Hypertrophic Cardiomyopathy With Apical Left Ventricular Aneurysm

Abstract

We report a case of hypertrophic cardiomyopathy (HCM) with apical left ventricular aneurysm, which is difficult to review because cases are so rare. A 54-year-old Japanese man was first found to have an electrocardiographic abnormality (T-wave inversion at rest) 19 years ago, and non-obstructive apical HCM without identifiable cause was diagnosed by echocardiography, left ventriculography, and clinical findings. After 19 years, he was admitted because of repeated episodes of palpitation and chest oppression at rest. Widespread left ventricular hypertrophy from the anteroseptal wall to the apex with an apical left ventricular aneurysm was detected by echocardiography, left ventriculography, and cardiac magnetic resonance imaging. Histologic examination of the hypertrophic apical myocardium surrounding the aneurysm showed that the myocardial tissue had been extensively replaced by fibrous tissue containing hypertrophic myocardial fibers, and uptakes of [123I]-metaiodobenzyl guanidine (MIBG) and [123I-] beta-methyliodophenyl pentadecanoic acid (BMIPP) in single-photon emission photography images were reduced despite high myocardial perfusion. On the other hand, histologic examination of the hypertrophic anterior wall revealed myocardial hypertrophy with disorganization; myocardial perfusion and the uptakes of MIBG and BMIPP were preserved. Abnormalities of myocardial fatty acid metabolism and sympathetic neuron activity with preserved perfusion flow and histologic changes such as fibrosis in the apical wall are indicative of apical myocardial injury or ischemia (infarction) without coronary artery stenosis; apical aneurysm may have occurred in severe apical HCM with cavity obliteration up to the midventricular level.

Association of ST elevation with apical aneurysm in hypertrophic cardiomyopathy

“…_ 2 ) T D $ F I G ] cases particularly between the MVO-HCM and ACM with a small non-obliterated area in ACM. 2,3,10,27 Supplementary video related to this article can be found at http://dx.doi.org/10.1016/j.ihj.2015.05.019.…”

Effect of Intravenous Propranolol on Left Ventricular Apical Ballooning Without Coronary Artery Stenosis (Ampulla Cardiomyopathy). Three Cases.

“…Although the configuration of left ventricular asynergy is different from that of midventricular obstruction, apical aneurysm formation in midventricular obstruction of hypertrophic cardiomyopathy has been reported. 18 Additionally, the improvement in apical asynergy in specific cases following acute propranolol injection suggests that dynamic intraventricular obstruction does play a role in the persistent hemodynamic, electrocardiographic, and echocardiographic wall motion abnormalities when there is a definite intraventricular pressure gradient. Secondary ischemia caused by the increased wall tension and dynamic midventricular obstruction, as in sigmoid septum and obstructive cardiomyopathy, might also be important contributory factors.…”

Hypertrophic cardiomyopathy with apical aneurysm: left ventricular reconstruction and cryoablation for ventricular tachycardia

“…Mid-ventricular obstruction is an uncommon variant of LV obstructive HCM. 6 It may lead to the development of an apical aneurysm, creating two distinct (basal and apical) LV chambers.…”