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 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 early diagnosis and advances in surgical and endovascular treatment. It is calculated that 85-95% of patients with congenital heart disease will reach adulthood. The survival of these patients create complications and sequelae that contribute to higher  rates of hospitalization and new surgeries, among other procedures.

It is estimated that 5 to 10% of patients with ACHD will develop pulmonary hypertension (PH) in its progression, making it one of the most common causes of pulmonary arterial hypertension (PAH), after connective tissues diseases. Its occurrence is related to a worse prognosis.

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