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Int J Surg Case Rep
Int J Surg Case Rep
International Journal of Surgery Case Reports
2210-2612
Elsevier

S2210-2612(24)00853-8
10.1016/j.ijscr.2024.110072
110072
Case Report
A rare case of large true main pulmonary artery aneurysm managed surgically: A case report
Sohail Abdul Ahad abdulahad7@hotmail.com
a⁎
Madni Varisha varisha.madni@scholar.aku.edu
b
Wahab Rida a
Ahmad Waris waris.ahmad@aku.edu
a
Inam Hina hina.inam@aku.edu
a
a Cardio-thoracic Surgery, Aga Khan University Hospital Karachi, Pakistan
b Aga Khan University Hospital, Pakistan
⁎ Corresponding author. abdulahad7@hotmail.com
26 7 2024
9 2024
26 7 2024
122 11007219 6 2024
16 7 2024
22 7 2024
© 2024 The Author(s)
2024
https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Introduction & importance

Pulmonary Artery Aneurysm is defined as the localized dilation of the pulmonary artery >1.5 times the upper normal limit or pulmonary artery measuring 4 cm. Pulmonary artery aneurysm is considered as a rare disorder having an incidence of 1 in 14,000 post-mortem examinations.

Case presentation

Presented below is a case of a 28 year old gentleman, who presented with exertional dyspnea and orthopnea and was diagnosed with having a pulmonary artery aneurysm of 76 cm × 56 cm × 53 cm arising from the main pulmonary artery upon Computed Tomography Scan which is a rare finding according to the available literature.

Clinical discussion

Clinical manifestations of pulmonary artery aneurysm are varying and rarely occur. However the use of radiological imaging has aided in the diagnosis. No specific treatment guidelines have been mentioned yet in the literature however, medical management, surgical resection and endovascular therapy are one of the multiple options available.

Conclusion

Pulmonary Artery Aneurysm presents with non-specific symptoms which makes the diagnosis very challenging for the physicians, in process, delaying the accurate management of the disease. However, pulmonary artery aneurysm must be considered as a differential diagnosis and appropriate management options, whether medical or surgical should be opted for keeping in mind the size and the complications of the disease.

Highlights

• Young male presented with chest pain, dyspnea, and weight loss

• CT scan revealed a saccular PA aneurysm arising from the main pulmonary trunk

• Excision of a large, calcified aneurysm (7 x 7 cm) arising from the main pulmonary artery

• The main pulmonary artery was repaired using a bovine pericardial patch.

• Histopathology was negative for granuloma or malignancy

Keywords

Pulmonary artery aneurysm
Pulmonary artery hypertension
Main pulmonary trunk
Left pulmonary artery
Surgical resection
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pmc1 Introduction

Pulmonary artery aneurysm (PAA) is an uncommon condition characterized by the localized dilation of the pulmonary artery (PA), involving all three layers of the vessel wall. Diagnosis typically occurs when the vessel dilation exceeds 1.5 times the normal upper limits [1] or when the pulmonary trunk measures >4 cm [2]. With an incidence of around one in 14,000 postmortem examinations, PAA is considered a rare disorder [3]. The origins of PAA can be traced to a variety of congenital and acquired factors, including congenital heart defects, connective tissue abnormalities, and pulmonary hypertension (PAH) [4]. PAH, defined as a mean PA pressure of ≥20 mmHg at rest [2], plays a significant role in PAA development and is closely associated with its clinical manifestation [5]. Simultaneous occurrence of pulmonary artery aneurysm (PAA) and pulmonary arterial hypertension (PAH) elevates the risk of aneurysm rupture and dissection of the vessel wall [6]. Additionally, PAA carries the potential of compressing adjacent structures such as the left main coronary artery, the primary bronchus, and/or the recurrent laryngeal nerve [6]. Among these critical complications, a ruptured PAA can lead to high mortality rates ranging from 50 to 100 % [7]. To mitigate the risk of life-threatening complications associated with PAA, early surgical intervention may be advisable in specific cases where patients present with both PAA and PAH. This work has been reported in line with the SCARE criteria [8].

2 Case presentation

We bring forth the case of 28-year-old gentleman, with a history of smoking, alcohol consumption and no known co-morbid conditions visited the outpatient department reporting chest pain, exertional dyspnea, and orthopnea persisting for the past four months, associated with significant loss of weight along with hemoptysis for which he received symptomatic treatment outside with no avail. A computed Tomography Pulmonary Angiography Scan was done which revealed a sizable saccular pulmonary artery aneurysm originating from the pulmonary trunk, measuring 76 cm × 56 cm × 53 cm after which he was referred for further management [Fig. 1]. A chest radiograph was done which revealed a large, well-defined radiodensity with a margin in the left hilum region [Fig. 2].Fig. 1 Computed Tomography Scans Chest Pulmonary Artery, axial and coronal views, showing a giant pulmonary artery aneurysm arising from the main pulmonary trunk.

