Para-Hisian Pacing for a Pediatric Patient with a Congenitally Corrected Transposition of the Great Arteries (SLL)

We report a pediatric patient with a congenitally corrected transposition of the great arteries (ccTGA)(SLL) in which permanent para-Hisian pacing (PPHP) could improve dyssynchrony-associated systemic ventricular (SV) dysfunction resulting from permanent morphologic left ventricular pacing for complete atrioventricular block. Since, in patients with ccTGA(SLL), an elongated His-bundle runs medially toward the upper septum to the site of the fibrous continuity between the right-sided mitral valve and pulmonary artery, the His-bundle may easily be captured by a pacing lead, unlike in normal hearts. Thus, PPHP may be an effective therapeutic strategy for the treatment of dyssynchrony-associated SV dysfunction associated with ccTGA (SLL). (PACE 2010; e4–e7)     

New Onset Complete Heart Block and Corrected Transposition of Great Arteries in the Seventh Decade

A 65-year-old female was hospitalized for syncope due to new onset complete heart block. An apical holosystolic murmur was detected on physical examination. Echocardiography revealed corrected transposition of great arteries, and Ebstein-like anomaly with regurgitation at the left-sided tricuspid valve. A single chamber (VVI) pacemaker was implanted and patient has been asymptomatic thereafter.     

成人大动脉转位及合并畸形彩色多普勒超声心动图特征及规律性研究

目的 探寻成人大动脉转位(TGA)及合并畸形彩色多普勒超声心动图(CDE)特征及规律性.方法 应用CDE检查50例成人TGA,47例经心血管造影对照,35例经手术证实.结果 根据CDE图像特征对50例成人TGA全部做出正确诊断.成人TGA及合并畸形CDE图像特征及规律性明显:(1)二维超声心动图(2DE)胸骨旁左室长轴切面矫正性TGA室间隔回声显示不清晰,主动脉后壁下方增厚即主动脉下圆锥回声;完全性TGA显示主动脉在前方发自右心室,肺动脉在后方发自左心室.(2)2DE心尖四腔心切面显示矫正性TGA房室连接不一致;完全性TGA房室连接一致.(3)2DE胸骨旁大动脉短轴切面显示2条大动脉呈2个环状回声,称2DE"双环征".根据两环相互位置关系判断转位的类型;根据两环内径比较判断是肺动脉狭窄还是肺动脉高压.(4)成人TGA矫正性(80.0%)明显多于完全性(20.0%).在矫正性中SLL型(82.5%)明显多于IDD型(17.5%);在完全性中SDD型(90.0%)明显多于其他类型.所有成人完全性TGA均合并室间隔缺损.(5)成人TGA合并肺动脉狭窄(84.0%)明显多于肺动脉高压(16.0%).(6)成人矫正性TGA心脏位置异常(62.5%)多见;完全性TGA心脏位置均正常.(7)成人矫正性TGA(55.0%)发绀;完全性TGA均有发绀.结论 成人TGA及合并畸形CDE图像特征及规律性明显,CDE对成人TGA及合并畸形有特异性诊断价值.应用2DE判断房室连接关系是鉴别诊断校正性TGA与完全性TGA的关键.     

