Pulmonary vein stenosisPPulmonary vein stenosisPulmonary vein stenosisEnglishCardiologyChild (0-12 years)Lungs;HeartLungs;Cardiovascular systemConditions and diseasesAdult (19+) CaregiversFatigue2018-03-22T04:00:00ZRachel Vanderlaan, MD;Christopher Caldarone, MD, FRCSC8.3000000000000059.30000000000001300.00000000000Health (A-Z) - ConditionsHealth A-Z<p>Pulmonary vein stenosis (PVS) is when there is a narrowing or blockage in the pulmonary veins. It is a rare disease and if it progresses, it can lead to pulmonary hypertension and right heart failure.</p><p>Pulmonary vein stenosis (PVS) affects the pulmonary veins. The pulmonary veins bring blood from the lungs back to the left atrium of the heart. In PVS, the pulmonary veins become narrowed. In some cases, the narrowing can cause the pulmonary veins to become blocked. This is caused by an abnormal build-up of cells inside the wall of the pulmonary vein. It is not known what causes the cells to build-up. PVS is a rare but very serious disease.</p><p>If multiple pulmonary veins are affected, it can cause pulmonary hypertension. As a result, the right heart has to work harder to pump blood through the lungs. If the lung pressure remains high, it may result in right heart failure.</p> <figure class="asset-c-80"><span class="asset-image-title">Pulmonary veins</span><img src="https://assets.aboutkidshealth.ca/AKHAssets/pulmonary_veins_EN.jpg" alt="" /><figcaption class="asset-image-caption">Typically, there are four pulmonary veins, two from each lung. These veins drain oxygen-rich blood from the lungs into the left atrium of the heart. This blood is then moved out into the body, supplying it with the oxygen needed for proper health and function.</figcaption> </figure> <h2>Key points</h2><ul><li>Each child is unique. The number of pulmonary veins affected and where the stenosis is located is not the same in every child.</li><li>There are two main categories: primary PVS and post-repair PVS. Primary PVS is when the child develops PVS without having any surgery on the pulmonary veins, while post-repair PVS develops after surgical repair of the pulmonary veins.</li><li>Progression of the disease can be clinically silent, meaning there are no symptoms.</li><li>PVS is a rare but very serious disease.</li><li>Children can have surgery or catheter-based interventions (balloon angioplasty or stents) to help relieve the narrowing and help blood flow better through the lungs. </li></ul> <h2>Signs and symptoms of PVS</h2><p>PVS can be clinically silent. This means a child might not have any symptoms of PVS until the disease is very advanced. </p><p>Some signs and symptoms of PVS may include:</p><ul><li>Fast breathing (tachypnea) </li><li>Fatigue or lack of energy</li><li>Some babies do not gain any weight, because their heart is working harder to pump blood through the lungs</li><li>Frequent <a href="/article?contentid=12&language=English">colds</a> or chest infections </li><li>Bleeding in the lungs</li></ul><p>None of the above signs or symptoms are specific to PVS. <br></p><h2>Understanding PVS</h2><p>The cause of PVS is not known. It is also not known why some children develop PVS that progresses, while others have PVS that remains more stable. No genetic cause for PVS has been identified. </p><p>Children who have more serious PVS are those who:</p><ul><li>have multiple pulmonary veins involved</li><li>have both lungs involved</li><li>have other heart defects </li> <li>are diagnosed at a very young age </li><li>have recurrent disease, meaning PVS continues to come back, despite treatment.</li></ul><p>Children who had a repair of anomalous pulmonary veins as an infant have around a 10% risk of developing pulmonary vein stenosis.</p><h2>Diagnosing PVS</h2><p>Because PVS can be clinically silent early on, many children will have a diagnosis of PVS made when they have tests for other reasons. </p><p>Children who are suspected of having PVS will have an <a href="/article?contentid=1274&language=English">echocardiogram</a> to look at the blood flow in the pulmonary veins and how well the right heart is pumping. </p><p>Your child’s doctor will also confirm the diagnosis with a <a href="/article?contentid=1272&language=English">CT scan</a>, <a href="/article?contentid=1270&language=English">MRI</a> or <a href="/article?contentid=1645&language=English">diagnostic catheterization</a>. Some health-care centres use <a href="/article?contentid=1301&language=English">lung perfusion scans</a> to help diagnose PVS. These tests let the doctors understand how many pulmonary veins are affected and how narrow the veins are. These tests are important to help plan the treatment of PVS.