Right coronary fistula and aneurysm draining to the right atrium

In a 3-year-old boy, a continuous heart murmur was heard. The echocardiogram showed a dilated right coronary artery suggesting the existence of a coronary fistula. A more detailed echocardiogram when the patient was sedated revealed a fistula leading to a large aneurysm and further to the right atrium. The accidental dissection and thrombosis during the interventional heart catheterization resulted in a closure of the fistula. A continuous heart murmur and a dilated coronary artery are the hallmarks of coronary fistula.
Conclusion:  Anatomic details of coronary fistula might be possible to see in an echocardiogram. Interventional heart catheterization is usually an adequate treatment option.     

A Novel Approach to Gathering and Acting on Relevant Clinical Information: SCAMPs

The current tools to adequately inform the process of improving health-care delivery consist primarily of retrospective studies, prospective trials, and clinical practice guidelines. We propose a novel and systematic approach that bridges the gap of our current tools to affect change, provides an infrastructure to improve health-care delivery, and identifies unnecessary resource utilization. The objective of this special article is to introduce the rationale and methods for this endeavor entitled “Standardized Clinical Assessment and Management Plans” (SCAMPs). SCAMPs take a relatively heterogeneous patient population and through a process of iterative analysis and modification of standardized assessment and management algorithms, SCAMPs allow the intrinsic biologic variability in a patient population to emerge and be understood. SCAMPs can be used to complement our currently available tools in order to result in incremental and sustained improvement in health-care delivery.     

Ilizarov Treatment of Congenital Pseudarthrosis of the Tibia: A Multi-Targeted Approach Using the Ilizarov Technique

Congenital pseudarthrosis of the tibia (CPT) is one of the most challenging problems in pediatric orthopaedics. The treatment goals are osteosynthesis, stabilization of the ankle mortise by fibular stabilization, and lower limb-length equalization. Each of these goals is difficult to accomplish but regardless of the surgical options, the basic biological considerations are the same: pseudarthrosis resection, biological bone bridging of the defect by stable fixation, and the correction of any angular deformity. The Ilizarov method is certainly valuable for the treatment of CPT because it can address not only pseudarthrosis but also all complex deformities associated with this condition. Leg-length discrepancy can be managed by proximal tibial lengthening using distraction osteogenesis combined with or without contralateral epiphysiodesis. However, treatment of CPT is fraught with complications due to the complex nature of the disease, and failure is common. Residual challenges, such as refracture, growth disturbance, and poor foot and ankle function with stiffness, are frequent and perplexing. Refracture is the most common and serious complication after primary healing and might result in the re-establishment of pseudarthrosis. Therefore, an effective, safe and practical treatment method that minimizes the residual challenges after healing and accomplishes the multiple goals of treatment is needed. This review describes a multi-targeted approach for tackling these challenges, which utilizes the Ilizarov technique in atrophic-type CPT.     

不同类型先天性心脏病并发二尖瓣反流手术效果随访分析

目的:随访观察不同类型先天性心脏病(先心病)合并二尖瓣反流(MR)患儿术后MR的转归。方法:回顾性分析MR术后病例229例(166例行二尖瓣成形术、63例二尖瓣未处理),比较不同类型先心病合并MR患儿行心脏畸形矫正后,二尖瓣处理组与未处理组术后早期、远期MR的转归情况。结果:144例室间隔缺损(VSD)修补同时二尖瓣成形组与29例仅行VSD修补二尖瓣未处理组患儿相比,术后早期和远期MR改善率前者均高于后者;40例动脉导管未闭(PDA)合并MR组与16例主动脉缩窄(CoA)合并MR组,术后早期二尖瓣成形组MR改善率均高于未处理组(P均0.05),而术后远期二尖瓣成形组与未处理组MR改善率均无统计学差异(P0.05)。结论:不同类型先心病合并MR可能需要采取不同的处理方案。VSD合并MR者,建议在修补缺损同时对较重度MR行二尖瓣成形术;而PDA或CoA合并MR患儿可先矫正心脏畸形,再根据患儿远期预后,决定是否行二尖瓣成形术。     

Early Pleural Effusions Related to the Myocardial Injury after Open-heart Surgery for Congenital Heart Disease

