Pulmonic stenosis is a defect of the pulmonic valve in which the valve is stiffened, causing an obstruction to flow. This disease is typically congenital, benign, and diagnosed in pediatric patients with potentially curative treatments. Pulmonic stenosis is also present in adults, usually in conjunction with severe cardiac structural diseases.
Pulmonic stenosis is commonly associated with congenital structural cardiac syndromes, including tetralogy of Fallot and Noonan syndrome. Maternal rubella syndrome is also a common cause of congenital pulmonic stenosis, albeit it is not a genetic-based defect. The onset of symptomatic pulmonic stenosis in adults may be seen in pregnancy of patients with undiagnosed pulmonic stenosis, or patients suffering from an underlying carcinoid syndrome. Furthermore, patients with rheumatic heart disease, previous cardiothoracic surgeries, or a cardiac tumor may acquire pulmonic stenosis.
Isolated pulmonic stenosis is divided in to valvular, subvalvular, and supravalvular obstruction.
Valvular pulmonic stenosis is the most common type. In typical valvular disease, the commissures are partially fused, and leaflets are thin. This structural anomaly results in a dome-shaped or conical outlet seen during systole. Less commonly, the pulmonic valves can be dysplastic, thickened, and without fusion. The associated hypoplastic annulus and proximal pulmonary artery are common in atypical presentations and are also associated with Noonan syndrome. The atypical presentation generally requires intervention in the early years of life. Bicuspid valves are associated with tetralogy of Fallot, while other variations in leaflet quantities, such as quadricuspid valves, have also been reported.
Subvavular pulmonic stenosis is a defect obstructing the infundibular region. A primary cause of fibromuscular narrowing of the right ventricular outflow tract may be associated with a double-chambered right ventricle or tetralogy of Fallot. Secondary causes of subvalvular pulmonic stenosis may be a result of primary valvular stenosis, inducing hypertrophy of the right ventricle. Secondary stenosis often regresses with valvotomy or valvuloplasty.
Supravalvular pulmonic stenosis, also known as peripheral pulmonary stenosis, is a functional obstruction originating from the pulmonary artery. The blockage may occur at the main artery, bifurcation point, at distal branches of the pulmonary artery, or any combination thereof. Supravalvular stenosis is associated with structural defects of atrial septal defects, ventricular septal defects, patent ductus arteriosus, and tetralogy of Fallot. Supravalvular stenosis may also be a result of surgical repair of transposition of the great arteries.
Most patients with pulmonic stenosis are asymptomatic, which may lead to a missed diagnosis until an insult occurs. Symptomatic patients typically experience dyspnea on exertion or associated fatigue, depending on the severity of obstruction and cardiac compensatory reserve. Rarely, patients can experience angina or sudden cardiac arrest. In patients with an associated septal defect, the patient may present with signs of cyanosis.
A physical exam may reveal multiple signs of pulmonic stenosis, depending on severity and practitioner skill. Cardiac examination may reveal a left parasternal heave, secondary to right ventricular hypertrophy. Auscultation at the left upper sternal border may reveal a systolic ejection murmur radiating to the back. Furthermore, there may be an associated ejection click and a split S2, rising from a delay in pulmonary closing. Finally, pulmonic auscultation may be associated with a 4th heart sound and tricuspid regurgitation.
Evaluation of pulmonic stenosis is best completed using echocardiography, due to its superior visualization of the pulmonic valve and surrounding structures. A transthoracic echocardiogram is sufficient in most cases. Transesophageal echocardiography should be reserved for suboptimal views or when particularly assessing for endocarditis.
Doppler studies using echocardiography provides flow gradients, which is used to grade severity. Guidelines from the European Association of Echocardiography, the American Society of Echocardiography, the American Heart Association, and American College of Cardiology (AHA/ACC) and the European Society of Cardiology have been summarized below.
