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.

• Pulmonary Valve Stenosis

  It should not be confused with a pulmonary valve stenosis, which is in the heart, but can have similar hemodynamic effects. Both stenosis of the pulmonary artery and pulmonary valve stenosis are causes of pulmonic stenosis. [en.wikipedia.org]

  The pulmonary valve is located between the right ventricle and the pulmonary artery. The valve acts as a doorway that lets blood into and out of the heart. Pulmonary valve stenosis is when the pulmonary valve does not open properly or wide enough. [healthline.com]

  Areas of potential narrowing include thickened muscle below the pulmonary valve, stenosis of the valve itself, or stenosis of the pulmonary artery above the valve. [cincinnatichildrens.org]

  […] of pulmonary valve stenosis. [medlineplus.gov]

  […] and onward to the lungs Truncus arteriosus – the formation of only one combined artery instead of the normal two outlets from the heart, the aorta and pulmonary artery Pulmonary valve stenosis – problems with the pulmonary valve (for example, development [my.clevelandclinic.org]

• Pain

  This condition causes back pain, often associated with pain that radiates into the legs, esp. when the patient is standing. Sitting often relieves the pain. [medical-dictionary.thefreedictionary.com]

  Seek medical treatment if you experience chest pain, shortness of breath, or fainting as a result of your pulmonary valve stenosis. These symptoms can indicate your condition is advancing. Mild stenosis may improve with time. [healthline.com]

  If the condition is severe, symptoms may include: Episodes of pain in the chest causing difficulty breathing Low energy Poor feeding Rapid breathing Tiring early with exercise Turning blue, a condition called cyanosis Because there may be no obvious symptoms [ucsfbenioffchildrens.org]

  Rauchen kann zu einem langsamen und schmerzhaften Tod führen - Smoking can lead to a slow and painful death. [germanchoices.blogspot.com]

• Atrial Septal Defect

  Two-dimensional echocardiography revealed two atrial septal defects (ASD), a ventricular septal defect (VSD) in the membranous area, continuous with primum ASD, resulting in an atrioventricular canal defect. [ncbi.nlm.nih.gov]

  NIH: National Heart, Lung, and Blood Institute Atrial septal defect (Medical Encyclopedia) Bicuspid aortic valve (Medical Encyclopedia) Congenital heart defect corrective surgeries (Medical Encyclopedia) Congenital heart disease (Medical Encyclopedia) [icdlist.com]

  With severe PS and decreased right ventricular chamber compliance, cyanosis can occur from right-to-left shunting if a concomitant patent foramen ovale, atrial septal defect, or ventricular septal defect is present. [emedicine.medscape.com]

  A case of congenital stenosis of the pul-monary artery associated with atrial septal defect of 15 years female was presented and a short review of the literatures concerning to that disease was carried out. [webview.isho.jp]

• Collapse

  Although it appears ideal for implantation in pulmonary artery stenoses in children, it has been unclear whether it has sufficient radial strength to prevent vessel recoil and stent collapse. [ncbi.nlm.nih.gov]

  The collapsed stent is introduced over a deflated balloon catheter into the femoral vein through a long and wide Mullins sheath. [rjmatthewsmd.com]

• Weakness

  Weak correlation was found between MPA diameter and its pressure (r = 0.399, p = 0.04) and moderate correlation was found between AAO diameter and pressure (r = 0.611; p = 0.01). [nature.com]

• Exertional Dyspnea

  A 60-year-old male with exertional dyspnea was referred to our hospital. Right pulmonary artery stenosis due to external compression by a calcified band was diagnosed by echocardiography, computed tomography, and magnetic resonance imaging. [ncbi.nlm.nih.gov]

  These patients typically present with exertional dyspnea and fatigue, and symptomatic improvement has been seen following balloon angioplasty.  Systemic vasculitis with pulmonary arterial involvement should be excluded.  The presence of murmurs consistent [slideshare.net]

• 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]

• Heart Disease

  Abstract Pulmonary artery stenosis is a challenging problem in the management of congenital heart disease. [ncbi.nlm.nih.gov]

  NIH: National Heart, Lung, and Blood Institute Atrial septal defect (Medical Encyclopedia) Bicuspid aortic valve (Medical Encyclopedia) Congenital heart defect corrective surgeries (Medical Encyclopedia) Congenital heart disease (Medical Encyclopedia) [icdlist.com]

  Implantation and intermediate-term follow-up of stents in congenital heart disease. Circulation. 1993;88:605–14. PubMed CrossRef Google Scholar 11. Stanger P, Heymann MA, Tarnoff H, et al. [link.springer.com]

• Heart Murmur

  This association, which has not been described before, may be clinically important in patients with lipodystrophy who present with impaired exercise tolerance or heart murmurs. [ncbi.nlm.nih.gov]

  The first sign of the condition often is a heart murmur, an extra sound heard during a chest examination. [ucsfbenioffchildrens.org]

  Pulmonary valve stenosis can cause a heart murmur. A heart murmur sounds like an extra click, blowing, whooshing, or rasping sound when a doctor listens to your heart. The murmur can be an initial indicator of pulmonary valve stenosis. [healthline.com]

  Cyanotic heart disease (Medical Encyclopedia) Dextrocardia (Medical Encyclopedia) Echocardiogram -- children (Medical Encyclopedia) Heart murmurs and other sounds (Medical Encyclopedia) Patent ductus arteriosus (Medical Encyclopedia) Ventricular septal [icdlist.com]

  The diagnosis of pulmonary stenosis is usually first suspected because a physician detects a heart murmur. The heart murmur of pulmonary stenosis is a turbulent noise caused by ejection of blood through the obstructed valve. [cincinnatichildrens.org]

• Chest Pain

  Seek medical treatment if you experience chest pain, shortness of breath, or fainting as a result of your pulmonary valve stenosis. These symptoms can indicate your condition is advancing. Mild stenosis may improve with time. [healthline.com]

  Alarming symptoms include anginal chest pain, syncope, and dyspnea on exertion. When these occur, surgery to repair or replace the diseased valve are necessary. [medical-dictionary.thefreedictionary.com]

  Other symptoms can include: shortness of breath (dyspnea); chest pain (angina); fainting (syncope), or cyanosis (blueness). If significant pulmonary stenosis is present then the child may feel tired when playing or doing any physical activity. [chd-uk.co.uk]

  pain Swelling in your legs or abdomen Cyanosis or high red blood cell count Diagnosis The most common diagnostic test used to confirm pulmonary stenosis is an echocardiogram. [achaheart.org]

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]

Cutting balloon angioplasty is a common treatment modality in the pediatric population for pulmonary artery stenosis. [ncbi.nlm.nih.gov]

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]

What is the prognosis? The prognosis for this condition is generally good, with survival without complications nearly 100%. In the long term, these children fare well and most have no symptoms and lead normal lives. [childrensheartcentre.com]

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]

∗∗ In the absence of other etiologies of LV dilatation. ψ Quantitative parameters can help sub-classify the moderate regurgitation group into mild-to-moderate and moderate-to-severe regurgitation as shown. [echopedia.org]

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]

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]

[…] doi.org/10.1093/ehjci/jew046 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]

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]

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