Pulmonary valve disease consists of an association of two conditions- pulmonary valve stenosis and regurgitation. It can be a congenital or acquired condition, secondary to infective endocarditis, carcinoid disease or surgical interventions for other cardiac conditions. In order to diagnose pulmonary valve disease, both stenosis and regurgitation must be concomitantly present, although they may occur at different moments in time. Furthermore, pulmonary valvular stenosis may be accompanied by subvalvular and supravalvular stenosis, as well as more or less distal pulmonary artery stenosis.
Symptoms of pulmonary valve disease are more often due to the stenosis component and depend on the severity of the condition. They consist of complaints resulting from decreased cardiac output caused by right ventricular dysfunction: dyspnea, fatigability, exertional syncope or dizziness, chest pain, peripheral edema and, in rare cases, sudden death. If a ventricular septal defect coexists, like in tetralogy of Fallot, cyanotic spells may occur. If foramen ovale is patent, right atrial pressure may exceed left atrial pressure, leading to right-to-left shunt and cyanosis .
Physical signs also depend on severity. If the stenosis component is mild, it gives rise to a short presystolic murmur and an ejection click that only appears if the valves are pliable, while in severe stenosis the murmur is more intense and becomes holo-systolic and the click disappears because of the right ventricular end-diastolic pressure being higher than the pulmonary arterial pressure. In mild cases, the second heart sound is widely split, while in severe disease patients the pulmonary component becomes inaudible. The pulmonary regurgitation, especially if severe and caused by pulmonary hypertension, causes a high-pitched diastolic murmur that might be misinterpreted as aortic regurgitation. In mild to moderate pulmonary regurgitation situations, the murmur may be absent. If infundibular or supravalvular stenosis coexists, the systolic murmur changes accordingly: it peaks later during the systole or becomes more prolonged. If the right ventricle is enlarged, in can be palpated in the epigastric region (Hartzer sign). The jugular pulse may have a prominent "A" wave. If the pulmonary regurgitation component is severe, the heart may be enlarged, pulsatile and painful .
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When symptoms appear, they may include a heart murmur, fatigue, shortness of breath, chest pain, fainting, and poor weight gain for infants. [ihimontanafoundation.org]
It can be diagnosed prenatally but often is not diagnosed until after birth when a doctor hears a heart murmur. Further testing would confirm pulmonary valve disease. [umm.edu]
Presentation See also the separate Heart Auscultation and Heart Murmurs in Children articles. Pulmonary regurgitation is usually asymptomatic unless severe, when it may lead to signs of right heart failure. [patient.info]
murmur Distention of the jugular vein in the neck Enlargement of the liver Retention of fluid in the ankles Tests may include: Chest X-ray Echocardiogram Cardiac catheterization Holter monitor Pulmonary Regurgitation Treatment Options You may not need [heart.uvahealth.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]
The pulmonary regurgitation, especially if severe and caused by pulmonary hypertension, causes a high-pitched diastolic murmur that might be misinterpreted as aortic regurgitation. [symptoma.com]
Soft diastolic murmur at the left upper sternal edge. Right ventricular hypertrophy. Loud P2 (pulmonary component of the second heart sound). May be differentiated from aortic regurgitation by lack of collapsing pulse. Investigations ECG. CXR. [patient.info]
murmur starts at S2-- descrendo (high pitch) signs of aortic regurg Corrigan's pulse Rapid forceful carotid upstroke followed by rapid decline signs of aortic regurg Quincke's pulse Diastolic blanching in nail bed when slightly compressed signs of aortic [brainscape.com]
Signs include a decrescendo diastolic murmur. Diagnosis is by echocardiography. Usually, no specific treatment is necessary except for management of pulmonary hypertension. [merckmanuals.com]
A high-pitched diastolic murmur following a prominent P 2 may be evident in patients with PR secondary to pulmonary hypertension. [revespcardiol.org]
If infundibular or supravalvular stenosis coexists, the systolic murmur changes accordingly: it peaks later during the systole or becomes more prolonged. If the right ventricle is enlarged, in can be palpated in the epigastric region (Hartzer sign). [symptoma.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]
General appearance was normal and cardiac auscultation revealed 2/6 systolic murmur in the left second intercostal space. ECG and X-ray chest were normal. [file.scirp.org]
Clinical assessment of infundibular pulmonary stenosis (PS) reveals a systolic murmur peaking in late systole and well-preserved, but delayed, P 2. Clinical assessment of supravalve PS often provides diagnostic clues. [revespcardiol.org]
Cyanosis in these patients is due to the right to left shunting at the level of the foramen ovale.   Mild Pulmonic Stenosis: Patients with mild stenosis are asymptomatic and are diagnosed by routine examination with an ejection systolic murmur [wikidoc.org]
Pulmonary Ejection Click
Pulmonary ejection click. Delayed second heart sound with severe stenosis. Parasternal thrill and heave. 'A waves' in the JVP. Investigations Echocardiography: will confirm the valve defect and measure the flow across the valve. [patient.info]
This seemingly paradoxical behavior of the pulmonary ejection click is explained by an inspiratory increase in right ventricular end-diastolic pressure, which opens the valve in late diastole and, hence, causes absence of systolic ejection click during [revespcardiol.org]
Pulmonary Systolic Murmur
Signs Soft pulmonary systolic murmurs are more easily heard with the patient lying down. They are often heard in healthy individuals and may be due to physiological changes associated with respiration. [patient.info]
Brain natriuretic peptide is measured initially and at follow-up visits, as it is a good indicator of disease evolution . The electrocardiogram shows right ventricular hypertrophy and right atrial dilatation, while posteroanterior and lateral chest radiography highlights a dilated pulmonary trunk and diminished pulmonary vascularization.
