INSUFICIENCIA CARDIACA

Insuf Card 2016;11 (3): 115-121

Pulmonary arterial hypertension in adult congenital heart disease  

Impact and results

Data from the GUTI-GUCH Registry

Claudio Gabriel Morós, Marisa Pacheco Otero, Gianina Faliva, María del Carmen Rubio,  Mariana López Daneri, Isabel Torres, Inés Abella, Álvaro Sosa Liprandi, Liliana Noemí Nicolosi, María Grippo

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 is estimated that 85-90% of patients with congenital heart disease will reach adulthood. The survival of these patients generates complications, and consequences that contribute to a higher rates of new surgeries, hospitalization, among other procedures.

It is estimated that 5 to 10% of patients with ACHD develop pulmonary hypertension (PH) in its evolution, making it currently the most frequent cause of pulmonary arterial hypertension (PAH) after connective tissue diseases. Its occurrence is related with the worse patient prognosis.

The objective of the study was to analyze the epidemiological, clinical characteristics, and pharmacological treatment of patients with PAH and their impact on survival.