0
ONLINE EXCLUSIVES
Chest Imaging and Pathology for Clinicians |

A 57-Year-Old Man With Insidious Dyspnea and Nonpleuritic Chest and Back Pain FREE TO VIEW

Kenta Nakamura, MD; George A. Alba, MD; Jonathan A. Scheske, MD; Nandini M. Meyersohn, MD; James R. Stone, MD, PhD; Gus J. Vlahakes, MD; Cameron D. Wright, MD; Brian B. Ghoshhajra, MD; David M. Dudzinski, MD, JD
Author and Funding Information

aDivision of Cardiology, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, MA

bDivision of Pulmonary and Critical Care Medicine, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, MA

cCardiovascular Imaging, Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA

dDepartment of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA

eDivision of Thoracic Surgery, Department of Surgery, Massachusetts General Hospital, Harvard Medical School, Boston, MA

fDivision of Cardiothoracic Surgery, Department of Surgery, Massachusetts General Hospital, Harvard Medical School, Boston, MA

CORRESPONDENCE TO: David M. Dudzinski, MD, JD, Massachusetts General Hospital, Corrigan Minehan Heart Center and Pulmonary Embolism Response Team, 55 Fruit St, Yawkey 5B, Boston, MA 02114


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2016;150(2):e41-e47. doi:10.1016/j.chest.2016.02.680
Text Size: A A A
Published online

A 57-year-old man with a history of DVT and pulmonary embolism, transient ischemic attacks, prior 60 pack-year smoking history, and oxygen-dependent COPD presented with insidiously worsening dyspnea associated with new pleuritic chest and back pain.

Figures in this Article

Three years before presentation, the patient developed left lower extremity edema shortly following air travel and he was ultimately diagnosed with a DVT and concomitant pulmonary embolism (PE). Additional evaluation revealed emphysema, COPD, and hypoxemia necessitating 3 to 4 L/min of supplemental oxygen. He was anticoagulated with Coumadin until 1 year before presentation when he suffered a mechanical fall with multiple rib fractures and anticoagulation was discontinued. He was otherwise treated for suspected oxygen-dependent COPD until the index presentation with acutely worsened dyspnea and chest pain.

On presentation, vital signs were remarkable for hypotension to 87/59 mm Hg, tachypnea to 24 breaths/min, and hypoxemia requiring 6 L/min supplemental oxygen by nasal cannula to maintain an oxygen saturation of 96%; he was afebrile and heart rate was 89 beats/min. Cardiac auscultation was remarkable for a III/VI holosystolic murmur best appreciated along the left sternal border and intensified with inspiration. Right ventricular (RV) lift and pulmonary artery “tap” were absent. Jugular venous pressure was elevated at 13 cm H2O (8 cm above the sternal angle of Louis). There was 2+ pitting edema of the bilateral lower extremities. Initial laboratory studies were notable for acute renal failure (creatinine, 2.1 mg/dL; reference, 0.60-1.50 mg/dL), elevated aminotransferases (aspartate transaminase, 2,801 U/L; alanine transaminase, 1,275 U/L; reference, 10-40 U/L; 10-55, U/L, respectively), elevated lactate (4.7 mmol/L; reference, 0.5-2.2 mmol/L), and elevated D-dimer (4970 ng/mL; reference, 110-250 ng/mL). Electrocardiogram revealed normal sinus rhythm with an ectopic atrial focus and premature atrial contraction, biatrial enlargement, nonspecific ST/T-wave changes in the right precordial (V1-4) leads, and right bundle branch block (Fig 1).

Figure 1
Figure Jump LinkFigure 1 Electrocardiogram showing biatrial enlargement and right bundle branch block.Grahic Jump Location

An anteroposterior chest radiograph was underpenetrated but remarkable for diffuse interstitial pulmonary markings suggestive of pulmonary edema, prominence of the pulmonary arteries, and suggestion of enlarged cardiac silhouette (Fig 2). Transthoracic echocardiogram demonstrated a 5.4-cm serpiginous echodensity seen in the both right and left atria straddling a probable patent foramen ovale (PFO) (Fig 3A, 3B; Video 1). The interatrial septum was displaced leftward in both systole and diastole and the right ventricle appeared dilated and hypokinetic with interventricular septal flattening. Moderate tricuspid regurgitation and a markedly elevated estimated pulmonary artery systolic pressure of 59 mm Hg plus estimated central venous pressure were noted. Contrast-enhanced CT imaging of the chest showed a hypodense mobile mass traversing the interatrial septum and additional filling defects in the RV apex (Fig 3C) and the right pulmonary arteries (Fig 4A). Mosaic attenuation (Fig 4B) and occlusive thrombi of segmental arteries were noted in the lower lobes of the right lung. The main pulmonary artery was enlarged at 37 mm in diameter (normal, < 29 mm). The right atrium and ventricle again appeared dilated with interventricular septal flattening and probable RV hypertrophy. Extensive emphysema and pulmonary edema were also noted.

