Pulmonary valve stenosis is responsible for approximately 1-5% of all congenital heart diseases (CHDs) [1] [2], and appears either as an isolated defect or as a constitutive feature of Noonan syndrome, an autosomal dominant genetic disease characterised by hypertrophic cardiomyopathy, atrial septal defect (ASD) and stenosis of the pulmonary valve [1] [3]. The clinical presentation is usually asymptomatic, as stenosis causes minimal obstruction of the pulmonary outflow tract in the majority of patients throughout childhood and adulthood [4] [5]. However, significant obstruction and consequent hypertrophy of the right ventricle can result in volume overload and the development of fatigue, dyspnea, and syncope [4] [5] [6]. Pregnancy is one of the conditions that exerts a higher load on the right ventricle and is associated with a risk of atrial arrhythmias with many women being diagnosed with pulmonary valve stenosis during pregnancy [1] [4]. On the other hand, individuals suffering from Noonan syndrome exhibit numerous congenital abnormalities in addition to CHD, most prominent being short stature, feeding difficulties, failure to thrive, cryptorchidism (either unilateral or bilateral), and a delayed onset of puberty [3].

• Atrial Septal Defect

  Abstract The familial association of pulmonary stenosis, atrial septal defect, and unique electrocardiographic abnormalities involving a mother and two children is reported. [ncbi.nlm.nih.gov]

• Fatigue

  However, significant obstruction and consequent hypertrophy of the right ventricle can result in volume overload and the development of fatigue, dyspnea, and syncope. [symptoma.com]

  Older children with severe pulmonary stenosis are more likely to may have fatigue and/or shortness of breath (see figure Heart Failure: Pumping and Filling Problems). [merckmanuals.com]

  Symptoms may include chest pain, fainting, shortness of breath, fatigue and a bluish tint to the skin (cyanosis). Learn more about a minimally invasive treatment option for pulmonary valve stenosis and regurgitation [emoryhealthcare.org]

  In moderate and severe pulmonary valve stenosis (if the gradient exceeds 80 mm Hg) there is a dyspnea during fatiguing (without heart failure). [remedyland.com]

• Exertional Dyspnea

  He became physically active and was free of exertional dyspnea at the 12-month follow-up. [ncbi.nlm.nih.gov]

  […] end-systolic volume収縮終（末）期容積、期末容積 end-to-end anastomosis端々吻合 end-to-side anastomosis端側吻合 epicardial心外膜　【形】 epicardium心外膜 ergometer exerciseエルゴメータ運動負荷 escaped beat補充収縮 escaped rhythm補充調律 evoked potential誘発電位 excitation-contraction (E-C) coupling興奮収縮連関 exertional [tokyo-med.ac.jp]

• Heart Disease

  Pulmonary valve stenosis is one of the most common types of congenital heart disease and is predominantly asymptomatic throughout childhood and adulthood. [symptoma.com]

  Several forms of congenital heart disease (CHD) are found in about 10% of patients with del (18p), but coexisting PDA and PS have not been reported. [ncbi.nlm.nih.gov]

  Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 11th ed. Philadelphia, PA: Elsevier; 2019:chap 70. Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease in the adult and pediatric patient. [nlm.nih.gov]

  Congenital heart disease in the adult and pediatric patient. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 11th ed. [mountsinai.org]

• Heart Murmur

  A heart murmur is a sound created by turbulent blood flow through narrowed or leaking heart valves or through abnormal heart structures. Echocardiography (ultrasonography of the heart) confirms the diagnosis. [merckmanuals.com]

  Pulmonary stenosis, which is rare among adults, is usually caused by a birth defect, also called a congenital heart defect. Moderate to severe PS is most often diagnosed during childhood due to the loud heart murmur associated with the condition. [heart.org]

  The main sign of heart valve disease is an unusual heartbeat sound called a heart murmur. Your doctor can hear a heart murmur with a stethoscope. But many people have heart murmurs without having a problem. [icdlist.com]

• Chest Pain

  Symptoms may include chest pain, fainting, shortness of breath, fatigue and a bluish tint to the skin (cyanosis). Learn more about a minimally invasive treatment option for pulmonary valve stenosis and regurgitation [emoryhealthcare.org]

  When to see a doctor Talk to your doctor if you or your child has: Shortness of breath Chest pain Fainting If you have pulmonary stenosis or another heart problem, prompt evaluation and treatment can help reduce your risk of complications. [mayoclinic.org]

  If you are experiencing severe shortness of breath or chest pain, seek immediate medical care by calling 911 or going to an emergency room for evaluation. [rush.edu]

  In patients with significant PV stenosis and RTLS through a PFO, clinical features include exertional syncope, chest pain, central cyanosis, erythema of fingers and toes, clubbing of digits, underdevelopment, moon face, a sustained and strong RV impulse [link.springer.com]

• Systolic Murmur

  He was found to have an ejection systolic murmur and evidence of right ventricular outflow tract obstruction, with a peak velocity of 4.5 m/s recorded by transthoracic Doppler echocardiography. [ncbi.nlm.nih.gov]

