International Journal of Surgery Case Reports 114 (2024) 109159

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International Journal of Surgery Case Reports

journal homepage: www.elsevier.com/locate/ijscr

Case report

Successful surgical management of large pulmonary artery thrombosis in a

patient with primary severe mitral regurgitation and complex congenital
heart disease: A case report
Phan Quang Thuan a, Pham Tran Viet Chuong a, Ho Duc Thang a, Dinh Long Nhiem a,
Nguyen Hoai Nam b, *, Nguyen Hoang Dinh a, b, **
a
Department of Adult Cardiovascular Surgery, University Medical Center HCMC, Viet Nam
b
Department of Cardiovascular and Thoracic Surgery, Faculty of Medicine, University of Medicine and Pharmacy at Ho Chi Minh City, Viet Nam

A R T I C L E I N F O A B S T R A C T

Keywords: Introduction: Pulmonary artery thrombosis presents a rare and formidable challenge, especially in individuals
Pulmonary artery thrombosis with untreated complex congenital heart diseases and concurrent valvular abnormalities.
Antiphospholipid antibodies Presentation of case: This case details a distinctive instance of pulmonary artery thrombosis in a patient with
Congenital heart disease
untreated congenital atrial septal defect, severe pulmonary hypertension, pulmonary aneurysm, valvular heart
Atrial septal defect
Valvular heart disease
disease, and triple-positive antiphospholipid antibodies. Collaborative decision-making by a heart team guided
Pulmonary hypertension the diagnostic and therapeutic processes, resulting in surgical interventions encompassing pulmonary throm­
Pulmonary artery aneurysm bectomy, mitral and tricuspid regurgitation repair, and atrial septal defect closure.
Heart team Discussion: The case underscores the nuanced management of pulmonary artery thrombosis within the realm of
congenital heart disease, highlighting the imperative role of a multidisciplinary approach in achieving accurate
diagnoses and timely interventions. The multifaceted treatment strategy implemented further accentuates the
need for continual monitoring and personalized interventions in intricate clinical scenarios.
Conclusion: In conclusion, this case emphasizes the complexity of addressing pulmonary artery thrombosis in the
context of congenital heart disease. The collaborative efforts of a multidisciplinary team proved pivotal in
navigating diagnostic uncertainties and formulating a tailored treatment plan. Ongoing monitoring and
personalized strategies remain crucial for optimizing outcomes in such intricate cases.

1. Introduction manner is not recommended in clinical practice.

Pulmonary artery thrombus (PAT) occurrence in congenital heart
disease is infrequent. However, the presence of concurrent valvular and
congenital heart diseases, severe pulmonary hypertension, and a pul­
monary aneurysm raises concerns about multiple predisposing factors
for pulmonary thromboembolism, even without deep vein thrombosis
(DVT) [1–3]. Furthermore, the added risk of venous thromboembolism
due to antiphospholipid antibody syndrome is noteworthy [4]. It's
important to note that treating patients with multiple issues in this
We report a rare case of successful surgical intervention in a patient
with a significant PAT, concomitant severe rheumatic mitral regurgita­
tion (MR), a large atrial septal defect (ASD), pulmonary artery aneurysm
(PAA), severe secondary pulmonary regurgitation (PR), severe pulmo­
nary hypertension (PH), and secondary tricuspid regurgitation (TR). The
patient also tested positive for all three groups of antiphospholipid an­
tibodies. This case highlights the complexity of the clinical scenario and
underscores the importance of a collaborative, multidisciplinary heart
team approach in both diagnosis and treatment.

Abbreviation: CHD, congenital heart disease; PAT, pulmonary artery thrombus; MR, mitral regurgitation; ASD, atrial septal defect; PAA, pulmonary artery
aneurysm; PR, pulmonary regurgitation; TR, tricuspid regurgitation; PAPm, mean pulmonary artery pressure; PAD, pulmonary artery dissection; PH, pulmonary
hypertension; CT, computed tomography; APLA, antiphospholipid antibodies; INR, international normalised ratio; DVT, deep vein thrombosis; ESC, European Society
of Cardiology.
* Co-corresponding author.
** Correspondence to: N.H. Dinh, Department of Adult Cardiovascular Surgery, University Medical Center HCMC, Viet Nam.
E-mail addresses: dr.hoainamnguyen@gmail.com (N.H. Nam), dinh.nh@umc.edu.vn (N.H. Dinh).

