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Pulmonary Valve Insufficiency
Leaky Pulmonary Valve

Pulmonary valve insufficiency or regurgitation is the valvulopathy characterized by the presence of diastolic retrograde flow from the pulmonary artery to the right ventricle. If severe, this regurgitation leads, in time, to the appearance of right ventricular dysfunction and heart failure. This condition can be primary (congenital- as a stand alone disease or in association with Marfan syndrome), caused by endocarditis, rheumatic or carcinoid heart disease or secondary to pulmonary hypertension, percutaneous or surgical dilatation of a pulmonary stenosis or dilated cardiomyopathy.

Images

WIKIDATA, Public Domain

Presentation

Pulmonary valve insufficiency patients are asymptomatic if disease severity is mild. However, one-third of them will become symptomatic before reaching the age of forty years [1]. Severe regurgitation leads to symptoms of right heart failure [2] like dyspnea, initially with physical effort and later at rest [3]; fatiguability, abdominal distention due to ascites, liver distention causing pain in the right hypochondrium, early satiety and peripheral edema. Non-specific signs include angina due to the inability of the coronary arteries to properly oxygenate a dilated right ventricle, palpitations caused by atrial [4] or ventricular arrhythmias, that have a potential to be life threatening [5] and syncope- an insufficient, dilated heart will not provide proper cerebral blood flow. Symptoms associated with the underlying disease causing secondary pulmonic regurgitation may also be encountered: fever in infective endocarditis, hemoptysis in pulmonary hypertension or articular symptoms in rheumatic heart disease. Furthermore, acute neonatal pulmonary valve insufficiency caused by a large patent ductus arteriosus in Ebstein's anomaly patients has also been described [6].

Physical examination reveals an accentuated and delayed pulmonary component of the second heart sound if pulmonary hypertension is present. The pulmonary insufficiency murmur is brief, early diastolic and decrescendo and increases with squatting and inspiration. Valsalva maneuver decreases its intensity. If pulmonary hypertension coexists, the murmur modifies its character by becoming high pitched and possibly holo- diastolic. A tricuspid regurgitation murmur may overlap, making auscultation more difficult. If the right ventricle is enlarged, an impulse may be palpated along the left sternal border. Signs of elevated jugular venous pressure signs may also be observed.

Entire Body System

  • Pulmonary Valve Stenosis

    valve disorder Pulmonary valve regurgitation Pulmonary valve regurgitation, nonrheumatic Pulmonary valve stenosis Pulmonary valve stenosis, nonrheumatic Pulmonic valve regurgitation Pulmonic valve stenosis Pulmonic valve stenosis (narrowing) Clinical [icd9data.com]

    Overview Pulmonary valve stenosis is a condition in which a deformity on or near your pulmonary valve narrows the pulmonary valve opening and slows the blood flow. [mayoclinic.org]

    Areas of potential narrowing include thickened muscle below the pulmonary valve, stenosis of the valve itself, or stenosis of the pulmonary artery above the valve. [cincinnatichildrens.org]

    What is pulmonary valve stenosis? Pulmonary valve stenosis is the term describing a narrowing in the opening of the pulmonary valve. [childrenshospital.org]

    In a neonate suffering from this syndrome, the pulmonary valve stenosis was relieved by rupturing the fused valve with a balloon catheter introduced transvenously. [link.springer.com]

  • Coronary Atherosclerosis

    English TRA 2°P - TIMI 50 was a multicenter, randomized, double-blind, placebo-controlled study conducted in patients who had evidence or a history of atherosclerosis involving the coronary, cerebral, or peripheral vascular systems. [mymemory.translated.net]

Gastrointestinal

  • Abdominal Fullness

    Elevated right atrial (RA) pressure also leads to peripheral edema and hepatic congestion with decreased appetite and abdominal fullness. Long-standing severe TR results in right-heart failure, ascites, and anasarca. [doi.org]

Cardiovascular

  • Heart Disease

    Complications tricuspid valve regurgitation congestive heart failure valvular heart disease general pulmonary valve pathologies: pulmonary valve stenosis pulmonary valve regurgitation specific pulmonary valve pathologies: congenital pulmonary valve stenosis [radiopaedia.org]

    Published on Mar 20, 2018 Mayo Clinic cardiologist Heidi Connolly, M.D., discusses pulmonary valve regurgitation in adult congenital heart disease. [youtube.com]

    […] in Adults With Tetralogy of Fallot: A Meta-analysis-A Report for the Writing Committee of the 2019 Update of the Canadian Cardiovascular Society Guidelines for the Management of Adults With Congenital Heart Disease. 61 Mongeon FP...Marelli A 31813508 [malacards.org]