Figure 2
Figure Jump LinkFigure 2 Chest radiograph showing diffusely increased reticular markings, prominence of the pulmonary arteries, and enlarged cardiac silhouette.Grahic Jump Location
Figure 3
Figure Jump LinkFigure 3 Transthoracic echocardiogram showing a 5.4-cm linear echodensity in the right atrium and left atrium straddling a patent foramen ovale (A, apical four-chamber view; B, subcostal four-chamber view). See Video 1 for cine. Contrast-enhanced CT scan of the chest shows a mobile mass extending across a patent foramen ovale as well as right atrial and right ventricular dilation and right ventricular hypertrophy (C), with a low attenuation filling defect in the RV apex concerning for thrombus. LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.Grahic Jump Location
Figure 4
Figure Jump LinkFigure 4 A, Contrast-enhanced CT imaging for pulmonary embolism showed a large eccentric filling defect in the main right-sided pulmonary artery as well as significantly dilated pulmonary artery. B, Color-inverted CT image showed a pattern of mosaic attenuation in the lower lobes of the right lung, suggestive of chronic thromboembolic pulmonary hypertension.Grahic Jump Location

Given the inherently unstable nature of thrombus-in-transit across the interatrial septum and in the RV cavity, the patient was taken for comprehensive surgical thromboembolectomy to remove thrombi from both atria and the right ventricle; thromboendarterectomy of the pulmonary, lobar, and subsegmental arteries; and closure of the PFO. Thrombus-in-transit was confirmed in the right atrium extending into the left atrium through a large PFO. Upon gross inspection, the right pulmonary artery and tributaries were occluded with extensive subacute and chronic material (Fig 5A). Following endarterectomy, the artery appeared thickened and inflamed with substantial scar tissue. Histology of the thrombus retrieved from the pulmonary artery revealed both organizing and unorganized thrombus with focal areas of intimal hyperplasia suggestive of at least a subacute process, consistent with presence of thrombosis of varying ages from acute to chronic (Fig 5B, 5C).

Figure 5
Figure Jump LinkFigure 5 A, Gross photograph of material retrieved from the right-sided pulmonary artery and tributaries included extensive subacute and chronic thrombus. Histology of the thrombus shows both unorganized (B) and organized (C) thrombus components. Intimal hyperplasia was also noted, consistent with the presence of organized thrombus and suggestive of chronicity (yellow arrows).Grahic Jump Location

What is the diagnosis?

Diagnosis: VTE causing acute, subacute, and chronic PE complicated by chronic thromboembolic pulmonary hypertension (CTEPH).

Clinical Discussion
The history of significant hypoxemia and significant pulmonary hypertension should raise suspicion for alternative diagnoses other than COPD. A comprehensive evaluation for additional etiologies of persistent hypoxemia and pulmonary hypertension following VTE and PE was warranted, including consideration for CTEPH. In review of the patient’s chart, an echocardiogram that had been performed at the time of initial PE several years before presentation revealed elevated estimated pulmonary artery systolic pressure. In the absence of evidence for left heart failure, such as enlarged left atrium, aortic or mitral valvulopathy, or left ventricular dysfunction, an echocardiogram suggestive of pulmonary hypertension warrants additional diagnostic investigation. Preliminary evaluation typically includes pulmonary function tests, ventilation-perfusion (V˙ /Q˙ ) (or perfusion-only) scintigraphy scan, overnight oximetry and polysomnography, and laboratory testing including autoimmune serologies, HIV, and liver function tests.,,V˙ /Q˙  scanning has been longer established than contrast-enhanced CT scanning for chronic thromboemboli; in this case, the CT scan is clearly preferable in this patient with extensive COPD and abnormal underlying lung parenchyma and ventilation, both factors that are known to decrease the sensitivity of scintigraphy. Although COPD was diagnosed at the time, the severity of hypoxemia was disproportionate to the parenchymal findings, and evaluation of pulmonary hypertension and consideration for confirmatory right heart catheterization were appropriate.

