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.

Circulating blood volumes in pulmonary hypertension associated with erythrocytosis--the effects of therapeutic hemodilution.

In the Eisenmenger syndrome, indirect estimation of blood volumes may provide quite inaccurate information when seeking to define therapeutic strategies. With this in mind, we analyzed directly the red cell mass, plasma volume, and total blood volume in patients with pulmonary hypertension associated with congenital cardiac defects and erythrocytosis, comparing the results with the respective estimated volumes, and examining the changes induced by therapeutic hemodilution. Thus, we studied 17 patients with the Eisenmenger syndrome, aged from 15 to 53 years, in the basal condition, studying 12 of them both before and after hemodilution. We also investigated five individuals with minimal cardiac lesions, aged from 14 to 42 years, as controls. Red cell mass and plasma volumes were measured using [51chromium]-sodium chromate and [131iodine]-albumin respectively. Hemodilution was planned so as to exchange 10% of the total blood volume, using 40,000 molecular weight dextran simultaneously to replace the removed volume. The mean values of the red cell mass, plasma volume and total blood volume as assessed by radionuclide techniques were 32%, 31% and 32% higher than the respective volumes as estimated using empirical mathematical formulas (p < 0.002). The measured total blood volume was also 19% higher in the patients compared with controls. Following a period of 5 days after hemodilution, we noted a 13% reduction in red cell mass (p = 0.046), and 10% reduction in total blood volume (p = 0.02), albeit with no changes in the plasma volume. We conclude that direct measurement of blood volumes is useful for proper management of these patients, and provides results that are considerably different from those obtained by empirical estimations.