Pulmonary Hypertension

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What the Radiologist needs to know - Source Radiographics journal

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Pulmonary hypertension: How the Radiologist can help:

Pulmonary hypertension : How the Radiologist can help RadioGraphics 2012; 32:9–32 Dr. Varun Babu MD Resident Department of Radiology AIMS

Introduction:

Introduction Definition Classification Pathophysiology Imaging algorithm Follow up Diseases with typical imaging findings RadioGraphics 2012; 32:9–32

Definition:

Definition Mean pulmonary arterial pressure < 20 mm Hg – normal 21-24 mm Hg – abnormal ≥ 25 mm Hg – Pulmonary hypertension Pulmonary arterial hypertension Elevated precapillary pulmonary resistance Normal pulmonary venous pressure: pulmonary wedge pressure ≤ 15mm Hg RadioGraphics 2012; 32:9–32

Classification:

Classification 4 th world symposium on pulmonary hypertension in California 2008 – updated clinical classification system RadioGraphics 2012; 32:9–32

Classification:

Classification RadioGraphics 2012; 32:9–32

Pathology and clinical course:

Pathology and clinical course RadioGraphics 2012; 32:9–32

Pathology and clinical course:

Pathology and clinical course RadioGraphics 2012; 32:9–32

Pathology and clinical course:

Pathology and clinical course RadioGraphics 2012; 32:9–32

Key point:

Key point The ability of the right ventricle to cope = main determinant of patients’ functional capacity and survival Right ventricular size & function – RV volumes and ejection fraction = cardiac MR imaging RadioGraphics 2012; 32:9–32

Diagnosis and assessment:

Diagnosis and assessment RadioGraphics 2012; 32:9–32

Diagnosis and assessment:

Diagnosis and assessment RadioGraphics 2012; 32:9–32

Diagnosis and assessment:

Diagnosis and assessment RadioGraphics 2012; 32:9–32

Chest radiography:

Chest radiography RadioGraphics 2012; 32:9–32

Chest radiography:

Chest radiography Central pulmonary arterial dilatation Pruning of peripheral arteries Increased diameter of right interlobar artery (15mm & 16mm in women and men respectively) Reduced retrosternal air space on lateral view – RV dilatation RadioGraphics 2012; 32:9–32

Multidetector CTPA:

Multidetector CTPA RadioGraphics 2012; 32:9–32

Vascular signs:

Vascular signs Main pulmonary artery diameter at the level of its bifurcation >29mm Positive predictive value 97% Sensitivity 87% Specificity 89% Segmental artery-to-bronchus diameter ratio of 1:1 or more in 3-4 lobes. RadioGraphics 2012; 32:9–32

Vascular signs:

Vascular signs Main pulmonary arterial diameter larger than that of ascending aorta Positive predictive value 96% Specificity 92% RadioGraphics 2012; 32:9–32

Vascular signs:

Vascular signs Devaraj et al Ratio of MPA to AA with ECHO derived RV systolic pressure improved detection Revel et al ECG gated CTPA to assess pulmonary artery distensibility CS Max – CS Min x 100 CS Max 16.5% Sensitivity 86%; specificity 96% RadioGraphics 2012; 32:9–32

Cardiac signs:

Cardiac signs RV hypertrophy – wall thickness >4mm Straightening or leftward bowing of interventricular septum RV dilatation – RV to LV diameter ratio more than 1:1 ( midventricular level axial images) Decreased RV ejection fraction Dilatation of IVC and hepatic veins Pericardial effusion RadioGraphics 2012; 32:9–32

Cardiac signs:

Cardiac signs RadioGraphics 2012; 32:9–32

Parenchymal signs:

Parenchymal signs Centrilobular ground glass nodules – common in IPAH Cholesterol granulomas due to ingestion of RBC by macrophages due to repeated pulmonary hemorrhage Neovascularity Tiny serpiginous intrapulmonary vessels emerging from centrilobular arterioles, not conforming to usual pulmonary arterial anatomy RadioGraphics 2012; 32:9–32

Key point:

Key point Catheterization of right side of heart – reference standard for diagnosing pulmonary hypertension Pulmonary pressures, resistance & cardiac output Invasive, involves radiation, no morphological information . RadioGraphics 2012; 32:9–32

Cardiac MR imaging :

Cardiac MR imaging RadioGraphics 2012; 32:9–32

Long standing left-to-right shunt (Group 1):

