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Although idiopathic pulmonary arterial hypertension is perceived as a progressive disease with a uniformly poor outcome, the natural history of disease is heterogeneous, with some patients dying within months of diagnosis and others living for decades. The course of the disease has also been altered by advances in medical therapies. The outcome of patients with other types of pulmonary arterial hypertension (PAH) has been less well characterized. Assessment of prognosis of such patients is important, as it influences both medical therapy and referral for transplantation. This chapter will provide evidence based recommendations to assess the prognosis of patients with PAH.

There is an increased awareness and recognition of pulmonary hypertensive disorders over the last 2 decades. This has paralleled the enhanced understanding of the pathogenesis and pathophysiology of these processes. Particularly, the genetic abnormalities in patients with familial primary pulmonary hypertension and primary pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia have been described. Fortunately, some of the improved knowledge has furthered treatment options and other novel therapies. Despite these new developments, there remains a significant need for improved treatment options for these patients; the survival can still be as short as 6 months in patients with World Health Organization (WHO)/New York Heart Association Class IV symptoms.

Pulmonary hypertension (PH) often complicates the course of patients with advanced lung disease, and it is associated with a worse prognosis. Per the recent classification of pulmonary hypertensive disorders, PH due to lung disease is considered as a separate category within a group of disorders that was previously referred to as “secondary” PH. Among the lung diseases associated with PH, the incidence and clinical course of PH is best known for patients with COPD. Per studies in patients with COPD and other lung disorders, it is evident that the pathophysiology and treatment of these disorders is generally distinct from that of pulmonary arterialhypertensive disorders. Changes in the pulmonary vasculature that accompany elevations in pulmonary vascular pressure are generally referred to as pulmonary vascular remodeling.Chronic hypoxia is well known to cause pulmonary vascular remodeling and PH, and it is the major mechanism implicated for the development of PH in patients with lung disease. Other mediators have also been implicated in the pathogenesis of PH in animal models and patients with PH, including patients with pulmonary diseases. General features of pulmonary vascular remodeling are discussed with particular emphasis on those changes that have been described in patients with lung diseases. Recent discoveries in these areas are also reviewed, and findings in pulmonary arterial hypertensive diseases are contrasted with those found in patients with PH due to lung diseases. Some of these discoveries have already led to new treatment strategies for patients with the most severe forms of PH. PH due to lung diseases shares some common pathophysiologic features with pulmonary arterial hypertension. Therefore, it is likely that these discoveries and new treatments will also be extended to benefit patients with PH due to lung disease.

Compared to idiopathic pulmonary arterial hypertension (IPAH), patients with portopulmonary hypertension (POPH) have worse survival. Health disparities may contribute to these differences but have not been studied. We sought to compare socioeconomic factors in patients with POPH and IPAH and to determine whether socioeconomic status and/or POPH diagnosis were associated with treatment and health-care utilization. We performed a cross-sectional study of adults enrolled in the Pulmonary Hypertension Association Registry. Patients with IPAH (n = 344) and POPH (n = 57) were compared. Compared with IPAH, patients with POPH were less likely to be college graduates (19.6% vs. 34.9%, p = 0.02) and more likely to be unemployed (54.7% vs. 30.5%, p < 0.001) and have an annual household income below poverty level (45.7% vs. 19.0%, p < 0.001). Patients with POPH had similar functional class, quality of life, 6-min walk distance, and mean pulmonary arterial pressure with a higher cardiac index. Compared with IPAH, patients with POPH were less likely to receive combination therapy (46.4% vs. 62.2%, p = 0.03) and endothelin receptor antagonists (28.6% vs. 55.1%, p < 0.001) at enrollment with similar treatment at follow-up. Patients with POPH had more emergency department visits (1.7 ± 2.1 vs. 0.9 ± 1.2, p = 0.009) and hospitalizations in the six months preceding enrollment (1.5 ± 2.1 vs. 0.8 ± 1.1, p = 0.02). Both POPH diagnosis and lower education level were independently associated with a higher number of emergency department visits. Compared to IPAH, patients with POPH have lower socioeconomic status, are less likely to receive initial combination therapy and endothelin receptor antagonists but have similar treatment at follow-up, and have increased health-care utilization.

To determine the utility of CT-determined main pulmonary artery diameter (MPAD) for predicting pulmonary hypertension (PH) in patients with parenchymal lung disease.

Pneumocystis jiroveciipneumonia (PJP) is an opportunistic pathogen known to occur exclusively in immunocompromised hosts. To our knowledge, among immunocompetent hosts, a total of 13 cases have been reported in the literature. We present a case of a nonimmunocompromised woman presenting with hypoxemic respiratory failure found to have PJP pneumonia. The pathogenesis of PJP in immunocompetent patients is not known. The most common symptoms reported in the immunocompetent population include fever and shortness of breath. Immunocompetent patients with PJP have a shorter duration of illness, a lower partial pressure of oxygen tension (PaO2), increased incidence of pleural effusion, and increased mortality. Cotrimoxazole remains the treatment of choice, but the duration of therapy and whether to add corticosteroids is unknown. Although rare in immunocompetent patients, in those presenting with compatible symptoms/signs and radiographic findings, an evaluation for PJP should be considered.