Submitral Aneurysms Research Articles

P480 SUBMITRAL LEFT VENTRICULAR ANEURYSM AND STROKE IN A YOUNG CAUCASIAN WOMAN

Abstract Submitral aneurysms are infrequent and they are generally described in populations from Subsaharian Africa. The aetiology is still unclear: it has been proposed that they could be caused by congenital alterations of ventricular wall, infectious events, chest trauma, cardiac surgery or Myocardial Infarction. Submitral aneurysms can induce arrhythmias, stroke, heart failure secondary to mitral insufficiency, and can facilitate infections. We report the case of a 39–year–old Caucasian woman who was hospitalized in our Institution after relapsing fever episodes, presenting with altered state of consciousness. The ECG showed sinus rhythm and normal morphology. When she was hospitalized, laboratory test findings included normocytic anaemia (Hb 9,8 g/dl), thrombocytopenia (94x103 platelets), neutrophilia (81% neutrophils), ESR 32 mm/h, CRP 8,84, low electolyte level, procalcitonin 11,54 ng/ml. Blood culture was positive for Candida Albicans. A transthoracic echocardiogram showed normal left ventricle dimensions and wall thickness, without abnormalities of global or segmental wall motion, thickening of mitral valve leaflets, which appeared slightly hyperechogen. It was found an aneurysmatic area in correspondence of subvalvular apparatus of mitral valve, between mitral annulus and posterior papillary muscle. Mitral annulus was shifted toward the atrial roof. These findings were confirmed by a thransesophageal echocardiogram. The aneurysm measured 33x22 mm in correspondence to the atrio–ventricular junction. A cardiac MRI confirmed these morphologic alterations without evidence of infarction areas, supposing the presence of an infectious lesion. A brain MRI showed multiple and diffuse areas of altered signal referred to stroke. The patient was referred to the Heart Surgery of our Institution. Intraoperatively, a large aneurysm arose from submitral area; moreover, the posterior leaflet of the mitral valve was characterized by some fissures. The aneurysm was excluded by a direct suturing and the mitral valve was replaced with a biological valve. In our patient, mitral annulus dislocation was evident, supporting the hypothesis of a congenital malformation that can have promoted a mycotic infection and the formation of thrombi responsible for the cerebral disease. In patients with cardiac arrhythmias or embolic events, without other motivating factors, it’s necessary to pay attention and to make a differential diagnosis with these types of aneurysms.

Acquired submitral aneurysms may be associated with mitral paravalvular leaks

Acquired submitral aneurysm after mitral valve replacement is caused by weakness of the annular tissues induced by inflammation, scarring, and ischemia from surgical trauma. The asynchronous stretch of the annulus caused by the submitral aneurysm may cause recurrent paravalvular leaks. In patients with acquired submitral aneurysms and paravalvular leaks, ideal solution is a repeat surgery to address both the aneurysm and the leak. However, when patients are at high risk for repeat surgeries on cardiopulmonary bypass through sternotomy, transcatheter closure of these paravalvar leaks may offer an alternative solution. Four such procedures in three patients are detailed in this report.

A Rare Case of Coexisting Subaortic and Submitral Left Ventricular Aneurysms

Subvalvar left ventricular aneurysm is a rare disorder. Commonly it occurs below the posterior annulus of the mitral valve. Rarely the aneurysm may be found below the aortic annulus. Coexistence of both aortic and submitral aneurysms is still rarer. Here is a 58-year-male who presented with heart failure. Echo revealed an aneurysm below the aortic annulus adjacent to right coronary cusp collapsing in diastole and enlarging in systole. Doppler interrogation showed flow into and out of aneurysm from left ventricle and mild aortic regurgitation. In addition there was a submitral aneurysm below the posterior mitral leaflet with a single localised neck and mild mitral regurgitation. There was evidence of moderate left ventricular systolic dysfunction and grade II diastolic dysfunction with features of right heart failure. He was given diuretics and referred for surgery.

