Ocular findings in familial adenomatous polyposis

Background: Retinal pigment epithelium (RPE)lesions are predictive congenital phenotypic markersfor familial adenomatous polyposis (FAP). Thisprospective screening study aims at assessing theincidence and significance of these lesions in FAPpatients and their family members.Methods: Sixty-two members from three familiesincluding five patients with the diagnosis of FAP havebeen ophthalmologically surveyed. All RPE lesions weredocumented with fundus photography and fluoresceinangiography was performed in 13 subjects.Sigmoidoscopy and/or radiological examination wereperformed annually in 9 family members with typicalRPE lesions during 4 years to allow early diagnosis ofFAP.Results: Typical RPE lesions were present infive FAP patients and 15 family members.Telangiectatic dilatations in the retinal peripherywith small dot-like hemorrhages were detected in 6subjects from 3 families These lesions wereparticularly evident on fluorescein angiography.Annual colon analysis showed polyps in 3 out of 9subjects who were positive for RPE lesions.Conclusion: RPE lesions are valuable as aclinical marker in predicting FAP. The co-existingperipheral vascular alterations which have not beenreported before, are probably related to FAP.     

干下漏斗部室间隔缺损的外科治疗

４６例干下漏斗部室间隔缺损，占同期室缺手术治疗的２３．２％（４６／１９８）。缺损位于肺动脉瓣下３４例，漏斗部１２例。用带垫片褥式缝合修补２１例，补片修补２５例。全组无死亡。术后直接缝合组有主动脉关闭不全２例、残余分流２例，残留杂音３例。笔者认为，适时手术，选择适当的进路、应用补片修补、正确判断和处理主动脉瓣病变及对合并肺动脉狭窄者予以流出道补片加宽，是提高本病手术疗效的关键。     

Blood pool ventriculography with a new technetium-labelled complex (99mTc-DEPIC) : a clinical evaluation

A new, single bolus method of in vivo blood pool imaging using a technetium Tc99m phosphine isocyanide complex (DEPIC) which binds to pre-albumin was evaluated in volunteers (n=4) and patients (n=20). DEPIC was assessed for its safety and possible drug interactions. Its duration of action and quality of ventriculography were compared with imaging using standard in vivo red cell labelling (PYP) during two 3-h scanning periods 1 week apart. DEPIC had a mean plasma halflife of 3.3 h. The count rate over the left ventricle was initially 42% higher with DEPIC than with PYP. However, removal of DEPIC by the liver resulted in equivalent count rates by 1 h, and by 3 h PYP count rates were 22% higher than DEPIC. Immediately post injection mean (SD) difference in the left ventricular ejection fraction between the two methods was 2.4% (7.7%). Satisfactory DEPIC scans were obtained up to 2 h post injection, but by 3 h there was a mean difference of 13% (11.3%). DEPIC was found to be a safe alternative to red all labelling for blood pool angiography, suitable for routine work. The single bolus methodology and high initial count rates offer improved efficiency and a capability for truly emergency scanning.     

心间隔缺损并哑性动脉导管未闭4例

我院治疗６６例心间隔缺损的病例中，有４例合并哑性动脉导管未闭（简称ＰＤＡ），发生率６％。全组病例术前均无ＰＤＡ临床体征，１例术前再次复查心脏彩超时发现，其余３例均漏诊。１例先处理ＰＤＡ，３例在纠正心内畸形同时处理ＰＤＡ。心间隔缺损合并哑性ＰＤＡ容易漏诊，但有其临床特点，手术可先处理ＰＤＡ，或在体外循环下处理ＰＤＡ。     

非瓣膜病房颤患者左心耳血流动力学的TEE研究

应用多平面经食管超声心动图技术（Ｍ－ＴＥＥ），对３０例健康人和３０例非瓣病房颤患者（ＮＶＡＦ）心底短轴切面测量了左心耳最大直径，同一心动周期内左心耳最大及最小面积及左心耳最大空速度，产将ＮＶＡＦ患者按照自发显影及血栓的有无分为２组。发现ＮＶＡＦ组左心耳增大，收缩力及血流速度均减低，且这种变化在有血栓和有血栓形成倾向的患者中更为明显。     

