It is thought to typically affect children and young adults Clinical presentation The clinical presentation overlaps that of primary pulmonary hypertension. Pathology Pathologically, PCH is characterized by proliferation of benign thin-walled capillary-sized blood vessels within the lung parenchyma 5. Proliferating capillaries invade the pulmonary interstitium and alveolar septae and occlude the pulmonary vasculature. Invasion of pulmonary veins and, less frequently, pulmonary arteries can be common 8. Radiographic features CT The typical radiographic appearance described is that of a diffuse bilateral reticulonodular pattern associated with enlarged central pulmonary arteries 3.
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This article has been cited by other articles in PMC. To the Editor: We read with interest the recent publications in the Brazilian Journal of Pulmonology highlighting the prevalence of pulmonary hypertension PH in Brazil, particularly as a complication of sickle cell anemia 1 and schistosomiasis, 2 and discussing the role of imaging methods in the evaluation of this disease.
A year-old male nonsmoker presented to the pulmonology department with a three-month history of rapidly progressive dyspnea. Physical examination revealed digital clubbing, and he was tachypneic and cyanotic, requiring supplementary oxygen. Heart auscultation and chest examination were unremarkable, and examination of the abdomen revealed a slightly enlarged liver. A transesophageal echocardiogram showed enlargement of the main pulmonary artery and right heart chamber.
Arterial blood gas analysis showed the following results: pH, 7. A chest X-ray showed dilatation of the main and central pulmonary arteries with a nonspecific interstitial infiltrate.
Chest CT angiography allowed us to exclude pulmonary embolism and revealed diffuse centrilobular ground-glass opacities GGOs and marked dilatation 36 mm of the main pulmonary artery Figure 1. The GGOs were bilateral and symmetrical, with no thickening of the interlobular septa. Anticoagulation therapy with heparin was started and initially improved the symptoms slightly. Pulmonary catheterization demonstrated a mean pulmonary artery pressure of 65 mmHg and a negative vasoactive intestinal peptide result.
The pulmonary artery occlusion pressure was 13 mmHg, and cardiac output was 2. Because of the diagnostic suspicion of PCH, the patient was placed on the waiting list for lung transplantation. Despite the treatment with heparin and furosemide, the condition of the patient gradually deteriorated, and he died two months after admission.
An autopsy revealed prominent dilation of the right heart chambers and of the pulmonary artery, as well as diffuse alveolar hemorrhage. Microscopic examination of the lungs showed well-demarcated areas with dense proliferation of capillary channels within alveolar walls and surrounding walls of pulmonary venules and veins, which supported the diagnosis of PCH Figure 2.
Pulmonary capillary hemangiomatosis: an uncommon cause of pulmonary hypertension
Fenrihn There was a problem providing the content you requested Different sizes of centrilobular ground glass opacities in chest high resolution computed tomography pulmobar patients with pulmonary veno-occlusive disease and patients with pulmonary capillary hemangiomatosis. The Journal is published both in Spanish and English. Proliferating capillaries invade the pulmonary interstitium and alveolar septae and occlude the pulmonary vasculature. Infobox medical condition new Pages using infobox medical condition with unknown parameters All stub articles. Loading Stack — 0 images remaining. Please cite this article as: Cardiovasc Path, 22pp.
Hemangiomatosis capilar pulmonar