Fatal Refractory Cardiogenic Shock in Apical Hypertrophic Cardiomyopathy: A Case Report

Abstract

Background: Apical hypertrophic cardiomyopathy (ApHCM) is a rare phenotypic variant of hypertrophic cardiomyopathy (HCM) characterised by hypertrophy confined to the left ventricular apex. Although traditionally considered to carry a more favourable prognosis than obstructive HCM, ApHCM is increasingly recognised as a potentially life-threatening condition, capable of causing severe arrhythmias, heart failure, and — in rare cases — fatal cardiogenic shock. Diagnosis is particularly challenging when concomitant coronary artery disease is present, as the clinical presentation may closely mimic acute coronary syndrome. Aim: The present case report aims to describe a diagnostically challenging and rapidly fatal presentation of ApHCM in a patient with pre-existing coronary artery disease. Case Summary: We report the case of a 75-year-old woman with known ischemic heart disease and a history of left circumflex artery stenting, who presented with acute chest pain and palpitations. Electrocardiography revealed atrial fibrillation with deep anteroapicolateral negative T-waves and episodes of non-sustained ventricular tachycardia (NSVT). Elevated high-sensitivity troponin initially suggested an ischemic aetiology; however, emergent coronary angiography excluded stent thrombosis or new obstructive coronary disease. Transthoracic echocardiography (TTE) identified apical wall hypertrophy of 16 mm, a characteristic 'ace of spades' left ventricular cavity configuration, and a resting intraventricular pressure gradient of 60 mmHg. The five-year HCM Risk-SCD score was estimated at 3.68%, placing the patient in the intermediate-risk category. Despite this classification, the clinical course was rapidly and fatally complicated by refractory cardiogenic shock. Conclusion: This case highlights the potential for a catastrophic course in ApHCM, even in patients classified as intermediate risk by validated prognostic tools. The coexistence of prior coronary artery disease, new-onset atrial fibrillation, NSVT, and significant intraventricular obstruction defines a particularly high-risk clinical phenotype. Early cardiac MRI, systematic arrhythmia monitoring, and timely reassessment of ICD candidacy are critical in complex ApHCM presentations.