Magnetic resonance imaging of pulmonary hypertension

Figures

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  Figure 1. A 10-year-old girl with partial anomalous connection of the right lower pulmonary vein to the right atrium with imaging findings of pulmonary hypertension (images courtesy of Kantha Bopha Children’s Hospital, Phnom Penh, Cambodia). a Axial static steady-state free-precession (SSFP) localiser image shows a dilated pulmonary trunk and branch pulmonary arteries. The pulmonary artery (MPA) to ascending aorta (AO) diameter ratio is increased (34 mm/19 mm = 1.8). b, c Axial (b) and short-axis (c) end-systolic SSFP cine images show a dilated and hypertrophic right ventricle with increased diameters of the right ventricle (RV) exceeding that of the left ventricle (LV) (arrows in (b)—RV diameter > LV diameter), leftward bowing of the interventricular septum, and thickened right ventricular myocardium (asterisk). d Posterior volume-rendered, contrast-enhanced angiographic image illustrates dilatation of the central pulmonary arteries (RPA, LPA) with pruning of the peripheral pulmonary arteries. e Coronal-oblique contrast-enhanced angiographic image shows the anomalous connection of the left lower pulmonary vein (asterisk) to the right atrium (RA) above the junction to the inferior vena…

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  Figure 2. A 5-month-old girl with trisomy 21 with echocardiographic findings of pulmonary hypertension and atrial septum defect (ASD). a Steady-state free-precession (SSFP) cine image in the horizontal long-axis plane (4-chamber view) shows the dilated right atrium (RA) and right ventricle (RV), a normal-sized left ventricle (LV), and an atrial septum defect (asterisk). The right ventricular myocardium is not thickened (arrow). b Volumetry in the short-axis plane at end-diastole shows an increased right ventricular end-diastolic volume index (RV EDV 81 ml/m2, z-value 5; LV EDV 41 ml/m2, z-value -1). c Coronal 4-D flow MR velocity map illustrates flow measurements in the main pulmonary artery (MPA) and ascending aorta (AAO) showing increased pulmonary blood flow with QP/QS = 2/1. d Anterior volume rendered angiographic view at peak contrast enhancement shows normal pulmonary vasculature. Transit time measured between the main pulmonary artery (MPA) and ascending aorta (AAO) is normal (3.4 s), which indicates a normal pulmonary vascular resistance

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  Figure 3. Scatterplot showing pulmonary transit times between the pulmonary trunk (MPA) and ascending aorta (AAO), obtained from dynamic contrast-enhanced time-resolved magnetic resonance angiography in 32 children without pulmonary hypertension (median age 5 years, age range 1 month to 17.8 years). Regression equation y = 2.6886 + 0.09991x, R2 0.3085. Dotted lines show 95% confidence intervals and outer lines 95% prediction intervals

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  Figure 4. A 2-year-old boy with cystic fibrosis and occlusion of the left pulmonary veins. a Coronal perfusion map shows homogenous perfusion of the right lung, but no perfusion of the peripheral areas of the left lung (arrows). The right pulmonary artery (RPA) is dilated and the left pulmonary artery (LPA) is small. b Coronal and (c) axial proton density-weighted fast spin echo images show a small left lung with peripheral reticular interstitial pattern, pleural thickening, and ill-defined increased perihilar soft tissue (asterisk in (b)), which likely is caused by diffuse pulmonary to systemic collateral vessels

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  Figure 5. A 2-year-old boy with severe bronchopulmonary dysplasia. a Axial proton density-weighted fast spin echo and (b) axial zero echo time images show architectural distortion with atelectatic areas side by side with hyperinflated areas (asterisk). c Coronal zero echo time image shows the normal trachea and central bronchi, mosaic pattern of lung signal intensity, and an air-filled cyst (asterisk). d Coronal perfusion map shows no perfusion in the air-filled cyst (asterisk) and decreased perfusion in the periphery of the right upper lobe (arrows)

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  Figure 6. A 12-year-old boy with bronchiolitis obliterans. a Coronal zero echo time image shows mosaic signal intensity with decreased lung signal in both upper lobes (asterisk). b Coronal subtraction angiographic image at peak lung enhancement shows corresponding perfusion defects (asterisk). c Coronal perfusion map shows corresponding decreased blood volume (asterisk). The pulmonary transit time between the pulmonary trunk and ascending aorta is normal (3 s), which indicates a normal pulmonary vascular resistance

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  Figure 7. A 17-year-old female with primary ciliary dyskinesia. a Axial respiratory gated T2-weighted fast spin echo image shows characteristic consolidation (asterisk) in the middle lobe and bronchial wall thickening of lower lobe segmental bronchi. b Coronal respiratory gated proton density-weighted fast spin echo image shows mucus plugging and tree-in-bud formations in the medial basal segment. c Coronal angiographic image and signal-time curves from the pulmonary trunk (MPA, yellow region of interest and curve) and ascending aorta (AAO, blue region of interest and curve) illustrate measurement of the transit time between peak enhancement of the MPA and AAO. The pulmonary transit time is prolonged (9.7 s), which indicates an increased pulmonary vascular resistance