Zwężenie zastawki płucnej
Leczenie
Leczenie zwężenia zastawki płucnej jest dostosowane do stopnia zwężenia, anatomii zastawki oraz obecności objawów klinicznych. Zwężenie łagodne (gradient <50 mmHg) zwykle nie wymaga interwencji, natomiast umiarkowane (50-79 mmHg) i ciężkie (≥80 mmHg) wskazują na konieczność leczenia, przy czym ciężkie zwężenie wymaga szybkiej interwencji balonowej lub chirurgicznej. Krytyczne zwężenie u noworodków wymaga natychmiastowego podania prostaglandyn (PGE1) w celu utrzymania drożności przewodu tętniczego, a następnie poszerzenia zastawki. Balonowa walwuloplastyka (BPV) jest metodą z wyboru w izolowanym umiarkowanym i ciężkim zwężeniu, charakteryzującą się wysokim odsetkiem sukcesu (~90%) i niskim ryzykiem powikłań (śmiertelność 0,24%, poważne powikłania 0,35%). Leczenie chirurgiczne stosuje się w przypadku niepowodzenia BPV lub współistniejących wad serca, obejmując walwotomię, walwektomię oraz poszerzenie łatą, z dobrym rokowaniem i krótkim czasem hospitalizacji (3-4 dni).
Leczenie zwężenia zastawki płucnej
Leczenie zwężenia zastawki płucnej (stenoza zastawki płucnej) zależy od stopnia nasilenia zwężenia, anatomii zastawki oraz obecności objawów klinicznych. Metody terapeutyczne różnią się w zależności od wieku pacjenta i współistniejących wad serca.12
Postępowanie w różnych stopniach nasilenia zwężenia
Łagodne zwężenie zastawki płucnej (gradient szczytowy poniżej 50 mmHg) zazwyczaj nie wymaga interwencji. Pacjenci z łagodnym zwężeniem zastawki płucnej są zdrowi, mogą prowadzić normalny tryb życia i uprawiać wszystkie rodzaje aktywności i sportów. Wymagają jedynie regularnych kontroli kardiologicznych w celu monitorowania ewentualnej progresji wady.12
Umiarkowane zwężenie zastawki płucnej (gradient 50-79 mmHg) wymaga interwencji w celu zmniejszenia obturacji. Moment wykonania zabiegu zależy od wyników badania echokardiograficznego i objawów klinicznych.12
Ciężkie zwężenie zastawki płucnej (gradient ≥80 mmHg) zawsze wymaga leczenia. W przypadku objawów niewydolności prawej komory serca należy zastosować leczenie przeciwzastoinowe, w tym glikozydy nasercowe i diuretyki. Jednak problem nie ustąpi do czasu usunięcia przeszkody, dlatego należy szybko przeprowadzić interwencję balonową lub chirurgiczną.23
Krytyczne zwężenie zastawki płucnej u noworodków jest stanem zagrażającym życiu i wymaga natychmiastowej interwencji. Początkowo stosuje się prostaglandyny (PGE1) w celu utrzymania drożności przewodu tętniczego, co zapewnia przepływ krwi do płuc. Po stabilizacji stanu dziecka wykonuje się zabieg poszerzenia zastawki.12
Balonowa walwuloplastyka
Balonowa walwuloplastyka (BPV) jest obecnie metodą z wyboru w leczeniu izolowanego, umiarkowanego do ciężkiego zwężenia zastawki płucnej. Zabieg ten wykonuje się w pracowni hemodynamiki, co pozwala uniknąć operacji na otwartym sercu.12
Technika zabiegu polega na wprowadzeniu cewnika z balonem na końcu przez żyłę, najczęściej w pachwinie, przeprowadzeniu go do serca i umieszczeniu w obrębie zwężonej zastawki. Pod kontrolą obrazowania rentgenowskiego balon jest napełniany, co powoduje rozciągnięcie płatków zastawki, poszerzenie jej ujścia i poprawę przepływu krwi. Następnie balon jest opróżniany i wraz z cewnikiem usuwany.12
Wyniki balonowej walwuloplastyki w leczeniu zwężenia zastawki płucnej są doskonałe. U większości pacjentów zabieg ten zmniejsza stopień zwężenia z ciężkiego do łagodnego. U dzieci i młodzieży z „typowym” zwężeniem zastawki płucnej, pojedynczy zabieg balonowej walwuloplastyki jest zwykle jedyną wymaganą terapią. Rzadko zdarza się, że u starszego dziecka po skutecznym zabiegu balonowej walwuloplastyki ponownie rozwinie się znaczne zwężenie zastawki płucnej.12
Zabieg ten jest obarczony niskim ryzykiem śmiertelności (0,24%) i niewielkim odsetkiem poważnych powikłań (0,35%). Odsetek natychmiastowego powodzenia zabiegu wynosi blisko 90%.1
Leczenie chirurgiczne
Leczenie chirurgiczne jest wskazane w przypadkach, gdy balonowa walwuloplastyka nie jest możliwa lub nie przyniosła oczekiwanych efektów. Dotyczy to szczególnie pacjentów z dysplastyczną zastawką płucną, hipoplazją pierścienia zastawki, ciężką niedomykalnością zastawki płucnej, podastawkowym lub nadastawkowym zwężeniem tętnicy płucnej, lub gdy występują inne wady serca wymagające interwencji chirurgicznej.12
Dostępne są następujące metody chirurgicznego leczenia zwężenia zastawki płucnej:
- Walwotomia chirurgiczna – polega na nacięciu zrośniętych płatków zastawki podczas operacji na otwartym sercu. Zabieg wykonuje się poprzez nacięcie klatki piersiowej (sternotomię pośrodkową). Podczas operacji stosuje się krążenie pozaustrojowe, co umożliwia otwarcie serca i naprawę zastawki.12
- Walwektomia (z lub bez łaty przezpierścieniowej) – to chirurgiczne usunięcie zastawki. Często stosuje się łatę, która ułatwia przepływ krwi z prawej komory do tętnicy płucnej. W przyszłości, gdy dziecko dorośnie, może być konieczna wymiana zastawki płucnej.12
- Poszerzenie łatą – stosuje się łaty do poszerzenia zwężonych obszarów. Mogą być dodane do prawej komory lub tętnicy płucnej.12
