INSUFICIENCIA CARDIACA

Received: 06/25/2016
Accepted: 08/15/2016

Insuf Card 2016; 11 (3): 115-121       Available at: http://www.insufcienciacardiaca.org/

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 treatments. It is estimated that 85-90% of patients with congenital heart disease will reach adulthood. The survival of these patients generates complications and sequelae that lead to higher hospitalization rates and new surgeries, among other procedures.
During its progression, it is estimated that 5 to 10% of patients with ACHD will develop pulmonary hypertension (PH), presently being the prominent cause of pulmonary arterial hypertension (PAH), after connective tissue diseases. Its emergence is linked to a worse prognosis for the patient.
The objective of the present study was to analyze the characteristics of epidemiological, clinical, and pharmacological treatment in patients with PAH and its impact on survival.