[A Successful Surgical Treatment of Hemolytic Anemia Caused by Aortic Valve Perivalvular Leakage and Severely Kinked Elephant Trunk:Report of a Case].

Both perivalvular leakage and kinked prosthetic graft may cause hemolysis. A 72-year-old man was refereed to our hospital because of hemolytic anemia. He has past histories of total aortic arch replacement and repeat aortic valve replacement for aortic aneurysm and prosthetic valve endocarditis. Pre-operative examinations demonstrated aortic valve perivalvular leakage and severe graft kinking of the elephant trunk. Repeat aortic valve replacement and axillo-femoral bypass were performed successfully. Hemolysis got better after the operation and the patient discharged home in stable condition.

Concurrent Cholecystectomy Does Not Increase Splenectomy Morbidity in Patients With Hemolytic Anemia: A Pediatric NSQIP Analysis.

PURPOSE: Children undergoing splenectomy for hemolytic anemia often have cholelithiasis, which may or may not be symptomatic. It is unclear whether concurrent cholecystectomy increases length of stay or morbidity after splenectomy. The purpose of this study was to compare morbidity among children undergoing laparoscopic splenectomy alone versus splenectomy with concurrent cholecystectomy in patients with hemolytic anemia. METHODS: We retrospectively evaluated children with hemolytic anemia undergoing non-traumatic laparoscopic splenectomy in the National Surgical Quality Improvement Program-Pediatric database (2012-2020). Outcomes were compared for patients undergoing splenectomy alone (n = 1010) versus splenectomy with cholecystectomy (n = 371). Pearson's Chi-square and Student's t-tests were utilized as appropriate. Propensity score-matching was completed, controlling for eight demographic and clinical variables. RESULTS: 1381 patients were identified, 73.1% undergoing splenectomy alone and 26.9% splenectomy with cholecystectomy. Splenectomy with cholecystectomy patients were older (10.9 years vs. 8.4 years, p < 0.01), more likely to have hereditary spherocytosis (56.1% vs. 40.8%, p < 0.01), less likely to have sickle cell disease (12.1% vs. 33.5%, p < 0.01), more likely ASA class 1 or 2 (49.3% vs. 42.1%, p < 0.01), and had similar preoperative hematocrit levels (29.6 vs. 29.3, p = 0.33). The splenectomy with cholecystectomy group was less likely to receive preoperative blood transfusions (13.5% vs. 25.4%, p < 0.01). There were 360 pairs selected on propensity score-matching, and splenectomy with cholecystectomy was associated with increased operative time (182 min vs. 145 min, p < 0.01) and decreased occurrences of a postoperative transfusion (4.2% vs. 8.9%, p = 0.01). Length of stay after surgery (2.5 days vs. 2.3 days, p = 0.13), composite morbidity (3.9% vs. 3.4%, p = 0.69), and 30-day readmission rates (3.3% vs. 7.4%, p = 0.08) were all similar. CONCLUSIONS: Splenectomy with cholecystectomy is associated with similar postoperative morbidity, length of stay and readmission rates compared to splenectomy alone. These data support the safety of concurrent cholecystectomy with splenectomy for children with cholelithiasis in the setting of hemolytic anemia. TYPE OF STUDY: Retrospective Cohort Study. LEVEL OF EVIDENCE: Level III.

Hemolytic anemia caused by kinked graft 6 months after aortic dissection repair.

BACKGROUND: Clinically insignificant hemolytic anemia is occasionally a complication of prosthetic valve replacement. However, hemolysis related to kinked grafts is a very rare complication after central repair for acute aortic dissection. CASE PRESENTATION: A 42-year-old man had undergone replacement of the ascending aorta and a root repair for type A aortic dissection 6 months previously. Laboratory data showed mild hemolysis 5 months later, and he began to complain of fatigue on exertion. The serum hemoglobin level reduced to 8.6 g/dL, and lactate dehydrogenase levels increased to 3071 IU/L with gross change in urine color, indicating hemoglobinuria. We diagnosed mechanical hemolytic anemia caused by a kinked graft and planned a repeat operation. The kinked graft was resected and graft-graft anastomosis was performed. Postoperatively, the clinical course was uneventful, and the hemolytic anemia completely resolved. CONCLUSION: We herein report a case of hemolytic anemia caused by kinking of the graft 6 months after acute aortic dissection repair. The diagnosis was swiftly made, and the patient was successfully managed with redo surgery.