https://doi.org/10.1016/j.ijscr.2023.109159
Received 3 November 2023; Received in revised form 6 December 2023; Accepted 8 December 2023
Available online 15 December 2023
2210-2612/© 2023 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license
(http://creativecommons.org/licenses/by-nc-nd/4.0/).
P.Q. Thuan et al.
The work has been reported in line with the SCARE 2020 criteria [5].
2. Case presentation
A 54-year-old male presented with progressive exertional dyspnea
and hemoptysis (2 months). Initially diagnosed with pulmonary embo­
lism and treated with oral anticoagulants, symptoms persisted, leading
to admission. His medical history included lumbar spine surgery but no
prior cardiovascular issues. Examination revealed dyspnea, muscle
contractions, lung rales, a systolic murmur, and hepatomegaly. Vital
signs were stable, except for oxygen saturation (85–90 %). Laboratory
tests showed elevated NT-proBNP and D-Dimer levels, and electrocar­
diography revealed an S1Q3T3 pattern and a right bundle branch block
(Fig. 1).
The patient underwent lower extremity ultrasound, ruling out
thrombus formation. Chest radiography revealed marked bilateral pul­
monary artery enlargement, consolidative findings in the right lung
(Fig. 2A). Echocardiography disclosed complex pathology: severe
rheumatic MR (type IIA2A3), extensive 51 mm ASD, severe TR (type
IIIb), mean pulmonary artery pressure of 60 mmHg, a 78 mm pulmonary
trunk aneurysm, enlarged right atrium and ventricle, reduced fractional
area change (20 %), and concurrent severe PR due to annular dilatation.
The CT scan findings are illustrated in Fig. 3A and C.
The patient's condition, assessed by a heart team, confirmed acute
pulmonary thrombosis with multiple related complications: acute res­
piratory and right ventricular failure, severe primary MR (IIA2A3), se­
vere TR (IIIb), a large ASD (51 mm diameter), and severe PR due to
annular dilation. A pulmonary artery trunk aneurysm (78 mm diameter)
coexisted with severe PH (PAPm 60 mmHg). Immediate treatment
included intravenous heparin, oxygen therapy (10 L/min), and urgent
surgical intervention to address the thrombus, repair mitral and
tricuspid valves, and close the ASD using a fenestrated technique.
In a complex surgical case necessitating a full sternotomy incision, a
conspicuously large aneurysm of the pulmonary trunk was prominently
identified (Fig. 4A). Establishment of cardiopulmonary bypass (CPB)
involved connecting the ascending thoracic aorta to both the superior
and inferior vena cava, accompanied by the administration of antegrade
cardioplegia. Induction of cardiac arrest facilitated access to the pul­
monary trunk and the subsequent division of the left and right main
pulmonary arteries. Thrombus removal was diligently executed, and
effective management of adhesions binding the thrombus to the arterial
walls was achieved (Fig. 4B-E). Concurrently, an endarterectomy pro­
cedure was performed to address the thrombus affixed to the two main
pulmonary arteries, with intermittent cardiac arrest totaling 4 min while
maintaining hypothermic conditions at 28 ◦ C. Subsequent interventions
encompassed a Waterson's groove incision, mitral valve repair with
annulus adjustment to 28 and closure sutures for the A2 and A3 margins,
incision of the right atrium, creation of a pericardial opening for the
correction of the atrial septal defect using a fenestrated technique (with
a diameter of 10 mm), and tricuspid valve repair with a size 30 annulus.
Post-surgical transesophageal echocardiography, conducted within the
operating room, revealed mild mitral valve and tricuspid regurgitation,
severe pulmonary regurgitation, a small residual atrial septal defect, and
a PAPm of 60 mmHg. Crucial time intervals during the procedure
Fig. 1. ECG displaying S1Q3T3 pattern with right bundle branch block (arrow).
2
International Journal of Surgery Case Reports 114 (2024) 109159
encompassed 188 min on CPB, 127 min of aortic clamping, and a brief
circulatory arrest period lasting 4 min.
The patient underwent a 5-day stay in the cardiac intensive care unit,
during which there was a significant improvement in right ventricular
function following the initiation of Nitric oxide (NO) ventilation within
24 h post-op, with a dose ranging from 5 to 10 ppm. Extubation took
place on the second day post-operation, and subsequent chest X-rays
indicated improved conditions compared to the pre-surgical state
(Fig. 2B). Discharge was granted on the 20th day postoperatively, along
with an oral Warfarin prescription to maintain an international nor­
malised ratio (INR) range of 2–3. The pre-discharge echocardiogram
showed the absence of MR, moderate TR, severe PR, and a decrease in
pulmonary artery pressure compared to preoperative values, with a
PAPm of 51 mmHg. Further investigation revealed positive results for
the thrombophilia test, along with other positive findings including
Anticardiolipin IgG antibodies, Anti-beta-2-glycoprotein-I antibodies
IgG, and Lupus anticoagulants, collectively indicative of anti­
phospholipid antibody syndrome. Anatomical pathology assessments
confirmed organized thrombosis within the vascular wall (Fig. 4F).
Following a re-evaluation after 2 months, the patient reported the
absence of exertional dyspnea and a return to normal functionality.
Subsequent echocardiography indicated the absence of MR, moderate
TR, and moderate PR, with a PAPm of 26 mmHg and a residual shunt
ASD measuring 1 mm. Chest X-ray examinations continued to display
prominent bilateral hilar regions without solidification imaging in the
right lung (Fig. 2C). CT examination of the pulmonary artery trunk
revealed measurements of 62 mm, with the right and left main pulmo­
nary arteries measuring 44 mm and 48 mm, respectively. Furthermore,
thrombus attachment to the wall of the left pulmonary artery was
observed (Fig. 3B), alongside the successful closure of the ASD (Fig. 3D).
3. Discussion
Analyzing the mechanisms of pulmonary thromboembolism in our
patient, several factors emerge. Their complex medical profile included
an ASD, PH, PAA, and all three antiphospholipid antibodies, elevating
the risk of pulmonary arterial thrombosis [6,7]. Alternative sources like
deep vein thrombosis were ruled out. Intraoperative examination
confirmed the absence of atrial thrombi. However, patients with PAA
and congenital heart disease (CHD) face an increased risk of pulmonary
artery dissection (PAD), which can lead to local thrombosis [8].
Although CT scans showed no PAD, intraoperative findings revealed
tissue thrombosis adhered to the pulmonary artery wall, necessitating
endothelium and thrombus removal. While rare, the possibility of pre­
vious PAD followed by thrombosis remains plausible [9]. Additionally,
PAA due to Grown-Up Congenital Heart can cause blood flow stasis and
thrombosis. Our patient thus experienced local pulmonary thrombosis
amidst numerous thrombotic risk factors and antiphospholipid antibody
syndrome-induced hypercoagulability, though the potential for prior
PAD cannot be ruled out definitively.
In terms of treatment, the patient, despite ongoing anticoagulation
therapy, continues to experience severe respiratory and right ventricular
failure. Hemoptysis and dyspnea symptoms (2 months) suggesting
thrombus organization led us to opt for surgery over fibrinolysis or
P.Q. Thuan et al. International Journal of Surgery Case Reports 114 (2024) 109159

