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 .
Liver, Gall & Pancreas
- Heart Murmur
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]
Diagnosis Heart valve disease is diagnosed by listening to the heart with a stethoscope; diseased heart valves make distinct clicking sounds or murmurs. [ihimontanafoundation.org]
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]
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]
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]
- Diastolic Murmur
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]
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]
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]
High-pitched, early diastolic decrescendo murmur On auscultation, the 1st heart sound (S 1 ) is normal. The S 2 may be split or single. [merckmanuals.com]
An apical diastolic thrill can be palpated. A holodiastolic murmur can be auscultated at the apex with a presystolic accentuation in sinus rhythm. The intensity of this murmur is determined by the transmitral gradient. [textbookofcardiology.org]
- Systolic Murmur
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]
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. [symptoma.com]
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]
Mild stenosis is characterized by a systolic ejection click and short early systolic murmur. With progressive severity, the murmur gets louder, longer and peaks later in systole. The ejection click is often more prominent in expiration. [revespcardiol.org]
Atrial septal defect : Presence of systolic ejection murmur, wide fixed split S2, EKG showing RVH. In ASD the split of the S2 is fixed, there is no ejection click. [wikidoc.org]
- Opening Snap
Tricuspid stenosis: atrial systolic murmur, tricuspid opening snap, and right atrial pressure pulse. Am J Med. 1985;78:375-84. Medline 12. Tei C, Shah PM, Tanaka H. Phonographic-echographic documentation of systolic honk in tricuspid prolapse. [revespcardiol.org]
Reduction of valve area to Clinical presentation A tricuspid opening snap and a characteristic mid-diastolic murmur may be audible along the left sternoid border on auscultation. [textbookofcardiology.org]
- Pulmonary Ejection Click
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]
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]
Treatment Medical Treatment The only medical treatment is palliative for relief of right heart failure with hepatic congestion and peripheral edema. [revespcardiol.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]
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]
Thus, emphasis should be placed on the importance of prophylaxis in affording a good prognosis for recovery even in patients with severe heart disease and prevention of recurrence to reduce cardiac morbidity from this disease. [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]
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]
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 can be organic or functional. [textbookofcardiology.org]
Valves made from animal or human tissue (are used for valve replacement), in adults metal valves can be used.   Epidemiology [ edit ] The epidemiology of pulmonary valve stenosis can be summed up by the congenital aspect which is the majority of [en.wikipedia.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]
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]
Seckeler, M.D. and Hoke, T.R. (2011) The Worldwide Epidemiology of Acute Rheumatic Fever and Rheumatic Heart Disease. Clinical Epidemiology, 3, 67-84. http://dx.doi.org/10.2147/CLEP.S12977 7. [file.scirp.org]
Linguistic Variant - Portuguese, Brazil) 2015AB LNC-RU-RU (LOINC Linguistic Variant - Russian, Russia) 2015AB LNC-TR-TR (LOINC Linguistic Variant - Turkish, Turkey) 2015AB LNC-ZH-CN (LOINC Linguistic Variant - Chinese, China) 2015AB MCM (Glossary of Clinical Epidemiologic [doctor.am]
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]
The cause in turn is divided into: valvular, external and intrinsic (when it is acquired).  Pathophysiology [ edit ] The pathophysiology of pulmonary valve stenosis consists of the valve leaflets becoming too thick (therefore not separate one from [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]
Abstract Pulmonary hypertension (PH) is a classic pathophysiological consequence of left-sided valvular heart disease (VHD). [imaging.onlinejacc.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]
It can help to prevent an infection of the valve. Not everyone with valve stenosis will need this step. [winchesterhospital.org]
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]
Prevention Get prompt treatment for a sore throat that lasts longer than 48 hours, especially if accompanied by a fever. Timely administration of antibiotics may prevent the development of rheumatic fever which can cause valvular heart disease. [hopkinsmedicine.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]
- Bonow RO, Carabello BA, Kanu C, et al. ACC/AHA 2006 guidelines for the management of patients with valvular heart disease;a report of the American College of Cardiology / American Heart Association Task Force on Practice Guidelines (writing committee to revise the 1998 Guidelines for the management of patients with Valvular Heart Disease): developed in collaboration with the Society of Cardiovascular Anesthesiologists: endorsed by the Society for Cardiovascular Angiography and Interventions and the Society of Throacic Surgeons. Circulation. 2006;114:e84-e231.
- Waller BF, Howard J, Fess S. Pathology of pulmonic valve stenosis and pure regurgitation. Clin Cardiol. 1995;18:45–50.
- Kitagawa A, Oka N, Kimura S, et al. Clinical utility of the plasma brain natriuretic peptide level in monitoring tetralogy of Fallot patients over the long term after initial intracardiac repair: considerations for pulmonary valve replacement. Pediatr Cardiol. 2015;36(4):752-758.
- Lei MH, Chen JJ, Ko YL, Cheng JJ, Kuan P, Lien WP. Reappraisal of quantitative evaluation of pulmonary regurgitation and estimation of pulmonary artery pressure by continuous wave Doppler echocardiography. Cardiology. 1995;86:249–256.
- Silvilairat S, Cabalka AK, Cetta F, Hagler DJ, O’Leary PW. Echocardiographic assessment of isolated pulmonary valve stenosis: which outpatient Doppler gradient has the most clinical validity? J Am Soc Echocardiogr. 2005;18:1137–1142.
- Lang RM, Bierig M, Devereux RB, et al. Recommendations for chamber quantification. Eur J Echocardiogr. 2006;7:79–108.
- Didier D, Ratib O, Lerch R, et al. Detection and quantification of valvular heart disease with dynamic cardiac MR imaging. Radiographics. 2000;20 (5): 1279-1299.
- Williams RV, Minich LL, Shaddy RE, et al. Comparison of Doppler echocardiography with angiography for determining the severity of pulmonary regurgitation. Am J Cardiol. 2002;89:1438–1441.