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(Circulation. 2008;118:1541-1549.)
© 2008 American Heart Association, Inc.

Heart Failure

Prevalence, Clinical Significance, and Natural History of Left Ventricular Apical Aneurysms in Hypertrophic Cardiomyopathy

Martin S. Maron, MD; John J. Finley, MD; J. Martijn Bos, MD; Thomas H. Hauser, MD, MMSc, MPH; Warren J. Manning, MD; Tammy S. Haas, RN; John R. Lesser, MD; James E. Udelson, MD; Michael J. Ackerman, MD, PhD; Barry J. Maron, MD

From the Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, Mass (M.S.M., J.J.F., J.E.U.); Department of Medicine, Pediatrics, and Molecular Pharmacology, Experimental Therapeutics/Divisions of Cardiovascular Diseases and Pediatric Cardiology, and the Mayo Clinic Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, Minn (J.M.B., M.J.A.); Departments of Medicine (Cardiovascular Division) (T.H.H., W.J.M.) and Radiology (W.J.M.), Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Mass; and Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minn (T.S.H., J.R.L., B.J.M.).

Correspondence to Martin S. Maron, MD, Tufts Medical Center, No. 70, 800 Washington St, Boston, MA 02111. E-mail mmaron{at}tuftsmedicalcenter.org

Received March 25, 2008; accepted July 29, 2008.

Background— Hypertrophic cardiomyopathy (HCM) is the most common genetic heart disease characterized by a diverse clinical and phenotypic spectrum. This study reports the prevalence, morphology, clinical course, and management of an underrecognized subgroup of HCM patients with left ventricular apical aneurysms.

Methods and Results— Of 1299 HCM patients, 28 (2%) were identified with left ventricular apical aneurysms, including a pair of identical twins. Aneurysms were recognized at a wide age range (26 to 83 years), including 12 patients (43%) who were 50 years of age. Apical aneurysms varied considerably in size (maximum dimension, 10 to 66 mm), were dyskinetic/akinetic with thin rims, and were associated with transmural (and often more extensive) myocardial scarring identified by late gadolinium enhancement cardiovascular magnetic resonance. Apical aneurysms were recognized by echocardiography in only 16 of 28 patients (57%) but by cardiovascular magnetic resonance in the 12 patients undetected by echocardiography. Left ventricular chamber morphology varied; however, 19 patients (68%) showed an "hourglass" contour, with midventricular hypertrophy producing muscular narrowing and intracavitary gradients in 9 patients (74±42 mm Hg). Sarcomeric protein missense mutations known to cause other phenotypic expressions of HCM were present in 3 patients. Over 4.1±3.7 years of follow-up, 12 patients (43%) with left ventricular apical aneurysms experienced adverse disease complications (event rate, 10.5%/y), including sudden death, appropriate implantable cardioverter-defibrillator discharges, nonfatal thromboembolic stroke, and progressive heart failure and death.

Conclusions— Patients with left ventricular apical aneurysms represent an underappreciated subset in the heterogeneous HCM disease spectrum with important clinical implications, often requiring a high index of suspicion and cardiovascular magnetic resonance for identification. Apical aneurysms in HCM are associated with substantial cardiovascular morbidity and mortality and raise novel treatment considerations.

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CLINICAL PERSPECTIVE

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