Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_3902_Библиотеки_им_академика_М_И_Перельмана
.pdf
24
A repeat CT angiography of the chest was performed and
directly reviewed. Although no acute pulmonary emboli were
seen, there was peripheral lining clot seen in the bilateral
lower lobes and right upper lobe, suggestive of chronic
thromboembolic disease.
As his suspected underlying diagnosis shifted from iPAH
to chronic thromboembolic pulmonary hypertension
(CTEPH), he underwent pulmonary angiography with repeat
right heart catheterization. Hemodynamics on triple PH
directed medical therapy included a right atrial pressure
(RAP) of 14mmHg, PAP 98/26 (50) mmHg, PCWP 10mmHg,
CO 4.1L/min, and PVR 10.5Wood Units. Pulmonary angiog-
raphy revealed evidence of bilateral multiple vascular nar-
rowings and obstructive lesions in the distal vessels, with
significantly reduced vascular markings in the right upper,
right middle, and left lower lobes (Fig.3.3).
Taken together, his hemodynamics, VQ scan, direct review
of his CT angiography, and pulmonary angiography con-
WASH IN
ANT
Q
LT LAT QRT LAT QLAO QRAO Q
POST QLPO
QR
PO Q
WASHOUT1WASHOUT2EQUIL
F . VQ scan shows bilateral mismatched segmental and
subsegmental perfusion defects
A. Vaidya et al.
https://t.me/med1917
25
firmed his diagnosis of CTEPH.Given the distal nature of
his thromboembolic disease, consultation with an experi-
enced PTE center performing distal endarterectomies was
requested.
Over the course of his initial evaluation with our Pulmonary
Hypertension Cardiology program, the patient continued to
deteriorate with worsening severe right heart failure. He lost
22lb of weight, pulse increased to 110 and blood pressure fell
to 101/50 mmHg. His jugular venous pressure was greater
than 20cm of water with abdominal ascites, pitting peripheral
edema, and he was admitted for IV diuresis and further titra-
tion of IV epoprostenol. His NTProBNP increased to 2147,
and creatinine increased to 1.45mg/dL.His 6min walk dis-
tance fell to 247m.
He was urgently evaluated at the referral PTE center,
where he underwent IVC filter placement followed by bilat-
eral pulmonary thromboendarterectomy (Fig.3.4).
F . Pulmonary angiogram demonstrating scattered distal
arterial narrowings (“webs”) and vessel attenuation particularly in
the right upper, right middle and left lower lobes
Chapter 3. Case 3: Distal Chronic Thromboembolic ....
https://t.me/med1917
26
Post-operatively, his hemodynamics (off PH-directed medi-
cal therapy) revealed RAP of 12mmHg, PA 58/12 (25) mmHg,
CO 5.3L/min, and PVR 2.3Wood Units. He was treated for
reperfusion lung injury with mechanical ventilator support
and diuresis, and ultimately discharged on post- operative day
#13. His post-operative V/Q scan revealed increased perfusion
to posterior and superior RLL, RML, LLL, and lingular ter-
ritories, with ‘perfusion steal’ from the LUL, superior segment
of the LLL, and persistent defects RUL.
Six months post-operatively, off all PH specific medical
therapy and oxygen supplementation, he had regained 28lb
of flesh weight, had WHO Functional Class I, pulse reduced
to 71, BP increased to 134/70, 6min walk distance increased
to 567 m without significant desaturation. NT ProBNP had
fallen to 847, and creatinine had fallen to 0.69 mg/dL. His
echocardiogram revealed no interventricular systolic septal
flattening, only mild RV enlargement, normal RV function,
and estimated PASP 30mmHg. His pulmonary hypertension
has been cured, managed solely with warfarin for anticoagu-
lation. His recovery and excellent clinical status has persisted
to 2years post-operatively (Fig.3.5).
F . Chronic thrombotic material removed at thromboendar-
terectomy. Ruler=15cm
A. Vaidya et al.
https://t.me/med1917
27
Radiographic Interpretation
To better assess this patient’s candidacy for PTE surgery,
selective pulmonary angiography with AP and lateral views
was performed to “map out” the extent and localization of
disease.
This study demonstrates numerous features of chronic
thromboembolic disease. On the right side (Fig.3.6a), there
were several “pouch” defects involving the segmental ante-
rior and posterior right upper lobe and basal lateral right
lower lobe arteries (solid arrows). A segmental web is present
in the apical branch of the right upper lobe artery (open
arrow). There is abrupt calibre change with diffuse irregular-
ity in a predominant distal distribution in the lower lobe ves-
sels. On the left (Fig.3.6b), “pouch” defects are present in the
anterior segment of the left upper lobe and medial basal left
lower lobe arteries and at the subsegmental level in the apical
branch of the left upper lobe vessel (solid arrows). There is a
web in the distal lingula and stenosis in the posterior basal
left lower lobe artery (open arrows). These findings are best
depicted on lateral views, and were not evident on the prior
AP pulmonary angiographic studies.
