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International J. of Healthcare and Biomedical Research, Volume: 04, Issue: 01, October 2015, Pages 174-178 Case Report: Congenital sub mitral left ventricle aneurysm with normal mitral valve - A rare presentation Dr. Santosh Kumar Pandey1*, Dr.SubhenduSekhar Mahapatra1, Dr.Uttam Kumar Mukhopadhyay1, Dr.Swarnendu Datta1, Dr.suruchi Pandey2, Dr.Gautham Shetty1, Dr.Debajyoti Mandal1 1Department of Cardiothoracic and vascular sciences, IPGME&R and SSKM Hospital,Kolkata,West Bengal, India zip code 700020. 2Department of cardiology, RTIICS, Mukundapur, Kolkata, West Bengal. *Corresponding author: Dr.Santosh Kumar Pandey, Mch – PDT, Department of Cardiothoracic and vascular sciences,IPGME&R and SSKM Hospital,Kolkata,West Bengal, India- 700020. Abstract Submitral Left Ventricular Aneurysm (SMLVA) is an unusual, non-ischemic Left Ventricular (LV) Aneurysm. SMLVA is rarely reported in Indian subcontinent. It is most commonly found in Black population, among the natives of south and West Africa.SMLVA is basically a congenital out-pouching of the LV wall occurring adjacent to the posterior leaflet of Mitral Valve2. It is typically diagnosed in young adults, presenting mostly with features of Mitral regurgitation, congestive heart failure, systemicembolization, arrhythmia and sometimes sudden death. But in our case mitral valve was almost normal and the patient presented with progressive worsening dyspnoea,palpitation and occasional chest pain secondary to gradual decline in ejection fraction .His coronary angiogram was normal. Differential diagnosis of SMLVA must be kept in mind when a young individual present with features of progressive LV dysfunction though it is rare. Key words: Submitral left ventricular aneurysm, LV dysfunction, congenital. Introduction Few case of SMA has been reported in Caucasians Sub Mitral Left Ventricular aneurysm is an in which all presented with significant Mitral uncommon regurgitation. cardiac lesion with very little description in the Indian literature. In 1962 Abrahams etal 1 reported 12 patients with an Our patient is a rare presentation of a large SMA without involvement of Mitral Valve Apparatus. unusual form of Left Ventricular aneurysm from He Nigeria and other African countries and they occasional chest pain and palpitation. Our case termed as “Annular Subvalvular left ventricle emphasise on considering a differential diagnosis aneurysm”. The anatomy of mitral valve annulus is of SMA in young adult presenting with features of such that its 2/3 portion is related to posterior low ejection fraction or LV dysfunction. leaflet which is attached to LV myocardium The typical location of the aneurysm with normal 3 presented with progressive dyspnoea, through annular ring . A weakness or disruption of coronaries on angiography confirms the diagnosis this muscular–fibrous union results in occurrence of SMLVA. Surgical correction is mandatory to of submitral aneurysm below mitral leaflet. Cases treat the cardiac failure secondary to increasing size of SMLVA has been reported from all parts of the of the aneurysm and to prevent the potential 4,5 world but very few from Indian subcontinent . cardiovascular events. 174 www.ijhbr.com ISSN: 2319-7072 International J. of Healthcare and Biomedical Research, Volume: 04, Issue: 01, October 2015, Pages 174-178 Case Report A 23 years young male presented to our department with progressive worsening dyspnoea for last 1 ½ years associated with occasional chest pain and palpitation. There was no past history of rheumatic fever, any episode of syncopal attack, asthma or Tuberculosis. No family history of any cardiovascular disease. On examination he had heart rate of 110 per minute, regular rate and rhythm, Blood pressure of 90/60mmHg, JVP was raised and had pedal oedema. The apex was in the th 6 left intercostal space,2 cm outside the mid Fig 1:- Showing relatively small LV with a large aneurysmal sac (AN) clavicular line. On auscultation, soft S1 and Left ventricular S3 