</p><h2>Treatment for PVS</h2><h3>Surgery</h3><p>Surgery can be done to relieve the narrowing (stenosis) in the pulmonary veins close to the atrium of the heart. The surgeon will relieve blocked areas of the pulmonary veins. This allows blood to flow unobstructed through the veins. Your child will be given a general anaesthesia to put them to sleep during the surgery.</p><h3>Catheter-based interventions</h3><p>In most cases, if only one pulmonary vein is affected, it may be treated with catheter-based interventions. These include <a href="/article?contentid=60&language=English">stent placement</a> and <a href="/article?contentid=1667&language=English">balloon angioplasty</a>. Both the stent and the balloon will open the area of stenosis and allow blood to flow through the veins.</p><p>One of the risks of catheter-based interventions is restenosis. This means that it is possible for narrowing of the pulmonary veins to redevelop in the future. </p><p>If the pulmonary vein is too narrowed to place a catheter through, surgery may be required. </p><h3>Other treatment options</h3> <p>A lung transplant may be an option for some children if they have progressive disease that cannot be treated by surgery or catheter-based interventions.</p><p>There are no proven medical therapies to treat PVS, but there are experimental therapies that are currently being tested. Your child may be given specific medications to help manage the symptoms of PVS.</p><h2>Complications of PVS</h2><p>Children with PVS may experience some complications including:</p><ul><li>Recurrent colds or infections that may require hospitalization</li><li>Some children may cough up blood from their lungs (hemoptysis) due to the high pressures in the lungs.</li></ul><p>Even after treatment, it is possible that PVS may recur (come back). It is important that your child undergo imaging tests of the pulmonary veins after any treatment to monitor stenosis. </p><p>Progressive PVS can lead to pulmonary hypertension and right heart failure.</p><h2>When to see a doctor</h2><p>You should see a doctor if: </p><ul><li>your child is breathing very fast</li><li>your child is not growing and keeping up with their peers</li> <li>your child develops repeated serious chest infections or coughs up blood. </li></ul><h2>At SickKids</h2><p>If your child is coming for surgery at SickKids, find important information here: <a href="http://www.sickkids.ca/visitingsickkids/coming-for-surgery/index.html">Coming for surgery</a></p><p>For more information on the Cardiology Clinic at SickKids: <a href="http://www.sickkids.ca/areas-of-care/clinics/cardiology-clinic.html">Cardiology Clinic</a></p> <h2>Resources</h2> <ul><li>PVS Network: <a href="http://www.pvsnetwork.org/">http://www.pvsnetwork.org</a></li></ul><h2>References</h2> <ul><li>M. Samanek, M. Voriskova. Congenital heart disease among 815,569 children born between 1980 and 1990 and their 15-year survival: a prospective Bohemia survival study Pediatric Cardiology, 20 (1999), pp. 411–417</li></ul>
Sténose des veines pulmonairesSSténose des veines pulmonairesPulmonary vein stenosisFrenchCardiologyChild (0-12 years)Lungs;HeartLungs;Cardiovascular systemConditions and diseasesAdult (19+) CaregiversFatigue2018-03-22T04:00:00ZRachel Vanderlaan, MD;Christopher Caldarone, MD, FRCSC8.3000000000000059.30000000000001300.00000000000Health (A-Z) - ConditionsHealth A-Z<p>La sténose des veines pulmonaires résulte du rétrécissement ou du blocage des veines pulmonaires. C’est une maladie rare qui évolue parfois vers l’hypertension pulmonaire et l’insuffisance congestive du cœur droit.</p><p>La sténose des veines pulmonaires affecte les veines pulmonaires, celles qui alimentent l’oreilllette gauche du cœur. La maladie réduit le volume de ces veines. Dans certains cas, cela peut aller jusqu’au blocage; on ignore la raison de la prolifération anormale des cellules et c’est leur accumulation sur la paroi des veines qui est responsable de la maladie. La sténose des veines pulmonaires est rare, mais très grave. L’atteinte de plusieurs veines pulmonaires peut causer l’hypertension pulmonaire. Le côté droit du cœur doit alors exercer plus de pression pour pomper le sang à travers les poumons. En cas de pression excessive pendant un certain temps, une insuffisance cardiaque droite peut se développer.