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Pulmonary Artery Stenosis

Pulmonary Artery Steis

Pulmonary artery stenosis (PAS) is a congenital or acquired disease that leads to the formation of narrowed sections of the main pulmonary artery or the peripheral branches of the vessel.


Presentation

Pulmonary artery stenosis (PAS) is largely divided into two distinct categories depending on the location of the stenosis: type 1 and type 2. Type 1 PAS involves one or multiple sites of arterial stenosis found in the main pulmonary artery or its major branches, whereas type 2 PAS results in one or more narrowings in the peripheral branches of the pulmonary arteries [1].

Patients may be pediatric but the condition might as well be diagnosed during adulthood. Some individuals with arterial stenoses that amount to less than 50% of the vascular diameter may experience no symptomatology. Patients with more profound stenosis usually exhibit dyspnea and fatigue, poor appetite, tachypnea, and low exercise tolerance, while cyanosis, tachycardia, peripheral edema, and ascites are also possible. Dyspnea and fatigue constitute the symptoms typically involved in the vast majority of PAS cases and are typically elicited following exercise; most individuals are in their second decade of life when symptoms are initially reported [2]. Hepatomegaly may also be found and together with peripheral edemas greatly suggest the existence of right ventricular failure on the grounds of PAS.

In general, most patients affected by type 1 pulmonary artery stenosis are asymptomatic. The most common finding that raises suspicion and necessitates further investigations is a continuous, ductus-like murmur heard during cardiac auscultation. On the other hand, type 2 PAS can either be an isolated abnormal finding or manifest as part of another clinical entity such as Alagille syndrome, Williams-Beuren syndrome (WBS), or following pulmonary artery surgical reconstruction [3] [4]. Therefore, patients with peripheral pulmonary artery stenosis may also exhibit symptoms directly associated with one of these disorders, such as dental malformation and mental retardation, that is caused by Williams-Beuren syndrome. Lastly, both Behçet disease and Takayasu arteritis encompass type 2 PAS in their clinical spectrum and patients affected by the aforementioned disorders are also expected to present with corresponding manifestations such as fever, hypertension, and heart failure in the case of Takayasu arteritis, or uveitis, oral aphthous ulcers, and genital ulcers in Behçet disease [5] [6] [7] [8] [9]. Pulmonary hypertension is a common manifestation of type 2 pulmonary artery stenosis.

Aspiration
  • There was 1 late, noncardiac death from aspiration pneumonia in a patient with a severe craniofacial defect. Follow-up echocardiograms in the intermediate term have demonstrated relief of pulmonary artery narrowing and unobstructed aortic flow.[ncbi.nlm.nih.gov]
Orthopnea
  • A 44-year-old man was admitted to our institution with orthopnea. Chest computed tomography showed an extrinsic compression at the bilateral main bronchus and right pulmonary artery due to a mediastinal mass.[ncbi.nlm.nih.gov]
Hepatomegaly
  • Hepatomegaly may also be found and together with peripheral edemas greatly suggest the existence of right ventricular failure on the grounds of PAS. In general, most patients affected by type 1 pulmonary artery stenosis are asymptomatic.[symptoma.com]
Aphthous Ulceration
Heart Disease
  • Abstract Pulmonary artery stenosis is a challenging problem in the management of congenital heart disease.[ncbi.nlm.nih.gov]
Vascular Disease
  • Her pulmonary ventilation and blood flow scintigraphy findings were suspicious of pulmonary vascular disease; the diagnosis was pulmonary hypertension and bilateral branch pulmonary artery stenosis.[ncbi.nlm.nih.gov]
Ulcer
Agitation
  • For further evaluation of the systolic murmur, agitated saline contrast echocardiography was performed.[ncbi.nlm.nih.gov]

Workup

Pulmonary artery stenosis often presents a challenge concerning its diagnosis, due to the fact that the disorder is often asymptomatic. The patients that do not experience PAS-related symptoms may be diagnosed incidentally during tests conducted for other cardiac abnormalities, whereas those who complain about significant symptomatology are required to undergo various procedures to reach an accurate diagnosis. A complete workup involves the following tests:

  • Thoracic radiograph. A chest X-ray may reveal regions of stenosed pulmonary arteries in some patients, by illustrating the dilated section following the stenosis. Pulmonary vessels might appear as diminished, augmented, or normal, depending on the particular characteristics of the vascular abnormality involved.
  • Electrocardiogram. It may reveal findings consistent with right ventricular hypertrophy which results from a systolic overload of the structure [10].
  • Selective angiocardiography. A procedure that greatly contributes to the diagnosis of PAS.
  • Catheter-guided pulmonary arteriography. It is employed to detect areas of stenosis and can also diagnose right ventricular hypertension [11]. It does constitute the gold standard for the detection of peripheral pulmonary artery stenosis [12].
  • Pulmonary perfusion imaging. The procedure may reveal localized imbalance in lung perfusion but remains a test with no specificity for PAS [12].
Right Axis Deviation
  • Electrocardiography (ECG) was in favour of right axis deviation and right ventricular prominence.[jpma.org.pk]

Treatment

  • The safety and efficacy of balloon-expandable stents for treatment of peripheral pulmonary artery stenosis (PPAS) is evaluated and analyzed.[ncbi.nlm.nih.gov]

