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Pathophysiological and metabolic manifestations of pulmonary vascular disease in children
1Division of Physiology and Pharmacology, University of London.
Insights
Pulmonary vascular disease in children with congenital heart disease is dangerous. Accelerated smooth muscle growth and abnormal vessel remodeling can lead to fatal pulmonary hypertensive crises, even before advanced disease develops.
Area of Science:
- Pediatric Cardiology
- Pulmonary Hypertension
- Congenital Heart Disease
Background:
- Pulmonary vascular disease in children with congenital heart disease (CHD) poses significant risks.
- Abnormal pulmonary vascular remodeling, including increased smooth muscle, can be fatal.
- This is particularly critical after Fontan procedures or heart transplantation.
Purpose of the Study:
- To examine the pathological changes in pulmonary vasculature in children with CHD and pulmonary hypertension.
- To understand the implications of these changes on clinical outcomes and treatment.
- To correlate structural and hemodynamic findings for improved disease prediction.
Main Methods:
- Review of pathological changes in pulmonary vasculature in pediatric patients with CHD.
- Analysis of smooth muscle cell differentiation and connective tissue deposition in hypertensive lungs.
- Correlation of lung biopsy findings with cardiac catheterization data.
Main Results:
- Accelerated smooth muscle differentiation and connective tissue deposition occur in hypertensive lungs.
- Intimal changes are observed in children with CHD.
- Pulmonary hypertensive crises can arise even with potentially reversible disease.
Conclusions:
- Pulmonary vascular disease in pediatric CHD is complex and can be fatal due to accelerated vascular remodeling.
- The reversibility of disease is linked to the type of pathological change present.
- Accurate prediction of disease progression requires further understanding of functional implications.
Abstract:
In children with congenital heart disease pulmonary vascular disease can be fatal for a variety of reasons. Even before the classical changes of advanced pulmonary vascular obstructive disease have developed, a marked increase in pulmonary vascular smooth muscle can be fatal due to pulmonary hypertensive crises. After the Fontan procedure, a modest increase in muscularity can jeopardise the outcome since there is no subpulmonary ventricle to support the pulmonary circulation. Following heart transplantation, a slight increase in muscularity can cause failure of the donor right ventricle unless that heart is already hypertrophied as in the domino procedure. In all children with pulmonary hypertension, either persistent pulmonary hypertension of the newborn or secondary to congenital heart disease the pulmonary vasculature fails to remodel normally after birth. Newborn vessels are characterized by the immaturity of the smooth muscle cells and the paucity of connective tissue. In the hypertensive lung smooth muscle differentiation and connective tissue deposition is accelerated. In children with congenital heart disease intimal changes follow. In these children the potential reversibility of disease following intracardiac repair is determined by the type of pathological change present at the time of repair. However, pulmonary hypertensive crises can occur in young children with potentially reversible disease. Operability is not synonymous with the potential reversibility of pathological lesions. Correlations between structural findings at lung biopsy and haemodynamic findings at cardiac catheterization have improved the accuracy with which the natural and unnatural history of pulmonary vascular disease can be predicted, but is still inadequate because we do not understand the functional implications of the changes.(ABSTRACT TRUNCATED AT 250 WORDS)