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Pulmonary Valve Disease

Pulmonic Valve Disease

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.


Presentation

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 [1].

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 [2].

Heart Murmur
  • 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]
  • 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]
  • 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]
  • 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 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]
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]
  • Signs include a decrescendo diastolic murmur. Diagnosis is by echocardiography. Usually, no specific treatment is necessary except for management of pulmonary hypertension.[merckmanuals.com]
Systolic Murmur
  • 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]
Exertional Syncope
  • 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.[symptoma.com]
  • Fainting or exertional syncope. Sudden death. 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]
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]
Suggestibility
  • 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[symptoma.com]
  • 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]
  • This symbol (with prefactor N) accomplishes the same tasks the δ- function had when doing the continuous Fourier transformation. first two Mobile PAMM Brokers Options Black–Scholes model Piracy The SIIA suggests Later in the dis- ease course, after treatment[automatedforextraders.com]

Workup

Brain natriuretic peptide is measured initially and at follow-up visits, as it is a good indicator of disease evolution [3]. 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 [4]. 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 [5]; 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 [6].

Computer tomography and magnetic resonance imaging are also useful in assessing disease severity and the cardiac status [7]. 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 [8].

Atelectasis
  • Complications Brain infection (abscess) Lung collapse (atelectasis) Pneumonia Right-sided heart failure Stroke Calling your health care provider Call your health care provider if your infant has symptoms of absent pulmonary valve.[mostellarmedical.org]
Right Axis Deviation
  • 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]
Enlargement of the Liver
  • […] 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 immediate treatment if you have mild or moderate symptoms.[heart.uvahealth.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]
Prominent A-Wave
  • The jugular pulse may have a prominent "A" wave. If the pulmonary regurgitation component is severe, the heart may be enlarged, pulsatile and painful.[symptoma.com]

Treatment

  • 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]
  • […] echo Doppler interrogation of aortic valve what is recommended treatment for symptomatic severe aortic stenosis valve replacement (must have strong indications) what is outlook for patients receiving aortic valve replacmeent 75% death within 3 yrs of[brainscape.com]

Prognosis

  • 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]

Etiology

  • 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]
  • 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]
  • 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]

Epidemiology

  • 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]
  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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]

Prevention

  • 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]
  • 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]
  • Repairing or replacing a valve can prevent lasting damage to your heart and sudden death.[nhlbi.nih.gov]
  • The goal of an operation for tricuspid regurgitation is to improve symptoms, and prevent progressive failure of the right ventricle, the hearts right-sided pumping chamber.[myheart.net]

References

Article

  1. 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.
  2. Waller BF, Howard J, Fess S. Pathology of pulmonic valve stenosis and pure regurgitation. Clin Cardiol. 1995;18:45–50.
  3. 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.
  4. 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.
  5. 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.
  6. Lang RM, Bierig M, Devereux RB, et al. Recommendations for chamber quantification. Eur J Echocardiogr. 2006;7:79–108.
  7. 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.
  8. 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.

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Last updated: 2019-07-11 20:44