The management of pulmonary arterial hypertension (PAH) has significantly evolved over the last decades in the wake of more sensitive diagnostics and specialized clinical programs that can provide focused medical care. In the current era of PAH care, 1-year survival rates have increased to 86%–90% from 65% in the 1980s, and average long-term survival has increased to 6 years from 2.8 years. The heterogeneity in the etiology and disease course has opened doors to focusing research in phenotyping the disease and understanding the pathophysiology at a cellular and genetic level. This may eventually lead to precision medicine and the development of medications that may prevent or reverse pulmonary vascular remodeling. With more insight, clinical trial designs and primary end-points may change to identify the true survival benefit of pharmacotherapy. Identifying responders from non-responders to therapy may help provide individualized patient-centered care rather than an algorithm-based approach. The purpose of this review is to highlight the latest advances in screening, diagnosis, and management of PAH.

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This article is one of ten reviews selected from the Annual Update in Intensive Care and Emergency Medicine 2021. Other selected articles can be found online at https:// www. biome dcent ral. com/ colle ctions/ annua lupda te2021. Further information about the Annual Update in Intensive Care and Emergency Medicine is available from https:// link. sprin ger. com/ books eries/ 8901.

Life-threatening hemoptysis (LTH) is any amount of hemoptysis that causes significant hemodynamic decompensation or respiratory distress which may lead to death if left untreated. While the amount of hemoptysis that qualifies as massive hemoptysis has continued to be debated, any amount between 100 to 1,000 mL/day is considered significant. Up to 15% cases of hemoptysis are LTH and need urgent life-saving intervention. Understanding of pulmonary vascular anatomy is of paramount importance to manage LTH. The goal of treatment lies in airway protection, appropriate oxygenation, and prevention of exsanguination. Once the airway is stabilized, a quick diagnosis and control of bleeding site is targeted. This chapter highlights current practices and approach to LTH including medical management, bronchoscopic approach, and advanced therapies such as bronchial artery embolization and surgical resection. We review situations, such as bronchiectasis, vascular malformation, diffuse alveolar hemorrhage, and tracheostomy bleed and specific approach to management of these conditions in a systematic and evidence-based manner.

Fibrosing mediastinitis (FM) is a rare disorder of inflammation and fibrosis involving the mediastinum. The formation of fibroinflammatory mass in the mediastinum can lead to obstruction of mediastinal structures and cause severe debilitating and life-threatening symptoms. Superior vena cava syndrome (SVCS) is a dreaded complication of FM with no medical therapy proven to be efficacious. Spiral vein grafting has long been utilized as first-line therapy for SVC syndrome due to FM. Endovascular repair with stents and angioplasty for malignant causes of SVC syndrome is well established. However, there are limited data on their utility in SVC syndrome due to FM. We present two cases of SVC syndrome due to FM treated with endovascular stenting and a detailed review of current literature on its utility in SVCS due to benign causes.

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