Wyniki chirurgicznego leczenia są również doskonałe. Pobyt w szpitalu jest krótki (3-4 dni), a powikłania są rzadkie. Łagodne resztkowe zwężenie jest powszechne, ale nie powoduje długoterminowych problemów. Może wystąpić również niedomykalność zastawki, która jest dobrze tolerowana bez konieczności wymiany zastawki.1
Wymiana zastawki płucnej
Wymiana zastawki płucnej może być konieczna w przypadku znacznej niedomykalności zastawki płucnej po wcześniejszym leczeniu (walwuloplastyce lub chirurgicznej naprawie), gdy poprzednie metody leczenia zawiodły lub gdy obecne jest znaczne powiększenie prawej komory serca.12
Wymianę zastawki płucnej można przeprowadzić metodą tradycyjną (chirurgia na otwartym sercu) lub metodami małoinwazyjnymi. Dostępne są następujące rodzaje zastawek do wszczepienia:
- Zastawki biologiczne (tkanki zwierzęce, np. świńskie lub wołowe)
- Zastawki mechaniczne
- Homografty (ludzkie zastawki od dawców)
- Zastawki przezskórne (np. zastawka Melody)12
Przezskórna wymiana zastawki płucnej (TPVR) jest małoinwazyjną alternatywą dla operacji na otwartym sercu u odpowiednio dobranych pacjentów. Zastawka Melody jest przezskórnym systemem zastawkowym zaprojektowanym do leczenia zwężenia lub niedomykalności protez konduitów umieszczonych między prawą komorą a tętnicami płucnymi.12
Specjalne przypadki terapeutyczne
Leczenie noworodków z krytycznym zwężeniem
Noworodki z krytycznym zwężeniem zastawki płucnej wymagają szczególnego podejścia. Pacjenci ci mogą prezentować obraz prawie całkowitej atrezji płucnej (wada sinicza) z małą i często niewystarczającą prawą komorą. Przeżywają oni dzięki drożnemu przewodowi tętniczemu.1
Początkowe leczenie obejmuje stabilizację stanu pacjenta poprzez utrzymanie czasowej drożności przewodu tętniczego za pomocą infuzji prostaglandyny E1. Ostateczne podejście terapeutyczne będzie zależeć od ciężkości współistniejących wad. Wczesne otwarcie zastawki jest wskazane u pacjentów z najbardziej korzystnym rokowaniem w zakresie wystarczająco rozwiniętej prawej komory i zastawki trójdzielnej oraz braku zależnego od prawej komory krążenia wieńcowego.1
Leczenie płodów
Płodowa walwuloplastyka płucna jest rzadką techniką mającą zastosowanie u płodów z prenatalnym rozpoznaniem krytycznego zwężenia zastawki płucnej lub atrezji zastawki płucnej i ryzykiem progresji w kierunku hipoplazji prawej komory. Zabieg wykonuje się często między 21. a 28. tygodniem ciąży. Celem jest promowanie lepszego wewnątrzmacicznego rozwoju struktur prawego serca, co sprzyja fizjologii dwukomorowej po urodzeniu.12
Leczenie farmakologiczne
Leczenie farmakologiczne ma głównie charakter wspomagający i jest stosowane w następujących sytuacjach:
- U noworodków z krytycznym zwężeniem zastawki płucnej – prostaglandyny (PGE1) do utrzymania drożności przewodu tętniczego
- U pacjentów z objawami niewydolności prawej komory – leki przeciwzastoinowe, w tym glikozydy nasercowe i diuretyki
- Po zabiegu wymiany zastawki płucnej – antybiotykoterapia profilaktyczna przed określonymi zabiegami stomatologicznymi lub operacjami w celu zapobiegania infekcyjnemu zapaleniu wsierdzia
- Po wszczepieniu stentu – antybiotyki i ewentualnie leki przeciwzakrzepowe12
Długoterminowa opieka i obserwacja
Wszyscy pacjenci po leczeniu zwężenia zastawki płucnej wymagają regularnej, długoterminowej opieki kardiologicznej. Celem kontroli jest monitorowanie funkcji serca i zastawki płucnej oraz wczesne wykrywanie ewentualnych powikłań, takich jak nawrót zwężenia czy pojawienie się istotnej niedomykalności zastawki.12
U niektórych pacjentów może być konieczne powtórzenie zabiegu balonowej walwuloplastyki lub operacji w przyszłości. Nawrót istotnego zwężenia zastawki płucnej występuje u 5-10% dzieci w ciągu 10 lat od leczenia. W przypadku znacznej niedomykalności zastawki płucnej po leczeniu, może być zalecana wymiana zastawki w okresie dojrzewania lub wczesnej dorosłości, aby zapobiec powikłaniom, takim jak powiększenie prawej komory, niewydolność serca i zaburzenia rytmu serca.12
Rokowanie i jakość życia
Rokowanie dla pacjentów po leczeniu zwężenia zastawki płucnej jest zazwyczaj doskonałe. Długoterminowe przeżycie po chirurgicznej walwotomii wynosi 95% w ciągu 25 lat, co jest porównywalne z populacją ogólną.1
Większość pacjentów, którzy przeszli zabieg balonowej walwuloplastyki lub chirurgicznej naprawy w dzieciństwie, będzie prowadzić normalne życie. Zazwyczaj nie wymagają oni ciągłego leczenia antybiotykami, chociaż może być ono zalecane dla niektórych osób z dodatkowymi powikłaniami. Prawdopodobieństwo konieczności powtórzenia zabiegu jest bardzo niskie – 5% w ciągu 25 lat.1
Warto podkreślić, że chociaż leczenie znacznie poprawia funkcję zastawki, nie może ona zostać całkowicie przywrócona do stanu normalnego. Zastawka płucna zawsze pozostanie w pewnym stopniu nieprawidłowa, co uzasadnia konieczność długoterminowej opieki kardiologicznej.12
Kolejne rozdziały
Zapraszamy do dalszego czytania naszego leksykonu.
