History

Fact Explanation
Onset Narrowing of the pulmonary valve is usually present at birth (congenital). and it is a condition developed during the fetal life. The cause is unknown, but genetics may play a role. It may be isolated pulmonary stenosis or combined with the other heart lesions[1].
Asymtomatic Some people( specially children) who are having pulmonary stenosis are totally asymptomatic and leads a normal life[1]
Shortness of breath on exertion In pulmonary stenosis, there is an obstruction to the blood flow from the right ventricle to the pulmonary artery due to narrowing of pulmonary valve. Areas of narrowing would be below the valve, above the valve or pulmonary valve itself which is the commonest.[3] There is thickening and fusion of the valvular commissures. There are 3 types of pulmonary stenosis, the typical dome-shaped pulmonary valve is characterised by a narrow central opening with a preserved, mobile valve, the dysplastic pulmonary valve is less common, the unicuspid or bicuspid pulmonary valve is generally a feature of tetralogy of Fallot[3]. Lowered cardiac indices before and after exercise in adults with severe pulmonary stenosis. can cause shortness of breath.
Fatigue and faintishness When the pulmonary valve is obstructed, the right ventricle must work harder to eject blood into the pulmonary artery. To compensate for this additional workload, the muscle of the right ventricle gradually thickens.[3] The thicker hypertrophied right ventricular muscle, then become weak, leading to reduced cardiac output and systemic circulation. [6]
Features of right heart failure-Ankle swelling, sacral oedema, abdominal sweling These are features of right heart failure. Pumping of the right ventricle against increased pressure causes the muscular wall of the ventricle to thicken and right ventricular hypertrophy. Transition of hypertrophy to heart failure occurs by fibrosis and myocyte degeneration. Cell loss, mainly by autophagy and oncosis, contributes significantly to the progression of left ventricular systolic dysfunction(11). Impaired right ventricular function can lead to right ventricular failure[6]
Palpitations Atrial fibrillation can presents with palpitations. [5,7]
Chest pain Coronary artery disease and markedly enlarged pulmonary artery aneurysms may rarely cause chest pain by compression of the left main coronary artery. [2]
Risk factors:-History of atrial fibrillation Pulmonary vein stenosis is a late complication of radiofrequency ablation for atrial fibrillation, frequently asymptomatic and it can be associated with severe respiratory symptoms that cause significant morbidity[7].
Risk factors:-History of rubella in mother during antenatl period Babies born to mothers who had rubella (German measles) during pregnancy were more likely to develop pulmonary stenosis along with deafness and patent ductus arteriosus[7].
Risk factors:-Rheumatic fever This is a complication of an infection caused by streptococcus pyogens, and patient may have a history of sore throat, skin sepsis followed by fever. Rheumatic fever may injure the heart valves causing stenosis[9].
Infective endocarditis may present with pyrexia People with structural heart problems, such as pulmonary stenosis, have a higher risk of developing infections in infectious endocarditis. The pulmonary valve is the least commonly involved valve in infective endocarditis, and isolated pulmonary valve endocarditis is extremely rare. [10]
