Pediatric valve replacement, 15 years experience in Oman

Background Despite improving surgical techniques, treatment of heart valve disease in children remains controversial. Somatic growth and adequate anticoagulation are of concern when children undergo valve replacement. We conducted this study to evaluate the performance of valves in this age group. Methods 42 children under the age of 13 years who underwent valve replacement were included in this study. Totally, 50 valves were implanted in 42 patients: 48 were mechanical prostheses, two were bioprosthetic both in pulmonary position. 37 (74%) valves were implanted in mitral position, 10 (20%) in aortic position, 1 (2%) in tricuspid position and 2 (4%) in pulmonary position. Preoperatively, 14 (33,3%) patients were in New York Heart Association (NYHA) class IV, while 27 (64.2%) were in NYHA class III. Results There were 2 (4.7%) hospital deaths and 2 (4.7%) late deaths while 2 (4.7%) patients were lost to follow up. The mean follow up period was 9.4 yrs. 35 (83.3%) patients are in NYHA Class I and free of all medications except warfarin. 3 (7.1%) patients have undergone 5 successful pregnancies. The median INR was 2.23. Major thrombo-embolic episode occurred in 1 (2.3%) patient. Conclusions In view of the problems of sizing, anticoagulation and need for re-operation at an early age, there is a reluctance to replace valves in children. This study shows that despite these problems, valve replacement can be undertaken safely and successfully in children, when repair has failed or not technically feasible.     

球囊二尖瓣扩张术后心房纤颤复律的临床研究

目的探讨二尖瓣狭窄合并心房纤颤患者在球囊二尖瓣扩张后,转复心房纤颤的临床效果及影响心房纤颤转复后窦性心律维持的相关因素.方法 206例二尖瓣狭窄合并心房纤颤患者在球囊扩张术后,采用口服胺碘酮及电复律治疗心房纤颤,对复律成功的患者给予小剂量胺碘酮维持窦性心律并随访1年.结果服用胺碘酮者58例,17例转复成功(29.3%);189例行电转复,178例转复成功(94.2%),电转复成功率明显高于胺碘酮转复(P＜0.01).心房纤颤复发组与保持窦性心律组相比,左心房内径明显扩大(P＜0.01),心房纤颤持续时间明显长(P＜0.01),中度二尖瓣关闭不全人数的比率明显高(P＜0.05).随访1年,有45例患者心房纤颤复发.影响心房纤颤转复后维持窦性心律的相关因素有左心房内径扩大、心房纤颤持续时间长及明显的二尖瓣关闭不全.结论对左心房内径明显扩大、心房纤颤持续时间过长及有明显的二尖瓣关闭不全的患者,在选择心房纤颤转复时应慎重.     

Unusual presentation and clinical variability in Belgian pedigrees with progressive external ophthalmoplegia and multiple deletions of mitochondrial DNA

We studied 14 patients from three unrelated Belgian pedigrees with a familial mitochondrial disorder and multiple deletions of mitochondrial DNA (mtDNA). In one family with an oculopharyngeal presentation there is a clear autosomal dominant inheritance. Progressive external ophthalmoplegia (PEO), “ragged red fibres” (RRF) and multiple deletions of mtDNA are common to all three families. Therefore a diagnosis of autosomal dominant progressive ophthalmoplegia with multiple deletions of mtDNA (adPEO) was made in one family at least. Our data confirm the previous observations that adPEO is a systemic disorder rather than a pure myopathy. In our pedigrees frequently associated features include axonal peripheral neuropathy, dysphagia, psychiatric illness, and sudden death. Mild ataxia, pes cavus and mitral valve prolapse with associated mitral insufficiency also occur. In some cases onset is atypical with neuropathy, adolescent onset myopathy or psychiatric illness. In such cases the common features of PEO and muscle weakness always complete the clinical phenotype later during the course of the disease. Biochemical studies on mitochondrial fractions prepared from one patient's muscle, revealed no abnormalities of respiratory chain enzyme activities.     

