Clinicopathological profiles of progressive heart failure in hypertrophic cardiomyopathy

Abstract

AimsHypertrophic cardiomyopathy (HCM) is an important cause of heart failure-related disability over a wide range of ages. Profiles of severe progressive heart failure symptoms and death, or heart transplantation deserve more complete definition within large patient cohorts.Methods and resultsClinical and morphological features of heart failure were assessed in 293 consecutive HCM patients over a median follow-up of 6 (inter-quartile range 2–11) years. Gross and histopathological features were analysed in 12 patients for whom the heart was available for inspection. Of the 293 patients, 50 (17%) developed severe progressive heart failure, including 18 who died or were transplanted. Three profiles of heart failure were identified predominantly associated with: (i) end-stage systolic dysfunction (ejection fraction <50%) (15; 30%); (ii) left ventricular (LV) outflow obstruction at rest (11; 22%); and (iii) non-obstructive with preserved systolic function (24; 48%). Overall, atrial fibrillation (AF) contributed to heart failure in 32 patients (64%) among the three profiles. Compared with other patients, those non-obstructive with preserved systolic function had earlier onset of heart failure symptoms mainly due to diastolic dysfunction, and the most accelerated progression to advanced heart failure and adverse outcome (P = 0.04). Thrombi were identified in the left atrial appendage of five gross heart specimens all belonging to patients with AF, including three of which were unrecognized clinically and had previously embolized. Extensive myocardial scarring with LV remodelling was evident in all end-stage patients; no or only focal scars were present in other patients.ConclusionProfiles of advanced heart failure in HCM are due to diverse pathophysiological mechanisms, including LV outflow obstruction and diastolic or global systolic ventricular dysfunction. Atrial fibrillation proved to be the most common disease variable associated with progressive heart failure. Recognition of the heterogeneous pathophysiology of heart failure in HCM is relevant, given the targeted management strategies necessary in this disease.

Relationships between P wave dispersion, atrial electromechanical delay, left atrial remodeling, and NT-proBNP levels, in patients with hypertrophic cardiomyopathy

“…These parameters and LA phasic functions have been used widely to predict development of atrial fibrillation (AF) episodes in patients with various heart diseases [5][6][7]. Patients with HCM are more prone to development of AF, which is suggested to be a strong determinant of HCM-related morbidity and is the most common disease variable associated with progressive heart failure [8].…”

Clinical variants and outcomes of progressive chronic heart failure in individuals with hypertrphic cardiomyopathy

“…Несмотря на разли-чия ЭхоКГ характеристик, исходы у всех неблагоприятны. Эти результаты подчеркивают необходимость активного контроля развития заболевания у пациентов с необструк-тивной ГКМП даже при наличии сохраненной систоли-ческой функции ЛЖ [16]. Возможность принятия реше-ния о необходимости ТС после возникновения симпто-мов выраженной ХСН может быть непродолжительной.…”

“…В нашей когорте пациентов с ГКМП при длительном наблюдении таких пациентов было 6,1 %. По данным предыдущих исследований, такая форма встречается у 15-20 % пациентов с ГКМП [14,16,18]. При опреде-лении этой стадии прогрессирования заболевания каж-дая из характеристик, таких как наличие ФВ ЛЖ в низ-ком или нормальном диапазоне [14], изменение диасто-лической функции от умеренной до выраженной [19], выраженная дилатация ЛП [17], увеличение области отсроченного конрастирования гадолинием [19], истон-чение стенки ЛЖ [13], эпизоды ФП [20,21], спонтан-ное снижение или утрата градиента выходного тракта ОРИГИНАЛЬНЫЕ СТАТЬИ § ЛЖ [22], описывались отдельно в когортах пациентов с ГКМП.…”

Biphasic Force-Frequency Relation Predicts Primary Cardiac Events in Patients With Hypertrophic Cardiomyopathy

“…Lastly, patients with atrial fibrillation were excluded and this condition has been reported as a RF for worsening heart failure and SCD among patients with HCM. 31 Because right atrial pacing via cardiac catheterization is necessary to analyze the FFR, our study was forced to exclude patients with HCM with atrial fibrillation because it is refractory to pacing.…”