OBJECTIVETo analyze the clinical and cardiac imaging characteristics of patients with left ventricular apical hypoplasia (LVAH).

METHODSFrom January 2008 to January 2012, seven patients [3 male/4 female, age: 6 - 44 (19.9 ± 14.2) years] with LVAH were included in this cohort. Transthoracic echocardiogram was performed in all patients, cardiovascular MRI was performed in 3 patients and cardiovascular CT in another 2 patients. In addition, one LVAH patient underwent cardiac catheterization and angiography examination.

RESULTSFour out of 7 patients complained chest discomfort. Precordial murmur was heard in 3 patients. Atrial fibrillation was evidenced by electrocardiogram in 3 patients. Left ventricular end-diastolic diameter [(57.9 ± 11.6) mm] increased while left ventricule (LV) longitudinal diameter reduced in all patients. Left ventricular systolic function was reduced in 2 patients and mean LVEF was (47.6 ± 17.2)%. The interventricular septum bulged towards the right, and the ventricular septum thickness was (7.3 ± 1.2) mm. The papillary muscles were dominant on the flattened LV anteroapical region. The right ventricle elongated and wrapped around the hypoplastic left ventricular apex, and the dimension of right ventricle was (19.7 ± 7.6) mm. Focal fat replacement of the left ventricular apical wall was evidenced in 5 patients underwent cardiovascular MRI or CT examinations.

CONCLUSIONSClinical symptoms are non-specific in LVAH patients. Truncated and spherical LV, abnormal origin of papillary muscles in the flattened LV anterior apex and an elongated right ventricle wrapping around the LV apex as well as focal fat replacement of the left ventricular apical wall are typical imaging characteristics of LVAH.