Risk factors:-Family history Pulmonary valve stenosis is a rare disorder. There may be a family history of pulmonary valve stenosis on certain situations. [1]
References
  1. ARAIN NI, MOLLER JH, PYLES LA, SIVANANDAM S. "Vanishing" pulmonary valve stenosis Ann Pediatr Cardiol [online] 2012, 5(1):47-50 [viewed 18 June 2014] Available from: doi:10.4103/0974-2069.93711
  2. WARNES C. A., WILLIAMS R. G., BASHORE T. M., CHILD J. S., CONNOLLY H. M., DEARANI J. A., DEL NIDO P., FASULES J. W., GRAHAM T. P., HIJAZI Z. M., HUNT S. A., KING M. E., LANDZBERG M. J., MINER P. D., RADFORD M. J., WALSH E. P., WEBB G. D.. 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): Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation [online] 2008 December, 118(23):e714-e833 [viewed 17 June 2014] Available from: doi:10.1161/CIRCULATIONAHA.108.190690
  3. NISHIMURA RA, PIERONI DR, BIERMAN FZ, COLAN SD, KAUFMAN S, SANDERS SP, SEWARD JB, TAJIK AJ, WIGGINS JW, ZAHKA KG. Second natural history study of congenital heart defects. Pulmonary stenosis: echocardiography. Circulation [online] 1993 Feb, 87(2 Suppl):I73-9 [viewed 18 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/8425325
  4. JESSUP M, BROZENA S. Heart failure. N Engl J Med [online] 2003 May 15, 348(20):2007-18 [viewed 18 June 2014] Available from: doi:10.1056/NEJMra021498
  5. PACKER DL, KEELAN P, MUNGER TM, BREEN JF, ASIRVATHAM S, PETERSON LA, MONAHAN KH, HAUSER MF, CHANDRASEKARAN K, SINAK LJ, HOLMES DR JR. Clinical presentation, investigation, and management of pulmonary vein stenosis complicating ablation for atrial fibrillation. Circulation [online] 2005 Feb 8, 111(5):546-54 [viewed 17 June 2014] Available from: doi:10.1161/01.CIR.0000154541.58478.36
  6. GORDON K, CHILD A. Systems and diseases. The heart, Part Ten. Heart failure-2. Nurs Times [online] 2000 Apr 20-26, 96(16):49-52 [viewed 18 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/11309947
  7. BEDOGNI F, BRAMBILLA N, LAUDISA ML, SALVADè P, CARMINATI M, MANTICA M, TONDO C. Acquired pulmonary vein stenosis after radiofrequency ablation treated by angioplasty and stent implantation. J Cardiovasc Med (Hagerstown) [online] 2007 Aug, 8(8):618-24 [viewed 17 June 2014] Available from: doi:10.2459/01.JCM.0000281696.08242.ac
  8. VENABLES AW. THE SYNDROME OF PULMONARY STENOSIS COMPLICATING MATERNAL RUBELLA Br Heart J [online] 1965 Jan, 27(1):49-55 [viewed 18 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC490133
  9. VASAN RS, SHRIVASTAVA S, VIJAYAKUMAR M, NARANG R, LISTER BC, NARULA J. Echocardiographic evaluation of patients with acute rheumatic fever and rheumatic carditis. Circulation [online] 1996 Jul 1, 94(1):73-82 [viewed 18 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/8964121
  10. HATEMI AC, GURSOY M, TONGUT A, BICAKHAN B, GUZELTAS A, CETIN G, KANSIZ E. Pulmonary Stenosis as a Predisposing Factor for Infective Endocarditis in a Patient with Noonan Syndrome Tex Heart Inst J [online] 2010, 37(1):99-101 [viewed 18 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2829787
  11. HEIN S, ARNON E, KOSTIN S, SCHöNBURG M, ELSäSSER A, POLYAKOVA V, BAUER EP, KLöVEKORN WP, SCHAPER J. Progression from compensated hypertrophy to failure in the pressure-overloaded human heart: structural deterioration and compensatory mechanisms. Circulation [online] 2003 Feb 25, 107(7):984-91 [viewed 18 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/12600911

