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      A 66-Year-Old Female with Apical Hypertrophic Cardiomyopathy Presenting with Hypertensive Crises and Type 2 Myocardial Infarction and a Normal Coronary Angiogram

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      1 , , 2 , 2 , 2
      Case Reports in Cardiology
      Hindawi

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          Abstract

          A 66-year-old female presented to the emergency room with an episode of chest pain that lasted for a few minutes before resolving spontaneously. Electrocardiogram showed a left bundle branch block, left ventricular hypertrophy, and T wave inversions in the lateral leads. Initial cardiac troponin level was 0.15 ng/ml, with levels of 4 ng/ml and 9 ng/ml obtained 6 and 12 hours later, respectively. The peak blood pressure recorded was 195/43 mmHg. Echocardiogram with DEFINITY showed a small left ventricular cavity with apical hypertrophy, and coronary angiogram showed no stenotic or occluding lesions in the coronary arteries. The patient was admitted for a type 2 myocardial infarction with hypertensive crises. She was diagnosed with having apical hypertrophic cardiomyopathy, which is a variant of hypertrophic cardiomyopathy (HCM) in which the hypertrophy predominantly involves the apex of the left ventricle resulting in midventricular obstruction, as opposed to the left ventricular outflow tract obstruction seen in HCM. Patients with apical HCM may present with angina, heart failure, myocardial infarction, syncope, or arrhythmias and are typically managed with medications like verapamil and beta-blockers for those who have symptoms and antiarrhythmic agents like amiodarone and procainamide for treatment of atrial fibrillation and ventricular arrhythmias. An implantable cardioverter defibrillator (ICD) is recommended for high-risk HCM patients with a history of previous cardiac arrest or sustained episodes of ventricular tachycardia, syncope, and a family history of sudden death.

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          Most cited references25

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          Phenotypic spectrum and patterns of left ventricular hypertrophy in hypertrophic cardiomyopathy: morphologic observations and significance as assessed by two-dimensional echocardiography in 600 patients.

          This study sought to achieve an understanding of the true structural heterogeneity of hypertrophic cardiomyopathy. The diversity and clinical significance of the morphologic expression of hypertrophic cardiomyopathy have not been fully defined within this broad disease spectrum. Patterns of left ventricular hypertrophy were characterized by two-dimensional echocardiography in a large study cohort of 600 patients (7 to 79 years old, mean age 45; 393 [66%] men) consecutively studied at two referral centers. Left ventricular wall thickness was 15 to 52 mm (mean [+/- SD] 22.3 +/- 5). A multitude of patterns of asymmetric left ventricular hypertrophy were identified, with the most common showing diffuse involvement of substantial portions of both ventricular septum and free wall. Of 16 possible patterns of left ventricular hypertrophy, 12 (78%) were identified among the 600 patients. Hypertrophy most commonly involved two left ventricular segments (228 patients [38%]) or three or more segments (202 patients [34%]), but was also localized to one segment in a substantial number of patients (170 [28%]). The anterior portion of the ventricular septum was the region of the left ventricle that most frequently showed thickening (573 patients [96%]), and was also the predominant site of hypertrophy in most patients (492 patients [83%]). Patterns of wall thickening that were either concentric (i.e., symmetric) or confined to the apex were particularly uncommon (in 1% each). 1) In hypertrophic cardiomyopathy, the distribution of left ventricular hypertrophy is characteristically asymmetric and particularly heterogeneous, encompassing most possible patterns of wall thickening, from extensive and diffuse to mild and segmental, and with no single morphologic expression considered typical or classic. 2) A greater extent of left ventricular hypertrophy was associated with younger age and more marked mitral valve systolic anterior motion and outflow obstruction but showed no relation to either magnitude of symptoms or gender.
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            Long-term outcome in patients with apical hypertrophic cardiomyopathy.

            The aim of this study was to describe long-term outcome in patients with apical hypertrophic cardiomyopathy (ApHCM) followed in a tertiary referral center. Apical hypertrophic cardiomyopathy is a relatively rare form of hypertrophic cardiomyopathy (HCM), first described in Japan. Initial reports, based on a limited number of patients, emphasized the benign nature of this condition. A retrospective study of 105 patients with ApHCM diagnosed at the Toronto General Hospital from 1975 to 2000 was performed. Symptoms, clinical findings, mortality and cardiovascular morbidity were analyzed. The mean age at presentation was 41.4 +/- 14.5 years. During a mean follow-up of 13.6 +/- 8.3 years from presentation, cardiovascular mortality was 1.9% (2/105) and annual cardiovascular mortality was 0.1%. Overall survival was 95% at 15 years. Thirty-two patients (30%) had one or more major morbid events, the most frequent being atrial fibrillation (12%) and myocardial infarction (10%). Probability of survival without morbid events was 74% at 15 years. Three predictors of cardiovascular morbidity were identified: age at presentation or = II at baseline. Forty-four percent of the patients were asymptomatic at the time of last follow-up. Apical hypertrophic cardiomyopathy in North American patients is not associated with sudden cardiac death and has a benign prognosis in terms of cardiovascular mortality. Nevertheless, one third of these patients experience serious cardiovascular complications, such as myocardial infarction and arrhythmias. These data are likely to influence the counseling and management of patients with ApHCM.
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              Detection of apical hypertrophic cardiomyopathy by cardiovascular magnetic resonance in patients with non-diagnostic echocardiography.

              To investigate the role of cardiovascular magnetic resonance (CMR) in a series of patients with ECG repolarisation changes and normal echocardiography. 10 patients with anterolateral T wave inversion for which there was no obvious pathological cause who had normal routine echocardiography without contrast for the exclusion of hypertrophic cardiomyopathy (HCM) also had CMR that was diagnostic of apical HCM. Apical HCM detected by CMR could be morphologically severe with wall thickness up to 28 mm, or mild. The extent of repolarisation abnormalities did not correlate to the morphological severity. In patients with unexplained repolarisation abnormalities, a normal routine echocardiogram without contrast does not exclude apical HCM. Further imaging with CMR or contrast echocardiography may be required. The reliance on routine echocardiography to exclude apical HCM may have led to underreporting of this condition.
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                Author and article information

                Contributors
                Journal
                Case Rep Cardiol
                Case Rep Cardiol
                CRIC
                Case Reports in Cardiology
                Hindawi
                2090-6404
                2090-6412
                2018
                25 October 2018
                : 2018
                : 7089149
                Affiliations
                1Department of Internal Medicine, Maimonides Medical Center, 4802 10th Avenue, Brooklyn, New York 11219, USA
                2Department of Cardiology, Maimonides Medical Center, 4802 10th Avenue, Brooklyn, New York 11219, USA
                Author notes

                Academic Editor: Konstantinos P. Letsas

                Author information
                http://orcid.org/0000-0002-3334-6417
                Article
                10.1155/2018/7089149
                6222212
                30498604
                9b90d753-93d7-49c4-8f24-9fe9eb07eee9
                Copyright © 2018 Vineet Meghrajani et al.

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 2 July 2018
                : 24 September 2018
                : 4 October 2018
                Categories
                Case Report

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