Aortic and Mitral Valve Replacement in a Patient with Severe Haemophilia A

Abstract: Aortic and mitral valve replacement in a patient with severe haemophilia A. P. D. Meagher, K. A. Rickard, J. G. Richards and D. K. Baird, Aust. N.Z. J . Med., 1981, 11, pp. 7679.
A forty-nine year old man with severe Haemophilia A developed left ventricular failure as a complication of valvular heart disease. On November 26th, 1979, open heart surgery was performed and both the aortic and mitral valves were replaced with porcine heterografts
Replacement therapy in the form of Factor Vlll concentrates maintained the Factor Vlll coagulant level at 100% until ten days post-operatively and a total dose of 116,600 units were given
The patient described is the first haemophiliac to undergo open heart surgery in Australia and the first reported case in the world to have had a successful double valve graft
With the current availability of factor Vlll concentrates, elective surgical procedures are being performed more frequently. Such procedures require careful pre-operative planning and post-operative monitoring of replacement therapy to ensure satisfactory haemostasis. Although haemostatic defects may occur after open heart surgery, this case report illustrates that the haemostatic problems of open heart surgery and haemophilia can be prevented by pre-operative planning and meticulous postoperative monitoring of replacement therapy     

Successful treatment with arsenic trioxide of a patient with ATRA-resistant relapse of acute promyelocytic leukemia

 Arsenic trioxide has recently been introduced as a promising new agent to treat refractory acute promyelocytic leukemia (APL). In the present study, arsenic trioxide was given intravenously for 42 days to a 56-year-old female patient suffering from chemotherapy/ATRA-resistant APL, with 43% APL blasts in the bone marrow and elevated D-dimers. During the first days of arsenic trioxide treatment a rapid decrease in the D-dimers was seen (normal values reached until day 7), together with a slight decrease in peripheral blood leukocytes. This initial coagulation response was followed by a second phase of hematological response (starting on days 15–20) characterized by leukocytosis, occurrence of myeloid progenitor cells in the peripheral blood, and a decrease in bone marrow blasts (<1% on days 28 and 36). Finally, the patient entered complete hematological and cytogenetic remission, although the PML-RARα fusion product was still detectable by PCR. These data confirm the therapeutic value of arsenic trioxide in relapsed/resistant APL. Received: February 10, 1999 / Accepted: March 29, 1999     

Valve Replacement with a Sutureless Aortic Prosthesis in a Patient with Concomitant Mitral Valve Disease and Severe Aortic Root Calcification

Aortic valve replacement with concomitant mitral valve surgery in the presence of severe aortic root calcification is technically difficult, with long cardiopulmonary bypass and aortic cross-clamp times.We performed sutureless aortic valve replacement and mitral valve annuloplasty in a 68-year-old man who had severe aortic stenosis and moderate-to-severe mitral regurgitation. Intraoperatively, we found severe calcification of the aortic root. We approached the aortic valve through a transverse aortotomy, performed in a higher position than usual, and we replaced the valve with a Sorin Perceval S sutureless prosthesis. In addition, we performed mitral annuloplasty with use of an open rigid ring.The aortic cross-clamp time was 63 minutes, and the cardiopulmonary bypass time was 83 minutes. No paravalvular leakage of the aortic prosthesis was detected 30 days postoperatively.Our case shows that the Perceval S sutureless bioprosthesis can be safely implanted in patients with aortic root calcification, even when mitral valve disease needs surgical correction.     

Successful Management of Chronic Refractory Immune Thrombocytopenia with Laparoscopic Splenectomy in a Patient with Acute Promyelocytic Leukemia

Acute promyelocytic leukemia (APL) is a particular type of acute myeloid leukemia with characteristic biological and clinical features, the frequent association at diagnosis of a severe hemorrhagic diathesis. Immune thrombocytopenia (ITP) is a common autoimmune disease characterized by low platelet counts and an increased risk of bleeding. Here we present a patient with the diagnosis of APL who achieved and maintained a remission with an induction consisting of idarubicin and ATRA, and then developed corticosteroid refractory ITP which is successfully treated with laparoscopic splenectomy.     