Pulmonic stenosis may be interpreted through electrocardiography, despite not being diagnostic. Criteria supporting right ventricular hypertrophy correlates well with the severity of the disease. Right axis deviation may also be seen in mild disease, while prominent R waves in V1 and R waves in AVR are more likely seen in severe cases.
Cardiac catheterization and pulmonary angiography are typically not required for the diagnosis of pulmonic stenosis due to echocardiography's efficacy and safety profile. Cardiac magnetic resonance imaging (CMR) is a viable alternative to echocardiography. In the setting of suboptimal echocardiographic viewing or complicated anatomy, cardiac magnetic resonance imaging may assist in proper diagnosis. CMR also provides a measurement of the right ventricular volume, right ventricular function, and pulmonary artery blood flow. Cardiac computed tomography (CCT) may be substituted for patients who cannot undergo CMR.
The sensitivity of plain film x-rays is not sufficient to diagnose pulmonic stenosis. Radiographic evidence of pulmonic stenosis is supported by the prominence of the pulmonary arteries or a prominent right heart border.
The American Heart Association and American College of Cardiology have recommended the following management plans in their 2008 guidelines:
Surgical intervention is recommended for severe valvular stenosis with severe pulmonary regurgitation, hypoplastic pulmonary annulus, subvalvular stenosis, or supravalvular stenosis. Unfortunately, balloon valvotomy is not as effective in dysplastic valves as in domed valves, making surgery the preferred option. Balloon valvotomy may be worthwhile if peak doppler gradient is >60 mm Hg in asymptomatic patients, or if peak doppler gradient is >50 mm Hg in symptomatic patients. Surgery should also be considered in patients undergoing concurrent cardiac surgical procedures. Pulmonary artery balloon angioplasty, with optional stent placement, is an acceptable treatment for supravalvular and subvalvular pulmonic stenosis.
Congenital structural cardiac defects include complex congenital heart disease associated with findings of pulmonary stenosis, double-chambered right ventricle, syndrome of absent pulmonary valves, and tetralogy of Fallot. Adult-onset symptoms, excluding undiagnosed congenital causes, include rheumatic valvular heart disease and carcinoid heart disease. Acute onset of symptoms should include myocardial infarction, pulmonary embolism, right heart failure, cardiac tumor, cardiac sarcoma, sinus of Valsalva aneurysm.
The prognosis for most patients is excellent. Except for critical stenosis during the neonatal period, most patients will live a typical life. For patients undergoing surgical revision, 67% of patients achieve 90% of maximal exercise capacity, with only 15-20% needing reintervention. 67% of reinterventions are for significant pulmonary regurgitation, and, if present, most supraventricular tachycardias (SVTs) resolved with reintervention.
For patients undergoing balloon valvotomy, a better prognosis was seen with dome-shaped valves over dysplastic valves. After the procedure, at years 1, 3, and 6, the reintervention rate was 0%, 3.5%, and 3.5%, respectively. Furthermore, 52% of adults were free of any symptoms. Complications are generally minor but could include a vagal response, catheter induced ventricular ectopy, right bundle branch block, and transient or permanent high-grade AV nodal block. A rare but serious side effect has been seen, dubbed "suicidal right ventricle," in which the infundibulum obstructs the right outflow tract; this may self-resolve or require reintervention.
Patients suffering from pulmonic stenosis can also undergo virtually all dental procedures. Antibiotics are only recommended with a history of prior endocarditis or a prosthetic valve placement. In pregnancy, patients should avoid vigorous exercise, especially in the second half, when they have moderate or severe gradients. Finally, in athletes with mild stenosis and gradients less than 50 mm Hg, there are no activity limitations; more severe cases should only participate in low-intensity competitive sports.
Complications of pulmonic stenosis are rare. Infective endocarditis is possible; however, it is the lowest likelihood of all valvular pathologies at 0.38 per 10,000 person-years. Other complications include arrhythmias, typically as premature atrial contractions, premature ventricular contractions, and ventricular couplets.
