Pulmonary artery intimal sarcoma is a rare disorder arising from the intima of the pulmonary artery. Histopathology reveals that it is a tumour cell of mesenchymal origin. The signs and symptoms include chronic shortness of breath and other features of right ventricular failure, which mimic chronic pulmonary thromboembolism. The definitive diagnosis can rarely be made based on the symptoms and signs alone, and other investigations including echocardiography, computed tomography, magnetic resonance imaging (MRI), and positron emission tomography (PET) are often required. The gold standard for diagnosis is tissue biopsy. The mainstay for treatment is surgery, and complete surgical resection with endarterectomy provides survival benefit. According to recent evidences, however, multimodal treatment provides better survival outcomes than monotherapy such as surgery alone. Despite the newer upcoming treatment strategies, patients with pulmonary intimal sarcoma continue to have a poor prognosis. We present a case of pulmonary artery intimal sarcoma and review the literature associated with the disease.

Pulmonary arterial hypertension (PAH) is characterized by muscularized pulmonary blood vessels, leading to right heart hypertrophy and cardiac failure. However, state-of-the-art therapeutics fail to target the ongoing remodeling process. Here, this study shows that matrix metalloproteinases (MMP)-1 and MMP-10 levels are increased in the medial layer of vessel wall, serum, and M1-polarized macrophages from patients with PAH and the lungs of monocrotaline-and hypoxia-induced PAH rodent models. MMP-10 regulates the malignant phenotype of pulmonary artery smooth muscle cells (PASMCs). The overexpression of active MMP-10 promotes PASMC proliferation and migration via upregulation of cyclin D1 and proliferating cell nuclear antigen, suggesting that MMP-10 produced by infiltrating macrophages contributes to vascular remodeling. Furthermore, inhibition of STAT1 inhibits hypoxia-induced MMP-10 but not MMP-1 expression in M1-polarized macrophages from patients with PAH. In conclusion, circulating MMP-10 could be used as a potential targeted therapy for PAH.

A B S T R A C TObjectives: Although the association between systemic infection and cardiovascular events has been identified, uncertainty remains regarding the incidence and prognosis of sepsis in acute myocardial infarction (AMI). The purpose of this research was to assess the impact of sepsis on survival after first AMI. Methods: This was a nationwide cohort study involving the analysis of data from the Taiwan National Health Insurance Research Database for the period 2000-2012, for patients with a primary diagnosis of first AMI. Among the 186 112 prospective patients, sepsis was diagnosed in 13 065 (7.0%). The propensity score matching technique was used to match 13 065 controls to the patients with sepsis and AMI with similar baseline characteristics. Cox proportional hazards regression models, including sepsis, percutaneous coronary intervention (PCI), and comorbidities, were performed to further evaluate the different influences on the mortality risk in patients hospitalized for first AMI. Results: Overall, the 12-year survival rate was lower in AMI patients with sepsis than in those without sepsis (log rank p-value <0.001); this was also shown in the different age and sex groups. The AMI patients with sepsis had a longer length of hospital stay than those without sepsis (32.5 days vs. 11.74 days, p < 0.001). In the Cox proportional hazards regression analysis, sepsis was an independent risk factor for mortality in patients after AMI (hazard ratio 1.78; 95% confidence interval 1.72-1.83). Interventional management with PCI or coronary artery bypass grafting improved survival in both the sepsis and non-sepsis patients after first AMI. Conclusions: In conclusion, sepsis significantly increased the mortality risk of patients after first AMI. PCI may improve the long-term survival of patients in comparison to those managed conservatively.

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