Idiopathic mitral valve prolapse with tricuspid, aortic …...myxomatous degeneration of their...
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I NDIAN J OURNAL OF P ATHOLOGY AND M ICROBIOLOGY - 58(2), A PRIL -J UNE 2015 217 Case Report ABSTRACT Mitral valve prolapse (MVP) is usually asymptomatic, but can be associated with complications such as infective endocarditis, mitral regurgitation, thromboembolism and sudden cardiac death. It has been very rarely reported to occur in association with other valvular involvement. A 55-year-old male patient was brought dead and at autopsy the mitral valve orifice was stenotic and the leaflets were enlarged, myxoid and bulging suggestive of MVP and chordae tendinae were thickened, stretched and elongated. Similar changes were seen in the tricuspid valve. The pulmonary and aortic valves also showed myxomatous degeneration of their cusps. Myxomatous degeneration is the most common cause of MVP and it can be associated with involvement of the other valves. Concomitant involvement of the aortic valve has been reported, however it is very rare and simultaneous involvement of the pulmonary valve has not been reported in the literature so far. We report a case of MVP associated with myxomatous degeneration of the tricuspid, pulmonary and aortic valves. KEY WORDS: Aortic, mitral valve prolapse, pulmonary, tricuspid Idiopathic mitral valve prolapse with tricuspid, aortic and pulmonary valve involvement: An autopsy case report Heena M. Desai, Gayathri P. Amonkar Department of Pathology, TN Medical College and BYL Nair Charitable Hospital, Mumbai, Maharashtra, India Address for correspondence: Dr. Heena M. Desai, Department of Pathology, TN Medical College and BYL Nair Charitable Hospital, Dr. A. L. Nair Road, Mumbai Central, Mumbai - 400 008, Maharashtra, India. E-mail: [email protected] INTRODUCTION Mitral valve prolapse (MVP) is an abnormal displacement of the mitral valve into the left atrium during systole. It is usually asymptomatic but can be associated with complications like infective endocarditis, mitral regurgitation, thromboembolism and sudden cardiac death. [1] MVP has been very rarely reported to occur in association with tricuspid and aortic valve involvement. [2] However, pulmonary valve involvement has not been reported with MVP. We report here a case of mitral and tricuspid valve prolapse associated with myxomatous degeneration of the aortic and pulmonary valves. CASE REPORT A 55-year-old male, chronic alcoholic and hypertensive was brought dead to our hospital. At autopsy, significant findings were seen in the heart and brain. The heart was globular and weighed 300 gms. The mitral valve orifice was stenotic, the leaflets were enlarged, myxoid and bulging towards the left atrium with interchordal hooding suggestive of MVP [Figure 1]. The chordae tendinae were thickened, stretched and elongated and there was a small focus of calcification in the posterior mitral leaflet [Figure 2]. Similar changes were seen in the tricuspid valve [Figure 3]. The pulmonary and aortic valves also showed myxomatous degeneration of their cusps [Figures 4 and 5]. There was left atrial dilatation and mild hypertrophy of the left ventricle. Brain showed a thin film of sub- arachnoid hemorrhage. The final cause of death was given as congestive cardiac failure in a case of prolapsed mitral and tricuspid valve with sub-arachnoid hemorrhage. DISCUSSION The prevalence of MVP is variable. The worldwide prevalence of MVP is between 0.4% and 35% whereas Indian prevalence by echocardiography studies is between 2.7% and 16%. [3,4] This wide prevalence could be due to the variety of populations studied, both hospital-based and healthy volunteers with a minority still being unrecognized since most are usually asymptomatic. There are very few autopsy studies on MVP with a reported incidence of about 4%-5% at autopsy. [5,6] MVP is more common in females as compared to males. [3] Myxomatous degeneration is the most common cause of MVP in which there is excessive accumulation of glycosaminoglycan Access this article online Website: www.ijpmonline.org PMID: xxxxxxxxx DOI: 10.4103/0377-4929.155319 Quick Response Code: [Downloaded free from http://www.ijpmonline.org on Monday, June 29, 2015, IP: 115.111.224.207]
Transcript of Idiopathic mitral valve prolapse with tricuspid, aortic …...myxomatous degeneration of their...
