Cardiac catheterization in children with pulmonary hypertensive vascular disease: consensus statement from the Pulmonary Vascular Research Institute, Pediatric and Congenital Heart Disease Task Forces

PVRI Member Authors: Maria Jesus del Cerro Marin, Shahin Moledina, Sheila Glennis Haworth, Dunbar Ivy, Gabriel Diaz, Alexandra Heath, Antonio Lopes, Ana Mocumbi, Goverdhan Dutt Puri, Dr. S. Harikrishnan, Lina Maria Caicedo, Omar Al-Tamimi, Ian Adatia

Abstract

Cardiac catheterization is important in the diagnosis and risk stratification of pulmonary hypertensive vascular disease (PHVD) in children. Acute vasoreactivity testing provides key information about management, prognosis, therapeutic strategies, and efficacy. Data obtained at cardiac catheterization continue to play an important role in determining the surgical options for children with congenital heart disease and clinical evidence of increased pulmonary vascular resistance. The Pediatric and Congenital Heart Disease Task Forces of the Pulmonary Vascular Research Institute met to develop a consensus statement regarding indications for, conduct of, acute vasoreactivity testing with, and pitfalls and risks of cardiac catheterization in children with PHVD. This document contains the essentials of those discussions to provide a rationale for the hemodynamic assessment by cardiac catheterization of children with PHVD.

Cardiac catheterization is important in the diagnosis and risk stratification of pulmonary hypertensive vascular disease (PHVD) in children. Acute vasoreactivity testing provides key information about management, prognosis, therapeutic strategies, and efficacy. Data obtained at cardiac catheterization continue to play an important role in determining the surgical options for children with congenital heart disease and clinical evidence of increased pulmonary vascular resistance. Hemodynamics (pressures, systemic and pulmonary blood flow, and vascular resistance index) obtained at cardiac catheterization relate directly to outcome.1 Cardiac catheterization can be applied uniformly across all age groups and neurodevelopmental stages to demonstrate disease progression or regression and response to therapy.2 Current guidelines for evaluation of pulmonary hypertension in adults3 include recommendations for cardiac catheterization, with specific definitions, and discussion of prognostic and therapeutic implications of a positive acute vasoreactivity test. The functional classification, diagnostic spectrum, phenotype, and progression of PHVD in children differ from those in adults and have been discussed by Pulmonary Vascular Research Institute (PVRI) task forces.2,4,5 These differences directly affect the conduct of, data required for, and value of cardiac catheterization undertaken in children with disorders of the pulmonary circulation.

The aim of this document is to provide a comprehensive, globally applicable guideline for cardiac catheterization in pediatric PHVD. It reflects the ideas and experience of the Pediatric Task Forces at the sixth and seventh PVRI meetings held in Cape Town, South Africa (February 2012) and Istanbul, Turkey (January 2013). Members of the Pediatric Task Forces included pediatric specialists from cardiology, pulmonology, anesthesiology, cardiac critical care, cardiac surgery, and neonatology, with global representation from 12 countries.

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Topics

Catheterization
Echocardiography
Hemodynamics
Pediatric
Pulmonary Hypertension
Right Ventricle: Structure, Function and Dysfunction

Authors

Maria Jesus del Cerro, Shahin Moledina, Sheila G. Haworth, Dunbar Ivy, Maha Al Dabbagh, Hanaa Banjar, Gabriel Diaz, Alexandria Heath-Freudenthal, Ahmed Nasser Galal, Tilman Humpl, Snehal Kulkarni, Antonio Lopes, Ana Olga Mocumbi, G. D. Puri, Beyra Rossouw, S. Harikrishnan, Anita Saxena, Patience Udo, Lina Caicedo, Omar Tamimi, Ian Adatia

Published in:

Pulmonary Circulation Vol 6: No 1 cover image

March 2016

Pulmonary Circulation Vol 6: No 1

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