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January 2003 articles...

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Lisa Salberg Find out more about Lisa Salberg
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  • January 2003 articles...

    J Am Coll Cardiol 2003 Jan 15;41(2):315-21 Related Articles, Links

    Maximum left ventricular thickness and risk of sudden death in patients with hypertrophic cardiomyopathy.

    Olivotto I, Gistri R, Petrone P, Pedemonte E, Vargiu D, Cecchi F.

    Cardiologia S. Luca, Azienda Ospedaliera Careggi, Florence, Italy

    We sought to assess the relationship between maximum left ventricular (LV) wall thickness and outcome in patients with hypertrophic cardiomyopathy (HCM).An association between maximum LV wall thickness and risk of sudden death was suggested in HCM. This finding requires further investigation, given the important implications for risk stratification and treatment.We analyzed the mortality and risk profile of 237 patients (age 41 +/- 17 years; 63% male) classified into five groups based on echocardiographic maximum LV thickness.During follow-up (12 +/- 7 years), 36 patients died of cardiovascular causes, including 16 sudden deaths. Maximum LV thickness was not associated with a risk of sudden death (p = 0.37) nor with overall cardiovascular mortality (p = 0.7). With the exception of the small subset with thickness values </=15 mm, with a consistently benign clinical course, the distribution of sudden death and overall cardiovascular mortality was not significantly different among the other four classes, ranging from 16 to 19 mm to >/=30 mm. Among 30 patients with extreme LV thickness (>/=30 mm), only one sudden event occurred among six patients diagnosed at <18 years of age (17%) and none among 24 diagnosed at >/=18 years of age. The prevalence of nonsustained ventricular tachycardia, syncope, an abnormal blood pressure response to exercise, and atrial fibrillation was similar among the five thickness classes.During 12-year follow-up, we observed no association between maximum LV thickness and cardiovascular mortality in a community-based population with HCM. The degree of maximum LV wall thickness should be considered in the context of a multifactorial approach to risk stratification, rather than as an isolated risk factor. Only in those patients diagnosed at a very young age might the presence of extreme LV wall thickness represent, per se, a potential marker of risk of sudden death.
    Knowledge is power ... Stay informed!
    YOU can make a difference - all you have to do is try!

    Dx age 12 current age 46 and counting!
    lost: 5 family members to HCM (SCD, Stroke, CHF)
    Others diagnosed living with HCM (or gene +) include - daughter, niece, nephew, cousin, sister and many many friends!
    Therapy - ICD (implanted 97, 01, 04 and 11, medication
    Currently not obstructed
    Complications - unnecessary pacemaker and stroke (unrelated to each other)

  • #2
    J Clin Invest 2003 Jan;111(2):209-16 Related Articles, Links


    Idiopathic restrictive cardiomyopathy is part of the clinical expression of cardiac troponin I mutations.

    Mogensen J, Kubo T, Duque M, Uribe W, Shaw A, Murphy R, Gimeno JR, Elliott P, McKenna WJ.

    Department of Cardiological Sciences, St. George's Hospital Medical School, London, United Kingdom. Department of Medicine and Geriatrics, Kochi Medical School, Kochi, Japan. Departamento de Cardiologia, Clinica Medellin, Medellin, Colombia.

    Restrictive cardiomyopathy (RCM) is an uncommon heart muscle disorder characterized by impaired filling of the ventricles with reduced volume in the presence of normal or near normal wall thickness and systolic function. The disease may be associated with systemic disease but is most often idiopathic. We recognized a large family in which individuals were affected by either idiopathic RCM or hypertrophic cardiomyopathy (HCM). Linkage analysis to selected sarcomeric contractile protein genes identified cardiac troponin I (TNNI3) as the likely disease gene. Subsequent mutation analysis revealed a novel missense mutation, which cosegregated with the disease in the family (lod score: 4.8). To determine if idiopathic RCM is part of the clinical expression of TNNI3 mutations, genetic investigations of the gene were performed in an additional nine unrelated RCM patients with restrictive filling patterns, bi-atrial dilatation, normal systolic function, and normal wall thickness. TNNI3 mutations were identified in six of these nine RCM patients. Two of the mutations identified in young individuals were de novo mutations. All mutations appeared in conserved and functionally important domains of the gene. This article was published online in advance of the print edition. The date of publication is available from the JCI website, http://www.jci.org.

    PMID: 12531876 [PubMed - in process]
    Knowledge is power ... Stay informed!
    YOU can make a difference - all you have to do is try!

    Dx age 12 current age 46 and counting!
    lost: 5 family members to HCM (SCD, Stroke, CHF)
    Others diagnosed living with HCM (or gene +) include - daughter, niece, nephew, cousin, sister and many many friends!
    Therapy - ICD (implanted 97, 01, 04 and 11, medication
    Currently not obstructed
    Complications - unnecessary pacemaker and stroke (unrelated to each other)

    Comment


    • #3
      Perinat Med 2002;30(6):517-21 Related Articles, Links


      A rare presentation of Pompe disease with massive hypertrophic cardiomyopathy at birth.

      Noori S, Acherman R, Siassi B, Luna C, Ebrahimi M, Pavlova Z, Ramanathan R.