I N D I A N J O U R N A L O F P A T H O L O G Y A N D M I C R O B I O L O G Y - 5 8 ( 2 ) , A P R I L - J U N E 2 0 1 5 217
Case R
eport
ABSTRACT
Mitral valve prolapse (MVP) is usually asymptomatic, but can be associated with complications such as infective endocarditis, mitral regurgitation, thromboembolism and sudden cardiac death. It has been very rarely reported to occur in association with other valvular involvement. A 55-year-old male patient was brought dead and at autopsy the mitral valve orifi ce was stenotic and the leafl ets were enlarged, myxoid and bulging suggestive of MVP and chordae tendinae were thickened, stretched and elongated. Similar changes were seen in the tricuspid valve. The pulmonary and aortic valves also showed myxomatous degeneration of their cusps. Myxomatous degeneration is the most common cause of MVP and it can be associated with involvement of the other valves. Concomitant involvement of the aortic valve has been reported, however it is very rare and simultaneous involvement of the pulmonary valve has not been reported in the literature so far. We report a case of MVP associated with myxomatous degeneration of the tricuspid, pulmonary and aortic valves.
KEY WORDS: Aortic, mitral valve prolapse, pulmonary, tricuspid
Idiopathic mitral valve prolapse with tricuspid, aortic and pulmonary valve involvement: An autopsy case reportHeena M. Desai, Gayathri P. AmonkarDepartment of Pathology, TN Medical College and BYL Nair Charitable Hospital, Mumbai, Maharashtra, India
Address for correspondence:Dr. Heena M. Desai, Department of Pathology, TN Medical College and BYL Nair Charitable Hospital, Dr. A. L. Nair Road, Mumbai Central, Mumbai - 400 008, Maharashtra, India. E-mail: [email protected]
INTRODUCTION
Mitral valve prolapse (MVP) is an abnormal displacement of the mitral valve into the left atrium during systole. It is usually asymptomatic but can be associated with complications like infective endocarditis, mitral regurgitation, thromboembolism and sudden cardiac death.[1] MVP has been very rarely reported to occur in association with tricuspid and aortic valve involvement.[2] However, pulmonary valve involvement has not been reported with MVP. We report here a case of mitral and tricuspid valve prolapse associated with myxomatous degeneration of the aortic and pulmonary valves.
CASE REPORT
A 55-year-old male, chronic alcoholic and hypertensive was brought dead to our hospital. At autopsy, significant findings were seen in the heart and brain. The heart was globular and weighed 300 gms. The mitral valve orifice was stenotic, the leaflets were enlarged, myxoid and bulging towards the left atrium with interchordal hooding suggestive of MVP [Figure 1]. The chordae tendinae were thickened, stretched and elongated and there was a small focus of calcification in the posterior mitral leaflet [Figure 2]. Similar changes were seen in the tricuspid valve [Figure 3]. The pulmonary and aortic valves also showed myxomatous degeneration of their cusps [Figures 4 and 5]. There was left atrial dilatation and mild hypertrophy of the left ventricle.
Brain showed a thin f i lm of sub-arachnoid hemorrhage. The final cause of death was given as congestive cardiac failure in a case of prolapsed mitral and tricuspid valve with sub-arachnoid hemorrhage.
DISCUSSION
The prevalence of MVP is variable. The worldwide prevalence of MVP is between 0.4% and 35% whereas Indian prevalence by echocardiography studies is between 2.7% and 16%.[3,4] This wide prevalence could be due to the variety of populations studied, both hospital-based and healthy volunteers with a minority still being unrecognized since most are usually asymptomatic. There are very few autopsy studies on MVP with a reported incidence of about 4%-5% at autopsy.[5,6]
MVP is more common in females as compared to males. [3] Myxomatous degeneration is the most common cause of MVP in which there is excessive accumulation of glycosaminoglycan
Access this article onlineWebsite: www.ijpmonline.orgPMID: xxxxxxxxxDOI: 10.4103/0377-4929.155319Quick Response Code:
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Desai and Amonkar: Idiopathic mitral valve prolapse
material within leaflets and cusps. It can be associated with diseases of tricuspid (commoner) and aortic (less common) valves. Other less common causes include dysfunctional papillary muscles, ruptured chordae tendinae or papillary muscles.[7]
The weight of the heart is increased only in symptomatic MVP but usually remains normal in asymptomatic patients.[8] According to Edwards,[7] the pathological criteria to label the mitral valve as MVP include interchordal hooding (the free margins of the mitral valve leaflet bulges between the chordal insertions) which should be more than 4 mm in height and should involve half of the anterior leaflets or more than 2/3rd of the posterior leaflet of the mitral valve. Whenever the leaflets are voluminous with marked bulging into the left atrium, MVP is usually of a long duration which was seen in our case.
