, cardiac catheterization, and electrophysiologic studies. Electrophysiologic studies included provocative challenge with parenteral adrenergic agents. RESULTS: Non-invasive studies were unremarkable. There was no evidence of prolonged QT syndrome or support for vasopressor syncope. Electrophysiologic studies revealed reproducible polymorphic ventricular tachycardia. This predisposition required a ventricular stimulation program of higher intensity while on mexilitine. CONCLUSIONS: This case of syncope associated with inhaled, short-acting beta 2-agonist resulted from a hyperexcitable conduction system rather than the presence of a long qt syndrome.
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