Prognostic Implications of the Doppler Restrictive Filling Pattern in Hypertrophic Cardiomyopathy

Biagini, Elena; Spirito, Paolo; Rocchi, Guido; Ferlito, Marinella; Rosmini, Stefania; Lai, Francesco; Lorenzini, Massimiliano; Terzi, Francesca; Reggiani, Maria Letizia Bacchi; Boriani, Giuseppe; Branzi, Angelo; Boni, Luca; Rapezzi, Claudio

doi:10.1016/j.amjcard.2009.07.057

Cited by 100 publications

(75 citation statements)

References 17 publications

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“…More than three-quarters of HCM patients in crosssectional studies belong to this stage (Figure 1). [23][24][25] The distribution of LV hypertrophy is typically regional and asymmetrical, generally involving the basal septum and anterior wall, but can develop in all imaginable patterns within the LV and involve the right ventricle and papillary muscles 3,7,10 ( Figure 3 and Table). Besides cardiac hypertrophy, the HCM phenotype includes a constellation of mitral valve and subvalvar abnormalities, subaortic, midventricular and right ventricular outflow obstruction, atrial remodeling, coronary myocardial bridging, crypts, and autonomic nervous system abnormalities.…”

mentioning

confidence: 99%

Patterns of Disease Progression in Hypertrophic Cardiomyopathy

Olivotto

Cecchi²,

Poggesi³

et al. 2012

Circ: Heart Failure

288

226

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A fter the recent celebrations of the 50 th anniversary of the modern description of hypertrophic cardiomyopathy (HCM) by Teare and Lord Brock, the time is ripe to reflect on what remains to be discovered. [1][2][3] With the full realization that a massive amount of information relating to the disease has already been uncovered, and paying tribute to all those involved in this process, it is essential to concentrate on the gaps in our knowledge that require concerted efforts to advance the field, particularly in relation to patient management, which continues to be perceived as less than optimal. 3 We believe that this is largely due to the partial disconnect between basic research, and an incomplete understanding of the fundamental mechanisms molding a continuously, often insidiously changing phenotype. A thorough comprehension of these processes requires a translational approach based on long-term clinical observation of large HCM cohorts, coupled with basic scientific research, and represents an essential step toward the development of innovative therapies which need to be both disease-and patient-specific. 2,3Traditionally, the focus of HCM literature has been polarized on 2 aspects of indisputable clinical relevance: the pathogenesis, clinical consequences, and management of dynamic left ventricular (LV) outflow obstruction, 1 and the issue of arrhythmic risk stratification and prevention of sudden cardiac death (SCD).4,5 By comparison, limited attention has been devoted to the life-long process of LV remodeling and progressive dysfunction that occur in a substantial proportion of HCM patients and culminates in the rare but dramatic clinical evolution termed as end-stage or burned-out phase.6-9 Consequently, the stages that precede this severe condition are still relatively unknown, representing an important target for research.3 Indeed, because of the slowly evolving nature of HCM, timely identification of patients at risk of developing advanced LV dysfunction and heart failure (HF) may allow effective preventive strategies over a time span of several years before clinical demise. [7][8][9] To aid the characterization of different phases of HCM in individual patients, we propose a simple framework for systematic clinical staging of the disease. To this purpose, 4 clinical stages are identified, with special emphasis on diagnosis, potential mechanisms, challenges for management, and targets for future investigation: these are defined as nonhypertrophic HCM, classic phenotype, adverse remodeling, and overt dysfunction (Figure 1 and Table). 3,6,7,10 Stage I: Nonhypertrophic HCM Definition and DiagnosisNonhypertrophic HCM is a state characterized by the absence of LV hypertrophy in individuals harboring HCM-causing mutations, investigated in the course of systematic family screenings. In most HCM patients, a hypertrophic phenotype is generally absent in newborn or very young children, and tends to manifest during the second decade of life.7,10,11 Due to incomplete penetrance and age-related onset, however, gen...

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mentioning

confidence: 99%

Patterns of Disease Progression in Hypertrophic Cardiomyopathy

Olivotto

Cecchi²,

Poggesi³

et al. 2012

Circ: Heart Failure

288

226

View full text Add to dashboard Cite

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“…On the other hand, there is another proposed LV remodeling in HCM, which may eventually lead to a "restrictive-stage'' phase with a small LV chamber, progression of biatrial dilatation, markedly restrictive diastolic filling, and poor outcome. [24][25][26] The D-HCM patients with non-dilated LV size in our study may be characterized partly by the restrictive type. Recently Wada, et al reported that the severity of fibrosis in myocardial biopsy and lower LV ejection fraction were associated with a greater risk of lethal arrhythmic events in HCM patients.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2017

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SummaryAlthough a subtype of hypertrophic cardiomyopathy (HCM), dilated phase of HCM (D-HCM) characterized by left ventricular (LV) systolic dysfunction, has been reported to have a poor prognosis, some patients with D-HCM survive for a relatively long period. The degree of LV dilatation and functional mitral regurgitation (MR) are generally thought to be important predictors of poor prognosis in patients with LV systolic dysfunction. However, there is little information available on the relations among LV size, presence of significant MR, and prognosis in D-HCM patients.We retrospectively studied 31 patients with D-HCM to determine whether echocardiographic assessment of LV size and MR provides incremental prognostic information.During a follow-up period of 5.6 ± 4.2 years, there were 13 cardiovascular deaths. When the patients were divided into two groups by LV size at diagnosis of D-HCM, a non-dilated LV group (LV end-diastolic diameter (LVEDD) < 50 mm, n = 9) and a dilated LV group (LVEDD ≥ 50 mm, n = 22), the clinical course in the non-dilated LV group was significantly worse. As for the clinical impact of MR, no patient in the non-dilated LV group showed significant MR and 7 of the patients with dilated LV size showed significant MR during follow-up. Once significant MR was reached, cardiovascular deaths were significantly more frequent in patients with MR.Patients with D-HCM, particularly those with less LV dilatation at diagnosis of dilated phase and with significant MR during follow-up, have a poor prognosis. (Int Heart J 2017; 58: 63-68)

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“…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. Однако эти характеристики имеют тенденцию «собираться или скапливаться» у отдельных индивиду-умов, являясь различными аспектами прогрессирования заболевания у одной и той же подгруппы пациентов.…”

Section: Discussionunclassified