Submitral Aneurysm Secondary to Infective Endocarditis: Review of Literature and Case Report

Submitral aneurysm is seldom reported worldwide. It is thought to be caused by weakness in the cardiac musculature due to abnormal embryogenesis. Mostly these aneurysms were reported from Africa in black African population. Apart from congenital cause, many other causes have been put forth as its etiology. Among these endocarditis is a known infective etiology. Submitral aneurysm secondary to left sided infective endocarditis is rare. The definitive diagnostic modality and treatment of submitral aneurysm remains echocardiography and surgical correction of aneurysm respectively. The submitral aneurysms as well as infective endocarditis both are known independent risk factors for thromboembolic events and subsequent stroke increasing both morbidity and mortality. The management of submitral aneurysm includes surgical correction by repair of the aneurysm but it requires careful consideration of surgical resection of diseased paravalvular tissue or diseased mitral valve and correction of mitral regurgitation by preforming mitral valve replacement. The surgical approach reduces the risk of recurrent endocarditis and thromboembolic events, thus reducing the risk of stroke. Prior to surgery, patient should receive prophylactic antibiotics for one to two weeks to control any active infective endocarditis. For us successful treatment of a patient suffering from submitral aneurysm due to infective endocarditis was challenging. We are reporting a case of submitral aneurysm secondary to infective endocarditis along with review of literature on infective endocarditis and submitral aneurysma.

Submitral Aneurysm: A Rare Cause of Complete Heart Block

Subvalvular left ventricular (LV) aneurysms are rare and are of two types – subaortic and submitral. More commonly, they are submitral in position. Submitral aneurysms (SMAs) are most commonly located below posterior mitral leaflet (PML), whereas subaortic aneurysms are present below intermediate portion of the left aortic sinus. SMA was first described by Corvisart in 1812, and since then, only about 100–120 cases have been described worldwide. Aneurysmal dilatation in submitral position behind PML that communicates with left ventricle helps in making the diagnosis. Color Doppler echocardiography reveals the severity of mitral regurgitation. Transesophageal echocardiography and contrast-enhanced computed tomography (CT) are helpful in diagnosis in cases of acute rupture of SMA, and contrast-enhanced CT helps in evaluation of coronaries. Surgical resection is the definitive treatment for these aneurysms. Our special case presented with complaints of giddiness and presyncope and was diagnosed as complete heart block on electrocardiogram and was then diagnosed as having a large SMA on two-dimensional echocardiogram with LV dysfunction. Usually, SMAs present with dyspnea, pansystolic murmurs due to mitral regurgitation, fever secondary to infective endocarditis, or as thromboembolism. However, this patient presented symptoms of complete heart block. To conclude, SMA should be considered in differential diagnosis of mitral regurgitation with LV dysfunction and heart failure in young patients. Although complete heart block is uncommon in these patients, it should be kept in mind.

Submitral Aneurysm After Pulmonary Artery Banding—A Case Report

Submitral aneurysms are rare clinical entities occurring predominantly in young adults of African descent. A host of etiologies have been proposed for this entity. We present a unique case of submitral aneurysm which developed after pulmonary artery banding in a three-year-old girl with complex congenital heart disease. The aneurysmal sac was burrowing into the interatrial septum.

A rare case of submitral left ventricular aneurysms.

A rare case of submitral left ventricular aneurysms.

Multislice computed tomography-guided surgical repair of acquired posterior left ventricular aneurysms: demonstration of mitral valve and left ventricular reverse remodelling.

Involvement of the mitral valve (MV) apparatus represents a challenge in surgical ventricular repair (SVR) of posterior left ventricular (LV) aneurysms. This study sought to investigate whether multislice computed tomography (MSCT) assessment can be used to optimize the surgical procedure for posterior LV aneurysms. Thirty patients (m : w = 24 : 6, age 38-78, median 66 years; mean New York Heart Association class 2.98) with posterior LV aneurysm were operated upon. MSCT was performed in 24 patients before and after surgery. End-diastolic and end-systolic volumes of LV and aneurysm were indexed to body surface area (LVEDVI/LVESVI, AEDVI/AESVI). The MV apparatus was characterized by coaptation distance (CD), tenting area (TA), MV closure angle (MVCA), MV annulus area (MVAA) and interpapillary muscle distance (IMD). Thirty-day mortality was 10% and 5-year survival rate was 83%. After surgery, LVEDVI decreased from 151.2 ± 84.1 to 85.7 ± 28.3 ml/m(2) (P = 0.001) and LVESVI from 110.6 ± 88.8 to 50.2 ± 22.9 ml/m(2) (P = 0.001). LV ejection fraction increased from 31.5 ± 15.1 to 43.4 ± 9.9% (P = 0.001). Preoperative MSCT showed significantly higher values of MVAA, CD and TA in patients who needed MV repair or replacement. Postoperative reduction of mitral regurgitation in patients without MV surgery corresponded with significant reduction in intercommissural diameter, anteroposterior diameter, MVAA, TA, CD, MVCA and IMD. MSCT represents an excellent diagnostic tool for the assessment of MV and LV geometry. MSCT-guided SVR of submitral LV aneurysms leads to excellent mid-term results. On the basis of the MSCT assessment, we propose an algorithm for surgical planning in posterior LV aneurysms.