[Graft Kink with Hemolytic Anemia Caused by Enlargement of the Residual False Lumen in the Aortic Root:Report of a Case].

A 67-year-old male underwent ascending aortic replacement for Stanford type A acute aortic dissection four years ago. Residual false lumen in the aortic root and mild to moderate aortic regurgitation were noted postoperatively. Two and a half years later, he presented with hemolytic anemia and shortness of breath. Computed tomography (CT) revealed aneurysmal aortic root and severely kinked vascular prosthesis, and echocardiography showed severe aortic regurgitation. It was considered that the proximal residual false lumen gradually enlarged, and the graft was pushed up distally and kinked, resulting in hemolytic anemia. Surgical treatment was indicated because of deteriorating shortness of breath due to hemolytic anemia (Hb 6.7 g/dl, LDH 1,528 U/l) and aortic regurgitation. Aortic root replacement was successfully performed and hemolytic anemia improved immediately after surgery.

Intractable mechanical hemolytic anemia complicating mitral valve surgery: a case series study.

BACKGROUND: Intractable, mechanical hemolytic anemia (IMHA) is a rare catastrophic complication following mitral valve surgery. We analyzed patient characteristics and IMHA management by reoperations after mitral valve surgery. METHODS: We collected medical records from mitral valve patients requiring reoperation due to IMHA. INCLUSION CRITERIA: hemoglobin < 100 g/L; positive hemolysis tests and echocardiography results; and exclusion of other hemolysis causes. RESULTS: Data from 25 IMHA cases included 10 (40%) early onset (1.3 (0.3,3.0) months) and 15 (60%) late onset (120 (24,204) months) cases. Early IMHA etiologies included surgical defects (6, 60%), uncontrolled infection (3, 30%) and Bechet's disease (1, 10%). Late IMHA etiologies included degeneration (13, 87%), new infection (1, 7%) and trauma (1, 7%). There were more mechanical valves (15, 88%) than bio-valves (2, 12%); the main valvular dysfunction was paravalvular leak (16, 64%). IMHA manifestations included jaundice (18, 72%), dark urine (21, 84%), heart failure (16, 64%), acute kidney injury (11, 44%), hepatomegaly (15, 60%), splenomegaly (15, 60%) and pancreatitis (1, 4%). Laboratory results showed decreased hemoglobin (70 ± 14 g/L) and increased bilirubin (72 ± 57 µmol/L), lactate dehydrogenase (2607 ± 2142 IU/L) and creatinine (136 ± 101 µmol/L) levels. Creatinine level negatively correlated with hemoglobin level (B = -3.33, S.E. B = 1.31, Exp(B) = 368.15, P = 0.018). Preoperative medications included iron supplements (20, 80%), erythropoietin (16, 64%) and beta-blocker (22, 88%). Two patients died of cardiac causes before reoperation. The other 23 underwent reoperation with long surgical times (aortic cross clamp 124 ± 50 min, cardiopulmonary bypass 182 ± 69 min) and blood transfusions (red blood cells 6 (6, 8) units, plasma 600 (400,800) ml, platelet 1(0,2) units). Postoperative complications included cardiac dysfunction (5, 22%), arrhythmia (10, 43%), sepsis (6, 26%), pulmonary infection (5, 22%), gastrointestinal bleeding (3, 13%), cerebral hemorrhage (2, 9%), chronic renal dysfunction (1, 4%) and surgical hemorrhage (1, 4%). Five (33%) patients died after reoperation from cardiac dysfunction (3, 60%), septic shock (1, 20%) and self-discharge (1, 20%). CONCLUSIONS: IMHA induces severe multi-organ dysfunction, contributing to high mortality. Perioperative management should focus on etiological treatment, organ protection, and blood management.

