Hypertrophic Cardiomyopathy With LV Apical Aneurysm

Study Questions:

What is the clinical course and prognosis of patients with hypertrophic cardiomyopathy (HCM) and left ventricular (LV) apical aneurysm?

Methods:

In a retrospective review, clinical data for 1,940 consecutive patients with HCM at two centers (Minneapolis Heart Institute, Tufts Medical Center) were analyzed. Of those, 93 (4.8%) were identified with LV apical aneurysms. Mean age was 56 ± 13 years and 69% were male. LV apical aneurysms were identified by echocardiography in 50 of the 93 patients (54%), including 32 patients with medium or large aneurysms and 18 patients with smaller aneurysms; of these 50 patients, identification of the apical aneurysm was enhanced by contrast in 21 (42%), including 11 identified solely by contrast enhancement. LV apical aneurysms were identified by cardiac magnetic resonance (CMR) only (n = 39) or computed tomography (CT) (n = 4) in 43 (46%) patients, including three in whom contrast echocardiography failed to identify a small aneurysm.

Results:

Over 4.4 ± 3.2 years, 3 of 93 patients (3%) died suddenly or of heart failure (HF), and 22 (24%) survived with contemporary treatment interventions: 18 experienced appropriate implantable cardioverter-defibrillator (ICD) discharges, two underwent heart transplants, and two were resuscitated after cardiac arrest. The sudden cardiac death (SCD) event rate was 4.7%/year. Notably, recurrent monomorphic ventricular tachycardia (VT) requiring ≥2 ICD shocks occurred in 13 patients, including six who underwent successful radiofrequency ablation of arrhythmic focus without VT recurrence. Five non-anticoagulated patients experienced non-fatal thromboembolic events (1.1 %/year), and 13 with apical thrombus and anticoagulation did not experience an embolic event. There was no consistent relationship between aneurysm size and adverse HCM-related events. Rate of HCM-related deaths combined with life-saving aborted disease-related events was 6.4%/year, threefold greater than the 2.0%/year event rate in 1,847 HCM patients without aneurysms (p < 0.001).

Conclusions:

Patients with LV apical aneurysms are at high risk for arrhythmic SCD and thromboembolic events. The authors concluded that identification of this HCM phenotype expands risk stratification and can lead to effective treatment interventions for potentially life-threatening complications.

Perspective:

Established major risk factors for SCD in HCM include nonsustained VT, maximal LV wall thickness ≥3 cm, family history of SCD, history of syncope, and an abnormal blood pressure response to exercise. LV apical aneurysm and HCM with LV ejection fraction <50% (end-stage HCM) are considered less common subgroups at increased risk. These data reinforce that patients with HCM and LV apical aneurysm represent a high-risk subgroup within the disease spectrum, with over 25% having either died or experienced an adverse complication of the disease. That not all apical LV aneurysms were detected on echocardiography reinforces a potential role for additional imaging modalities (CT or CMR) among patients with HCM.


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