Mitral valve and left ventricular reverse remodeling after surgical repair of submitral left ventricular aneurysms assessed with multi-slice computed tomography

Mitral valve and left ventricular reverse remodeling after surgical repair of submitral left ventricular aneurysms assessed with multi-slice computed tomography

Submitral Aneurysm: An Echocardiography Study in a Tertiary Center in Angola

Submitral Aneurysms (SMA) are a relatively rare and poorly understood cardiac condition, although there are multiple reports of its incidence in specific populations [1-5]. Clinically it is manifested by symptoms and signs of heart failure due to mitral regurgitation, and/or ventricular arrhythmias. Transthoracic echocardiography (TTE) plays a key role in the definitive diagnosis of this pathology. Transesophageal echocardiography is of paramount importance in assessing the rupture of the aneurysm into the left atrium [1].

Successful management of multiple infected sub-mitral aneurysms of left ventricle

Sub-mitral aneurysms are rarely reported clinical entity. Though a variety of etiologies are proposed, congenital weakness of the mitral valve annulus is the most widely accepted one. A 17-year-old boy with sub-mitral aneurysm presented with severe mitral regurgitation, which was diagnosed by echocardiography and successfully managed surgically. The aneurysm wall was positive for Staphylococcus aureus, and patient was treated with intensive antibiotics.

Multimodality Imaging of Submitral Left Ventricular Aneurysm

Submitral left ventricular aneurysms are a rare nonischemic aneurysm thought to be developmental in origin. These aneurysms invariably occur at the site of posterior mitral annulus and lead to mitral valve incompetence. We hereby describe a case of a 30-year-old adult with a rare form of submitral left ventricular aneurysm involving whole of the posterior mitral annulus. Multimodality imaging with two-dimensional echocardiography, three-dimensional echocardiography, computed tomography and invasive left ventriculography helped the patient to undergo successful resection of the aneurysm along with mitral valve replacement.

Assessment of left ventricular and mitral valve geometry and function after surgical repair of submitral left ventricular aneurysms with multi-slice computed tomography

Assessment of left ventricular and mitral valve geometry and function after surgical repair of submitral left ventricular aneurysms with multi-slice computed tomography

Abstract 18544: Geometry and Function of Left Ventricle and Mitral Valve After Surgical Repair of Submitral Left Ventricular Aneurysms Assessed with Multi-slice Computed Tomography

Objectives: Surgical repair of submitral aneurysms presents a technical challenge especially if the mitral valve (MV) apparatus is involved. We present our single center surgical experience and the potential of multi-slice computed tomography (MSCT) in the assessment of aneurysm morphology and changes in MV and LV geometry. Methods: In 05/06-04/12, 21 patients (m:w=17:4, ages 39 to 78, mean 63 yrs; mean NYHA class 2.8) with submitral LV aneurysm were operated upon. Echocardiography and MSCT (Somatom Definition, Siemens) were performed before and after surgery. LV and aneurysm end-diastolic/end-systolic volume were measured and indexed to body surface area (LV-EDVI/LV-ESVI, A-EDVI/A-ESVI). LV ejection fraction (LVEF), cardiac output (CO) and cardiac index (CI) were calculated on the basis of MSCT data. MV geometry was characterized by mid-systolic coaptation distance (CD), tenting area (TA) and MV closure angle (MVCA). Results: Thirty-day and 5-year survival was 88.9%±7.4% and 82.1%±9.5%. Aneurysms were localized in myocardial segments 4/10/11. Preoperative A-EDVI showed a slight systolic increase, demonstrating adverse volume shift. There was reduction of LV-EDVI (141.4±21.6 to 72.7±12.9 ml/sqm, p=0.006) and LV-ESVI (102.2±24.9 to 42.5±9.9 ml/sqm, p=0.024), LVEF improvement (32% to 45%, p=0.016) and significant increase in CO and CI after surgical repair. Mitral repair/replacement was necessary in three patients. Postoperative reduction of mitral regurgitation (from grade 0.94 to 0.24, p=0.003) corresponded with improvement of MV geometry (CD (reduced from 10.2 to 7.9 mm, p=0.06) and of TA (1.8 to 1.5 sqcm, p=0.04) and MVCA (103° to 116°, p=0.04). Conclusions: Reconstruction of submitral LV aneurysms can be performed with good mid-term results. MSCT provides valuable information about aneurysm localization, its extent and effect on MV and LV geometry and reverse remodeling, and also functional changes after surgical restoration.