</p> <figure class="asset-c-80"> <span class="asset-image-title">Veines pulmonaires</span> <img src="https://assets.aboutkidshealth.ca/AKHAssets/pulmonary_veins_FR.jpg" alt="" /> <figcaption class="asset-image-caption">On trouve d’ordinaire quatre veines pulmonaires, soit deux pour chaque poumon. Elles transportent du sang oxygéné depuis les poumons vers l’oreillette gauche du cœur. Le sang se dirige par la suite dans l’ensemble de l’organisme pour lui fournir l’oxygène dont il a besoin pour assurer son bon maintien et son fonctionnement normal.</figcaption> </figure> <h2>À retenir</h2><ul><li>Chaque enfant est unique. Le nombre de veines pulmonaires atteintes et l’emplacement de la sténose varient pour chaque enfant.<br></li><li>Les sténoses appartiennent à deux principaux types : les sténoses des veines pulmonaires dites primaires et celles qui surviennent à la suite d’une chirurgie réparatrice. Les sténoses primaires chez l’enfant apparaissent sans qu’il ait subi de chirurgie tandis que les autres sténoses font suite à une chirurgie réparatrice, d’où leur nom.</li><li>La maladie peut évoluer de manière asymptomatique et cliniquement silencieuse.</li><li>La sténose des veines pulmonaires, bien que rare, est une maladie très grave.</li><li>Une chirurgie pédiatrique ou des interventions par cathéter (angioplastie par ballonnet ou endoprothèses) peuvent être pratiquées dans le but d’atténuer le rétrécissement et d’améliorer la circulation sanguine dans les poumons.</li></ul><h2>Symptômes de la sténose des veines pulmonaires</h2><p>La maladie peut être cliniquement silencieuse. L’enfant peut ne présenter aucun symptôme jusqu’à ce que la maladie soit à un stade avancé.</p><p>Voici certains symptômes de la maladie :</p><ul><li>Un rythme respiratoire accéléré (tachypnée)</li><li>Une sensation de fatigue ou un manque d’énergie</li><li>Certains bébés ne présentent aucun gain pondéral en raison de l’effort exercé par le cœur pour pomper le sang à travers les poumons</li><li>Infections thoraciques ou <a href="/article?contentid=12&language=French">rhumes</a> fréquents</li><li>Saignements dans les poumons</li></ul><p>Aucun des symptômes susmentionnés n’est propre à la sténose des veines pulmonaires.</p><h2>Comprendre la sténose des veines pulmonaires</h2><p>La cause de cette maladie est inconnue. On ignore également pourquoi la maladie progresse chez certains enfants alors qu’elle est stable chez d’autres. Aucune cause génétique n’a été décelée.</p><p>Les cas les plus graves sont observés chez les enfants :</p><ul><li>dont un grand nombre de veines pulmonaires sont affectées</li><li>dont les deux poumons sont affectés</li><li>qui présentent d’autres malformations cardiaques </li><li>ayant fait l’objet d’un diagnostic de la maladie à un très jeune âge</li><li>chez qui la maladie est récurrente, ce qui signifie qu’en dépit des traitements, la maladie réapparait.</li></ul><p>Les enfants qui ont subi une chirurgie pour réparer des anomalies des veines pulmonaires courent un risque évalué à 10 % de développer la sténose des veines pulmonaires.</p><h2>Le diagnostic de la sténose des veines pulmonaires</h2><p>En raison de l’absence de symptômes cliniques au début de la maladie, le diagnostic est souvent posé lors de tests de routine.</p><p>Les enfants chez qui l’on soupçonne une maladie seront soumis à un <a href="/Article?contentid=1274&language=French">échocardiogramme</a> afin de constater la circulation du sang dans les veines pulmonaires et l’efficacité de la partie droite du cœur. </p> <p>Le pédiatre confirmera également le diagnostic à l’aide du <a href="/article?contentid=1272&language=French">tomodensitogramme</a>, de l’<a href="/article?contentid=1270&language=French">imagerie par résonance magnétique</a> ou du <a href="/article?contentid=1645&language=French">cathétérisme diagnostique</a>. Certains centres de soins de santé utilisent la <a href="/article?contentid=1301&language=French">technique d’exploration par perfusion du poumon</a> pour poser un diagnostic. Ces tests permettent au médecin d’établir le nombre de veines qui sont touchées et le degré de rétrécissement. Les tests sont importants car ils aident à préparer un plan de traitement de la sténose des veines pulmonaires.