Jorge Caceres

Spa 4003

INSUFICIENCIA CARDIACA

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

Pulmonary arterial hypertension in adult congenital heart diseases

Impact and outcome

Data from the GUTI-GUCH Registry

Claudio Gabriel Morós¹, Marisa Pacheco Otero², Gianina Faliva³, María del Carmen Rubio⁴, Mariana López Daneri⁵, Isabel Torres⁶, Inés Abella⁷, Álvaro Sosa Liprandi⁸, Liliana Noemí Nicolosi⁹, María Grippo¹⁰

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 85-90% of patients with congenital heart diseases reach adulthood. The survival of these patients generates complications and sequelae that contribute to a higher rate of hospital admissions and new surgeries, among other procedures^1,2.

It is estimated that 5 to 10% of patients with ACHD develop pulmonary hypertension (PH) during its progression^3,4, making it the most frequent cause of pulmonary arterial hypertension (PAH) after connective tissue diseases^5,6. Its’ appearance is related to a worse prognosis for the patient.

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