In the setting of pregnancy, a high-risk obstetrician should be consulted as the hemodynamic changes associated with pregnancy can exacerbate pulmonic stenosis symptoms. Cardiology consultation for a pregnant patient may be warranted if the symptoms are severe. Consult of a pediatric cardiologist is warranted for newborns experiencing symptoms of pulmonic stenosis. Depending on the severity, pediatric critical care may also be necessary. In cases of suspected symptomatic pulmonic stenosis in an adult, a cardiology consult may be warranted.
Parents of patients diagnosed with pulmonic stenosis should be educated about complications and treatments available to pediatrics patients. Adult patients, especially those who are planning to become pregnant, should be educated about the risk of such decisions exacerbating their underlying condition.
An interprofessional team approach will ensure the best outcome during pregnancy and labor for patients with pulmonic stenosis. Obstetricians and cardiologists should be in communication for the affected mother's health, as well as with a pediatric intensivist and cardiologist for the affected neonate.
|||Balzer D, Pulmonary Valve Replacement for Tetralogy of Fallot. Methodist DeBakey cardiovascular journal. 2019 Apr-Jun; [PubMed PMID: 31384375]|
|||Hill G, Repair and follow-up of Tetralogy of Fallot with pulmonary stenosis. Congenital heart disease. 2013 Mar-Apr; [PubMed PMID: 23448360]|
|||Anderson K,Cnota J,James J,Miller EM,Parrott A,Pilipenko V,Weaver KN,Shikany A, Prevalence of Noonan spectrum disorders in a pediatric population with valvar pulmonary stenosis. Congenital heart disease. 2019 Mar; [PubMed PMID: 30556322]|
|||Yazigi A,De Pecoulas AE,Vauloup-Fellous C,Grangeot-Keros L,Ayoubi JM,Picone O, Fetal and neonatal abnormalities due to congenital rubella syndrome: a review of literature. The journal of maternal-fetal [PubMed PMID: 27002428]|
|||Khanra D,Shrivastava Y,Duggal B,Soni S, Congenital supravalvular and subvalvular pulmonary stenosis with hypoplastic pulmonary annulus associated with congenital rubella syndrome. BMJ case reports. 2019 Jul 10; [PubMed PMID: 31296628]|
|||Simula DV,Edwards WD,Tazelaar HD,Connolly HM,Schaff HV, Surgical pathology of carcinoid heart disease: a study of 139 valves from 75 patients spanning 20 years. Mayo Clinic proceedings. 2002 Feb; [PubMed PMID: 11838647]|
|||Karimi A,Pourafshar N,Fudge JC, Pulmonary and tricuspid valvuloplasty in carcinoid heart disease. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography [PubMed PMID: 28029208]|
|||Nguyen A,Schaff HV,Abel MD,Luis SA,Lahr BD,Halfdanarson TR,Connolly HM, Improving outcome of valve replacement for carcinoid heart disease. The Journal of thoracic and cardiovascular surgery. 2019 Jul; [PubMed PMID: 30527716]|
|||Samánek M,Slavík Z,Zborilová B,Hrobonová V,Vorísková M,Skovránek J, Prevalence, treatment, and outcome of heart disease in live-born children: a prospective analysis of 91,823 live-born children. Pediatric cardiology. 1989 Fall; [PubMed PMID: 2687820]|
|||Stephensen SS,Sigfusson G,Eiriksson H,Sverrisson JT,Torfason B,Haraldsson A,Helgason H, Congenital cardiac malformations in Iceland from 1990 through 1999. Cardiology in the young. 2004 Aug; [PubMed PMID: 15680046]|
