Presentation
Chronic dry cough, progressive dyspnea, syncopal episodes with exertion, hemoptysis.
Patient Data
Extensive subcentimeter poorly defined groundglass nodules in a centrilobular and reticulonodular configuration.
Relative enlargement of the right heart. No significant pericardial or pleural effusion. The main pulmonary artery is dilated up to 3.9 cm in diameter. There is also enlargement of the central pulmonary arteries, with peripheral tapering compatible with pulmonary hypertension. There is no interlobular septal thickening. Extensive poorly-defined subcentimeter ground-glass nodules of both lungs which demonstrate centrilobular and reticulonodular configuration are compatible with known history of pulmonary capillary hemangiomatosis.
Enlargement of main pulmonary artery measuring 3.9 cm, consistent with pulmonary hypertension.
Case Discussion
This patient was referred to our transplant center with a diagnosis of pulmonary capillary hemgangiomatosis. Because of his history of hemoptysis a biopsy has not been obtained. Imaging features of poorly-defined diffuse sub-centimeter centrilobular and reticulonodular ground-glass opacities alongside his clinical symptoms support the diagnosis of pulmonary capillary hemangiomatosis. The patient has not elected for transplantation at this time.
There is overlap in the clinical, histopathological and imaging findings in pulmonary capillary
hemangiomatosis (PCH) and pulmonary veno-occlusive disease (PVOD) and the differences are subtle in these rare causes of WHO group 1 pulmonary arterial hypertension.
In both cases, the typical imaging manifestations of pulmonary arterial hypertension are present
Definitive diagnosis of both PCH and PVOD requires microscopic examination of lung tissue.
This patient with known PCH has several clinical and imaging features distinguishing it from PVOD:
1. Hemoptysis, present in this patient, occurs more frequently in PCH than in PVOD.
2. PCH characteristically shows diffuse poorly defined centrilobular ground-glass opacities, as opposed to PVOD in which the ground-glass opacities are sharply defined.
3. Nodular or smooth interlobular septal thickening is often absent in PCH. When present, it is usually less pronounced than in patients with PVOD, which is marked by smoothly thickened interlobular septa.
4. Pleural effusions are less common in PCH than PVOD.
Both PCH and PVOD must be distinguished from other causes of pulmonary hypertension, as potent vasodilators (typical pharmacological management of pulmonary hypertension) can trigger fatal pulmonary edema in these patients. Definitive treatment of both disorders is timely evaluation for lung transplantation.
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