Introduction: Apical hyperthrophic cardiomyopathy (AHCM) is an uncommon form of hyperthrophic car-diomyopathy (HCM) with less prevalent detection of gene mutations and sudden cardiac death compared with other forms of HCM.
Methods: We present the case of a 76 years old patient with multiple cardiovascular risk factors (hypertensi-on, dyslipidemia, obesity, former smoker) with histo-ry of unprovoked pulmonary embolism – PE (2018), without evidence of deep venous thrombosis, in treat-ment with rivaroxaban, who presented with worsening severe dyspnea at effort and peripheral edemas, symp-toms started a few days before admission. He denied angina or palpitations. On clinical examination we identified obesity grade II and bilateral leg edema and routine laboratory tests revealed controlled dyslipide-mia.The electrocardiogram (ECG) showed atrial flutter (AF) with block 5:1, heart rate 50/min, with negative T waves in DI, aVL and V2-V6. Transthoracic echocardi-ography with contrast was performed showing no wall motion abnormalities otherwise with a mild concentric left ventricle hypertrophy (LVH) except for the apex where there was severe LVH suggestive for AHCM; there was an increased aortic velocity with an aortic valve with degenerative changes. We thought that the changes on the ECG were most likely due to AHCM. A thoracic tomography scan with contrast was also per-formed because of associated dyspnea which excluded an acute recurrence of PE. The next day the T waves on ECG normalized, putting forward for consideration an acute coronary syndrome. We performed a corona-rography which confirmed a 80% stenosis of proximal left anterior descending (LAD) artery with subsequent placement of a drug eluting stent.
Results: Sometimes heart failure might occur due to multiple etiological factors and mechanisms of decom-pensation. We had a patient with AHCM with an ECG that could be interpreted as typical for this pathology but with concomitent severely affected LAD coronary artery, with AF and high grade atrioventricular block plus mild aortic stenosis with a bicuspid aortic valve with indication for strict follow up.
Conclusions: AHCM was first described in Japan whe-re has the highest prevalence, but is also documented in other countries (rare in Caucasian population). Note that AHCM often mimic acute coronary syndromes through clinical manifestations and electrocardiogra-phic aspects. Our case report showed a Caucasian pati-ent with AHCM with concomitent severe atherosclero-tic desease and aortic stenosis on a bicuspid valve.