Clinical Features of the Dilated Phase of Hypertrophic Cardiomyopathy in Comparison With Those of Dilated Cardiomyopathy

Abstract

Patients with D-HCM were more symptomatic at diagnosis, although LV dilatation and impaired fractional shortening seemed more severe in patients with DCM. The prognosis for D-HCM patients was worse than that for patients with DCM despite similar or even more intensive treatment for heart failure.

45-year-old man with hypertrophic cardiomyopathy after alcohol ablation, progression to dilated cardiomyopathy (RCD code: III‑1B.8a)

“…End-stage HCM, the stadium in which our patient was admitted to the hospital, is also characterized with higher incidence of ventricular arrhythmias and poor prognosis [7]. Of note, patients with end-stage HCM are more often male.…”

Prognostic Significance of Non-Dilated Left Ventricular Size and Mitral Regurgitation in Patients With Dilated Phase of Hypertrophic Cardiomyopathy

“…[1][2][3]17,18) Although LV systolic function is supernormal or preserved in most cases of HCM, progression to systolic impairment occurs in about 5-10% of patients when they are followed for a long period. [4][5][6][7][8][9] It is usually associated with LV remodeling with wall thinning and cavity dilatation, resembling the morphologic features of dilated cardiomyopathy (DCM). [4][5][6][7][8][9] Although this subtype of HCM, so-called D-HCM, has been reported to have a poor prognosis, some patients with D-HCM survive for a relatively long period in the current era of advanced arrhythmia and heart failure management.…”

“…[4][5][6][7][8][9] It is usually associated with LV remodeling with wall thinning and cavity dilatation, resembling the morphologic features of dilated cardiomyopathy (DCM). [4][5][6][7][8][9] Although this subtype of HCM, so-called D-HCM, has been reported to have a poor prognosis, some patients with D-HCM survive for a relatively long period in the current era of advanced arrhythmia and heart failure management. 2,11) In the present study, less LV enlargement at diagnosis of D-HCM and presence of significant MR during follow-up were associated with markedly worse clinical outcome, although cardiovascular mortality was originally poor in the whole cohort of D-HCM.…”

“…[1][2][3] On the other hand, it is well known that HCM in a subset of patients progresses to ''dilated'' or ''end-stage'' phase characterized by left ventricular (LV) systolic dysfunction, and patients with dilated phase of HCM (D-HCM) have a poor prognosis. [4][5][6][7][8][9][10] However, various treatments such as the use of an implantable cardioverter-defibrillator (ICD) for prevention of sudden cardiac death and several medications and devices for prevention of heart failure are now available even for patients with D-HCM. 2,11) In patients with LV systolic dysfunction, the degree of LV dilatation associated with LV remodeling is generally thought to be one of the predictors of poor prognosis.…”

Dilated Phase of Hypertrophic Cardiomyopathy Caused by Two Different Sarcomere Mutations, Treated with Surgical Left Ventricular Reconstruction and Cardiac Resynchronization Therapy with a Defibrillator

“…It was reported that CRT might be useful in a subset of D-HCM patients with wide QRS prolongation and LV dyssynchrony (30)(31)(32). Sustained or non-sustained ventricular tachycardia is frequently seen in D-HCM patients, and sudden death often occurs in the presence of severe heart failure (33). Several reports have noted that the observation of late gadolinium enhancement on MRI, presumed to represent fibrosis and replacement scarring, was probably largely responsible for the malignant ventricular tachyarrhythmias observed in these patients (34)(35)(36).…”