eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Pulmonary Stenosis, Valvar

Author: P Syamasundar Rao, MD, Professor of Pediatrics and Medicine, University of Texas-Houston Medical School; Director, Division of Pediatric Cardiology, Children's Memorial Hermann Hospital; Professor of Pediatrics, MD Anderson Cancer Center, University of Texas
Coauthor(s): Kurt Pflieger, MD, FAAP, Active Staff, Department of Pediatrics, Lake Pointe Medical Center
Contributor Information and Disclosures

Updated: Jul 6, 2009

Introduction

Background

Diseases of the pulmonary valve are most often congenital, and only rarely do acquired disorders such as carcinoid and rheumatic fever affect the pulmonary valve.1 The pulmonary valve may be stenotic or atretic, or the leaflets of the valve may be absent. Pulmonary stenosis may be valvar, supravalvar, or subvalvar (ie, double-chambered right ventricle); it may also be in the branch pulmonary arteries. These lesions are collectively associated with obstruction of the right ventricular outflow tract. In this article, only valvar pulmonary stenosis is reviewed.

A stenotic pulmonary valve may occur without associated congenital abnormalities, although it is most often associated with other structural abnormalities of the heart. To distinguish the former from the latter, terms such as pulmonary stenosis with a normal aortic root or pulmonary stenosis with an intact ventricular septum have been used. However, the term isolated pulmonary valve stenosis is preferred.1 The term isolated may be used even when a patent foramen ovale or a small atrial septal defect is present. The term congenital need not be used because most are congenital.

Pathologic Anatomy

 Pathologic features of the stenotic pulmonary valve vary.2 The most common pathology is a dome-shaped pulmonary valve. The fused leaflets of the pulmonary valve protrude from their attachment into the pulmonary artery as a conical, windsock-like structure. The size of the pulmonary valve orifice varies from a pinhole to several millimeters. The orifice is most usually central but can be eccentric. Raphae, presumably fused commissures of the valve, extend from the stenotic orifice to a variable distance down into the base of the dome-shaped valve. The number of the raphe may vary from 0-7. Relatively uncommon variants are unicommissural, bicuspid, and tricuspid valves. The valve annulus is abnormal in most cases, and the fibrous back bone is partially or completely lacking; therefore, a true annulus may not be present.2

Hypoplasia of the pulmonary valve ring and dysplastic pulmonary valves may be present in a few of patients. Pulmonary valve dysplasia is characterized by thickened, nodular, and redundant valvular leaflets with minimal or no commissural fusion; hypoplasia of the valve ring; and lack of poststenotic dilatation of the pulmonary artery.3,4 The obstruction is mainly related to thickened, myxomatous, immobile pulmonary valve cusps and hypoplasia of the valve ring.

Changes secondary to pulmonary valve obstruction occur in the right ventricle and pulmonary artery.1,2 Hypertrophy of the right ventricular muscle is proportional to the degree (and perhaps the duration) of obstruction. The muscle hypertrophy is particularly prominent in the infundibular region and may become physiologically important; this appears to be related to the degree and duration of obstruction.5 Mild dilatation of the right ventricular cavity is present. In extremely severe or critical obstruction, the right ventricular cavity may be markedly dilated. In rare cases, the right ventricle may be hypoplastic.

The main pulmonary artery is dilated in almost all cases. This dilatation is independent of the severity of the pulmonary valve obstruction and presumably related to a high-velocity jet across the stenotic valve.6,7 As noted above, such poststenotic dilatation is remarkably absent in patients with dysplastic pulmonary valves.

An interatrial communication, a patent foramen ovale or an atrial septal defect may be present and may be the seat for right-to-left shunt in patients with severe or long-standing pulmonary stenosis.

Pulmonary valve stenosis is the most common cardiac lesion in Noonan syndrome (phenotypically Turner syndrome and genotypically normal [XX or XY]).8,9

Supravalvar pulmonary stenosis is often associated with rubella syndrome and Williams syndrome (ie, elfin facies, supravalvar aortic stenosis, and hypercalcemia with or without mental retardation).

Isolated infundibular or subvalvar pulmonary stenosis is uncommon and usually associated with a ventricular septal defect (VSD), such as in tetralogy of Fallot.

Peripheral pulmonary stenosis is frequently observed in newborns. It is related to relative narrowing of the branch pulmonary arteries and the acute angle of the origin of the branch pulmonary arteries at this age. This represents fetal pattern and, in most cases, resolves over time.

