Natural history and treatment of pulmonic stenosis in adults
- Karen Stout, MD
Karen Stout, MD
- Cardiology/Medicine and Pediatrics
- University of Washington
Nearly all cases of valvular pulmonic stenosis are congenital in origin, and most cases occur as an isolated lesion. Acquired cases of stenosis of the native pulmonary valve are encountered less commonly but may be caused by the carcinoid syndrome or rheumatic fever (in which case pulmonic stenosis is always associated with other valve abnormalities). There are increasing numbers of patients with stenosis of bioprosthetic or valved conduits used in repair of more complex congenital lesions affecting the pulmonary valve, such as tetralogy of Fallot, pulmonary atresia, or truncus arteriosus. (See "Clinical manifestations and diagnosis of pulmonic stenosis in adults", section on 'Introduction'.)
Congenital valvular pulmonic stenosis exists in three predominant forms :
●Most cases involve dome-type pulmonic valve stenosis, which is characterized by a narrow central opening with preserved valve motion. Three rudimentary raphes are generally present without clear-cut commissures. The pulmonary trunk may be dilated due to a medial abnormality. Calcification of the valve can be seen in older patients.
●Approximately 20 percent of cases involve pulmonary valve dysplasia, with thickened valve leaflets and relative immobility of the valve (image 1). Pulmonary artery dilation is not commonly associated with dysplastic valves. Dysplastic pulmonary valves are a common component of Noonan syndrome, occurring in up to half of patients in some studies. (See "Clinical manifestations and diagnosis of pulmonic stenosis in adults", section on 'Introduction'.)
●More complex congenital heart disease, such as cases of tetralogy of Fallot or transposition of the great arteries, may be associated with a unicuspid or bicuspid pulmonary valve, which may be obstructive. (See "Pathophysiology, clinical features, and diagnosis of tetralogy of Fallot", section on 'Right ventricular outflow obstruction'.)
- Warnes CA, Williams RG, Bashore TM, et al. 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; 118:e714.
- Khambadkone S, Coats L, Taylor A, et al. Percutaneous pulmonary valve implantation in humans: results in 59 consecutive patients. Circulation 2005; 112:1189.
- McElhinney DB, Hellenbrand WE, Zahn EM, et al. Short- and medium-term outcomes after transcatheter pulmonary valve placement in the expanded multicenter US melody valve trial. Circulation 2010; 122:507.
- Baumgartner H, Hung J, Bermejo J, et al. Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice. J Am Soc Echocardiogr 2009; 22:1.
- Baumgartner H, Bonhoeffer P, De Groot NM, et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J 2010; 31:2915.
- Nishimura RA, Otto CM, Bonow RO, et al. 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. J Am Coll Cardiol 2014; 63:e57.
- Silvilairat S, Cabalka AK, Cetta F, et al. Echocardiographic assessment of isolated pulmonary valve stenosis: which outpatient Doppler gradient has the most clinical validity? J Am Soc Echocardiogr 2005; 18:1137.
- Hayes CJ, Gersony WM, Driscoll DJ, et al. Second natural history study of congenital heart defects. Results of treatment of patients with pulmonary valvar stenosis. Circulation 1993; 87:I28.
- Nugent EW, Freedom RM, Nora JJ, et al. Clinical course in pulmonary stenosis. Circulation 1977; 56 [Suppl I]:38.
- Nadas AS. Pulmonic stenosis--indications for surgery in children and adults. N Engl J Med 1972; 287:1196.
- Earing MG, Connolly HM, Dearani JA, et al. Long-term follow-up of patients after surgical treatment for isolated pulmonary valve stenosis. Mayo Clin Proc 2005; 80:871.
- Roos-Hesselink JW, Meijboom FJ, Spitaels SE, et al. Long-term outcome after surgery for pulmonary stenosis (a longitudinal study of 22-33 years). Eur Heart J 2006; 27:482.
- Rao PS. Percutaneous balloon pulmonary valvuloplasty: state of the art. Catheter Cardiovasc Interv 2007; 69:747.
- Stanger P, Cassidy SC, Girod DA, et al. Balloon pulmonary valvuloplasty: results of the Valvuloplasty and Angioplasty of Congenital Anomalies Registry. Am J Cardiol 1990; 65:775.
- Ben-Shachar G, Cohen MH, Sivakoff MC, et al. Development of infundibular obstruction after percutaneous pulmonary balloon valvuloplasty. J Am Coll Cardiol 1985; 5:754.
- Fawzy ME, Galal O, Dunn B, et al. Regression of infundibular pulmonary stenosis after successful balloon pulmonary valvuloplasty in adults. Cathet Cardiovasc Diagn 1990; 21:77.
- Chen CR, Cheng TO, Huang T, et al. Percutaneous balloon valvuloplasty for pulmonic stenosis in adolescents and adults. N Engl J Med 1996; 335:21.
- Jarrar M, Betbout F, Farhat MB, et al. Long-term invasive and noninvasive results of percutaneous balloon pulmonary valvuloplasty in children, adolescents, and adults. Am Heart J 1999; 138:950.
- Mullen MP, Landzberg MJ. Care of adults with congenital heart disease. In: Cardiovascular Therapeutics: A Companion to Braunwald's Heart Disease, 2nd ed, Antman E (Ed), W.B. Saunders, Philadelphia 2002. p.1062.
- Rao PS, Galal O, Patnana M, et al. Results of three to 10 year follow up of balloon dilatation of the pulmonary valve. Heart 1998; 80:591.
- Peterson C, Schilthuis JJ, Dodge-Khatami A, et al. Comparative long-term results of surgery versus balloon valvuloplasty for pulmonary valve stenosis in infants and children. Ann Thorac Surg 2003; 76:1078.
- GRADING SEVERITY
- NATURAL HISTORY
- Mild stenosis
- Moderate stenosis
- Severe stenosis
- Pulmonary regurgitation following relief of pulmonary stenosis
- INDICATIONS FOR INTERVENTION
- LONG-TERM SURGICAL FOLLOW-UP
- BALLOON VALVOTOMY
- Procedural considerations
- Acute response
- Long-term efficacy
- SUMMARY AND RECOMMENDATIONS