One of the many faces of hypertrophic cardiomyopathy

Introduction: Echocardiography-based epidemiologic studies have shown a prevalence of 1 case per 500 pe-ople for hypertrophic cardiomyopathy, which accounts for approximately 20 million people globally. What is the meaning of this numbers besides statistics? This is the most common monogenic cardiovascular disorder, diverse in clinical presentation, including sudden death as first manifestation, but underrecognized in 90% of the cases.

Objective: The purpose of this paper is to present a patient with apical hypertrophic cardiomyopathy (AHCM), diagnosed starting from nonspecific symptoms and signs. A high level of suspicion and thorough investigations are required in order to diagnose such a chameleonic disease when survival is a race against the clock.

Case presentation: We present the case of a 72 year old woman, hypertensive, with a personal history of cured thyroid cancer, with favourable outcome, euthyroid at presentation, who was addressed to the hospital for acute coronary syndrome based on electrocardiographic (EKG) changes. The patient presented atypical chest pain, dispnea on minimal-moderate exertion and palpitations. Clinical and paraclinical examinations were performed, centered on the cardiovascular system – EKG at rest, echocardiography, EKG Holter monito-ring, cardiac magnetic resonance (CMR). The clinical and biologic findings were within normal limits. The EKG revealed left ventricular hypertrophy (LVH) and deep, negative T waves in the anterior leads. The echocardiography identified apical LVH and api-cally displaced hypertrophic pappilary muscles, without obstruction, confirmed by transpulmonary contrast administration. Mild left atrium dilatation and second degree mitral regurgitation were associated. CMR con-firmed the AHCM diagnostic, without cardiac fibro-sis. A long nocturnal episode of atrial fibrillation was identified. The anticoagulant was initiated, together with antiarrythmic therapy with amiodarone. The an-tihypertensive treatment with angiotensin receptor blocker and diuretic and the antiarrythmic therapy im-proved the symptoms. In this clinical picture dizziness and fainting appeared after the initiation of amiodaro-ne, although with small doses the heart rate was convenient. The case particularities include the late onset, with non-specific presentation, hypertrophic cardiomiopathy be-ing a multifaceted disease, sometimes difficult to differentiate from acute cardiac ischemia based on the EKG changes. The rapid and accurate diagnosis is essential in order to select the optimal treatment for these life threatening diseases. An active approach, prevention or early treatment initiation is recommended for the complications of AHCM. In this particular case radi-ofrequency ablation is being considered regarding the poor tolerance of the arrythmia and of the antiarrythmic treatment.

ISSN
ISSN – online: 2734 – 6382
ISSN-L 1220-658X
ISSN – print: 1220-658X
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CNCSIS B+
CODE: 379
CME Credits: 10 (Romanian College of Physicians)
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