HEMANGIOMATOSIS CAPILAR PULMONAR PDF

O eletrocardiograma mostrava sinais de sobre-carga ventricular direita. Case records of the Massachusetts General Hospital: weekly clinicopathological exercises; case A year-old woman with exertional dyspnea, hemoptysis, and pulmonary nodules. N Engl J Med.

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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.

The PH syndrome is a heterogeneous group of conditions with the common feature of pulmonary artery involvement, resulting in increased pulmonary vascular resistance, hypertrophy, and right ventricular dilatation that can lead to significant cardiac dysfunction cor pulmonale and death. PCH is an extremely rare cause of primary PH and occurs most commonly in adults aged years, but it has been reported to occur in all age groups and shows no sex predilection.

The clinical presentation of PCH is very nonspecific and most frequently includes dyspnea and fatigue. Other complaints are chest pain, chronic cough, peripheral edema, cyanosis, and syncope. On chest X-rays, PCH appears as diffuse or bibasilar reticulonodular opacities. Signs of pulmonary arterial hypertension PAH , such as enlarged central pulmonary arteries and dilatation on the right side of the heart, can also be present.

The final diagnosis of PCH is made by lung biopsy. Open lung biopsy is the most accurate method, but various patients do not have the necessary clinical conditions to undergo such a procedure. Transbronchial biopsy has been considered a highly risky procedure due to the possibility of bleeding. The proliferation of capillary channels within alveolar walls is the main histological characteristic of PCH. This condition must be considered when a patient with PAH presents hemoptysis or hemorrhagic pleural effusion and interstitial lung infiltrates, especially in the presence of centrilobular GGOs and sparse, smoothly thickened interlobular septa.

Currently, biopsy is the only method to confirm the diagnosis of PCH. National Center for Biotechnology Information , U. Journal List J Bras Pneumol v. J Bras Pneumol. Author information Copyright and License information Disclaimer.

Copyright notice. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. 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.

Open in a separate window. Figure 1. In B, chest CT angiography image demonstrating marked dilatation of the main pulmonary artery diameter, 36 mm and bilateral pleural effusion. Figure 2. References 1. Sickle cell anemia: a significant potential cause of pulmonary hypertension in Brazil.

Treatment of schistosomiasis-associated pulmonary hypertension. Magnetic resonance of the lung: a step forward in the study of lung disease. Vonk Noordegraaf A. The image of pulmonary hypertension. The role of imaging techniques in the assessment of pulmonary circulation. Pulmonary capillary hemangiomatosis associated with primary pulmonary hypertension: report of 2 new cases and review of 35 cases from the literature. Medicine Baltimore ; 81 6 — Support Center Support Center.

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Pulmonary capillary hemangiomatosis: an uncommon cause of pulmonary hypertension

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.

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Pulmonary capillary hemangiomatosis PCH is a disease affecting the blood vessels of the lungs, where abnormal capillary proliferation and venous fibrous intimal thickening result in progressive increase in vascular resistance. Indeed, there is some evidence to suggest that PCH and pulmonary veno-occlusive disease are different forms of a similar disease process. These are non specific. Typical symptoms include dyspnea , cough , chest pain and fatigue. At least some cases appear to be due to mutations in the eukaryotic translation initiation factor 2-alpha kinase 4 EIF2AK4 gene. Lung biopsy is essential to make this diagnosis.

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Pulmonary capillary hemangiomatosis

Pulmonary capillary haemangiomatosis PCH is a rare vascular proliferative condition that can lead to pulmonary hypertension. It is characterized by multiple angiomatous lesions composed of proliferating capillary vessels in the lung parenchyma and usually progresses to fatal pulmonary hypertension 1. At the time of initial writing, the etiology and inheritance pattern was not well known 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.

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