F . Echocardiogram 6months after pulmonary thromboen-
darterectomy showing a significant reduction in right atrial and right
ventricular size with improved RV function
Chapter 3. Case 3: Distal Chronic Thromboembolic ....
https://t.me/med1917
28
Right PA
Left PA
Right Lat
Left Lat
a
b
F . (a) Right PA and lateral digital subtraction pulmonary
angiogram. (b) Left PA and lateral digital subtraction pulmonary
angiogram. Arrows identify areas of severe stenosis or occlusion
from chronic thromboembolic disease
A. Vaidya et al.
https://t.me/med1917
29
Clinical Comments
This case provides a number of important lessons in the diag-
nosis and treatment of patients with CTEPH, especially for
those with disease primarily in the segmental and subseg-
mental vessels. The initial point of discussion, however, is that
the evaluation of a patient with pulmonary hypertension
should include a V/Q scan to screen for chronic thromboem-
bolic pulmonary hypertension. Observations from Tunariu
and colleagues have demonstrated that the V/Q scan has a
sensitivity of greater than 96% for detecting CTEPH, better
than the 51% sensitivity of CT angiography of the chest.
Although with contemporary CT capabilities and expert
interpretation the value of CT in screening for this disease
has been shown to be nearly comparable to lung scintigraphy,
“missing” CTEPH by CT… as was the issue in the above
case… can result in considerable delay in diagnosis, and as a
result, a missed opportunity to offer the most appropriate and
effective intervention.
The essential lesson from this case comes from what
occurred once the patient was referred to a center experi-
enced in the evaluation and treatment of CTEPH.Once the
perfusion scan revealed unmatched defects, the diagnostic
approach was appropriately directed to establishing the basis
for these defects. A review of the CT angiogram raised suspi-
cions for the presence of organized thrombi in the distal vas-
cular bed, but the lack of certainty prompted what many feel
to be the “gold standard” procedure for the diagnosis of
chronic thromboembolic disease….catheter based
pulmonary
angiography. And in fact, at the segmental and subsegmental
level, a properly performed pulmonary angiogram has been
shown to be of greater sensitivity for the detection of chronic
thromboembolic lesions compared to CT angiography (see
Suguira etal. 2013). This approach in this case led to the cor-
rect diagnosis, ultimately prompting a referral to a center
with surgical experience in the removal of distal vessel
chronic thromboembolic disease.
Chapter 3. Case 3: Distal Chronic Thromboembolic ....
https://t.me/med1917
30
The task for the expert surgical center than becomes one of
not only confirming the diagnosis, but adequately defining the
extent of disease and localizing or “mapping out” the lesions.
An assessment of surgical candidacy is dependent on the qual-
ity of pre-operative imaging and, most importantly, the capa-
bilities of the surgical team. A significant prognostic factor for
successful outcomes following PTE surgery relates to the
experience of the CTEPH physicians and institution, and this
is especially the case with segmental (Level III) and subseg-
mental (Level IV) resection. Selective pulmonary angiography
including lateral views in this patient more clearly defined the
extent and localization of the chronic thromboembolic disease
present, thereby allowing the surgical team to assess whether
or not endarterectomy was feasible within their capabilities.
Finally, the importance of recognizing distal vessel CTEPH
as potentially amenable to thromboendarterectomy is under-
scored by the observation that significant pulmonary hemo-
dynamic benefit, and in most instances, resolution of a
patient’s pulmonary hypertension and RV failure can be
achieved with surgery (see D’Armini etal. 2014).
This case is also an example of a known risk factor for
developing distal vessel CTEPH. The presence of chronic
indwelling catheters, pacemaker wires, or ventriculoatrial
shunts are far more prevalent in patients with Level III and
Level IV disease than in proximal vessel CTEPH.Given the
relatively small thrombus formation associated with such
devices, it is not surprising that the chronic thromboembolic
disease in this patient’s case was distal, as opposed to the
more proximal disease associated with known lower
extremity
deep vein thrombosis (DVT) or a history of a massive or
submassive pulmonary embolus.
Points ofEmphasis
In experienced CTEPH centers, resection of distal vessel
chronic thromboembolic disease is technically feasible and
can result in meaningful improvement in pulmonary hemody-
namics and clinical status.
A. Vaidya et al.
https://t.me/med1917
31
Diagnosis
1. Distal vessel chronic thromboembolic pulmonary
hypertension.
Further Reading
Bonderman D, Wilkens H, Wakounig S, et al. Risk factors for
chronic thromboembolic pulmonary hypertension. Eur Respir J.
2009;33:325–31.
