Microvascular Cardiomyopathy: A Distinct Driver Of RV Dysfunction In CTD-PAH (HFSA 2026) - Oct 7, 2026 - Abstract #6; Pres time: Oct 9, 2026; 05:12 PM - 05:22 PM; Location: 129 AB - PCC North; "Lymph node biopsy excluded malignancy, and high-dose corticosteroids and hydroxychloroquine were initiated...She was initiated on high-dose corticosteroids with subsequent pulse-dose methylprednisolone for ongoing inflammatory activity, followed by mycophenolate mofetil and rituximab for disease control...She was initially treated with phosphodiesterase-5 inhibition (sildenafil, later transitioned to tadalafil) and endothelin receptor antagonist therapy (ambrisentan), later discontinued due to volume overload. With improved cardiac output, dobutamine was weaned and selexipag initiated. Worsening digital ischemia prompted transition to IV treprostinil for microvascular disease, later de-escalated with resumption of selexipag... In SLE/MCTD, severe RV failure may reflect complement-mediated microvascular cardiomyopathy rather than fixed pulmonary vascular disease. Prioritizing early immunosuppression, alongside carefully tailored pulmonary vasodilator therapy, can lead..."
Cardiomyopathy • Cardiovascular • Immunology • Inflammatory Arthritis • Lupus • Musculoskeletal Pain • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Systemic Lupus Erythematosus • Vasculitis
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Reema Bhatt; Mohil Garg; Danielle Howell; David Kim; Jennifer McLeod; Ayan Purkayastha; Ningxin Wan
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