Examination

Fact Explanation
Dysmorphic features Pulmonary stenosis is the most common cardiac anomaly in Noonan patients. Features of noonan's syndrome include congenital heart defect (typically pulmonary valve stenosis) also ASD, hypertrophic cardiomyopathy, short stature, learning problems, pectus excavatum, and a characteristic configuration of facial features including a webbed neck and a flat nose bridge[1].
Cyanosis When there is a severe obstruction in the pulmonary valve, right ventricle can not eject sufficient blood to the pulmonary artery, oxygen saturation can not be maintained at normal level. There can be associated ASD or a substantial increase in right atrial pressure and right-to-left shunting through the foramen ovale, causing blue discoloration of the body due to lower oxygen levels[2].
Irregular pulse Arrhythmias can exist. Patient can present with atrial fibrillation and other tachyarrhythmias[3].
Hypertension There can be associated hypertension as a comorbid factor[2].
Features of right heart failure: Ankle oedema, sacral oedema, ascites, hepatomegally, elevated jugular venous pressure Pumping of the right ventricle against increased pressure causes thickening of the muscular wall and right ventricular hypertrophy. Eventually, the heart becomes stiff and may become weakened causing right heart failure[4].
Shifted cardiac apex There can be enlargement of heart causing cardiomegally in severe situations. There can be latera or downward displacement of the apex [4,5]
Features of pulmonary hypertension:-Loud second heart sound, wide split second heart sound This is due to associated peripheral pulmonary artery stenosis. Patients who had a systemic–to–pulmonary artery shunt as a child may have branch pulmonary artery stenosis at the site of the anastomosis[7].
Murmur There can be ejection click or a ejection systolic murmur, which is best radiates to the left shoulder. In severe pulmonary stenosis, the murmur become longer and second heart sound will become inaudible [6].
Early diastolic decrescendo in pulmonary regurgitation Pulmonary regurgitation is due to the leakage of blood back into the right ventricle after repair. There will be early diastolic decrescendo murmur at the left sternal edge and a Graham Steel murmur if associated with pulmonary hypertension and mitral stenosis[8].
References
  1. HATEMI AC, GURSOY M, TONGUT A, BICAKHAN B, GUZELTAS A, CETIN G, KANSIZ E. Pulmonary Stenosis as a Predisposing Factor for Infective Endocarditis in a Patient with Noonan Syndrome Tex Heart Inst J [online] 2010, 37(1):99-101 [viewed 18 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2829787
  2. WARNES C. A., WILLIAMS R. G., BASHORE T. M., CHILD J. S., CONNOLLY H. M., DEARANI J. A., DEL NIDO P., FASULES J. W., GRAHAM T. P., HIJAZI Z. M., HUNT S. A., KING M. E., LANDZBERG M. J., MINER P. D., RADFORD M. J., WALSH E. P., WEBB G. D.. 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): Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation [online] 2008 December, 118(23):e714-e833 [viewed 17 June 2014] Available from: doi:10.1161/CIRCULATIONAHA.108.190690
  3. 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 [online] 1993 Feb, 87(2 Suppl):I89-101 [viewed 18 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/8425327
  4. NISHIMURA RA, PIERONI DR, BIERMAN FZ, COLAN SD, KAUFMAN S, SANDERS SP, SEWARD JB, TAJIK AJ, WIGGINS JW, ZAHKA KG. Second natural history study of congenital heart defects. Pulmonary stenosis: echocardiography. Circulation [online] 1993 Feb, 87(2 Suppl):I73-9 [viewed 18 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/8425325
  5. JESSUP M, BROZENA S. Heart failure. N Engl J Med [online] 2003 May 15, 348(20):2007-18 [viewed 18 June 2014] Available from: doi:10.1056/NEJMra021498
  6. BONOW RO, CARABELLO B, DE LEON AC JR, EDMUNDS LH JR, FEDDERLY BJ, FREED MD, GAASCH WH, MCKAY CR, NISHIMURA RA, O'GARA PT, O'ROURKE RA, RAHIMTOOLA SH, RITCHIE JL, CHEITLIN MD, EAGLE KA, GARDNER TJ, GARSON A JR, GIBBONS RJ, RUSSELL RO, RYAN TJ, SMITH SC JR. Guidelines for the management of patients with valvular heart disease: executive summary. A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee on Management of Patients with Valvular Heart Disease). Circulation [online] 1998 Nov 3, 98(18):1949-84 [viewed 18 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/9799219
  7. SMITH WG. Pulmonary Hypertension and a Continuous Murmur Due to Multiple Peripheral Stenoses of the Pulmonary Arteries Thorax [online] 1958 Sep, 13(3):194-200 [viewed 18 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1018437
  8. YOO BW, PARK HK. Pulmonary stenosis and pulmonary regurgitation: both ends of the spectrum in residual hemodynamic impairment after tetralogy of Fallot repair. Korean J Pediatr [online] 2013 Jun, 56(6):235-41 [viewed 18 June 2014] Available from: doi:10.3345/kjp.2013.56.6.235

