Clinical–imaging–pathological correlation in pulmonary hypertension associated with left heart disease

Figures

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  FIGURE 1. Case 1. Pulmonary hypertension associated with heart failure with preserved ejection fraction: echocardiography and computed tomography (CT). a) Transthoracic echocardiography shows right atrial (RA) and right ventricular (RV) dilatation. In addition, the left atrium (LA) is dilated. Left ventricular (LV) dimensions and systolic function are preserved. b) Maximum tricuspid regurgitation velocity measured by continuous-wave Doppler over the tricuspid valve indicates elevated pulmonary pressures. c) Pulsed-wave (PW) Doppler over the RV outflow tract demonstrates a short acceleration time and a mid-systolic notch (arrows). d) LV diastolic function is severely impaired as illustrated by an increased E:A ratio measured by PW Doppler over the mitral valve inflow. Tissue doppler imaging demonstrates reduced e′ velocity over the e) septal wall and f) lateral LV wall. g) CT of the thorax shows a dilated LA. h) CT shows no signs of parenchymal lung disease.

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  FIGURE 2. Case 1. Pulmonary hypertension (PH) associated with heart failure with preserved ejection fraction: lung histopathology. a, c, d) Haematoxylin and eosin staining and b) Elastica van Gieson staining; magnification ×100 (all). a) Collagen-rich remodelling with constrictive intimal fibrosis of a septal vein; the small branch (arrowhead) beneath the larger vein (arrow) appears to be completely occluded. b) Arterialisation of a septal vein with duplication of the elastic lamina (arrowheads): these veins resemble arteries, but lack the adjacent bronchioles; note the beige oedema filling the alveoli in part. c) Two pulmonary arterial branches (and their adjacent bronchioles), with substantial intimal and medial thickening and representing the morphological correlate to the pre-capillary PH component. d) Capillary congestion with the beginning of haemangiomatosis-like changes (area below the dotted line); note the numerous haemosiderophages (arrowheads) and the ossified fragment (arrow), both typical in long-lasting congestion due to left heart failure.

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  FIGURE 3. An overview of the diagnostic evaluation of patients with heart failure with preserved ejection fraction (HfpEF). Reproduced from Borlaug et al. [17] with permission. BMI: body mass index; BNP: brain natriuretic peptide; echo: echocardiography; E/e′: early diastolic transmitral flow velocity/mitral annular early wall motion velocity ratio; NT-proBNP: N-terminal pro-brain natriuretic peptide; H2FPEF: heavy, hypertension, atrial fibrillation, pulmonary hypertension, elder age, elevated filling pressures.

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  FIGURE 4. Case 2. Pulmonary hypertension (PH) related to primary mitral valve regurgitation: echocardiography, cardiac magnetic resonance imaging (MRI) and lung histopathology. a) Transthoracic echocardiography illustrates severe mitral regurgitation. b) Transoesophageal echocardiography illustrates a prolapse of the mitral valve. c) Cardiac MRI shows bi-atrial and biventricular dilatation. d) Cardiac MRI with late-gadolinium enhancement demonstrates fibrosis of the left ventricular (LV) inferolateral wall (arrow). e) Lungs from the patient with PH due to mitral valve disease (autopsy). Haematoxylin and eosin staining (all); magnification: e) ×40 and f) and g) ×400. e) Pulmonary arteries (circle on the left) and pulmonary septal veins (circle on the right) are almost indistinguishable due to venous arterialisation; note the thickened alveolar septa in between that are magnified in g). f) Microvessel, either arteriole or venule, of about 30 µm in diameter; note the perfectly round, single-layered muscularisation of this vessel that is under normal conditions totally devoid of smooth muscle cells. g) Alveolar septa are thickened due to congestion and clear capillary haemangiomatosis, similar…

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  FIGURE 5. Case 3. Pulmonary (arterial) hypertension (P(A)H) with cardiopulmonary comorbidities: echocardiography, computed tomography (CT) and histopathology. a, b) Transthoracic echocardiography shows bi-atrial and right ventricular (RV) dilatation. There is flattening of the interventricular septum. c) High-resolution CT showing minimal parenchymal emphysema, some septal lines, but no (fibrotic) interstitial lung disease. d) Lungs from the patient classified as P(A)H with cardiopulmonary comorbidities (autopsy) d, e, g). Haematoxylin and eosin staining and f) Elastica van Gieson staining; magnification: d) ×40, e) ×100, and f) and g) ×200. d) Pulmonary arteries (arrows) display important medial hyperplasia; congestion is present on every slide, with thick alveolar septa and enlarged capillaries; note the brownish haemosiderophages (top). e) and f) Microvessels are muscularised throughout the lung, down to the smallest pre- and post-capillary level, with near-occlusive remodelling. g) Chronic inflammatory infiltrate can be observed within the interstitium and in the vicinity of remodelled microvessels. LA: left atrium; LV: left ventricle; RA: right atrium.