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Pulmonary hypertension in congenital heart disease: irreversible vascular changes in young infants
Insights
Severe pulmonary hypertension is rare in infants with congenital heart disease. This study found an unexpected increase in severe cases, possibly due to improved care and high altitude, suggesting earlier surgical intervention.
Area of Science:
- Pediatric Cardiology
- Pulmonary Hypertension
- Congenital Heart Disease
Background:
- Pulmonary hypertensive arteriopathy (grade IV or higher) is considered rare in infants under one year.
- Congenital heart disease (CHD) can lead to pulmonary hypertension.
- Autopsy data from infants with CHD provides insight into disease progression.
Observation:
- Six cases of grade IV pulmonary artery hypertension were identified in 280 infants with CHD over 20 years.
- All identified cases occurred in the latter years of the study period.
- Affected infants had conditions such as ventricular septal defect and atrioventricular canal.
Findings:
- The frequency of severe pulmonary hypertensive arteriopathy appears to be increasing in infants.
- Improved supportive care may allow infants to survive longer, developing more severe disease.
- High altitude may accelerate the development of pulmonary arteriopathy.
Implications:
- The rising incidence warrants consideration of early surgical correction for underlying cardiovascular malformations.
- Early intervention may be particularly crucial for infants in high-altitude regions.
- Further research is needed to understand the contributing factors to this observed increase.
Abstract:
Among 280 infants under 1 year of age with congenital heart disease autopsied at the University of Colorado Health Sciences Center between 1959 and 1978, there were six instances of grade IV1 pulmonary artery hypertension. Five were patients with ventricular septal defect (four associated with other cardiovascular malformations). The sixth was a patient with atrioventricular canal. The youngest was 2 1/2 months of age. Advanced degrees of pulmonary hypertensive arteriopathy (grade IV or more) have been said to be rare in infants, especially under the age of 1 year. The fact that all of these cases occurred within the last few years of the study suggests the possibility of improved supportive care leading to the prolonged survival of infants who might otherwise have died prior to developing severe disease. In addition, the role of altitude in accelerating the arteriopathy must be considered in the present series. In any case, this unexpected increase in the frequency of severe pulmonary hypertensive arteriopathy should stimulate consideration of early surgical correction of the underlying cardiovascular malformation, especially in areas of relatively high altitude.