Differential Diagnoses

Fact Explanation
Atrial septal defect On auscultation, systolic outflow tract murmur, wide and fixed splitting of S2, but there is no ejection click as in pulmonary stenosis[1,7].
Aortic valve stenosis On auscultation, ejection systolic murmur in upper right sternal border, radiating into carotids[2].
Innocent murmur Usually patient is asymptomatic.On auscultation, soft systolic murmur, usually shorter than with pulmonary stenosis and no ejection click[3].
Tetralogy of fallot This condition is comprised of 4 elements. Overriding aorta, right ventricular hypertrophy, infundibular stenosis and ventricular septal defect. Patients usually present as neonates, with cyanosis of varying intensity. The aetiology is multifactorial, but there is an association with untreated maternal diabetes, phenylketonuria, and intake of retinoic acid. Cyanosis more prominent than in pulmonary stenosis and no ejection click[5,6].
Right bundle branch block This shows rSR pattern on ECG[4].
References
  1. WARNES C. A., WILLIAMS R. G., BASHORE T. M., CHILD J. S., CONNOLLY H. M., DEARANI J. A., DEL NIDO P., FASULES J. W., GRAHAM T. P., HIJAZI Z. M., HUNT S. A., KING M. E., LANDZBERG M. J., MINER P. D., RADFORD M. J., WALSH E. P., WEBB G. D.. 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): Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation [online] 2008 December, 118(23):e714-e833 [viewed 17 June 2014] Available from: doi:10.1161/CIRCULATIONAHA.108.190690
  2. RAJAMANNAN NM, BONOW RO, RAHIMTOOLA SH. Calcific aortic stenosis: an update. Nat Clin Pract Cardiovasc Med [online] 2007 May, 4(5):254-62 [viewed 17 June 2014] Available from: doi:10.1038/ncpcardio0827
  3. SMITH KM. The innocent heart murmur in children. J Pediatr Health Care [online] 1997 Sep-Oct, 11(5):207-14 [viewed 17 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/9369637
  4. SCHNEIDER JF, THOMAS HE, KREGER BE, MCNAMARA PM, SORLIE P, KANNEL WB. Newly acquired right bundle-branch block: The Framingham Study. Ann Intern Med [online] 1980 Jan, 92(1):37-44 [viewed 17 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/7350871
  5. BAILLIARD F, ANDERSON RH. Tetralogy of Fallot. Orphanet J Rare Dis [online] 2009 Jan 13:2 [viewed 17 June 2014] Available from: doi:10.1186/1750-1172-4-2
  6. PODDAR B, BASU S. Approach to a child with a heart murmur. Indian J Pediatr [online] 2004 Jan, 71(1):63-6 [viewed 18 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/14979389
  7. ADVANI N, MENAHEM S, WILKINSON JL. The diagnosis of innocent murmurs in childhood. Cardiol Young [online] 2000 Oct, 10(4):340-2 [viewed 18 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/10950330

