Disorders of the Pulmonary Circulatory System

9 Disorders of the Pulmonary Circulatory System


Pulmonary Embolism


Definition


Thrombotic occlusion of the pulmonary arterial system.


image Epidemiology


Common cause of acute chest pain with respiratory distress image In 80% of cases however, acute pulmonary embolism remains asymptomatic.


image Etiology, pathophysiology, pathogenesis


Usually originating in the pelvic or leg veins, the thrombi restrict the blood supply to the lung image This leads to capillary damage, transudation, hemorrhage, and occasionally necrosis.


Imaging Signs


image Modality of choice


CTA and, to a lesser extent, ventilation/perfusion scanning (lung scan).


image Radiographic findings


Nonspecific inconclusive findings: Platelike atelectasis image High-riding diaphragm image Pleural effusion image Local oligemia (Westermark sign) image Rarely pulmonary infarction, appearing as a wedge-shaped opacity with a pleural base.


image CT findings


Directly demonstrates embolisms in the pulmonary arterial system (filling defects).


image Nuclear medicine


Wedge-shaped perfusion defect.


image Pathognomonic findings


Intraluminal contrast filling defects image Signs of right heart strain.


Clinical Aspects


image Typical presentation


Asymptomatic in about 80% of cases, rendering clinical diagnosis difficult image Typical triad of chest pain, respiratory distress, and hemoptysis occurs in only about 5% image Deep venous thrombosis in the pelvis or lower extremity is present in less than 50%.


image Therapeutic options


Anticoagulation and fibrinolysis image A venal caval filter may be indicated in deep venous thrombosis in the pelvis or lower extremity where medical treatment is ineffective or contraindicated.


image Course and prognosis


Good with therapy image Fatal in about 20% of cases if left untreated.


image What does the clinician want to know?


Confirm or exclude diagnosis image Extent (unilateral or bilateral, central or peripheral).


image


Fig. 9.1 Massive bilateral pulmonary embolism in a 79-year-old man with acute coronary syndrome. CTA shows thrombi incompletely blocking the blood flow in both main pulmonary trunks, the superior and inferior lobe arteries, and the segmental branches: findings are consistent with acute pulmonary embolism. The plain chest radiographs were normal.


image


Fig. 9.2 Pulmonary infarct in a 47-year-old woman with protein S deficiency. The plain chest radiograph shows a faint wedge-shaped area of opacification with a pleural base in the right upper lung field and a second smaller area in the middle lung field consistent with infarct pneumonia. The patient had a known history of thromboembolic disease, which had led to occlusion of the inferior vena cava. Collateralization via the expanded azygos vein (*, arrows).


Differential Diagnosis










Pneumonia


– Fever


– One must consider the possibility of embolism where nonspecific shadows are present


Tips and Pitfalls


Insufficient filling of the pulmonary arteries image Breathing artifacts.


Selected Reference

Guilabert JP et al. Can multislice CT alone rule out reliably pulmonary embolism? A prospective study. Eur J Radiol 2007; 62: 220–226


Pulmonary Arterial Hypertension


Definition


Abnormally elevated blood pressure in the pulmonary artery (mean pulmonary arterial pressure at rest > 25 mmHg, with exercise > 30 mmHg).


image Epidemiology


Idiopathic form is rare image Secondary forms are far more common.


image Etiology, pathophysiology, pathogenesis


Increase in pulmonary arterial pressure due to cardiac pathology (left-to-right shunt, mitral stenosis, anomalous pulmonary venous connection, etc.) or pulmonary pathology (thromboembolic disease [CTEPH], emphysema, pulmonary fibrosis, etc.) image This leads to dilatation of the central pulmonary arteries image Findings in idiopathic pulmonary arterial hypertension include fibrosis and proliferative muscularization of arterioles image Pulmonary arterial hypertension is classified as idiopathic, familial, or associated; the latter occurs in disorders such as venous occlusive disease or capillary hemangiomatosis.


Imaging Signs


image Modality of choice


Radiographs, CTA, pulmonary angiography.


image Radiographic findings


Dilated central pulmonary arteries (diameter of the middle part of the right pulmonary artery > 16 mm in men, > 14 mm in women) with abrupt changes in caliber toward the periphery image Signs of right heart strain—enlarged area of contact between the anterior wall of the heart and the sternum, prominent pulmonary trunk, prominent main pulmonary artery segment.


image CTA findings


The pulmonary trunk is wider than the ascending aorta image Abrupt changes in caliber image In CTEPH there are mural irregularities, intraluminal webs and bands, ste-noses, and/or thromboembolic vascular occlusion image Mosaic perfusion image Signs of right heart strain—right ventricular dilatation and hypertrophy with protrusion of the interventricular septum against the left ventricle.


image Pulmonary angiographic findings


Vascular picture is identical to CTA.


image Pathognomonic findings


Dilated central pulmonary arteries with abrupt changes in caliber toward the periphery.


Clinical Aspects


image Typical presentation


Symptoms are nonspecific—dyspnea during exercise image Limited exercise tolerance image Fatigue image Advanced-stage disease shows signs of right heart failure.


image Therapeutic options

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Feb 28, 2016 | Posted by in RESPIRATORY IMAGING | Comments Off on Disorders of the Pulmonary Circulatory System

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