More information is added by echocardiography with regard to both stenosis and regurgitation components. The physician should assess valvular morphology, as well as supplementary supravalvular and subvalvular stenosis. Color doppler helps quantify the severity of the stenosis in a qualitative manner and assess the number of regurgitant flows, while pulsed and continuous wave doppler measure jet velocity and pressure gradients . Flow velocities of regurgitant jets should be measured, as well as jet acceleration time. Planimetry is not feasible, and continuity equation and proximal isovelocity surface area methods are not validated for pulmonary valve disease. Still, it is important to measure pulmonary artery pressure: systolic, mean and diastolic ; the fact that systolic pulmonary artery pressure may be as high as or exceed the systemic pressure is also suggested by an immobile or abnormal motion interventricular septum. Right ventricular enlargement should also be quantified .
Computer tomography and magnetic resonance imaging are also useful in assessing disease severity and the cardiac status . Cardiac catheterization is not usually needed in this condition unless echocardiographic and clinical findings are very different. It can more reliable to assess right ventricular and pulmonary artery pressure by "pull back" technique .
Humanitas Research Humanitas is a world-famous center of excellence for research and treatment of immune system-related disease. [humanitas.net]
Pulmonary Valve Disease Treatment Cardiovascular treatments are not required for most cases of pulmonary valve disease. Pulmonary regurgitation as the result of pulmonary hypertension may require you to take medication. [dignityhealth.org]
Options You may not need immediate treatment if you have mild or moderate symptoms. [heart.uvahealth.com]
The treatment options for tricuspid stenosis include balloon valvuloplasty and surgical valve repair. [ncbi.nlm.nih.gov]
Symptoms of Pulmonary Valve Disease Patients with pulmonary valve disease may not notice symptoms, but may need treatment before symptoms develop if the pulmonary valve is severely affected. [houstonmethodist.org]
Prognosis Pulmonary regurgitation is usually well tolerated in childhood. [patient.info]
Support Groups Expectations (prognosis) Without surgery, most infants who have severe lung complications will die. Surgery can treat the condition and relieve symptoms in many cases. [mostellarmedical.org]
Tompkins, D.G., Boxerbaun, B. and Liebman, J. (1972) Long-Term Prognosis of Rheumatic Fever Patients Receiving Regular Intramuscular Benzathine Penicillin, Circulation, 45, 543-551. 25. [file.scirp.org]
Fortunately, the prognosis for children with this condition has greatly improved over the last several decades (assuming proper diagnosis and treatment is administered). [advancingyourhealth.org]
Etiology, pathogenesis and hemodynamics of diseases of both valves have been dealt in simple and comprehensible manner. Special effort has been made to explain the clinical features (Symptoms and signs) of disease. [authorhouse.com]
Symptoms caused by underlying etiology such as flushing, diarrhea, abdominal pain, etc. associated with carcinoid heart disease point to the etiology. [revespcardiol.org]
Etiological Aspects 3.1.1. Congenital Diseases of the pulmonary valve are most often congenital, and only rarely due to acquired disorders such as carcinoid, rheumatic and infective endocarditis . [file.scirp.org]
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]
Over the past 60 years, the etiology of most valvular heart diseases in industrialized countries shifted towards degenerative etiologies, mainly because of a decrease in acute rheumatic fever. [textbookofcardiology.org]
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]
Seckeler, M.D. and Hoke, T.R. (2011) The Worldwide Epidemiology of Acute Rheumatic Fever and Rheumatic Heart Disease. Clinical Epidemiology, 3, 67-84. 7. [file.scirp.org]
Explore these free sample topics: -- The first section of this topic is shown below -- Basics Description Deformity of pulmonary valve, most commonly congenital, resulting in obstruction of right ventricular (RV) outflow Epidemiology Incidence Predominant [unboundmedicine.com]
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Epidemiology Valvular heart diseases are a major burden to society and it is expected that the prevalence will increase. Rheumatic valve disease used to be the most prevalent etiology of valvular cardiac diseases worldwide. [textbookofcardiology.org]
These haemodynamic findings are similar to recent studies in bicuspid aortic disease, and suggest the importance of flow patterns in the pathophysiology of vessel dilation in both aortic and pulmonary bicuspid valve disease. [rdm.ox.ac.uk]
[…] 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]
Abstract Pulmonary hypertension (PH) is a classic pathophysiological consequence of left-sided valvular heart disease (VHD). [imaging.onlinejacc.org]
"Pathophysiology of Aortic Valve Disease." Cardiac Surgery in the Adult. Ed. Cohn L.H. Third Edition ed. McGraw-Hill Education, 2012. 825-40. [textbookofcardiology.org]
In addition, the TV morphology provides clues of underlying etiology and pathophysiology of valve dysfunction. 13 Tricuspid valve morphology: Ebstein's anomaly is characterized by apical displacement of the septal tricuspid leaflet into the right ventricle [revespcardiol.org]
Prevention Though pulmonary valve disease cannot be prevented, there are a few measures that can be taken to improve overall health and slow the development of heart disease. [humanitas.net]
Children’s heart conditions can’t be prevented, but a lot can be done to improve and often completely repair their hearts at any age. [nemours.org]
Preventative Antibiotics and Heart Valve Disease Patients with heart valve disease who have an abnormal heart or who have had heart surgery risk developing endocarditis. [ihimontanafoundation.org]
Prevention Although there is no way to prevent this condition, families may be evaluated to determinetheir risk ofcongenital defects. References Park, MK. Park : Pediatric Cardiology for Practitioners. 5th ed. Philadelphia, Pa: Mosby; 2008. [mostellarmedical.org]
Treatment Therapy is directed towards preventing recurrent rheumatic heart disease in children and monitoring for the complications and sequelae of chronic rheumatic heart disease in adults. [file.scirp.org]
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