      Division of Neonatology, Women's and Children's Hospital, Keck School of Medicine, University of Southern California, Los Angeles, USA. [email protected]

      We report a term infant with Pompe disease presenting in the immediate newborn period. The infant was born at 40 weeks' gestation, weighing 3600 g to a 32 year-old black female. Infant presented at delivery with massive hypertrophic cardiomyopathy and pulmonary hypertension. Diagnosis was confirmed by low alpha-glucosidase activity. The histopathology and electron microscopic findings were consistent with Pompe disease. This is the second reported case of Pompe disease presenting at delivery.

      PMID: 12530110 [PubMed - in process]
      Knowledge is power ... Stay informed!
      YOU can make a difference - all you have to do is try!

      Dx age 12 current age 46 and counting!
      lost: 5 family members to HCM (SCD, Stroke, CHF)
      Others diagnosed living with HCM (or gene +) include - daughter, niece, nephew, cousin, sister and many many friends!
      Therapy - ICD (implanted 97, 01, 04 and 11, medication
      Currently not obstructed
      Complications - unnecessary pacemaker and stroke (unrelated to each other)

      Comment


      • #4
        J Cardiovasc Electrophysiol 2002 Dec;13(12):1300-2 Related Articles, Links


        Pulmonary vein firing triggering atrial fibrillation after open heart surgery.

        Saad EB, Saliba WI, Marrouche NF, Natale A.

        The Center for Atrial Fibrillation, Department of Cardiovascular Medicine, Section of Cardiac Pacing and Electrophysiology, The Cleveland Clinic Foundation, Cleveland Ohio 44195, USA.

        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.

        PMID: 12521350 [PubMed - in process]
        Knowledge is power ... Stay informed!
        YOU can make a difference - all you have to do is try!

        Dx age 12 current age 46 and counting!
        lost: 5 family members to HCM (SCD, Stroke, CHF)
        Others diagnosed living with HCM (or gene +) include - daughter, niece, nephew, cousin, sister and many many friends!
        Therapy - ICD (implanted 97, 01, 04 and 11, medication
        Currently not obstructed
        Complications - unnecessary pacemaker and stroke (unrelated to each other)

        Comment


        • #5
          Radiology 2003 Jan;226(1):129-37 Related Articles, Links


          First-Pass MR Imaging in the Assessment of Perfusion Impairment in Patients with Hypertrophic Cardiomyopathy and the Asp175Asn Mutation of the alpha-Tropomyosin Gene.

          Sipola P, Lauerma K, Husso-Saastamoinen M, Kuikka JT, Vanninen E, Laitinen T, Manninen H, Niemi P, Peuhkurinen K, Jaaskelainen P, Laakso M, Kuusisto J, Aronen HJ.

          Depts of Clin Radiology (P.S., H.M., H.J.A.), Medicine (K.P., P.J., M.L., J.K.), and Clin Physiology and Nuclear Medicine (M.H.S., J.T.K., E.V., T.L.), Kuopio Univ Hosp, Puijonlaaksontie 2, 70210 Kuopio, Finland.

          PURPOSE: To assess first-pass magnetic resonance (MR) imaging in the evaluation of perfusion impairment in a genetically homogeneous population of patients with hypertrophic cardiomyopathy (HCM) and the Asp175Asn mutation of the alpha-tropomyosin gene and to evaluate the association between hypertrophy and perfusion. MATERIALS AND METHODS: Rest-stress first-pass MR imaging with gadopentetate dimeglumine was performed in 17 patients with HCM and the Asp175Asn substitution in the alpha-tropomyosin gene and in five control subjects. Global and segmental first-pass reserve index (FPR) measurements were derived from signal intensity versus time curves. Left ventricular (LV) wall thickness and LV mass index were measured on cine MR images. The Mann-Whitney test was used to evaluate the difference in FPR between the patient group and the control group. The Spearman correlation was used to evaluate the association between LV hypertrophy and FPR. RESULTS: Global FPR was significantly lower in the patients with HCM than in the control subjects (1.12 +/- 0.35 vs 1.80 +/- 0.58, P =.015). In patients with HCM, maximal LV wall thickness and LV mass index correlated negatively with global FPR (r = -0.723, P =.001 and r = -0.598, P =.011, respectively). At the regional level, segmental FPR correlated inversely with LV wall thickness (r = -0.389; P <.001) in patients with HCM. CONCLUSION: First-pass MR imaging facilitates global and regional evaluation of perfusion impairment in patients with HCM. The severity of perfusion impairment is associated with the degree of LV hypertrophy. Copyright RSNA, 2002

          PMID: 12511681 [PubMed - in process]
          Knowledge is power ... Stay informed!
          YOU can make a difference - all you have to do is try!

          Dx age 12 current age 46 and counting!
          lost: 5 family members to HCM (SCD, Stroke, CHF)
          Others diagnosed living with HCM (or gene +) include - daughter, niece, nephew, cousin, sister and many many friends!
          Therapy - ICD (implanted 97, 01, 04 and 11, medication
          Currently not obstructed
          Complications - unnecessary pacemaker and stroke (unrelated to each other)

          Comment

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