were present. Chest auscultation revealed bilateral basal crepitation. All peripheral pulses were bilaterally and equally palpable. ECG showed evidence of sinus tachycardia, Chest X- ray revealed cardiomegaly with evidence of pulmonary venous congestion. Echocardiography finding revealed normalsize Right and left atrium. Right ventricular outflow tract was also normal in size.Left ventricle was dilated(60mm) with a large aneurysm,present in the inferior wall in submitral position. Mitral valve, Tricuspid valve and Aortic valve were normal with significant reduction of Fig.2- Showing normal angiogram. ejection fraction (<30%), Interventricular septum He was normal and moderate pericardial effusion was Conventional present. Cardiopulmonary bypass was achieved by aortic Cardiac MRI was done which shows normal LA and bicaval cannulation. The aorta was cross and LV was relatively small. LV shows a large clamped saccular defect in the inferior wall, in submitral myocardial protection was provided with cold region. The saccular lesion measures 6.1x4.5x4.0 blood antegrade root cardioplegia.We found dense cm with a neck measuring 4.4cm in diameter. The adhesion between the sac andpericardium.We angiogram is suggestive of normal coronaries. approached through left atrium. Mitral valve was was planned median under for surgical sternotomy moderate correction. was hypothermia done. and assessed after opening left atrium which was normal. The aneurysm was approached from outside(external approach). Aneurysm was opened, the clots and debris was removed, and excess wall of the aneurysm was excised keeping healthy LV margins. The ‘neck’ of the aneurysm was closed with interrupted pledgetted 3–0 Polypropylene 175 www.ijhbr.com ISSN: 2319-7072 International J. of Healthcare and Biomedical Research, Volume: 04, Issue: 01, October 2015, Pages 174-178 horizontal mattress sutures in such a way that, the sutures goes through the neck of the aneurysm and through the posterior mitral annulus. The defect in the left ventricle was narrowed down with 2–0 Polypropylene purse string suture and the defect was closed Polypropylene with PTFE interrupted Felt using pledgetted 2–0 sutures. Mitral valve was tested and was found to be competent. Left atrium was closed directly. Intraoperative pictures: Fig.5-The aneurysmal sac being opened and the clots and debris removed. Fig.3- Showing dense adhesion of the sac with the pericardium. Fig.6-The neck of the sac being closed with PTFE graft. Fig.4-The aneurysmal sac is separated with a patch of pericardium which could not be separated. Fig.7- The aneurysmal sac cut edge being closed. Postoperative echocardiography was done which shows normal mitral valve with no gradient across 176 www.ijhbr.com ISSN: 2319-7072 International J. of Healthcare and Biomedical Research, Volume: 04, Issue: 01, October 2015, Pages 174-178 the valve. Patient was discharged in stable absence of coronary artery disease on angiography condition after 7 days. He was followed up after 3 confirm the diagnosis of SMLVA. Surgical months with echo which suggest no mitral treatment is mandatory as soon as the diagnosis of regurgitation and ejection fraction of 51%. SLVA is made, in order to treat the cardiac failure Discussion that arises from expansion of the aneurysm and to LV aneurysm is more common occurrence in post avoid other potential cardiovascular events. The myocardial inherent infarction patients.Submitral Left Ventricular Aneurysm (SMLVA) is a rare lesion 1 that occurs most often in the blacks and still rarest 4,5 difficulties extracardiac associated approach with included the inadequate exposure of the mitral annulus, residual mitral in Indian population . The prevalence of this regurgitation, lesion among blacks appears to indicate a approaching the aneurysm due to adhesions. 