Myocardial Damages in Systemic Sclerosis Detected by Gated Myocardial Perfusion SPECT and Sympathetic Imaging

Nakajima, Kénichi; Kawano, Masaya; Hasegawa, Minoru; Taki, Junichi; Fujimoto, Manabu; Takehara, Kazuhiko; Tonami, Norihisa

doi:10.1253/circj.70.1481

Cited by 20 publications

(16 citation statements)

References 21 publications

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“…However, one study found an independent inverse association between the myocardial systolic velocity obtained with tissue Doppler at tricuspid lateral annulus and PAP, indicating that the levels of PAP are deter- minative in the development of right ventricular systolic function [6]. Diastolic dysfunction can be altered very early in the course of the disease [7]. Strain and strain rate analyze regional functions, and not the global systolic function of the right ventricle.…”

Section: Discussionmentioning

confidence: 99%

“…These data do not agree with other studies performed in patients with systemic sclerosis. However, the majority of the studies included symptomatic patients and/or with pulmonary hypertension; one study that had included patients with a mean disease duration of 11.8 AE 8.7 years and used conventional and tissue Doppler echocardiography, found, in comparison with controls, no alterations in left ventricular function (systolic and diastolic) or in right ventricular systolic function, but they demonstrated abnormal right ventricular diastolic function [7]. However, one study [22] had demonstrated abnormal systolic right ventricular function in patients with systemic sclerosis and both normal PAP, early manifestations of the disease (<5 years of evolution) or cutaneous subtypes.…”

Section: Ss Controlmentioning

confidence: 99%

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Speckle-tracking-derived strain and strain-rate analysis: a technique for the evaluation of early alterations in right ventricle systolic function in patients with systemic sclerosis and normal pulmonary artery pressure

Matias

Isla²,

Vasconcelos³

et al. 2009

Journal of Cardiovascular Medicine

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Section: Discussionmentioning

confidence: 99%

Section: Ss Controlmentioning

confidence: 99%

Speckle-tracking-derived strain and strain-rate analysis: a technique for the evaluation of early alterations in right ventricle systolic function in patients with systemic sclerosis and normal pulmonary artery pressure

Matias

Isla²,

Vasconcelos³

et al. 2009

Journal of Cardiovascular Medicine

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“…It is well known that myocardial RP provokes silent episodes of coronary vasoconstriction with transient hypoxia 1. In our patient, the septic state with reduction of haemoglobin together with the possible activation of the sympathetic system9 10 might have triggered coronary vasoconstriction leading to myocardial ischaemia. This may have favoured ballooning, depression in T wave on ECG and a mild increase of troponine.…”

mentioning

confidence: 62%

Tako-tsubo-like syndrome in systemic sclerosis: a sign of myocardial Raynaud phenomenon?

Melchiorre¹,

Bernardo²,

Conforti³

et al. 2008

Annals of the Rheumatic Diseases

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“…68,69 Patients with SSc and myocardial perfusion defects have a specific phenotype with diffuse rather than limited scleroderma, positive anti-Scl70 antibody, more extensive and rapid progression of skin involvement, and a trend toward longer disease duration and greater severity. 69,70 One study determined that thallium perfusion defect scoring was the single most powerful predictor of both mortality and subsequent development of clinical cardiac disease in patients with either limited or diffuse scleroderma. 71 Clearly, more is to be learned from these imaging modalities.…”

Section: Nuclear Imagingmentioning

confidence: 99%

The Right Ventricle in Scleroderma (2013 Grover Conference Series)

Hassoun

2015

Pulm. circ.

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Pulmonary arterial hypertension (PAH) results from severe remodeling of the distal lung vessels leading irremediably to death through right ventricular (RV) failure. PAH (Group 1 of the World Health Organization classification of pulmonary hypertension) can be idiopathic (IPAH) or associated with other disorders, such as connective tissue diseases. Prominent among the latter is systemic sclerosis (SSc), a heterogeneous disorder characterized by endothelium dysfunction, dysregulation of fibroblasts resulting in excessive collagen production, and immune abnormalities. For as-yet-unknown reasons, SSc-associated PAH (SSc-PAH) carries a significantly worse prognosis compared with any other form of PAH in Group 1, including IPAH. We have previously shown that patients with SSc-PAH have a median survival of only 3 years, compared with 8 years for IPAH, despite modern PAH therapy. Because death is principally due to RV failure, we speculated that RV adaptation to PAH differed between the two entities due to disparate pulmonary artery loading, perhaps from vessel stiffening, or intrinsic RV myocardial disease that might limit function and adaptation to high afterload. In SSc, RV function may also be impaired by inflammatory processes, excess fibrosis of the myocardium, or altered angiogenesis, which may all contribute to impaired contractile reserve exacerbating cardiopulmonary impedance mismatch. This is now suggested by recent findings from our group that demonstrate that, although pulmonary vascular load may be similar between patients with IPAH and those with SSc-PAH, the latter display reduced myocardial contractility as assessed by pressure-volume loop measurements. This review focuses on fundamental hemodynamic, structural, and functional differences in RV from patients with SSc-PAH compared with IPAH, which may account for survival discrepancies between the two populations. Possible underlying basic mechanisms are discussed.Pulmonary arterial hypertension (PAH) is a leading cause of mortality in patients with systemic sclerosis (SSc), a heterogeneous disorder characterized by dysfunction of the endothelium, dysregulation of fibroblasts resulting in excessive production of collagen, and abnormalities of the immune system. PAH leads almost uniformly to death through right ventricular (RV) failure. However, for reasons that remain unclear, SSc-associated PAH (SSc-PAH) carries a significantly worse prognosis compared with any other form of PAH in Group 1 of the pulmonary hypertension classification. 1 Indeed, we and others have shown that patients with SSc-PAH are four times more likely to die than patients with idiopathic PAH (IPAH) and have a median survival of only 3 years, compared with 8 years for IPAH, despite modern PAH therapy. 2,3 From a broad perspective, a disparate RV adaptation to PAH between these two entities could actually result from a greater load, perhaps due to pulmonary arterial stiffening, as reported in systemic arteries. Alternatively, the RV myocardium could be diseased, limiting function and ...

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Myocardial Damages in Systemic Sclerosis Detected by Gated Myocardial Perfusion SPECT and Sympathetic Imaging

Cited by 20 publications

References 21 publications

Speckle-tracking-derived strain and strain-rate analysis: a technique for the evaluation of early alterations in right ventricle systolic function in patients with systemic sclerosis and normal pulmonary artery pressure

Speckle-tracking-derived strain and strain-rate analysis: a technique for the evaluation of early alterations in right ventricle systolic function in patients with systemic sclerosis and normal pulmonary artery pressure

Tako-tsubo-like syndrome in systemic sclerosis: a sign of myocardial Raynaud phenomenon?

The Right Ventricle in Scleroderma (2013 Grover Conference Series)

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