Case

Reports

Perforated Submitral
Left Ventricular Aneurysm
Resulting in Severe Mitral Annular Regurgitation

Leo Simpson, MD
J. Michael Duncan, MD
Raymond F. Stainback, MD

Annular submitral left ventricular aneurysm, which predominantly occurs in blacks who
live in tropical regions of Africa, is a relatively unknown cardiac condition in the United
States. We describe a patient with submitral left ventricular aneurysm who underwent
resection of the mass and of the native mitral valve, followed by mitral valve replacement.
(Tex Heart Inst J 2006;33:492-4)

lthough annular submitral left ventricular aneurysm (SLVA) is an unusual

and relatively unknown cardiac condition in the United States, it has been
reported periodically in the world medical literature. Initially described by
Abrahams and colleagues1 in 1962 and subsequently by Chesler and coworkers,2 this
disorder occurs predominantly in blacks who live in tropical regions of Africa,3 but it
has been described in other racial groups as well.4-6 A diagnosis of SLVA is typically
made in the 2nd or 3rd decade of life. Associated findings on presentation include severe mitral regurgitation, heart failure, systemic embolism, and sudden cardiac death.
We report the case of a patient with symptomatic, perforated SLVA who required
surgical resection of the defect and mitral valve replacement.

Case Report

Key words: African continental ancestry group;

aneurysm, submitral left
ventricular; heart aneurysm;
mitral valve/abnormalities;
mitral valve replacement
From: Department of Adult
Cardiology, Texas Heart
Institute at St. Lukes
Episcopal Hospital,
Houston, Texas 77030
Address for reprints:
Raymond F. Stainback, MD,
Texas Heart Institute at St.
Lukes Episcopal Hospital,
P.O. Box 20345, MC 1-133,
Houston, TX 77225-0345
E-mail:
rstainback@sleh.com
2006 by the Texas Heart
Institute, Houston

492

A 35-year-old American black man with no history of medical problems came to

our institution with a 1-month history of fatigability, weight loss, exertional shortness of breath, orthopnea, and paroxysmal nocturnal dyspnea. The physical examination revealed clinical signs of congestive heart failure. Cardiac auscultation
revealed a displaced apex, a soft S1, a left ventricular S3, and a grade 3/6 holosystolic
murmur at the apex that radiated to the left shoulder and back. Transthoracic and
transesophageal echocardiograms showed a 2.5 3.5-cm thick-walled aneurysm
attached to the intervalvular fibrosa, anterior mitral valve leaflet, and anterior and
lateral mitral annulus. The aneurysm occupied the anterior and lateral 50% of the
left atrium (Figs. 1A, 2, and 3A). Echocardiographic 2-dimensional and color Doppler imaging revealed an extensive communicating neck within the mitral annulus,
through which left ventricular blood entered a balloon-like aneurysmal cavity and
then escaped into the left atrium via an aneurysmal perforation (Figs. 1B and 3B).
Perforation of the extensive annular aneurysm (possibly acutely) produced the hemodynamic equivalent of severe mitral regurgitation and heart failure, although leakage
along the mitral leaflet coaptation line could not be demonstrated.
The patient underwent resection of the mass and of the native mitral valve, followed by mitral valve replacement with a St. Jude Silzone valve prosthesis* (St.
Jude Medical, Inc.; St. Paul, Minn). Intraoperative findings included a gelatinous
tumor that involved the anterior leaflet of the mitral valve and extended into
the left ventricle. Extensive mitral annular connective tissue degeneration precluded
preservation of the native leaflets. Histopathologic examination of the resected valve
showed a benign myxofibromatous lesion in a collagenous, vascularized stroma, with
focal areas of fibrinoid necrosis. Results of a Gram stain and stains for fungal and
atypical organisms were negative, as were bacterial, fungal, and acid-fast bacillus
* FDA approval for the Silzone-coated valve came in March 1998 (for the SJM Masters) and in October 1998 (for the Seguin Ring); on 21 January 2000, St. Jude Medical recalled all products with
the Silzone coating, after reports of endocarditis and paravalvular leak. In our judgment, this defect
had no bearing on the outcome of the case reported here.

