|Year : 2014 | Volume
| Issue : 2 | Page : 225-228
A case of submitral left ventricular aneurysm (SMA), presenting with acute rheumatic fever with HIV infection
Rajeshwari S Vohra1, Deelip B Kadam2, Narendra C Kale2
1 Department of Emergency Medicine, Padmashree Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth, Pune, Maharashtra, India
2 Department of Medicine, B. J. Medical College and Sassoon General Hospital, Pune, Maharashtra, India
|Date of Web Publication||4-Feb-2014|
Rajeshwari S Vohra
D3 Clover Garden, 4 Naylor Road, Off Mangaldas Road, Pune - 411 001, Maharashtra
Source of Support: None, Conflict of Interest: None
Submitral aneurysm (SMA) is a rare congenital out-pouching of left ventricular wall, invariably occurring adjacent to the posterior leaflet of mitral valve.
We describe a patient who had rare co existence of SMA, rheumatic heart disease and HIV infection. Our patient presented with acute rheumatic fever in acute decompensated heart failure (ADHF) profile B. Patient was also detected to be having HIV infection. On echocardiography submitral left ventricular aneurysm posteriorely below the posterior mitral leaflet (PML) was detected.
Keywords: Acute rheumatic fever, Submitral left ventricular aneurysm, HIV infection
|How to cite this article:|
Vohra RS, Kadam DB, Kale NC. A case of submitral left ventricular aneurysm (SMA), presenting with acute rheumatic fever with HIV infection. Med J DY Patil Univ 2014;7:225-8
|How to cite this URL:|
Vohra RS, Kadam DB, Kale NC. A case of submitral left ventricular aneurysm (SMA), presenting with acute rheumatic fever with HIV infection. Med J DY Patil Univ [serial online] 2014 [cited 2019 Nov 20];7:225-8. Available from: http://www.mjdrdypu.org/text.asp?2014/7/2/225/126355
| Introduction|| |
Submitral left ventricular aneurysm is a rare cardiac pathology, recognized but with relatively sparse description in the literature. It is a congenital out-pouching of left ventricular wall, invariably occurring adjacent to the posterior leaflet of mitral valve.  Two-thirds of the mitral annulus is related to the posterior leaflet which is attached to the myocardium of the left ventricle (LV) by annular ring. The immediate external relationship of the mitral ring is the epicardium of the atrioventricular groove. A dehiscence of this muscular-fibrous union results in the invariable occurrence of the SMA below the mitral leaflet.  SMA was first described in 1812 by Corvisart and, since then, only 100-120 cases of these aneurysms have been reported worldwide. 
It was first reported from Nigeria and other African countries by Abrahams et al. where 12 patients with an unusual form of left ventricular aneurysm were described which they termed as ''annular left ventricular aneurysm.''  Cases have been described from all over the world with few case reports from India as well. 
The case we describe highlights the importance of considering SMA in the differential diagnosis of mitral regurgitation with left ventricular dysfunction and heart failure in the young.
| Case Report|| |
A 21-year-old married female admitted in medical ward was presented with history of palpitations, orthopnea, joint pain and swelling, edema of feet since 8 to 10 days. All symptoms were progressively increasing in severity. Patient had similar episode 8 to 10 months ago for which she was admitted and treated in hospital and was partially relieved. She had history of spontaneous abortion at 4 to 6 weeks 3 to 4 months prior to her complaints. No major medical problems in past was recorded. Patient had normal age of menarche and regular menstrual periods.
On physical examination she had toxic look, heart rate was 140/min regular, BP was 140/80 mm of mercury, respiratory rate was 30/min, Jvp was raised, bilateral pitting edema feet was present. She had swelling of both ankle joints. On CVS examination she had systolic thrill in mitral area. She had pansystolic murmur of grade 4 by 6, summation gallop with P2 loud. Clinical diagnosis of acute rheumatic fever, with moderate to severe MR with pulmonary hypertension with cardiac failure was made. Differential diagnosis of acute bacterial endocarditis was also kept.
Laboratory parameters revealed that her Hb was 10.1 and ESR was 50. ASO was positive (350 t odds unit), and CRP was raised. Throat swab and its culture showed Gram positive Cocci in chains, blood cultures were sent which were negative for growth. HIV was positive by ELISA. Apart from sinus tachycardia no other abnormality was detected on ECG. She underwent transthoracic two dimensional echocardiography (2-D Echo) with color Doppler study which revealed grade 4 mitral regurgitation, grade 2 aortic regurgitation, and submitral aneurysm seen posteriorly below PML, [Figure 1]. SMA was seen as well-defined out pouching in left AV groove in its posterior aspect in submitral location communicating with left ventricle [Figure 2]. LV was dilated with poor contractility. There was no evidence of vegetation. A to-and-fro flow was seen on color Doppler [Figure 3]. With these findings the patient was diagnosed as having a submitral aneurysm of the left ventricle.
