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Pulmonary Valve Insufficiency

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.


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.

Pulmonary Valve Stenosis
  • 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]
  • […] 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]
  • , including pulmonary valve stenosis.[childrenshospital.org]
  • Pulmonary valve stenosis Lake, Carol L.; Booker, Peter D. (2005-01-01). Pediatric Cardiac Anesthesia. Lippincott Williams & Wilkins. ISBN 9780781751759. Bruce, Charles J.; Connolly, Heidi M. (2009-05-26).[en.wikipedia.org]
  • Types of pulmonary valve disease include: Pulmonary valve stenosis . Pulmonary valve stenosis is a condition in which a deformity on or near your pulmonary valve reduces the blood flow from the heart to the pulmonary artery and to the lungs.[mayoclinic.org]
Coarctation of the Aorta
  • […] of the Aorta 581 The Heart in Pregnancy معلومات المراجع[books.google.com]
Early Satiety
  • Severe regurgitation leads to symptoms of right heart failure like dyspnea, initially with physical effort and later at rest; fatiguability, abdominal distention due to ascites, liver distention causing pain in the right hypochondrium, early satiety and[symptoma.com]
Heart Disease
  • 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]
  • Stenosis - when the valve doesn't open enough and blocks blood flow Valve problems can be present at birth or caused by infections, heart attacks, or heart disease or damage.[icdlist.com]
  • Caring for adults with congenital heart disease: successes and challenges for 2007 and beyond . Cardiol Young 2007 S ep 17 ( Suppl 2 ): 87 – 96 ; Review. PMID: 18039402. 22.[cambridge.org]
  • Diseases 561 Abnormalities of Right Ventricular Outflow 571 Infarction 446 Abnormal Vascular Connections and Structures 600 Suggested Readings 632 INDEX Masses Tumors and Source of Embolus 711 Echocardiography in Systemic Disease and Clinical Problem[books.google.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]
  • 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]
  • […] diagnose pulmonary regurgitation after a primary care doctor detects a heart murmur and refers a child to the Cardiac Center.[chop.edu]
  • Regurgitation results in turbulence and the generation of characteristic heart murmurs .[cvphysiology.com]
  • 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]
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]
  • Signs include a decrescendo diastolic murmur. Diagnosis is by echocardiography. Usually, no specific treatment is necessary except for management of pulmonary hypertension.[merckmanuals.com]
  • The backward flow of blood into the ventricular chamber during diastole results in a diastolic murmur .[cvphysiology.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]
Venous Insufficiency
  • insufficiency inadequacy of the venous valves and impairment of venous return from the lower limbs ( venous stasis ), often with edema and sometimes with stasis ulcers at the ankle.[medical-dictionary.thefreedictionary.com]
Ulcer
  • […] condition such as cleft palate or muscular dysfunction, resulting in defective speech. venous insufficiency inadequacy of the venous valves and impairment of venous return from the lower limbs ( venous stasis ), often with edema and sometimes with stasis ulcers[medical-dictionary.thefreedictionary.com]

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.

Incomplete Right Bundle Branch Block
  • 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[symptoma.com]

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]
  • Treatment consisted of a tricuspid valve annuloplasty and creation of an autologous monocusp valve using the anterior wall of the pulmonary artery.[ncbi.nlm.nih.gov]
  • The most common complication of the surgical treatment is the pulmonary valve insufficiency which usually requires reintervention, as was the case with our patient.[ingentaconnect.com]
  • BACKGROUND: Pulmonary valve (PV) insufficiency is often an acquired condition after treatment for pulmonary stenosis. It is recognized that PV insufficiency has serious deleterious effects.[ncbi.nlm.nih.gov]

Prognosis

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

Etiology

  • Pulmonary valve insufficiency is also rare, with reported etiologies comprising mainly pulmonary valve anomalies. A comparative review of the literature relating to the diagnosis and therapeutic management of the condition is provided.[ncbi.nlm.nih.gov]
  • 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

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • Among the causes of pulmonary insufficiency are: The pathophysiology is due to diastolic pressure variations between the pulmonary artery and right ventricle, differences are often very small, but increase regurgitation.[en.wikipedia.org]
  • 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]
  • In this era of increasing awareness and improved treatment options for patients with valve disease, it is important that clinicians consider the diagnosis of right-sided valve disease, understand its pathophysiology, choose appropriate confirmatory testing[doi.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]
  • 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]
  • 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]
  • Careful attention must be taken to prevent transannular leak at this level. The patch is folded and the ring of the valve is fixed to the transannular patch at the level of the fold.[ctsnet.org]

References

Article

  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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Last updated: 2018-06-21 19:22