Hemolytic Anemia in a Patient with Subacute Bacterial Endocarditis by Cardiobacterium hominis

Hemolytic anemia is a rarely occurring manifestation of native valve infective endocarditis. We herein report an afebrile patient with hemolytic anemia caused by Cardiobacterium hominis endocarditis. A 60-year-old Japanese man had a history of aortic root replacement and the gradual onset of general fatigue. He had hemolytic anemia. Blood cultures detected C. hominis. A transthoracic echocardiogram showed aortic valve vegetation and periannular abscess with perforation of the non-coronary cusp. Intravascular hemolysis recovered after antimicrobial therapy, surgical removal of the vegetation and abscess, and aortic valve replacement. Subacute endocarditis should be considered if patients develop hemolytic anemia with signs of chronic inflammation without a fever.     

Clinical and hemodynamic follow-up of a patient after operation for dissection of an ascending aortic aneurysm secondary to coarctation of the aorta

We present clinical follow-up of a 20-year-old male with an aortic aneurysm secondary to aortic coarctation. The diagnosis of aortic aneurysm secondary to aortic coarctation was made in 1997. The patient did not agree to undergo any invasive or therapeutic procedures at that time. He presented to an emergency unit with severe chest pain after chest trauma obtained during judo exercises in 1998. Two-dimensional echocardiography showed bicuspid aortic valves, an ascending aortic aneurysm 6 cm in diameter with an intimal flap and false lumen, aortic coarctation distal to the left subclavian artery, and aortic insufficiency secondary to annular dilatation. Type II aortic dissection was confirmed by transesophageal echocardiography, which showed the dissection was confined to the ascending aorta. The dissection extended to the beginning of the arcus aorta. Following stabilization of the patient's clinical condition, balloon coarctation angioplasty was performed to reduce afterload and hypertension and to facilitate femoral artery cannulation for cardiopulmonary bypass. Surgical procedures included resection of the aortic valve and prosthetic valve implantation, resection of the ascending aorta, and interposition of a 22 mm Hamashied tubular vascular graft. At a follow-up visit 6 years later, the patient reported being easily fatigued and having palpitations. He had been suffering from hemolytic anemia and mild renal function impairment. Cardiac catheterisation and angiography showed a 40 mmHg gradient due to kinking of the aortic graft and no gradient at the coarctation site. We postulated the kinking of the aortic vascular graft may be related to an inappropriate vascular graft length. We also thought that the severe hemolysis was attributable to the disturbance of blood flow by a jet of blood at the site of the kinking aortic vascular graft. A second operation was performed because the renal function of the patient had decreased progressively and hemolysis symptoms increased. After the second operation, hemolysis on peripheral blood smears had disappeared and renal function had shown progressive improvements.     

Partial dehiscence of a porcine prosthesis in association with aortic annular separation producing a false aneurysm of the ascending aorta

A 44-year-old man developed intermittent left bundle branch block followed by complete heart block 18 months after aortic valve replacement, and aortic insufficiency and hemolytic anemia 5 years postoperatively. These abnormalities were associated with operative findings (7 years after aortic valve replacement) of total disruption of the aorta at the level of the aortic annulus. Echocardiographic findings of an enlarged and unusually shaped "aortic root" and angiographic findings of abnormally high origin of the coronary arteries relative to the valve ring, which should have suggested the diagnosis 2 years prior to successful surgical repair, are presented.     

Hemolytic anemia caused by graft-versus-host reaction in ABO-nonidentical renal transplants from blood group O donors

Acute hemolytic anemia is one of the side effects associated with cyclosporin and tacrolimus therapy, and three mechanisms have been described to account for hemolytic anemia in patients receiving these drugs: drug induced hemolysis, autoimmune hemolysis and alloimmune hemolysis resulting from donor lymphocytes derived from the allograft (passenger lymphocyte syndrome). We report four cases of renal transplant recipients who developed alloimmune hemolytic anemia due to minor ABO incompatibility while under treatment with cyclosporin (two) and tacrolimus (two). The anti-erythrocyte antibodies responsible for hemolysis were of the IgG isotype and showed anti-A or anti-B specificity. These findings suggest that the hemolysis could be related to alloantibodies derived from the clonal development of donor B lymphocytes in the recipients (microchimerism). In summary, hemolytic anemia due to ABO-minor incompatibility occurs infrequently after renal transplantation. Risks are higher for patients A, B or AB blood group receiving an O blood group graft under treatment with cyclosporin or tacrolimus. Follow-up of these patients is warranted for the early detection and optimal management may be achieved by reduction of immunosuppression and change to mycophenolate mofetil.     