1例安置埋藏式自动复律除颤器病人的随访

埋藏式自动复律除颤器(AICD)能降低恶性快速室性心律失常患者的猝死发生率,已经得到基本肯定,但其猝死率的降低,在相当程度上取决于能否对置入AICD患者进行认真随访,不断完善其治疗。本文报告1例随访16个月经验,并就随访结果,结合文献,略予讨论。     

Conversion of Ouabain-Induced Ventricular Tachycardia in Dogs with Epicardial Lidocaine: Pharmacodynamics and Functional Effects

Epicardial antiarrhythmic drug administration was studied as a therapeutic approach for experimental ventricular tachycardia (VT) in an open-chest dog model. Lidocaine-polyurethane matrices (28%, w/w) were formulated as a model system. Matrices were placed on the left ventricular epicardium in each of 23 anesthetized open-chest dogs with ouabain-induced VT, to evaluate effectiveness in restoring sinus rhythm. Conversion occurred in all animals treated with matrices containing 300 mg or more of lidocaine after 1.5 to 7.0 min. The matrix lidocaine content correlated linearly with the time required for conversion to sinus rhythm (r = 0.75, P = 0.0002); irrespective of matrix size the myocardial/plasma lidocaine ratio was 20.1 ± 4.2 (mean ± SD) at the time of conversion. In a separate series of five dogs without ventricular tachycardia, systolic wall thickening measured with sonomicrometers after 5 min of controlled-release lidocaine administration (500- to 1000-mg matrix lidocaine content, 7.48 ± 3.49-mg/kg dose) was only minimally diminished (–14.1%) and this effect was observed only at the site of matrix placement on the anterior-apical epicardium. In contrast, intracoronary injection of 0.3 or 1.0 mg/kg of lidocaine-HCl resulted in complete elimination of wall thickening or replacement by systolic thinning. Thus epicardial administration of lidocaine from polyurethane matrices was an effective means of treating ouabain-induced ventricular tachycardia. Regional myocardial function in the vicinity of the matrices was modified to a very limited degree, supporting the view that the matrices can be used safely, without serious risk to ventricular contractile performance.     

慢性肺心病左心功能探讨

通过42例重型肺心病、7例轻型肺心病、16例肺心病伴发冠心病患者和30例正常人在静息状态下的左右心脏射血分数及其临床资料的分析表明,重型肺心病患者在病情的急性发作期,存在有不同程度的左心功能不全,而轻型肺心病患者则无左心功能不全的表现。表明慢性肺心病仅在病程晚期,才由以右心为主的疾病发展成为全心病。本文还对重型肺心病与肺心病伴发冠心病患者的左心功能进行比较,结果表明,肺心病伴发冠心病患者左室功能受损程度更为明显,双重病因导致的左室功能减退,常造成心肌更严重的损害而且不容易恢复。     

术前左室射血分数及短缩分数对冠状动脉旁路术后恶性心律失常的预测价值

为了评价冠状动脉旁路术 (CABG)术前左室射血分数 (LVEF)及左室短缩分数 (LVFS)对术后室性心律失常 (VA)预测的准确性 ,采用术前及术后 2周心脏彩超EF、FS值 (面积长轴法 )、心室晚电位 (VLP)、心肌酶、持续心电监测的方法 ,对我院 1 5 0例行CABG术的患者进行分析。结果 :1 )术前心肌梗死 (MI)、室壁瘤、VA及VLP阳性患者EF、FS值明显减低 ;2 )术前左心功能不全 (LVD)患者术后EF、FS值明显改善 ;3 )术前LVD、VA、VLP阳性及室壁瘤患者术后VA发生率明显高于其他患者。提示 :1 )面积长轴法EF、FS值是反映左心功能的敏感指标 ;2 )术前LVD患者术后短期左心功能明显好转 ,获益最大 ;3 )非LVD患者术后因心肌顿抑导致近期心功能暂时下降 ;4 )EF≤ 4 0 %和(或 )FS≤ 2 4 %是预测术后VA的独立指标 ,FS较EF更能准确地反映心脏收缩功能 ;5 )LVD、VLP、室壁瘤等综合指标分析有助于提高对术后VA预期的敏感性、特异性和准确性