小婴儿一期矫治完全性大动脉错位或Taussig-Bing合并主动脉弓畸形的早中期转归

目的：回顾性研究大动脉转换术同时进行主动脉弓矫治的Ⅰ期手术治疗完全性大动脉错位或Taussig-Bing合并主动脉弓畸形的早中期效果。方法：2000年1月至2008年12月,连续对26例存在主动脉弓畸形的完全性大动脉错位或Taussig-Bing畸形的小婴儿进行了Ⅰ期手术矫治,其中完全性大动脉错位13例（TGA/VSD 11例,TGA/IVS 2例）,Taussig-Bing 13例;主动脉弓畸形中主动脉弓中断（A型）7例,CoA19例,6例伴有冠状动脉异常类型。平均手术年龄（28±35）d,〈2个月占62%,手术平均体重为（4.19±1.15）kg。在深低温停循环或深低温低流量下进行主动脉弓畸形矫治,采用自身组织直接吻合扩大或重建弓,伴有弓部发育不良者补片扩大成形。伴有冠状动脉畸形者在大动脉转换手术中冠状动脉移植方法予改良处理。结果：手术住院死亡3例（11.5%）,死因与冠脉移植无关。平均插管时间102 h,监护室时间平均8 d。术后早期生存者主动脉瓣上压力阶差〉30 mmHg有2例,主动脉瓣反流轻度2例。单因素分析中伴有冠状动脉异常类型者与术后早期死亡或并发症的风险相关,多因素分析示其与手术年龄、肺动脉高压、术前FS、主动脉阻断时间、术后血清乳酸水平相关。随访期3个月～7年,无死亡,术后5年实际生存率为88.5%（95%可信度范围CI 76%～96%）,术后1年、5年无需介入干预或手术分别为91.4%、87%。结论：TGA和Taussig-Bing伴有主动脉弓畸形者Ⅰ期进行大动脉转换术和主动脉弓畸形矫治早中期效果良好,早期手术并发症和死亡的风险因素为年龄偏大,肺高压严重,把握手术时机是手术成功要则之一。     

II. Indices of Pain Intensity Derived From Ecological Momentary Assessments and Their Relationships With Patient Functioning: An Individual Patient Data Meta-analysis

Pain intensity is a complex and dynamic experience. A focus on assessing patients’ average pain levels may miss important aspects of pain that impact functioning in daily life. In this second of 3 articles investigating alternative indices of pain intensity derived from Ecological Momentary Assessments (EMA), we examine the indices’ associations with physical and psychosocial functioning. EMA data from 10 studies (2,660 patients) were reanalyzed to construct indices of Average Pain, Maximum Pain, Minimum Pain, Pain Variability, Time in High Pain, Time in Low Pain, Pain after Wake-up. Three sets of individual patient data meta-analyses examined 1) the test-retest reliability of the pain indices, 2) their convergent validity in relation to physical functioning, fatigue, depression, mental health, and social functioning, and 3) the incremental validity of alternative indices above Average Pain. Reliabilities approaching or exceeding a level of .7 were observed for all indices, and most correlated significantly with all functioning domains, with small to medium effect sizes. Controlling for Average Pain, Maximum Pain and Pain Variability uniquely predicted all functioning measures, and Time in High Pain predicted physical and social functioning. We suggest that alternative pain indices can provide new perspectives for understanding functioning in chronic pain.PerspectiveAlternative summary measures of pain intensity derived from EMA have the potential to help better understand patients’ pain experience. Utilizing EMA for the assessment of Maximum Pain, Pain Variability, and Time in High Pain may provide an enhanced window into the relationships between pain and patients’ physical and psychosocial functioning.     

P49First UK Report of a Case of Glycoprotein VI Deficiency in a 33-Year-Old Patient with Moderate Thrombocytopenia and Autoantibodies Against the Immunoglobulin-Like Domains of the Receptor

