INSUFICIENCIA CARDIACA                                                                                                 ISSN 1850-1044

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

Pulmonary arterial hypertension in adult congenital heart disease

Impact and outcome

Data from GUTI-GUCH registry

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

Introduction

Adult congenital heart disease (ACHD) is one of the fastest growing cardiology subgroups in recent years due to the early diagnostics and advance 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 sequelae which cause a great number of hospitalizations and new surgeries, among other procedures.

It is estimated that 5 – 10% of ACHD patients will develop pulmonary hypertension (PH) during development, being in reality the most frequent cause for pulmonary arterial hypertension (PAH) after connective tissue diseases. Its presence is related to a worst patient’s prognosis.

This work objective was to analyze the epidemiologic characteristics and clinical and pharmacological treatment of patients with PAH and their impact on survival.