Pathophysiology

Clinically significant narrowing of a valve or a blood vessel increases pressure proximal to the obstruction. This pressure gradient is necessary to maintain flow across the stenotic site. In pulmonic stenosis, hypertrophy of the right ventricle ensues and maintains this forward flow. The magnitude of right ventricular pressure and the pressure gradient across the pulmonary valve are generally proportional to the degree of obstruction. Under usual circumstances, proportional right ventricular hypertrophy maintains normal pulmonary blood flow. If the normal output is not maintained, right-sided heart failure ensues. This occurs in neonates with critical pulmonary stenosis and in patients with severe obstruction that occurs in childhood or adulthood.

Changes in the geometry of the left ventricle and decreased left ventricular function can also occur.10,11 The changes are proportional to the degree of right ventricular hypertrophy; however, they revert to normal after obstruction of the right ventricular outflow tract is relieved.

With increasing right ventricular hypertrophy, right ventricular compliance decreases with a resultant increase in end-diastolic pressure and with prominent a waves in the right atrium. As right atrial pressure rises, a right-to-left shunt may occur if the foramen ovale is patent or if an atrial septal defect is present; this change results in systemic arterial desaturation and clinically discernible cyanosis. This shunting may occur even without measurable elevation of right atrial pressure and is attributable to decreased right ventricular compliance.12 Such a right-to-left shunt can also occur in patients with an underdeveloped (hypoplastic) right ventricle.13

Frequency

United States

Pulmonary stenosis represents 8-12% of all congenital heart defects in children.14,15 In adults, pulmonary stenosis represents approximately 15% of all congenital heart defects.16,17,18 Isolated valvar pulmonary stenosis with an intact ventricular septum is the second most common congenital cardiac defect in children. It may occur in as many as 50% of all patients with congenital heart disease associated with other congenital cardiac lesions.

Mortality/Morbidity

The severity of the valvar dysplasia determines its related morbidity and mortality. Mild-to-moderate valvar pulmonary stenosis is extremely well tolerated. Severe pulmonary stenosis can be associated with decreased pulmonary flow, right ventricular hypertrophy, early congestive heart failure (CHF) with critical obstructions, and cyanosis. The morbidity formerly associated with surgery is lessened with balloon pulmonary valvuloplasty.

Race

Racial difference in the prevalence of pulmonary stenosis is unlikely.19

Sex

The male-to-female ratio is 1:1.16

Age

The patient's age at presentation is related to the severity of the obstruction. If the stenosis is severe, patients may present in the neonatal period or in infancy. Patients with mild obstruction may present in childhood with asymptomatic murmurs.

Clinical

History

  • Most children with pulmonic stenosis, particularly those with trivial and mild pulmonary stenosis, present with asymptomatic cardiac murmurs that are detected during routine examination.
  • Patients with moderate or severe pulmonary stenosis may have mild exertional dyspnea. Adults may be asymptomatic irrespective of the severity of their obstruction.16,19,20
  • Patients with severe or critical obstruction may present with signs of systemic venous congestion, which are usually interpreted as signs of congestive heart failure (CHF). The signs are due to severe right ventricular dysfunction or to cyanosis secondary to a right-to-left shunt across a patent foramen ovale or an atrial septal defect.
  • Lightheadedness, syncope, and chest pain that resembles angina pectoris are rare, even in patients with severe obstruction.
  • Of note, many patients with moderate or severe pulmonary stenosis remain asymptomatic.