Investigations - for Diagnosis

Fact Explanation
Echocardiogram This investigation demonstrates the pulmonary valvular stenosis, condition of the other valves and associated valvular lesions and any septal defects such as atrial septal defect or ventricular septal defect. This may be normal in the presence of mild pulmonary stenosis. With moderate-to-severe pulmonary stenosis there will be enlargement of the right ventricle and thickening of its muscle[4,5].
Electrocardiogram There will be features of severe obstruction such as right atrial enlargement, right-axis deviation, and right ventricular hypertrophy[3]. T inversions and ST depressions may be there extending to the chest lead V6[3,5].
Chest X-ray In isolated pulmonary stenosis, the hypertrophy may be hidden within the cardiac shadow and of the heart is very small. So, when there is a visible enlargement of the cardiac shadow, it has to be taken seriously. The heart size on chest x-ray is normal unless there is an associated cardiac lesion. Vascular fullness in the left lung base greater than the right base (Chen’s sign), dilatation of the main pulmonary artery are some of the findings. Calcification of the valve may rarely be seen in older patients. Right atrium may also be enlarged[4].
Cardiac catheterization Cardiac catheterization is rarely necessary for diagnosis. Cardiac catheterization is a test used to measure pressure and blood flow in the heart's chambers and in the great vessels, examine the arteries of the heart, perform a biopsy on the heart muscle repair the certain types of heart defects, open a narrowed (stenotic) heart valve and angioplasty with or without stenting. A peak right ventricular systolic pressure of less than 35 mm Hg and a systolic pulmonary valve gradient of less than 10 mmHg are considered the upper limits of normal. Angiography helps define contractile function, the presence of infundibular obstruction, and mobility of the pulmonary valve[2].
MRI and CT scan Magnetic resonance imaging and CT scans are sometimes used to confirm the diagnosis of pulmonary valve stenosis. These studies do provide excellent imaging of the main, branch, and peripheral pulmonary arteries and are useful when there is associated lesions such as pulmonary regurgitation or tricuspid regurgitation[4].
Angiography Angiography of the pulmonary artery can assess the degree of pulmonary regurgitation and any stenotic lesions in the main, branch, or peripheral pulmonary arteries[4].
References
  1. PACKER DL, KEELAN P, MUNGER TM, BREEN JF, ASIRVATHAM S, PETERSON LA, MONAHAN KH, HAUSER MF, CHANDRASEKARAN K, SINAK LJ, HOLMES DR JR. Clinical presentation, investigation, and management of pulmonary vein stenosis complicating ablation for atrial fibrillation. Circulation [online] 2005 Feb 8, 111(5):546-54 [viewed 16 June 2014] Available from: doi:10.1161/01.CIR.0000154541.58478.36
  2. BROCK SR. THE SURGICAL TREATMENT OF PULMONARY STENOSIS Br Heart J [online] 1961 Jul, 23(4):337-356 [viewed 17 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1017778
  3. MUNGER TM, WU LQ, SHEN WK. Atrial fibrillation. J Biomed Res [online] 2014 Jan, 28(1):1-17 [viewed 17 June 2014] Available from: doi:10.7555/JBR.28.20130191
  4. WARNES C. A., WILLIAMS R. G., BASHORE T. M., CHILD J. S., CONNOLLY H. M., DEARANI J. A., DEL NIDO P., FASULES J. W., GRAHAM T. P., HIJAZI Z. M., HUNT S. A., KING M. E., LANDZBERG M. J., MINER P. D., RADFORD M. J., WALSH E. P., WEBB G. D.. 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): Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation [online] 2008 December, 118(23):e714-e833 [viewed 17 June 2014] Available from: doi:10.1161/CIRCULATIONAHA.108.190690
  5. BIRKEBAEK NH, HANSEN LK, OXHøJ H. Diagnostic value of chest radiography and electrocardiography in the evaluation of asymptomatic children with a cardiac murmur. Acta Paediatr [online] 1995 Dec, 84(12):1379-81 [viewed 18 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/8645955

