IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES Rapidly Progressive Pulmonary Hypertension in a Patient with Pulmonary Tumor Thrombotic Microangiopathy Marc Buser1, Monika Felizeter-Kessler2, Daniela Lenggenhager3, and Micha T. Maeder1 1
Division of Cardiology, 2Division of Oncology, and 3Division of Pathology, Kantonsspital St. Gallen, St. Gallen, Switzerland
A 79-year-old woman with metastasizing breast cancer was admitted with progressive dyspnea. Pulmonary embolism was excluded by computed tomography. Echocardiography showed right ventricular dilation and dysfunction (Figure 1A; see Video E1 in the online supplement). Right heart catheterization revealed significant pulmonary hypertension with a mean pulmonary artery pressure of 51 mm Hg (mPAP; Figure 1B). The pulmonary artery occlusion pressure (PAOP) was difficult to measure; the best tracing suggested a mean PAOP (mPAOP) of 34 mm Hg (Figure 1C); the left ventricular enddiastolic pressure (LVEDP) was 8 mm Hg (Figure 1D; same scale, in mm Hg, for Figures 1B–1D). In the following days, the patient experienced recurrent syncope on minimal exertion. Follow-up echocardiography revealed progression of right ventricular dilation and dysfunction (Figure 1E; see Video E2). Despite Figure 1. (A) Transthoracic echocardiography, apical four-chamber view showing right ventricular off-label treatment with ambrisentan, the dilation and dysfunction (LV = left ventricle, RV = right ventricle, LA = left atrium, RA = right atrium; see patient died 10 days later. At autopsy, Video E1). (B–D) Pressure tracings from right and left heart catheterization (dashed red line: mPAP = multiple peripheral pulmonary arteries mean pulmonary artery pressure, mPAOP = mean pulmonary artery occlusion pressure, LVEDP = left were densely packed with intraluminal tuventricular end-diastolic pressure; same scale, in mm Hg, for B–D). (E) Follow-up transthoracic mor cells (Figure 1F; hematoxylin–eosin; echocardiography, apical four-chamber view, revealing progression of right ventricular dilation and dysfunction (same abbreviations as in A; see Video E2). (F) Histology of peripheral pulmonary artery original magnification, 320) immunoposidensely packed with intraluminal tumor cells (hematoxylin–eosin; original magnification, 320). (G) tive for estrogen receptor (Figure 1G; origPeripheral pulmonary artery packed with intraluminal tumor immunopositive for estrogen receptor inal magnification, 320), consistent with (original magnification, 320). (H) Pulmonary arteriole completely occluded due to fibrin thrombi and tumor emboli of the metastasizing breast concentric stenotic fibrocellular intimal proliferation (elastic van Gieson; original magnification, 340). carcinoma; as a morphologic sign of the locally activated coagulation cascade, multiple pulmonary arterioles showed complete occlusion (Figure 1H; elastic van Gieson; original magnification, 340) due to fibrin thrombi and concentric stenotic fibrocellular intimal proliferation. Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare and often fatal form of pulmonary hypertension. The discordance between PAOP and LVEDP is an intriguing hemodynamic feature. Because of thrombotic occlusion of the pulmonary terminal vascular bed the backward transmission of left atrial pressure to the balloon catheter is disrupted; rather, the measured “PAOP” represents the reflected
This article has an online supplement, which is accessible from this issue’s table of contents at www.atsjournals.org Am J Respir Crit Care Med Vol 191, Iss 6, pp 711–712, Mar 15, 2015 Copyright © 2015 by the American Thoracic Society DOI: 10.1164/rccm.201501-0004IM Internet address: www.atsjournals.org
Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences
711
IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES pulmonary artery pressure waveform (1). The diagnosis in patients with PTTM is most often made postmortem. Cytology of blood specimens aspirated from a “wedged” position by the pulmonary artery catheter might allow obtaining the diagnosis premortem in selected cases if there is a high suspicion of PTTM already at the time of cardiac catheterization (2). n Author disclosures are available with the text of this article at www.atsjournals.org.
References 1. McCabe JM, Bhave PD, McGlothlin D, Teerlink JR. Running from her past: a case of rapidly progressive dyspnea on exertion. Circulation 2011;124:2355–2361.
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2. Abati A, Landucci D, Danner RL, Solomon D. Diagnosis of pulmonary microvascular metastases by cytologic evaluation of pulmonary artery catheter-derived blood specimens. Hum Pathol 1994;25: 257–262.