Late relapse of acute promyelocytic leukemia treated with all-<Emphasis Type="Italic">trans</Emphasis> retinoic acid and chemotherapy: report of two cases

Two patients with acute promyelocytic leukemia (APL) relapsed at 111 and 84 months after achievement of complete remission (CR) induced by a combination of all-trans retinoic acid and chemotherapy. In both patients molecular remission, obtained after consolidation, had been confirmed at 60 months from CR achievement. At relapse, morphological, immunophenotypic, cytogenetic, and molecular analyses showed findings identical to those at diagnosis. Hematological and molecular remission was induced with the identical treatment applied at diagnosis. We conclude that, although infrequently, patients with APL treated with modern combination therapy can experience very late relapse and can be rescued with treatment similar to that administered at diagnosis.Grant from COFIN (MIUR, Rome, Italy), Regione Campania (Italy), CNR (Rome, Italy), AIL (Rome, Italy)     

Outcomes of high-risk acute promyelocytic leukemia patients treated with arsenic trioxide (ATO)/all trans retinoic acid (ATRA) based induction and consolidation without maintenance phase: A case Series

Patients with high-risk acute promyelocytic leukemia (APL) have inferior outcomes compared with patients with low-risk APL, predominantly due to higher risk of early mortality related to hemorrhage. The majority of regimens contain prolonged maintenance, but the impact of this phase is not clear in the era of all trans retinoic acid (ATRA) and arsenic trioxide (ATO). We present a retrospective analysis of 10 patients that were treated for high risk APL based on the consolidation treatment phase of APL 0406 study without subsequent maintenance. With a median follow up of 38 months, all patients remain in remission.     

Staged Balloon Aortic Valvuloplasty before Standard Aortic Valve Replacement in Selected Patients with Severe Aortic Valve Stenosis

This study evaluated preoperative balloon aortic valvuloplasty (BAV) as a technique to decrease aortic valve replacement (AVR) risk in patients who have severe symptomatic aortic valve stenosis with substantial comorbidity.We report the outcomes of 18 high-risk patients who received BAV within 180 days before AVR from November 1993 through December 2011. Their median age was 78 years (range, 51–93 yr), and there were 11 men (61%). The pre-BAV median calculated Society of Thoracic Surgeons Predicted Risk of Mortality (STS PROM) was 18.3% (range, 9.4%–50.7%). Preoperative left ventricular ejection fraction measured a median of 0.23 (range, 0.05–0.68), and the median aortic valve area index was 0.4 cm²/m² (range, 0.2–0.7 cm²/m²). The median interval from BAV to AVR was 28 days (range, 1–155 d). There were no strokes or deaths after BAV; however, 4 patients (22%) required mechanical circulatory support, 3 (17%) required femoral artery operation, and 1 (6%) developed severe aortic valve regurgitation. After BAV, the median STS PROM fell to 9.1% (range, 2.6%–25.7%) (compared with pre-BAV, P <0.001). Echocardiography before AVR showed that the median left ventricular ejection fraction had improved to 0.35 (range, 0.15–0.66), and the aortic valve area index to 0.5 cm²/m² (range, 0.3–0.7 cm²/m²) (compared with pre-BAV, both P <0.05). All patients received AVR. Operative death occurred in 2 patients (11%), and combined operative death and morbidity in 7 patients (39%).Staged BAV substantially reduces the operative risk associated with AVR in selected patients.     

Valve-Sparing Aortic Root Replacement and Tricuspidization in a Patient with an Asymmetric Bicuspid Aortic Valve

Aortic valve replacement is the standard surgical procedure for severe aortic regurgitation. Due to advances over the past decade, there have been substantial improvements in aortic root graft design, in aortic valve repair techniques, and in the understanding of valvular function in the remodeled aortic root. Herein, we describe the case of a dyspneic patient with an asymmetric bicuspid aortic valve who underwent valve-sparing aortic root replacement and tricuspidization. The patient subsequently resumed strenuous physical activity and was asymptomatic 2 years after the operation.Key words: Aortic diseases/pathology/surgery/ultrasonography, aortic valve/abnormalities, aortic valve insufficiency/complications/surgery/ultrasonography, cardiac surgical procedures/methods, suture techniques, treatment outcomeThe conventional surgery for severe aortic regurgitation has been aortic valve replacement. This procedure, however, may soon become obsolete due to advances in aortic valve repair. Over the past decade, there have been substantial improvements in aortic root graft design, in aortic valve repair technique, and in the understanding of valvular function in the remodeled aortic root.^1–7 Herein, we describe the case of a patient with complex aortic root disease who underwent surgical repair.     

24例重型再生障碍性贫血患者抗胸腺细胞球蛋白联合环孢素治疗的疗效分析

目的：分析重型再生障碍性贫血（再障）患者采用ATG和环孢素联合免疫抑制治疗（IST）的疗效。方法：回顾性分析使用IST的24例重型再障患者临床资料,并随访8～79个月,分析IST的疗效及可能影响疗效的因素。结果：使用IST方案总体有效率达75.0%。对IST治疗有效的患者,白细胞恢复时间为15～42d,中位恢复时间为24d。获得治愈的6例患者,血小板恢复时间为2～6个月。合用雄激素的患者治疗反应率为82.4%,高于未合用雄激素患者的57.1%。＋30d时中性粒细胞绝对值（N）≥1.5×109/L的患者有效率为86.7%,N〈1.5×109/L患者的有效率为50.0%。有18例患者拟诊为真菌感染,1例合并浸润性肺结核。＋30d时N〈1.5×109/L的8例患者均出现典型的肺部真菌感染灶。结论：IST可有效减少输血依赖。合用雄激素可增加治疗反应率;治疗＋30d中性粒细胞绝对值≥1.5×109/L可能提示患者对IST反应好。中性粒细胞减少时间长的患者,感染发生率高,尤其是真菌感染。     

