There is an increase in mortality (2001-2010) associated with PH among men, women, and all race and ethnic groups, and from several conditions commonly associated with PH (hypertension and coronary heart disease, aortic stenosis, liver disease and cirrhosis, and autoimmune disease) but also from renal disease and diabetes, while finding that PH-associated mortality decreased over time from deaths due to congenital malformations among newborns, and for emphysema, chronic lower respiratory disease, and pulmonary embolism.

Approximately one-half of deaths associated with PH occur among those under 75 years of age. Although PH death rates have been stable for those aged 1 to 74 years over the past decade, the identified increases in PH death among those with a UCOD from hypertension, coronary heart disease, and valvular disease may be avoidable with improved public health efforts in the primary prevention of heart disease. The increased mortality from autoimmune conditions in association with PH requires further study. Increases in hospitalizations may reflect both improved recognition of PH as well as an increase in treatment options. The decline in PH mortality due to congenital malformations, chronic lower respiratory disease, and emphysema over time is encouraging.

More in blacks and women who are smoking

After 2003 death rates for women were higher than for men. Death rates throughout the reporting period 1999–2008 were higher for blacks than for whites. Hospitalization rates in women were 1.3–1.6 times higher than in men. Conclusions. Pulmonary hypertension mortality and hospitalization numbers and rates increased from 1999 to 2008.

Explanations for the older age include a change in the population age distribution, in the natural history of the disease itself (e.g., a change in some unrecognized intrinsic or extrinsic factor that delays disease manifestations), improved survival with therapy, or an increased prevalence of chronic lung disease in women due to secular trends in smoking among women. Our findings of excess PH mortality in blacks are generally consistent with the association between race and excess mortality from disease of the circulatory system in the United States

Associated with hereditary, connective tissue diseases, HIV

Familial PAH is now referred to as heritable, with further breakdown into the genetic abnormality identified, if any. Schistosomiasis and chronic hemolytic anemia are now part of category 1 disease as associated conditions to reflect their unique importance as causative factors of PAH. Chronic thromboembolic pulmonary hypertension is no longer divided into proximal and distal, as improvements in surgical technique make this partitioning obsolete. Finally, the miscellaneous category is expanded, and now includes many conditions previously included in the “other” category of associated PAH. The latter change recognizes the complexity and our poor understanding of the link between these conditions and pulmonary hypertension. PAH can be associated with a variety of known diseases, such as connective tissue diseases, portal hypertension, and human immunodeficiency virus (HIV) infection, in addition to the classic idiopathic form (see Box 1).


Box 1: Updated Clinical Classification of Pulmonary Hypertension, Dana Point 2008
1. Pulmonary arterial hypertension (PAH)
  • 1.1. Idiopathic PAH (IPAH)
  • 1.2. Heritable PAH
    • 1.2.1. BMPR2
    • 1.2.2. ALK1, endoglin (with or without hereditary hemorrhagic telangiectasia)
    • 1.2.3. Unknown
  • 1.3. Drug and toxin-induced
  • 1.4. Associated with (APAH)
    • 1.4.1. Connective tissue disease
    • 1.4.2. HIV infection
    • 1.4.3. Portal hypertension
    • 1.4.4. Congenital heart diseases
    • 1.4.5. Schistosomiasis
    • 1.4..6. Chronic hemolytic anemia
  • 1.5. Persistent pulmonary hypertension of the newborn (PPHN)

1′ Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH)

2. Pulmonary hypertension owing to left heart diseases
  • 2.1. Systolic dysfunction
  • 2.2. Diastolic dysfunction
  • 2.3. Valvular disease
3. Pulmonary hypertension owing to lung diseases and/or hypoxia
  • 3.1. Chronic obstructive pulmonary disease
  • 3.2. Interstitial lung disease
  • 3.3. Other pulmonary diseases with mixed restrictive and obstructive pattern
  • 3.4. Sleep-disordered breathing
  • 3.5. Alveolar hypoventilation disorders
  • 3.6. Chronic exposure to high altitude
  • 3.7. Developmental abnormalities
4. Chronic thromboembolic pulmonary hypertension (CTEPH)
5. Miscellaneous
  • 5.1. Hematologic disorders: myeloproliferative disorders, splenectomy
  • 5.2. Systemic disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleimyomatosis, neurofibromatosis, vasculitis
  • 5.3. Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders
  • 5.4. Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure on dialysis