9 Disorders of the Pulmonary Circulatory System Thrombotic occlusion of the pulmonary arterial system. Epidemiology Common cause of acute chest pain with respiratory distress In 80% of cases however, acute pulmonary embolism remains asymptomatic. Etiology, pathophysiology, pathogenesis Usually originating in the pelvic or leg veins, the thrombi restrict the blood supply to the lung This leads to capillary damage, transudation, hemorrhage, and occasionally necrosis. Modality of choice CTA and, to a lesser extent, ventilation/perfusion scanning (lung scan). Radiographic findings Nonspecific inconclusive findings: Platelike atelectasis High-riding diaphragm Pleural effusion Local oligemia (Westermark sign) Rarely pulmonary infarction, appearing as a wedge-shaped opacity with a pleural base. CT findings Directly demonstrates embolisms in the pulmonary arterial system (filling defects). Nuclear medicine Wedge-shaped perfusion defect. Pathognomonic findings Intraluminal contrast filling defects Signs of right heart strain. Typical presentation Asymptomatic in about 80% of cases, rendering clinical diagnosis difficult Typical triad of chest pain, respiratory distress, and hemoptysis occurs in only about 5% Deep venous thrombosis in the pelvis or lower extremity is present in less than 50%. Therapeutic options Anticoagulation and fibrinolysis A venal caval filter may be indicated in deep venous thrombosis in the pelvis or lower extremity where medical treatment is ineffective or contraindicated. Course and prognosis Good with therapy Fatal in about 20% of cases if left untreated. What does the clinician want to know? Confirm or exclude diagnosis Extent (unilateral or bilateral, central or peripheral).
Pulmonary Embolism
Definition
Imaging Signs
Clinical Aspects
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 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).
Epidemiology
Idiopathic form is rare Secondary forms are far more common.
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.) This leads to dilatation of the central pulmonary arteries Findings in idiopathic pulmonary arterial hypertension include fibrosis and proliferative muscularization of arterioles 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
Modality of choice
Radiographs, CTA, pulmonary angiography.
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 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.
CTA findings
The pulmonary trunk is wider than the ascending aorta Abrupt changes in caliber In CTEPH there are mural irregularities, intraluminal webs and bands, ste-noses, and/or thromboembolic vascular occlusion Mosaic perfusion Signs of right heart strain—right ventricular dilatation and hypertrophy with protrusion of the interventricular septum against the left ventricle.
Pulmonary angiographic findings
Vascular picture is identical to CTA.
Pathognomonic findings
Dilated central pulmonary arteries with abrupt changes in caliber toward the periphery.
Clinical Aspects
Typical presentation
Symptoms are nonspecific—dyspnea during exercise Limited exercise tolerance Fatigue Advanced-stage disease shows signs of right heart failure.
Therapeutic options