[Apical sequestration in hypertrophic cardiomyopathy: its clinical features and pathophysiology]

The clinical and pathophysiological significance of apical sequestration, in which an apical cavity was sequestered from the remainder of the left ventricle by cavity obliteration was investigated in patients with hypertrophic cardiomyopathy (HCM). Among 196 consecutive patients, 24 with apical sequestration and 70 control subjects proven to have no sequestration with left ventriculography were selected for this study using echocardiography combined with Doppler color flow imaging. Various cardiac disorders occurred significantly more frequently in patients with apical sequestration than in the 70 controls: NYHA > or = II, 83% vs 51%; thromboembolism, 17% vs 3%; ventricular tachycardia, 47% vs 11%; and apical asynergy, 75% vs 4%. Continuous Doppler ultrasound revealed that all 24 patients with sequestration had a high systolic blood flow velocity across the obliterated cavity (2.7 +/- 0.9 m/s). During isovolumic relaxation or early diastolic filling or both, 21 of them had paradoxical jet flow directed toward the basal cavity away from the apex, with the peak flow velocity ranging from 1.0 m/s to 3.5 m/s (mean 1.9 +/- 0.7). The maximal diastolic pressure gradient across the obliterated cavity ranged between 4 mmHg and 49 mmHg using the simple Bernoulli's equation, which suggested a significantly higher pressure in the sequestered apical chamber during early diastole. Patients with sequestration were classified into 2 groups; 17 with (group A) and 7 without (group B) apical hypertrophy. The time interval from the closing of the aortic valve to the onset of filling into the sequestered cavity was longer in group A than in group B (401 +/- 191 vs 131 +/- 145 ms, p < 0.01) as assessed by the pulsed Doppler technique. Angiographic asynergy of the apex was more frequent in group A than in group B (100 vs 29%, p < 0.01). In group B, the midventricular cavity was incompletely obliterated throughout the cardiac cycle; whereas, in group A, it was obliterated completely in systole and partially in diastole. Apical sequestration is not uncommon in HCM; it is accompanied by abnormal segmental wall motion, which may be related to ventricular arrhythmias and thromboembolism. Prolonged cavity obliteration with a higher systolic apical pressure and a persistent diastolic intraventricular gradient may play a pathogenic role in apical aneurysmal formation in the absence of fixed coronary artery disease, particularly in patients with apical hypertrophy.