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.
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 for Pulmonary Valve Insufficiency depends on the severity of the condition and the symptoms. Mild cases may not require treatment and can be managed with regular monitoring. In more severe cases, medications may be prescribed to manage symptoms, such as diuretics to reduce fluid retention. In cases where the valve dysfunction significantly affects heart function, surgical intervention may be necessary. This could involve valve repair or replacement.
Prognosis
The prognosis for individuals with PVI varies based on the severity of the condition and the presence of other health issues. Many people with mild PVI live normal lives without significant complications. However, severe cases can lead to heart failure if not properly managed. With appropriate treatment and monitoring, most patients can maintain a good quality of life.
Etiology
Pulmonary Valve Insufficiency can be caused by several factors. Congenital heart defects, where the valve is malformed from birth, are a common cause. Other causes include damage from infections such as endocarditis, rheumatic fever, or carcinoid syndrome. Additionally, conditions that cause the pulmonary artery to dilate can lead to valve insufficiency.
Epidemiology
PVI is less common than other types of heart valve diseases. It can occur at any age but is often associated with congenital heart defects, which are present from birth. The exact prevalence is not well-documented, but it is considered a rare condition compared to other valvular heart diseases.
Pathophysiology
In PVI, the pulmonary valve fails to close completely, allowing blood to flow back into the right ventricle. This backward flow increases the volume of blood the right ventricle must handle, potentially leading to right ventricular enlargement and dysfunction over time. The heart may compensate initially, but prolonged stress can lead to heart failure.
Prevention
Preventing PVI involves managing risk factors and underlying conditions that could lead to valve damage. This includes maintaining good dental hygiene to prevent infections that could affect the heart valves, managing blood pressure, and treating any infections promptly. Regular check-ups can help detect early signs of valve problems.
Summary
Pulmonary Valve Insufficiency is a condition where the pulmonary valve does not close properly, leading to the backward flow of blood into the heart. While some individuals may not experience symptoms, others may have fatigue, shortness of breath, and swelling. Diagnosis involves physical exams and imaging tests, and treatment ranges from monitoring to surgery, depending on severity. With proper management, many patients can lead normal lives.
Patient Information
If you have been diagnosed with Pulmonary Valve Insufficiency, it's important to follow your doctor's recommendations and attend regular check-ups. Keep track of any symptoms and report changes to your healthcare provider. Lifestyle changes, such as a healthy diet and regular exercise, can support heart health. Understanding your condition and treatment options can empower you to manage your health effectively.
References
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.