    Heart Valve Diseases Also called: Valvular heart disease Your heart has four valves. Normally, these valves open to let blood flow through or out of your heart, and then shut to keep it from flowing backward. But sometimes they don't work properly. [icdlist.com]

    Symptoms associated with the underlying disease causing secondary pulmonic regurgitation may also be encountered: fever in infective endocarditis, hemoptysis in pulmonary hypertension or articular symptoms in rheumatic heart disease. [symptoma.com]

  • 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 If pulmonary valve insufficiency is asymptomatic, its main manifestation is a heart murmur. The most precise diagnostic methods are: Echocardiography is commonly used for diagnosis of this disease. [bookinghealth.com]

    […] that indicates pulmonary valve regurgitation is a certain type of heart murmur that can be heard during a physical exam. [mercy.com]

    […] diagnose pulmonary regurgitation after a primary care doctor detects a heart murmur and refers a child to the Cardiac Center. [chop.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]

  • Diastolic Murmur

    Abstract To study the transient diastolic murmur associated with renal failure, we used Doppler echocardiography to characterize flow across the semilunar valves in 10 patients on chronic hemodialysis with a diastolic murmur (group A), 26 patients on [annals.org]

    Clinical examination classically reveals a right ventricular heave on praecordial palpation, and an early diastolic murmur that is heard on praecordial auscultation known as the Graham Steell murmur 1-3. [radiopaedia.org]

    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 (S1) is normal. The S2 may be split or single. [merckmanuals.com]

  • Graham Steell Murmur

    If it is secondary to pulmonary hypertension it is referred to as a Graham Steell murmur. Example sentences with "pulmonary valve insufficiency", translation memory add example No translation memories found. [glosbe.com]

    The murmur of pulmonic regurgitation increases in intensity during inspiration, unlike that of aortic regurgitation. The murmur of pulmonic regurgitation is classically referred to as the "Graham-Steell murmur" after it's initial describers. [healio.com]

    Steell's murmur (pulmonic regurgitation) (see also Endocarditis, pulmonary) 424.3 Incompetency, incompetence, incompetent annular pulmonary valve (heart) (see also Endocarditis, pulmonary) 424.3 Insufficiency, insufficient Lesion(s) Murmur (cardiac) [icd9data.com]

    Clinical examination classically reveals a right ventricular heave on praecordial palpation, and an early diastolic murmur that is heard on praecordial auscultation known as the Graham Steell murmur 1-3. [radiopaedia.org]

  • Pulmonary 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]

Workup

Blood workup should include brain natriuretic peptide plasma level that has proven useful in monitoring disease severity and right ventricular end-diastolic pressure [7].

Thoracic radiography is useful especially in pulmonary hypertension patients, where the typical aspect of enlarged central pulmonary and hilar vessels and diminished peripheral vascularization is observed. Cardiomegaly may also be present.

The electrocardiogram may show right ventricular hypertrophy (tall R or QR aspect in V1, negative T wave in V1-V3 and right axis deviation) or dilatation (complete or incomplete right bundle branch block and right axis deviation) and right atrial enlargement.

Echocardiography further characterizes chamber dimensions, wall thickness, and kinetics, as well as the aspect of the pulmonary valve, that may be dilated, perforated, ruptured or have attached vegetations. An immobile or abnormal motion of the interventricular septum is an indirect indicator of pulmonary hypertension and increased right ventricular pressures. Color flow Doppler offers a qualitative assessment of the regurgitant pulmonary jet in terms of dimensions and orientation. If regurgitation is holodiastolic or pulmonary acceleration time (normally 140 msec) is decreased, pulmonary hypertension must be suspected and demonstrated by calculating pulmonary artery systolic and diastolic pressures, both good predictors of cardiac status [8] [9]. Vena contracta has not yet been validated for pulmonary regurgitation. Regurgitant volume and regurgitant fraction can be calculated using pulsed Doppler [10] and stroke volume.

Pulmonary artery angiography is indicated when pulmonary embolism as a cause of pulmonary hypertension and pulmonary valve insufficiency is suspected. The same information can be obtained with less invasive methods, such as computer tomography angiography or ventilation/perfusion scanning, while cardiac magnetic resonance imaging offers high quality characterization of pulmonary valve anatomy and regurgitation mechanism.

