Table 3 Analysis of PAH according to sex, separate from genetic PAH

 Heritable PAH

 Congenital PAH

Female-dominant PAH

 Heritable PAH: estrogen-driven BMPR2 polymorphisms

 Idiopathic PAH

 CTD-associated PAH

 Rheumatic autoimmune disease-associated PAH (i.e., SSc, systemic lupus erythematosus, rheumatoid arthritis, myositis/ dermatomyositis, Raynaud’s syndrome, CREST, Sjögren’s syndrome)

 SSc-associated PAH/lcSSc (female-dominant form of SSc)

 Other female dominant autoimmune diseases associated with PAH (i.e., thyroiditis)

 Anorexigen-associated PAH

Male-dominant PAH

 HIV-associated PAH with myocarditis (myocarditis is male dominant and can be caused by HIV or Schistosoma mansoni infection)

 SSc-associated PAH/dcSSc (male-dominant form of SSc)

 Portopulmonary hypertension^a