Giant high-pressure pulmonary artery aneurysm (PAA) in a patient with COPD and chronic pulmonary embolism (PE)
- 1 Department of Cardiovascular Medicine, UC Davis Medical Center, Sacramento, California, USA
- 2 University of Missouri Health Care, Columbia, Missouri, USA
- Correspondence to Dr Hee Kong Fong; heekong@gmail.com
Abstract
A woman in her 60s with a history of known severe chronic obstructive pulmonary disease (COPD), former smoker of 50 pack-years and small patent foramen ovale (PFO) without significant shunt was admitted for acute on chronic hypoxic respiratory failure. Diagnostic workup showed severe dilatation of main pulmonary artery (MPA) (75.5 mm axial view and 86.6 mm sagittal view) and left and right PAs measuring 40 mm and 34 mm, respectively, on CT angiography of the chest. Right heart catheterisation showed severe pulmonary hypertension (PH). A diagnosis of giant high-pressure pulmonary arterial aneurysm (PAA) secondary to PH, induced by COPD, and chronic pulmonary embolism was made. Despite aggressive medical management, she passed away on comfort care. Giant high-pressure PAAs are rarely reported. This is a unique case that demonstrates this very rare condition in a living patient. The management of giant PAA is controversial. Experts recommend medical management or aneurysmectomy for sizes of >55–60 mm. Death could have been prevented if our patient was screened earlier and received appropriate medical care.
Background
Pulmonary arterial aneurysms (PAAs) are rare, often found by autopsy, and are defined as giant PAAs by some authors when the diameter is >50 mm. There is no clear consensus on management of giant PAAs, which make them difficult to manage and treat.
We report a case of a patient who was diagnosed with a giant high-pressure PAA secondary to PH, induced by chronic obstructive pulmonary disease (COPD), and chronic PE. This patient was not diagnosed with the PAA until her admission when she was undergoing diagnostic workup. Admission was for acute on chronic hypoxic respiratory failure and despite aggressive medical management, outside of intubation, the patient passed on comfort care.
PAAs may be more common than we know and earlier screening and management of patients at risk may benefit these patients by preventing progression of the aneurysm, as aneurysm enlargement could possibly lead to rupture or dissection and ultimately death. Considering earlier medical therapy and treatment of PH as well as long-term conservative management and assessments may help prevent the enlargement of the aneurysm and the need for aneurysmectomy for sizes of >55–60 mm.
Case presentation
A woman in her 60s with a history of known severe COPD, former smoker of 50 pack-years, mild coronary artery disease, first-degree atrioventricular (AV) block and small PFO without significant atrial septal defect (ASD) presented with 3 weeks of progressive debilitating shortness of breath and new abdominal and lower extremity oedema.
She presented initially requiring high flow nasal cannula 50 L/min at 50% with diffuse bilateral expiratory wheezes, grade 3 pansystolic murmur at tricuspid area and 2+ bilateral pitting pedal oedema. N-terminal-pro hormone BNP (NT-proBNP) of 6488 pg/mL with undetectable serum troponin. Twelve-lead ECG showed first degree AV block, right axis deviation and non-specific ST and T wave abnormality. Chest radiograph (figures 1 and 2) showed massively dilated pulmonary arteries (PAs) with diffuse interstitial opacities consistent with pulmonary hypertension (PH). Transthoracic echocardiogram revealed severely dilated right ventricle (RV) with moderately reduced systolic function (video 1), severely dilated main PA (MPA) (figure 3), severe tricuspid regurgitation (video 2) with estimated right ventricular systolic pressure (RVSP) of 90 mm Hg (figure 4) and dilated inferior vena cava with poor inspiratory collapse (figure 5). CT angiography of the chest (figures 6 and 7) confirmed severe dilatation of MPA (75.5 mm axial view and 86.6 mm sagittal view), left and right PAs measuring 40 mm and 34 mm, respectively, and chronic, non-occlusive thrombus in right lower lobe subsegmental PA. Parenchymal window of CT of the chest (figure 8) demonstrated moderate emphysematous changes.
Chest radiograph (frontal view) shows massively dilated main pulmonary artery (arrow) and dilated right interlobar artery (arrowhead).
Chest radiograph (lateral view) shows dilated pulmonary artery and right ventricular enlargement.
Two-dimensional echocardiography (parasternal short-axis view) shows severely dilated main pulmonary artery up to 7.4 cm.
Discussion
PAAs are rare and often an autopsy finding.1 2 Giant PAAs are defined by some authors when diameter is >50 mm.3 4 Aetiology for PAA without arteriovenous (AV) communication include infection such as schistosomiasis, syphilis and tuberculosis; structural cardiac or vascular abnormalities; and PH.5 6 PH is associated with 66% cases of PAA.2 Based on WHO classification, she was diagnosed as group III (COPD) and group IV (chronic thromboembolic pulmonary hypertension (CTEPH)) PH.7 To the best of our knowledge, only one giant PAA case due to COPD has been reported.8 Other potential causes of PAAs are vasculitides such as Behcet disease and Hughes-Stovin syndrome, as well as heritable connective tissues diseases such as Ehlers-Danlos and Marfans syndromes.9–11 This case is unique that it illustrates giant high-pressure PAA in a living patient with both COPD and chronic PE. Other authors have reported giant PAAs (max 120 mm) due to idiopathic pulmonary arterial hypertension.12 13 Even larger aneurysmal sacs associated with AV communication, particularly patent ductus arteriosus, have been described.3 14
Diagnostic modalities of PAA include ECG, chest radiography, transthoracic/transoesophageal echocardiography, MRI, angiography and the gold standard contrast-enhanced CT.5 15
There is no consensus on management of giant high-pressure PAAs due to its rarity, but most authors recommend surgical intervention with aneurysmectomy for sizes of >55–60 mm for risks of impending rupture and dissection.15–17 Medical therapy is warranted in PH irrespective of surgical candidacy, either for the purpose of controlling PH before surgery or as long-term conservative management with regular assessments.15 18
Learning points
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Giant pulmonary artery aneurysms (PAAs) are very rare and are often an autopsy finding.
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Our case is unique that it demonstrates giant high-pressure PAA in a living patient with both chronic obstructive pulmonary disease and chronic pulmonary embolism.
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Surgical intervention is recommended for giant high-pressure PAAs of >55–60 mm, with preoperative medical therapy for adequate control of pulmonary hypertension.
Ethics statements
Patient consent for publication
Footnotes
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Contributors HKF, AP, BB and FS played a role in planning, conducting, reporting, conception and design, acquisition, analysis and interpretation of data. Each author participated equally in this case report.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
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Competing interests None declared.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.
References
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