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 Torres, Ines Abella, Álvaro Sosa Lipandri, Liliana Noemí Nicolosi, María Grippo.

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 is estimated that between 85-90% of the congenital heart disease patients will reach adulthood. The survival of these patients generates complications and sequels that result in a higher rate of hospitalizations and new surgeries, among other procedures.

It is estimated that between from 5% to 10% of patients with AHCD 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 work’s objective was to analyze the epidemiological, clinical and pharmacological characteristics on patients with PAH and the impact on the survival rate.