Esplenectomía en enfermedades hemolíticas. Influencia de la enfermedad de Gilbert en la aparición de complicaciones biliares / No disponible

Introducción: En pacientes con enfermedades hemolíticas (EH) se recomienda esplenectomía entre 6-12 años. En aquellos con enfermedad de Gilbert (EG) asociada se ha descrito mayor riesgo de complicaciones biliares (CB), sin establecerse edad quirúrgica óptima. Nuestro objetivo es cuantificar el riesgo de CB en pacientes con EH y EG para valorar el beneficio de esplenectomía temprana. Material y métodos: Estudio retrospectivo de las esplenectomías realizadas en pacientes con EH entre 2000-2017. Se analizó la incidencia de CB, su repercusión clínica (ingreso o tratamiento invasivo) y momento de aparición. Se consideraron dos grupos: pacientes con EG y sin EG. Se obtuvieron curvas de supervivencia y se compararon mediante log-rank test. Resultados: Se realizaron 44 esplenectomías, 15 de ellas (34,1%) en pacientes con EH+EG. La edad mediana en la cirugía fue 10,3 años (rango 5,4-14,8). Veintinueve (65,9%) presentaron CB. El 50% de los pacientes con EG las presentaron antes de los 8 años vs.10,5 años en los casos sin EG (log-rank 3,9; p= 0,05). Los pacientes con EG presentaron más CB (86,7% vs. 55,2%; c2= 4,37, p= 0,037). En el grupo EH+EG, 8 casos (53%) necesitaron ingreso vs. 8 (31%) en el grupo sin EG (c2= 2, p= 0,1). El tratamiento invasivo fue necesario en 2 pacientes (13%) del grupo EH+EG y 2 pacientes (7,6%) del grupo sin EG (c2= 0,3, p= 0,6). Conclusiones: En nuestra serie, la incidencia de CB fue superior en los pacientes con EG. Existió una tendencia a la presentación más temprana de CB en este grupo, pero ni este dato ni su repercusión clínica nos permiten recomendar la esplenectomía temprana

Introduction: In patients with hemolytic disorders (HD) splenectomy is recommended between 6-12 years. A higher risk of biliary complications (BC) has been described in those with associated Gilbert’s disease (GD), but the ideal surgical age has not been stablished yet. Our aim is to quantify the risk of BC in patients with HD and GD to assess the benefit of early splenectomy. Material and methods: Retrospective study of splenectomies performed in patients with HD between 2000-2017. The incidence of BC, its clinical consequences (admission or invasive treatment) and time of onset were analyzed. Two groups were considered: patients with GD and without GD. Survival curves were obtained and compared with log-rank test. Results: Fourty-four patients underwent splenectomy, 15 of them (34.1%) with HD+GD. The median age at surgery was 10.3 years (range 5.4-14.8). Twenty-nine (65.9%) had BC. Half of the patients with GD had BC before 8 years vs. 10,5 years in the cases without GD (log-rank 3.9, p= 0.05). Patients with GD had more BC (86.7% vs. 55.2%; c2= 4.37, p= 0.037). In the HD+GD group, 8 cases (53%) required admission vs.8 patients (31%) in the group HD without GD (c2= 2, p= 0.1). Invasive treatment was performed in 2 patients (13%) in the HD+GD group and 2 others (7.6%) in the group HD without GD (c2= 0.3, p= 0.6). Conclusions: In our series, the BC incidence was higher in patients with HD and GD. There was a trend towards an earlier presentation of BC in this group, but neither this data nor its clinical consequences allow us to recommend early splenectomy

New Presentation of Hemolysis After Papillary Muscles Approximation for Mitral Valve Repair.

Hemolysis is a well-recognized complication after prosthetic valve replacement, especially with perivalvular leaks. Hemolytic anemia associated with mitral valve (MV) repair is less common. We report the case of a young man with severe hemolytic anemia caused by turbulence of blood flow through a very small quadrangle orifice due to early failure of MV repair. The patient underwent redo MV biologic prosthesis replacement and tricuspid valve annuloplasty. The hemolysis completely disappeared few months later. In this case, we describe a new presentation of mechanical hemolysis due to early failure of MV repair that has not been described in the literature.

Hemolytic Anemia due to Right Ventricular to Pulmonary Artery Conduit Stenosis.

Hemolytic anemia is a well-recognized complication in patients with left-sided mechanical heart valves. It is rare to see hemolysis with a bioprosthetic valve in the right ventricular outflow tract. We report a 4-year-old-girl with history of truncus arteriosus status-post repair who developed hemolytic anemia as a result of a calcified and obstructed bioprosthetic right ventricular to pulmonary artery-valved conduit. The hemolysis was alleviated by replacing the obstructed conduit with a larger valved conduit.

