Introduction

Adult congenital heart disease (ACHD) is one of the fastest growing cardiology subgroups in recent years due to early diagnosis and advances in surgical and endovascular treatment. It’s calculated that 85-90% of patients with congenital heart disease will reach adulthood. The survival rate of these patients results in complications and sequelae that redound in a greater rate of hospitalization and new surgeries, among other procedures.

It is estimated that 5-10% of patients with ACHD develops Pulmonary hypertension (PH) through its progression, and currently being the most frequent cause for Pulmonary arterial hypertension (PAH), after connective tissue diseases. Its appearance is linked to a worst prognosis.

The objective of the present study is to analyze the epidemiological characteristics, clinical and pharmacological treatments in patients with PAH and its impact with PAH and its impact on the survival rate.