The patient’s current unstable presentation 3 years later was consistent with acute PE as manifested clinically by chest pain, tachypnea, and hypoxemia. The prior history of VTE was an important historical risk factor for recurrence. Hypotension with biochemical evidence of end-organ dysfunction constituted shock resulting from cardiovascular compromise. The patient was felt to have “massive” or “high-risk” PE based on hemodynamic instability and prolonged hypotension. Classification of PE stratifies patients into those at high risk for death that warrant consideration of invasive and/or thrombolytic therapies as opposed to conservative management with anticoagulation alone.,, However, the presence of thrombus-in-transit with embolization to the left heart, presumably acute, as visualized on echocardiography and CT imaging, presented an urgent need for procedural intervention to mitigate risk of further embolization with high likelihood of systemic embolization. Thrombus-in-transit is a relatively rare finding, observed in 4% of all patients with PE, but in 18% in those requiring intensive care. Transit of thrombus through an intracardiac shunt into the left atrium is rarer. The scenarios of mobile thrombus are not directly addressed in treatment guidelines,; however, thrombus-in-transit carries high mortality and warrants aggressive intervention.,, Treatment strategy for mobile thrombus may be generally stratified according to hemodynamic status (Table 1), but requires careful multidisciplinary discussion and care coordination.,, At our institution and others, a Pulmonary Embolism Response Team has been organized for questions such as mobile thrombus where patient-specific decision-making is required to integrate comorbidities, hemodynamic status, burden of disease, need for additional surgical or percutaneous procedures, institutional-specific expertise with surgical or percutaneous procedures, and risk of undertreatment with anticoagulation alone or a percutaneous approach.,,, Given impaired hemodynamics and oxygen exchange, this patient would likely not have tolerated the additional burden of emboli to the pulmonary circulation and ongoing risk of paradoxical arterial embolization predisposing to myocardial infarction and further stroke.,,

Table Graphic Jump Location
Table 1 Proposed Treatment Strategy for Thrombus-in-Transit (Right Heart Thrombi) in Acute Pulmonary Embolism, Classified According to Hemodynamic Status and Surgical Riska,,,,
a Optimal treatment strategy requires patient-specific, multidisciplinary discussion and care coordination. There are no consensus guidelines because of a paucity of data and so treatment must be individualized for high-risk patient scenarios, for example in the case of impending systemic embolization as in the patient presented.
Imaging Discussion

Echocardiography and contrast-enhanced CT clearly demonstrated thrombus-in-transit with dramatic visualization of a highly mobile and unstable right atrial thrombus traversing a PFO and reaching into the left atrium (Fig 3). CT also revealed a large thrombus in the RV apex not clearly visualized on echocardiogram, highlighting the importance of multimodality imaging in the management of complex thromboembolic disease. The large eccentric filling defect in the main right-sided pulmonary artery (Fig 4A) and occlusive thrombi in the segmental arteries of the right middle and lower lobes were consistent with both acute/subacute and chronic PE, respectively. Furthermore, the mosaic pattern of attenuation noted on the right lower lung field suggests irregular perfusion and strongly supports CTEPH (Fig 4B), albeit confounded in this case by the expected hypoperfusion in segments with underlying parenchymal abnormality resulting from emphysema.

The imaging is suggestive of both acute and chronic RV failure. A significantly dilated main pulmonary artery at 37 mm (normal, < 29 mm) together with RV dilation and septal flattening may suggest acute RV strain. Demonstration of RV hypertrophy supports a component of chronic cardiopulmonary insult because hypertrophy cannot develop immediately following acute PE. The estimated pulmonary artery systolic pressure in excess of 50 mm Hg makes pulmonary hypertension a likely explanation. The absence of left atrial enlargement argues against chronically elevated left ventricular filling pressure or volume as an etiology of both right heart failure and elevated pulmonary artery pressure. In considering the patient’s prior history of VTE and PE, his preexisting oxygen dependence may have been better explained by occult CTEPH than COPD alone.

PFO is a congenital vestige of the left-to-right atrial shunt physiologically necessary in utero but may persist in up to one-third of adults. The slitlike flap of the interatrial septum typically seals shortly after birth with reversal of right- and left-sided filling pressures following the first breaths of life and dramatic reduction in pulmonary vascular resistance. The majority of PFOs are undetected and asymptomatic because higher left atrial pressure prevents right-to-left flow of blood under normal physiologic conditions. However, increased right heart chamber pressures may cause paradoxical right-to-left flow across the PFO. The systolic murmur with respiratory augmentation suggests a right-sided lesion and taken together with left sternal location is highly suggestive of tricuspid regurgitation, consistent with RV dysfunction, dilation, and pressure overload. We believe that the patient in our study presented with preexisting RV pressure overload resulting from occult CTEPH and was thus at increased risk for paradoxical embolism through the PFO; this phenomenon may also explain the patient’s history of transient ischemic attacks.