|||CAMPBELL M, Factors in the aetiology of pulmonary stenosis. British heart journal. 1962 Sep; [PubMed PMID: 14018144]|
|||Snellen HA,Hartman H,Buis-Liem TN,Kole EH,Rohmer J, Pulmonic stenosis. Circulation. 1968 Jul; [PubMed PMID: 4889601]|
|||Linde LM,Turner SW,Sparkes RS, Pulmonary valvular dysplasia. A cardiofacial syndrome. British heart journal. 1973 Mar; [PubMed PMID: 4692662]|
|||Robertson M,Benson LN,Smallhorn JS,Musewe N,Freedom RM,Moes CA,Burrows P,Johnston AE,Burrows FA,Rowe RD, The morphology of the right ventricular outflow tract after percutaneous pulmonary valvotomy: long term follow up. British heart journal. 1987 Sep; [PubMed PMID: 3663423]|
|||Kumar M,Turrentine MW,Rodefeld MD,Bell T,Brown JW, Right Ventricular Outflow Tract Reconstruction With a Polytetrafluoroethylene Monocusp Valve: A 20-Year Experience. Seminars in thoracic and cardiovascular surgery. 2016 Summer; [PubMed PMID: 28043462]|
|||Cheatham SL,Holzer RJ,Chisolm JL,Cheatham JP, The Medtronic Melody® transcatheter pulmonary valve implanted at 24-mm diameter--it works. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography [PubMed PMID: 23359563]|
|||Fitzgerald KP,Lim MJ, The pulmonary valve. Cardiology clinics. 2011 May; [PubMed PMID: 21459245]|
|||Hultgren HN,Reeve R,Cohn K,McLeod R, The ejection click of valvular pulmonic stenosis. Circulation. 1969 Nov; [PubMed PMID: 5377205]|
|||Stern J,Delman AJ, Normal splitting of the second heart sound in significant valvular pulmonic stenosis. American heart journal. 1968 Jul; [PubMed PMID: 5659268]|
|||Hayes CJ,Gersony WM,Driscoll DJ,Keane JF,Kidd L,O'Fallon WM,Pieroni DR,Wolfe RR,Weidman WH, Second natural history study of congenital heart defects. Results of treatment of patients with pulmonary valvar stenosis. Circulation. 1993 Feb; [PubMed PMID: 8425320]|
|||Fathallah M,Krasuski RA, Pulmonic Valve Disease: Review of Pathology and Current Treatment Options. Current cardiology reports. 2017 Sep 16; [PubMed PMID: 28916901]|
|||Nishimura RA,Otto CM,Bonow RO,Carabello BA,Erwin JP 3rd,Guyton RA,O'Gara PT,Ruiz CE,Skubas NJ,Sorajja P,Sundt TM 3rd,Thomas JD, 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Journal of the American College of Cardiology. 2014 Jun 10; [PubMed PMID: 24603191]|
|||Baumgartner H,Hung J,Bermejo J,Chambers JB,Evangelista A,Griffin BP,Iung B,Otto CM,Pellikka PA,Quiñones M, Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography. 2009 Jan; [PubMed PMID: 19130998]|
|||Weyman AE,Hurwitz RA,Girod DA,Dillon JC,Feigenbaum H,Green D, Cross-sectional echocardiographic visualization of the stenotic pulmonary valve. Circulation. 1977 Nov; [PubMed PMID: 912836]|
|||Lima CO,Sahn DJ,Valdes-Cruz LM,Goldberg SJ,Barron JV,Allen HD,Grenadier E, Noninvasive prediction of transvalvular pressure gradient in patients with pulmonary stenosis by quantitative two-dimensional echocardiographic Doppler studies. Circulation. 1983 Apr; [PubMed PMID: 6825241]|
|||Warnes CA,Williams RG,Bashore TM,Child JS,Connolly HM,Dearani JA,Del Nido P,Fasules JW,Graham TP Jr,Hijazi ZM,Hunt SA,King ME,Landzberg MJ,Miner PD,Radford MJ,Walsh EP,Webb GD, ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: Executive Summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease). Circulation. 2008 Dec 2; [PubMed PMID: 18997168]|