Wybierz kolejny rozdział z menu poniżej, aby otworzyć nową podstronę kompedium wiedzy i uzyskać szczegółowe informację o leku, substancji lub chorobie.
Materiały źródłowe
- #1 Pulmonary valve stenosis | Altru Health Systemhttps://www.altru.org/health-library/conditions/pulmonary-valve-stenosis
If you have moderate or severe pulmonary valve stenosis, you may need a heart procedure or heart surgery. The type of procedure or surgery done depends on your overall health and the appearance of your pulmonary valve. […] Pulmonary valve stenosis treatment may include: […] Balloon valvuloplasty. The provider inserts a flexible tube with a balloon on the tip into an artery, usually in the groin. X-rays help guide the tube, called a catheter, to the narrowed valve in the heart. The balloon inflates, making the valve opening larger. The balloon is deflated. The catheter and balloon are removed. Valvuloplasty may improve blood flow through the heart and reduce pulmonary valve stenosis symptoms. But the valve may narrow again. Some people need valve repair or replacement in the future. […] Pulmonary valve replacement. If balloon valvuloplasty isn’t an option, open-heart surgery or a catheter procedure may be done to replace the pulmonary valve. If there are other heart problems, the surgeon may repair those during the same surgery. People who have had pulmonary valve replacement need to take antibiotics before certain dental procedures or surgeries to prevent endocarditis.
- #1 Pulmonary (Valve) Stenosis | Symptoms, Diagnosis & Treatmenthttps://www.cincinnatichildrens.org/health/p/pvs
Children with mild pulmonary valve stenosis do not need treatment. Patients with mild pulmonary valve stenosis are healthy. They can do all types of activities and sports. They can lead normal lives. […] Children with moderate-to-severe degrees of pulmonary stenosis need treatment. The timing of which is often not urgent and driven by the echo findings and any symptoms that may be present in an older child. […] The type of treatment needed depends on the type of valve defect present. Often, the pulmonary valve is of normal size. The obstruction is due to the commissures or lines being stuck together. This „typical” form of pulmonary valve stenosis responds well to balloon dilation. Balloon dilation valvuloplasty (a tube catheter is placed inside the femoral artery and threaded up to the heart) is done at the time of cardiac catheterization. It does not need open-heart surgery. In older children, the procedure is done electively on an outpatient basis.
- #1 Valvar Pulmonary Stenosis Treatment & Management: Approach Considerations, Medical Care, Surgical Carehttps://emedicine.medscape.com/article/891729-treatment
The neonate with critical pulmonary valve stenosis requires special consideration. Patients with critical pulmonary stenosis may present with nearpulmonary atresia (cyanotic lesion) with a small and often inadequate right ventricle. These patients survive because of a patent ductus arteriosus (PDA). Although balloon pulmonary valvuloplasty produces good results, nearly 25% patients require reintervention to address related complications, restenosis, and associated defects. […] Patients with associated severe infundibular or supravalvar pulmonary stenosis require surgical intervention. […] Definitive repair may not be possible if the right ventricle is hypoplastic or if single ventricular palliation (eg, the Fontan procedure or a variation of this) is needed. The modified Fontan procedure currently used is staged cavopulmonary connection.
- #1 Pulmonary Stenosis | University of Michigan Healthhttps://www.uofmhealth.org/conditions-treatments/ped-heart/conditions/pulmonary-stenosis
Treatment options for pulmonary stenosis include open-heart surgery or balloon angioplasty. The primary indication for treatment is the degree of narrowing and treatment is timed to prevent damage to the right heart. […] Balloon angioplasty has proven to be an excellent treatment option for children with pulmonary stenosis. This procedure is done in the heart catheterization laboratory and avoids the need for surgery. During the procedure catheters (thin plastic tubes) are placed into the large blood vessels in the legs and gently guided to the heart. The catheter tip is placed across the pulmonary valve and the balloon tip is inflated. The balloon gently dilates the narrowed area. This procedure achieves a good result in most patients and no further treatment is ever needed. […] Surgical valvotomy is another treatment option for patients with severe pulmonary stenosis is direct incision of the fused leaflets of the valve during open-heart surgery. During this procedure an incision, called a midline sternotomy, is made down the middle of the chest. A heart lung machine is used to support the patient while the heart is opened and the pulmonary valve is repaired. Surgical repair is preferred when there is more extensive narrowing in addition to the pulmonary valve itself, often as a result of narrowing below and/or above the valve. In these situations, a patch may be needed to enlarge narrowed areas in addition to valve repair. The results from this procedure are also excellent. Hospital length of stay is short (3 or 4 days) and complications are rare. Mild residual narrowing is common but will not result in long-term problems. Valve leakage may also occur but is very well tolerated without the need for valve replacement.
