Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Endocavitary fulguration was performed in 31 cases of high-risk ventricular tachycardia (VT) for which antiarrhythmics, including Amiodarone and Class-I antiarrhythmic agents, given alone or in combination, proved ineffective. Permanent VT was incessant in nine patients at the time of fulguration: five of these were moribund, and two were unconscious. Included in the series were ten cases of arrhythmogenic right ventricular dysplasia, 13 cases of chronic VT after myocardial infarction, five cases of idiopathic dilated cardiomyopathy, two cases of idiopathic VT, and one case that involved a congenital anomaly. Combined fulguration and antiarrhythmic therapy succeeded in preventing VT in the 27 patients who survived the initial period of treatment. Thirteen patients needed two or more sessions, however, before their VT was brought under control. Three of the four early deaths were probably related to an imperfect technique. Four late deaths were due to spontaneous evolution of the disease. One patient without cardiac failure died suddenly. The follow-up period ranged from a minimum of 13 to a maximum of 34 months, with an average follow-up of nearly 2 years. The success rate for fulguration alone or for antiarrhythmic drugs is about 90%. This group of patients is compared with a group that involved 73 less severe cases treated with drugs. Fulguration appears to be the better form of treatment.
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PMID:Treatment of resistant ventricular tachycardia with endocavitary fulguration and antiarrhythmic therapy, compared to antiarrhythmic therapy alone: experience in 111 consecutive cases with a mean follow-up of 18 months. 1522 49

A retrospective study was done on 14 cats and 36 dogs diagnosed with right atrioventricular valve malformations (RAVM), either tricuspid dysplasia (n = 38) or Ebstein's anomaly (n = 12). Comparison with a large reference population including 85 250 animals allowed an epidemiologic analysis of these RAVM, demonstrating that Labrador, Boxer and Chartreux cats had a 35, 7 and 11-fold higher risk respectively to be affected by one of these heart diseases than other breeds, without sexual predisposition. In 93% of cases, cardiac auscultation revealed a heart murmur, whose grade was not significantly correlated with the echographic stages of the valvular disease. The echocardiographic examination confirmed the frequent association between RAVM and other congenital anomalies, particularly mitral dysplasia, in 84% and 20% of tricuspid dysplasia and Ebstein anomaly respectively. Last, the prognosis of RAVM was worsened by atrial fibrillation and right-sided heart failure.
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PMID:[Congenital malformations of the tricuspid valve in domestic carnivores: a retrospective study of 50 cases]. 1524 46

Since Kerr described programmed cell death (apoptosis) as a process distinct from necrosis, there have been many studies of apoptosis in disease, especially of immunological origin. Because cardiac myocytes are terminally differentiated cells, they have typically been assumed to die exclusively by necrosis. However, during the last decade this view has been challenged by several studies demonstrating that a significant number of cardiac myocytes undergo apoptosis in myocardial infarction, heart failure, myocarditis, arrhythmogenic right ventricular dysplasia, and immune rejection after cardiac transplantation, as well as in other conditions of stress. These are potentially relevant observations, because apoptosis--unlike necrosis--can be blocked or reversed at early stages. Specific inhibition of this process may confer a considerable degree of cardioprotection, but requires a thorough understanding of the underlying mechanisms. Recent progress includes a better understanding of the importance of mitochondria-initiated events in cardiac myocyte apoptosis, of factors inducing apoptosis in heart failure and during hypoxia, and of the dual pro-apoptotic and anti-apoptotic effects of hypertrophic stimuli such as beta-adrenoceptor agonists, angiotensin converting enzyme inhibitors, nitric oxide and calcineurin. The investigation of cytoprotective and apoptotic signal transduction pathways has revealed important new insights into the roles of the mitogen-activated protein kinases p38, extracellular signal regulated kinase and c-Jun N-terminal kinase in cardiac cell fate. Our present review focuses on the intracellular signal transduction pathways of cardiac myocyte apoptosis and the possibility of specific inhibition of the process.
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PMID:Possible therapeutic targets in cardiac myocyte apoptosis. 1532 Jul 55

