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Further Reading

Kane RD, Hawkins HK, Miller JA, Noce PS. Microscopic pul-

monary tumor emboli associated with dyspnea. Cancer.

1975;36(4):1473–82.

Miyoshi S, Hamada H, Katayama H, Hamaguchi N, etal. Pulmonary

tumor thrombotic microangiopathy associated with lung cancer.

J Cardiol Cases. 2010;1:e120–3.

Roberts KE, Hamele-Bena D, Saqi A, Stein CA, Cole RP.Pulmonary

tumor embolism: a review of the literature. Am J Med.

2003;115:228–32.

Chapter 10. Case 10: Chronic Thrombus andMetastatic…

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99© Springer Nature Switzerland AG 2020

W. R. Auger, D. Gopalan (eds.), Clinical Cases in Chronic

Thromboembolic Pulmonary Hypertension, Clinical Cases in

Cardiology, https://doi.org/10.1007/978-3-030-17366-1_11

Case Presentation

A 50 year old white female presented with complaints of grip-

ping, squeezing substernal chest pain awakening her from sleep

at 1 A.M. The pain spontaneously resolved after 10 min.

Evaluation in the emergency department revealed hypoxemia

Chapter 11

Case 11: Don’t Forget

About thePulmonary

Veins!

TerenceK.Trow, DeepaGopalan, andWilliamR.Auger

T. K. Trow (*)

Pulmonary, Critical Care, and Sleep Medicine, DynaMed Plus,

EBSCO Health Information Services, Ipswich, MA, USA

Yale Pulmonary Vascular Disease Program, Yale School of

Medicine, New Haven, CT, USA

e-mail: ttrow@ebsco.com

D. Gopalan

Department of Radiology, Imperial College Hospitals, London, UK

e-mail: d.gopalan@nhs.net

W. R. Auger

Lewis Katz School of Medicine, Temple University,

Philadelphia, PA, USA

e-mail: Bill.Auger@tuhs.temple.edu

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100

with oxygen saturations of 82% on room air normalizing with

three liters nasal cannula (LNC) supplementation. Vital signs

showed a pulse of 54, BP of 156/87, respiratory rate of 24, and a

temperature of 98.2. Physical exam was notable for some repro-

ducible chest wall tenderness that was not relieved by leaning

forward, a grade 1/6 holosystolic murmur heard best at left

upper sternal border as well as a grade 1/6 diastolic murmur, and

rales at both lung bases. No dullness to percussion of the lungs

was appreciated. No pulmonary bruit was heard between the

scapulas. The lower extremities showed no clubbing, cyanosis, or

edema. An initial troponin was 0.03μg/L.N-Terminal proBrain

natriuretic peptide (NT-proBNP) was 5330pg/ml. An electro-

cardiogram (ECG) revealed a right bundle branch block with

left posterior fascicular block and T wave inversions in V1-V4

with some ectopic atrial beats. A chest X-ray (Fig.11.1) revealed

enlarged pulmonary arteries with perihilar pulmonary vascular

congestion and some alveolar opacities suggestive of pulmonary

edema. A dose of 40 mg of furosemide was administered. She

was admitted for further evaluation and treatment.

F . PA and lateral chest radiograph showing cardiomegaly

and enlarged central pulmonary arteries

T. K. Trow et al.

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101

Past medical history included hypertension, gastroesopha-

geal reflux disease, depression, and a history of congenital

atrial septal defect with repair at age 10in 1975 followed by

mitral valve repair with 28mm Physio II annuloplasty ring as

well as closure of A2 plus A3 and release of P3 chordae in

2010. A tricuspid valve annuloplasty and patch reconstruc-

tion of the right superior pulmonary vein were also per-

formed at that time.

Social history was notable for no alcohol or illicit drug use

but ongoing tobacco abuse with a 20 pack-year history.

Hospital Course: The patient was diuresed with gradual

improvement in dyspnea and oxygen requirement.

Nonsteroidal anti-inflammatory therapy improved her muscu-

loskeletal chest wall pain. An initial echocardiogram revealed

severe pulmonic regurgitation with severe pulmonary hyper-

tension with a pulmonary artery systolic pressure (PASP)

estimate of 72, moderate right ventricular dysfunction and

dilatation, with mild tricuspid regurgitation. Her left atrium

was severely dilated and moderate diastolic relaxation impair-

ment was noted. Mild mitral regurgitation was noted. No

pericardial effusion was present. She was diuresed further and

symptomatically improved with a repeat echocardiogram

showing a PAPs estimate of 42. Bubble contrast administra-

tion revealed no evidence of shunting. Consultation with the

pulmonary hypertension and cardiology service resulted in a

differential diagnosis of WHO group 2 pulmonary venous

hypertension, WHO group 4 chronic thromboembolic pulmo-

nary hypertension (CTEPH), or chronic obstructive pulmo-

nary disease (COPD) associated WHO group 3 pulmonary

hypertension. Congenital heart disease associated pulmonary

hypertension (PH) was deemed unlikely due to previous

echocardiograms showing no PH as recently as 2010. Serologies

for connective tissue disease, inherited or acquired hyperco-

agulability and human immunodeficiency virus were negative.

Pulmonary function tests revealed a forced expiratory volume

of 1.67L (52% of predicted), forced vital capacity of 2.08L

(51% of predicted), a ratio between them of 80, total lung

capacity of 3.66L (52% of predicted), and a diffusion capacity

Chapter 11. Case 11: Don’t Forget About .....

