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.

• 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]

• Fever

  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 [symptoma.com]

  Though the fever subsided for 6-8 hours with the help of an anti-pyretic, it recurred shortly after that. According to her mother, the baby was born in a cyanosed state, and was kept on a ventilator for one day. [jpma.org.pk]

  Conditions that can have pulmonary valve stenosis as a complication include rheumatic fever and carcinoid tumors in the digestive system. Untreated pulmonary valve stenosis can lead to a number of harmful and deadly complications. [healthline.com]

  When to seek medical advice Call your healthcare provider right away if any of these occur: Increased pain, swelling, redness, bleeding, or drainage at the catheter insertion site Fever An irregular heartbeat Breathing difficulty, shortness of breath, [fairview.org]

  Call your healthcare provider if you have fever, increased draining from the wound, or any severe symptoms. Follow all the instructions your healthcare provider gives you for medicine, exercise, diet, and wound care. [hopkinsmedicine.org]

• Cyanotic Congenital Heart Disease

  Keywords: Double outlet right ventricle, Cyanotic heart disease, Atrioventricular canal defect, Pulmonary stenosis. [jpma.org.pk]

  Noninvasive quantitative evaluation of the morphology of the major pulmonary artery branches in cyanotic congenital heart disease. Angiocardiographic and echocardiographic correlative study. Circulation. 1994;89:1306–16. [link.springer.com]

  […] for the management of adults with congenital heart disease). [nature.com]

• Hypoxemia

  OBJECTIVE: After lung transplantation, pulmonary artery stenosis (PAS) may occur at the anastomotic site, resulting in poor graft function and hypoxemia. [ncbi.nlm.nih.gov]

  By then, his functional status started becoming worse with progressive increased dyspnea, pulmonary infections, cyanosis due to a severe chronic hypoxemia and secondary erythrocytosis. [cardiothoracicsurgery.biomedcentral.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]

• Systolic Murmur

  Right ventricular systolic pressure was estimated as 55-60 mmHg from the tricuspid regurgitation jet. For further evaluation of the systolic murmur, agitated saline contrast echocardiography was performed. [ncbi.nlm.nih.gov]

  Systolic Murmurs - Pulmonary Stenosis You are listening to a typical example of the murmur caused by pulmonary valve stenosis. [med.ucla.edu]

  Functional or physiologic PPS is a common cause of a systolic murmur in infants. It occurs in both premature and full-term infants; with time, the pulmonary artery grows, and the murmur usually disappears within a few months. [emedicine.medscape.com]

  There was an ejection systolic murmur of grade 3/6 at upper left sternal border. Rest of the systemic examination was unremarkable. [jpma.org.pk]

• Ejection Murmur

  Signs include a crescendo-decrescendo ejection murmur. Diagnosis is by echocardiography. Symptomatic patients and those with large gradients require balloon valvuloplasty. [merckmanuals.com]

  • Pulmonic stenosis without VSD= 8% of all CHD • Mostly asymptomatic • When symptomatic, cyanosis and heart failure • Loud systolic ejection murmur • Cor pulmonale • Rare calcification of pulmonary valve in older adults X-ray • Enlarged main pulmonary [learningradiology.com]

  A systolic ejection murmur of Grade 3/6 intensity was audible in left second intercostal space. Another peculiar finding was a continuous murmur in the back and both axilla. [jcecho.org]

• Diastolic Murmur

  On examination, apart from right upper zone crapitations, a diastolic murmur was heard. The skin of the entire body was extremely loose, hanging in folds, soft and inelastic. The changes were remarkable on face, neck, and flexures. [idoj.in]

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]

• Ventricular Hypertrophy

  It may reveal findings consistent with right ventricular hypertrophy which results from a systolic overload of the structure. Selective angiocardiography. A procedure that greatly contributes to the diagnosis of PAS. [symptoma.com]

  Concentric ventricular hypertrophy occurs in response to pressure overload. [pedsinreview.aappublications.org]

  With severe valvular PS, subvalvular right ventricular hypertrophy can cause infundibular narrowing and contribute to the right ventricular outflow obstruction. This often regresses after correction of valvular stenosis. [emedicine.medscape.com]

  The heart's apical impulse may be laterally and inferiorly displaced as a result of left ventricular hypertrophy. Alarming symptoms include anginal chest pain, syncope, and dyspnea on exertion. [medical-dictionary.thefreedictionary.com]

  One example is right ventricular hypertrophy, or heart enlargement. This can weaken and permanently damage the heart. It may also cause irregular heartbeats. [healthline.com]

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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