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]

  I37 Nonrheumatic pulmonary valve disorders Excludes1 pulmonary valve disorder specified as congenital ( Q22.1, Q22.2, Q22.3 ) pulmonary valve disorder specified as rheumatic ( I09.89 ) I37.0 Nonrheumatic pulmonary valve stenosis I37.1 Nonrheumatic pulmonary [icd10coded.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]

  valve stenosis I37.1 Nonrheumatic pulmonary valve insufficiency I37.2 Nonrheumatic pulmonary valve stenosis with insufficiency I37.8 Other nonrheumatic pulmonary valve disorders I37.9 Nonrheumatic pulmonary valve disorder, unspecified I38 Endocarditis [icd10data.com]

  valve stenosis Q22.2 - Congenital pulmonary valve insufficiency Q22.3 - Other congenital malformations of pulmonary valve Q22.4 - Congenital tricuspid stenosis Q22.5 - Ebstein's anomaly Q22.6 - Hypoplastic right heart syndrome Q22.8 - Other congenital [unboundmedicine.com]

• Fishing

  Box 30105, Bethesda, MD 20824-0105. (301) 251-1222. . insufficiency [ in″sŭ-fish en-se ] inability to perform properly an allotted function; called also incompetence. coronary insufficiency decreased supply of blood to the myocardium resulting from constriction [medical-dictionary.thefreedictionary.com]

• Coronary Atherosclerosis

  English The European Medicines Agency has waived the obligation to submit the results of studies with Clopidogrel/Acetylsalicylic acid Zentiva in all subsets of the paediatric population in the treatment of coronary atherosclerosis (see section 4.2 for [mymemory.translated.net]

• Liver Fibrosis

  fibrosis markers and risk of liver carcinoma in adult patients after repair of tetralogy of Fallot. 61 Yamamura K...Ohga S 31006598 2019 35 Echocardiographic parameters of severe pulmonary regurgitation after surgical repair of tetralogy of Fallot. 61 [malacards.org]

• 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]

  […] 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]

  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]

  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]

  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]

  pulmonary insufficiency following surgery (J95.1-J95.2) valve I37.1 Murmur (cardiac) (heart) (organic) R01.1 ICD-10-CM Diagnosis Code R01.1 Cardiac murmur, unspecified 2016 2017 2018 2019 2020 Billable/Specific Code Applicable To Cardiac bruit NOS Heart [icd10data.com]

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

• 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]

  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]

  The backward flow of blood into the ventricular chamber during diastole results in a diastolic murmur. [cvphysiology.com]

  Signs include a decrescendo diastolic murmur. Diagnosis is by echocardiography. Usually, no specific treatment is necessary except for management of pulmonary hypertension. [merckmanuals.com]

• 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]

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.

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.