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]

  Atrial septal defects An atrial septal defect (ASD) is where there's a hole between the two collecting chambers of the heart (the left and right atria). [nhs.uk]

• 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]

  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]

  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). [msdmanuals.com]

  […] usually have symptoms shortly after birth, which may include: cyanosis (bluish tinge to the skin) lack of energy pale, cool or clammy skin In rare cases, older children can have symptoms that include: feeling unable to exercise heavy and rapid breathing fatigue [childrenshospital.org]

• Congestive Heart Failure

  II included 22 patients with severe pulmonary valve stenosis and congestive right heart failure and/or central cyanosis. [ncbi.nlm.nih.gov]

  Eventually, the heart becomes stiff and may become weakened. Congestive heart failure. If the right ventricle becomes weak and unable to pump efficiently, congestive heart failure develops. [knowyourdisease.com]

  If pulmonary stenosis is severe, congestive heart failure occurs, and systemic venous engorgement will be noted. [en.wikipedia.org]

  […] pulmonic valvular stenosis, with pressure gradient greater than 50 mm Hg is more often associated with decreased cardiac output, right ventricular hypertrophy, early congestive heart failure (CHF), and cyanosis. [emedicine.com]

• Dentist

  Children do not need to take antibiotics before visits to the dentist or surgeries. The following are some English-language resources that may be useful. Please note that THE MANUAL is not responsible for the content of these resources. [msdmanuals.com]

  Discuss with your dentist or doctor whether the dosage of Coumain (warfarin) needs to be decreased or stopped before any of the above procedures. [hopkinsmedicine.org]

  See your dentist for regular visits. [webmd.com]

  Talk to your doctor and dentist about antibiotics before any dental procedures. It can help to prevent an infection of the valve. Not everyone with valve stenosis will need this step. [health.cvs.com]

• Severe Pain

  But you shouldn’t feel severe pain. Pain medicine is available if needed. You may need to lie flat for several hours after the procedure without bending your legs. This will help prevent bleeding. [hopkinsmedicine.org]

• 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]

• Hepatomegaly

   heavy or rapid breathing shortness of breath Fatigue,fainting rapid heart rate swelling in the feet, face, eyelids, and/or abdomen Cyanosis in terminal stages Severe cases: Distended jugular veins, hepatomegaly, ascites 8.  How is pulmonary [slideshare.net]

  Similarly, no patient had signs of systemic venous congestion such as hepatomegaly, ascites, or peripheral edema. [revespcardiol.org]

• Heart Disease

  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]

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

  Guidelines on the management of valvular heart disease: The Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology. Eur Heart J. 2007 Jan. 28(2):230-68. [Medline]. [emedicine.medscape.com]

  Offering a broad perspective on these diseases, Valvular Heart Disease expands on the recent guidelines developed by the major heart societies in the United State and Europe. [books.google.com]

• 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. [msdmanuals.com]

  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]

  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]

• Heart Failure

  II included 22 patients with severe pulmonary valve stenosis and congestive right heart failure and/or central cyanosis. [ncbi.nlm.nih.gov]

  In most children, the only symptom is a heart murmur, but, if the narrowing is severe in an infant, a bluish color to the skin (cyanosis) and signs of right heart failure (such as fatigue and enlargement of the liver) are possible. [msdmanuals.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]

  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]

  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]

  Seek medical treatment if you experience chest pain, shortness of breath, or fainting as a result of your pulmonary valve stenosis. These symptoms can indicate your condition is advancing. Mild stenosis may improve with time. [healthline.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]

  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]

• Hydrops Fetalis

  We report a case of fatal hydrops fetalis owing to adenoviral endomyocarditis with aortic and pulmonary valve stenosis. [ncbi.nlm.nih.gov]

• 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]

  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]

  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]

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].

• Enlargement of the Liver

  In most children, the only symptom is a heart murmur, but, if the narrowing is severe in an infant, a bluish color to the skin (cyanosis) and signs of right heart failure (such as fatigue and enlargement of the liver) are possible. [msdmanuals.com]

• 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]

Although these procedures have been applied in isolation as methods of treatment, this case demonstrates the feasibility and effectiveness of a combined percutaneous treatment. [ncbi.nlm.nih.gov]

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

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]

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]

Head Trauma 234 Sepsis and Septic Shock 242 Nonpolio Enteroviruses 332 Respiratory Syncytial Virus 334 Rhinoviruses 336 Roseola and Human Herpesviruses 337 Rubella 338 Rubeola 339 VaricellaZoster Virus 342 Viral Gastroenteritis 344 Disorders of Unknown Etiology [books.google.com]

(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]

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]

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]

[…] 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]

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

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]

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.