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

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.

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).

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.

Repair of congenital heart disease with associated pulmonaryhypertension in children: what are the minimal investigativeprocedures? Consensus statement from the Congenital HeartDisease and Pediatric Task Forces, Pulmonary VascularResearch Institute (PVRI)

Standardization of the diagnostic routine for children with congenital heart disease associatedwith pulmonary arterial hypertension (PAH-CHD) is crucial, in particular since inappropriate assignmentto 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 HeartDisease and Pediatric Task Forces of the Pulmonary Vascular Research Institute decided to conduct asurvey aimed at collecting expert opinion from different institutions in several countries, covering manyaspects of the management of PAH-CHD, from clinical recognition to noninvasive and invasive diagnosticprocedures and immediate postoperative support. In privileged communities, the vast majority of childrenwith 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 percentageof patients (older at presentation, with extracardiac syndromes or absence of clinical features of increasedpulmonary blood flow, thus suggesting elevated pulmonary vascular resistance) remain at a higher risk ofcomplications and unfavorable outcomes. These patients need a more sophisticated diagnostic approach,including invasive procedures. The authors emphasize that decision making regarding operability is basednot only on cardiac catheterization data but also on the complete diagnostic picture, which includes theclinical history, physical examination, and all aspects of noninvasive evaluation.