Pathologic Discussion

Gross pathology of the pulmonary artery thrombectomy specimen was consistent with subacute and chronic thromboemboli with evidence of remodeling and scar formation of the right-sided pulmonary and segmental arteries. Histopathology supported this diagnosis as evidenced by organized thrombus, though the thrombus-in-transit retrieved from the atria showed components of unorganized and thus acute thrombus. Extensive involvement of the proximal pulmonary vasculature was consistent with the diagnosis of CTEPH as opposed to primary pulmonary arterial hypertension, which typically involves distal, smaller arteries < 300 μm in size. The development of CTEPH is thought to be a secondary arteriopathy, grossly mediated by thromboemboli resulting in arterial occlusion, inadequate thrombus resolution, and inflamed and scarred arterial tissue. Right-sided cardiomyopathy is observed with CTEPH, likely through the pathophysiology of increased pulmonary vascular resistance leading to RV dilation and dysfunction., Given the chronicity of CTEPH, the right ventricle adversely remodels; both dilation and hypertrophy were observed in the patient in our study, findings that would be unusual for an isolated acute process. The mixed acute and chronic nature of this patient’s thromboembolic pathologies is explained by thromboembolic phenomena over the course of hours to years. In summary, this patient likely developed CTEPH in the years following initial VTE and PE and presented several years later with recurrent acute and subacute PE and thrombus-in-transit.

Treatment Discussion
In general, the sequelae of acute PE, even those with hemodynamic significance and RV compromise, typically normalize with anticoagulation within 6 weeks of PE and resolves without significant long-term sequelae. A small minority of acute PE patients, approximately 1% to 8%, progress to CTEPH. Given the persistent involvement of the larger, proximal vasculature typically observed in CTEPH, there is an opportunity for surgical revascularization with pulmonary thromboendarterectomy to confer a survival benefit., If deemed inoperable, balloon angioplasty or medical therapy alone may be considered as alternatives. The patient in our study underwent thromboendarterectomy at the time of thromboembolectomy for acute thrombi. Despite this aggressive revascularization of all lobar and subsegmental pulmonary arteries, a V˙ /Q˙  scintigraphy scan performed 1 week following surgery revealed an unmatched V˙ /Q˙  defect in the right lower lobe (Fig 6) consistent with persistent chronic thromboembolism, likely from distal thrombotic burden or irreversible vasculopathy not reversible with surgery alone. A 2-week postoperative echocardiogram revealed a persistently dilated and hypokinetic RV with an elevated pulmonary artery systolic pressure of 69 mm Hg plus central venous pressure. Right heart catheterization revealed persistent precapillary pulmonary hypertension (pulmonary artery systolic pressure 55 mm Hg, mean pulmonary artery pressure of 37 mm Hg, pulmonary artery occlusion pressure of 10 mm Hg, pulmonary vascular resistance of 7.8 Wood units, and reduced cardiac index of 1.89 L/min/m2). The patient was ultimately discharged to home on the inhaled vasodilator treprostinil four times daily, oral sildenafil, and continued supplemental oxygen at 6 L/min. Persistent pulmonary hypertension following pulmonary thromboendarterectomy occurs in up to one-third of patients at 3 month follow-up resulting from distal, surgically inaccessible thrombus or irreversibly impaired vasculopathy.,, Nevertheless, surgical intervention confers mortality benefit compared with medical therapy alone despite persistence of pulmonary hypertension.
Figure 6
Figure Jump LinkFigure 6 Ventilation-perfusion scintigraphy scan 1 week following comprehensive surgical thromboendarterectomy of all lobar and segmental pulmonary arteries showed an unmatched ventilation perfusion defect in the right (R) lower lobe (arrow) consistent with persistent chronic thromboembolism and chronic thromboembolic pulmonary hypertension. A matched defect in the right lower lobe (*) is suggestive of atelectasis. L = left.Grahic Jump Location

The patient in our study exhibited a spectrum of acute, subacute, and chronic PE, representing thromboembolic events hours, days to weeks, and years after initial VTE, respectively. Initial management focused on hemodynamic stabilization and prophylactic retrieval of a right- to left-sided thrombus that posed a catastrophic risk of both pulmonary and systemic embolization. Suspected by history and confirmed at the time of surgery, CTEPH was appropriately considered, and the patient underwent concomitant thromboembolectomy, thromboendarterectomy, and PFO closure. Multidisciplinary expertise and multimodality imaging were instrumental in diagnosis and ultimately identifying the optimal management strategy for this complicated patient.

Financial/nonfinancial disclosures: The authors have reported to CHEST the following: J. R. S. has received payments for expert witness testimony involving cases of PE. None declared (K. N., G. A. A., N. M M., G. J. V., C. D. W., B. B. G., D. M. D.).

Other contributions:CHEST worked with the authors to ensure that the Journal policies on patient consent to report information were met.

Additional information: The Video can be found in the Multimedia section of the online article.