</p><h2>Le traitement de la la sténose des veines pulmonaires</h2><h3>Chirurgie</h3><p>Une chirurgie peut être pratiquée pour atténuer le rétrécissement (sténose) des veines pulmonaires situées près de l’oreillette du cœur. En libérant les régions bloquées, le chirurgien permettra au sang de s’écouler librement dans les veines. Votre enfant sera sous anesthésie générale pendant l’opération.</p><h3>Interventions au moyen d’un cathéter</h3><p>Dans la plupart des cas, lorsqu’une seule veine pulmonaire est affectée, on peut utiliser le cathéter à des fins de traitement. Ce qui comprend l’<a href="/article?contentid=1667&language=French">angioplastie par ballonnet</a> ou l’<a href="/article?contentid=60&language=French">insertion d’une endoprothèse</a>. Tant une méthode que l’autre permet d’élargir la région affectée par la sténose et de rétablir le passage du sang dans les veines.</p><p>La resténose est un des risques associés à ce type d’interven-tions. Ce terme fait référence à la possibilité que les veines pulmonaires se rétrécissent à l’avenir en dépit du traitement effectué.</p><p>La chirurgie peut se révéler nécessaire dans les cas où la veine pulmonaire est trop étroite pour y introduire un cathéter.</p><h3>Autres traitements possibles</h3><p>Une greffe pulmonaire peut être envisagée pour certains enfants qui souffrent d’une maladie progressive qui ne peut être traitée par la chirurgie ou une intervention par cathéter.</p><p>Il n’existe aucune thérapie médicale éprouvée pour le traitement de la sténose des veines pulmonaires, bien qu’il existe des thérapies expérimentales présentement à l’essai. Il se peut que l’on administre des médicaments spécifiques à votre enfant pour l’aider à gérer ses symptômes.</p><h2>Complications de la sténose</h2><p>Les enfants atteints de la maladie peuvent faire l’expérience de certaines complications, y compris :</p><ul><li>Des infections ou des rhumes récurrents pouvant nécessiter l’hospitalisation</li><li>Certains enfants peuvent expectorer du sang provenant des poumons (hémoptysie) en raison de la pression élevée présente dans les poumons.</li></ul><p>Même une fois le traitement terminé, il est possible d’observer une récidive. Quel que soit le traitement subi, il est important que votre enfant ait recours à des techniques d’imagerie impliquant les veines pulmonaires afin de surveiller la sténose. La sténose des veines pulmonaires qui évolue librement peut entraîner l’hypertension pulmonaire et l’insuffisance cardiaque droite.</p><h2>Quand consulter un médecin</h2><p>Nous vous recommandons de consulter un médecin dans les cas suivants :</p><ul><li>Votre enfant respire à un rythme accéléré</li><li>Sa croissance est lente et ne se compare pas à celle de ses pairs</li><li>Il est en proie de manière répétée à des infections thoraciques graves et il crache du sang.</li></ul><h2>À l’hôpital SickKids</h2><p>Si votre enfant doit subir une chirurgie à l’hôpital SickKids, nous vous invitons à prendre connaissance d’importantes informations qui figurent sur le site à l’adresse : Se présenter pour une chirurgie à l’hôpital SickKids.</p><p>Pour obtenir de plus amples informations sur la clinique de cardiologie à l’hôpital SickKids, consultez le site à l’adresse Cardiology Clinic (en anglais seulement).</p><h2>Ressources</h2> <ul><li>PVS Network: <a href="http://www.pvsnetwork.org/">http://www.pvsnetwork.org</a></li></ul><ul><li>M. Samanek, M. Voriskova. Congenital heart disease among 815,569 children born between 1980 and 1990 and their 15-year survival: a prospective Bohemia survival study Pediatric Cardiology, 20 (1999), pp. 411–417</li></ul><br>

 

 