二尖瓣置换合并射频迷宫术的体外循环管理

目的探讨二尖瓣置换合并射频迷宫术的体外循环 (CPB)方法。方法 :76例风湿性心脏二尖瓣病变合并房颤患者实施瓣膜置换时 ,采用心内直视射频消融迷宫术对其合并的心房纤颤予以治疗。先期的 13例 (A组 )患者在CPB转流阻闭升主动脉后进行射频消融术和二尖瓣置换 ;后期 6 3例 (B组 )采用阻断上下腔静脉后先射频消融右心房 ,再行二尖瓣置换和左房射频消融。结果 :A组 13例患者CPB转流 (10 4± 2 5 )min ,主动脉阻断 (6 1± 11)min。B组 6 3例患者CPB转流 (81± 19)min ,主动脉阻断 (47± 11)min ;应用冷晶体心肌保护患者开放升主动脉后心脏自动复跳率 5 5 .9% ,应用冷血心肌保护患者心脏自动复跳率 71.4 %。术后恢复窦性心律 6 4例 ,占 84 .2 %。结论 :在二尖瓣置换合并射频迷宫术的体外循环中阻闭升主动脉前先射频消融左房可以明显缩短体外循环时间和主动脉阻断时间 ;应用冷血心肌保护液可以加强心肌保护效果且射频消融迷宫术治疗顽固性房颤效果明显     

吸入一氧化氮对心瓣膜置换术后肺动脉高压患者血流动力学的影响

目的：观察吸入一氧化氮（ＮＯ）对心瓣膜置换术后肺动脉高压患者血流动力学的影响。方法：选择９例心瓣膜置换术后伴肺动脉高压的病人，吸入ＮＯ０．００３％，观察三个时象点：吸入ＮＯ前；开始吸入ＮＯ后１５分钟；停止吸入ＮＯ后１５分钟。结果：吸入ＮＯ能显著降低肺动脉压和肺循环阻力指数（Ｐ＜０．０１），停止吸入ＮＯ１５分钟后，肺动脉压和肺循环阻力指数恢复到原有水平。在整个观察过程中，心率、平均动脉压、中心静脉压、肺动脉楔压、体循环阻力指数和心脏指数均无显著变化（Ｐ＞０．０５）。结论：吸入ＮＯ具有选择性肺血管扩张作用，是治疗心瓣膜置换术后肺动脉高压的较理想药物。     

二尖瓣环钙化与颈动脉粥样硬化关系的超声研究

目的 了解二尖瓣环钙化（MAC）与颈动脉粥样硬化之间的关系，探讨MAC能否作为颈动脉粥样硬化的独立预测因子。方法 采用高频超声对156例MAC患者（其中55例为重度MAC患者）及118例年龄、性别与之相匹配的无MAC对照者的颈动脉进行了检测。结果 ①与对照组比较，MAC组及重度MAC组患者高血压和糖尿病的患病率明显增加（P〈0．05）；②MAC组颈动脉粥样硬化斑块发生率，颈动脉狭窄≥20％、≥40％及双侧颈动脉狭窄≥40％的发生率均显著高于对照组（P〈0．01），重度MAC组上述指标的改变更为显著（P〈0．01）。此外，重度MAC组颈动脉狭窄≥60％的发生率明显高于对照组（P〈0．05）；③MAC组及重度MAc组颈动脉内-中膜厚度（IMT）显著高于对照组（P〈0．01）；④单因素及多因素Logistic回归分析结果均表明：MAC是颈动脉狭窄≥40％最有意义的预测因子（P〈0．01）。结论 MAC与颈动脉粥样硬化之间存在十分密切的关系，通过对MAC的检测，能够预测颈动脉粥样硬化的存在及其程度，在临床实际工作中，对于有二尖瓣环钙化的患者应常规进行颈动脉粥样硬化的检查。     

从二尖瓣瓣环三维形态的研究探讨合理的瓣环成形术

观察１５个固定心脏标本、１２个非固定心脏标本二尖瓣瓣环三维形态，运用动态三维超声心动图重建了１１名健康成年人的二尖瓣瓣环。发现二尖瓣瓣环为非平面的“马鞍”形，在心动周期中二尖瓣瓣环始终保持着程度不同的“马鞍”形。结果认为用硬质人工二尖瓣瓣环作瓣环成形术后的某些并发症，可能与改变了二尖瓣瓣环的天然三维形态有关。