Pulmonic Regurgitation Clinical Presentation
- Author: Xiushui (Mike) Ren, MD; Chief Editor: Richard A Lange, MD, MBA more...
Pulmonic regurgitation is seldom clinically significant. However, symptoms of right-sided heart failure can occur when the severity and duration of the regurgitation result in right ventricular enlargement and decompensation. Dyspnea on exertion is the most common complaint. Easy fatigability, light-headedness, peripheral edema, chest pain, palpitations, and frank syncope may occur in patients with any cause of right-sided heart failure and do little to elucidate the etiology of the right-sided failure. Patients who experience these symptoms may attribute them to poor physical fitness or anxiety, delaying evaluation until their condition worsens. In more advanced presentations of right-sided heart failure, abdominal distension secondary to ascites, right upper quadrant pain secondary to hepatic distension, and early satiety may occur.
Other symptoms specific to the underlying disease process causing pulmonic regurgitation may occur. Such disease processes include connective-tissue disease, infective endocarditis, carcinoid heart disease, rheumatic heart disease, and primary or secondary pulmonary hypertension. For example, hemoptysis is generally not associated with pulmonic regurgitation per se, but, in severe pulmonary hypertension causing pulmonic regurgitation, it may occur as a result of the associated pulmonary arteriole rupture and hemorrhage and/or parenchymal inflammation.
Jugular venous pressure (JVP) is usually increased. Often, an increased A wave is present, but this may be less apparent when significant tricuspid regurgitation with a dominant V wave is also present. When right ventricular enlargement is present, a palpable impulse (lift or heave) is usually present at the left lower sternal border. Palpable pulmonary artery pulsation at the left upper sternal border may be present in the setting of significant pulmonary artery dilatation. With significant pulmonary hypertension, pulmonic valve closure can be palpated.
The pulmonic component of the second heart sound (P2) is inaudible in the absence of a pulmonic valve, whether congenital or secondary to surgical resection. In pulmonic regurgitation due to pulmonary hypertension, P2 is accentuated; with increased right ventricular end-diastolic volume, the ejection time is increased, P2 is delayed, and the S2 split is widened.
A low-pressure regurgitant flow across the pulmonic valve, as occurs when the pulmonary arterial pressure is normal, is heard as a brief, decrescendo early diastolic murmur at the upper left sternal border. It is made louder by squatting or inspiration and softer by Valsalva maneuvers or expiration. An S3 or S4 may be noted at the left mid-to-lower sternal border because of the presence of right ventricular hypertrophy or failure and is augmented by inspiration.
The Graham Steell murmur of pulmonary hypertension is a high-pitched, early diastolic decrescendo murmur noted over the left upper-to-left midsternal area and is a result of high-velocity regurgitant flow across an incompetent pulmonic valve. The regurgitant flow murmur may be present during the whole of diastole because there is a pulmonary-to-right ventricular pressure gradient throughout this time period. Typically, the murmur occurs in severe pulmonary hypertension when the pulmonary artery systolic pressure is more than 60 mm Hg. The quality of this high-pitched early decrescendo diastolic murmur is identical to that of aortic insufficiency. However, the peripheral manifestations of aortic insufficiency are absent. The associated findings of tricuspid regurgitation are frequently present, that is, prominent JVP with surging V waves, holosystolic murmur at the lower left sternal border (louder with inspiration), and enlarged, pulsatile liver.
Significant pulmonic regurgitation occurs variably as a complication of various conditions.
Primary and pulmonary hypertension
Primary pulmonary hypertension occurs in approximately 1 instance per 500,000 cases. This diagnosis can be made only after all other causes have been excluded.
Secondary pulmonary hypertension (multiple causes) is the most common cause of pulmonic regurgitation in adults.
Tetralogy of Fallot
Tetralogy of Fallot, especially with congenital absence of the pulmonary valve or postoperative following surgical repair of this condition (eg, pulmonary valvotomy), may cause significant pulmonic regurgitation.
In rare cases, infective endocarditis results in significant pulmonic regurgitation. It may occur in an intravenous drug user or an individual with an atrial septal defect and a large left-to-right intracardiac shunt.
