[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.

[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.

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.

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.

Review of surgical prosthetic paravalvular leaks: diagnosis and catheter-based closure.

Paravalvular leak (PVL) is an uncommon yet serious complication associated with surgical prosthetic valve implantation. Paravalvular leak can have significant clinical consequence such as congestive heart failure, haemolytic anaemia, and infective endocarditis. Recently, transcatheter therapy has been applied to the treatment of this disorder with reasonable procedural and clinical success. This review discusses the current state of PVLs, the utilization of multi-modality imaging in their diagnosis and treatment, and the available therapeutic options. Further aim of this review is to examine transcatheter therapy of PVLs including the principles, outcomes, and procedural-related complications.

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.

Intractable hemolytic anemia after mitral valve repair: a report of three cases.

Although hemolytic anemia after mitral valve replacement (MVR) is well recognized, hemolytic anemia associated with mitral valve repair is an uncommon condition. Furthermore, persistent and severe hemolytic anemia despite of medical treatment subsequently requiring reoperation is extremely rare. We here report three cases of intractable hemolytic anemia after mitral valve repair leading to MVR. Collision of regurgitant jet into the annuloplasty ring was the speculated mechanism of hemolysis in all cases. After MVR, all of them experienced immediate resolution of the hemolytic anemia.

Hemolytic anemia case caused by an inverted inner felt after bentall operation.

A 26-yr-old male patient reported worsened dyspnea, dizziness one year after an emergency Bentall operation for type A aortic dissection. There was evidence of hemolytic anemia and aortogram revealed a significant stenosis at the distal anastomosis site. During the reoperation, we found the inner felt at the distal anastomosis was inverted causing a significant stenosis. The reoperation successfully resolved this problem. Here, we report a rare case of hemolytic anemia caused by an inverted inner felt after Bentall operation.

Hemolysis after mitral valve repair.

Hemolysis is a potential complication of mitral valve repair. We report a case of hemolytic anemia after mitral valve repair successfully treated with re-repair, and review the literature on reoperation after failure of mitral valve repair with associated hemolysis.

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.

Pathogenesis of paravalvular leakage as a complication occurring in the late phase after surgery.

Paravalvular leakage (PVL) remains an unavoidable late complication after valve surgery. We indicate surgery only cases with progressive congestive heart failure and/or hemolytic anemia. We review our clinical experiences of PVL surgery. Between 1992 and 2009 we experienced 8 cases of aortic PVL-6 subjects after primary aortic valve replacement (AVR) and 2 subjects after re-AVR-and 10 subjects with mitral PVL-5 cases after primary mitral valve replacement (MVR) and 5 cases after re-MVR. Mitral PVL began in the later phases after surgery and had more severe symptoms, because of heart failure and/or hemolytic anemia, than aortic PVL. Aortic PVL occurred more frequently because of laxation of sutured threads without frequent sites. Conversely, mitral PVL was mainly caused by cutting annulus tissue around the anterior commisurae after primary MVR, and by a valve-on-valve structure on the middle scallop of the posterior leaflet or circumferentially after re-MVR. All operations were performed safely and all patients were discharged uneventfully. No cases experienced recurrence of PVL in the follow-up period. The symptoms of PVL became exacerbated, and our surgical indications based on these symptoms were validated. Valve-on-valve replacement, which was a major cause of PVL after re-MVR, should be avoided in a re-MVR procedure. Cautious follow-up is necessary, even in the late phase after surgery, especially for patients who have undergone MVR.

Liver-kidney transplantation to cure atypical hemolytic uremic syndrome.

Atypical hemolytic uremic syndrome is often associated with mutations in genes encoding complement regulatory proteins and secondary disorders of complement regulation. Progression to kidney failure and recurrence with graft loss after kidney transplantation are frequent. The most common mutation is in the gene encoding complement factor H. Combined liver-kidney transplantation may correct this complement abnormality and prevent recurrence when the defect involves genes encoding circulating proteins that are synthesized in the liver, such as factor H or I. Good outcomes have been reported when surgery is associated with intensified plasma therapy. A consensus conference to establish treatment guidelines for atypical hemolytic uremic syndrome was held in Bergamo in December 2007. The recommendations in this article are the result of combined clinical experience, shared research expertise, and a review of the literature and registry information. This statement defines groups in which isolated kidney transplantation is extremely unlikely to be successful and a combined liver-kidney transplant is recommended and also defines those for whom kidney transplant remains a viable option. Although combined liver-kidney or isolated liver transplantation is the preferred therapeutic option in many cases, the gravity of risk associated with the procedure has not been eliminated completely, and assessment of risk and benefit requires careful and individual attention.

[Robotic splenectomy--a personal view]. / Splenectomia robotica.

Until now 40 robotic splenectomies were performed in our department, the first case being done on February 25, 2008. Our data show that robotic splenectomy with the DaVinci surgical system is technically feasible and safe, with good results and without complications. The main advantages are a better tridimensional view and an increased versatility of the surgical instruments. The DaVinci system allows an accurate dissection around the splenic hilum and preservation of the splenic remnant vessels in partial splenectomy. Robotic splenectomy will probably not replace the laparoscopic splenectomy for the most common indications like ITP, hemolytic anemia. It may be a very useful surgical tool in difficult splenectomy: partial splenectomy, splenectomy in liver cirrhosis, splenic tumors or malignant hemopathies. In these cases the robotic approach may shorten the operative time, decrease the blood loss and the risk of remorrhagic complications during surgery and even make possible a minimally invasive splenectomy very difficult to be performed by classical laparoscopy.

Severe intravascular haemolysis as delayed manifestation of perivalvular leak in patients with mitral valve replacement: a report of two cases.

Haemolytic anaemia following mitral valve replacement is uncommon, however in patients who suffer from some degree of perivalvular leak, severe and potentially fatal recurrent intravascular haemolysis can be an annoying problem. We report the cases of two patients with severe haemolytic anaemia observed some years after mitral valve replacement. In one of the two patients the presence of an association between a valvular leak after mitral valve replacement and a calcific atrial wall produced severe and recurrent haemolysis that required multiple blood transfusions. In the second patient the presence of a single valvular leak after mitral valve replacement induced an episode of haemolytic anaemia some years after the operation. These cases point out that in case of unexplained worsening anaemia, a transthoracic (TT) and transesophageal (TE) echocardiogram should be performed, and the possibility of atrial wall alterations in the producing of anaemia should be kept in consideration. In these cases reoperation resolved the recurrence of anemization.

Eclampsia complicated by abdominal compartment syndrome.

A primigravida with eclampsia and hemolytic anemia, elevated liver enzymes, and low platelet count (HELLP syndrome) developed intra-abdominal compartment syndrome requiring a decompressive laparotomy, underlining the importance of including abdominal compartment syndrome in the differential diagnosis in pregnant women.