Transcatheter Aortic Valve Replacement in Low-Risk Patients With Symptomatic Severe Bicuspid Aortic Valve Stenosis

ObjectivesThe aim of this study was to evaluate clinical outcomes and transcatheter heart valve hemodynamic parameters after transcatheter aortic valve replacement (TAVR) in low-risk patients with bicuspid aortic stenosis (AS).BackgroundTAVR is approved for low-risk patients in the United States. However, patients with bicuspid AS were excluded from the randomized cohorts of the pivotal low-risk trials.MethodsThe LRT (Low Risk TAVR) trial was an investigator-initiated, prospective, multicenter study and was the first and only U.S. Food and Drug Administration–approved investigational device exemption trial to evaluate the feasibility of TAVR with either balloon-expandable or self-expanding valves in low-risk patients with bicuspid AS. The primary endpoint was all-cause mortality at 30 days. Baseline and follow-up echocardiography and computed tomography to detect leaflet thickening were analyzed in an independent core laboratory.ResultsSixty-one low-risk patients with symptomatic, severe AS and bicuspid aortic valves (78.3% Sievers type 1 morphology) underwent TAVR at 6 centers from 2016 to 2019. The mean age was 68.6 years, and 42.6% were men. At 30 days, there was zero mortality and no disabling strokes. The rate of new permanent pacemaker implantation was 13.1%; just 1 patient had a moderate paravalvular leak at 30 days. Hypoattenuated leaflet thickening was observed in 10% of patients at 30 days.ConclusionsTAVR appears to be safe in patients with bicuspid AS, with short length of hospital stay, zero mortality, and no disabling strokes at 30 days. Subclinical leaflet thrombosis was observed in a minority of patients at 30 days but did not appear to be associated with clinical events.     

Alcohol Septal Ablation in a Young Patient after Aortic Valve Replacement

A 38-year-old male presented with heart failure symptoms and was diagnosed with aortic valve endocarditis and underlying aortic stenosis in the absence of concentric hypertrophy or bicuspid aortic valve and underwent aortic valve replacement but continued to have symptoms which were then attributed to hypertrophic cardiomyopathy with dynamic left ventricular outflow tract obstruction. He was determined to be unsuitable for myomectomy and underwent successful alcohol septal ablation using transthoracic echocardiographic Doppler and continuous wave velocity monitoring without requiring to cross the aortic valve or to perform transatrial septostomy and left ventricular pressure monitoring. When crossing the aortic valve is a relative or absolute contraindication like in our index case, continuous Doppler velocity recording is a safe and effective alternative approach to monitor the outflow gradient while performing alcohol septal ablation .     

重度主动脉瓣狭窄手术的麻醉处理经验

目的:总结重度主动脉瓣狭窄手术的麻醉处理经验.方法:选自2005-01至2009-10我院成人重度主动脉瓣狭窄并实施主动脉瓣置换术患者118例,其中男85例、女33例,年龄37~74(53±11)岁,体重57~103(68±10)kg.根据术前主动脉瓣收缩期跨瓣压差大小将患者分为:压差>90 mmHg组(η=62)和压差≤90 mmHg组(η=56).对两组患者的麻醉方法,血流动力学管理及术后转归进行回顾性总结.结果:压差>90 mmHg组患者与压差≤90 mmHg组比较,主动脉瓣收缩期压差增大[(121±26.9)mmHg与(69.8±14.1)mmHg,P<0.01]、左心室壁厚度增厚[(13.6±2.8)mm与(12.4±2.5)mm,P<0.05]、自麻醉诱导至体外循环前低血压发生率增加(35.5%与16.1%,P<0.01)、术中多巴胺使用率增加(66.7%与56.5%,P<0.01)、心脏复苏困难发生率增加(24.2%与14.3%,P<0.01),术后气管插管留置时间延长[(14.8±7.1)h与(10.2±4.1)h,P<0.01]、重症监护病房停留时间延长[(45.3±27.6)h与(31.9±19.1)h,P<0.01],住院期间死亡率增加(3.2%与0,P<0.01),差异均有统计学意义.结论:主动脉瓣狭窄程度决定患者术中并发症的发生率和术后的转归.麻醉管理的目标是避免麻醉后发生低血压.