Clinical study of double-chamber stimulation. Apropos of 50 cases followed-up for 1 to 5 years

The authors report their experience of dual chamber pacing in 29 men and 21 women of mean age 71 +/- 4 years. 35 had sinus node dysfunction associated with node-His bundle conduction disorders; 31 presented with neurological symptoms and 4 with heart failure (due to pacemaker syndrome in 1 case). Sinus node dysfunction was diagnosed by surface ECG in 25 cases and after electrophysiological studied in only 10 cases. Fifteen patients had atrioventricular block without sinus node dysfunction: 2 of them were young subjects, 1 had pacemaker syndrome and 12 were actual or potential heart failure patients for whom preservation of the atrial systole was justified. Nine patients presented with neurological symptoms. 43 (86%) had cardiac or arterial disease associated with cardiac rhythm and conduction disorders. The percutaneous single subclavian vein approach was used in 36 cases (78%). 41 active and 9 passive fixation electrodes were utilized. The mean follow-up period was 25 months (12 to 70 months), with a cumulative figure of 1,253 months/patients. Two late re-operations for displacement of the atrial electrode were performed. Dual chamber pacing was abandoned, 14 months on average after implantation, in 9 patients (18%), on account of arrhythmias in 4 of them. Three cases of tachycardia from "electronic re-entry" and 6 cases of supraventricular arrhythmia transferred to the ventricle by the pacemaker were observed. Sixteen patients (32%) died 12 +/- 4 months on average after surgery: 12 (33%) had sinus node dysfunction and 4 (26%) had AV block. Death was caused by a cardiovascular disease in 12 cases.(ABSTRACT TRUNCATED AT 250 WORDS)     

Lambert-Eaton syndrome. Apropos of 2 cases]

Lambert-Eaton syndrome is a myasthenia-like syndrome of paraneoplastic origin which is often associated with anaplastic small-cell lung cancer. It seems to be an autoimmune disease responsible for a deficit of acetylcholine ejection in the motor end plate. On the occasion of two recent cases, we review the clinical, physiopathological and diagnostic aspects of this paraneoplastic syndrome.     

Outcome of prognostic factors of infectious endocarditis over a 16 year period. Apropos of 471 cases]

Four hundred and seventy one cases of infective endocarditis (IE) were reviewed: 338 native valve IE and 133 prosthetic valve IE (42 early and 91 late IE). Two periods were compared: 1973-1980 (250 cases) and 1981-1988 (221 cases). There was a decrease in native valve IE (78% to 64%) and an increase in late prosthetic valve IE (13% to 27%), little change with respect to age, causal cardiac disease, delay in diagnosis (except in native valve IE, 39 to 29 days), or frequency of complications, especially cardiac (50% and 51%). However, global mortality decreased from 41% to 27% (p < 0.001). The evolution of the frequency of cardiac complications, cardiac surgery and mortality for the two periods was: for native valve IE respectively 53% to 42%, 41% to 37%, 37% to 20% (p < 0.005); for early prosthetic valve IE respectively, 45% to 55%, 41% to 55%, and 82% to 50% (p < 0.05); for late prosthetic IE, respectively 34% to 69%, 34% to 69% and 37% to 36%. The frequency of surgery had therefore little influence on prognosis except in early prosthetic valve IE. The percentage of infections which could not be controlled medically decreased from 17% to 11%. The mortality of unoperated patients decreased from 46% to 28% (p < 0.01), suggesting more effective antibiotherapy, and the mortality of operated patients fell from 34% to 26%. Global surgical mortality was 35% in the acute phase (positive valve culture), 14% after sterilisation (p < 0.001) and the corresponding frequencies of paravalvular leaks was 17% and 4% (p < 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)     