|||Miranda WR,Connolly HM,DeSimone DC,Phillips SD,Wilson WR,Sohail MR,Steckelberg JM,Baddour LM, Infective Endocarditis Involving the Pulmonary Valve. The American journal of cardiology. 2015 Dec 15; [PubMed PMID: 26611123]|
|||Warnes CA,Williams RG,Bashore TM,Child JS,Connolly HM,Dearani JA,del Nido P,Fasules JW,Graham TP Jr,Hijazi ZM,Hunt SA,King ME,Landzberg MJ,Miner PD,Radford MJ,Walsh EP,Webb GD, ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation. 2008 Dec 2; [PubMed PMID: 18997169]|
|||Baumgartner H,Bonhoeffer P,De Groot NM,de Haan F,Deanfield JE,Galie N,Gatzoulis MA,Gohlke-Baerwolf C,Kaemmerer H,Kilner P,Meijboom F,Mulder BJ,Oechslin E,Oliver JM,Serraf A,Szatmari A,Thaulow E,Vouhe PR,Walma E, ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). European heart journal. 2010 Dec; [PubMed PMID: 20801927]|
|||Zaidi A,Ionescu A,Sharma R,Heatley M, Echocardiographic surveillance of aortic valve stenosis: towards a standardized approach. The Journal of heart valve disease. 2012 Nov; [PubMed PMID: 23409349]|
|||Fernando RJ,Garner C,Slaughter TF, A Review of the 2017 American Society of Echocardiography Guidelines for Evaluation of Aortic Stenosis: Considerations for Perioperative Echocardiography. Journal of cardiothoracic and vascular anesthesia. 2018 Aug; [PubMed PMID: 29735221]|
|||Galiè N,Humbert M,Vachiery JL,Gibbs S,Lang I,Torbicki A,Simonneau G,Peacock A,Vonk Noordegraaf A,Beghetti M,Ghofrani A,Gomez Sanchez MA,Hansmann G,Klepetko W,Lancellotti P,Matucci M,McDonagh T,Pierard LA,Trindade PT,Zompatori M,Hoeper M, 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). European heart journal. 2016 Jan 1; [PubMed PMID: 26320113]|
|||Fratz S,Hess J,Schwaiger M,Martinoff S,Stern HC, More accurate quantification of pulmonary blood flow by magnetic resonance imaging than by lung perfusion scintigraphy in patients with fontan circulation. Circulation. 2002 Sep 17; [PubMed PMID: 12234957]|
|||Kutty S,Rathod RH,Danford DA,Celermajer DS, Role of imaging in the evaluation of single ventricle with the Fontan palliation. Heart (British Cardiac Society). 2016 Feb; [PubMed PMID: 26567230]|
|||Sridharan S,Derrick G,Deanfield J,Taylor AM, Assessment of differential branch pulmonary blood flow: a comparative study of phase contrast magnetic resonance imaging and radionuclide lung perfusion imaging. Heart (British Cardiac Society). 2006 Jul; [PubMed PMID: 16775104]|
|||Steeden JA,Atkinson D,Hansen MS,Taylor AM,Muthurangu V, Rapid flow assessment of congenital heart disease with high-spatiotemporal-resolution gated spiral phase-contrast MR imaging. Radiology. 2011 Jul; [PubMed PMID: 21415248]|
|||Roman KS,Kellenberger CJ,Farooq S,MacGowan CK,Gilday DL,Yoo SJ, Comparative imaging of differential pulmonary blood flow in patients with congenital heart disease: magnetic resonance imaging versus lung perfusion scintigraphy. Pediatric radiology. 2005 Mar; [PubMed PMID: 15490145]|
|||Shaath G,Al Mutairi M,Tamimi O,Alakhfash A,Abolfotouh M,Alhabshan F, Predictors of reintervention in neonates with critical pulmonary stenosis or pulmonary atresia with intact ventricular septum. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography [PubMed PMID: 21954133]|
