Lipomatous hypertrophy of the interatrial septum: an overview.
ABSTRACT Lipomatous hypertrophy of the interatrial septum is a rare, but increasingly recognized, anomalous developmental or neoplastic lesion of the heart. This entity was first described in 1964 at autopsy and is identified before death based on its distinctive characteristics on echocardiography, computed tomography, and magnetic resonance imaging. Although it is often asymptomatic, the mass has been associated with supraventricular arrhythmias and sudden death. In rare patients who experience intractable symptoms, surgical excision of the lesion may provide relief. Therefore, lipomatous hypertrophy of the interatrial septum is of interest to the pathologist when a cardiac mass is received for evaluation or at autopsy when a patient has experienced sudden death from an unknown cause.
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ABSTRACT: ZET İnteratriyal septumun lipomatöz hipertrofisi nadir görülen, interatriyal septumda kalınlaşmaya neden olan yağlı infiltrasyonla karakterize benign bir durumdur. Genellikle benign ve asemptomatiktir ancak nadiren klinik olarak önemli atriyal fibrilasyon, atriyoventriküler blok ve vena cava süperior obstrüksiyonuyla birlikte olabilir. Kadınlarda, yaşlılarda ve obezlerde daha sık görülür. BT ve MR görüntülemede fossa ovalisin korunmasına bağlı tipik 'dumbbell' veya 'bilobed' şekli oluşur. Anahtar Kelimeler: İnteratriyal septum. Lipomatöz hipertrofi. BT. MR. Lipomatous hypertrophy of the interatrial septum ABSTRACT Lipomatous hypertrophy of the interatrial septum is a rare entity, characterized by excessive deposition of adipose tissue that may result in marked thickening of the atrial septum. Although benign and frequently asymptomatic, it is of clinical importance as it is associated with atrial fibrillation, atrioventricular block and vena cava superior obstruction. Typically occurs in the female, elderly and obese people. The fossa ovalis is generally spared, giving it a characteristic 'dumbbell' or 'bilobed' shape on the CT and MR imaging. Key Words: Interatrial septum. Lipomatous hypertrophy. CT. MR imaging. Lipomatöz hipertrofi, interatriyal septumun myokar-dial lifleri arasında aşırı yağ depolanması ile kalınlaş-ması sonucu oluşan benign, nadir bir durumdur 1,2 . Genellikle rastlantısal olarak saptanır. Kadınlarda, yaşlılarda ve obezlerde daha sık görülür. Görülme sıklığı %1-8 oranında değişmektedir 3,4 . Karakteristik yağ içeriği nedeniyle bilgisayarlı tomografi (BT) ile tanısı kolaylıkla konur. Bu yazıda, nadir görülmesi nedeniyle interatriyal septumun lipomatöz hipertrofisi (İSLH)'nin BT bul-guları sunulmuştur.
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ABSTRACT: We present the case of a 58-year-old woman who had large lipomatous hypertrophy of the interventricular septum, a condition that is reported very infrequently. Preoperative cardiac magnetic resonance images revealed an inhomogeneous, infiltrating mass that was suppressed in fat-suppression mode. The extensive mass was causing right ventricular dysfunction, so we excised it through a right ventricular approach. The findings on histologic analysis of the mass were consistent with lipomatous hypertrophy. The patient died of septic shock on the 28th postoperative day. In addition to the patient's case, we discuss the characteristics and diagnosis of this rare entity.Texas Heart Institute journal / from the Texas Heart Institute of St. Luke's Episcopal Hospital, Texas Children's Hospital 04/2014; 41(2):231-3. DOI:10.14503/THIJ-13-3336 · 0.63 Impact Factor
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ABSTRACT: Benign lipomatous lesions of the heart encompass an apparently etiologically diverse group of entities, including neoplastic, congenital, and reparative phenomena. Among these, lipomas and lipomatous hypertrophy of the atrial septum (LHAS) represent two commonly encountered mass lesions. To date, no study has systematically and comparatively evaluated the morphologic and genetic characteristics of these lesions. Tissue registry archives of Mayo Clinic were queried for cases of cardiac lipoma and LHAS (1994–2011). Clinical, imaging and pathological findings were reviewed. Representative cases in each cohort were evaluated by fluorescence in situ hybridization (FISH) for HMGA1 and HMGA2 loci rearrangement and for MDM2/CPM locus amplification. Five cases of cardiac lipoma were identified (mean age 67 years, range 48–101; 3 men); 4 right atrial and 1 left ventricular. 42 cases of LHAS were identified (mean age 75.6 years, range 45–95, 20 men), 39 of which were autopsy-derived. The median size was 3.4 cm for lipomas, and 2.8 cm for LHAS (n = 14). A single case each of cardiac lipoma and LHAS were found to harbor HMGA2 rearrangement, while no case showed cytogenetic abnormality of HMGA1 or CPM. This represents the largest series of histopathologically confirmed cardiac lipomas from a single institution. Additionally, it is the first to evaluate cardiac lipomas and LHAS for genetic alterations associated with extracardiac lipomatous lesions. The genetic and morphologic similarities found provide evidence in support the neoplastic classification of cardiac lipomas. A single case of LHAS contained an HMGA2 rearrangement, challenging the currently accepted hypothesis of pathogenesis for this lesion.Human pathology 09/2014; 45(9). DOI:10.1016/j.humpath.2014.05.003 · 2.81 Impact Factor