BACKGROUND Echocardiographic parameters could be implicated in the development of apical asynergy (characterized by apical sequestration or apical aneurysm) and worse cardiovascular outcome in patients with apical hypertrophic cardiomyopathy (ApHCM). OBJECTIVE Echocardiographic parameters and morphological patterns of left ventriculograms are associated with cardiovascular morbidity and mortality in patients with ApHCM. METHODS We followed 47 cases with echocardiographically documented ApHCM. Echocardiographic findings of the extent and degree of hypertrophy, sustained cavity obliteration, and paradoxical diastolic jet flow were measured. All patients underwent a cardiac catheterization except for the cases whose informed consent was not acquired. The clinical manifestations were assessed and recorded by the attending physicians during 35.4 ± 23.7 months follow-up. RESULTS Among the 47 patients with ApHCM, 30 patients presented as the "pure" form and 17 patients present as the "mixed" form. Seventeen of 28 patients with sustained cavity obliteration showed paradoxical flow by echocardiography. Thirty-one underwent left ventriculograms and showed morphological abnormalities, including "ace-of-spades" configuration (15/31), apical sequestration (12/31), and apical aneurysm (4/31). The results demonstrated that cardiovascular morbidities occurred in 21 of 47 patients and were closely related to the presence of mixed form ApHCM, cavity obliteration, and paradoxical flow by univariate and multivariate Cox analysis. During the period of follow-up, 4 patients (9.5%) died, and among them 3 had concomitant apical aneurysm. CONCLUSIONS We concluded that detection of cavity obliteration and paradoxical flow and discrimination of pure form from mixed form by echocardiography, as well apical sequestration from apical aneurysm in ApHCM patients, is warranted.