Carvedilol therapy for patients with Eisenmenger syndrome

Background: Eisenmenger syndrome is characterized by the development of pulmonary arterial hypertension with resultant intracardiac right-to-left shunt and hypoxemia in patients with congenital heart defects. In clinical practice, we found that these patients might benefit from carvedilol therapy. Thus, we designed this prospective, open label, observational study to evaluate the efficacy of carvedilol for patients with Eisenmenger syndrome. Methods Twenty patients(17.5 ± 4.8 years) with Eisenmenger syndrome were treated with carvedilol for 6 weeks. The efficacy of carvedilol on 6-minute walking distance, World Health Organization(WHO) functional class, arterial oxygen saturation and right ventricle systolic pressure were assessed.Results At the end of observation, change from baseline in 6-minute walking distance increased 36.35 meters(95% confidence intervals [CI] 25.43 to 47.27 m, P 0.01). WHO functional class was also significantly improved(P 0.05). Change from baseline in right ventricular systolic pressure assessed by echo was reduced by 8.11 mm Hg(95% confidence intervals [CI],-10.78 to-5.44 mm Hg, P 0.05). However, arterial oxygen saturation remained unchanged(87.5 ± 3.02 % at baseline versus 87.80 ± 7.29 % at the end of observation). Conclusions Carvedilol can improve clinical function, symptoms as well as exercise capacity in patients with Eisenmenger syndrome. These findings need to be confirmed in further randomized clinical trials.     

Budd-Chiari syndrome treated successfully by percutaneous transluminal balloon angioplasty: two cases followed-up for 6 years.

We report 2 cases of Budd-Chiari syndrome that are due to complete membranous obstruction between the junction of the inferior vena cava and the right atrium. Both cases were treated successfully by King's bioptome breakthrough followed by balloon dilatation of the membrane. The patients showed remarkable symptomatic improvement and promising hemodynamic and angiographic results immediately after the procedure and 6 years later. We conclude that balloon angioplasty is a safe and effective method for treating this rare disease in selected cases.     

Endobronchial lipomas. Apropos of 16 cases

Intrabronchial lipomas are rare and benign tumours. Sixteen cases treated between 1956 and 1988 at the Marie Lannelongue Surgical Centre, Paris, are reviewed. The patients presented with various symptoms such as cough, haemoptysis, infection or dyspnoea. Radiography was abnormal in 15 cases, and the lesion was regularly located at bronchoscopy. Treatment was chiefly surgical. Owing to the slow and silent growth of intrabronchial lipomas, there is a risk of progressive bronchial obstruction resulting in bronchopulmonary lesions. With an early detection, treatment should be as conservative as possible.     

腹腔镜袖状胃切除术治疗代谢综合征50例疗效分析(两年随访)

目的探讨腹腔镜袖状胃切除术（LSG）治疗代谢综合征的安全性和有效性。 方法回顾性分析2013年2月至2017年6月在解放军第一五三中心医院接受腹腔镜袖状胃切除手术的50例患者临床资料,记录并比较术前及术后1个月、3个月、6个月、1年、2年的体质量、体质量指数（BMI）、平均动脉压、血清甘油三脂、高密度脂蛋白、空腹血糖(FPG)、糖化血红蛋白(HbA1c)及糖尿病、高血压、高脂血症、睡眠呼吸暂停综合征、脂肪肝、关节退行性变改善情况。 结果50例患者均顺利完成LSG,手术时间(70.7±26.3) min,术后住院时间(7.0±1.7) d。所有患者术后随访时间均超过2年,无围手术期死亡及术后严重并发症发生,体质量、BMI在术后3个月可观察到明显下降（P<0.05）;24例合并高血压,平均动脉压在术后1个月出现明显下降然后保持相对稳定（P<0.05）,术后两年内高血压完全缓解率63%;35例合并高甘油三脂血症,血清甘油三酯在术后6个月出现明显下降（P<0.05）,而高密度脂蛋白在术后1年出现有统计学意义的升高（P<0.05）;32例合并糖尿病,FPG在术后1个月出现明显下降（P<0.05）,HbA1c同样在术后1个月出现明显下降（P<0.05）,其中12例完全治愈而且不需进行药物治疗,治愈率为37.5%。术后2年内患者脂肪肝、睡眠呼吸暂停及关节退行性变也得到不同程度的改善。 结论LSG治疗肥胖症安全性好、效果显著,在明显减轻体重的同时还能治愈或显著改善其合并的代谢综合征。     

Nocturnal oxygen therapy in patients with the Eisenmenger syndrome.