Aortic stenosis and mitral regurgitation complicated by hemolytic anemia and positive Direct Coombs test: a case report

A 83-year-old man was admitted because of heart failure due to severe aortic stenosis and mitral regurgitation secondary to chordal rupture of the anterior leaflet. Mild anemia and elevated serum lactate dehydrogenase were present with reticulocytosis and haptoglobinemia. Direct Coombs test was positive. Coexistence of autoimmune hemolytic anemia was identified, but the main cause of his hemolysis was thought to be mechanical hemolysis due to stenotic valve and/or ruptured chordae because of the presence of red cell fragmentation. The patient successfully underwent double valve replacement. Improvement of anemia was coupled with reduction of the serum lactate dehydrogenase level. Valvular shear stress on the red cells and reduction of red cell deformability secondary to autoimmune hemolytic anemia were thought to be responsible for his hemolysis.     

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.     

A new surgical technique for treatments of left ventricular outflow tract obstructions. Report of a case and reexamination of our experience (author's transl)]

Aortic valvotomy, aortic valve replacement, resection of the subaortic membrane and aortic patch angioplasty do not always provide satisfactory relief of left ventricular outflow tract obstruction. From August 1975 through January 1977, 15 patients underwent creation of a left ventricular vent by means of a Dacron fabric conduit containing a heterograft valve going from the left ventricle to the supraceliac portion of the abdominal aorta (Cooley's point). All patients had valvular, subvalvular, supravalvular aortic stenosis, or combined defects. One patient had severe hemolytic anemia following aortic valve replacement. Eleven of our 15 patients had undergone one or more previous surgical procedures for relief of aortic stenosis. The operative mortality has been one patient. At postoperative catheterization, there was a normalization of pressure gradients, and the blood flow through the conduit was 40% of the cardiac output. An interesting case of supravalvular aortic stenosis associated with stenosis of the vessels of the arch is presented.     

Valve dysfunction of the cloth-covered Starr-Edwards ball valve.

BACKGROUND: Between June 1968 and March 1977, Starr-Edwards cloth-covered ball valves were used for valve replacement on a routine basis. METHODS AND RESULTS: Among the 66 operative survivors who underwent an isolated aortic or mitral valve replacement, 20 patients required reoperation 22 times because of valve dysfunction, thromboembolic complication, paravalvular leakage, hemolytic anemia, and/or prosthetic valve endocarditis. Reoperation was performed at a mean of 15.9+/-9.8 years after initial replacement. Excised valves were examined and reoperation after initial operation was reviewed. Operative mortality was 10.0%. Freedom from reoperation for aortic valve replacement and mitral valve replacement was 56.2% at 34 years and 61.0% at 37 years after initial operation, respectively. Cloth wear or pannus formation were observed in all excised prostheses. Orifice cloth was more markedly worn in mitral valves than in aortic valves, particularly in mitral valves of more than 20 years old. Pannus overgrowth contributed to valve regurgitation in the older valves. CONCLUSIONS: Early diagnosis of valve dysfunction and reoperation are recommended as soon as symptoms appear.     

先天性主动脉缩窄合并严重主动脉瓣病变的一次性外科矫治

目的 :探讨先天性主动脉缩窄合并严重主动脉瓣病变的外科治疗方法。方法 :采用升主动脉—腹主动脉人工血管转流术 +主动脉瓣置换术一次性治疗患者 9例 ,其中 4例对手术方法和体外循环技术进行了改进。结果 :术中死于体外循环意外 1例 ;术后部分肠梗阻 1例 ,8例患者痊愈出院。上肢收缩压术后较术前下降〔平均12 2 /78mmHgvs .178/6 5mmHg( 1mmHg =0 133kPa)〕 ,有显著性差异 (P <0 0 5 ) ,术后上、下肢血压无明显压差。左心室舒张末期内径术后较术前缩小 ( 4 9 40± 6 88mmvs .6 7 33± 17 2 9mm) ,有显著性差异 (P <0 0 5 )。结论 :该类患者采用升主动脉—腹主动脉人工血管转流术 ,在常规体外循环下行主动脉瓣置换术即简便又安全有效     

Hb Alesha [β67(E11)Val→Met (GTG>ATG); HBB: c.202G > A] Found in a Chinese Girl

Mutations that cause destabilization of the hemoglobin (Hb) tetramer are a rare cause of hemolytic anemia. In contrast to the hemolytic anemia caused by enzyme deficiencies, a dominant mode of inheritance characterizes the unstable Hbs. Hb Alesha [β67(E11)Val→Met; HBB: c.202G>A] is caused by a G>A mutation at codon 67 of the β-globin gene, resulting in a valine to methionine substitution at helix E11. This replacement disrupts the apolar bonds between valine and the heme group, producing an unstable Hb and severe hemolysis. We report this rare hemoglobinopathy in a Chinese girl with severe hemolytic anemia, splenomegaly and frequent requirement for red blood cell (RBC) transfusions.     