- #1 Pulmonary (Valve) Stenosis | Symptoms, Diagnosis & Treatmenthttps://www.cincinnatichildrens.org/health/p/pvs
Open-heart surgical procedures are needed for more complex valves, when balloon dilation does not work. These valves may be blocked by thick and abnormal leaflet tissue. The width of the valve may be small in some cases. […] For these conditions, surgical pulmonary valvotomy (opening of the valve), partial valvectomy (removal of a portion of the leaflet), and a transannular patch (patch from the right ventricle to pulmonary artery) may be needed during the open-heart surgery repair. […] Results of balloon dilation valvuloplasty for pulmonary stenosis have been excellent. Balloon dilation cannot make an abnormal valve „normal.” The procedure decreases the degree of pulmonary valve obstruction from severe to mild in most patients. […] For children and teens with „typical” pulmonary valve stenosis, a single balloon dilation procedure is usually the only therapy needed. It is rare that an older child will have significant pulmonary valve obstruction again after a successful balloon dilation procedure.
- #1 Percutaneous treatment of pulmonary valve and arteries for the management of congenital heart diseasehttps://recintervcardiol.org/en/review-articles/percutaneous-treatment-of-pulmonary-valve-and-arteries-for-the-management-of-congenital-heart-disease
The rate of immediate procedural success is close to 90% with a very low mortality rate (0.24%) and scarce major complications (0.35%). […] In the absence of severe-to-mild PR, repeating the percutaneous valvuloplasty is the selection of choice except for the management of valve dysplasia where surgery can be indicated. […] The management of these patients includes early stabilization by keeping the temporal maintenance of ductal patency with prostaglandin E1 infusion. The ultimate therapeutic approach will depend on the severity of the associated lesions. The early opening of the valve is indicated in patients with the most favorable prognosis in terms of sufficiently developed RV and tricuspid valve and lack of RV-dependent coronary circulation. […] Fetal pulmonary valvuloplasty is a rare technique applicable to fetuses with prenatal diagnosis of critical pulmonary valve stenosis or PVA and risk of progression towards RV hypoplasia. It is often performed between the 21st and 28th weeks of pregnancy. The goal here is to promote a better intrauterine development of right heart structures, thus favoring biventricular physiology after birth. […] The current technological advances made in imaging techniques, materials, and devices has revolutionized the possibilities regarding the percutaneous management of pulmonary trunk, valve, and PB lesions. That is why the indications published in the clinical practice guidelines are being changed.
- #1 Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniq | VHRMhttps://www.dovepress.com/pulmonary-valve-stenosis-from-diagnosis-to-current-management-techniqu-peer-reviewed-fulltext-article-VHRM
According to the American Heart Association/American College of Cardiology (AHA/ACC) guidelines, in case of domed pulmonic valve with moderate or severe valvular stenosis and less than moderate pulmonic valve regurgitation, balloon valvotomy is recommended. […] Otherwise, surgical repair is recommended in case of symptomatic patients with moderate or severe valvular pulmonic stenosis who are ineligible for balloon valvotomy or who have failed it. This includes patients with severe PS and an associated hypoplastic pulmonary annulus, severe pulmonary regurgitation (PR), sub-valvular PS or supravalvular pulmonic stenosis. Surgery is also preferred for most dysplastic valves and when there is associated severe tricuspid regurgitation or other cardiopathy that warrants operative intervention.
- #1 Pulmonary Stenosis: Symptoms, Diagnosis & Treatment -Children’s Hospital of Orange Countyhttps://choc.org/heart/congenital-heart-defects/pulmonary-stenosis/
Specific treatment for pulmonary stenosis will be determined by your childâs doctor based on: […] Mild pulmonary stenosis often does not require treatment. Moderate or severe stenosis is treated with repair of the obstructed valve. Several options are currently available. […] Repair options include the following: […] Balloon dilation or valvuloplasty. An interventional cardiac catheterization procedure is the most common treatment choice. […] Valvotomy. Valvotomy is the surgical release of scar tissue within the aortic valve leaflets that are preventing the valve leaflets from opening properly. […] Valvectomy (with or without transannular patch). Valvectomy is the surgical removal of the valve and is frequently accompanied by the placement of an outflow patch to improve blood flow from the right ventricle into the pulmonary artery.
- #1 Pulmonary Stenosis: Symptoms, Diagnosis & Treatment -Children’s Hospital of Orange Countyhttps://choc.org/heart/congenital-heart-defects/pulmonary-stenosis/
Patch enlargement. Patches are used to enlarge the narrowed areas. […] Pulmonary valve replacement. Replacement of the pulmonary valve is a surgical procedure that is often recommended in adulthood in the case of leaky pulmonary valves. […] Your childâs cardiologist may recommend that antibiotics be given to prevent bacterial endocarditis for a period of time following surgery and always for all valve replacements. […] Regular follow-up care at a center offering pediatric or adult congenital cardiac care should continue throughout the individualâs lifespan.
- #1 Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniq | VHRMhttps://www.dovepress.com/pulmonary-valve-stenosis-from-diagnosis-to-current-management-techniqu-peer-reviewed-fulltext-article-VHRM
Pulmonary valve replacement is also the main reintervention in patients with PS who have previously undergone valvotomy with subsequent PR and/or residual PS. […] There are several options for pulmonary valve replacement, including: Homograft conduits; Bovine jugular vein grafts; Mechanical prosthetic valves; Biological prosthetic valves. […] The Melody transcatheter pulmonary valve is a percutaneous valve system designed to treat obstruction or regurgitation of prosthetic conduits positioned between the RV and PAs. […] The main indications for Melody Valve use are the following: Age 5 years or weight 30 kg; Original conduit diameter 16 cm; Echocardiographic signs of RVOT conduit dysfunction; Patients in NYHA class I; Doppler mean gradient 35 mmHg or moderate PR; Patients in NYHA class I with Doppler mean gradient 40 mmHg or severe PR associated with TV annulus Z-score 2 RVEF 40%. […] If the Melody Valve was originally used only for stenotic RVOT conduits, the introduction of larger Edwards SAPIEN valves allowed tPVR to be performed also in native and wider RVOTs. […] The short outcomes show excellent results with all types of RVOT.