Physical

  • Physical findings depend on the degree of obstruction.
    • Most patients with pulmonary stenosis appear healthy and are well developed. Indeed, the chubby and rounded faces, described as moon facies, were initially thought to be characteristic for this anomaly,15,17 but this facial appearance is not a helpful diagnostic tool.21
    • Most patients with trivial, mild, or moderate stenosis, and many with severe stenosis, are acyanotic. However, some may have cyanosis secondary to an interatrial right-to-left shunt.
    • The jugular venous pulse is normal. However, in patients with decreased right ventricular compliance (ie, severe stenosis), a prominent α wave may be visualized in the neck pulsation. Patients may have a concomitant presystolic pulsation in the liver as well.
    • In patients with trivial or mild obstruction, the right ventricular impulse is normal. When the pulmonary stenosis is moderate to severe, a sustained and forceful right ventricular impulse and a right ventricular heave are felt.
    • A thrill may be felt in the suprasternal notch and at the left upper sternal border (pulmonic area). The precordial thrill is most likely to be associated with severe obstruction, although no consistent relationship is observed between the thrill and the degree of obstruction.
    • Upon auscultation, the first heart sound may be normal in intensity or may be loud. The second heart sound is widely split. The width of the split increases with worsening stenosis. The intensity of pulmonary component of the second heart sound may be loud (in mild stenosis) or may be soft, diminished, or absent, depending on the severity of obstruction. A fourth heart sound may be heard at the left lower sternal border in patients with severe obstruction and is usually associated with prominent α wave in the jugular pulse.
    • An ejection systolic click is heard along the left sternal border and varies with respiration (decreases or disappears during inspiration). With increasing severity, the click comes closer to the first heart sound.
    • An ejection systolic murmur of grade II-VI to V-VI is best heard at the left upper sternal border with radiation into infraclavicular regions, axillae, or back. The intensity of the murmur is not necessarily related to the severity of pulmonary valve obstruction, but the duration and timing of peaking of the murmur are related to the severity of stenosis.
    • An early diastolic decrescendo murmur of pulmonary regurgitation is not usually heard in the typical case of pulmonary stenosis. Previous surgical or balloon intervention or valvar calcification may result in such a murmur.
    • A holosystolic murmur at the left lower sternal border, which indicates tricuspid regurgitation, may be audible in some patients with extremely severe pulmonary stenosis.
    • Hepatosplenomegaly may develop in cases of CHF.
    • Peripheral pulmonary stenosis (commonly encountered in the neonate) is usually associated with a grade II/VI systolic murmur that radiates into the posterior lung fields and axillae. The pathology of peripheral pulmonary stenosis is related to branch pulmonary arteries that are relatively small compared with the large main pulmonary artery, as well as to the acute angular takeoff of the branch pulmonary arteries from the main pulmonary artery specific to a neonate's anatomy. This condition and the associated murmur usually resolve spontaneously in the first month of life.
  • Clinical assessment of severity
    • The severity of the obstruction of the pulmonary valve can often be estimated by carefully analyzing the ausculatory findings.22,23 The timing of the ejection click, the extent of splitting of the second heart sound, the intensity of the pulmonary component of the second sound, the duration of the systolic murmur, and the timing of the peaking of the ejection murmur usually indicate the severity of pulmonary valve stenosis (see Media file 1).

      In valvar pulmonic stenosis, the severity of obst...

      In valvar pulmonic stenosis, the severity of obstruction may be judged by auscultatory findings. In mild stenosis, the ejection click (EC) is clearly separated from the first heart sound (S1). The murmur starts with the click, peaks in early systole, and ends way before the aortic component of the second heart sound (A2) The pulmonary component of the second heart sound (P2) is normal to increased in intensity. In moderate pulmonic stenosis, the click is closer to the first heart sound, the ejection murmur peaks later in the systole and the murmur reaches the A2, and the second heart sound is widely split with soft pulmonary component. In severe valvar obstruction, the click is either absent or occurs so close to S1 that it cannot be heard separately, and the murmur peaks late in systole and extends beyond the A2. The second heart sound is widely split with an extremely soft or inaudible P2. Reproduced from Rao PS: Evaluation of cardiac murmur in children. Indian J Pediatr 1991 Jul-Aug; 58(4): 471-91.

      [ CLOSE WINDOW ]
      In valvar pulmonic stenosis, the severity of obst...

      In valvar pulmonic stenosis, the severity of obstruction may be judged by auscultatory findings. In mild stenosis, the ejection click (EC) is clearly separated from the first heart sound (S1). The murmur starts with the click, peaks in early systole, and ends way before the aortic component of the second heart sound (A2) The pulmonary component of the second heart sound (P2) is normal to increased in intensity. In moderate pulmonic stenosis, the click is closer to the first heart sound, the ejection murmur peaks later in the systole and the murmur reaches the A2, and the second heart sound is widely split with soft pulmonary component. In severe valvar obstruction, the click is either absent or occurs so close to S1 that it cannot be heard separately, and the murmur peaks late in systole and extends beyond the A2. The second heart sound is widely split with an extremely soft or inaudible P2. Reproduced from Rao PS: Evaluation of cardiac murmur in children. Indian J Pediatr 1991 Jul-Aug; 58(4): 471-91.