  Functional or physiologic PPS is a common cause of a systolic murmur in infants. It occurs in both premature and full-term infants; with time, the pulmonary artery grows, and the murmur usually disappears within a few months. [emedicine.medscape.com]

  Ejection systolic murmur along the left upper edge of the sternum. Pulmonary ejection click. Delayed second heart sound with severe stenosis. Parasternal thrill and heave. 'A waves' in the JVP. [patient.info]

• Mitral Valve Prolapse

  If they don't, you could have Regurgitation - when blood leaks back through the valve in the wrong direction Mitral valve prolapse - when one of the valves, the mitral valve, has "floppy" flaps and doesn't close tightly. [icdlist.com]

  Valve Disease 425 Mitral Valve Prolapse 427 Anomalous Origin of the Left Coronary Artery 429 Cardiomyopathy 433 Infective Endocarditis 439 Myocarditis 444 Rheumatic Heart Disease 447 Aortic Arch and Pulmonary Artery Abnormalities Vascular Rings and Slings [books.google.com]

  Mitral valve prolapse (MVP) is a very common condition, affecting 1% to 2% of the population. MVP causes the leaflets of the mitral valve to flop back into the left atrium during the heart's contraction. [webmd.com]

  […] regurgitation (MR)僧帽弁逆流［症］ mitral stenosis (MS) 僧帽弁狭窄［症］ mitral valve 僧帽弁 mitral valve area僧帽弁口面積 mitral valve prolapse (MVP)僧帽弁逸脱［症］ mitral valve replacement僧帽弁置換術 Mobitz type I blockモビッツI型ブロック Mobitz type II blockモビッツII型ブロック moist rale湿性ラ音 mucocutaneous [tokyo-med.ac.jp]

• Cryptorchidism

  On the other hand, individuals suffering from Noonan syndrome exhibit numerous congenital abnormalities in addition to CHD, most prominent being short stature, feeding difficulties, failure to thrive, cryptorchidism (either unilateral or bilateral), and [symptoma.com]

• Dizziness

  Among some of the symptoms consistent with pulmonary valve stenosis are the following: Heart murmur Cyanosis Dyspnea Dizziness Upper thorax pain Developmental disorders In regards to the cause of pulmonary valve stenosis a very high percentage are congenital [en.wikipedia.org]

  If the condition is moderate to severe, then you may have following signs and symptoms: heart murmur prominent and enlarged jugular vein bluish discoloration of the skin chest pain dizziness heart palpitations unexplained fatigue failure to thrive shortness [gleneaglesglobalhospitals.com]

  However, when symptoms do develop, many of them are similar to those associated with heart failure: Shortness of breath, especially with exercise Palpitations Chest pain (may be mild) Fatigue Dizziness or fainting Bluish nails, lips or skin Heart murmur [houstonmethodist.org]

  Children past infancy may also have these symptoms: Feeling short of breath when active Having pain, pressure or tightness in their chest Fainting or feeling weak or dizzy when active Being more tired than normal Diagnosing Pulmonary Stenosis To diagnose [seattlechildrens.org]

  Dizziness, pain in the chest and the right ventricular hypertrophy occurs in severe cases and it is accompanied by edema, increased dyspnea and fatigue. [remedyland.com]

The diagnosis of a pulmonary valve stenosis can be made by performing a thorough clinical examination, with an emphasis on cardiac auscultation. Common findings are widening of the second heart sound (S2), the presence of a fourth heart sound (S4), a harsh mid-systolic ejection murmur (also known as crescendo-decrescendo) heard at the 2nd left parasternal intercostal space that may be accompanied by a thrill, as well as increased jugular venous pressure [4] [5] [6]. Patient history is also an integral part of the workup, as information regarding the onset and course of symptoms are highly useful for further evaluation. Electrocardiography (ECG) is usually the first method used in assessment of cardiac disease, but the cornerstone in diagnosing pulmonary valve stenosis is cardiac ultrasonography and Doppler echocardiography, which directly determines the severity of obstruction by measuring the peak gradient flow (< 36 mmHg is considered as mild obstruction, 36-64 mmHg as moderate and > 64 mmHg as severe) [5] [6] [7]. Where available and when necessary, cardiac magnetic resonance (CMR) imaging is recommended in order to obtain a better view of the right ventricle and measure its size and function, while cardiac catheterization is indicated when inconclusive results are obtained, or when both supravalvular and subvalvular obstruction is concomitantly present [6] [7]. Plain radiography usually reveals a normal heart shadow, but a reduced visibility of the pulmonary vasculature is often seen in severe stenosis [5].

