Microvascular Function Is Selectively Impaired in Patients With Hypertrophic Cardiomyopathy and Sarcomere Myofilament Gene Mutations

Olivotto, Iacopo; Girolami, Francesca; Sciagrà, Roberto; Ackerman, Michael J.; Sotgia, Barbara; Bos, J. Martijn; Nistri, Stefano; Sgalambro, A; Grifoni, Camilla; Torricelli, Francesca; Camici, Paolo G.; Cecchi, Fabiola

doi:10.1016/j.jacc.2011.05.018

Cited by 150 publications

(104 citation statements)

References 44 publications

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“…These structural changes, in addition to the increased extravascular compression, cause severe CMD that can be demonstrated by a severely blunted maximal MBF and reduced CFR. 44, 45 The anatomical basis of CMD is supported by the…”

Section: Cmd In Primary Lvhmentioning

confidence: 99%

From Left Ventricular Hypertrophy to Dysfunction and Failure

Lazzeroni

Rimoldi

Camici

2016

Circ J

124

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Left ventricular hypertrophy (LVH) is growth in left ventricular mass caused by increased cardiomyocyte size. LVH can be a physiological adaptation to strenuous physical exercise, as in athletes, or it can be a pathological condition, which is either genetic or secondary to LV overload. Physiological LVH is usually benign and regresses upon reduction/cessation of physical activity. Pathological LVH is a compensatory phenomenon, which eventually may become maladaptive and evolve towards progressive LV dysfunction and heart failure (HF). Both interstitial and replacement fibrosis play a major role in the progressive decompensation of the hypertrophied LV. Coronary microvascular dysfunction (CMD) and myocardial ischemia, which have been demonstrated in most forms of pathological LVH, have an important pathogenetic role in the formation of replacement fibrosis and both contribute to the evolution towards LV dysfunction and HF. Noninvasive imaging allows detection of myocardial fibrosis and CMD, thus providing unique information for the stratification of patients with LVH. DCM is generally caused by viral/bacterial myocarditis, exposure to cardiotoxic substances or is a manifestation of a systemic disease. Before cell death and fibrotic repair occur, multiple factors contribute to contractile dysfunction, including abnormalities in sarcolemmal integrity, energy production, calcium handling and force transmission. 10 Both systolic and diastolic dysfunction worsen with time and EF is progressively reduced, with symptoms and signs of HF. Secondary LVHCardiomyocyte hypertrophy is the primary mechanism by which the heart counteracts a protracted increase in stress (or load) on the ventricular wall. 11 According to Laplace's law, 12 wall stress is directly related to LV cavity size and intracavitary pressure and inversely related to wall thickness. Thus, wall thickening will counteract, at least in part, the increased stress and oxygen demand caused by pressure or volume overload, although, in the long run, this compensatory mechanism may become maladaptive. On the other hand, several other nonhemodynamic factors, including activation of the adrenergic and renin-angiotensin-aldosterone (RAAS) systems, release of growth factors and cytokines, contribute to the development of LVH. 13,14 Pressure Overload In most cases, pressure overload manifests as arterial hypertension (AH) or aortic stenosis (AS).Hypertensive Heart The most common condition associated with an increase in cardiac afterload and wall stress is AH. 15 Approximately 20-60% of patients with uncomplicated AH have evidence of increased LVM on echocardiography. 16 LVM can increase from wall thickening, chamber dilatation or both. Wall thickening occurs more commonly in response to pressure overload whereas chamber dilation occurs more commonly in response to volume overload. Although LV wall thickness is more closely related to systemic diastolic blood pressure (concentric hypertrophy), reflecting pure pressure load caused by increased systemic vascular resistance...

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Section: Cmd In Primary Lvhmentioning

confidence: 99%

From Left Ventricular Hypertrophy to Dysfunction and Failure

Lazzeroni

Rimoldi

Camici

2016

Circ J

124

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“…Microvascular ischemia, which is most pronounced in-but not confined to-the hypertrophied regions, is important in the pathogenesis of disease progression and LV dysfunction and is a likely substrate for ventricular arrhythmias (22,73,74). Of note, microvascular dysfunction is more pronounced in gene-positive HCM patients compared with those without gene mutations (75), suggesting a molecular link between sarcomere mutations and microvascular remodeling (76).…”

Section: Microvascular Ischemia Myocardial Fibrosis and Disease Promentioning

confidence: 99%

MR Imaging in Hypertrophic Cardiomyopathy: From Magnet to Bedside

Bogaert¹,

Olivotto²

2014

Radiology

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“…Użycie głowicy przezprzełykowej z kolei ułatwia ciągły udział w procedurze i zapewnia dobrą jakość badania, ale wymaga zastosowania znieczulenia ogólnego. Pomiaru gradientu w LVOT można dokonać z projekcji przezżołądkowej koniuszkowej czterojamowej (0 o ) oraz środkowoprzełykowej w osi długiej na poziomie zastawki aortalnej (120)(121)(122)(123)(124)(125)(126)(127)(128)(129)(130) o ). Należy pamiętać, że zmierzony gradient w LVOT może być zaniżony z powodu sedacji ogólnej.…”

Section: Powiększenie Lewego Przedsionkaunclassified

“…Ocena niedokrwienia, zazwyczaj indukowanego wysiłkiem, jest istotna u pacjentów z HCM ze względu na jego znaczenie prognostyczne (związek z potencjalnie letalnymi arytmiami i niekorzystną przebudową serca: rozstrzeń, jawna dysfunkcja skurczowa [121,126]. Metody radioizotopowe, takie jak SPECT (Thallium-201, 99mTc) czy PET (N-amoniak) z powodzeniem są wykorzystywane w ocenie zaburzeń perfuzji mięśnia sercowego wywołanych wysiłkiem czy wazodylatacją (dipiridamol, adenozyna) [122,123,127,128]. Można w ten sposób zobrazować nie tylko odwracalne ogniska niedokrwienia, ale też trwałe zmiany, świadczące o obecności blizny, porównywalne z obszarami zwłóknienia w CMR [128,129].…”

Section: Obrazowanie Radioizotopoweunclassified