On gross examination of the valve, the deposits can be focal/generalized. With generalized myxomatous degeneration, both atrioventricular and semilunar valves can be affected as was seen
in our case. Semilunar valve cusps are thinner and translucent which makes them prone to aneurysm and rupture.[8] The spongiosa layer of the rough zone is the main site of myxomatous change in MVP.[5]
Patients of MVP with generalized myxomatous degeneration can have simultaneous involvement of other valves as seen in our case where MVP was seen to be associated with tricuspid, aortic and pulmonary valve prolapse. Kasper et al.[9] studied 27 patients with tricuspid valve prolapse and found 8 patients (29.6%) to have concomitant mitral and tricuspid valve prolapse which was statistically significant (P < 0.02). Concomitant involvement of the aortic valve has been reported, however it is very rare and simultaneous involvement of the pulmonary valve has not been reported in the literature so far.[2]
MVP can be associated with complications like infective endocarditis, mitral regurgitation, thromboembolism and sudden cardiac death. Mitral regurgitation is the most common complication of MVP. Sudden death in MVP occurs in less than
Figure 1: Le atrial view showing the steno c mitral valve orifi ce and enlarged, myxoid leafl ets bulging towards the le atrium with interchordal hooding
Figure 2: Opened up mitral valve showing thickened, stretched and elongated chordae tendinae
Figure 3: Cut open tricuspid valve showing enlarged myxoid leafl ets sugges ve of tricuspid valve prolapse
Figure 4: Opened up aor c valve showing myxomatous degenera on of its cusps
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Desai and Amonkar: Idiopathic mitral valve prolapse
1% of cases; usually occurring secondary to ruptured chordae tendinae and mitral valve leaflets. Arrythmias can occur but are rare.
In our case, we had a stenotic mitral valve, which is extremely rare in MVP. Furthermore, to the best of our knowledge, this is the first reported case of quadruple valve involvement in association with MVP.
REFERENCES
1. St John Sutton M, Weyman AE. Mitral valve prolapse prevalence and complications: An ongoing dialogue. Circulation 2002;106:1305-7.
2. Ribeiro CL, Ginefra P, Albanesi Filho FM, Christiani LA, Quaresma JC, Gomes Filho JB. Prevalence of tricuspid and aortic valve prolapse in patients with mitral valve prolapse. Arq Bras Cardiol 1989;53:251-5.
3. Sattur S, Bates S, Movahed MR. Prevalence of mitral valve prolapse and associated valvular regurgitations in healthy teenagers undergoing screening echocardiography. Exp Clin Cardiol 2010;15:e13-5.
4. Cheng TO. Mitral valve prolapse: The Merchant of Venice or The Tales of Hoffman? European Heart Journal 2002; 23, 87-8.
5. Watanabe C, Sugiura M, Ohkawa S, Ito Y, Toku A, Maeda S, et al. Pathology and histochemistry of mitral valve prolapse. J Cardiol 1993;23:69-77.
6. Anders S, Said S, Schulz F, Püschel K. Mitral valve prolapse syndrome as cause of sudden death in young adults. Forensic Sci Int 2007;171:127-30.
7. Edwards JE. Pathology of mitral incompetence. In: Silver MD, editor. Cardiovascular Pathology. New York, Edinburg: Churchill Livingstone; 1983. p. 575-98.
8. Virmani R, Atkinson JB, Forman MB. The pathology of mitral valve prolapse. Herz 1988;13:215-26.
9. Kasper W, Meinertz T, Weber T, Geibel A, Just H. Incidence of tricuspid valve prolapse. Z Kardiol 1991;80:333-7.
Figure 5: Opened up pulmonary valve showing myxomatous degenera on of its cusps
How to cite this article: Desai HM, Amonkar GP. Idiopathic mitral valve prolapse with tricuspid, aortic and pulmonary valve involvement: An autopsy case report. Indian J Pathol Microbiol 2015;58:217-9.
Source of Support: Nil, Confl ict of Interest: None declared.
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