Progression of pulmonary artery dilatation in patients with pulmonary hypertension coexisting with a pulmonary artery aneurysm

Akagi, Satoshi; Nakamura, Kazufumi; Sarashina, Toshihiro; Ejiri, Kentaro; Kasahara, Shingo; Ito, Hiroshi

doi:10.1016/j.jjcc.2017.11.005

Cited by 11 publications

(19 citation statements)

References 18 publications

(20 reference statements)

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“…Other case report of 65-year-old patient with proximal PAA and PH which had been treated conservatively with intravenous epoprostenol showed that after 20 months of treatment, pulmonary artery pressure was nearly normalized and the size of PAA remains the same [18]. However, recent studies found that the progression of PAA dilatation is independent of the change in pulmonary artery pressure [19,20]. We argue that the difference between our findings or the previous case reports and the previous cohort studies was because of the difference in the follow-up period.…”

Section: A B Cmentioning

confidence: 99%

Proximal Pulmonary Artery Aneurysm Secondary to Suspected Pulmonary Hypertension Treated with Conservative Therapy in Limited Resource Setting

Intan

Hasibuan²,

Nugraha

et al. 2019

Am J Case Rep

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Rare disease Background: Pulmonary artery aneurysm (PAA) is a rare disease in cardiovascular system. This disease is difficult to diagnose and less often considered due to its non-specific clinical manifestations. Until now there are no clear guidelines about its optimal management because of the small number of reported cases. Case Report: We report a 56-year-old male with chief complain of atypical bilateral chest pain and shortness of breath. Initial electrocardiogram (ECG) and laboratory evaluation showed no sign of ischemic heart disease. After the patient was stabilized, he was evaluated using chest x-ray, transthoracic echocardiography (TTE), and multi slice computed tomography (MSCT). The patient was then diagnosed with PAA secondary to suspicion of pulmonary hypertension (PH) with chronic obstructive pulmonary disease and heart failure. Conservative treatment was chosen because of the limited resources for surgery and patient's refusal to be referred. The treatment aims to lower the pulmonary artery pressure while monitoring the aneurysm. His 6-month follow-up evaluation showed an improvement in pulmonary artery pressure and persistent of the PAA without any increasement of the diameter. Conclusions: PAA is a rare disease that is difficult to diagnose because of its non-specific nature. Persistent atypical chest pain can be an early symptom of PAA, thus clinicans should be aware in a high-risk patient suffered persistent chest pain, despite normal ECG and laboratory findings. TTE and MSCT evaluation are reliable for diagnosing PH and PAA. With conservative treatment and routine follow-up, patient with PAA secondary to PH could be managed well.

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Section: A B Cmentioning

confidence: 99%

Proximal Pulmonary Artery Aneurysm Secondary to Suspected Pulmonary Hypertension Treated with Conservative Therapy in Limited Resource Setting

Intan

Hasibuan²,

Nugraha

et al. 2019

Am J Case Rep

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“…However, despite PAA being common among PAH patients, hemodynamic severity of PAH has not shown to be an independent risk factor for PAA development in this population [1]. Furthermore, effective PA pressure reduction with pulmonary vasodilators have failed to stop progressive PA enlargement [2,7]. Thus, it seems that a chronic exposure to an increased afterload worsen irreversible ECM changes leading to PA growth [1].…”

Section: Hemodynamic Overload Contributes To Development Of Both Aortmentioning

confidence: 99%

“…However, not every patient exposed to an increased intravascular pressure develops a great vessel aneurysm. Furthermore, the effect of antihypertensive drugs or pulmonary vasodilators on artery diameter change is debatable [2,7]. Thus, besides increased intravascular pressure, patients who finally develop abnormal artery growth must present a genetic susceptibility triggering ECM changes responsible of aneurysm development.…”

Section: Hemodynamic Overload Contributes To Development Of Both Aortmentioning

confidence: 99%

“…However, this finding is controversial since other published works have shown that a worse hemodynamic profile does not necessarily lead to PAA development [1]. Furthermore, it seems that PA pressure reduction with pulmonary vasodilators is not able to stop PA enlargement in PAH patients [2,7]. PA dilatation is related to an increased risk of sudden cardiac death [8] and among patients with PAA a non-significant higher proportion of sudden cardiac death has been described [1].…”

Section: Introductionmentioning

confidence: 99%

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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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“…Aneurysm and pseudoaneurysm are uncommon, but potentially life-threatening abnormalities of the pulmonary arteries. Aneurysm of the main pulmonary artery (MPA) defined as MPA diameter over 40 mm was reported in 1 : 14 000 autopsies [1,2]. By definition, an aneurysm is a local dilatation of a blood vessel, involving all three layers of the vessel wall: the tunica intima, tunica media, tunica adventitia [3].…”

Section: Introductionmentioning

confidence: 99%

Pulmonary artery aneurysm as a result of group 2 pulmonary artery hypertension in a patient with significant mitral and aortic valve disease – review and case report

Marzec¹,

Jaworska-Wilczyńska²,

Grobelny³

et al. 2020

kitp

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Aneurysms are uncommon, but potentially life-threatening abnormalities of the pulmonary arteries. Aneurysm of the main pulmonary artery (MPA) defined as MPA diameter over 40 mm was reported in 1 : 14 000 autopsies. The most frequent location is the main pulmonary artery (89% of cases), whereas the maximum described diameter is 106-170 mm. Clinical manifestations are usually nonspecific or asymptomatic. Right heart failure symptoms, pulmonary regurgitation, trachea or bronchi compression or pulmonary emboli caused by enlarged MPA are the most commonly described clinical manifestations. Pulmonary artery aneurysm dissection is an uncommon complication but associated with a high mortality rate. Unfortunately, guidelines regulating the optimal time for the surgical intervention still have not been developed. We present the history of 76-year-old patient suffering from an aneurysm of the pulmonary artery (74 × 61 mm), as well as mitral and aortic valve disease, who was successfully operated on in our hospital.

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Progression of pulmonary artery dilatation in patients with pulmonary hypertension coexisting with a pulmonary artery aneurysm

Abstract: PA dilatation progressed in patients with a PA aneurysm despite treatment of PH. The progression of PA dilatation is independent of reduction of PA pressure by PH treatment.

Cited by 11 publications

References 18 publications

Proximal Pulmonary Artery Aneurysm Secondary to Suspected Pulmonary Hypertension Treated with Conservative Therapy in Limited Resource Setting

Proximal Pulmonary Artery Aneurysm Secondary to Suspected Pulmonary Hypertension Treated with Conservative Therapy in Limited Resource Setting

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

Pulmonary artery aneurysm as a result of group 2 pulmonary artery hypertension in a patient with significant mitral and aortic valve disease – review and case report

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