- #1 Pulmonary Valve Stenosis Treatment at Emory Heart & Vascularhttps://www.emoryhealthcare.org/services/heart-vascular/treatments/pulmonary-valve-stenosis
Restoring normal blood flow to your lungs is critical. The cardiovascular experts at Emory Heart Vascular offer a full range of medical and surgical therapies. They will work with you to identify the best treatment for you. […] Balloon valvuloplasty: This treatment uses a tiny balloon attached to a thin tube called a catheter. Our specialist places the balloon into your narrowed valve. They inflate it to widen the valve and improve blood flow. […] Pulmonary valve repair and replacement: Our specialists surgically repair or replace your pulmonary valve. […] Transcatheter pulmonary valve replacement (TPVR): This minimally invasive procedure is not open surgery. Our specialists can replace the pulmonary valve without open heart surgery in certain patients.
- #1 Valvar Pulmonary Stenosis Treatment & Management: Approach Considerations, Medical Care, Surgical Carehttps://emedicine.medscape.com/article/891729-treatment
Patients with trivial (gradient 25 mm Hg) or mild (gradient 50 mm Hg) pulmonary stenosis do not need intervention to relieve the obstruction of the pulmonary valve. […] Patients with moderate (gradient, 50-79 mm Hg) and severe (gradient, 80 mm Hg) obstruction should undergo intervention to relieve the stenosis of the pulmonary valve. […] Patients with signs of right ventricular failure should be promptly treated with anticongestive measures, including digitalis and diuretics. However, the problem does not resolve until the obstruction is relieved. Therefore, prompt balloon or surgical intervention should be undertaken. […] A fetus with critical pulmonary stenosis or atresia with intact ventricular septum may benefit from pulmonary balloon valvuloplasty in utero, which promotes growth of the right ventricle.
- #1 Pulmonary (Valve) Stenosis | Symptoms, Diagnosis & Treatmenthttps://www.cincinnatichildrens.org/health/p/pvs
Newborns and young infants with severe pulmonary valvar obstruction have an excellent response to balloon dilation. Outcomes can be less robust (that is, significant valve stenosis can remain) if the valve is underdeveloped in size. Recurrence of significant pulmonary stenosis does occur in 5-10% of children within 10 years of treatment. Occasionally, these patients may need a second balloon dilation, or an open-heart surgical procedure if the valve is small. […] Because balloon dilation is the only treatment necessary for most patients, open-heart surgery is only done in patients with more complex forms of pulmonary valve obstruction. Patients with very thick valve leaflets or underdeveloped pulmonary valve sizes also have great long-term outcomes after open-heart surgical repair. If there is no associated heart disease, these children are expected to lead normal, active lives. […] All children with pulmonary valve stenosis need to be seen at regular intervals. Long-term follow-up with evaluation by a cardiologist is important to help give the highest quality outcome for patients with pulmonary valve stenosis.
- #1 Pulmonary Stenosis | Conditions | UCSF Healthhttps://www.ucsfhealth.org/conditions/pulmonary-stenosis
Children and adults whose pulmonary valves are still flexible may be treated with a balloon valvuloplasty, a non-surgical procedure in which a catheter a thin, flexible, plastic tube is inserted into the heart via blood vessels from the leg. A balloon at the tip of the catheter is inserted into the narrow opening in the valve and then inflated to stretch the valve opening and separate the valve leaflets. This is usually very successful and permanent. If, however, the valve is unusually thick, then the balloon procedure is unlikely to be successful and the surgeon will have to open up the valve with a scalpel. […] In adults with severe stenosis, the valve is often stiff and calcified, and treatment is more likely to include surgical replacement with either a manufactured valve or a valve from an animal, usually a pig. Another surgical repair involved removing obstructive tissue below the valve, which may be done in conjunction with valve replacement. Studies indicate that surgical valvotomy has a 25-year survival rate of 95 percent, or about the same as the normal population.
- #1 Pulmonary Stenosis | Conditions | UCSF Healthhttps://www.ucsfhealth.org/conditions/pulmonary-stenosis
Most patients who have been treated surgically or with balloon valvuloplasty in childhood will have normal lives. They usually do not require continuous antibiotic treatment but it may be recommended for some people who have additional complications. The likelihood of requiring a repeat procedure is very low 5 percent at 25 years. However, it is recommended that all patients be evaluated by a cardiologist specializing in adult congenital heart disease at least once. At that time, an echocardiogram should be performed. Thereafter, yearly physical examinations are recommended with repeat echocardiogram should there be a change in the physical examination or new symptoms.
- #1 Pulmonary Valve Stenosis  | American Heart Associationhttps://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-defects/pulmonary-valve-stenosis
The pulmonary valve can be treated to improve the obstruction and leak, but the valve can’t be made normal. […] Treatment is needed when the pressure in the right ventricle is high (even though there may be no symptoms). In most children the obstruction can be relieved during cardiac catheterization by balloon valvuloplasty. In this procedure, a special tool, a catheter containing a balloon, is placed across the pulmonary valve. The balloon is inflated for a short time to stretch open the valve. Some children may need surgery. […] The valve can be treated to improve the obstruction, but the valve can’t be made normal. […] Treatment is needed when the pressure in the right ventricle is high (even though there may be no symptoms). In most patients the obstruction can be relieved during cardiac catheterization (referred to as interventional or therapeutic catheterization) by balloon valvuloplasty. In this procedure, a special catheter containing a balloon is placed across the pulmonary valve. The balloon is inflated for a short time to stretch open the valve.