Acute fulminant hemolysis after transcatheter mitral valve replacement for mitral annular calcification

Transcatheter mitral valve replacement (TMVR) is emerging as an alternative treatment strategy to surgery for patients with severe mitral annular calcification (MAC) who are not candidates for traditional mitral valve surgery. Paravalvular leak (PVL) is common following TMVR for severe MAC and can lead to heart failure symptoms and/or intravascular hemolysis, the latter of which usually is clinically stable. We report the case of a 67‐year‐old woman with symptomatic severe aortic stenosis and mitral stenosis with MAC in the setting of prior chest irradiation who was treated initially with transcatheter aortic valve replacement followed by TMVR at a later date (Sapien S3 system; Edwards Lifesciences). Immediately following TMVR, she developed acute profound hemolysis which manifested with hemoglobinuria, transfusion‐dependent anemia, and acute renal failure requiring renal replacement therapy. She was treated with post‐dilation balloon valvuloplasty after failed transcatheter PVL closure 10 days following TMVR with resulting improvement in the PVL. The hemolytic anemia resolved and renal function recovered without the need for continued hemodialysis 2 months later and stabilization of glomerular filtration rate at 6 months. This case highlights a potential severe complication of TMVR in MAC and suggests that improvement in hemolysis and late recovery of renal function may occur following treatment of PVL.     

Stanford A型主动脉夹层的外科治疗

目的 总结A型主动脉夹层外科治疗经验,探讨治疗A型主动脉夹层安全有效的术式和方法.方法 我院2008年1月至2013年11月对40例A型主动脉夹层患者予以外科治疗.Bentall（带瓣人造血管替代升主动脉根部和主动脉瓣膜,并移植左右冠状动脉）手术17例,其中10例同期行主动脉弓部替换＋降主动脉象鼻支架置入术;单纯升主动脉人工血管置换术8例;窦部成形＋主动脉瓣交界悬吊术6例,窦部替换＋主动脉瓣成形＋升主动脉半弓替换5例;升主动脉人工血管置换术＋主动脉全弓替换4例.采用深低温停循环技术（DHCA）12例,其余为浅中低温体外循环.采用冷血心脏停搏液灌注12例,组氨酸-色氨酸-酮戊二酸（HTK）停搏液灌注7例,冷晶体心脏停搏液21例.采用改良超滤技术19例.结果 手术死亡1例,围术期死亡4例,死亡率12.5％（5/40）,余均痊愈出院.结论 细化A型主动脉夹层的分型有利于制订个体化手术方案.术中止血彻底及心肌、脑保护确切可提高手术成功率.     

Reduction of hemolysis without reoperation following mitral valve repair.

Hemolytic anemia following mitral valve repair and annular ring placement is uncommon compared with mitral valve replacement. A 60-year-old man, who had undergone mitral valve repair with a Duran ring, developed hemolytic anemia and needed a blood transfusion. Transesophageal echocardiography revealed a paravalvular mitral regurgitation jet colliding with the Duran ring. Most cases of severe hemolysis after mitral valve repair have undergone reoperation, but in the present case study, the hemolysis after mitral valve repair reduced without the need for reoperation, although the paravalvular mitral regurgitation jet continued to collide with the Duran ring.     

Reversed root for entirely biological aortic valve and ascending aorta replacement in a patient with endocarditis and mediastinitis

A 59-year-old patient developed endocarditis with coagulase-negative staphylococci after aortic composite graft replacement and pacemaker implantation. She underwent complete pacemaker removal and tricuspid valve reconstruction. Pus was present in and around the aortic graft. Re-replacement of the aortic root and ascending aorta using only biological material was performed. A 23-mm full root stentless prosthesis (Vascutek Root Elan, Vascutec, Terumo, Leeds, UK) was used for aortic root replacement, and a second 25-mm full root prosthesis with removed cusps implanted in a reversed fashion was used for ascending aorta and proximal aortic arch replacement.     