Investigations - Fitness for Management

Fact Explanation
Full blood count Anaemia (low haemoglobin) needs correction as it may be an aggravating factor for heart failure[3]. There may be features of associated complications such as infective endocarditis (Neutrophil leucocytosis, low platelets).
Doppler studies Doppler studies are done to see the relationship between the peak-to-peak invasive haemodynamic gradient and the doppler peak instantaneous gradient as they are important in evaluating the patient for invasive interventions[1,2]
References
  1. WARNES C. A., WILLIAMS R. G., BASHORE T. M., CHILD J. S., CONNOLLY H. M., DEARANI J. A., DEL NIDO P., FASULES J. W., GRAHAM T. P., HIJAZI Z. M., HUNT S. A., KING M. E., LANDZBERG M. J., MINER P. D., RADFORD M. J., WALSH E. P., WEBB G. D.. 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): Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation [online] 2008 December, 118(23):e714-e833 [viewed 17 June 2014] Available from: doi:10.1161/CIRCULATIONAHA.108.190690
  2. PACKER DL, KEELAN P, MUNGER TM, BREEN JF, ASIRVATHAM S, PETERSON LA, MONAHAN KH, HAUSER MF, CHANDRASEKARAN K, SINAK LJ, HOLMES DR JR. Clinical presentation, investigation, and management of pulmonary vein stenosis complicating ablation for atrial fibrillation. Circulation [online] 2005 Feb 8, 111(5):546-54 [viewed 17 June 2014] Available from: doi:10.1161/01.CIR.0000154541.58478.36
  3. GORDON K, CHILD A. Systems and diseases. The heart, Part Ten. Heart failure-2. Nurs Times [online] 2000 Apr 20-26, 96(16):49-52 [viewed 18 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/11309947

Investigations - Followup

Fact Explanation
Echocardiogram and doppler studies These patients after balloon valvotomy, should be followed up for the development of pulmonary regurgitation and re-stenosis [3]. Doppler studies and echocardiographic evaluation is important during this follow up. Degree of pulmonary regurgitation right ventricular pressure, size, and function and tricuspid regurgitation are assessed by echocardiography[1,2]. Repeat balloon dilatation has to be performed in some patients and surgical intervention is needed for subvalvular or supravalvular stenosis in small number of patients. Age, balloon to annulus ratio, and valve anatomy were not significant predictors for the late development of moderate and severe valve regurgitation[4,5].
Electrocardiogram There will be regression of right ventricular hypertrophy( on ECG after balloon dilatation), However, evidence of hemodynamic improvement in ECG does not become apparent until 6 months after valvuloplasty. features of right ventricular hypertrophy may be reversed, but sometimes features of right ventricular strain such as T inversions may be not settling[5].
Chest X-ray After pulmonary valvuloplasty the heart size should be normal on chest x-ray. A progressively increasing heart size should prompt the search for pulmonary regurgitation or another lesion. The development of atrial arrhythmias should also prompt a search for residual hemodynamic lesions such as pulmonary regurgitation[5].
References
  1. RAO PS, GALAL O, PATNANA M, BUCK SH, WILSON AD. Results of three to 10 year follow up of balloon dilatation of the pulmonary valve. Heart [online] 1998 Dec, 80(6):591-5 [viewed 17 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/10065029
  2. MCCRINDLE BW. Independent predictors of long-term results after balloon pulmonary valvuloplasty. Valvuloplasty and Angioplasty of Congenital Anomalies (VACA) Registry Investigators. Circulation [online] 1994 Apr, 89(4):1751-9 [viewed 17 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/8149541
  3. AL BALUSHI AY, AL SHUAILI H, AL KHABORI M, AL MASKRI S. Pulmonary valve regurgitation following balloon valvuloplasty for pulmonary valve stenosis: Single center experience. Ann Pediatr Cardiol [online] 2013 Jul, 6(2):141-4 [viewed 17 June 2014] Available from: doi:10.4103/0974-2069.115258
  4. BROCK SR. THE SURGICAL TREATMENT OF PULMONARY STENOSIS Br Heart J [online] 1961 Jul, 23(4):337-356 [viewed 17 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1017778
  5. WARNES C. A., WILLIAMS R. G., BASHORE T. M., CHILD J. S., CONNOLLY H. M., DEARANI J. A., DEL NIDO P., FASULES J. W., GRAHAM T. P., HIJAZI Z. M., HUNT S. A., KING M. E., LANDZBERG M. J., MINER P. D., RADFORD M. J., WALSH E. P., WEBB G. D.. 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): Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation [online] 2008 December, 118(23):e714-e833 [viewed 17 June 2014] Available from: doi:10.1161/CIRCULATIONAHA.108.190690

