Format

Send to

Choose Destination
Pulm Circ. 2014 Jun;4(2):330-41. doi: 10.1086/675995.

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).

Author information

1
Heart Institute, University of São Paulo, São Paulo, Brazil ; Leader of the Congenital Heart Disease Task Force, PVRI.
2
Columbia University College of Physicians and Surgeons, New York, New York, USA ; In memoriam.
3
University College, London, United Kingdom.
4
Stanford University School of Medicine, Stanford, California, USA ; Leader of the Congenital Heart Disease Task Force, PVRI.
5
King Fahd Armed Forces Hospital, Jeddah, Saudi Arabia.
6
La Paz Children's Hospital, Madrid, Spain ; Leader of the Pediatric Task Force, PVRI.
7
Children's Hospital Colorado, Aurora, Colorado, USA.
8
King Saud University, Riyadh, Saudi Arabia.
9
Amrita Institute of Medical Sciences, Kerala, India.
10
Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India.
11
Second University of Naples, Naples, Italy.
12
Heart Institute, University of São Paulo, São Paulo, Brazil.
13
National Institute of Health, Maputo, Mozambique.
14
Dante Pazzanese Institute, São Paulo, Brazil.
15
King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
16
King Abdulaziz Cardiac Center, Riyadh, Saudi Arabia.
17
Kardiozentrum, La Paz, Bolivia.
18
Hospital Patronato Municipal San Jose Sur, Quito, Ecuador.
19
Universidad Nacional de Colombia, Bogota, Colombia.
20
National Institute of Cardiology, Mexico City, Mexico.
21
All India Institute of Medical Sciences, New Delhi, India.
22
Great Ormond Street Hospital, London, United Kingdom.
23
Federal University of Minas Gerais, Belo Horizonte, Brazil.
24
Stollery Children's Hospital, University of Alberta, Edmonton, Alberta, Canada ; Leader of the Pediatric Task Force, PVRI.

Erratum in

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.

KEYWORDS:

cardiac catheterization; congenital heart disease; pediatric cardiac surgery; postoperative care; pulmonary hypertension

Supplemental Content

Full text links

Icon for Atypon Icon for PubMed Central
Loading ...
Support Center