PMC6624456_01
Right ventricular systolic pressure according to transthoracic echocardiography (mmHg) and 6-min walk distance (m) prior to lung transplant.. Abbreviations: RVSP, right ventricular systolic pressure; 6-MWD, 6-min walk distance.
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After a period with stable cardiopulmonary hemodynamics, her functional status worsened, and she progressed to require 3L of oxygen continuously (Fig. 1).
High resolution computed tomography (HRCT) images pre-transplant. A. gross dilatation of the main pulmonary arteries associated with rapid peripheral tapering, consistent with pulmonary arterial hypertension, and no evident filling defects to indicate venous thromboembolism. B. Cardiomegaly with four chamber enlargement, dilatation of the right ventricle and flattening of the inter-ventricular septum. C. Increased interstitial lung markings consistent with pulmonary edema, but overall preserved pulmonary parenchyma.
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Computed tomography pulmonary embolism protocol did not show any evidence of pulmonary embolus but showed new increased interstitial markings in the lung parenchyma consistent with congestive heart failure (Fig. 2).
Pathological specimens from lung explants demonstrating features of pulmonary arterial hypertension. A. Low-power micrograph of a plexiform-dilation lesion. The arrow indicates the classical "glomeruloid-like" plexus of narrow slit-like channels lined by endothelium. The arrowheads indicate the post-stenotic dilation and angiomatoid lesions typifying plexogenic arteriopathy. Original magnification 40X. Movat pentachrome stain. B. Intra-lobular arteriole (arrow) showing severe concentric laminar intimal fibrosis (arrow). Note the tunica media of normal thickness. Movat pentachrome stain; original magnification 100x.
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The explanted lungs demonstrated severe pulmonary arterial hypertensive changes, including intimal proliferation, plexiform and angiomatoid lesions (Fig. 3).