Truong Q.A. .Massaro J.M. .Rogers I.S. .et al Reference values for normal pulmonary artery dimensions by noncontrast cardiac computed tomography: the Framingham Heart Study. Circ Cardiovasc Imaging. 2012;5:147-154 [PubMed]journal. [CrossRef] [PubMed]
 
McLaughlin V.V. .Archer S.L. .Badesch D.B. .et al ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association. Circulation. 2009;119:2250-2294 [PubMed]journal. [CrossRef] [PubMed]
 
Hoeper M.M. .Bogaard H.J. .Condliffe R. .et al Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62:D42-D50 [PubMed]journal. [CrossRef] [PubMed]
 
Galie N. .Hoeper M.M. .Humbert M. .et al Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J. 2009;30:2493-2537 [PubMed]journal. [CrossRef] [PubMed]
 
Jaff M.R. .McMurtry M.S. .Archer S.L. .et al Management of massive and submassive pulmonary embolism, iliofemoral deep vein thrombosis, and chronic thromboembolic pulmonary hypertension: a scientific statement from the American Heart Association. Circulation. 2011;123:1788-1830 [PubMed]journal. [CrossRef] [PubMed]
 
Konstantinides S.V. .Torbicki A. .Agnelli G. .et al 2014 ESC guidelines on the diagnosis and management of acute pulmonary embolism. Eur Heart J. 2014;35:3033-3069 [PubMed]journal. [CrossRef] [PubMed]
 
Kearon C. .Akl E.A. .Ornelas J. .et al Antithrombotic therapy for VTE disease: Chest Guideline and Expert Panel Report. Chest. 2016;149:315-352 [PubMed]journal. [CrossRef] [PubMed]
 
Torbicki A. .Galie N. .Covezzoli A. .et al Right heart thrombi in pulmonary embolism: results from the International Cooperative Pulmonary Embolism Registry. J Am Coll Cardiol. 2003;41:2245-2251 [PubMed]journal. [CrossRef] [PubMed]
 
Casazza F. .Bongarzoni A. .Centonze F. .Morpurgo M. . Prevalence and prognostic significance of right-sided cardiac mobile thrombi in acute massive pulmonary embolism. Am J Cardiol. 1997;79:1433-1435 [PubMed]journal. [CrossRef] [PubMed]
 
Athappan G. .Sengodan P. .Chacko P. .Gandhi S. . Comparative efficacy of different modalities for treatment of right heart thrombi in transit: a pooled analysis. Vasc Med. 2015;20:131-138 [PubMed]journal. [CrossRef] [PubMed]
 
Dudzinski D.M. .Piazza G. . Multidisciplinary pulmonary embolism response teams. Circulation. 2016;133:98-103 [PubMed]journal. [CrossRef] [PubMed]
 
Kabrhel C. .Rempell J.S. .Avery L.L. .Dudzinski D.M. .Weinberg I. . Case records of the Massachusetts General Hospital. Case 29-2014. A 60-year-old woman with syncope. N Engl J Med. 2014;371:1143-1150 [PubMed]journal. [CrossRef] [PubMed]
 
Reza N. .Dudzinski D.M. . Pulmonary embolism response teams. Curr Treatment Options Cardiovasc Med. 2015;17:387- [PubMed]journal
 
Provias T. .Dudzinski D.M. .Jaff M.R. .et al The Massachusetts General Hospital Pulmonary Embolism Response Team (MGH PERT): creation of a multidisciplinary program to improve care of patients with massive and submassive pulmonary embolism. Hosp Pract. 2014;42:31-37 [PubMed]journal
 
Aggarwal K. .Jayam V.K. .Meyer M.A. .Nayak A.K. .Nathan S. . Thrombus-in-transit and paradoxical embolism. J Am Soc Echocardiogr. 2002;15:1021-1022 [PubMed]journal. [CrossRef] [PubMed]
 
Bugra Z. .Hunerel D. .Tayyareci Y. .et al Echocardiographic diagnosis of a giant thrombus passing through a patent foramen ovale from right atrium to the left atrium. J Thromb Thrombolysis. 2008;25:297-299 [PubMed]journal. [CrossRef] [PubMed]
 
King M.A. .Ysrael M. .Bergin C.J. . Chronic thromboembolic pulmonary hypertension: CT findings. AJR Am J Roentgenol. 1998;170:955-960 [PubMed]journal. [CrossRef] [PubMed]
 
Hagen P.T. .Scholz D.G. .Edwards W.D. . Incidence and size of patent foramen ovale during the first 10 decades of life: an autopsy study of 965 normal hearts. Mayo Clinic Proc. 1984;59:17-20 [PubMed]journal. [CrossRef]
 
Lang I.M. .Pesavento R. .Bonderman D. .Yuan J.X. . Risk factors and basic mechanisms of chronic thromboembolic pulmonary hypertension: a current understanding. Eur Respir J. 2013;41:462-468 [PubMed]journal. [CrossRef] [PubMed]
 
