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Pulmonary Valve Stenosis

Valvular Pulmonary Stenosis

Pulmonary valve stenosis is one of the most common types of congenital heart disease and is predominantly asymptomatic throughout childhood and adulthood. In the case of severe stenosis, the effort of the right ventricle is increased due to reduced blood flow into the pulmonary trunk, causing fatigue and dyspnea. The anomaly is visible with echocardiography and the diagnosis is often incidental.


Presentation

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].

Hemophilia A
  • We treated three unrelated patients with hemophilia A and congenital pulmonary valve stenosis. In two patients, the occurrence of the cardiac malformation was sporadic and in one familial.[ncbi.nlm.nih.gov]
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]
  • A successful catheter closure of an atrial septal defect was performed with a dramatic increase in systemic oxygen saturation alleviating the need for a surgical systemic-to-pulmonary artery shunt.[ncbi.nlm.nih.gov]
  • A 13-month-old boy with a diagnosis of atrial septal defect and pulmonary valve stenosis was admitted for corrective surgery. Right juxtaposition of the atrial appendages with a tunnel between the atrial appendages was detected during the surgery.[ncbi.nlm.nih.gov]
  • Pulmonary valve stenosis and atrial septal defect are common forms of congenital heart disease; however, their association is relatively rare.[ncbi.nlm.nih.gov]
  • The infant was centrally cyanosed and had a systolic murmur from a moderately severe pulmonary valve stenosis and a small atrial septal defect.[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]
  • 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]
  • […] 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]
  • We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: May 12, 2019[patientslikeme.com]
  • This results in swelling of the legs and abdomen and can cause fatigue and shortness of breath. Irregular heartbeat (arrhythmia). People with pulmonary stenosis are more likely to have an irregular heartbeat.[mayoclinic.org]
Exertional Dyspnea
  • He became physically active and was free of exertional dyspnea at the 12-month follow-up.[ncbi.nlm.nih.gov]
  • We describe the case of a 70-year-old man who presented with increasing exertional dyspnea.[ncbi.nlm.nih.gov]
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]
  • Pulmonary valve stenosis and atrial septal defect are common forms of congenital heart disease; however, their association is relatively rare.[ncbi.nlm.nih.gov]
  • 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]
  • Transcatheter therapy of valvular pulmonary stenosis is one of first catheter interventions facilitating its application in field of structural heart disease and now treatment of choice for significant pulmonary stenosis.[ncbi.nlm.nih.gov]
Heart Murmur
  • This paper describes an asymptomatic 6-year-old boy who was referred because of a heart murmur. An electrocardiogram and two-dimensional echocardiography revealed right heart volume overload.[ncbi.nlm.nih.gov]
  • 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]
  • Moderate to severe PS is most often diagnosed during childhood due to the loud heart murmur associated with the condition. What are the symptoms? If PS is mild, there probably won't be any noticeable symptoms.[heart.org]
  • In most children, the only symptom is a heart murmur, but a bluish color to the skin (cyanosis) and signs of right heart failure (such as fatigue and shortness of breath) are possible.[msdmanuals.com]
  • Pulmonary valve stenosis can cause a heart murmur. A heart murmur sounds like an extra click, blowing, whooshing, or rasping sound when a doctor listens to your heart. The murmur can be an initial indicator of pulmonary valve stenosis.[healthline.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]
  • 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]
  • 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
  • The infant was centrally cyanosed and had a systolic murmur from a moderately severe pulmonary valve stenosis and a small atrial septal defect.[ncbi.nlm.nih.gov]
  • 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]
  • Sixteen patients were asymptomatic with pulmonary systolic murmurs, although 6 patients presented with dyspnea.[ncbi.nlm.nih.gov]
  • Additionally in our study, we found no correlation between the PV gradient and the intensity of the systolic murmur, presence of a systolic click, or the PV morphology by trans-thoracic echocardiogram at initial evaluation.[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]
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 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 452 Diseases of the[books.google.com]
Hydrops Fetalis
  • We report a case of fatal hydrops fetalis owing to adenoviral endomyocarditis with aortic and pulmonary valve stenosis.[ncbi.nlm.nih.gov]
Short Arm
  • We report on a patient with a partial deletion on the short arm of chromosome 18 (del 18p), who presented with dysmorphic features and delayed developmental milestones as well as with a patent ductus arteriosus (PDA) and pulmonary valve stenosis (PS).[ncbi.nlm.nih.gov]
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]
  • 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]
  • 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]

Workup

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].

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]
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]

Treatment

  • 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]
  • Twelve hours after the dental treatment, the patient's core temperature fell to 37.4 degrees C. After hospitalization for 4 days, the patient was discharged in good condition.[ncbi.nlm.nih.gov]
  • The mean diameter of the pulmonary annulus was 7 mm ( 1.19); 33.3% had a dysplastic pulmonary valve, and 92% were started on prostaglandin E1 treatment.[ncbi.nlm.nih.gov]
  • After starting phentolamine or captopril treatment, prostaglandin could be discontinued after a mean time of 15.86 hours.[ncbi.nlm.nih.gov]
  • Abstract Balloon valvuloplasty for pulmonary valve stenosis by static dilation is the treatment of choice for the treatment of isolated valvular pulmonary stenosis in the infant, child, and adolescent.[ncbi.nlm.nih.gov]

Prognosis

  • 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 for pulmonary valve stenosis Patients with moderate stenosis can have a normal life. Bacterial endocarditis can be developed.[remedyland.com]
  • Prognosis Pulmonary regurgitation is usually well tolerated in childhood.[patient.info]
  • Prognosis Mild pulmonic valvular stenosis has a good overall prognosis. Life expectancy approaches that of someone without valvular disease. [9] Patients with moderately severe to severe stenosis have clinically progressing disease.[emedicine.com]

Etiology

  • 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]
  • 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]
  • 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]
  • 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]
  • Other etiologies result in dysplastic valves, which do not open and close normally. [19] Coexisting cardiac malformations (eg, ventricular septal defect, atrial septal defect, patent ductus arteriosus) may complicate the anatomy, physiology, and clinical[emedicine.medscape.com]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • 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]
  • Pathophysiology [ edit ] When pulmonic stenosis (PS) is present, resistance to blood flow causes right ventricular hypertrophy.[en.wikipedia.org]
  • […] analyzed the treatment, quality of life, echocardiography findings, complications, exercise responses, and predisposition to endocarditis with regards to cardiac valvular disease, and pulmonary stenosis was found to be the most benign valvular lesion. [9] Pathophysiology[emedicine.com]

Prevention

  • 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]
  • 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]

References

Article

  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.

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Last updated: 2019-06-28 10:17