Investigations - Screening/Staging

Fact Explanation
Cardiac magnetic resonance (CMR) The excellent visualisation of the RV outflow tract with CMR facilitates easy identification of the site and severity of pulmonary stenosis[1].
References
  1. MYERSON SAUL G. Heart valve disease: investigation by cardiovascular magnetic resonance. Array [online] 2012 December [viewed 18 June 2014] Available from: doi:10.1186/1532-429X-14-7

Management - General Measures

Fact Explanation
Physical activities If the patients have only mild-to-moderate PS, there is no need for limit the physical activities. If it's severe, need to limit activity until the valve is dilated or replaced as the there is a risk of arrhythmias. Patient should get advice from the doctor before any exercising programme[1].
Medical follow-up Medical follow-up is important to check for the symptoms or problems related to. In mild or moderate, patient need ongoing follow-up.Also need opinion from a Cardiologist before any surgery or invasive procedure[1].
Prevention of endocarditis No need of taking antibiotics to prevent endocarditis unless the patient had endocarditis in the past or had valve replacement with a prosthetic material. People who had valve replacement need preventive antibiotics before dental and other procedures[2].
Pregnancy Pregnancy will be well tolerated in mild or moderate PS.But in severe PS, it is considered as a high risk. In these cases cardiologist's opinion should be taken during the pregnancy and delivery[1].
Diet Balanced healthy diet is necessary for these people. A diet that is low in sodium, refine sugar, cholesterol and saturated fat is important to reduce the cardiovascular risk factors. Adding fruits ,vegetables , fish and whole grain is beneficial[1].
Quitting smoking Smoking is a significant risk factor for heart disease[1].
References
  1. WARNES C. A., WILLIAMS R. G., BASHORE T. M., CHILD J. S., CONNOLLY H. M., DEARANI J. A., DEL NIDO P., FASULES J. W., GRAHAM T. P., HIJAZI Z. M., HUNT S. A., KING M. E., LANDZBERG M. J., MINER P. D., RADFORD M. J., WALSH E. P., WEBB G. D.. 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): Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation [online] 2008 December, 118(23):e714-e833 [viewed 17 June 2014] Available from: doi:10.1161/CIRCULATIONAHA.108.190690
  2. BONOW RO, CARABELLO B, DE LEON AC JR, EDMUNDS LH JR, FEDDERLY BJ, FREED MD, GAASCH WH, MCKAY CR, NISHIMURA RA, O'GARA PT, O'ROURKE RA, RAHIMTOOLA SH, RITCHIE JL, CHEITLIN MD, EAGLE KA, GARDNER TJ, GARSON A JR, GIBBONS RJ, RUSSELL RO, RYAN TJ, SMITH SC JR. Guidelines for the management of patients with valvular heart disease: executive summary. A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee on Management of Patients with Valvular Heart Disease). Circulation [online] 1998 Nov 3, 98(18):1949-84 [viewed 18 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/9799219