|Figure 2: Trans Thoracic 2 D Echocardiography showing out-pouching of PML|
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|Figure 3: Trans thoracic color Doppler showing to and fro movement of blood in SMA|
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She was started with treatment of acute rheumatic fever with aspirin; 80 mg/ kg body wt. and benzathine penicillin 1.2 million unit IM. She was also treated with treatment of cardiac failure. (frusemide, digoxin). Symptomatically patient did not improve and tachycardia persisted. She was then started on prednisolone 1 mg / kg body weight. In view of positive throat swab culture she was given antibiotics. Her symptoms improved and murmur and gallop disappeared. Heart rate was 80/min. Our patient had a rare combination of acute rheumatic fever, HIV infection and SMA.
In view of HIV positive status, she was investigated with CD4 count and viral load and started with anti retroviral therapy (ART). She was advised definitive surgery for SMA.
Discussion and Review of Literature
As our patient had coexisting acute rheumatic fever, HIV infection, and SMA, the literature was reviewed and it was found that the etiology of heart muscle disease in HIV infection is unknown.  Given that most HIV infection occurs in the developing world, conditions such as rheumatic heart disease may contribute to cardiac complications in HIV infection even though a significant interaction has not yet been noted. 
In our patient SMA was congenital or secondary to acute rheumatic fever and/or HIV infection could not be ascertained.
Patients with HIV infection are often unable to mount an appropriate serological response. Rheumatic heart disease should be considered in HIV positive patients from endemic areas who have evidence of heart disease. Clinical and laboratory features may be modified by immunodeficiency associated with HIV. 
A study conducted in Bostwana to assess how HIV influences spectrum of heart disease in Bostwana showed 8.4% had Echocardiographic evidence of rheumatic heart disease in HIV positive patients. 
As our patient had rare coexistence of acute rheumatic fever, HIV infection and SMA literature was reviewed for SMA and it was found that Submitral aneurysm is a rare cardiac pathology mostly seen in African countries, particularly in the black population. It is considered to be a false aneurysm caused by a congenital defect in the posterior portion of the mitral annulus. Other etiologies such as tuberculosis, Takayasu arteritis, and mitral valve endocarditis have been proposed. Although it can present rarely with life threatening complications such as ventricular tachycardia caused by compression of the left main coronary artery, the most common presentation is as severe mitral regurgitation.
Echocardiography usually shows it as a subpericardial echo-free space below the mural leaflet of the mitral valve, which communicates with the ventricular cavity. 
Diagnosis of SMA 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.
African blacks account for most of the reported SMA cases. Many of these patients present with New York Heart Association (NYHA) functional class III or IV symptoms. ,
Other reported complications of SMA, including thromboembolism, coronary compression, diastolic dysfunction, and malignant arrhythmias, can rapidly progress to death. ,
A submitral aneurysm is a congenital out pouching 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. ,, In most cases, the neck of the aneurysm is found beneath the posterior mitral valve annulus, at any site between the anterolateral and posteromedial commissures.  It has been postulated that the disease may be secondary to congenital weakness in the left ventricular wall-mitral apparatus junction, but further research is needed. , Acquired cases of SMA have been reported in conjunction with Takayasu's arteritis, following mitral valve endocarditis or mitral valve replacement, and as a sequela to electrophysiology studies. ,
Doppler echocardiography has proved to be a useful technique for the noninvasive diagnosis of submitral aneurysms in the clinical setting. Management of SMA involves initial medical stabilization with diuretics and afterload-reducing agents. Surgical repair is the definitive treatment and includes pericardial patch repair,  valvuloplasty through a transmitral approach,  transatrial repairs with sutures,  or mitral valve replacement. 
The literature is reviewed to increase the awareness of clinicians, especially echocardiographers about this rare cardiac disorder when coming across congestive cardiac failure with mitral regurgitation in young population. 
Submitral aneurysm is rare and most cases have been described in black and Negroid people. Sporadic case reports in the last two decades have documented their existence in the Indian population. Even though the etiology of the condition is thought to be congenital Takayasu's arteritis  and tuberculous pericarditis  has been reported. Definitive treatment is surgical.  Mortality is high in un-operated cases and operative repair under cardiopulmonary bypass is the most appropriate management. Post-operative outcome is directly related to size of the aneurysm and severity of LV dysfunction.
| Conclusion|| |
SMA is a rare condition, which can be primary or secondary. It should be considered in young patients who have presented with severe mitral regurgitation in decompensated state. Rheumatic heart disease should be considered in HIV positive patients from endemic areas who have evidence of heart disease.
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[Figure 1], [Figure 2], [Figure 3]