Submitral Left Ventricular Aneurysm

Volume 33, Number 4, 2006

LV

Ao

Fig. 1 A) Transesophageal echocardiogram (TEE), 4-chamber

view, shows lateral periannular communication (broken arrow)
between the left ventricle (LV) and the submitral left ventricular
aneurysm that is situated within the left atrium (solid arrow).
B) Color-flow Doppler in the same TEE view shows torrential
systolic flow (broken arrow) from the LV into the aneurysm, with
subsequent aneurysm-to-left atrial flow via the aneurysmal perforation (arrowhead).

Fig. 3 A) Transesophageal echocardiogram (TEE), 3-chamber

view shows the aneurysm extending to the intervalvular fibrosa
(arrow). B) Color-flow Doppler in the same TEE view demonstrates turbulent high velocity systolic flow within the aneurysm.
Ao = aortic root; LV = left ventricle
Real-time motion images are available at texasheart.org/journal.

Real-time motion image is available at texasheart.org/journal.

LV

Postoperatively, the patient did well at first, and he

was discharged. Six months later, he was readmitted to
the hospital for recurrent congestive heart failure due to
partial prosthetic valve dehiscence. He eventually died
of recurrent valve dehiscence due to inadequate annular
connective tissue.

Discussion
LA

Fig. 2 Transesophageal echocardiogram, 2-chamber view,

further demonstrates the site of aneurysmal perforation (arrowhead).
A = aneurysm; LA = left atrium; LV = left ventricle
Real-time motion image is available at texasheart.org/journal.

(AFB) cultures. Results of rheumatologic and infectious workups were also negative.
Texas Heart Institute Journal

African blacks account for most of the reported SLVA

cases. Many of these patients present with New York
Heart Association (NYHA) functional class III or IV
symptoms.4,7 Other reported complications of SLVA,
including thromboembolism, coronary compression,
diastolic dysfunction, and malignant arrhythmias, can
rapidly progress to death.8-11 Our patient eventually died
of multiple instances of prosthetic valve dehiscence secondary to inadequate annular connective tissue.
A submitral aneurysm is a congenital outpouching of
the left ventricular wall, invariably occurring adjacent
to the posterior leaflet of the mitral valve, often in the
region of the atrioventricular groove and in the absence
of ischemic, infective, or traumatic disease.1,12,13 In most
Submitral Left Ventricular Aneurysm

493

cases, the neck of the aneurysm is found beneath the

posterior mitral valve annulus, at any site between the
anterolateral and posteromedial commissures.7 It has
been postulated that the disease may be secondary to
congenital weakness in the left ventricular wallmitral
apparatus junction, but further research is needed.14,15
Acquired cases of SLVA have been reported in conjunction with Takayasus arteritis, following mitral valve endocarditis or mitral valve replacement, and as a sequela
to electrophysiology studies.16,17
Doppler echocardiography has proved to be a useful
technique for the noninvasive diagnosis of submitral aneurysms in the clinical setting. Management of SLVA
involves initial medical stabilization with diuretics and
afterload-reducing agents. Surgical repair is the definitive
treatment and includes pericardial patch repair,18 valvuloplasty through a transmitral approach,7 transatrial repairs
with sutures,19 or mitral valve replacement.20