Fig. 2. A: shows an X-ray of the patient before surgery, B shows an X-ray taken 6 days after surgery, with enlargement of the two hilars and consolidation of the right
lung (arrow). C shows an X-ray of a patient two months after surgery with enlarged two hilum (arrow).

Fig. 3. A: Preoperative CT showed an enlarged pulmonary artery of the trunk

and two main branches, nearly occlusion thrombosis of the left main pulmonary
artery, and thrombus of the right main pulmonary artery (arrow). B: CT of the
patient two months after surgery showed that the pulmonary aneurysm has
reduced in size, and there is little wall thrombus in the left main pulmonary
artery (arrow). C shows a preoperative CT with ASD (arrow). D: Postoperative
CT with patched ASD (arrow), and mitral annulus, tricuspid annulus (star).
catheter-based thrombectomy. Given the patient's multiple valvular le­
sions and ASD, surgical thromboendarterectomy and valvular repair
were deemed suitable. As per the European Society of Cardiology (ESC)
guidelines for adult CHD, patients with secondary ASD and PH should
undergo cardiac catheterization to assess pulmonary resistance (<3
wood units) [10]. However, due to the patient's acute pulmonary
thrombosis and inadequate response to anticoagulation, immediate
pulmonary revascularization was crucial, potentially saving the patient's
life. We implemented a fenestrated patch technique to minimize post­
operative acute pulmonary edema [11]. Simultaneous mitral and
tricuspid valve surgery was performed based on ESC guidelines [12].
Regarding the PAA, it wasn't addressed at this time to avoid
Fig. 4. A: shows an aneurysm of the trunk pulmonary artery (arrow). B: shows
thrombosis of the left main pulmonary artery (arrow). C: shows thrombosis of
the right main pulmonary artery (arrow). D: shows a large ASD (arrow). E:
displays an image of thrombus after removal from the pulmonary artery. F:
shows pathology with organized thrombosis and vascular wall.
prolonging surgery. The lack of established treatment recommendations
for pulmonary vascular disease in CHD with PH, coupled with the rarity
of aneurysm progression following ASD resolution, factored into this
decision [9,13]. Concerning severe PR and the PAA, it was expected to
improve after ASD correction. Immediate treatment was deferred, with
percutaneous pulmonary valve replacement an option for the future
[14]. Building on the diagnostic and therapeutic insights, a compre­
hensive Heart team meeting was convened to discuss diagnosis and

