Apical hypertrophic cardiomyopathy

Abstract

We describe a patient with asymptomatic apical hypertrophic cardiomyopathy (AHCM) who later developed cardiac arrhythmias, and briefly discuss the diagnostic modalities, differential diagnosis and treatment option for this condition. AHCM is a rare form of hypertrophic cardiomyopathy which classically involves the apex of the left ventricle. AHCM can be an incidental finding, or patients may present with chest pain, palpitations, dyspnea, syncope, atrial fibrillation, myocardial infarction, embolic events, ventricular fibrillation and congestive heart failure. AHCM is frequently sporadic, but autosomal dominant inheritance has been reported in few families. The most frequent and classic electrocardiogram findings are giant negative T-waves in the precordial leads which are found in the majority of the patients followed by left ventricular (LV) hypertrophy. A transthoracic echocardiogram is the initial diagnostic tool in the evaluation of ACHM and shows hypertrophy of the LV apex. AHCM may mimic other conditions such as LV apical cardiac tumors, LV apical thrombus, isolated ventricular non-compaction, endomyocardial fibrosis and coronary artery disease. Other modalities, including left ventriculography, multislice spiral computed tomography, and cardiac magnetic resonance imagings are also valuable tools and are frequently used to differentiate AHCH from other conditions. Medications used to treat symptomatic patients with AHCM include verapamil, beta-blockers and antiarrhythmic agents such as amiodarone and procainamide. An implantable cardioverter defibrillator is recommended for high risk patients.

Апікальна Гіпертрофічна Кардіоміопатія. Клінічний Випадок І Огляд Літератури

“…АГКМП является наиболее редкой и сложной для диагностики формой гипертрофической кардиомио-патии, в патологический процесс может быть изоли-рованно вовлечена анатомическая верхушка ЛЖ или также апикальная треть МЖП [17,22,33,41,62].…”

“…Для АГКМП наряду с воль-тажными признаками гипертрофии ЛЖ характерны нарушения процессов реполяризации -глубокие (гигантские) более 10 мм отрицательные зубцы Т в левых грудных отведениях, иногда в отведениях от конечностей, с одновременной глубокой косо-нисходящей депрессией сегмента ST [3,5,22,62]. Выраженная гипертрофия МЖП приводит к по-явлению патологических зубцов Q, что может об-условить ошибочную диагностику острого инфар-кта миокарда.…”

“…Дифференциальную диагностику у больных с ГКМП следует также проводить такими нозологи-ями, как некомпактная кардиомиопатия ЛЖ [54], эндомиокардиальный фиброз [62].…”

Case Report - Isolated Apical Hypertrophic Cardiomyopathy

“…8 The diagnostic criteria for apical hypertrophic cardiomyopathy included demonstration of asymmetric hypertrophy, confined predominantly to the apex with an apical wall thickness ≥ 15 mm and a ratio of maximal apical to posterior wall thickness ≥ 1.5, based on echocardiography. 9 In this case, asymmetric apical wall is 26 mm and its posterior wall is 4.2 mm and a ratio of maximal apical to posterior wall thickness 6.19, satisfying the diagnosis of apical left ventricular hypertrophic cardiomyopathy. The degree of thickness increases from base to apex resulting in a markedly diminished apical cavity and a spade-shaped left ventricular cavity suggesting apical left ventricular hypertrophic cardiomyopathy in the apical 4 chamber view of twodimensional echocardiographic image of this patient.…”

Diagnosis of Cardiomyopathies: Tips and Tricks for Internists and General Practitioners

“…45,46 The unremarkable recent coronary angiogram performed seemed to exclude a CAD. To better characterize the apical region a CMR was proposed but declined by the patient because of claustrophobia.…”