References
1. Abrahams DG, Barton CJ, Cockshott WP, Edington GM,
Weaver EJ. Annular subvalvular left ventricular aneurysms.
Q J Med 1962;31:345-60.
2. Chesler E, Joffe N, Schamroth L, Meyers A. Annular subvalvular left ventricular aneurysms in the South African Bantu.
Circulation 1965;32:43-51.
3. Gaultier Y, Cenac A, Aoua HO, Toure I. Idiopathic annular
submitral aneurysm. Contribution of echography apropos
of 5 cases [in French]. Arch Mal Coeur Vaiss 1989;82:897902.
4. Sharma S, Daxini BV, Loya YS. Profile of submitral left ventricular aneurysms in Indian patients. Indian Heart J 1990;
42:153-6.
5. Guimaraes AC, Filho AS, Esteves JP, Abreu WN, Vinhaes
LA, de Almeida Souza JA, Machado A. Annular subvalvular
left ventricular aneurysm in Bahia, Brazil. Br Heart J 1976;
38:1080-5.
6. Ribeiro PJ, Mendes RG, Vicente WV, Menardi AC, Evora
PR. Submitral left ventricular aneurysm. Case report and review of published Brazilian cases. Arq Bras Cardiol 2001;76:
395-402.

494

Submitral Left Ventricular Aneurysm

7. Antunes MJ. Submitral left ventricular aneurysms. Correction by a new transatrial approach. J Thorac Cardiovasc Surg
1987;94:241-5.
8. Janeira LF, Talit U, Parker R, Hughes CE, Tuna IC. Surgical management of ventricular tachycardia in subannular left
ventricular aneurysm. Ann Thorac Surg 1995;60:438-40.
9. Normann SJ. Annular subaortic aneurysm resulting in sudden death. Clin Cardiol 1991;14:68-72.
10. Chi NH, Yu HY, Chang CI, Lin FY, Wang SS. Clinical surgical experience of congenital submitral left ventricular aneurysm. Thorac Cardiovasc Surg 2004;52:115-6.
11. Purushotham S, Manohar SR, Sivasubramaniam S, Neela
kandhan KS. Submitral left ventricular aneurysm: the location
of the circumflex coronary artery in relation to the aneurysm influences the surgical approach and outcome. J Thorac
Cardiovasc Surg 2005;129:1175-7.
12. Chockalingam A, Gnanavelu G, Alagesan R, Subramaniam
T. Congenital submitral aneurysm and sinus of valsalva
aneurysm. Echocardiography 2004;21:325-8.
13. Chesler E, Mitha AS, Edwards JE. Congenital aneurysms
adjacent to the anuli of the aortic and/or mitral valves. Chest
1982;82:334-7.
14. Dalvi BV, Sathe SV, Lokhandwala YY, Kulkarni HL, Kale
PA. Coexistence of congenital submitral and aortic sinus aneurysms. Am Heart J 1990;119(2 Pt 1):419-21.
15. Chen CC, Hsiung MC, Wei J, Chang WT, Yin WH, Young
MS. Mitral annular subvalvular left ventricular aneurysm.
Echocardiography 2005;22:434-7.
16. Rose AG, Folb J, Sinclair-Smith CC, Schneider JW. Idio
pathic annular submitral aneurysm associated with Takayasus aortitis. A report of two cases. Arch Pathol Lab Med
1995;119:831-5.
17. Sutorius DJ, Helmsworth JA, Majeski JA, Miller SF. Repair
of a subvalvular left ventricular aneurysm following mitral
valve replacement. Ann Thorac Surg 1981;32:92-6.
18. Almeida-Filho OC, Schmidt A, Sgarbieri R, Marin-Neto
JA, Maciel BC. Large submitral left ventricular aneurysm
associated with mitral valve aneurysm. Echocardiography
2002;19:391-3.
19. Gokhale AG, Lal N, Ashok B, Jacob J, Krishnaswami S,
Jairaj PS. Mitral valve replacement for an annular submitral
aneurysm of the left ventricle. Thorax 1993;48:676-7.
20. Esposito F, Renzulli A, Festa M, Cerasuolo F, Caruso A, Sarnicola P, Cotrufo M. Submitral left ventricular aneurysm.
Report of 2 surgical cases. Tex Heart Inst J 1996;23:51-3.

Volume 33, Number 4, 2006