- #2 Pulmonary (Valve) Stenosis | Symptoms, Diagnosis & Treatmenthttps://www.cincinnatichildrens.org/health/p/pvs
Children with mild pulmonary valve stenosis do not need treatment. Patients with mild pulmonary valve stenosis are healthy. They can do all types of activities and sports. They can lead normal lives. […] Children with moderate-to-severe degrees of pulmonary stenosis need treatment. The timing of which is often not urgent and driven by the echo findings and any symptoms that may be present in an older child. […] The type of treatment needed depends on the type of valve defect present. Often, the pulmonary valve is of normal size. The obstruction is due to the commissures or lines being stuck together. This „typical” form of pulmonary valve stenosis responds well to balloon dilation. Balloon dilation valvuloplasty (a tube catheter is placed inside the femoral artery and threaded up to the heart) is done at the time of cardiac catheterization. It does not need open-heart surgery. In older children, the procedure is done electively on an outpatient basis.
- #2 Valvar Pulmonary Stenosis Treatment & Management: Approach Considerations, Medical Care, Surgical Carehttps://emedicine.medscape.com/article/891729-treatment
Patients with trivial (gradient 25 mm Hg) or mild (gradient 50 mm Hg) pulmonary stenosis do not need intervention to relieve the obstruction of the pulmonary valve. […] Patients with moderate (gradient, 50-79 mm Hg) and severe (gradient, 80 mm Hg) obstruction should undergo intervention to relieve the stenosis of the pulmonary valve. […] Patients with signs of right ventricular failure should be promptly treated with anticongestive measures, including digitalis and diuretics. However, the problem does not resolve until the obstruction is relieved. Therefore, prompt balloon or surgical intervention should be undertaken. […] A fetus with critical pulmonary stenosis or atresia with intact ventricular septum may benefit from pulmonary balloon valvuloplasty in utero, which promotes growth of the right ventricle.
- #2 Percutaneous treatment of pulmonary valve and arteries for the management of congenital heart diseasehttps://recintervcardiol.org/en/review-articles/percutaneous-treatment-of-pulmonary-valve-and-arteries-for-the-management-of-congenital-heart-disease
The rate of immediate procedural success is close to 90% with a very low mortality rate (0.24%) and scarce major complications (0.35%). […] In the absence of severe-to-mild PR, repeating the percutaneous valvuloplasty is the selection of choice except for the management of valve dysplasia where surgery can be indicated. […] The management of these patients includes early stabilization by keeping the temporal maintenance of ductal patency with prostaglandin E1 infusion. The ultimate therapeutic approach will depend on the severity of the associated lesions. The early opening of the valve is indicated in patients with the most favorable prognosis in terms of sufficiently developed RV and tricuspid valve and lack of RV-dependent coronary circulation. […] Fetal pulmonary valvuloplasty is a rare technique applicable to fetuses with prenatal diagnosis of critical pulmonary valve stenosis or PVA and risk of progression towards RV hypoplasia. It is often performed between the 21st and 28th weeks of pregnancy. The goal here is to promote a better intrauterine development of right heart structures, thus favoring biventricular physiology after birth. […] The current technological advances made in imaging techniques, materials, and devices has revolutionized the possibilities regarding the percutaneous management of pulmonary trunk, valve, and PB lesions. That is why the indications published in the clinical practice guidelines are being changed.
- #2 Pulmonary Stenosis | University of Michigan Healthhttps://www.uofmhealth.org/conditions-treatments/ped-heart/conditions/pulmonary-stenosis
Treatment options for pulmonary stenosis include open-heart surgery or balloon angioplasty. The primary indication for treatment is the degree of narrowing and treatment is timed to prevent damage to the right heart. […] Balloon angioplasty has proven to be an excellent treatment option for children with pulmonary stenosis. This procedure is done in the heart catheterization laboratory and avoids the need for surgery. During the procedure catheters (thin plastic tubes) are placed into the large blood vessels in the legs and gently guided to the heart. The catheter tip is placed across the pulmonary valve and the balloon tip is inflated. The balloon gently dilates the narrowed area. This procedure achieves a good result in most patients and no further treatment is ever needed. […] Surgical valvotomy is another treatment option for patients with severe pulmonary stenosis is direct incision of the fused leaflets of the valve during open-heart surgery. During this procedure an incision, called a midline sternotomy, is made down the middle of the chest. A heart lung machine is used to support the patient while the heart is opened and the pulmonary valve is repaired. Surgical repair is preferred when there is more extensive narrowing in addition to the pulmonary valve itself, often as a result of narrowing below and/or above the valve. In these situations, a patch may be needed to enlarge narrowed areas in addition to valve repair. The results from this procedure are also excellent. Hospital length of stay is short (3 or 4 days) and complications are rare. Mild residual narrowing is common but will not result in long-term problems. Valve leakage may also occur but is very well tolerated without the need for valve replacement.
- #2 Valvar Pulmonary Stenosis Treatment & Management: Approach Considerations, Medical Care, Surgical Carehttps://emedicine.medscape.com/article/891729-treatment
The general consensus based on current data is that balloon valvuloplasty is the treatment of choice for managing isolated pulmonary valve stenosis. […] In general, indications for balloon pulmonary valvuloplasty are similar to those used in surgical pulmonary valvotomy (ie, moderate pulmonary valve stenosis with a peak-to-peak gradient 50 mm Hg with a normal cardiac index). […] The technique of balloon pulmonary valvuloplasty involves positioning a balloon catheter across the stenotic valve, usually over an extra-stiff exchange-length guide wire and inflating the balloon with diluted contrast material to accomplish valvotomy. […] The results of balloon pulmonary valvuloplasty for patients with dysplastic pulmonary valves are generally poor with the use of conventional balloon pulmonary valvuloplasty techniques.