Witkin A.S. .Channick R.N. . Chronic thromboembolic pulmonary hypertension: the end result of pulmonary embolism. Curr Cardiol Rep. 2015;17:63- [PubMed]journal. [CrossRef] [PubMed]
 
Delcroix M. .Vonk Noordegraaf A. .Fadel E. .Lang I. .Simonneau G. .Naeije R. . Vascular and right ventricular remodelling in chronic thromboembolic pulmonary hypertension. Eur Respir J. 2013;41:224-232 [PubMed]journal. [CrossRef] [PubMed]
 
Nakamura K. .Bechis M.Z. .Yee A.J. .Dudzinski D.M. . Progressive onset of extracardiac and myocardial symptoms: right heart failure and cor pulmonale in a young man with debilitating polyneuropathy and monoclonal gammopathy. Circulation. 2015;132:59-67 [PubMed]journal. [CrossRef] [PubMed]
 
Ribeiro A. .Lindmarker P. .Johnsson H. .Juhlin-Dannfelt A. .Jorfeldt L. . Pulmonary embolism: one-year follow-up with echocardiography doppler and five-year survival analysis. Circulation. 1999;99:1325-1330 [PubMed]journal. [CrossRef] [PubMed]
 
Jenkins D.P. .Madani M. .Mayer E. .et al Surgical treatment of chronic thromboembolic pulmonary hypertension. Eur Respir J. 2013;41:735-742 [PubMed]journal. [CrossRef] [PubMed]
 
Pepke-Zaba J. .Jansa P. .Kim N.H. .Naeije R. .Simonneau G. . Chronic thromboembolic pulmonary hypertension: role of medical therapy. Eur Respir J. 2013;41:985-990 [PubMed]journal. [CrossRef] [PubMed]
 
Freed D.H. .Thomson B.M. .Berman M. .et al Survival after pulmonary thromboendarterectomy: effect of residual pulmonary hypertension. J Thorac Cardiovasc Surg. 2011;141:383-387 [PubMed]journal. [CrossRef] [PubMed]
 
Condliffe R. .Kiely D.G. .Gibbs J.S. .et al Improved outcomes in medically and surgically treated chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med. 2008;177:1122-1127 [PubMed]journal. [CrossRef] [PubMed]
 
Fedullo P. .Kerr K.M. .Kim N.H. .Auger W.R. . Chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med. 2011;183:1605-1613 [PubMed]journal. [CrossRef] [PubMed]
 
Chartier L. .Bera J. .Delomez M. .et al Free-floating thrombi in the right heart: diagnosis, management, and prognostic indexes in 38 consecutive patients. Circulation. 1999;99:2779-2783 [PubMed]journal. [CrossRef] [PubMed]
 
Goldhaber S.Z. .Visani L. .De Rosa M. . Acute pulmonary embolism: clinical outcomes in the International Cooperative Pulmonary Embolism Registry (ICOPER). Lancet. 1999;353:1386-1389 [PubMed]journal. [CrossRef] [PubMed]
 
Donaldson C.W. .Baker J.N. .Narayan R.L. .et al Thrombectomy using suction filtration and veno-venous bypass: single center experience with a novel device. Catheter Cardiovasc Interv. 2015;86:E81-E87 [PubMed]journal. [CrossRef] [PubMed]
 

Figures

Figure Jump LinkFigure 1 Electrocardiogram showing biatrial enlargement and right bundle branch block.Grahic Jump Location
Figure Jump LinkFigure 2 Chest radiograph showing diffusely increased reticular markings, prominence of the pulmonary arteries, and enlarged cardiac silhouette.Grahic Jump Location
Figure Jump LinkFigure 3 Transthoracic echocardiogram showing a 5.4-cm linear echodensity in the right atrium and left atrium straddling a patent foramen ovale (A, apical four-chamber view; B, subcostal four-chamber view). See Video 1 for cine. Contrast-enhanced CT scan of the chest shows a mobile mass extending across a patent foramen ovale as well as right atrial and right ventricular dilation and right ventricular hypertrophy (C), with a low attenuation filling defect in the RV apex concerning for thrombus. LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.Grahic Jump Location
Figure Jump LinkFigure 4 A, Contrast-enhanced CT imaging for pulmonary embolism showed a large eccentric filling defect in the main right-sided pulmonary artery as well as significantly dilated pulmonary artery. B, Color-inverted CT image showed a pattern of mosaic attenuation in the lower lobes of the right lung, suggestive of chronic thromboembolic pulmonary hypertension.Grahic Jump Location
Figure Jump LinkFigure 5 A, Gross photograph of material retrieved from the right-sided pulmonary artery and tributaries included extensive subacute and chronic thrombus. Histology of the thrombus shows both unorganized (B) and organized (C) thrombus components. Intimal hyperplasia was also noted, consistent with the presence of organized thrombus and suggestive of chronicity (yellow arrows).Grahic Jump Location
Figure Jump LinkFigure 6 Ventilation-perfusion scintigraphy scan 1 week following comprehensive surgical thromboendarterectomy of all lobar and segmental pulmonary arteries showed an unmatched ventilation perfusion defect in the right (R) lower lobe (arrow) consistent with persistent chronic thromboembolism and chronic thromboembolic pulmonary hypertension. A matched defect in the right lower lobe (*) is suggestive of atelectasis. L = left.Grahic Jump Location