Hemolytic anemia caused by aortic flap and inversion of felt strip after ascending aorta replacement.

BACKGROUNDS: Hemolysis related to a kinked prosthetic graft or inner felt strip is a very rare complication after aortic surgery. We describe herein a case of hemolytic anemia that developed due to aortic flap of the dissection and inversion of an inner felt strip that was applied at the proximal anastomosis of a replaced ascending aorta 10 years previously. CASE PRESENTATION: A 74-year-old woman presented with consistent hemolytic anemia 10 years after replacement of the ascending aorta to treat Stanford type A acute aortic dissection. The cause of hemolysis was attributed to mechanical injury of red blood cells at a site of stenosis caused by aortic flap of the dissection and inversion of the felt strip used for the proximal anastomosis. Repeated resection of the strip and graft replacement of the ascending aorta resolved this problem. CONCLUSIONS: We considered that blood flow disrupted by a jet of blood at the site of the proximal inner felt strip was the cause of severe hemolysis, we describe rare hemolytic anemia at the site of aortic flap and inverted felt strip after replacement of the ascending aorta.

Anemia hemolítica secundaria a plastía mitral: caso clínico local y revisión de la literatura / Hemolytic anemia after mitral valve repair: case report

Una mujer de 54 años sometida a una reparación mitral se presenta 2 meses después con anemia hemolítica. El mecanismo de ésta correspondió a dehiscencia de la cuadrantectomía y a falta de endotelización de un sector del anillo, que se evidenció mediante ecografía transesofágica y se confirmó por hallazgos operatorios. Se resecó el anillo mitral y se implantó una prótesis de Saint Jude, y no hubo recurrencia de la hemólisis. Se incluye una discusión con revisión de la literatura.

A 54 year old woman was subjected to a mitral valve repair of a mixomatous degenerative valve with severe mitral insufficiency. Quadrantectomy, mitral chords transfer from P2 to a A2 and implantation of a rigid Edwards 28 ring were performed. Two months later the patient was diagnosed with severe hemolytic anemia. Trans esophageal echocardiography revealed severe mitral insufficiency and at surgery a dehiscence at the base of the qudrantectomy in addition to a non endothelysed sector of the mitral ring were found. A Saint-Jude mitral prosthesis was inserted. At follow up, no recurrence of hemolysis occurred.

Apicoaortic conduit for severe hemolytic anemia after aortic valve replacement.

We describe the case of an 82-year-old woman who had undergone aortic mechanical valve replacement for aortic stenosis with a small annulus, and coronary artery bypass grafting. Four years after the operation, she began to experience hemolysis. Prosthetic valve obstruction was observed but there was no paravalvular leakage or aortic regurgitation through the mechanical valve. We elected to perform apicoaortic bypass in this patient with severe hemolytic anemia secondary to a mechanical valve malfunction.

Elephant trunk in a small-calibre true lumen for chronic aortic dissection: cause of haemolytic anaemia?

OBJECTIVES: The elephant trunk technique for aortic dissection is useful for reducing false lumen pressure; however, a folded vascular prosthesis inside the aorta can cause haemolysis. The purpose of this study was to investigate whether an elephant trunk in a small-calibre lumen can cause haemolysis. METHODS: Inpatient and outpatient records were retrospectively reviewed. RESULTS: Two cases of haemolytic anaemia after aortic surgery using the elephant trunk technique were identified from 2011 to 2013. A 64-year-old man, who underwent graft replacement of the ascending aorta for acute Stanford type A aortic dissection, presented with enlargement of the chronic dissection of the descending aorta and moderate aortic regurgitation. A two-stage surgery was scheduled. Total arch replacement with an elephant trunk in the true lumen and concomitant aortic valve replacement were performed. Postoperatively, he developed severe haemolytic anaemia because of the folded elephant trunk. The anaemia improved after the second surgery, including graft replacement of the descending aorta. Similarly, a 61-year-old man, who underwent total arch replacement for acute Stanford type A aortic dissection, presented with enlargement of the chronic dissection of the descending aorta. Graft replacement of the descending aorta with an elephant trunk inserted into the true lumen was performed. The patient postoperatively developed haemolytic anaemia because of the folded elephant trunk, which improved after additional stent grafting into the elephant trunk. CONCLUSIONS: A folded elephant trunk in a small-calibre lumen can cause haemolysis. Therefore, inserting an elephant trunk in a small-calibre true lumen during surgery for chronic aortic dissection should be avoided.