Ryanodine receptors (RyRs) are the major sarcoplasmic reticulum calcium-release channels required for excitation-contraction coupling in skeletal and cardiac muscle. Mutations in RyRs have been linked to several human diseases. Mutations in the cardiac isoform of RyR2 are associated with catecholaminergic polymorphic ventricular arrhythmias (CPVT), and arrhythmogenic right ventricular dysplasia type 2 (ARVD2), whereas mutations in the skeletal muscle isoform (RyR1) are linked to malignant hyperthermia (MH) and central core disease (CCD). RyRs are modulated by several other proteins, including the FK506 binding proteins (FKBPs), FKBP12 and FKBP12.6. These immunophilins appear to stabilize a closed state of the channel and are important for cooperative interactions among the subunits of RyRs. This review discusses the regulation of RyRs by FKBPs and the possibility that defective modulation of RyR2 by FKBP12.6 could play a role in heart failure, CPVT, and ARVD2. Also discussed are the consequences of FKBP12 depletion to skeletal muscle and the possibility of FKBP12 involvement in certain forms of MH or CCD.
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PMID:Regulation of ryanodine receptors by FK506 binding proteins. 1545 14

Arrhythmogenic right ventricular cardiomyopathy, also known as right ventricular dysplasia, is a genetically determined heart muscle disease associated with arrhythmia, heart failure, and sudden death. Autosomal dominant inheritance is typical. The identification of causative mutations in cell adhesion proteins has shed new light on its pathogenesis. Fibrofatty replacement of the myocardium, the hallmark pathologic feature, may be a response to injury caused by myocyte detachment. Sudden death is often the first manifestation in probands, emphasizing the importance of evaluating asymptomatic relatives for the disease. Standardized guidelines facilitate the clinical diagnosis of right ventricular dysplasia. However, familial studies have highlighted the need to broaden the diagnostic criteria, which are highly specific but lack sensitivity for early disease. Modifications have been proposed for the diagnosis of right ventricular dysplasia in relatives. Early right ventricular dysplasia is characterized by a "concealed phase" in which electrocardiographic and imaging abnormalities are often absent, but patients may nonetheless be at risk for arrhythmic events. Detection at this stage remains a clinical challenge, underscoring the potential value of mutation analysis in identifying affected persons. Serial evaluation of patients with suspected right ventricular dysplasia is recommended as clinical features may develop during the follow-up period. The onset of symptoms such as palpitation or syncope may herald an active phase of a previously quiescent disease, during which patients are at increased risk for sudden death. Greater awareness of right ventricular dysplasia among physicians and judicious use of implantable cardioverter-defibrillators may help to prevent unnecessary deaths.
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PMID:Arrhythmogenic right ventricular cardiomyopathy: clinical presentation, diagnosis, and management. 1550 Dec 7

In a cardiological department of a non-referral hospital responsible for 80,000 inhabitants with 2500 in-hospital patients and 1500 out-hospital patients per year, the prevalence, symptoms and prognosis of arrhythmogenic right ventricular dysplasia-cardiomyopathy (ARVD/C) were examined retrospectively. From 1997 to 2002, ARVD/C was diagnosed in 35 females and 45 males (overall prevalence 1 in 1000 inhabitants) with a mean age of 45.6 years. Symptoms were chest pain (80%), palpitations (60%) and syncopes (30%), and clinical findings were repetitive ventricular premature beats (50%), supraventricular arrhythmias (30%), ventricular tachycardia (20%), aborted sudden death due to ventricular fibrillation (1%), right heart failure (4%), biventricular heart failure (1%) and high grade AV nodal block (4%). Endomyocardial biopsies were not performed. Aborted sudden death occurred in only one patient (0.3%) before the diagnosis was made, annual heart failure rate was 1%. No deaths appeared in a follow-up of 1-5 (mean 2.4) years with clinical assessment as the basis of diagnosis. The prevalence of ARVD/C is much higher and the prognosis better than expected from results of reference centers.
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PMID:Prevalence of right ventricular dysplasia-cardiomyopathy in a non-referral hospital. 1556 39

Congestive cardiac failure could be as important as cardiac arrhythmias in the natural history of arrhythmogenic right ventricular dysplasia. This can be related to the progressive replacement of myocardium by fat and fibrosis of the right ventricle. The left ventricle may also be involved by the same disease process. Moreover, inflammation can be superimposed on ARVD, resulting in a wide spectrum of clinical presentation which can mimick idiopathic dilated cardiomyopathy. Right ventricular cardiac failure has been controlled by anterior dynamic cardiomyoplasty.
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PMID:[Arrhythmogenic right ventricular dysplasia]. 1560 20