Tables

Table Graphic Jump Location
Table 1 Proposed Treatment Strategy for Thrombus-in-Transit (Right Heart Thrombi) in Acute Pulmonary Embolism, Classified According to Hemodynamic Status and Surgical Riska,,,,
a Optimal treatment strategy requires patient-specific, multidisciplinary discussion and care coordination. There are no consensus guidelines because of a paucity of data and so treatment must be individualized for high-risk patient scenarios, for example in the case of impending systemic embolization as in the patient presented.

References

Truong Q.A. .Massaro J.M. .Rogers I.S. .et al Reference values for normal pulmonary artery dimensions by noncontrast cardiac computed tomography: the Framingham Heart Study. Circ Cardiovasc Imaging. 2012;5:147-154 [PubMed]journal. [CrossRef] [PubMed]
 
McLaughlin V.V. .Archer S.L. .Badesch D.B. .et al ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association. Circulation. 2009;119:2250-2294 [PubMed]journal. [CrossRef] [PubMed]
 
Hoeper M.M. .Bogaard H.J. .Condliffe R. .et al Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62:D42-D50 [PubMed]journal. [CrossRef] [PubMed]
 
Galie N. .Hoeper M.M. .Humbert M. .et al Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J. 2009;30:2493-2537 [PubMed]journal. [CrossRef] [PubMed]
 
Jaff M.R. .McMurtry M.S. .Archer S.L. .et al Management of massive and submassive pulmonary embolism, iliofemoral deep vein thrombosis, and chronic thromboembolic pulmonary hypertension: a scientific statement from the American Heart Association. Circulation. 2011;123:1788-1830 [PubMed]journal. [CrossRef] [PubMed]
 
Konstantinides S.V. .Torbicki A. .Agnelli G. .et al 2014 ESC guidelines on the diagnosis and management of acute pulmonary embolism. Eur Heart J. 2014;35:3033-3069 [PubMed]journal. [CrossRef] [PubMed]
 
Kearon C. .Akl E.A. .Ornelas J. .et al Antithrombotic therapy for VTE disease: Chest Guideline and Expert Panel Report. Chest. 2016;149:315-352 [PubMed]journal. [CrossRef] [PubMed]
 
Torbicki A. .Galie N. .Covezzoli A. .et al Right heart thrombi in pulmonary embolism: results from the International Cooperative Pulmonary Embolism Registry. J Am Coll Cardiol. 2003;41:2245-2251 [PubMed]journal. [CrossRef] [PubMed]
 
Casazza F. .Bongarzoni A. .Centonze F. .Morpurgo M. . Prevalence and prognostic significance of right-sided cardiac mobile thrombi in acute massive pulmonary embolism. Am J Cardiol. 1997;79:1433-1435 [PubMed]journal. [CrossRef] [PubMed]
 
Athappan G. .Sengodan P. .Chacko P. .Gandhi S. . Comparative efficacy of different modalities for treatment of right heart thrombi in transit: a pooled analysis. Vasc Med. 2015;20:131-138 [PubMed]journal. [CrossRef] [PubMed]
 
Dudzinski D.M. .Piazza G. . Multidisciplinary pulmonary embolism response teams. Circulation. 2016;133:98-103 [PubMed]journal. [CrossRef] [PubMed]
 
Kabrhel C. .Rempell J.S. .Avery L.L. .Dudzinski D.M. .Weinberg I. . Case records of the Massachusetts General Hospital. Case 29-2014. A 60-year-old woman with syncope. N Engl J Med. 2014;371:1143-1150 [PubMed]journal. [CrossRef] [PubMed]
 
Reza N. .Dudzinski D.M. . Pulmonary embolism response teams. Curr Treatment Options Cardiovasc Med. 2015;17:387- [PubMed]journal
 
Provias T. .Dudzinski D.M. .Jaff M.R. .et al The Massachusetts General Hospital Pulmonary Embolism Response Team (MGH PERT): creation of a multidisciplinary program to improve care of patients with massive and submassive pulmonary embolism. Hosp Pract. 2014;42:31-37 [PubMed]journal
 