Pulmonary hypertension after splenectomy in hereditary stomatocytosis

Hereditary stomatocytosis is an autosomal dominant, congenital hemolytic form of anemia, and thromboembolic complications may ensue after a splenectomy is performed in a patient with hereditary stomatocytosis. However, little is known about the relationship between pulmonary hypertension and hereditary stomatocytosis. We describe here a rare case of a patient with hereditary stomatocytosis who developed pulmonary hypertension due to thromboembolic disease after splenectomy. At the first presentation, he was diagnosed with hemolytic anemia, but the cause was unknown, and he was also diagnosed with pulmonary hypertension. Thereafter, we linked his pulmonary hypertension with hemolytic anemia because his red blood cells appeared as leptocytes, acanthytes, and stomatocytes on scanning electron microscopy. It is important to confirm the diagnosis of hemolytic anemia, and splenectomy should be performed with due consideration of whether patients have hemolytic anemia.     

Aortic insufficiency in a patient with Marfan syndrome after aortic root reconstruction with a tailored-sinus graft

A patient with Marfan syndrome underwent valve-preserving aortic root reconstruction with a Robicsek-Thubrikar graft. Intraoperative transesophageal echocardiography showed aortic insufficiency after extracorporeal circulation was discontinued. Placing 3 subcommissural annuloplasty sutures corrected the severe aortic insufficiency. Herein, we discuss the mechanism and prevention of aortic regurgitation after aortic root replacement with a new graft that contains pre-designed aortic sinuses.     

Intravascular hemolysis in aortic stenosis

A 67-year-old woman with rheumatic aortic stenosis for 20 years was admitted to our hospital. Although she had no overt symptoms, she had severe aortic valve stenosis with a transvalvular pressure gradient of more than 150 mmHg. She had also been suffering from anemia and mild chronic renal failure. A peripheral blood smear showed numerous fragmented erythrocytes. Hemoglobin was 8.4 g/dl, lactate dehydrogenase was 316 IU/l, haptoglobin was less than 7.3 mg/dl, and hemosiderinuria was evident. We diagnosed intravascular hemolysis related to aortic stenosis. After we performed an aortic valve replacement, fragmentation on the peripheral blood smear dramatically disappeared.     

Production of the effector cytokine interleukin-17, rather than interferon-γ, is more strongly associated with autoimmune hemolytic anemia

Background Interleukin-17A is the signature cytokine of the Th17 subset and drives inflammatory pathology, but its relevance to autoantibody-mediated diseases is unclear. Th1 cells secreting interferon-γ have been implicated in autoimmune hemolytic anemia, so the aim was to determine which cytokine is more closely associated with disease severity. DESIGN AND METHODS: Interferon-γ and interleukin-17A were measured in the sera of patients with autoimmune hemolytic anemia and healthy donors, and in peripheral blood mononuclear cell cultures stimulated with autologous red blood cells, or a panel of peptides spanning red blood cell autoantigen. RESULTS: Serum interleukin-17A, but not interferon-γ, was significantly raised in patients with autoimmune hemolytic anemia (P <0.001), and correlated with the degree of anemia. Interleukin-17A was also more prominent in the responses of peripheral blood mononuclear cells from patients with autoimmune hemolytic anemia to red blood cells, and, again unlike interferon-γ, significantly associated with more severe anemia (P <0.005). There were no interleukin-17A responses to red blood cells by peripheral blood mononuclear cells from healthy donors. Specific autoantigenic peptides were identified that elicit patients' interleukin-17A responses. Conclusions Interleukin-17A makes a previously unrecognized contribution to the autoimmune response in autoimmune hemolytic anemia, challenging the model that the disease is driven primarily by Th1 cells. This raises the possibility that Th17, rather than Th1, cells should be the target for therapy.     

Acute supravalvular aortic stenosis following the replacement of the ascending aorta.

We report a very rare case of ascending aortic replacement complicated by acute supravalvular aortic stenosis. A 53-year-old man was referred to our Institution for evaluation of a systolic murmur and congestive heart failure. He had undergone elective ascending aortic replacement one month previously due to acute type A aortic dissection. On admission, transesophageal echocardiography revealed a proximal leak with a pseudoaneurysm compressing the aortic graft and generating a systolic gradient of 84 mmHg. The patient underwent reoperation, the graft was removed and a new Dacron graft was put in place.     

Successful repositioning of apically tethered transcatheter mitral valve replacement in the off‐bypass,beating heart

A 73‐year‐old man with severe, symptomatic secondary mitral regurgitation (MR) underwent successful transcatheter mitral valve replacement using the Tendyne? mitral valve system. The device was deployed from the left ventricular (LV) apex and secured in position by a tether attached to an epicardial pad. Three days postoperatively, the patient developed hemolytic anemia and a paravalvular leak (PVL) associated with indentation of the LV apex. Adjustment of the tether tension and placement of an epicardial disc under the pad resulted in reduction in the PVL, and resolution of the hemolytic anemia.