Clinical manifestations and diagnosis of pulmonic stenosis in adults
- Karen Stout, MD
Karen Stout, MD
- Cardiology/Medicine and Pediatrics
- University of Washington
Stenosis of the pulmonary valve is a relatively common congenital defect, occurring in approximately 10 percent of children with congenital heart disease . There is a slight female predominance, and familial occurrence has been reported in 2 percent of cases .
Pulmonic stenosis in children is usually associated with a benign clinical course, and excellent treatment options are available for severe cases. Thus, there is a high rate of survival into adulthood. This topic will review the pathophysiology, clinical manifestations, and diagnosis of pulmonic stenosis. The natural history and treatment of this disorder are discussed separately. (See "Natural history and treatment of pulmonic stenosis in adults".)
Pulmonic stenosis can occur at three locations:
- Rocchini AP, Emmanouilides GC. Moss and Adams Heart Disease in Infants, Children, and Adolescents, 5th ed, Emmanouilides GC, Riemenschneider TA, Allen HD, Gutgesell HP (Eds), Williams & Wilkins, Baltimore 1995.
- CAMPBELL M. Factors in the aetiology of pulmonary stenosis. Br Heart J 1962; 24:625.
- Snellen HA, Hartman H, Buis-Liem TN, et al. Pulmonic stenosis. Circulation 1968; 38:93.
- Gabriele OF, Scatliff JH. Pulmonary valve calcification. Am Heart J 1970; 80:299.
- Keith JD, Rowe RD, Vlad P. Heart Disease in Infancy and Childhood, 3rd ed, MacMillan, New York 1978.
- Koretzky ED, Moller JH, Korns ME, et al. Congenital pulmonary stenosis resulting from dysplasia of valve. Circulation 1969; 40:43.
- Linde LM, Turner SW, Sparkes RS. Pulmonary valvular dysplasia. A cardiofacial syndrome. Br Heart J 1973; 35:301.
- Rowe RD. Cardiovascular disease in the rubella syndrome. Cardiovasc Clin 1973; 5:61.
- Simula DV, Edwards WD, Tazelaar HD, et al. Surgical pathology of carcinoid heart disease: a study of 139 valves from 75 patients spanning 20 years. Mayo Clin Proc 2002; 77:139.
- Cabrera A, Martinez P, Rumoroso JR, et al. Double-chambered right ventricle. Eur Heart J 1995; 16:682.
- Robertson M, Benson LN, Smallhorn JS, et al. The morphology of the right ventricular outflow tract after percutaneous pulmonary valvotomy: long term follow up. Br Heart J 1987; 58:239.
- Hultgren HN, Reeve R, Cohn K, McLeod R. The ejection click of valvular pulmonic stenosis. Circulation 1969; 40:631.
- 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.
- 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.
- Weyman AE, Hurwitz RA, Girod DA, et al. Cross-sectional echocardiographic visualization of the stenotic pulmonary valve. Circulation 1977; 56:769.
- Musewe NN, Robertson MA, Benson LN, et al. The dysplastic pulmonary valve: echocardiographic features and results of balloon dilatation. Br Heart J 1987; 57:364.
- Pieroni DR, Nishimura RA, Bierman FZ, et al. Second natural history study of congenital heart defects. Ventricular septal defect: echocardiography. Circulation 1993; 87:I80.
- Lima CO, Sahn DJ, Valdes-Cruz LM, et al. Noninvasive prediction of transvalvular pressure gradient in patients with pulmonary stenosis by quantitative two-dimensional echocardiographic Doppler studies. Circulation 1983; 67:866.
- Johnson GL, Kwan OL, Handshoe S, et al. Accuracy of combined two-dimensional echocardiography and continuous wave Doppler recordings in the estimation of pressure gradient in right ventricular outlet obstruction. J Am Coll Cardiol 1984; 3:1013.
- Takao S, Miyatake K, Izumi S, et al. Clinical implications of pulmonary regurgitation in healthy individuals: detection by cross sectional pulsed Doppler echocardiography. Br Heart J 1988; 59:542.
- Rocchini AP, Beekman RH. Balloon angioplasty in the treatment of pulmonary valve stenosis and coarctation of the aorta. Tex Heart Inst J 1986; 13:377.
- Shapiro SM, Young E, Ginzton LE, Bayer AS. Pulmonic valve endocarditis as an underdiagnosed disease: role of transesophageal echocardiography. J Am Soc Echocardiogr 1992; 5:48.
- Warnes CA, Williams RG, Bashore TM, et al. 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; 118:2395.
- Gersony WM, Hayes CJ, Driscoll DJ, et al. Bacterial endocarditis in patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect. Circulation 1993; 87:I121.
- Bonow RO, Carabello BA, Chatterjee K, et al. 2008 Focused update incorporated into the ACC/AHA 2006 guidelines 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 (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation 2008; 118:e523.
- Wilson W, Taubert KA, Gewitz M, et al. 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. Circulation 2007; 115 published online April 19, 2007. www.circ.ahajournals.org/cgi/reprint/CIRCULATIONAHA.106.183095v1 (Accessed on May 04, 2007).
- Wolfe RR, Driscoll DJ, Gersony WM, et al. Arrhythmias in patients with valvar aortic stenosis, valvar pulmonary stenosis, and ventricular septal defect. Results of 24-hour ECG monitoring. Circulation 1993; 87:I89.
- Valvular pulmonic stenosis
- - Associated conditions
- Subvalvular pulmonic stenosis
- Supravalvular pulmonic stenosis
- CLINICAL MANIFESTATIONS
- ADDITIONAL TESTS
- Cardiac catheterization
- Cardiac magnetic resonance imaging
- Infective endocarditis
- SUMMARY AND RECOMMENDATIONS