Long standing left-to-right shunt (Group 1) Congenital systemic to pulmonary shunts which may be surgically corrected or treated. Most intra cardiac shunts detected in ECHO (ASD, VSD) Some left-to-right shunts like sinus venosus defects, PDA and anomalous pulmonary venous return may be missed RadioGraphics 2012; 32:9–32

PowerPoint Presentation:

RadioGraphics 2012; 32:9–32

Pulmonary capillary hemangiomatosis and veno occlusive disease (Group 1’):

Pulmonary capillary hemangiomatosis and veno occlusive disease (Group 1’) Rare, seen in children, young adults Definitive diagnosis – lung biopsy HRCT Thickened interlobular septa, poorly defined centrilobular ground glass nodules, pleural effusion, lymphadenopathy Important to identify as patients may develop fatal pulmonary edema secondary to vasodilator agents RadioGraphics 2012; 32:9–32

PowerPoint Presentation:

RadioGraphics 2012; 32:9–32

Left sided heart disease (Group 2):

Left sided heart disease (Group 2) Backward transmission of elevated LA pressure into pulmonary venous circulation Left ventricular systolic or diastolic dysfunction, valvular disorders, left atrial tumors TTE – modality of choice, incidentally may be seen in CTPA RadioGraphics 2012; 32:9–32

Lung disease (Group 3):

Lung disease (Group 3) Restrictive and obstructive lung diseases COPD, ILD, connective tissue disease, sarcoidosis , pulmonary Langherhan’s cell histiocytosis 50% patients with COPD develop mild pulmonary HTN RadioGraphics 2012; 32:9–32

PowerPoint Presentation:

Combination of emphysema in upper lobes and pulmonary fibrosis in lower lobes is associated with higher prevalence of pulmonary HTN Pulmonary artery diameter to ascending aorta diameter more reliable RadioGraphics 2012; 32:9–32

Chronic thromboembolic disease (Group 4):

Chronic thromboembolic disease (Group 4) 4% patients with acute PTE Symptomatic only when ≥60% of arterial bed is obstructed May be successfully treated with pulmonary thromboendarterectomy Main, lobar or proximal segmental arteries Parenchymal and vascular signs RadioGraphics 2012; 32:9–32

CTPA findings:

CTPA findings RadioGraphics 2012; 32:9–32

Parenchymal signs:

Parenchymal signs RadioGraphics 2012; 32:9–32

Vascular signs:

Vascular signs RadioGraphics 2012; 32:9–32

Role of cardiac MR:

Role of cardiac MR Functional information Asymmetric blood flow to each lung Substantial differences in flow between systemic arterial and pulmonary circulation Secondary to bronchial systemic supply to lungs or intra arterial right to left shunt due to stretching of foramen ovale . Post thromboendarterectomy assessment RadioGraphics 2012; 32:9–32

PowerPoint Presentation:

RadioGraphics 2012; 32:9–32

Primary pulmonary artery sarcoma:

Primary pulmonary artery sarcoma Rare, unilateral Originates in MPA, extends retrograde into RV Filling defects tend to be nodular, form acute angles with arterial wall May completely fill & expand the arterial lumen May extend into lung parenchyma or mediastinum & show delayed enhancement Shows increased FDG uptake in PET RadioGraphics 2012; 32:9–32

Primary pulmonary artery sarcoma:

Primary pulmonary artery sarcoma RadioGraphics 2012; 32:9–32

Takayasu arteritis:

Takayasu arteritis Idiopathic arteritis Involves medium and large vessels Pulmonary artery involvement 50-80% Cardiac MR/ CTPA Smooth, concentric, arterial mural thickening or occlusion Delayed mural contrast enhancement RadioGraphics 2012; 32:9–32

Takayasu arteritis:

Takayasu arteritis RadioGraphics 2012; 32:9–32

Peripheral & central tumor emboli:

Peripheral & central tumor emboli Primary renal cell, hepatocellular , breast, gastric, prostate carcinoma Intravascular filling defects or small, multifocal beading and dilatation of peripheral pulmonary arteries CT helps identify other signs of malignancy ( lymphadenpathy , lymphangitis carcinomatosa , intra abdominal masses) RadioGraphics 2012; 32:9–32

Other conditions (Group 5):

Other conditions (Group 5) Fibrosing mediastinitis Talcosis RadioGraphics 2012; 32:9–32

Summary:

Summary Establishing diagnosis, defining cause, quantifying hemodynamic compromise, therapeutic planning and monitoring Be aware of the classification, the advantages and disadvantages of different modalities and be familiar with the imaging findings RadioGraphics 2012; 32:9–32

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