Right Ventricular Myofilament Functional Differences in Humans With Systemic Sclerosis–Associated Versus Idiopathic Pulmonary Arterial Hypertension | Zendy

Steven Hsu | Zendy; Kristen M. Kokkonen-Simon | Zendy; Jonathan A. Kirk | Zendy; Todd M. Kolb | Zendy; Rachel L. Damico | Zendy; Stephen C. Mathai | Zendy; Monica Mukherjee | Zendy; Ami A. Shah | Zendy; Fredrick M. Wigley | Zendy; Kenneth B. Margulies | Zendy; Paul M. Hassoun | Zendy; Marc K. Halushka | Zendy; Ryan J. Tedford | Zendy; David A. Kass | Zendy

Open Access

Right Ventricular Myofilament Functional Differences in Humans With Systemic Sclerosis–Associated Versus Idiopathic Pulmonary Arterial Hypertension

Author(s) -

Steven Hsu,

Kristen M. Kokkonen-Simon,

Jonathan A. Kirk,

Todd M. Kolb,

Rachel L. Damico,

Stephen C. Mathai,

Monica Mukherjee,

Ami A. Shah,

Fredrick M. Wigley,

Kenneth B. Margulies,

Paul M. Hassoun,

Marc K. Halushka,

Ryan J. Tedford,

David A. Kass

Publication year - 2018

Publication title -

circulation

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 7.795

H-Index - 607

eISSN - 1524-4539

pISSN - 0009-7322

DOI - 10.1161/circulationaha.117.033147

Subject(s) - medicine , contractility , cardiology , pulmonary hypertension , myofilament , myocyte , pulmonary artery , fibrosis , systemic scleroderma , heart failure , dermatomyositis

Patients with systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH) have a far worse prognosis than those with idiopathic PAH (IPAH). In the intact heart, SSc-PAH exhibits depressed rest and reserve right ventricular (RV) contractility compared with IPAH. We tested whether this disparity involves underlying differences in myofilament function.

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