• Prominent A-Wave

  ECG of pulmonary valve stenosis Electrical axis deviation to right or right ventricular hypertrophy, prominent P wave. With cardiac catheterization is possible to determine the pressure gradient as the tip of the catheter pass pulmonary valves. [remedyland.com]

  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. [emedicine.medscape.com]

• Right Atrial Hypertrophy

  ECG: may show right ventricular hypertrophy, right atrial hypertrophy and right axis deviation. Angiography: may be required in children with multiple cardiac abnormalities. [patient.info]

After starting phentolamine or captopril treatment, prostaglandin could be discontinued after a mean time of 15.86 hours. [ncbi.nlm.nih.gov]

The prognosis is very poor. The survival time after resection varies from several months to several years depending on the presence of recurrence or metastasis. [ncbi.nlm.nih.gov]

Prognosis Pulmonary regurgitation is usually well tolerated in childhood. [patient.info]

Congenital etiologies cause an abnormal development of distal bulbus cordis secondary to: Rubella embryopathy (most important etiology) Isolated branch pulmonary artery stenosis (stenosis of a sling of the ductus arteriosus) Congenital/genetic syndromes [unboundmedicine.com]

Abstract Congenital heart disease (CHD) is the most common birth defect in humans, and the etiology of most CHD remains to be elusive. Atrial septal defect (ASD) makes up 30–40% of all adult CHDs and is thought to be genetically heterogeneous. [ncbi.nlm.nih.gov]

Specific features depend on the etiology, which affects the level at which the obstruction to right ventricular outflow occurs: valvular pulmonic stenosis most common cause level of obstruction thickened and/or structurally abnormal valve leaflets leaflets [radiopaedia.org]

(Etiology) Pulmonary Valve Stenosis is a narrowing of the pulmonary valve which occurs at birth. The exact cause of the condition is unknown. [dovemed.com]

The epidemiology of pulmonary valve stenosis can be summed up by the congenital aspect which is the majority of cases, in broad terms PVS is rare in the general population. [en.wikipedia.org]

Explore these free sample topics: -- The first section of this topic is shown below -- Basics Description Deformity of pulmonary valve, resulting in obstruction of right ventricular (RV) outflow Epidemiology Incidence Predominant age: congenital, present [unboundmedicine.com]

Both pulmonary stenosis (PS) and pulmonary regurgitation are often well tolerated for long periods but the overload of the right ventricle has a progressively detrimental effect on right ventricular function. [ 1 ] Epidemiology Pulmonary valve disease [patient.info]

As this process worsens, the asymptomatic adult becomes gradually symptomatic. [11, 12] Epidemiology Occurrence in the United States Approximately 5 out of 1000 infants are born with a congenital cardiac malformation. [5] Cardiac malformation is the most [emedicine.com]

The pathophysiology of pulmonary valve stenosis consists of the valve leaflets becoming too thick (therefore not separate one from another), which can cause high pulmonary pressure, and pulmonary hypertension. [en.wikipedia.org]

With the development of potential new treatments, it is crucial for cardiac physicians to be well informed on the pathophysiology, assessment, treatment options and their outcomes of valvular diseases. [books.google.com]

[…] male ( 1 ) African Americans (46.5%), Hispanics (33.1%), and Caucasians (20.4%) ( 1 ) Prevalence 10% of all cases of congenital heart disease In association with other lesions, may be as high as 25–30% of congenital heart disease ( 1 ) Etiology and Pathophysiology [unboundmedicine.com]

Pathophysiology The right ventricle - work harder It fails to pump forward efficiently. Pressure builds up in the right atrium Fluid retention and swelling may occur. There is a higher than average chance of developing an infection in the lining [slideshare.net]

When the two conditions are present simultaneously, significant left-to-right shunt is often prevented by the outflow obstruction, which protects the pulmonary bed until adulthood. [ncbi.nlm.nih.gov]

Prevention : Most of the time, pulmonary stenosis can't be prevented because it's typically present at birth or arises later as the result of another condition. [knowyourdisease.com]

It can help to prevent an infection of the valve. Not everyone with valve stenosis will need this step. [winchesterhospital.org]

The pulmonary valve functions as a one-way valve that allows blood to move from the right ventricle (pumping chamber) into the artery to the lungs and prevents blood from leaking back into the right ventricle. [childrenshospital.org]

1. Harris IS. Management of Pregnancy in Patients with Congenital Heart Disease. Prog Cardiovasc Dis. 2011;53(4):305-311.
2. Todros T, Paladini D, Chiappa E, et al. Pulmonary stenosis and atresia with intact ventricular septum during prenatal life. Ultrasound Obstet Gynecol. 2003;21(3):228-233.
3. Roberts AE, Allanson JE, Tartaglia M, Gelb BD. Noonan syndrome. Lancet. 2013;381(9863):333-342.
4. Ayad RF, Johnston SB, Grayburn PA, Schmidt TT, Choi JW. Congenital pulmonic stenosis in a 77-year-old woman successfully treated with percutaneous balloon valvuloplasty. Proc (Bayl Univ Med Cent). 2010;23(1):21-23.
5. Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson J, Loscalzo J. eds. Harrison's Principles of Internal Medicine, 18e. New York, NY: McGraw-Hill; 2012.
6. Porter RS, Kaplan JL. Merck Manual of Diagnosis and Therapy. 19th Edition. Merck Sharp & Dohme Corp. Whitehouse Station, N.J; 2011.
7. Davlouros PA, Niwa K, Webb G, Gatzoulis MA. The right ventricle in congenital heart disease. Heart. 2006;92(Suppl 1):i27-i38.