American Journal of Respiratory and Critical Care Medicine Volume 191 Number 6 | March 15 2015
Division of Cardiology, 2Division of Oncology, and 3Division of Pathology, Kantonsspital St. Gallen, St. Gallen, Switzerland
A 79-year-old woman with metastasizing breast cancer was admitted with progressive dyspnea. Pulmonary embolism was excluded by computed tomography. Echocardiography showed right ventricular dilation and dysfunction (Figure 1A; see Video E1 in the online supplement). Right heart catheterization revealed significant pulmonary hypertension with a mean pulmonary artery pressure of 51 mm Hg (mPAP; Figure 1B). The pulmonary artery occlusion pressure (PAOP) was difficult to measure; the best tracing suggested a mean PAOP (mPAOP) of 34 mm Hg (Figure 1C); the left ventricular enddiastolic pressure (LVEDP) was 8 mm Hg (Figure 1D; same scale, in mm Hg, for Figures 1B–1D). In the following days, the patient experienced recurrent syncope on minimal exertion. Follow-up echocardiography revealed progression of right ventricular dilation and dysfunction (Figure 1E; see Video E2). Despite Figure 1. (A) Transthoracic echocardiography, apical four-chamber view showing right ventricular off-label treatment with ambrisentan, the dilation and dysfunction (LV = left ventricle, RV = right ventricle, LA = left atrium, RA = right atrium; see patient died 10 days later. At autopsy, Video E1). (B–D) Pressure tracings from right and left heart catheterization (dashed red line: mPAP = multiple peripheral pulmonary arteries mean pulmonary artery pressure, mPAOP = mean pulmonary artery occlusion pressure, LVEDP = left were densely packed with intraluminal tuventricular end-diastolic pressure; same scale, in mm Hg, for B–D). (E) Follow-up transthoracic mor cells (Figure 1F; hematoxylin–eosin; echocardiography, apical four-chamber view, revealing progression of right ventricular dilation and dysfunction (same abbreviations as in A; see Video E2). (F) Histology of peripheral pulmonary artery original magnification, 320) immunoposidensely packed with intraluminal tumor cells (hematoxylin–eosin; original magnification, 320). (G) tive for estrogen receptor (Figure 1G; origPeripheral pulmonary artery packed with intraluminal tumor immunopositive for estrogen receptor inal magnification, 320), consistent with (original magnification, 320). (H) Pulmonary arteriole completely occluded due to fibrin thrombi and tumor emboli of the metastasizing breast concentric stenotic fibrocellular intimal proliferation (elastic van Gieson; original magnification, 340). carcinoma; as a morphologic sign of the locally activated coagulation cascade, multiple pulmonary arterioles showed complete occlusion (Figure 1H; elastic van Gieson; original magnification, 340) due to fibrin thrombi and concentric stenotic fibrocellular intimal proliferation. Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare and often fatal form of pulmonary hypertension. The discordance between PAOP and LVEDP is an intriguing hemodynamic feature. Because of thrombotic occlusion of the pulmonary terminal vascular bed the backward transmission of left atrial pressure to the balloon catheter is disrupted; rather, the measured “PAOP” represents the reflected
This article has an online supplement, which is accessible from this issue’s table of contents at www.atsjournals.org Am J Respir Crit Care Med Vol 191, Iss 6, pp 711–712, Mar 15, 2015 Copyright © 2015 by the American Thoracic Society DOI: 10.1164/rccm.201501-0004IM Internet address: www.atsjournals.org
Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences
711
IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES pulmonary artery pressure waveform (1). The diagnosis in patients with PTTM is most often made postmortem. Cytology of blood specimens aspirated from a “wedged” position by the pulmonary artery catheter might allow obtaining the diagnosis premortem in selected cases if there is a high suspicion of PTTM already at the time of cardiac catheterization (2). n Author disclosures are available with the text of this article at www.atsjournals.org.
References 1. McCabe JM, Bhave PD, McGlothlin D, Teerlink JR. Running from her past: a case of rapidly progressive dyspnea on exertion. Circulation 2011;124:2355–2361.
712
2. Abati A, Landucci D, Danner RL, Solomon D. Diagnosis of pulmonary microvascular metastases by cytologic evaluation of pulmonary artery catheter-derived blood specimens. Hum Pathol 1994;25: 257–262.
American Journal of Respiratory and Critical Care Medicine Volume 191 Number 6 | March 15 2015
