INSUFICIENCIA CARDIACA

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

Pulmonary arterial hypertension in adult congenital heart disease

Impact and outcome

Data from the GUTI-GUCH Registry

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

Introduction

The 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 estimated that the 85-95% of patients with congenital heart disease will reach adulthood. The survival of these patients generates complications and sequelae that results in a greater number of hospitalizations and new surgeries, among other procedures.

It is estimated that between 5 and 10% of the patients with ACHD will develop pulmonary hypertension (PH) during their evolution, being nowadays the most frequent cause of pulmonary arterial hypertension (PAH) after the connective tissue diseases. Its presence it is related with the worst patient’s prognosis.

The present study’s objective was to analyze the epidemiological characteristics as well as clinical and pharmacological treatments on patients with PAH and the impact on the survival rate.