Pulmonary arterial hypertension in adult congenital heart disease
Impact and outcome
Data from the GUTI-GUCH Registry
Claudio Gabriel Moros1, 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 subgroups within cardiology 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.