Treatment

Treatment On its own, pulmonary valve insufficiency is seldom severe enough to require treatment. Antibiotics are usually recommended before dental work to reduce the possibility of bacterial endocarditis. [medical-dictionary.thefreedictionary.com]

The newest types of pulmonary valve insufficiency treatment include pulmonary homograft and the Melody valve treatment. A pulmonary homograft is usually used for the treatment of children, because it can adapt to the body as the child grows. [bookinghealth.com]

Treatment for pulmonary valve regurgitation The goal of treatment for pulmonary valve regurgitation is to treat the underlying condition causing the valve problem. Pulmonary valve replacement is rare. [mercy.com]

Options You may not need immediate treatment if you have mild or moderate symptoms. [uvahealth.com]

Prognosis

Most patients with with PR following repair of tetralogy of Fallot carry an excellent prognosis. [emedicine.medscape.com]

Patients who have not undergone a radical surgical intervention have a poor prognosis, whereas the prognosis is much better for patients who have been operated. [content.sciendo.com]

Etiology

Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]

Depending on the etiology, patients may have clinical features of pulmonary hypertension 1,2. [radiopaedia.org]

Morbidity, mortality, and complications As noted, the morbidity and mortality rates associated with pulmonic regurgitation vary considerably, depending on the underlying etiology. [emedicine.medscape.com]

Epidemiology

Neurosurgery (79) Dentistry (76) Histology, Embryology (68) Emergency Medicine (55) Medical Chemistry and Biochemistry (46) Otorhinolaryngology (45) Radiology and Imaging (45) Biophysics (36) Endocrinology, Metabolism (35) Obstetrics, Gynaecology (29) Epidemiology [portal.mefanet.cz]

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]

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]

Pathophysiology

[medical citation needed] Causes[edit] Among the causes of pulmonary insufficiency are: Pathophysiology[edit] The pathophysiology is due to diastolic pressure variations between the pulmonary artery and right ventricle, differences are often very small [en.wikipedia.org]

Surgery, Traumatology and Orthopaedics (765) Internal Medicine (290) Pathology and Forensic Medicine (248) Paediatrics, Neonatology (178) Cardiology, Angiology (148) Anatomy (138) Neurology (138) Physiology and Pathophysiology (116) Oncology, Radiation [portal.mefanet.cz]

Pathophysiology Pulmonary Regurgitation results in right ventricle volume overload which eventually leads to right ventricular enlargment and dysfunction; the augmented stroke volume may lead to secondary dilation of the pulmonary main artery which in [wikiecho.org]

Pathophysiology of PR The functional situation of a severely or freely regurgitant pulmonary valve is different from that of a severely regurgitant aortic valve for two reasons. [academic.oup.com]

"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]

Prevention

Prevention Pulmonary valve insufficiency resulting from chronic lung diseases can be prevented by behaviors and interventions to prevent those primary diseases. [medical-dictionary.thefreedictionary.com]

[…] translation and definition " pulmonary valve insufficiency ", English-Arabic Dictionary online pulmonary valve insufficiency قصور الصمام الرئوي A pulmonary valve disease that occurs when the pulmonary valve is not strong enough to prevent backflow into [glosbe.com]

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]

References

  1. Shimazaki Y, Blackstone E, Kirklin J. The natural history of isolated congenital pulmonary valve incompetence: surgical implications. Thorac Cardiovasc Surg. 1984;32(4):257-259.
  2. Kondo C, Nakazawa M, Kusakabe K, et al. Left ventricular dysfunction on exercise long-term after total repair of tetralogy of Fallot. Circulation. 1995;1;92(9):II250-255.
  3. Wessel H, Cunningham W, Paul M, et al. Exercise performance in tetralogy of Fallot after intracardiac repair. J Thorac Cardiovasc Surg. 1980;80(4):582-593.
  4. Gatzoulis M, Balaji S, Webber S, et al. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet. 2000;16;356(9234):975-981.
  5. Gatzoulis M, Till J, Somerville J, et al. Mechanoelectrical interaction in tetralogy of Fallot. QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death. Circulation. 1995;15;92(2):231-237.
  6. Wald R, Adatia I, Van Arsdell G, et al. Relation of limiting ductal patency to survival in neonatal Ebstein's anomaly. Am J Cardiol. 2005;15;96(6):851-856.
  7. 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.
  8. Ristow B, Ahmed S, Wang L, et al. Pulmonary regurgitation end-diastolic gradient is a Doppler marker of cardiac status: data from the Heart and Soul Study. J Am Soc Echocardiogr. 2005;18(9):885-891.
  9. Ristow B, Ali S, Ren X, et al. Elevated pulmonary artery pressure by Doppler echocardiography predicts hospitalization for heart failure and mortality in ambulatory stable coronary artery disease: the Heart and Soul Study. J Am Coll Cardiol. 2007;49(1):43-49.
  10. Marx G, Hicks R, Allen H, et al. Noninvasive assessment of hemodynamic responses to exercise in pulmonary regurgitation after operations to correct pulmonary outflow obstruction. Am J Cardiol. 1988;61:595–601.
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