Pulmonary arteriovenous fistulas are vascular malformations, which, by virtue of producing abnormal vascular connections proximal to the units of gas exchange, result in intrapulmonary right-to-left shunting. These malformations or fistulas reflect at least in part disordered angiogenesis, and less commonly recruitment and dilation of pre-existing vascular channels. Pulmonary arteriovenous fistulas occur in a number of diverse clinical settings. Such fistulas are a well-established feature of the Weber-Osler-Rendu complex, or hereditary haemorrhagic telangiectasia, an autosomal dominant vascular dysplasia characterized by mucocutaneous telangiectasis, epistaxis, gastrointestinal haemorrhage, and arteriovenous malformations in the lung, brain, liver and elsewhere. They are also seen in the patient with acute or chronic liver disease, disease that is usually but not invariably severe, or those with non-cirrhotic portal hypertension. They may occur as congenital malformations, single or diffuse, large or small in isolation, and when large or extensive enough may result in hypoxaemia, clinical cyanosis, and heart failure. Cerebral vascular accidents are also a well-known complication of this disorder. An extensive literature has accumulated with regard to the pulmonary arteriovenous fistulas seen in the setting of the Weber-Osler-Rendu complex, and there is considerable information on the genetics, basic biology, clinical findings, complications and therapeutic interventions of these malformations in the setting of this syndrome. These issues, however, are not the primary considerations of this review, although some aspects of this fascinating disorder will be discussed later. Rather the focus will be on pulmonary arteriovenous malformations that develop in the setting of cavopulmonary surgery, and their relationship to the pulmonary arteriovenous fistulas occurring in the hepatopulmonary syndrome. The complex tapestry of these overlapping and intersecting clinical observations will be unfolded in the light of their chronology.
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PMID:The biological "scrabble" of pulmonary arteriovenous malformations: considerations in the setting of cavopulmonary surgery. 1568 49

Geleophysic dysplasia is an autosomal recessive short-limbed, dysmorphic syndrome. The condition is frequently associated with cardiac valvular disease, which may result in secondary hypertrophy and cardiac failure. We describe two distantly related Pakistani children with classical features of geleophysic dysplasia. Pulmonary stenosis was recognized in both within a few months of birth. The younger child shows no signs of cardiac decompensation at the age of 7 years whilst her older cousin has developed a significant pulmonary gradient requiring surgical intervention in the second decade of life. The natural history of his disorder highlights that the cardiac involvement seen in apparently stable forms of geleophysic dysplasia is frequently progressive.
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PMID:Natural history of cardiac involvement in geleophysic dysplasia. 1569 Mar 80

As for the preceding years, important studies regarding several remaining clinical issues for electrophysiologists have been reported in 2004. Large randomized studies have underlined the need for an EP study in asymptomatic patients with overt ventricular preexcitation. In addition to a short antegrade refractory period, arrhythmia induction (atrial fibrillation or reciprocating tachycardia) argues for accessory pathway ablation. Although currently leading to fairly good results, atrial fibrillation ablation technique is still evolving. Encircling pulmonary vein and the surrounding atrial tissue seems to give better long term clinical results as compared to ostial pulmonary vein disconnection. Large series have confirmed that whatever cardiomyopathy etiology, prophylactic ICD implantation was associated with a reduction of sudden arrhythmic death during follow-up in patients with low ejection fraction. However, in order to save one patient more and more patients have to be implanted because of the increasing efficacy of pharmacological treatment for heart failure. Three clinical series of arrhythmogenic right ventricular dysplasia implanted with AICD have been published this year. The prognostic factors for the occurrence of severe ventricular arrhythmia are hemodynamically ill tolerated ventricular tachycardia, and VT induction during EP study. Management of patients with Brugada syndrome is still far from being well defined. Interestingly in a recent report, hydroquinidine has been found to reduce the incidence of ventricular arrhythmia in the follow-up as well as the rate of ventricular arrhythmia induction in the EP lab. Yet, prophylactic ICD implantation remains the treatment of choice in symptomatic and inducible patients.
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PMID:[The best of arrhythmia in 2004]. 1571 65


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