Acquired Left Ventricular Submitral Aneurysms in the Course of Takayasu Arteritis in a Child

A 9-year-old black African boy was hospitalized for heart failure revealing a severe left ventricular dysfunction associated with dilated cardiomyopathy, two submitral aneurysms, occlusion of the circumflex artery and a giant coronary artery aneurysm on the proximal left anterior descending artery. The boy was coinfected with human immunodeficiency virus and Mycobacterium tuberculosis. Though rare, association of Takayasu arteritis and submitral aneurysm leads to rethinking the pathogenesis of submitral aneurysm and suggests that some of them may be acquired. In our case, a common inflammatory process, possibly triggered by tuberculosis or HIV, may underlie Takayasu and submitral aneurysms.

Congenital Submitral Aneurysm with Rupture into the Left Atrium: Assessment by 2D and 3D Transesophageal Echocardiography

We describe two cases of congenital submitral aneurysms (SMAs) in which three-dimensional transesophageal echocardiography (3D TEE) proved useful to define the spatial extent of these aneurysms. In both cases, rupture into the left atrium was accurately delineated. 3D TEE was useful in case 1 as it depicted the precise site of rupture into the left atrium as well as pseudoprolapse of the P2 segment of the mitral valve. In case 2 it also localized the rupture into the left atrium in relation to the annulus to be adjacent to the anterolateral commissure. In addition, a cleft between the A1 and A2 scallops were identified and together with failure of the leaflets to coapt enabled the mechanisms contributing to the mitral regurgitation to be elucidated. Thus, imaging from the left atrial perspective using 3D TEE provided superior spatial anatomical delineation of the rupture and its relationship to the mitral valve, as well as accurate anatomical definition of the mitral leaflets. This information provides added benefit to the surgeon in planning a transatrial surgical repair of the SMAs. 3D TEE is superior to conventional 2D TEE in defining the spatial anatomy of SMAs as well as the mechanisms contributing to mitral regurgitation.

Submitral aneurysm with left atrial communication

Annular sub-mitral aneurysms are rare lesions of varied etiology. A sub-mitral membranous curtain may be a potential area of weakness through which these lesions expand. Initially described in young males of African origin and reported from varying geographical areas, these lesions arise from the atrioventricular groove in close relation to the mural leaflet. They may cause pressure effects, lead to mitral incompetence and left ventricular dysfunction. Key issues during repair are proximity to the circumflex coronary artery, atrioventricular junction and progressive involvement of the mitral valve. This case report of a calcified bi-lobed sub-mitral aneurysm with communication to the left atrium discusses the anatomical basis of the lesion, the role of computed tomogram angiography in pre-operative evaluation and surgical management.

