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      Bisoprolol Successfully Improved the Intraventricular Pressure Gradient in a Patient with Midventricular Obstructive Hypertrophic Cardiomyopathy with an Apex Aneurysm due to Apical Myocardial Damage

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          Abstract

          Midventricular obstructive hypertrophic cardiomyopathy (MVOHCM) is a rare form of hypertrophic cardiomyopathy (HCM). An 80-year-old man was administered bisoprolol and warfarin therapies as treatment for MVOHCM with an apex aneurysm due to myocardial damage and intra-aneurysmal thrombus not complicated by atrial fibrillation. The pressure gradient in the midventricle successfully improved from 53.9 to 21.8 mmHg, and the intra-aneurysmal thrombus disappeared.

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          Pharmacological treatment of hypertrophic cardiomyopathy: current practice and novel perspectives.

          Enrico Ammirati, Rachele Contri, Raffaele Coppini … (2016)
          Hypertrophic cardiomyopathy (HCM) is entering a phase of intense translational research that holds promise for major advances in disease-specific pharmacological therapy. For over 50 years, however, HCM has largely remained an orphan disease, and patients are still treated with old drugs developed for other conditions. While judicious use of the available armamentarium may control the clinical manifestations of HCM in most patients, specific experience is required in challenging situations, including deciding when not to treat. The present review revisits the time-honoured therapies available for HCM, in a practical perspective reflecting real-world scenarios. Specific agents are presented with doses, titration strategies, pros and cons. Peculiar HCM dilemmas such as treatment of dynamic outflow obstruction, heart failure caused by end-stage progression and prevention of atrial fibrillation and ventricular arrhythmias are assessed. In the near future, the field of HCM drug therapy will rapidly expand, based on ongoing efforts. Approaches such as myocardial metabolic modulation, late sodium current inhibition and allosteric myosin inhibition have moved from pre-clinical to clinical research, and reflect a surge of scientific as well as economic interest by academia and industry alike. These exciting developments, and their implications for future research, are discussed.
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            Echocardiographic diagnosis of the different phenotypes of hypertrophic cardiomyopathy

            Vito Maurizio Parato, Valeria Antoncecchi, Fabiola Sozzi … (2016)
            Hypertrophic Cardiomyopathy (HCM) is an inherited cardiovascular disorder of great genetic heterogeneity and has a prevalence of 0.1 – 0.2 % in the general population. Several hundred mutations in more than 27 genes, most of which encode sarcomeric structures, are associated with the HCM phenotype. Then, HCM is an extremely heterogeneous disease and several phenotypes have been described over the years. Originally only two phenotypes were considered, a more common, obstructive type (HOCM, 70 %) and a less common, non-obstructive type (HNCM, 30 %) (Maron BJ, et al. Am J Cardiol 48:418 –28, 1981). Wigle et al. (Circ 92:1680–92, 1995) considered three types of functional phenotypes: subaortic obstruction, midventricular obstruction and cavity obliteration. A leader american working group suggested that HCM should be defined genetically and not morphologically (Maron BJ, et al. Circ 113:1807–16, 2006). The European Society of Cardiology Working Group on Myocardial and Pericardial Diseases recommended otherwise a morphological classification (Elliott P, et al. Eur Heart J 29:270–6, 2008). Echocardiography is still the principal tool for the diagnosis, prognosis and clinical management of HCM. It is well known that the echocardiographic picture may have a clinical and prognostic impact. For this reason, in this article, we summarize the state of the art regarding the echocardiographic pattern of the HCM phenotypes and its impact on clinical course and prognosis.
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              Progression of hypertrophic cardiomyopathy into a hypokinetic left ventricle: higher incidence in patients with midventricular obstruction.

              Sayid Fighali, Zvonimir Krajcer, Sidney K. Edelman … (1987)
              The development of segmental or generalized left ventricular hypokinesia is an unusual occurrence in patients with hypertrophic cardiomyopathy. To determine the incidence and possible pathophysiologic mechanisms responsible for this process, the serial clinical and laboratory data of 62 patients with the diagnosis of hypertrophic cardiomyopathy were analyzed. During a mean follow-up period of 8 years (range 2 to 21), 5 patients (Group A) developed left ventricular hypokinesia, whereas the remaining 57 patients (Group B) continued to exhibit the clinical and laboratory findings of hypertrophic cardiomyopathy. Three patients developed a dilated left ventricle with generalized hypokinesia; two other patients had segmental left ventricular wall motion abnormalities. None of these five patients who developed left ventricular hypokinesia had fixed coronary artery disease. The mean age, sex, mean duration of follow-up, presence of coronary myocardial bridges and angina pectoris, and an interventricular gradient were all similar in Groups A and B. Midventricular obliteration was seen in 4 (80%) of the 5 patients in Group A and in 4 (7%) of the 57 patients in Group B (p less than 0.001). Findings from this study reveal that segmental or generalized left ventricular hypokinesia can develop in patients with hypertrophic cardiomyopathy in the absence of fixed coronary artery disease. Such hypokinesia can occur after an acute myocardial infarction or it can develop gradually without clinical or electrocardiographic evidence of infarction. Patients with the mid-ventricular obliteration variant of hypertrophic cardiomyopathy are at a higher risk of developing segmental or diffuse left ventricular hypokinesia.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Intern Med
                Journal ID (iso-abbrev): Intern. Med
                Title: Internal Medicine
                Publisher: The Japanese Society of Internal Medicine
                ISSN (Print): 0918-2918
                ISSN (Electronic): 1349-7235
                Publication date (Electronic): 17 October 2018
                Publication date (Print): 15 February 2019
                Volume: 58
                Issue: 4
                Pages: 535-539
                Affiliations
                [1 ]Departments of Cardiovascular Medicine, Japanese Red Cross Kagoshima Hospital, Japan
                [2 ]Departments of Clinical Laboratory Unit, Japanese Red Cross Kagoshima Hospital, Japan
                [3 ]Departments of Neurosurgery, Japanese Red Cross Kagoshima Hospital, Japan
                [4 ]Departments of Cardiovascular Medicine and Hypertension, Graduate School of Medical and Dental Sciences, Kagoshima University, Japan
                Author notes

                Correspondence to Dr.　Kenjuro Higo, khigo@ 123456m2.kufm.kagoshima-u.ac.jp

                Article
                DOI: 10.2169/internalmedicine.0997-18
                PMC ID: 6421145
                PubMed ID: 30333393
                SO-VID: e6c88259-8133-4939-895f-3c0b97225a71
                Copyright © Copyright © 2019 by The Japanese Society of Internal Medicine
                License:

                The Internal Medicine is an Open Access journal distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit ( https://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                Date received : 12 February 2018
                Date accepted : 20 May 2018
                Categories
                Subject: Case Report

                Keywords: midventricular obstructive hypertrophic cardiomyopathy,bisoprolol,beta-blocker
                Data availability:
                Keywords: midventricular obstructive hypertrophic cardiomyopathy, bisoprolol, beta-blocker

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