Aims Cardiac disease in systemic sclerosis (SSc) may be primary or secondary to other disease manifestations of SSc. The prevalence of the primary cardiomyopathy of SSc is unknown. Cardiovascular magnetic resonance imaging (CMR) can help accurately determine the presence and cause of cardiomyopathy. We aimed to investigate the prevalence, the CMR features, and the prognostic implications of the primary cardiomyopathy of SSc. Methods and Results We conducted a retrospective cohort study of consecutive patients with SSc who had a clinical CMR for suspected cardiac involvement. We identified the prevalence, the CMR features of the primary cardiomyopathy of SSc, and its association with the long-term incidence of death or major adverse cardiac events (MACE): heart failure hospitalization, ventricular assist device implantation, heart transplantation, and sustained ventricular tachycardia. Of 130 patients with SSc, 80% were women, and the median age was 58 years. On CMR, 22% had an abnormal left ventricular ejection fraction (LVEF), and 40% had late gadolinium enhancement (LGE). The prevalence of the primary cardiomyopathy of SSc was 21%. A third of these patients had a distinct LGE phenotype. Over a median follow-up of 3.6 years after the CMR, patients with the primary cardiomyopathy of SSc had a greater incidence of death or MACE (adjusted hazard ratio 2.01; 95% confidence interval 1.03-3.92; p=0.041). Conclusion The prevalence of the primary cardiomyopathy of SSc was 21%, with a third demonstrating a distinct LGE phenotype. The primary cardiomyopathy of SSc was independently associated with a greater long-term incidence of death or MACE.

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In patients with CTEPH who are not eligible for PTE, BPA may be a treatment option. It is important to diagnose and treat patients early since delays are associated with worse clinical outcomes. We present a case of CTEPH where early diagnosis and treatment resulted in normalization of PA pressures. CAse RePoRt Check for updates Learning Objectives• To realize the importance of early diagnosis in CTE-PH given the association with better outcomes.• To highlight that early diagnosis of CTEPH can be challenging.• To recognize BPA as a treatment option for patients not eligible for PTE. Case PresentationA 35-year-old male with a history of hereditary spherocytosis with splenectomy fifteen years prior, presented to cardiology clinic for evaluation of patent foramen ovale (PFO), right ventricular enlargement and elevated right-sided pressures observed on echocardiogram. Two months prior, he experienced an acute ischemic stroke, and during the work-up for his CVA an echocardiogram revealed severe pulmonary hypertension with a right ventricular systolic pressure (RVSP) of 80 mmHg. His right ventricle (RV) was dilated, had reduced systolic function, and there was intraventricular septal flattening during systole and diastole consistent with pressure and volume overload. The study also revealed a patent PFO. Transesophageal echocardiography later confirmed the presence of a moderate-sized PFO, which was successfully closed percutaneously with a 25 mm Gore Cardioform device. Right heart catheterization (RHC) performed at the time of PFO closure showed mean pulmonary artery pressure (mPAP) of 39

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