Usefulness of Magnetic Resonance Imaging Evaluation of Congenital Left Ventricular Aneurysms

Congenital left ventricular (LV) aneurysm is a rare malformation of unknown cause that is often associated with a poor prognosis. This study was undertaken to evaluate the usefulness of cardiac magnetic resonance imaging in patients with congenital LV aneurysms and to determine the relation between clinical manifestations and the morphologic and functional characteristics of the aneurysms. Among the 26 consecutive patients with congenital LV aneurysms included, the anomalies involved the apex or free wall in 15 and the submitral myocardium in 11. Cardiac magnetic resonance provided detailed anatomic and functional assessment of the aneurysms in all patients. Compared with submitral aneurysms, apical or free-wall aneurysms were larger (24 +/- 29 vs 3 +/- 2 ml/m(2), p = 0.02), were more frequently associated with scar tissue by myocardial delayed enhancement imaging (71% vs 0%, p = 0.03), and tended to be more commonly associated with symptoms (53% vs 18%, p = 0.08). Aneurysm volume but not location correlated with LV size (r = 0.735, p <0.0001) and the ejection fraction (r = 0.774, p <0.0001). Apical or free-wall aneurysms were resected in 5 patients, with no mortality. There was 1 death after aortic valve replacement in a patient with type B Niemann-Pick disease, which was not clearly related to the LV aneurysm. The remaining 25 patients are alive at a median age of 13.5 years. In conclusion, the experience with this cohort illustrates that cardiac magnetic resonance is well suited for the morphologic and functional evaluation of congenital LV aneurysms.

Sub-mitral membranous curtain: A potential anatomical basis for congenital Sub-mitral aneurysms

To seek an anatomic basis for congenital sub-mitral aneurysms. 75 normal human autopsy heatts were incised between the right and left coronary cusps up to the apex. The membranous septum was trans illuminated from the right side. The mitral valve was lifted anteriorly to observe extension of any membranous tissue to the sub-mitral region. In 17 out of 75 hearts, the tissue of the membranous septum extended beyond the nadir of the non-coronary cusp, beneath the aortic leaflet of the mitral valve, up to the postero-medial commissural line. In 28 hearts, this sub-mitral membranous extension continued beyond the postero-medial commissural line beneath the annulus of the mural leaflet, to a distance varying from 4mm to 21mm, with height between 0.5mm to 4mm. This extension of the membranous tissue beyond the nadir of the non coronary cusp, caudal to the aortic and mural leaflets of the mitral valve is preferably labeled sub-mitral curtain. The sub-mitral curtain was in continuity with the membranous, septum in 40 hearts. In 5 hearts, trans illumination was interrupted between membranous septum and sub-mitral curtain by varying extent of opaque area due to tissue of the central fibrous body, right fibrous trigone and ventricular musculature. The membranous sub-mitral curtain of the mitral valve seen in 45 out of 75 hearts is a potentially weak area, through which congenital sub-mitral true aneurysms can occur.

Rheumatic and Nonrheumatic Valvular Heart Disease

Unlike the Western world, valvular disease ranks among the major cardiovascular afflictions in Africa. Acute rheumatic fever and chronic rheumatic valvular disease in their most virulent form are still commonly encountered and impose a huge burden on limited healthcare resources. We performed a systematic review of the literature with PubMed using rheumatic fever, rheumatic heart disease, valvular disease, warfarin anticoagulation, and pregnancy as search items. Literature emanating from Africa was emphasized. Epidemiology, current concepts on pathogenesis, and aspects of the medical and surgical management of this disease as seen from an African perspective are presented. The association of pregnancy with mitral stenosis is common and may be fatal if not managed appropriately. A practical approach to these patients is presented to optimize maternal and fetal outcome. Pregnant patients with mechanical valves require careful attention to ensure maternal survival and prevent fetal warfarin embryopathy. Prolonged subcutaneous heparin and frequent monitoring of the partial thromboplastin time are impractical in this setting, and the merits of different anticoagulation regimens are discussed. Congenital submitral aneurysms are a unique cause of mitral regurgitation, with the vast majority of cases originating from sub-Saharan Africa. Although the precise etiology is as yet unclear, the clinical and echocardiographic features are sufficiently characteristic to allow a preoperative diagnosis to be made. Transesophageal echocardiography allows much better definition of the size and anatomic relationships of the aneurysm. Surgical resection can be difficult but is usually curative. Mitral valve prolapse and endocarditis constitute the remaining frequent causes of valvular disease and are discussed briefly. The spectrum and presentation of valvular disease in Africa are uniquely different from elsewhere. Together with socioeconomic issues and the HIV pandemic, this fact makes it imperative that further epidemiological and clinical studies be undertaken and guidelines developed that are appropriate to the practice of medicine on the African continent.