{"orpha":263352,"name":"Postcardiotomy right ventricular failure","type":"Particular clinical situation in a disease or syndrome","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare cardiac condition characterized by acute severe right ventricular failure with subsequent hemodynamic instability following a cardiac surgical procedure. Predisposing factors include suboptimal myocardial protection during surgery, long cardiopulmonary bypass time, right ventricular myocardial ischemia or infarction, atrial arrhythmias, reperfusion lung injury with secondary pulmonary hypertension, post-operative pulmonary micro- or macro-embolism, and pre-existing pulmonary vascular disease, among others.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-263352/","orphanet_url":"https://www.orpha.net/en/disease/detail/263352","names":{"fr":{"name":"Insuffisance cardiaque droite post-cardiotomie","definition":"Maladie cardiaque rare caractérisée par une insuffisance ventriculaire droite aiguë et sévère associée à une instabilité hémodynamique consécutive à une intervention de chirurgie cardiaque. Les facteurs prédisposants sont, entre autres, une protection myocardique sous-optimale pendant l'opération, une durée prolongée de pontage cardio-pulmonaire, une ischémie ou un infarctus du myocarde du ventricule droit, des arythmies auriculaires, des lésions d'ischémie-reperfusion avec hypertension pulmonaire secondaire, des micro- ou macro-embolies pulmonaires post-opératoires et une maladie vasculaire pulmonaire préexistante."},"de":{"name":"Postkardiotomie-Syndrom mit rechtsventrikulären Versagen","definition":"Eine seltene kardiale Störung, die durch akutes schweres rechtsventrikuläres Versagen mit anschließender hämodynamischer Instabilität nach einem herzchirurgischen Eingriff gekennzeichnet ist. Zu den prädisponierenden Faktoren gehören unter anderem eine suboptimale Myokardprotektion während des Eingriffs, eine lange kardiopulmonale Bypasszeit, eine rechtsventrikuläre Myokardischämie oder ein Myokardinfarkt, Vorhofarrhythmien, eine Reperfusionslunge mit sekundärer pulmonaler Hypertonie, eine postoperative pulmonale Mikro- oder Makroembolie und eine vorbestehende pulmonale Gefäßerkrankung."},"es":{"name":"Insuficiencia ventricular derecha postcardiotomía","definition":"Es una afección cardíaca poco frecuente caracterizada por insuficiencia ventricular derecha grave aguda con inestabilidad hemodinámica posterior a un procedimiento quirúrgico cardíaco. Los factores predisponentes incluyen: protección miocárdica subóptima durante la cirugía, tiempo prolongado de derivación cardiopulmonar, isquemia o infarto de miocardio ventricular derecho, arritmias auriculares, daño pulmonar por reperfusión con hipertensión pulmonar secundaria, micro- o macroembolismo pulmonar posoperatorio y enfermedad vascular pulmonar preexistente, entre otros."},"it":{"name":"Insufficienza ventricolare destra post-cardiotomica"},"nl":{"name":"Postcardiotomie-rechterventrikelfalen","definition":"Een zeldzame aandoening van hart, gekenmerkt door acuut ernstig falen van rechterventrikel met vervolgens hemodynamische instabiliteit na een hartoperatie. Bevorderende factoren zijn onder meer suboptimale bescherming van myocard tijdens chirurgie, verlengde cardiopulmonale bypass, myocardiale ischemie of infarct van rechterventrikel, atriale aritmie, reperfusieletsel van long met secundaire pulmonale hypertensie, postoperatieve pulmonale micro- of macro-embolie, en pre-existente aandoening van longcirculatie."},"pt":{"name":"Falência do ventrículo direito pós-cardiotomia","definition":"Condição cardíaca rara caracterizada por insuficiência ventricular direita aguda e grave com subsequente instabilidade hemodinâmica após procedimento cirúrgico cardíaco. Os fatores predisponentes incluem proteção miocárdica subótima durante a cirurgia, longo tempo de circulação extracorpórea, isquemia ou enfarte do miocárdio ventricular direito, arritmias atriais, lesão pulmonar de reperfusão com hipertensão pulmonar secundária, micro ou macroembolia pulmonar pós-operatória e doença vascular pulmonar pré-existente, entre outros."},"pl":{"name":"Pokardiotomijna niewydolność prawej komory","definition":"Rzadko występujące zaburzenie kardiologiczne opisywane jako ostra, ciężka prawokomorowa niewydolność serca skutkująca niestabilnością hemodynamiczną, która występuje po zabiegu kardiochirurgicznym."},"cs":{"name":"Postkardiotomické selhání pravé komory"},"uk":{"name":"Посткардіохірургічна правошлуночкова недостатність","definition":"A rare cardiac condition characterized by acute severe right ventricular failure with subsequent hemodynamic instability following a cardiac surgical procedure. Predisposing factors include suboptimal myocardial protection during surgery, long cardiopulmonary bypass time, right ventricular myocardial ischemia or infarction, atrial arrhythmias, reperfusion lung injury with secondary pulmonary hypertension, post-operative pulmonary micro- or macro-embolism, and pre-existing pulmonary vascular disease, among others."},"zh":{"name":"心脏术后右心衰竭"},"tr":{"name":"Postkardiyotomi sağ ventrikül yetmezliği"}},"xrefs":[{"source":"ICD-10","code":"I97.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"UMLS","code":"C5680731","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"20898","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97929,"name":"Rare cardiac disease"},"parents":[{"orpha":97929,"name":"Rare cardiac disease","classifications":[146]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"1-9 / 100 000","value":0.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2010[OTHER]"}],"phenotypes":null}