This case refers to a 69-year-old woman with a history of hypertrophic cardiomyopathy (HCM) with severe mid-ventricular obstruction complicated by apical aneurysm and restrictive phenotype. This patient had no left ventricular outflow tract obstruction and previous examinations showed moderate mitral regurgitation (MR) with suboptimal echocardiographic follow-up. ICD was previously implanted for primary prevention. Disease progression was complicated by episodes of acute heart failure (HF) caused by atrial fibrillation/tachycardia (AF or AT) with rapid ventricular response despite pharmacological therapy and a transcatheter ablation attempt.
The patient was admitted to our Cardiac Intensive Care Unit with acute pulmonary oedema and extreme general frailty. EKG showed normal sinus rhythm alternating with AT with high ventricular rate. Transthoracic (TTE) and transesophageal (TEE) echocardiograms gave evidence of severe hypertrophy of left ventricular (LV) mid-cavity segments (mid IVS = 26 mm) with mid-ventricular systolic obliteration (with no more evidence of significant gradient) and a large apical aneurysm (37×35 mm) resulting in an “hourglass” shaped LV cavity (Figure 1). In addition the examinations highlighted systolic dysfunction with severe stroke volume reduction and severe MR caused by annulus dilatation with anterior jet direction due to leaflets abnormal coaptation (Figure 2).
HF was multifactorial, caused not only by MR and atrial tachyarrhythmias but also due to the abnormal large apical aneurysm and the small left ventricular chamber with low compliance.
The anatomical features obtained from TEE and cardiac CT (Figure 3) were analyzed by the Heart Team. Surgery with aneurysmectomy and mitral valve repair or replacement was excluded due to patient's frailty. Despite the suboptimal valve and LV chamber anatomy, percutaneous mitral valve repair with MitraClip was considered but also deferred owing to the unstable hemodynamic conditions. Further hemodynamic deterioration led to the patient’s exitus.
Discussion: HCM with mid-ventricular obstruction may be complicated by apical aneurysm. Large apical aneurysms can reduce LV contractile efficiency and the combination with severe MR can cause further loss of anterograde cardiac output leading to a progressive and fatal deterioration. A better outcome can only be achieved through careful follow-up and early detection and management of disease complications.