Background  Studies of cases of acquired and inherited GPVI deficiency have made critical contributions to the understanding of the pivotal role of GPVI in collagen mediated platelet signaling.
Aim  To characterize the first UK case of GPVI deficiency.
Methods  Numerous platelet function tests and immunophenotyping assays, western blotting and genomic sequencing.
Results  A female patient of 33 years with mild thrombocytopenia (80–120 × 10⁹ L⁻¹), had experienced moderate bleeding with atypical locations, including bruises and petechiae on neck and chest, and a perineal haematoma and postnatal bleeding. Bone marrow examination revealed plentiful megakaryocytes, compatible with a state of compensated thrombocytolysis. PFA 100 response with the collagen/epinephrine cartridge was >295 seconds, and aggregometry showed a profoundly reduced response to collagen and collagen-related peptide. Response to ADP and PAR1 peptide was moderately reduced, but ristocetin induced agglutination was normal. By flow cytometry, normal surface expression was observed to many platelet receptors, including GPIIbIIIa, GPIaIIa and PECAM-1. Expression of PAR-1 was mildly reduced, whereas for GPVI a 90% reduction in surface expression was observed. Total GPVI levels as measured by blot densitometry were also reduced, although not as profoundly. The sequence of the GP6 exons was identical to the reference sequence. A potent GPVI autoantibody was detected in the patients' plasma by a sandwich ELISA (MAIPA), and reactivity was mapped to the collagen-binding domains using recombinant GPVI fragments.
Conclusions  The patient has GPVI autoantibodies of the IgG class, resulting in an acquired GPVI deficiency and with a bleeding phenotype apparently more severe than other reported cases of GPVI deficiency.     

Self-Medication With Over-the-Counter Analgesics: A Survey of Patient Characteristics and Concerns About Pain Medication

Pain is a common reason for self-medication with over-the-counter (OTC) analgesics. However, this self-treating population has remained largely uncharacterized. This cross-sectional observational study investigated individuals who self-medicate their pain with OTC analgesics to elucidate their pain characteristics and medication use. In addition, presence of and risk factors for concerns about pain medication were examined. The clinical profile of the participants (n?=?1,889) was worse than expected with long-standing pain complaints (median pain duration of 9 years), pain located at multiple body sites (median of 4, and 13% with ≥10 painful body areas), about one-third suffering from daily pain and about 40% experiencing substantial pain-related disability. Head (58.6% of sample), low back (43.6%), and neck (30.7%) were the most common pain locations. About 73% had a physician diagnosis, mainly migraine and osteoarthritis. Paracetamol (used by 68.6% of patients) and nonsteroidal anti-inflammatory drugs (46.8%) were the most frequently used pain medications. About 40% of our sample showed substantial concern about the perceived need for pain medication and the perceived potential for harmful effects (eg, fear for addiction). These findings highlight the importance for health professionals to systematically probe pain patients about their self-medication practices and explore attitudes about pain medication.Perspective: This study found that the clinical picture of people who self-medicate their pain with OTC analgesics looked worse than expected. We also identified substantial concerns about pain medication. Therefore, we recommend that health professionals systematically probe pain patients about their self-medication practices and explore concerns about pain medication.     

Use of Personalized Decision Analysis in Decision Making for Palliative vs. Surgical Management of the Oldest-Old Patients With Localized Skin Cancer in a Culturally Sensitive Environment: A Case Study of a 96-Year-Old Male Taiwanese Patient

Clinical management of older cancer patients is challenging, especially for those in the oldest-old age group. In the Chinese culture, the preference toward noninvasive care for patients in this age group may create a difficult situation for clinicians when the cancer is potentially curable. Palliative care may not always be the obvious choice, especially if patients suffer from quality-of-life impairment because of symptoms related to progression of the untreated cancer. Balancing between higher rates of toxicities and potential gains in quality of life from anticancer therapies among the oldest old presents a real challenge in clinical practice. Decision analysis is an analytical tool that has a long history of successful application in clinical decision making. In this case study of a 96-year-old male with localized skin cancer, we demonstrate that personalized decision analysis can be a helpful tool to assist decision making in the clinical management of cancer patients in the oldest-old age group, especially in situations in which the evidence-based literature provides little guidance. This was achieved by providing information to help the key decision makers to better understand the risk-benefit trade offs and make an informed decision. We found that the option of surgical management was associated with higher expected quality-adjusted life years (0.894) than best supportive care (0.853). Based on this finding and various scenarios explored in sensitivity analyses, the family members recommended surgery for the patient. The patient recovered well from surgery and there is no evidence of recurrence to date.