3
P.Q. Thuan et al.
treatment. ESC recommendations and collaborative expertise provided a
more precise and unbiased perspective, vital in complex cases [12,14].
The heart team's input played a pivotal role in ensuring timely and ac­
curate care for the patient.
For the surgical approach, we chose to address pulmonary artery
thrombosis during cardiac arrest due to the challenging prognosis of
obtaining a sizable thrombus mass and the potential for pulmonary ar­
tery endarterectomy. Simultaneously, we repaired MR, TR, and the ASD.
Our CPB, aortic clamp, and circulatory arrest times were 188, 127, and
4 min, respectively, all reasonable for this complex procedure. While
literature reports surgical removal of pulmonary artery thrombosis in
CHD with PH [15,16], our case is unique, with no documented instances
in English literature combining pulmonary artery thromboendarter­
ectomy, mitral valve repair, ASD patching, and tricuspid valve repair,
making comparisons challenging.
Individuals with pulmonary thrombosis, especially those at high
thrombotic risk like congenital heart disease with pulmonary hyper­
tension patients, should undergo thrombophilia testing per the Amer­
ican Society of Hematology 2023 Guidelines for Venous
Thromboembolism Management [17]. In our case, testing revealed all
APLA types, diagnosing antiphospholipid antibody syndrome,
increasing recurrent thromboembolism risk [18]. Following ESC 2019
guidelines for pulmonary embolism management, recommending
vitamin K antagonist anticoagulation for relapse prevention (level I,
evidence B), we initiated warfarin therapy to maintain INR between 2
and 3, reducing thrombosis risk [18,19]. Regular follow-ups tracked
pulmonary artery pressure reductions and PR, supported by CT scans
assessing pulmonary aneurysm size and identifying new pulmonary ar­
tery thrombosis, reaffirming the decision to postpone surgery for the
aneurysm and severe PR.
4. Conclusions
In conclusion, the occurrence of pulmonary artery thrombosis in
patients with chronic untreated heart disease, particularly those with
complex secondary lesions and acquired left valvular heart disease, is
exceptionally rare. This rarity is further heightened when concomitant
with a pulmonary aneurysm in adult congenital heart disease and the
presence of three groups of antiphospholipid antibodies, potentially
leading to localized thrombosis. The challenges in obtaining a definitive
diagnosis and making treatment decisions are compounded by the lack
of clinical evidence in this domain. The recommendations of a multi­
disciplinary heart team are crucial in navigating diagnostic uncertainties
and determining the most suitable and timely course of treatment,
particularly in urgent situations. Our documented approach adds a
valuable experiential contribution to the global medical literature,
showcasing successful management of this complex case through
collaborative efforts and emphasizing the need for further research in
this underexplored area of pulmonary artery thrombosis.
Consent
Written informed consent was obtained from the patient for publi­
cation of this case report and accompanying images.
Ethical approval
This case was reviewed and approved by the Institutional Review
Board at University Medical Center, University of Medicine and Phar­
macy at Ho Chi Minh city (approval number: 105/GCN-HDDD-
BVDHYD). All methods were performed in accordance with the Decla­
ration of Helsinki.
Funding
No funding was received for this study.
4
International Journal of Surgery Case Reports 114 (2024) 109159
Author contribution
Writing: PQT and NHD. Critical review and revision: all authors.
Final approval of the article: all authors. Accountability for all aspects of
the work: all authors.
Guarantor
Nguyen Hoang Dinh.
Conflict of interest statement
The author(s) declare no conflict of interest in preparing this article.
Data availability
All of the material is available and owned by the authors and/or no
permissions are required.
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