Esplenectomía laparoscópica por enfermedades hematológicas en niños. Análisis de 11 años / Laparoscopic splenectomy in hematologic diseases in children. Analysis of 11-year experience

Introducción. Una indicación frecuente de esplenectomía en niños son las enfermedades hematológicas. Se muestran las indicaciones, curso clínico y complicaciones en niños con esplenectomía laparoscópica y se analizan los factores de conversión a cirugía abierta. Material y métodos. Estudio de cohorte, longitudinal, descriptivo de esplenectomía laparoscópica en un periodo de 11 años y su seguimiento a largo plazo. Resultados. Se analizaron 88 esplenectomías laparoscópicas. Edad promedio 9 años y peso 30 kg. La indicación fue anemia hemolítica en el 77% y púrpura trombocitopénica en el 18%. El 11% se operó con trombocitopenia grave y dos pacientes en forma urgente, se hizo colecistectomía en el 13%. La mediana de tiempo quirúrgico fue de 180 minutos con sangrado de 100 ml. Se convirtió el 5,7% por sangrado persistente, siendo los factores de riesgo el sangrado mayor a 300 ml y la necesidad de transfusión de concentrado eritrocitario. La mediana de estancia fue 3 días, superior en los pacientes afectos de púrpura. Tres pacientes desarrollaron neumonía y uno, absceso intra-abdominal. El seguimiento promedio es de 3,6 años con dos complicaciones tardías. Hubo curación con normalización del hemograma y sin necesidad de transfusiones en el 72%, mejoría con necesidad de transfusiones ocasionales en el 11,3% y fallo con requerimientos transfusionales iguales a los preoperatorios en el 6,8% de los pacientes. Discusión. Es posible operar a pacientes menores de 5 años y a pacientes con anemia o trombocitopenia en el momento de la cirugía, sin mayor complicación. La tasa de reconversión es baja y los factores de riesgo para reconversión son un sangrado mayor de 300 ml y la necesidad de transfundir concentrado de hematíes

Objectives. A common indication for splenectomy in children is hematologic diseases. Indications, clinical course and complications of laparoscopic splenectomy are shown; factors associated with open conversion are analyzed. Methods. Retrospective, longitudinal, descriptive series of laparoscopic splenectomy during 11 years and its long-term follow-up. Results. We studied 88 laparoscopic splenectomies. Average age was 9 years and weight 30 kg. The indication was hemolytic anemia in 77% and thrombocytopenic purpura in 18%. Eleven percent was operated with severe thrombocytopenia and two patients urgently; cholecystectomy was done in 13%. The median surgical time was 180 minutes and bleeding 100 ml. Conversion rate was 5.7% mainly because of persistent bleeding, being risk bleeding greater than 300 ml and the need for red cell concentrate transfusion. The median length of stay was 3 days, longer in those affected by purpura. Three patients developed pneumonia and one, intra-abdominal abscess. Follow-up was 3.6 years average with two late complications. Treatment response was cure in 72%, improvement in 11.3% and failure in 6.8%.Discussion. It is feasible to operate patients under 5 years of age and patients with anemia or thrombocytopenia at the time of surgery without major problems. The conversion rate is low and risk factors for conversion are bleeding more than 300 ml and needing to transfuse erythrocyte concentrate

Splenectomy-related red cell lysis resistance causing analytical difficulty in a patient with a hematological malignancy.

A patient with a history of chronic lymphocytic leukaemia and a previous splenectomy underwent full blood count analysis in a general hospital. Her medical care had previously taken place in a different institution. A CELL- DYN Sapphire analyser measured her lymphocyte count at ten-fold higher than her known baseline. The sample was sent to her previous hospital, where the laboratory utilises an ADVIA-2120i analyser. The results of this analysis were in keeping with her baseline. The spurious result appears to be related to red cell lysis resistance following splenectomy; however, this resistance appeared to be specific to the analytical method used.