Pulmonary vein stenosis2902.00000000000Pulmonary vein stenosisPulmonary vein stenosisPEnglishCardiologyChild (0-12 years)Lungs;HeartLungs;Cardiovascular systemConditions and diseasesAdult (19+) CaregiversFatigue2018-03-22T04:00:00ZRachel Vanderlaan, MD;Christopher Caldarone, MD, FRCSC8.3000000000000059.30000000000001300.00000000000Health (A-Z) - ConditionsHealth A-Z<p>Pulmonary vein stenosis (PVS) is when there is a narrowing or blockage in the pulmonary veins. It is a rare disease and if it progresses, it can lead to pulmonary hypertension and right heart failure.</p><p>Pulmonary vein stenosis (PVS) affects the pulmonary veins. The pulmonary veins bring blood from the lungs back to the left atrium of the heart. In PVS, the pulmonary veins become narrowed. In some cases, the narrowing can cause the pulmonary veins to become blocked. This is caused by an abnormal build-up of cells inside the wall of the pulmonary vein. It is not known what causes the cells to build-up. PVS is a rare but very serious disease.</p><p>If multiple pulmonary veins are affected, it can cause pulmonary hypertension. As a result, the right heart has to work harder to pump blood through the lungs. If the lung pressure remains high, it may result in right heart failure.</p> <figure class="asset-c-80"><span class="asset-image-title">Pulmonary veins</span><img src="https://assets.aboutkidshealth.ca/AKHAssets/pulmonary_veins_EN.jpg" alt="" /><figcaption class="asset-image-caption">Typically, there are four pulmonary veins, two from each lung. These veins drain oxygen-rich blood from the lungs into the left atrium of the heart. This blood is then moved out into the body, supplying it with the oxygen needed for proper health and function.</figcaption> </figure> <h2>What is PVS</h2><p>Narrowing or blockage can affect any of the pulmonary veins. The narrowing is not the same in all children. In some children, only a small part of the vein will be narrowed or blocked. Others may have long segments of the vein blocked or even the entire vein.</p><p>Some children may only have PVS in one pulmonary vein. Other children may have multiple veins affected, in either one or both lungs.</p> <figure class="asset-c-80"> <span class="asset-image-title">Two examples of pulmonary vein stenosis (PVS)</span><img src="https://assets.aboutkidshealth.ca/AKHAssets/pulmonary_vein_stenosis_EN.jpg" alt="" /><figcaption class="asset-image-caption">Pulmonary vein stenosis is a narrowing or, at times, a blockage of one or more of the four pulmonary veins. Narrowing or blockage can occur in a small part of one of the veins or in larger parts of one or more veins. This reduces the amount of blood that is able to drain from the lungs back into the heart.</figcaption> </figure> <p>There are two main categories of PVS:</p><ul><li>Primary, or congenital, PVS can occur in children who do not have a history of heart disease. </li><li>Post-repair PVS can develop after a child has surgery to repair anomalous (abnormally connected) pulmonary veins.<br></li></ul><h2>Key points</h2><ul><li>Each child is unique. The number of pulmonary veins affected and where the stenosis is located is not the same in every child.</li><li>There are two main categories: primary PVS and post-repair PVS. Primary PVS is when the child develops PVS without having any surgery on the pulmonary veins, while post-repair PVS develops after surgical repair of the pulmonary veins.</li><li>Progression of the disease can be clinically silent, meaning there are no symptoms.</li><li>PVS is a rare but very serious disease.</li><li>Children can have surgery or catheter-based interventions (balloon angioplasty or stents) to help relieve the narrowing and help blood flow better through the lungs. </li></ul> <h2>Signs and symptoms of PVS</h2><p>PVS can be clinically silent. This means a child might not have any symptoms of PVS until the disease is very advanced. </p><p>Some signs and symptoms of PVS may include:</p><ul><li>Fast breathing (tachypnea) </li><li>Fatigue or lack of energy</li><li>Some babies do not gain any weight, because their heart is working harder to pump blood through the lungs</li><li>Frequent <a href="/article?contentid=12&language=English">colds</a> or chest infections </li><li>Bleeding in the lungs</li></ul><p>None of the above signs or symptoms are specific to PVS. <br></p><h2>Understanding PVS</h2><p>The cause of PVS is not known. It is also not known why some children develop PVS that progresses, while others have PVS that remains more stable. No genetic cause for PVS has been identified. </p><p>Children who have more serious PVS are those who:</p><ul><li>have multiple pulmonary veins involved</li><li>have both lungs involved</li><li>have other heart defects </li> <li>are diagnosed at a very young age </li><li>have recurrent disease, meaning PVS continues to come back, despite treatment.