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- ACE, angiotensin converting enzyme
- I/D, insertion/deletion
- NYHA, New York Heart Association
- PCR, polymerase chain reaction
- PPH, primary pulmonary hypertension
Primary pulmonary hypertension (PPH) is caused by progressive obliteration of the pulmonary vascular bed that leads to a right ventricular adaptive response—that is, right ventricular hypertrophy and dilatation—and eventually results in right heart failure. In patients with PPH, low cardiac output and elevated right atrial pressures are indicators of poor survival1 and both variables are closely related to right ventricular systolic and diastolic performance. It is unknown why some patients seem to adapt quite well to elevated pulmonary vascular resistances while others develop right heart failure early in the course of their disease. Apparently, adaptation of the right heart varies substantially among individuals. The mechanisms involved in the right ventricular response to elevated pulmonary vascular resistance are unknown. Earlier studies have suggested that the angiotensin converting enzyme (ACE) genotype may be closely related to right heart function in patients with pulmonary hypertension.2 The cloning of the ACE gene has led to the identification of a deletion (D)-insertion (I) polymorphism that affects the level of serum and tissue ACE activity.3 The D/D genotype is associated with the highest ACE level of activity and has been linked to left ventricular hypertrophy.4 This association has been attributed to increased formation of angiotensin …