title: Repair of congenital heart disease with associated pulmonary hypertension in children: what are the minimal investigative procedures? Consensus statement from the Congenital Heart Disease and Pediatric Task Forces, Pulmonary Vascular Research Institute (PVRI).
abstract: Standardization of the diagnostic routine for children with congenital heart disease associated with pulmonary arterial hypertension (PAH-CHD) is crucial, in particular since inappropriate assignment to repair of the cardiac lesions (e.g., surgical repair in patients with elevated pulmonary vascular resistance) may be detrimental and associated with poor outcomes. Thus, members of the Congenital Heart Disease and Pediatric Task Forces of the Pulmonary Vascular Research Institute decided to conduct a survey aimed at collecting expert opinion from different institutions in several countries, covering many aspects of the management of PAH-CHD, from clinical recognition to noninvasive and invasive diagnostic procedures and immediate postoperative support. In privileged communities, the vast majority of children with congenital cardiac shunts are now treated early in life, on the basis of noninvasive diagnostic evaluation, and have an uneventful postoperative course, with no residual PAH. However, a small percentage of patients (older at presentation, with extracardiac syndromes or absence of clinical features of increased pulmonary blood flow, thus suggesting elevated pulmonary vascular resistance) remain at a higher risk of complications and unfavorable outcomes. These patients need a more sophisticated diagnostic approach, including invasive procedures. The authors emphasize that decision making regarding operability is based not only on cardiac catheterization data but also on the complete diagnostic picture, which includes the clinical history, physical examination, and all aspects of noninvasive evaluation.
authors.affiliation: Heart Institute, University of São Paulo, São Paulo, Brazil ; Leader of the Congenital Heart Disease Task Force, PVRI.
authors.forename: Antonio Augusto
authors.initials: AA
authors.lastname: Lopes
authors.affiliation: Columbia University College of Physicians and Surgeons, New York, New York, USA ; In memoriam.
authors.forename: Robyn J
authors.initials: RJ
authors.lastname: Barst
authors.affiliation: University College, London, United Kingdom.
authors.forename: Sheila Glennis
authors.initials: SG
authors.lastname: Haworth
authors.affiliation: Stanford University School of Medicine, Stanford, California, USA ; Leader of the Congenital Heart Disease Task Force, PVRI.
authors.forename: Marlene
authors.initials: M
authors.lastname: Rabinovitch
authors.affiliation: King Fahd Armed Forces Hospital, Jeddah, Saudi Arabia.
authors.forename: Maha
authors.initials: M
authors.lastname: Al Dabbagh
authors.affiliation: La Paz Children's Hospital, Madrid, Spain ; Leader of the Pediatric Task Force, PVRI.
authors.forename: Maria Jesus
authors.initials: MJ
authors.lastname: Del Cerro
authors.affiliation: Children's Hospital Colorado, Aurora, Colorado, USA.
authors.forename: Dunbar
authors.initials: D
authors.lastname: Ivy
authors.affiliation: King Saud University, Riyadh, Saudi Arabia.
authors.forename: Tarek
authors.initials: T
authors.lastname: Kashour
authors.affiliation: Amrita Institute of Medical Sciences, Kerala, India.
authors.forename: Krishna
authors.initials: K
authors.lastname: Kumar
authors.affiliation: Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India.
authors.forename: S
authors.initials: S
authors.lastname: Harikrishnan
authors.affiliation: Second University of Naples, Naples, Italy.
authors.forename: Michele
authors.initials: M
authors.lastname: D'Alto
authors.affiliation: Heart Institute, University of São Paulo, São Paulo, Brazil.
authors.forename: Ana Maria
authors.initials: AM
authors.lastname: Thomaz
authors.affiliation: Heart Institute, University of São Paulo, São Paulo, Brazil.
authors.forename: Leína
authors.initials: L
authors.lastname: Zorzanelli
authors.affiliation: Heart Institute, University of São Paulo, São Paulo, Brazil.
authors.forename: Vera D
authors.initials: VD
authors.lastname: Aiello
authors.affiliation: National Institute of Health, Maputo, Mozambique.
authors.forename: Ana Olga
authors.initials: AO
authors.lastname: Mocumbi
authors.affiliation: Dante Pazzanese Institute, São Paulo, Brazil.
authors.forename: Maria Virginia T
authors.initials: MV
authors.lastname: Santana
authors.affiliation: King Fahd Armed Forces Hospital, Jeddah, Saudi Arabia.
authors.forename: Ahmed Nasser
authors.initials: AN
authors.lastname: Galal
authors.affiliation: King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
authors.forename: Hanaa
authors.initials: H
authors.lastname: Banjar
authors.affiliation: King Abdulaziz Cardiac Center, Riyadh, Saudi Arabia.
authors.forename: Omar
authors.initials: O
authors.lastname: Tamimi
authors.affiliation: Kardiozentrum, La Paz, Bolivia.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Heath
authors.affiliation: Hospital Patronato Municipal San Jose Sur, Quito, Ecuador.
authors.forename: Patricia C
authors.initials: PC
authors.lastname: Flores
authors.affiliation: Universidad Nacional de Colombia, Bogota, Colombia.
authors.forename: Gabriel
authors.initials: G
authors.lastname: Diaz
authors.affiliation: National Institute of Cardiology, Mexico City, Mexico.
authors.forename: Julio
authors.initials: J
authors.lastname: Sandoval
authors.affiliation: All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Shyam
authors.initials: S
authors.lastname: Kothari
authors.affiliation: Great Ormond Street Hospital, London, United Kingdom.
authors.forename: Shahin
authors.initials: S
authors.lastname: Moledina
authors.affiliation: Heart Institute, University of São Paulo, São Paulo, Brazil.
authors.forename: Rilvani C
authors.initials: RC
authors.lastname: Gonçalves
authors.affiliation: Heart Institute, University of São Paulo, São Paulo, Brazil.
authors.forename: Alessandra C
authors.initials: AC
authors.lastname: Barreto
authors.affiliation: Heart Institute, University of São Paulo, São Paulo, Brazil.
authors.forename: Maria Angélica
authors.initials: MA
authors.lastname: Binotto
authors.affiliation: Federal University of Minas Gerais, Belo Horizonte, Brazil.
authors.forename: Margarida
authors.initials: M
authors.lastname: Maia
authors.affiliation: King Abdulaziz Cardiac Center, Riyadh, Saudi Arabia.
authors.forename: Fahad
authors.initials: F
authors.lastname: Al Habshan
authors.affiliation: Stollery Children's Hospital, University of Alberta, Edmonton, Alberta, Canada ; Leader of the Pediatric Task Force, PVRI.
authors.forename: Ian
authors.initials: I
authors.lastname: Adatia
keywords: cardiac catheterization; congenital heart disease; pediatric cardiac surgery; postoperative care; pulmonary hypertension