Prognosis

  • Pulmonary artery compression in adults resulting from tumors is an uncommon condition often associated with poor prognosis.[ncbi.nlm.nih.gov]
  • Although the long-term prognosis in our patient remains uncertain, the percutaneous insertion of a stent restored perfusion to the transplanted lung and avoided the risk of further surgery.[err.ersjournals.com]
  • Pulmonary artery stenosis usually improves and only rarely limits prognosis. Received September 28, 1989. Revision received December 20, 1989. Accepted January 10, 1990.[onlinejacc.org]
  • Cardiopulmonary abnormalities are common in Type I recessive CL and are the main factors to determine the prognosis and life expectancy.[idoj.in]

Etiology

  • The surgery indicated a probable etiology of chronic pericarditis. This is an extremely rare case of adult pulmonary artery stenosis without a known history of congenital disease, constrictive pericarditis, tuberculosis, or surgery.[ncbi.nlm.nih.gov]
  • […] ses ) an abnormal narrowing or contraction of a body passage or opening; called also arctation, coarctation, and stricture. aortic stenosis obstruction to the outflow of blood from the left ventricle into the aorta; in the majority of adult cases the etiology[medical-dictionary.thefreedictionary.com]
  • Pulmonary vein stenosis: Etiology, diagnosis and management. World J Cardiol 2016;8:81-8. 4. Seale AN, Daubeney PE, Magee AG, Rigby ML. Pulmonary vein stenosis: Initial experience with cutting balloon angioplasty. Heart 2006;92:815-20. 5.[jcecho.org]

Epidemiology

  • According to epidemiological estimates, pregnant women who smoke have almost double the risk of low birth weight and three times the risk of premature birth than pregnant women who do not smoke.[germanchoices.blogspot.com]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology A systolic pressure gradient between the pumping chamber and its downstream artery is the primary hemodynamic abnormality of stenosis regardless of whether the obstruction is right- or left-sided.[pedsinreview.aappublications.org]
  • February 2017, Pages 180–185, Download citation file: Ris (Zotero) EndNote BibTex Medlars ProCite RefWorks Reference Manager 2019 Oxford University Press Close Navbar Search Filter Mobile Microsite Search Term Close search filter search input Aims The pathophysiology[academic.oup.com]

Prevention

  • However, it is not always easy to prevent the right pulmonary artery twisting after implantation of the extracardiac conduit.[ncbi.nlm.nih.gov]
  • […] ventricular septal defect, 2) a narrowing at or just beneath the pulmonary valve, 3) a right ventricle that is more muscular than normal, 4) an aorta that lies directly over the ventricular septal defect Pulmonary atresia – absence of a pulmonary valve, preventing[my.clevelandclinic.org]

References

Article

  1. Baum D, Khoury GH, Ongley PA, Swan HJC, Kincaid OW. Congenital Stenosis of the Pulmonary Artery Branches. Circulation.1964;29:680-687.
  2. Kushner T, Halperin JL, Nair AP, Fuster V, Love BA. Peripheral pulmonary artery stenosis masquerading as pulmonary hypertension: a diagnostic and therapeutic challenge. Vasc Med 2012;17(4):235–238.
  3. Trivedi KR, Benson LN. Interventional strategies in the management of peripheral pulmonary artery stenosis. J Interv Cardiol. 2003;16(2):171–188.
  4. Inglessis I, Landzberg MJ. Interventional catheterization in adult congenital heart disease. Circulation. 2007;115(12):1622–1633.
  5. Luo Q, Zhang HL, Liu ZH, Xiong CM, Ni XH. Percutaneous transluminal angioplasty and stenting for pulmonary stenosis due to Takayasu’s arteritis: clinical outcome and four-year follow-up. Clin Cardiol. 2009;32(11):639–643.
  6. Pelage JP, El Hajjam M, Lagrange C, et al. Pulmonary artery interventions: an overview. Radiographics 2005;25(6):1653–1667.
  7. Toledano K, Guralnik L, Lorber A, et al. Pulmonary arteries involvement in Takayasu’s arteritis: two cases and literature review. Semin Arthritis Rheum. 2011;41(3):461–470.
  8. Fei Y, Li X, Lin S, et al. Major vascular involvement in Behçet’s disease: a retrospective study of 796 patients. Clin Rheumatic. 2013;32(6):845–852.
  9. Seyahi E, Melikoglu M, Akman C, et al. Pulmonary artery involvement and associated lung disease in Behçet disease: a series of 47 patients. Medicine (Baltimore). 2012;91(1):35–48.
  10. Eldridge F, Selzer A, Hultgren H. Stenosis of a Branch of the Pulmonary Artery:An Additional Cause of Continuous Murmurs over the Chest. Circulation. 1957;15(6):865-874.
  11. Kreutzer J, Landzberg MJ, Preminger TJ, et al. Isolated Peripheral Pulmonary Artery Stenoses in the Adult. Circulation. 1996;93:1417-1423
  12. Tonelli AR, Ahmed M, Hamed F, Prieto LR. Peripheral pulmonary artery stenosis as a cause of pulmonary hypertension in adults. Pulm Circ. 2015; 5(1): 204–210.

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Last updated: 2019-07-11 20:28