- #2 Interventional Treatment of Pulmonary Valve Stenosis: A Single Center Experiencehttps://pmc.ncbi.nlm.nih.gov/articles/PMC4877828/
Percutaneous pulmonary valvuloplasty is well established treatment of choice in pulmonary valve stenosis. […] Percutaneous pulmonary valvuloplasty is an effective procedure in treatment of pulmonary stenosis with good short and mid-term results. […] Up until 1982 this condition was treated surgically, the same year the technique of percutaneous balloon pulmonary valvuloplasty(BVP) was introduced by Kan et al. and since then it has become the established method of treatment of moderate and severe pulmonary stenosis in most cardiac centers in developed countries. […] Balloon valvuloplasty is the treatment of choice in the management of moderate to severe pulmonary stenosis with excellent short- and mid-term results.
- #2 Pulmonary (Valve) Stenosis | Symptoms, Diagnosis & Treatmenthttps://www.cincinnatichildrens.org/health/p/pvs
Open-heart surgical procedures are needed for more complex valves, when balloon dilation does not work. These valves may be blocked by thick and abnormal leaflet tissue. The width of the valve may be small in some cases. […] For these conditions, surgical pulmonary valvotomy (opening of the valve), partial valvectomy (removal of a portion of the leaflet), and a transannular patch (patch from the right ventricle to pulmonary artery) may be needed during the open-heart surgery repair. […] Results of balloon dilation valvuloplasty for pulmonary stenosis have been excellent. Balloon dilation cannot make an abnormal valve „normal.” The procedure decreases the degree of pulmonary valve obstruction from severe to mild in most patients. […] For children and teens with „typical” pulmonary valve stenosis, a single balloon dilation procedure is usually the only therapy needed. It is rare that an older child will have significant pulmonary valve obstruction again after a successful balloon dilation procedure.
- #2 Treatment for Pulmonary Stenosis – Leeds Teaching Hospitals NHS Trusthttps://www.leedsth.nhs.uk/patients/resources/treatment-for-pulmonary-stenosis/
This leaflet aims to give you an overview of a condition called pulmonary stenosis and itâs treatment using a keyhole approach. […] If the valve is severely narrowed, treatment may be needed; which is usually keyhole treatment rather than open heart surgery. Most cases can be treated by stretching the narrow valve open with a balloon (called âballoon valvuloplastyâ). This is done under general anaesthetic and involves passing a long tube with a collapsed balloon on the end of it (a balloon catheter) into the vein at the top of the leg and feeding it into the heart across the narrow valve. […] Sometimes the pulmonary valve cannot be stretched open using a balloon and open heart surgery will be needed. This involves opening the chest (usually in the middle at the front) and the function of the heart and lungs being taken over by a machine so the surgeon can open the heart and cut the narrow valve open.
- #2 Pulmonary Stenosis in Children – Stanford Medicine Children’s Healthhttps://www.stanfordchildrens.org/en/topic/default?id=pulmonary-stenosis-in-children-90-P01815
Valvotomy. This is surgery to remove scar tissue from the pulmonary valve leaflets. This lets the valve open as it should. […] Valvectomy. This is surgery to remove the valve. Often a patch is used to help the blood flow from the right ventricle into the pulmonary artery. The pulmonary valve may need to be replaced when the child is an adult. […] Patch enlargement. Patches are used to enlarge narrowed areas. They may be added to the right ventricle or the pulmonary artery. […] Pulmonary valve replacement. Some children may need to have the pulmonary valve replaced. A tissue valve (pig or human) may be used. Children who have had valve replacement will need to take antibiotics before medical and dental procedures in the future.
- #2 Pulmonary Stenosis in Children | Valley Children’s Healthcarehttps://www.valleychildrens.org/services/heart/conditions-we-treat/pulmonary-stenosis
Valvotomy. This is surgery to remove scar tissue from the pulmonary valve leaflets. This lets the valve open as it should. […] Valvectomy. This is surgery to remove the valve. Often a patch is used to help the blood flow from the right ventricle into the pulmonary artery. The pulmonary valve may need to be replaced when the child is an adult. […] Patch enlargement. Patches are used to enlarge narrowed areas. They may be added to the right ventricle or the pulmonary artery. […] Pulmonary valve replacement. Some children may need to have the pulmonary valve replaced. A tissue valve (pig or human) may be used. Children who have had valve replacement will need to take antibiotics before medical and dental procedures in the future.
- #2 Pulmonary Stenosis | Schneeweiss Adult Congenital Heart Centerhttps://www.congenitalheart.cuimc.columbia.edu/conditions-we-treat/pulmonary-stenosis
Management of valvular pulmonary stenosis is determined by the degree of obstruction, which ranges from mild to severe. Patients with mild pulmonary stenosis typically have no cardiac symptoms and do not require intervention on the valve. Patients with moderate or more valvular pulmonary stenosis can generally be treated by balloon valvuloplasty, which replaced surgical valvotomy during the 1980s. If the valve leaflets cannot be opened with a balloon, then surgical repair is usually performed. This might be a valvotomy, in which the leaflets are incised to relieve the obstruction, or a pulmonary valve replacement. […] In the patients with marked right ventricular enlargement, pulmonary valve replacement is often advised to improve symptoms and decrease the size of the right ventricle. […] In patients who develop severe obstruction within the body of the right ventricle, an entity known as double chambered right ventricle, surgical resection of the excess muscle bundles can be performed with effective relief of obstruction. In patients with obstruction within the pulmonary artery, careful study of the location, severity, and symptoms helps determine whether surgical or catheter-based procedures are indicated.
