Pulmonary arterial calcification with reversible pulmonary hypertension

Calcification within the pulmonary arteries evident on chest radiography usually represents prolonged and severe pulmonary hypertension that has resulted in calcific atherosclerosis, although calcification has occasionally been reported in diseases without hypertension. In those patients with pulmonary hypertension secondary to cardiac disease, the underlying cause has been patent ductus arteriosus, ventricular septal defect, surgical aortopulmonary shunts, and atrial defect. All these reported cases have shown evidence of shunt reversal secondary to advanced pulmonary vascular disease. Pulmonary artery calcification has thus been implicated as evidence of high pulmonary vascular resistance and irreversible vascular disease. We report a patient with ventricular septal defect, long-standing pulmonary hypertension, and extensive pulmonary artery calcification who had a large left-to-right shunt, near-normal pulmonary vascular resistance, and no irreversible pulmonary vascular disease.