INSUFICIENCIA CARDIACA                                                                                          ISSN 1850-1044

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

Pulmonary Arterial Hypertension in Adult Congenital Heart Disease

Impact and Outcomes

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

Introduction

Adult congenital heart disease (ACHD) is one of the fastest-growing cardiology subgroups in recent years, due to the early diagnosis and advances in surgical and endovascular treatment. It is estimated that 85-90% of patients with congenital heart disease reach adult age. The survival of these patients generate complications and sequels that result in high rates of hospitalizations and new surgeries, among other procedures.

It is estimated that from 5 to 10% of the patients with ACHD develop pulmonary hypertension (PH) during their progression, which in reality the most frequent case of pulmonary arterial hypertension (PAH) after the diseases of connective tissue. Their appearance is related to a worse prognosis for the patient.

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