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

Pulmonary Stenosis, Valvar: Follow-up

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

Follow-up

Further Inpatient Care

  • The neonate with critical pulmonary valve stenosis requires special consideration.
    • Patients with critical pulmonary stenosis may present with near–pulmonary 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.

Further Outpatient Care

  • Clinical, ECG, and Doppler echocardiographic evaluation are recommended at 1 month, 6 months, and 12 months after balloon pulmonary valvuloplasty and yearly thereafter.
  • Patients with trivial and mild pulmonary stenosis do not need intervention to relieve the pulmonary valve obstruction. However, they should be clinically followed up at periodic intervals (eg, on a yearly basis).
  • Routine well-child care, including immunizations, as per the primary physician, is suggested.
  • Physical activity should be normal.
  • Most patients with pulmonary stenosis are given prophylaxis for subacute bacterial endocarditis (SBE).
  • Opinions differ about the need for SBE prophylaxis in patients with valvar pulmonary stenosis because of the extremely low incidence of pulmonary valve endocarditis in this relatively large subpopulation. The author recommends SBE prophylaxis for all patients with valvar pulmonary stenosis.

Transfer

  • Transfer patients with symptomatic pulmonary stenosis to a tertiary care center offering pediatric cardiology and pediatric cardiothoracic surgery services.

Complications

  • Hypercontractile residual obstructing muscular hypertrophy in the infundibulum: This complication is associated with surgical or balloon valuloplasty for severe pulmonary stenosis.
  • Infundibular obstruction: This phenomenon, which occurs after valvar stenosis repair by means of surgery or valvuloplasty, has led to the designation of a suicide right ventricle. Beta-blockers and volume replacement are used to treat this condition, which occurs more frequently in relatively old patients with long-standing pulmonary stenosis.
  • Late atrial arrhythmias
  • Persistent repolarization abnormalities

Prognosis

  • Mild valvar pulmonary stenosis usually does not progress, but moderate-to-severe disease does tend to progress.
  • After the stenosis is relieved by means of balloon valvuloplasty, 8-10% patients require repeat intervention. The condition does not recur in the remaining patients, and right ventricular hypertrophy regresses.
  • Pulmonary valve regurgitation may need reintervention and further long-term studies are required to assess the impact of this issue on long-term outcome.  
  • The outcome after balloon or surgical valvulotomy is generally excellent. The probability of survival is similar to that of the general population, and the vast majority of patients are asymptomatic.

Patient Education

  • Reassure patients and parents of children with mild valvar pulmonary stenosis that this condition is not related to or associated with coronary artery disease, dysrhythmia, or sudden death.
  • Insurability may become a factor in obtaining further care. Patients are no more at risk for disastrous health consequences than is the usual population.
  • Provided the patient is asymptomatic and acyanotic and provided that initial Doppler echocardiograms show only mild valvar pulmonary stenosis, yearly screening examination and ECG are prudent follow-up care.
  • If evaluations performed a few years after the initial evaluation reveal no clinically significant change, the patient may be followed up once every 3-5 years.
  • For excellent patient education resources, visit eMedicine's Heart Center. Also, see eMedicine's patient education article Tetralogy of Fallot.

Miscellaneous

Medicolegal Pitfalls

  • Failure to exclude associated congenital anomalies and to detect the presence of cyanosis or a ductal-dependent lesion is a major error.
  • Failure to diagnose a serious congenital heart defect, such as tetralogy of Fallot is problematic.
    • Acyanotic patients with tetralogy of Fallot and those with a mild obstruction of the right ventricular outflow tract may have similar presentations and physical findings.
    • Tetralogy of Fallot is a lesion that is surgically correctable and that can be corrected safely, even in the neonatal period.
    • A tet spell, or hypercyanotic spell, is potentially lethal but frequently aborted with simple maneuvers. Such spells and can occur in previously acyanotic patients with tetralogy of Fallot ("pink tets").
    • Echocardiography can reliably confirm the precise diagnosis and help in differentiating valvar pulmonary stenosis from tetralogy of Fallot.
  • Echocardiography should not be withheld if complex anatomy is suspected.
  • Complications associated with balloon pulmonary valvuloplasty, such a rupture or perforation of the pulmonary artery or right ventricular outflow tract, are uncommon but can occur. This possibility should be explained to all parents.
 


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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