Fig. 1

Fig. 2 Chest Radiograph showing large, well-defined, rounded radiodensity with calcified margin in the left hilum.

Fig. 2

Initial assessment was unremarkable, and patient was received in a vitally and hemodynamically stable condition. Patient was planned for surgical resection under general anesthesia. After standard prep and drape midline sternotomy and pericardiotomy was done revealing the aneurysm tethered to the left atrium, causing bleeding. Aortic and bi-caval cannulation was done and cardiopulmonary bypass was initiated. Careful dissection of the aneurysm from the surrounding pericardium followed, leading to the excision of a large, calcified aneurysm (7 × 7 cm) arising from the distal main pulmonary artery [Fig. 3].Fig. 3 Intra-operative images showing; A. the resected aneurysm, B. large aneurysm arising from the main pulmonary artery trunk.

Fig. 3

Intra-pericardial adhesions necessitated the resection of the left phrenic nerve. The main pulmonary artery was repaired using a bovine pericardial patch. Atrial septal defect closure was performed and, left diaphragmatic plication done. After decannulation, the patient was successfully weaned off the cardiopulmonary bypass and hemostasis was achieved and the patient was transferred to critical care unit post-operatively.

Postoperatively, the patient remained vitally and hemodynamically stable. The hospital stay remained uneventful and he was discharged home as planned. Histopathology was negative for granuloma or malignancy. He was now doing well with regular clinic follow-ups.

3 Discussion

A pulmonary artery aneurysm is defined as a localized dilatation of a vessel, encompassing all three layers of the vessel wall. These aneurysms have a diameter exceeding 4 cm when measured in the main trunk of the pulmonary artery [2].

The presenting symptoms of PAA is primarily vague, leading to majority of the patients going undiagnosed, even in cases of substantial aneurysm size, due to its typically asymptomatic progression. However, clinical symptoms may manifest in the presence of complications such as bronchial or tracheal compression (resulting in cough and dyspnea), dissection, or rupture (resulting in hemoptysis) as shown in our patient [2].

In diagnostic imaging, computed tomography pulmonary angiography (CTPA) may reveal indicators such as right ventricle (RV) dilatation, right atrium dilatation, main pulmonary artery enlargement (diameter > 29 mm), or a main pulmonary artery/ascending aorta diameter ratio > 1, all suggestive of pulmonary hypertension (PH). The right heart catheterization (RHC) serves as the gold standard for diagnosis, being a low-risk outpatient procedure with a mortality rate below 0.1 % when performed in expert centers [1]. Echocardiography (transthoracic or trans-esophageal) helps in evaluating the cardiac function and also provides information regarding the presence of Pulmonary Artery Aneurysm [5]. Moreover, Magnetic Resonance Imaging can help in characterizing aortic and pulmonary hemodynamics as well [5].

Treatment options for the management of Pulmonary Artery Aneurysm are unpredictable and hence decided based upon the underlying causes, co-morbid conditions and hemodynamic stability of the patient [1]. The conservative management of pulmonary artery aneurysm includes treatment of the underlying etiology and lose follow-up with routine radiographic imaging to determine the increasing size of the aneurysm [1]. Interventional approach such as coil embolization is a seemingly new approach for iatrogenic and idiopathic aneurysms [5]. Surgical resection of the pulmonary artery aneurysm can be opted for, when the aneurysm measures>5.5 cm or with the manifestation of disease complications [2].

In the above presented case, we had opted for the surgical resection since the disease seemed to be refractory to medical management and the patient had presented with worsening symptoms. Furthermore, the size of the aneurysm was 7 × 7 cm which is a rare finding on the main pulmonary trunk according to the literature found.

4 Conclusion

Pulmonary Artery Aneurysms do not manifest any significant symptoms hence the diagnosis becomes very challenging [1]. Computed Tomography Scans of chest has helped in the increasing diagnosis of Pulmonary Artery Aneurysms [1]. No specific guidelines have been designed till yet with regard to treatment options; however, surgical resection has been advised in gradually increasing aneurysms [1].

Consent

Informed consent was taken from the patient.

Ethical approval

Our case report has been exempted from ethical review of our institute.

Funding

None.

Author contribution

1. Abdul Ahad Sohail: Visualization, Initial draft, Supervision.

2. Varisha Madni: Data collection, Initial draft, Editing of the original draft.

3. Rida Wahab: Data collection, Initial draft, Editing of the original draft.

4. Waris Ahmed: Project Administration and Supervision.

5. Hina Inam: Project Administration and Supervision.

Guarantor

Abdul Ahad Sohail.

Research registration number

Does not apply.

Conflict of interest statement

None.
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