    • With trivial and mild cases of pulmonary valve obstruction, the click is clearly separated from the first heart sound. Almost normal splitting of the second heart sound with normal or slightly increased pulmonary component of the second heart sound is heard. An ejection systolic, diamond-shaped murmur that peaks early in systole and that ends much before the aortic component of the second heart sound is appreciated.
    • Findings in moderate pulmonary valve stenosis include an ejection systolic click that is closer to the first heart sound than it is in mild forms, a widely split second sound with a diminished pulmonary component, and an ejection systolic murmur that peaks in mid-to-late systole and that ends just before the aortic component of the second heart sound.
    • In severe narrowing of the pulmonary valve, ausculatory features are an ejection systolic click that is absent or that occurs so close to the first heart sound that it becomes inseparable from it, markedly increased splitting with a soft or inaudible pulmonary component of the second heart sound, and a long ejection systolic murmur that peaks late in systole and that extends beyond the aortic component of the second heart sound so that the latter cannot be heard.
    • The duration and time of peaking of the ejection systolic murmur, and not its intensity, indicate the severity of the pulmonary valve obstruction. The longer the murmur and the later it peaks, the more severe the obstruction. Likewise, the shorter the interval between the first heart sound and ejection click, the wider the splitting of the second heart sound, and the softer the pulmonary component, the more severe the stenosis of the pulmonary valve.

Causes

  • Pulmonary valve stenosis is primarily due to maldevelopment of the pulmonary valve tissue and the distal portion of the bulbus cordis, which is characterized by fusion of leaflet commissures, resulting in a thickened and domed appearance of the valve.
  • Although familial forms of pulmonary stenosis are described, it is generally considered to be multifactorial in origin.24 Rates of recurrence in siblings are on the order of 2-3%.25,26 The prevalence of pulmonary stenosis in the offspring of a parent with pulmonary stenosis is 3.6%.
  • Aberrant flow patterns in utero may also be partly associated with maldevelopment of the pulmonary valve.27

More on Pulmonary Stenosis, Valvar

Overview: Pulmonary Stenosis, Valvar
Differential Diagnoses & Workup: Pulmonary Stenosis, Valvar
Treatment & Medication: Pulmonary Stenosis, Valvar
Follow-up: Pulmonary Stenosis, Valvar
Multimedia: Pulmonary Stenosis, Valvar
References
Further Reading
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Keywords

valvar pulmonary stenosis, pulmonary artery, right ventricle, valvular pulmonary stenosis, valvate pulmonary stenosis, pulmonary stenosis with a normal aortic root, pulmonary stenosis with an intact ventricular septum, isolated pulmonary valve stenosis, ventricular septal defect, rheumatic fever, pulmonary valve dysplasia, atrial septal defect, Noonan syndrome, Turner syndrome, rubella syndrome, Williams syndrome, ventricular septal defect, VSD, tetralogy of Fallot, heart failure, patent foramen ovale, underdeveloped right ventricle, hypoplastic right ventricle, syncope, angina pectoris, tricuspid regurgitation, hepatosplenomegaly, treatment, diagnosis

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Contributor Information and Disclosures

Author

P Syamasundar Rao, MD, Professor of Pediatrics and Medicine, University of Texas-Houston Medical School; Director, Division of Pediatric Cardiology, Children's Memorial Hermann Hospital; Professor of Pediatrics, MD Anderson Cancer Center, University of Texas
P Syamasundar Rao, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, American Pediatric Society, Medical Association of Georgia, Society for Cardiac Angiography and Interventions, Society for Pediatric Research, Southern Society for Pediatric Research, and Western Society for Pediatric Research
Disclosure: Nothing to disclose.

Coauthor(s)

Kurt Pflieger, MD, FAAP, Active Staff, Department of Pediatrics, Lake Pointe Medical Center
Kurt Pflieger, MD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Emergency Physicians, American Heart Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Jeffrey Allen Towbin, MD, MSc, FAAP, FACC, FAHA, Professor, Departments of Pediatrics (Cardiology), Cardiovascular Sciences, and Molecular and Human Genetics, Baylor College of Medicine; Chief of Pediatric Cardiology, Foundation Chair in Pediatric Cardiac Research, Texas Children's Hospital
Jeffrey Allen Towbin, MD, MSc, FAAP, FACC, FAHA is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American College of Cardiology, American College of Sports Medicine, American Heart Association, American Medical Association, American Society of Human Genetics, Cardiac Electrophysiology Society, New York Academy of Sciences, Society for Pediatric Research, Texas Medical Association, and Texas Pediatric Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

John W Moore, MD, MPH, Professor of Clinical Pediatrics, Section of Pediatric Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital
John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

CME Editor

Gilbert Z Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD, Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin
Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

 
 
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