{"orpha":99092,"name":"Interventricular septum aneurysm","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Interventricular septum aneurysm is a rare, non-syndromic, congenital heart malformation characterized by the presence of a congenital aneurysm of the membranous portion of the interventricular septum. Patients may be asymptomatic or may present with ventricular or supraventricular tachycardia, fatigue, exertional dyspnea, palpitations, and cardiac murmur. Ventricular septal defects and conduction defects, such as first-degree atrio-ventricular block or incomplete right bundle branch block, may also be also associated.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99092/","orphanet_url":"https://www.orpha.net/en/disease/detail/99092","names":{"fr":{"name":"Anévrisme du septum interventriculaire","definition":"L'anévrisme du septum interventriculaire est une malformation cardiaque congénitale, non syndromique et rare. Elle se caractérise par la présence d'un anévrisme congénital de la portion membraneuse du septum interventriculaire. Les patients peuvent être asymptomatiques ou présenter une tachycardie ventriculaire ou supraventriculaire, une fatigue, une dyspnée d'effort, des palpitations et un souffle cardiaque. Des communications interventriculaires et des troubles de la conduction peuvent aussi être associés, comme un bloc auriculo-ventriculaire de premier degré ou un bloc de branche droite incomplet."},"de":{"name":"Septumaneurysma, interventrikuläres","definition":"Das Interventrikelseptumaneurysma ist eine seltene, nicht-syndromale, angeborene Herzfehlbildung, die durch das Vorhandensein eines angeborenen Aneurysmas des membranösen Teils des Interventrikelseptums gekennzeichnet ist. Die Patienten können asymptomatisch sein oder ventrikuläre oder supraventrikuläre Tachykardien, Müdigkeit, Anstrengungsdyspnoe, Herzklopfen und Herzgeräusche zeigen. Weitere Manifestationen sind Ventrikelseptumdefekte und Überleitungsstörungen, wie z. B. ein atrio-ventrikulärer Block ersten Grades oder ein inkompletter Rechtsschenkelblock."},"es":{"name":"Aneurisma del septo interventricular","definition":"El aneurisma del tabique interventricular es una malformación cardíaca congénita no-sindrómica poco frecuente que se caracteriza por la presencia de un aneurisma congénito de la porción membranosa del tabique interventricular. Los afectados pueden estar asintomáticos o presentar taquicardia ventricular o supraventricular, fatiga, disnea de esfuerzo, palpitaciones y soplo cardíaco. También pueden asociarse defectos del tabique ventricular y defectos de conducción, tales como bloqueo auriculoventricular de primer grado o bloqueo de rama derecha incompleto."},"it":{"name":"Aneurisma del setto interventricolare","definition":"L'aneurisma del setto interventricolare è una cardiopatia congenita rara non sindromica, caratterizzata dalla presenza di un aneurisma congenito della porzione membranosa del setto interventricolare. I pazienti possono essere asintomatici, oppure presentare tachicardia ventricolare o sopraventricolare, affaticamento, dispnea da sforzo, palpitazioni e soffio cardiaco. Posso essere presenti difetti del setto ventricolare e anomalie della conduzione, come il blocco atrio-ventricolare di primo grado o il blocco di branca destra incompleto."},"nl":{"name":"Interventriculair septumaneurysma","definition":"Interventriculair septumaneurysma is een zeldzame, niet-syndromale, congenitale hartmalformatie die gekarakteriseerd wordt door de aanwezigheid van een congenitaal aneurysma van het membraangedeelte van het interventriculair septum. Patiënten kunnen asymptomatisch zijn, of ventriculaire of supraventriculaire tachycardie, vermoeidheid, inspanningsafhankelijke dyspneu, hartkloppingen, en hartruis vertonen. Ventrikelseptumdefecten en hartgeleidingsdefecten, zoals eerstegraads atrioventriculair blok of onvolledig rechterbundeltakblok, kunnen ook geassocieerd zijn."},"pt":{"name":"Aneurisma do septo intraventricular"},"pl":{"name":"Tętniak przegrody międzykomorowej"},"cs":{"name":"Aneuryzma mezikomorového septa"},"uk":{"name":"Аневризма міжшлуночкової перегородки","definition":"Interventricular septum aneurysm is a rare, non-syndromic, congenital heart malformation characterized by the presence of a congenital aneurysm of the membranous portion of the interventricular septum. Patients may be asymptomatic or may present with ventricular or supraventricular tachycardia, fatigue, exertional dyspnea, palpitations, and cardiac murmur. Ventricular septal defects and conduction defects, such as first-degree atrio-ventricular block or incomplete right bundle branch block, may also be also associated."},"zh":{"name":"室间隔动脉瘤","definition":"室间隔动脉瘤是一种罕见、非综合征的先天性心脏畸形，其特征是室间隔膜部存在先天性动脉瘤。患者可能没有症状，也可能出现室性或室上性心动过速、疲劳、劳力性呼吸困难、心悸和心脏杂音。该病也可能与室间隔缺损和传导缺陷（例如，一级房室传导阻滞或不完全性右束支传导阻滞）相关。"},"tr":{"name":"İnterventriküler septum anevrizması"}},"xrefs":[{"source":"OMIM","code":"105805","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"Q21.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LA88.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0007112","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C563239","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1387721","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":474347,"name":"Rare congenital anomaly of ventricular septum","classifications":[147,148]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}