Objectives. The degree of effusion immediately after cardiopulmonary bypass (CPB) can vary and may reflect several factors including the degree of myocardial injury. We compared the degree of pleural effusions after CPB to the overall myocardial injury as determined by serum cardiac troponin I (cTnI) levels after elective repair of a variety of congenital heart defects, including univentricular surgeries via cavopulmonary shunts. Methods. Serum was collected pre-CPB, post-CPB, and daily after that and cTnI level measured. The postoperative pleural effusion was measured each day until the chest tube was removed. Results. The 21 study patients were of average age of 5.5 years (±5.6). The duration of chest-tube drainage after open-heart surgery was 4.3 days (±3.5) and the amount was 2.4 mL/kg/hour (±2.9). For the biventricular repairs, cTnI levels on the postoperative day (POD) 1 best correlated with amount of effusion (n = 16, r = 0.5, P = 0.02) and the average (POD 0–3) cTnI levels with the total duration (n = 16, r = 0.4, P = 0.01) and also the amount (n = 16, r = 0.5, P = 0.02) of effusions. For the cavopulmonary shunts, the post-CBP cTnI level best correlated with the duration (n = 5, r = 0.8, P = 0.02) and amount (n = 5, r = 0.9, P = 0.02) of effusions. A cTnI level on the first postoperative day ≥15 µg/L was associated with effusions >2 days (sensitivity of 81% and specificity of 80%). Conclusion. We found that higher the cTnI released, especially ≥15 µg/L, longer the duration and greater the amount of early pleural effusions for a variety of congenital heart surgeries including cavopulmonary shunts. A number of factors may lead to excessive pleural effusions and the degree of myocardial injury may be one of them.     

Prevalence of asymptomatic mitral valve malformations

Introduction  Congenital abnormalities of the mitral valve are considered to be very rare cardiac anomalies. In particular, more severe malformations, such as the complete absence of either aortic (anterior) or mural (posterior) mitral leaflet, are usually considered to be incompatible with life. Ebstein-like malformation of the mitral valve is an extremely rare form of mitral valve deformity hitherto unreported in an asymptomatic adult patient. Materials and Methods  The detection of such a malformation prompted us to perform a prospective analysis of 26,484 consecutive comprehensive 2D-echocardiographic examinations, conducted at our tertiary care university hospital between April 2007 and July 2008, with regard to the presence of malformations of the mitral valvular apparatus. Results  In total, we found three cases of hypoplastic or even absent functional mural valve leaflets. All were diagnosed in adult patients who attended our outpatient department and were surprisingly asymptomatic regarding this finding. From our patient cohort, we calculate an actual prevalence of asymptomatic hypoplasia of the mitral valve of 1:8,800. Conclusions  Our findings broaden the spectrum of known mitral valve pathologies. The comparatively high prevalence of this malformation in our preselected patient cohort might indicate that this particular malformation has so far been under-diagnosed. In the context of this observation, both embryological development of the atrioventricular (AV) valves and recent functional insights into mitral valve physiology gained by mitral valve reconstructive surgery are discussed. Electronic supplementary material  The online version of this article (doi:) contains supplementary material, which is available to authorized users.     

先天性白内障人工晶状体植入术后近视漂移的分析

目的 分析不同年龄的先天性白内障患者的人工晶状体度数数选择.方法 回顾性分析先天性白内障患儿36例(62只眼),植入折叠IOL,术后随访时间12～36(24.52±7.54)个月.分为三组:A组1.5～4岁,B组4.1～6岁,C组6.1～8岁.结果 近视移动范围为-0.19～-3.25D,A组平均漂移-1.03D/年,B组平均漂移-0.65D/年,C组平均漂移-0.35D/年.3组屈光度数变化率(D/年)比较,差异有统计学意义(F=41.430,P=0.000),三组之间两两比较,差异也具有统计学意义(P=0.000).结论 先天性白内障植入IOL后最好保留轻中度的远视,补偿发育带来的近视趋势,年龄越大,欠矫的度数应越少.     

SPECT全身显像在131I治疗分化型甲状腺癌中的应用

目的：探讨SPECT显像对^131I治疗分化型甲状腺癌的价值。方法：对63例甲状腺癌患者术后及^131I治疗后分别进行SPECT显像。结果：63例患者术后SPECT扫描全部显示残留甲状腺。结论：甲状腺癌患者术后要辅以^131I治疗，且需SPECT显像进行监测。