Introduction: Apical hypertrophic cardiomyopathy (HCM) is an uncommon variant of hypertrophic cardiomyopathy, in which thickened apical segments produce a „spade-shaped,” small apical cavity, and is often first detected by transthoracic echocardiography or cardiac MRI after suggestive electrocardiographic (ECG) changes (deep T waves in lateral leads). Apical HCM has been considered until recently to hold a mild prognosis. The prevalence of significant coronary artery disease (CAD) in HCM has been estimated to be just over 10%, and the significant ECG changes of the apical form can make difficult the differential diagnosis.
Case presentation: We present the case of a 50 year old man, former heavy smoker and dyslipidemic, asymptomatic until 2015, when he develops progressive shortness of breath, up to IV NYHA class phenomena, for which he was admitted to hospital. No angina described. His cardiac family history was unremarkable. The ECG showed old anterior and inferior infarction. Transthoracic echocardiography showed dilated left cavites, with severe systolic dysfunction (LVEF of 25%) and localized wall motion abnormality – left ventricular (LV) aneurysm of the basal inferolateral wall, but also a peculiar localized severe thickening of the apical anterior and lateral wall segments. Coronarography was performed and showed subocclusion of the first diagonal artery, as well as of the mid-segment of the right coronary artery which also showed distal cronic occlusion. He was stabilized at the time by pharmaco-logic therapy, and remained asymptomatic until 2017, when he was admitted to hospital in acute pulmonary cardiogenic edema. In order to establish his revascularization indication, a cardiac MRI was performed and confirmed a dilated LV cavity, with severe systolic dys-function (LVEF 24%) and moderate right ventricular dysfunction (RVEF 37%), with severe thickening of the apical anterolateral segments. It also showed an old inferolateral transmural infarction, with late gadolinium enhancement more than 75% of the inferolateral wall and apical aneurism. Myocardial viability was also tested by exercise SPECT which showed irreversible myocardial perfusion uptake of the inferior, inferolateral and apical wall segments, therefore showing no eligible criteria for revascularization. His Holter monitoring showed several rung of nonsustained ventricular tachycardia. The patient was eligible for primary prevention of sudden cardiac death and a cardiac defibrillator was implanted. The key of the complete diagnosis was in old medical findings, as the patient was asked for older documents and a routine electrocardiogram performed in 2011 that showed deep, negative T waves in precordial leads and his echocardiography described anterior LVwall of 16 mm at basis and 20 mm at the apex, confirming HCM as his primary disease.
Conclusions: This case underlines the importance of complete echo acquisitions when specific ECG changes suggest apical HCM. It also magnifies the importance of multimodality imaging for full functional and structural diagnosis and viability assessment. The association of HCM and CAD appears to magnify sudden cardiac death.