|||Roos-Hesselink JW,Meijboom FJ,Spitaels SE,vanDomburg RT,vanRijen EH,Utens EM,Bogers AJ,Simoons ML, Long-term outcome after surgery for pulmonary stenosis (a longitudinal study of 22-33 years). European heart journal. 2006 Feb; [PubMed PMID: 16361324]|
|||Nielsen EA,Hjortdal VE, Surgically treated pulmonary stenosis: over 50 years of follow-up. Cardiology in the young. 2016 Jun; [PubMed PMID: 26403466]|
|||Sehar T,Qureshi AU,Kazmi U,Mehmood A,Hyder SN,Sadiq M, Balloon valvuloplasty in dysplastic pulmonary valve stenosis: immediate and intermediate outcomes. Journal of the College of Physicians and Surgeons--Pakistan : JCPSP. 2015 Jan; [PubMed PMID: 25604363]|
|||Parent JJ,Ross MM,Bendaly EA,Breinholt JP, Results of pulmonary balloon valvuloplasty persist and improve at late follow-up in isolated pulmonary valve stenosis. Cardiology in the young. 2017 Oct; [PubMed PMID: 28651667]|
|||Taggart NW,Cetta F,Cabalka AK,Hagler DJ, Outcomes for balloon pulmonary valvuloplasty in adults: comparison with a concurrent pediatric cohort. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography [PubMed PMID: 23613310]|
|||Rao PS, Percutaneous balloon pulmonary valvuloplasty: state of the art. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography [PubMed PMID: 17330270]|
|||Ben-Shachar G,Cohen MH,Sivakoff MC,Portman MA,Riemenschneider TA,Van Heeckeren DW, Development of infundibular obstruction after percutaneous pulmonary balloon valvuloplasty. Journal of the American College of Cardiology. 1985 Mar; [PubMed PMID: 3156173]|
|||Ahmadi A,Sabri M, Percutaneous balloon valvuloplasty inpatients with pulmonary valve stenosis: a single center experiment. JPMA. The Journal of the Pakistan Medical Association. 2012 Mar; [PubMed PMID: 22768462]|
|||Wilson W,Taubert KA,Gewitz M,Lockhart PB,Baddour LM,Levison M,Bolger A,Cabell CH,Takahashi M,Baltimore RS,Newburger JW,Strom BL,Tani LY,Gerber M,Bonow RO,Pallasch T,Shulman ST,Rowley AH,Burns JC,Ferrieri P,Gardner T,Goff D,Durack DT, Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Journal of the American Dental Association (1939). 2008 Jan; [PubMed PMID: 18167394]|
|||Gersony WM,Hayes CJ,Driscoll DJ,Keane JF,Kidd L,O'Fallon WM,Pieroni DR,Wolfe RR,Weidman WH, Bacterial endocarditis in patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect. Circulation. 1993 Feb; [PubMed PMID: 8425318]|
|||Gersony WM,Hayes CJ, Bacterial endocarditis in patients with pulmonary stenosis, aortic stenosis, or ventricular septal defect. Circulation. 1977 Aug; [PubMed PMID: 872351]|
|||Wolfe RR,Driscoll DJ,Gersony WM,Hayes CJ,Keane JF,Kidd L,O'Fallon WM,Pieroni DR,Weidman WH, Arrhythmias in patients with valvar aortic stenosis, valvar pulmonary stenosis, and ventricular septal defect. Results of 24-hour ECG monitoring. Circulation. 1993 Feb; [PubMed PMID: 8425327]|
|||Cuypers JA,Menting ME,Opić P,Utens EM,Helbing WA,Witsenburg M,van den Bosch AE,van Domburg RT,Baart SJ,Boersma E,Meijboom FJ,Bogers AJ,Roos-Hesselink JW, The unnatural history of pulmonary stenosis up to 40 years after surgical repair. Heart (British Cardiac Society). 2017 Feb 15; [PubMed PMID: 27515953]|
|||Ruckdeschel ES,Schuller J,Nguyen DT, Ventricular Tachycardia in Congenital Pulmonary Stenosis. Cardiac electrophysiology clinics. 2016 Mar; [PubMed PMID: 26920196]|