- #2 Pulmonary Stenosis (for Parents) | Nemours KidsHealthhttps://kidshealth.org/en/parents/pulmonary-stenosis.html
Valve replacement involves using an artificial valve or a valve from a donor. […] A challenge for some kids with pulmonary stenosis is that it can come back again after treatment. This can happen for different reasons, including scar tissue that forms after a procedure or a valve replacement that doesnât grow as kids get bigger. So some kids might need several procedures to keep the valve healthy. […] Because pulmonary stenosis can be a lifelong condition, kids who have the defect will need to see a cardiologist (a doctor who treats heart problems) regularly to make sure the narrowing isnât getting worse.
- #2 Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniq | VHRMhttps://www.dovepress.com/pulmonary-valve-stenosis-from-diagnosis-to-current-management-techniqu-peer-reviewed-fulltext-article-VHRM
Pulmonary valve replacement is also the main reintervention in patients with PS who have previously undergone valvotomy with subsequent PR and/or residual PS. […] There are several options for pulmonary valve replacement, including: Homograft conduits; Bovine jugular vein grafts; Mechanical prosthetic valves; Biological prosthetic valves. […] The Melody transcatheter pulmonary valve is a percutaneous valve system designed to treat obstruction or regurgitation of prosthetic conduits positioned between the RV and PAs. […] The main indications for Melody Valve use are the following: Age 5 years or weight 30 kg; Original conduit diameter 16 cm; Echocardiographic signs of RVOT conduit dysfunction; Patients in NYHA class I; Doppler mean gradient 35 mmHg or moderate PR; Patients in NYHA class I with Doppler mean gradient 40 mmHg or severe PR associated with TV annulus Z-score 2 RVEF 40%. […] If the Melody Valve was originally used only for stenotic RVOT conduits, the introduction of larger Edwards SAPIEN valves allowed tPVR to be performed also in native and wider RVOTs. […] The short outcomes show excellent results with all types of RVOT.
- #2 Pulmonary Valve Stenosis: Causes, Symptoms, and Diagnosishttps://www.healthline.com/health/pulmonary-valve-stenosis
Pulmonary valve stenosis does not always require medical treatment. However, some people may need medications or surgeries to correct the condition. […] Your doctor will use imaging scans and other examinations to determine the best course of treatment for pulmonary valve stenosis. They might not recommend intervention if the stenosis is mild and not causing symptoms. […] 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. […] A doctor may prescribe medications that make it easier for blood to flow through the hearts chambers. Examples of these medications include: prostaglandins to improve blood flow, blood thinners to reduce clotting, water pills to reduce excess fluid in the blood stream, pills that prevent irregular heart rhythms.
- #2 Pulmonary Valve Stenosis  | American Heart Associationhttps://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-defects/pulmonary-valve-stenosis
In some cases the valve cannot be treated by balloon valvuloplasty. Then surgery is needed to relieve/correct the narrowing. […] The long-term outlook after balloon valvuloplasty or surgery is excellent, and usually no medicines are needed. Your cardiologist will examine you regularly to look for problems such as worsening of the obstruction or leakage of the pulmonary valve.
- #2 Pulmonary Stenosis – Conditions and Treatments | Children’s National Hospitalhttps://www.childrensnational.org/get-care/health-library/pulmonary-stenosis
The outlook for children with pulmonary stenosis is usually excellent. For a period of time, your child’s cardiologist may advise that your child take antibiotics to prevent infection of the heart lining and valves (bacterial endocarditis). Your child may need to take them before medical and dental procedures. […] In some cases, a child may need to have procedures done again over time to stretch the valve open. Your child may need a pulmonary valve replacement as a teen or young adult to prevent complications. […] Your child’s cardiologist may recommend that antibiotics be given to prevent bacterial endocarditis for a period of time following surgery and always for all valve replacements. […] Occasionally, repeat interventional cath lab procedures may be necessary during infancy and childhood to stretch the valve open. Replacement of the pulmonary valve may be recommended later during adolescence or early adulthood to prevent complications such as enlargement of the right ventricle, heart failure and arrhythmias (irregular or fast heartbeats).
- #2 When Your Child Has Pulmonary Stenosis (PS) | Saint Luke’s Health Systemhttps://www.saintlukeskc.org/health-library/when-your-child-has-pulmonary-stenosis-ps
Mild or moderate PS usually needs no treatment. Your child should have regular visits with a cardiologist. This is to make sure that narrowing of the valve doesnt get worse over time. […] Severe or critical PS needs treatment. Your child may be given a medicine for a time to keep the ductus arteriosus open. This lets blood flow to the pulmonary artery and lungs from the aorta, bypassing the blocked area. The main treatment for PS is a procedure called balloon valvuloplasty. This procedure is described below. In some cases, open heart surgery to repair or replace the valve is also a choice. The cardiologist will tell you more about heart surgery if its needed. Surgery is the treatment of choice for subvalvar and supravalvar PS. […] All treatment choices for PS are done to relieve symptoms. This means that the pulmonary valve is not repaired. It will always be somewhat abnormal. Your child may have problems with the valve again in the future.
- #3 Problem: Pulmonary Valve Stenosis | American Heart Associationhttps://www.heart.org/en/health-topics/heart-valve-problems-and-disease/heart-valve-problems-and-causes/problem-pulmonary-valve-stenosis
Treatment is needed when the pressure in the right ventricle is high (whether or not there are symptoms). High pressure in the ventricle can lead to enlargement of the heart and heart failure. […] However, depending on the severity of the stenosis, the valve may be repaired or replaced by surgery, or by a minimally invasive procedure.