Management - Specific Treatments

Fact Explanation
Percutaneous Balloon Pulmonary Valvotomy Indicated in moderate to severe pulmonary stenosis with a peak-to-peak gradient >50 mmHg with normal cardiac index. Previous surgery and pulmonary valve dysplasia are not contraindications for balloon valvuloplasty[4,5]. This procedure is also applicable to pulmonary stenosis associated with other complex cardiac defects and stenosis of bioprosthetic valves in pulmonary position. The procedure is usually performed from the right femoral vein. The technique involves positioning one or more balloon catheters across the stenotic valve, usually over an extra-stiff guide wire and inflating the balloons with diluted contrast material, thus producing valvotomy. Balloon valvuloplasty generally reduces the peak systolic pressure gradient by more than 50 per cent. The currently recommended balloon/annulus ratio is 1.2 to 1.25. Predictors of restenosis include balloon/annulus ratio <1.2 and immediate post-valvuloplasty gradient >or=30 mmHg[3].
Surgical Pulmonary Valvotomy or Valve Replacement In patients with pulmonary stenosis and significant valvular regurgitation, valve replacement may be required. Mechanical valve replacement is rarely used because of concerns regarding thrombosis and the potential need for measurement of pulmonary pressures. Mechanical PVR can be considered in selected patients who have had multiple previous operations and are undergoing warfarin therapy because of another mechanical valve prosthesis(6). Percutaneous balloon valvotomy thus appears to be an excellent alternative to surgical valvuloplasty or valve replacement in most patients with classic, doming, valvular pulmonary stenosis. Its use in patients with a dysplastic valve is much less established. Mortality and morbidity rates are generally higher after surgery[2,5,6].
Stenting PVS stenting with stent sizes >or=10 mm seems to be an adequate therapy modality for treatment of severe acquired pulmonary stenosis. Example of one such aquired condition is severe pulmonary vein stenosis after catheter ablation of atrial fibrillation[1].
References
  1. PACKER DL, KEELAN P, MUNGER TM, BREEN JF, ASIRVATHAM S, PETERSON LA, MONAHAN KH, HAUSER MF, CHANDRASEKARAN K, SINAK LJ, HOLMES DR JR. Clinical presentation, investigation, and management of pulmonary vein stenosis complicating ablation for atrial fibrillation. Circulation [online] 2005 Feb 8, 111(5):546-54 [viewed 16 June 2014] Available from: doi:10.1161/01.CIR.0000154541.58478.36
  2. RADTKE W, KEANE JF, FELLOWS KE, LANG P, LOCK JE. Percutaneous balloon valvotomy of congenital pulmonary stenosis using oversized balloons. J Am Coll Cardiol [online] 1986 Oct, 8(4):909-15 [viewed 17 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/3760363
  3. CHEN CR, CHENG TO, HUANG T, ZHOU YL, CHEN JY, HUANG YG, LI HJ. Percutaneous balloon valvuloplasty for pulmonic stenosis in adolescents and adults. N Engl J Med [online] 1996 Jul 4, 335(1):21-5 [viewed 17 June 2014] Available from: doi:10.1056/NEJM199607043350104
  4. RADTKE W, KEANE JF, FELLOWS KE, LANG P, LOCK JE. Percutaneous balloon valvotomy of congenital pulmonary stenosis using oversized balloons. J Am Coll Cardiol [online] 1986 Oct, 8(4):909-15 [viewed 17 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/3760363
  5. BONOW RO, CARABELLO B, DE LEON AC JR, EDMUNDS LH JR, FEDDERLY BJ, FREED MD, GAASCH WH, MCKAY CR, NISHIMURA RA, O'GARA PT, O'ROURKE RA, RAHIMTOOLA SH, RITCHIE JL, CHEITLIN MD, EAGLE KA, GARDNER TJ, GARSON A JR, GIBBONS RJ, RUSSELL RO, RYAN TJ, SMITH SC JR. Guidelines for the management of patients with valvular heart disease: executive summary. A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee on Management of Patients with Valvular Heart Disease). Circulation [online] 1998 Nov 3, 98(18):1949-84 [viewed 18 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/9799219
  6. WARNES C. A., WILLIAMS R. G., BASHORE T. M., CHILD J. S., CONNOLLY H. M., DEARANI J. A., DEL NIDO P., FASULES J. W., GRAHAM T. P., HIJAZI Z. M., HUNT S. A., KING M. E., LANDZBERG M. J., MINER P. D., RADFORD M. J., WALSH E. P., WEBB G. D.. 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): Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation [online] 2008 December, 118(23):e714-e833 [viewed 17 June 2014] Available from: doi:10.1161/CIRCULATIONAHA.108.190690