INSUFICIENCIA CARDIACA                                                                                                 ISSN 1850-1044

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

Pulmonary arterial hypertension in adult congenital heart disease

Impact and outcome

Data from GUTI-GUCH registry

Claudio Gabriel Morós, Marisa Pacheco Otero, Gianina Faliva, Maria del Carmen Rubio, Mariana López Daneri, Isabel Torres, Inés Abella, Alvaro Sosa Liprandi, Liliana Noemí Nicolosi, Maria Grippo

Introduction

Adult congenital heart disease (ACHD) is one of the fastest growing cardiology subgroups in recent years due to the early diagnostics and advance in surgical and endovascular treatment. It is estimated that 85-90% of patients with congenital heart disease will reach adulthood. The survival of these patients generates complications and sequelae which cause a great number of hospitalizations and new surgeries, among other procedures.

It is estimated that 5 – 10% of ACHD patients will develop pulmonary hypertension (PH) during development, being in reality the most frequent cause for pulmonary arterial hypertension (PAH) after connective tissue diseases. Its presence is related to a worst patient’s prognosis.

This work objective was to analyze the epidemiologic characteristics and clinical and pharmacological treatment of patients with PAH and their impact on survival.

Pulmonary arterial hypertension in adult congenital heart disease

Impact and Outcome

Data from the GUTI-GUCH Registry

Claudio Gabriel Morós, Marisa Pacheco Otero, Gianina Faliya, María del Carmen Rubio, Mariana López Daneri, Isabel Torres, Ines Abella, Álvaro Sosa Lipandri, 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 estimated that between 85-90% of the congenital heart disease patients will reach adulthood. The survival of these patients generates complications and sequels that result in a higher rate of hospitalizations and new surgeries, among other procedures.

It is estimated that between from 5% to 10% of patients with AHCD will develop pulmonary hypertension (PH) during their evolution being nowadays the most frequent cause of pulmonary arterial hypertension (PAH) after the connective tissue diseases. Its presence it is related with the worst patient’s prognosis.

The present work’s objective was to analyze the epidemiological, clinical and pharmacological characteristics on patients with PAH and the impact on the survival rate.

Orlando Herrera
SPA 4003
December 6, 2016
INSUFICIENCIA CARDIACA
Noviembre de 2016

Pulmonary arterial hypertension in adult congenital heart disease
Impact and outcome
Data from GUTI-GUCH Register

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 fastest growing cardiology subgroups in latest years, due to early diagnosis and advances in surgical and endovascular treatment. 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 result in a higher rate 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 their evolution and being currently the most frequent cause of pulmonary arterial hypertension (PAH) after connective tissue disease. Its occurrence is related to a worse prognosis for the patient.
The objective of the present study was to analyze the epidemiological, clinical and pharmacotherapy characteristics of patients with PAH and their impact on survival.

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Hipertensión arterial pulmonar en cardiopatías congénitas del adulto
Impacto y resultados
Datos del Registro GUTI-GUCH

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

Introducción
La cardiopatía congénita del adulto (CCA) es uno de los subgrupos de mayor crecimiento en los últimos años dentro de la cardiología, debido al diagnóstico precoz y al avance de los tratamientos quirúrgico y endovascular. Se calcula que el 85-90% de los pacientes con cardiopatías congénitas llegarán a la vida adulta. La sobrevida de estos pacientes genera complicaciones y secuelas que redundan en una mayor tasa de internaciones y nuevas cirugías, entre otros procedimientos.
Se estima que del 5 al 10% de los pacientes con CCA desarrollarán hipertensión pulmonar (HP) en su evolución siendo en la actualidad la causa más frecuente de hipertensión arterial pulmonar (HAP) después de las enfermedades del tejido conectivo
Su aparición se relaciona con peor pronóstico para el paciente.
El objetivo del presente trabajo fue analizar las características epidemiológicas, clínicas y de tratamiento farmacológico en pacientes con HAP y su impacto en la sobrevida.