6 congenital predisposition . Antunes described the transatrial approach in SMLVA is basically a congenital outpouching of 19872. We approached through both extra and intra the LV wall occurring adjacent to the posterior cardiac approach. Surgical failure can be related to 2 and technical difficulties in leaflet of Mitral Valve .It is typically diagnosed in the failure to identify additional aneurysm necks or young adults,presenting mostly with features of inadequate closure of the aneurysm and lack of Mitral heart support to the mitral annulus leading to recurrent and aneurysm formation. Successful repair is dependent regurgitation, failure,systemic congestive embolization,arrhythmia sometimes sudden death. Submitral left ventricular on aneurysm seems to be caused by a junctional defect relationship between the aneurysm, the mitral valve between the cardiac muscle and the fibrous and its annulus. If mitral regurgitation is associated 2 the appropriate under-standing of the structure of the heart . The anatomy of mitral valve with aneurysm then it is advisable to do mitral ring annulus is such that its 2/3 portion is related to annuloplasty than direct suturing to prevent posterior recurrence leaflet which is attached to LV of regurgitation. Mitral Valve myocardium through annular ring. A weakness or Replacement (MVR) is one more option in cases disruption of this muscular–fibrous union results in where mitral valve leaflets are distorted or occurrence of submitral aneurysm below mitral damaged and when it is non-repairable. Also the 3 leaflet . Clinical symptoms arise as a result of mitral valve replacement may be an option in cases valvular of postoperative severe mitral regurgitation. regurgitation compression of or cardiac occasionally structures by from the 2 Conclusion aneurysmal sac or patient can be asymptomatic for SMLVA is very rare cardiac lesion in Indian many years5].In our patient mitral valve was subcontinent. The patient may not always present almost normal and the patient presented with with symptoms of mitral regurgitation but with a progressive worsening dyspnoea,palpitation and normal mitral valve, as in our case. . Differential occasional chest pain secondary to gradual decline diagnosis of SMLVA must be kept in mind when a in ejection fraction..]. Diagnosis by chest x ray is young easy if calcification is present in the aneurysm progressive LV dysfunction though it is rare. 7 wall . Now days, the transthoracic echocardiography is the most accurate diagnostic individual present with features of Echocardiography is sufficient to make the diagnosis. Surgery is the treatment of choice. 8 tool . The typical location of the aneurysm and the 177 www.ijhbr.com ISSN: 2319-7072 International J. of Healthcare and Biomedical Research, Volume: 04, Issue: 01, October 2015, Pages 174-178 Acknowledgment : I am very thankful to the Professor S. Bhattacharya and Professor Gautam cardiology department of our institute for their Sengupta encouraged us to prepare this case report. prompt support. Our head of the department Dr. Conflict of Interest: There is no conflict of interest. References 1. Abrahams DG, Barton CJ, Cockshott WP, Edington GM, Weaver EJ. Annular subvalvular left ventricular aneurysm. Q J Med. 1962;31:345–60. 2. Antunes MJ. Submitral left ventricular aneurysms. Correction by a new transatrial approach. J ThoracCardiovascSurg 1987; 94: 241-245. 3. Chesler E, Mitha AS, Edwards JE. Congenital aneurysms adjacent to the annuli of the aortic and/or mitral valves. Chest 1982; 82: 334-337 4. Chockalingam A, Gnanavelu G, Alagesan R, Subramaniam T. Congenital submitral aneurysm. Echocardiography 2004; 21: 325-328 5. Sharma S, Daxini BV, Loya YS. Profile of Submitral Left Ventricular Aneurysm in Indian Patients. IHJ 1990; 42:153-6. 6. Beck W, Schrire V. Idiopathic mitral sub annular left ventricular aneurysm in the Bantu. Am Heart J. 1969;78:28–33 7. Szarnicki RJ, De Leval MR, Stark J. Calcified left ventricular aneurysm in 6-year-old Caucasian boy. Br Heart J. 1981;45:464–6. 8. Davis MD, Caspi A, Lewis BS, Milner S, Colsen PR, Barlow JB. Two-dimensional echocardiographic features of submitral left ventricular aneurysm. Am Heart J. 1982;103:289–90. 178 www.ijhbr.com ISSN: 2319-7072