</li></ul><p>Children who had a repair of anomalous pulmonary veins as an infant have around a 10% risk of developing pulmonary vein stenosis.</p><h2>Diagnosing PVS</h2><p>Because PVS can be clinically silent early on, many children will have a diagnosis of PVS made when they have tests for other reasons. </p><p>Children who are suspected of having PVS will have an <a href="/article?contentid=1274&language=English">echocardiogram</a> to look at the blood flow in the pulmonary veins and how well the right heart is pumping. </p><p>Your child’s doctor will also confirm the diagnosis with a <a href="/article?contentid=1272&language=English">CT scan</a>, <a href="/article?contentid=1270&language=English">MRI</a> or <a href="/article?contentid=1645&language=English">diagnostic catheterization</a>. Some health-care centres use <a href="/article?contentid=1301&language=English">lung perfusion scans</a> to help diagnose PVS. These tests let the doctors understand how many pulmonary veins are affected and how narrow the veins are. These tests are important to help plan the treatment of PVS.</p><h2>Treatment for PVS</h2><h3>Surgery</h3><p>Surgery can be done to relieve the narrowing (stenosis) in the pulmonary veins close to the atrium of the heart. The surgeon will relieve blocked areas of the pulmonary veins. This allows blood to flow unobstructed through the veins. Your child will be given a general anaesthesia to put them to sleep during the surgery.</p><h3>Catheter-based interventions</h3><p>In most cases, if only one pulmonary vein is affected, it may be treated with catheter-based interventions. These include <a href="/article?contentid=60&language=English">stent placement</a> and <a href="/article?contentid=1667&language=English">balloon angioplasty</a>. Both the stent and the balloon will open the area of stenosis and allow blood to flow through the veins.</p><p>One of the risks of catheter-based interventions is restenosis. This means that it is possible for narrowing of the pulmonary veins to redevelop in the future. </p><p>If the pulmonary vein is too narrowed to place a catheter through, surgery may be required. </p><h3>Other treatment options</h3> <p>A lung transplant may be an option for some children if they have progressive disease that cannot be treated by surgery or catheter-based interventions.</p><p>There are no proven medical therapies to treat PVS, but there are experimental therapies that are currently being tested. Your child may be given specific medications to help manage the symptoms of PVS.</p><h2>Complications of PVS</h2><p>Children with PVS may experience some complications including:</p><ul><li>Recurrent colds or infections that may require hospitalization</li><li>Some children may cough up blood from their lungs (hemoptysis) due to the high pressures in the lungs.</li></ul><p>Even after treatment, it is possible that PVS may recur (come back). It is important that your child undergo imaging tests of the pulmonary veins after any treatment to monitor stenosis. </p><p>Progressive PVS can lead to pulmonary hypertension and right heart failure.</p><h2>When to see a doctor</h2><p>You should see a doctor if: </p><ul><li>your child is breathing very fast</li><li>your child is not growing and keeping up with their peers</li> <li>your child develops repeated serious chest infections or coughs up blood. </li></ul><h2>At SickKids</h2><p>If your child is coming for surgery at SickKids, find important information here: <a href="http://www.sickkids.ca/visitingsickkids/coming-for-surgery/index.html">Coming for surgery</a></p><p>For more information on the Cardiology Clinic at SickKids: <a href="http://www.sickkids.ca/areas-of-care/clinics/cardiology-clinic.html">Cardiology Clinic</a></p> <h2>Resources</h2> <ul><li>PVS Network: <a href="http://www.pvsnetwork.org/">http://www.pvsnetwork.org</a></li></ul><h2>References</h2> <ul><li>M. Samanek, M. Voriskova. Congenital heart disease among 815,569 children born between 1980 and 1990 and their 15-year survival: a prospective Bohemia survival study Pediatric Cardiology, 20 (1999), pp. 411–417</li></ul>https://assets.aboutkidshealth.ca/AKHAssets/pulmonary_veins_EN.jpgPulmonary vein stenosisFalse

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