D’Armini AM, Morsolini M, Mattiucci G, etal. Pulmonary endarter-
ectomy for distal chronic thromboembolic pulmonary hyperten-
sion. J Thorac Cardiovasc Surg. 2014;148:1005–12.
He J, Fang W, Lv B, et al. Diagnosis of chronic thromboembolic
pulmonary hypertension: comparison of ventilation/perfusion
scanning and multidetector computed tomography pulmonary
angiography with pulmonary angiography. Nucl Med Commun.
2012;33:459–63.
Madani MM, Jamieson SW.Technical advances of pulmonary endar-
terectomy for chronic thromboembolic pulmonary hypertension.
Semin Thorac Cardiovasc Surg. 2006;18:243–9.
Madani M, Mayer E, Fadel E, Jenkins DP. Pulmonary endarterec-
tomy: patient selection, technical challenges, and outcomes. Ann
Am Thorac Soc. 2016;13(Suppl 3):S240–7.
Mayer E, Jenkins D, Lindner J, et al. Surgical management and
outcomes of patients with chronic thromboembolic pulmonary
hypertension: results from an international prospective registry.
J Thorac Cardiovasc Surg. 2011;141:702.
Tunariu N, Gibbs SJR, Win Z, etal. Ventilation-perfusion scintig-
raphy is more sensitive than multidetector CTPA in detecting
chronic thromboembolic pulmonary disease as a treatable cause
of pulmonary hypertension. J Nucl Med. 2007;48:680–4.
Sugiura T, Tanabe N, Matsuura Y, etal. Role of 320-slice CT imaging
in the diagnostic workup of patients with chronic thromboem-
bolic pulmonary hypertension. Chest. 2013;143:1070–7.
Chapter 3. Case 3: Distal Chronic Thromboembolic ....
https://t.me/med1917
33© 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_4
Case Presentation
A 27-year-old female with no significant past medical history
developed dyspnea and a mild non-productive cough 2 years
prior to presentation. She was evaluated by her internist at that
time and placed on a proton pump inhibitor and an inhaled cor-
ticosteroid with some improvement. Approximately 1year later
she developed sudden onset of left arm discomfort, swelling and
“purplish” discoloration. An ultrasound showed a left arm DVT,
Chapter 4
Case 4: TheCase
forBalloon Pulmonary
Angioplasty
DavidS.Poch, DeepaGopalan, andWilliamR.Auger
D. S. Poch (*)
University of California, San Diego, La Jolla, CA, USA
e-mail: dspoch@ucsd.edu
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
https://t.me/med1917
34
and a CT angiogram of her chest showed segmental and sub-
segmental pulmonary emboli. An echocardiogram at that time
showed RV dilatation and an estimated RVSP of 46 mmHg.
Lower extremity duplex showed no evidence of DVT.She was
started on rivaroxaban with improvement in her left upper
extremity but only modest improvement in her dyspnea.
Three months after starting rivaroxaban her symptoms
of dyspnea persisted and a follow up echocardiogram dem-
onstrated RV enlargement and worsening pulmonary
hypertension. V/Q scan was performed and demonstrated
large, bilateral perfusion defects. The patient was told she
had thoracic outlet syndrome, although she had no history
of repetitive upper extremity motions that might provoke
this syndrome (such as those occurring in sports like vol-
leyball or swimming). Additionally, she never experienced
an accident or trauma to her thoracic cavity. Based on the
suspicion for chronic thromboembolic pulmonary hyper-
tension (CTEPH) she was referred to a specialized CTEPH
center with expertise in pulmonary thromboendarterec-
tomy (PTE) surgery.
At the time of evaluation for PTE she was NYHA func-
tional class III.She had difficulty climbing stairs and could
walk less than 300 ft on flat ground without becoming
winded. She also noted right-sided chest pain and intermit-
tent right arm numbness with exertion. She reported frequent
palpitations and a persistent non-productive cough. She had
no episodes of syncope, no edema or abdominal distention.
Review of symptoms revealed a history of Raynaud’s phe-
nomenon but no other stigmata of connective tissue disease.
She had no other identifiable risk factors for WHO Group I
pulmonary arterial hypertension and no signs or symptoms
consistent with systemic vasculitis. She had no history of
tobacco use, alcohol consumption or illicit drug use.
Physical examination revealed a tall (6 ft), 54 kg female
with a systemic blood pressure of 112/72, pulse rate of 76 BPM,
respiratory rate of 16 BPM and saturation of 99% on room air.
Cardiac exam was notable for a loud S2, a tricuspid regurgitant
murmur, soft RV heave, but no S3 gallop. Lung exam revealed
clear lung fields and no pulmonary flow murmurs. There was
D. S. Poch et al.
https://t.me/med1917
Соседние файлы в папке Библиотека им академика М.И. Перельмана