Pulmonary Hypertension, Primary, 4 (PPH4)

Pulmonary Hypertension, Primary, 4, also known as pph4, is related to ataxia with vitamin e deficiency and mucopolysaccharidosis iv. An important gene associated with Pulmonary Hypertension, Primary, 4 is KCNK3 (Potassium Two Pore Domain Channel Subfamily K Member 3). The drugs Spironolactone and Mineralocorticoid Receptor Antagonists have been mentioned in the context of this disorder. Affiliated tissues include endothelial and lung, and related phenotypes are pulmonary arterial hypertension with lack of acute response to no challenge and pulmonary arterial hypertension