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Paediatric Pulmonology and Allergology
2003 April, Vol. VI, No. 1 (pp.2134-2147)
The child with pulmonary hypertension
Andrew Bush
The commonest causes of pulmonary hypertension are secondary to endstage pulmonary disease or congenital heart disease (including structural abnormalities of the pulmonary veins). Less obvious causes which should always be considered include sleep disorder breathing due to obstructive sleep apnoea or considered include and occult interstitial lung disease. When these have been excluded, the primary pulmonary vascular diseases should be considered. These are primary pulmonary hypertension; pulmonary veno-occlusive disease; pulmonary embolic disease (thromboembolism, and non-thrombotic embolism); and invasive pulmonary capillary haemangiomatosis. The clinical signs and chest X-ray appearances are often non-specific. Echocardiography can often allow an estimate of pulmonary artery pressure and exclude congenital heart disease. Right heard catheterization is usually needed to confirm the diagnosis, estimate any reversibility of elevation of pulmonary vascular resistance, and exclude other causes of pulmonary hypertension. Precise diagnosis may require an open lung biopsy. For many of these conditions, treatment is difficult and the prognosis poor unless the child has a lung transplant. Recent novel therapies such as the phosphdiesterase 5 inhibitor sildenafil and the endothelin receptor antagoist Bosanten may offer new hope for medical treatment of these patients.