Aggarwal K. .Jayam V.K. .Meyer M.A. .Nayak A.K. .Nathan S. . Thrombus-in-transit and paradoxical embolism. J Am Soc Echocardiogr. 2002;15:1021-1022 [PubMed]journal. [CrossRef] [PubMed]
 
Bugra Z. .Hunerel D. .Tayyareci Y. .et al Echocardiographic diagnosis of a giant thrombus passing through a patent foramen ovale from right atrium to the left atrium. J Thromb Thrombolysis. 2008;25:297-299 [PubMed]journal. [CrossRef] [PubMed]
 
King M.A. .Ysrael M. .Bergin C.J. . Chronic thromboembolic pulmonary hypertension: CT findings. AJR Am J Roentgenol. 1998;170:955-960 [PubMed]journal. [CrossRef] [PubMed]
 
Hagen P.T. .Scholz D.G. .Edwards W.D. . Incidence and size of patent foramen ovale during the first 10 decades of life: an autopsy study of 965 normal hearts. Mayo Clinic Proc. 1984;59:17-20 [PubMed]journal. [CrossRef]
 
Lang I.M. .Pesavento R. .Bonderman D. .Yuan J.X. . Risk factors and basic mechanisms of chronic thromboembolic pulmonary hypertension: a current understanding. Eur Respir J. 2013;41:462-468 [PubMed]journal. [CrossRef] [PubMed]
 
Witkin A.S. .Channick R.N. . Chronic thromboembolic pulmonary hypertension: the end result of pulmonary embolism. Curr Cardiol Rep. 2015;17:63- [PubMed]journal. [CrossRef] [PubMed]
 
Delcroix M. .Vonk Noordegraaf A. .Fadel E. .Lang I. .Simonneau G. .Naeije R. . Vascular and right ventricular remodelling in chronic thromboembolic pulmonary hypertension. Eur Respir J. 2013;41:224-232 [PubMed]journal. [CrossRef] [PubMed]
 
Nakamura K. .Bechis M.Z. .Yee A.J. .Dudzinski D.M. . Progressive onset of extracardiac and myocardial symptoms: right heart failure and cor pulmonale in a young man with debilitating polyneuropathy and monoclonal gammopathy. Circulation. 2015;132:59-67 [PubMed]journal. [CrossRef] [PubMed]
 
Ribeiro A. .Lindmarker P. .Johnsson H. .Juhlin-Dannfelt A. .Jorfeldt L. . Pulmonary embolism: one-year follow-up with echocardiography doppler and five-year survival analysis. Circulation. 1999;99:1325-1330 [PubMed]journal. [CrossRef] [PubMed]
 
Jenkins D.P. .Madani M. .Mayer E. .et al Surgical treatment of chronic thromboembolic pulmonary hypertension. Eur Respir J. 2013;41:735-742 [PubMed]journal. [CrossRef] [PubMed]
 
Pepke-Zaba J. .Jansa P. .Kim N.H. .Naeije R. .Simonneau G. . Chronic thromboembolic pulmonary hypertension: role of medical therapy. Eur Respir J. 2013;41:985-990 [PubMed]journal. [CrossRef] [PubMed]
 
Freed D.H. .Thomson B.M. .Berman M. .et al Survival after pulmonary thromboendarterectomy: effect of residual pulmonary hypertension. J Thorac Cardiovasc Surg. 2011;141:383-387 [PubMed]journal. [CrossRef] [PubMed]
 
Condliffe R. .Kiely D.G. .Gibbs J.S. .et al Improved outcomes in medically and surgically treated chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med. 2008;177:1122-1127 [PubMed]journal. [CrossRef] [PubMed]
 
Fedullo P. .Kerr K.M. .Kim N.H. .Auger W.R. . Chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med. 2011;183:1605-1613 [PubMed]journal. [CrossRef] [PubMed]
 
Chartier L. .Bera J. .Delomez M. .et al Free-floating thrombi in the right heart: diagnosis, management, and prognostic indexes in 38 consecutive patients. Circulation. 1999;99:2779-2783 [PubMed]journal. [CrossRef] [PubMed]
 
Goldhaber S.Z. .Visani L. .De Rosa M. . Acute pulmonary embolism: clinical outcomes in the International Cooperative Pulmonary Embolism Registry (ICOPER). Lancet. 1999;353:1386-1389 [PubMed]journal. [CrossRef] [PubMed]
 
Donaldson C.W. .Baker J.N. .Narayan R.L. .et al Thrombectomy using suction filtration and veno-venous bypass: single center experience with a novel device. Catheter Cardiovasc Interv. 2015;86:E81-E87 [PubMed]journal. [CrossRef] [PubMed]
 
NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Video 1

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Find Similar Articles
CHEST Journal Articles
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543