Giant Pulmonary Artery Aneurysm in a Patient With Marfan Syndrome and Pulmonary Hypertension

Chiu, Peter; Irons, Mallory L.; Rijn, Matt van de; Liang, David; Miller, D. Craig

doi:10.1161/circulationaha.115.020537

Cited by 8 publications

(7 citation statements)

References 4 publications

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“…A review of the current literature revealed three cases of PAA associated with Marfan’s syndrome, 30 α1 antitrypsin deficiency, 31 and tuberous sclerosis. 32 Ehler–Danlos syndrome is also known to cause PAA, but we did not identify any reported cases during our extensive literature review.…”

Section: Etiologiesmentioning

confidence: 99%

Pulmonary artery aneurysm: a review

et al. 2020

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Pulmonary artery aneurysm is a rare but important entity in the spectrum of pulmonary vascular diseases. The etiologies can be varied and patients can present with non-specific symptoms with the diagnosis being incidental. There is limited consensus regarding the diagnostic criteria and follow-up imaging for patients diagnosed with this entity. Further the management strategies can be variable depending upon underlying disease, etiology, center dependent expertise, and resources available. We review the etiologies, epidemiology, classification, clinical manifestations, and imaging features of pulmonary artery aneurysm. We also review the current management strategies and suggest an algorithmic approach to these patients.

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Section: Etiologiesmentioning

confidence: 99%

Pulmonary artery aneurysm: a review

et al. 2020

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“…Pulmonary artery aneurysms constitute <1% of aneurysms occurring in the thoracic cavity [ 29 ]. The cause could be idiopathic but many medical conditions including congenital heart defects, connective tissue disorders, systemic vasculitides, and infections are often associated with PAA [ 3 , 4 , 8 , 9 , 14 , 16 – 24 ]. Clinical presentation is invariably nonspecific and roughly depends on the underlying etiology, location, and size of the aneurysm [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

“…Causes of PAA are numerous and diverse in pathogenesis, however, congenital cardiac defects (patent ductus arteriosus, ventricular and atrial septal defects) are implicated in about 50% of cases [ 19 , 20 ]. Other causes include infections (tuberculosis, syphilis, mycotic aneurysms), systemic vasculitides (Behcet’s disease, giant cell arteritis), connective tissue disorders (Marfan’s syndrome, Hughes-Stovin syndrome), degenerative diseases (atherosclerosis), chest trauma, and idiopathic PAA [ 3 , 4 , 8 , 9 , 14 , 16 – 24 ]. The clinical manifestations of PAA are largely nonspecific but dyspnea, palpitations, chest pain, cough, and hemoptysis are frequently reported in symptomatic patients [ 1 – 28 ].…”

Section: Introductionmentioning

confidence: 99%

Bilateral multiple pulmonary artery aneurysms associated with cavitary pulmonary tuberculosis: a case report

Pallangyo¹,

Lyimo

Bhalia³

et al. 2017

J Med Case Reports

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BackgroundPulmonary artery aneurysms constitute <1% of aneurysms occurring in the thoracic cavity. Congenital cardiac defects are responsible for the majority (>50%) of cases, however, pulmonary artery aneurysm is a rare sequelae of pulmonary tuberculosis reported in about 5% of patients with chronic cavitary tuberculosis on autopsy. The natural history of this potentially fatal condition remains poorly understood and guidelines for optimal management are controversial.Case presentationA 24-year-old man, a nursing student of African descent, was referred to us from an up-country regional hospital with a 4-week history of recurrent episodes of breathlessness, awareness of heartbeats and coughing blood 3 weeks after completing a 6-month course of anti-tuberculosis drugs. A physical examination revealed conjuctival and palmar pallor but there were no stigmata of connective tissue disorders, systemic vasculitides or congenital heart disease. An examination of the cardiovascular system revealed accentuated second heart sound (S2) with early diastolic (grade 1/6) and holosystolic (grade 2/6) murmurs at the pulmonic and tricuspid areas respectively. Blood tests showed iron deficiency anemia, prolonged bleeding time, and mild hyponatremia. A chest radiograph revealed bilateral ovoid-shaped perihilar opacities while a computed tomography scan showed bilateral multiple pulmonary artery pseudoaneurysms with surrounding hematoma together with adjacent cystic changes, consolidations, and tree-in-bud appearance. Our patient refused to undergo surgery and died of aneurismal rupture after 9 days of hospitalization.ConclusionsThe presence of intractable hemoptysis among patients with tuberculosis even after completion of anti-tuberculosis course should raise an index of suspicion for pulmonary artery aneurysm. Furthermore, despite of its rarity, early recognition and timely surgical intervention of pulmonary artery aneurysm is crucial to reducing morbidity and preventing the attributed mortality.

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“…Marfan syndrome: The dilatation of the pulmonary artery was a minor cardiovascular criteria for Marfan Syndrome diagnosis until the definition of the revised Ghent nosology, where it was not included [38,71]. However, up to a 74% of patients with Marfan Syndrome present a PA dilatation [72] and 15% a PAA [73], including PAA and PA dissection, which confirms that histological changes also affect the pulmonary vasculature [39,40].…”

Section: Meester-loeys Syndromementioning

confidence: 97%

Potential Molecular Pathways Related to Pulmonary Artery Aneurysm Development: Lessons to Learn from the Aorta

Nuche

Palomino-Doza

Arribas

et al. 2020

IJMS

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Pulmonary arterial hypertension (PAH) is a rare disease caused by pulmonary vascular remodeling. Current vasodilator treatments have substantially improved patients' survival. This improved survival has led to the appearance of complications related to conditions previously underdiagnosed or even ignored, such as pulmonary artery aneurysm (PAA). The presence of a dilated pulmonary artery has been shown to be related to an increased risk of sudden cardiac death among PAH patients. This increased risk could be associated to the development of left main coronary artery compression or pulmonary artery dissection. Nevertheless, very little is currently known about the molecular mechanisms related to PAA. Thoracic aortic aneurysm (TAA) is a well-known condition with an increased risk of sudden death caused by acute aortic dissection. TAA may be secondary to chronic exposure to classic cardiovascular risk factors. In addition, a number of genetic variants have been shown to be related to a marked risk of TAA and dissection as part of multisystemic syndromes or isolated familial TAA. The molecular pathways implied in the development of TAA have been widely studied and described. Many of these molecular pathways are involved in the pathogenesis of PAH and could be involved in PAA. This review aims to describe all these common pathways to open new research lines that could help lead to a better understanding of the pathophysiology of PAH and PAA and their clinical implications.

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Giant Pulmonary Artery Aneurysm in a Patient With Marfan Syndrome and Pulmonary Hypertension

Cited by 8 publications

References 4 publications

Pulmonary artery aneurysm: a review

Pulmonary artery aneurysm: a review

Bilateral multiple pulmonary artery aneurysms associated with cavitary pulmonary tuberculosis: a case report

Potential Molecular Pathways Related to Pulmonary Artery Aneurysm Development: Lessons to Learn from the Aorta

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