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102

adjusted for hemoglobin of 19.28 (72% of predicted) consis-

tent with a moderate restrictive ventilatory defect with mildly

impaired diffusion but no COPD. A ventilation-perfusion

scan was performed (Fig.11.2) and revealed unmatched per-

fusion defects to the entire right upper lobe (blue arrow).

A computerized tomography pulmonary angiogram

revealed no filling defects to suggest acute pulmonary emboli,

with linear densities in the lower lobes and RUL suggestive

of atelectasis.

Concern over CTEPH prompted a combined right heart

catheterization with pulmonary angiography. This revealed a

right atrial pressure (RAP) of 10, pulmonary artery pressure

(PAP) of 45/25 with a mean of 31, a pulmonary wedge pres-

sure (PAWP) of 12, cardiac output (CO) of 4.61L/min, car-

diac index (CI) of 2.14 L/min, resulting in a pulmonary

vascular resistance (PVR) of 4.12Wood units. A saturation

run revealed a high superior vena cava saturation of 66%,

RA sat of 65.9 %, PA sat of 68.1%, with a systemic saturation

of 95.5%. Inhaled nitric oxide at 40ppm resulted in PAP of

50/23; mean of 32. A 500 cc rapid fluid bolus resulted in a

doubling of the PAWP to 24. Pulmonary angiography

(Fig. 11.3) revealed markedly decreased right upper lobe

(RUL) perfusion with smooth stenosis of the truncus anterior.

F . Lung scintigraphy. Perfusion to right upper lobe is

decreased (arrow) with intact ventilation

T. K. Trow et al.

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103

Taken together this was interpreted as PH from heart fail-

ure with preserved ejection fraction (HFpEF) and possibly

CTEPH. Consultation with a CTEPH center experienced

with pulmonary thromboendarterectomy surgery was

requested.

A review of the chest CT angiogram in this patient con-

firmed the reduction in RUL arterial density relative to the

left upper lobe (LUL), as well as, the absence of intra-arterial

organized thrombus. However, with the history of a previous

cardiac surgical procedure which included reconstruction of

the right superior pulmonary vein, assessment of the medias-

tinum in the region of the pulmonary veins (Fig. 11.4)

revealed complete obstruction of the right superior pulmo-

nary vein near the juncture with the left atrium (open arrow).

F . Right pulmonary arteriogram: intact right upper lobe

vessels with distal pruning and absent venous return (arrow)

Chapter 11. Case 11: Don’t Forget About .....

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104

Radiographic Interpretation

The chest radiograph also demonstrates evidence for a previ-

ous sternotomy, tricuspid valve annuloplasty, and mitral valve

repair (best viewed on lateral film). In addition to generalized

“haziness” over the lower zones, the cardiac silhouette is

enlarged and the vascular markings in the right upper lobe

appear less prominent relative to the left upper lobe.

The anterior view of the V/Q scintigraphy shows overall

poor perfusion to the right upper lobe (blue arrow) as the

only significant unmatched defect. Notable is the “type” of

perfusion abnormality, a graded reduction without definitive

segmental or subsegmental defects.

The pulmonary angiogram is remarkable for the early

opacification of the right upper lobe vessels, and distal vessel

“pruning” as contrast fails to opacify the most distal vascula-

ture on sequential cuts. As the venous phase appears, there is

no drainage of contrast from the upper lobe of the right lung.

Important to note is the absence of angiographic irregulari-

F . CT angiogram demonstrating occlusion of the right

superior pulmonary vein

T. K. Trow et al.

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105

ties to suggest chronic thromboembolic disease; the overall

architecture of the right upper lobe vessels is basically nor-

mal. The white arrow points to some reflux of contrast into

the right superior pulmonary vein from the left atrium. A

coronal view of the same CT angiogram better demonstrates

the obstructed pulmonary vein (Fig.11.5).

Clinical Comments

This case illustrates that impedance of outflow from a lung

region due to obstruction or narrowing of a large pulmonary

vein may result in perfusion defects on lung scintigraphy. As

F . Coronal view of the CT angiogram demonstrated

thrombus in the right superior pulmonary vein (block arrow, left

panel). Note the contrast filled patent left superior pulmonary vein

(block arrow, right panel) for comparison (LA: Left atrium; RPA:

right pulmonary artery)

Chapter 11. Case 11: Don’t Forget About .....

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106

seen in this patient, arterial “pruning” may result from

diminished perfusion of the distal vessels, and should not be

interpreted as an angiographic feature of chronic thrombo-

embolic disease. In the example above, the basis for the poor

perfusion of the right upper lobe was occlusion of the right

superior pulmonary vein, likely chronically thrombosed fol-

lowing surgical reconstruction of this vessel several years

previously. By itself, it is unlikely this finding contributes

significantly to her symptoms, or plays a major role in her

pulmonary hypertension. Impressive was the considerable

rise in pulmonary arterial wedge pressure with a fluid chal-

lenge, in keeping with her diagnosis of WHO group II pul-

monary hypertension.

There are also other disease states that may compromise

the pulmonary veins as they enter the mediastinal space and

drain into the left atrium. Mediastinal fibrosis, adenopathy

and/or neoplasms that can compress the major pulmonary

veins and pulmonary vein stenosis post atrial fibrillation

ablation procedures are among the more common

considerations.

Points ofEmphasis

Not all perfusion defects are due to pulmonary arterial dis-

ease. Large vessel pulmonary venous narrowing or occlusion

can similarly cause perfusion defects seen on lung scintigra-

phy and CT.

Diagnoses

1. Right upper lobe perfusion defect due to occlusion of the

right superior pulmonary vein (post-surgical).

2. WHO group II pulmonary hypertension.

T. K. Trow et al.

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107