PURPOSE: Characteristics, therapeutic trends, in-hospital and follow - up outcome in PAH patients (pts) in a Latin American population is unknown, until our knowledge.
METHODS: Prospective registry, conducted in two centers. Pts on group I and IV were included. Diagnosis and severity of PAH were established by right heart catheterization (RHC). Biomarkers were used as risk stratification. Alpert criteria were used to improve quality of registry. Clinical, hemodynamic, echocardiography, V/Q lung scan, pulmonary function tests, treatment and major cardiovascular adverse events (heart failure, cardiogenic shock, pulmonary embolism and mortality) were recorded. Six year follow - up was established. The protocol was approved by institutional review board. Statistical analysis: Chi square, Yates corrected, Wilcoxon test. Logistic regression model. Data are expressed in percent, median, mean and SD.
RESULTS: From 2004 to 2010, 120 consecutive pts were included. Pts were female (76%) and young (36 ± 14). The etiology: idiopathic PAH (34%) and associated with congenital heart disease (31%), connective tissue diseases (22%), anorexigens (2%), HIV (2%), and chronic thromboembolic PAH (10%). The majority was enrolled in class II (79%) (WHO). Mean six -minute walk distance was 414 ± 101 m. RHC was performed in 84% with an mPAP of 61 ± 21 mmHg. ECHO was done in 100% with systolic pulmonary arterial pressure of 93 ± 28 mmHg. Acute vasodilator challenge was performed in IPAH with adenosine (86%) and inhaled iloprost (14%), 35% of them were positive. In a six year follow - up cardiovascular mortality was 14%.
CONCLUSION: This evidence provides important and reliable information on the complete spectrum, outcome, quality of care, and identifies areas for further quality improvement. RENEHAP broadens our knowledge about the outcome in the current era in pts with PAH and what needs to be improved in real life practice in Mexico.
CLINICAL IMPLICATIONS: This could be the first and modern evidence regarding PAH in Mexican pts. The incidence of congenital heart disease and functional class II at enrrolment is different than previous registries.
DISCLOSURE: Edgar Garcia Badillo, No Financial Disclosure Information; No Product/Research Disclosure Information