Rheumatic and carcinoid heart diseases
In rheumatic heart disease leading to significant pulmonic regurgitation, the pulmonary valve is affected following mitral, aortic, and tricuspid valve involvement.
Medications that act via serotoninergic pathways may result in significant pulmonic regurgitation (eg, methysergide, pergolide, fenfluramine).
Disorders that dilate the pulmonic valve ring to create valvular incompetence
These are the most common cause of pulmonic regurgitation and include primary or secondary pulmonary hypertension, dilatation of the pulmonary trunk in Marfan syndrome or Takayasu arteritis, and idiopathic causes
Acquired disorders that alter pulmonic valve morphology
These conditions include the following:
Rheumatic heart disease: In most cases, the other valves (ie, mitral, aortic, tricuspid) are also substantially affected.
Trauma from a Swan-Ganz catheter: This cause is unusual, but it can result if the catheter tip is withdrawn across the pulmonic valve with the balloon inflated.
Complications related to therapeutic balloon catheter dilatation of a stenotic pulmonic valve (eg, pulmonary balloon valvuloplasty). Such complications are not uncommon; however, in most cases, the degree of regurgitation is clinically insignificant, rendering pulmonic valve balloon catheter dilation a safe and effective treatment for moderate-to-severe pulmonic stenosis in adult and pediatric patients.
Carcinoid heart disease: The heart is affected in up to 60% of patients in whom carcinoid has metastasized to the liver, most commonly manifesting as valvular disease. In Pellikka and colleagues' 1993 series of 74 patients, the pulmonic valve was involved in 88%. Of those, 49% exhibited significant pulmonic stenosis, and 81% had significant pulmonic regurgitation. 
Congenital disorders that produce an incompetent pulmonic valve
These include complete absence of the pulmonic valve and valvular abnormalities (eg, fenestrations or redundant leaflets).
Liu S, Xu X, Liu G, Ding X, Zhao X, Qin Y. Comparison of immediate and long-term results between the single balloon and Inoue balloon techniques for percutaneous pulmonary valvuloplasty. Heart Lung Circ. 2015 Jan. 24(1):40-5. [Medline].
Eyskens B, Brown SC, Claus P, Dymarkowski S, Gewillig M, Bogaert J, et al. The influence of pulmonary regurgitation on regional right ventricular function in children after surgical repair of tetralogy of Fallot. Eur J Echocardiogr. 2010 Jan 19. [Medline].
Harrild DM, Powell AJ, Trang TX, Geva T, Lock JE, Rhodes J, et al. Long-term pulmonary regurgitation following balloon valvuloplasty for pulmonary stenosis risk factors and relationship to exercise capacity and ventricular volume and function. J Am Coll Cardiol. 2010 Mar 9. 55(10):1041-7. [Medline].
Pellikka PA, Tajik AJ, Khandheria BK, Seward JB, Callahan JA, Pitot HC, et al. Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients. Circulation. 1993 Apr. 87(4):1188-96. [Medline].
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. 2014 Dec 12. [Medline].
Ristow B, Ahmed S, Wang L, Liu H, Angeja BG, Whooley MA. Pulmonary regurgitation end-diastolic gradient is a Doppler marker of cardiac status: data from the Heart and Soul Study. J Am Soc Echocardiogr. 2005 Sep. 18(9):885-91. [Medline].
Ristow B, Ali S, Ren X, Whooley MA, Schiller NB. 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 Jan 2. 49(1):43-9. [Medline].
[Guideline] Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP 3rd, Guyton RA, et al. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2014 Jun 10. 63(22):2438-88. [Medline].
McElhinney DB, Hellenbrand WE, Zahn EM, Jones TK, Cheatham JP, Lock JE, et al. Short- and Medium-Term Outcomes After Transcatheter Pulmonary Valve Placement in the Expanded Multicenter US Melody Valve Trial. Circulation. 2010 Aug 3. 122(5):507-16. [Medline].
Dodo H, Perloff JK, Child JS, et al. Are high-velocity tricuspid and pulmonary regurgitation endocarditis risk substrates?. Am Heart J. 1998 Jul. 136(1):109-14. [Medline].