This prospective and controlled pilot study evaluates the long-term effects of nocturnal oxygen therapy (NOT) on exercise endurance, hematology variables, quality of life, and survival of 23 adult patients (mean age, 32 +/- 6 yr) with post-tricuspid congenital heart defects (ventricular septal defect = 10; patent ductus arteriosus = 13) and Eisenmenger Syndrome. All had pulmonary hypertension (mean pulmonary artery pressure = 88 +/- 20 mm Hg), severe hypoxemia (Pa(O(2)) = 44 +/- 5 mm Hg), and secondary erythrocytosis (hematocrit = 61.5 +/- 7%). Exercise endurance (6-min walk test = 380 +/- 88 m) was limited. In a random fashion, NOT was given to one group of patients (n = 12) but withheld from a comparable control group (n = 11). At 2 yr of close follow-up, two patients in the group of control patients, and three in the treatment group died. Mean survival estimates were similar in both groups (20.7 versus 20.8 mo; chi-square log-rank, 0.08; p = NS). Likewise, none of the hematology, exercise capacity, and quality of life variables examined showed statistically significant changes that were dependent on treatment regimen. We conclude that NOT does not modify the natural history of patients with advanced Eisenmenger Syndrome.     

Bifascicular block: its course and prognostic significance in 52 cases followed-up for 15 years]

The authors report the results of a 15 years follow-up in a group of 52 patients with right bundle branch block and left anterior hemiblock. The incidence of complete AV block and sudden death in this group is compared to the one observed in a suitable control group. Among the bifascicular block group there was only one sudden death and one case of complete AV block. However, the groups did not differ statistically for mortality rates and average survival time. The authors suggest that bifascicular blocks should be regarded as a mere risk factor for complete AV block.     

艾森曼格综合征肺动脉高压靶向治疗效果观察

目的 观察肺动脉高压靶向药物治疗对艾森曼格综合征的临床效果。方法 选择艾森曼格综合征患者36例,年龄9~51（39±18）岁。其中房间隔缺损（ASD）11例、室间隔缺损（VSD）14例、动脉导管未闭（PDA）6例、VSD+PDA 3例、ASD+PDA 2例。应用波生坦或西地那非治疗,根据服药情况分为长期用药组和间断用药组,2年后评估其疗效。结果 长期用药组2年后经皮血氧饱和度(SpO2)、6 min步行距离(6MWTD)较用药前显著升高（P<0.05）,Borg评分、纽约心脏病学会心功能分级(NYHA)、脑钠尿肽(BNP)、肺动脉收缩压(sPAP)较用药前显著减低（P<0.05）。与用药前相比,肺循环血流量 /体循环血流量(Qp/Qs)显著升高（P<0.05）,肺动脉平均压(mPAP)、肺动脉收缩压/体循环收缩压(Pp/Ps)显著减低（P<0.05）; 肺血管阻力(PVR)也有不同程度降低,但差异无统计学意义。间断用药组SpO2、6MWTD较用药前亦显著升高（P<0.05）,BNP较用药前显著减低（P<0.05）;Borg评分、NYHA、sPAP虽有不同程度改善,但差异无统计学意义。长期用药组与间断用药组相比,6MWTD、Borg评分、NYHA、BNP、sPAP等指标均无明显差异。但长期用药组SpO2显著升高（P<0.05）。结论 间断靶向治疗可用于部分艾森曼格综合征患者。     

Proteus syndrome. Expansion of the phenotype. Apropos of 3 pediatric cases]

In 1979 Cohen et Hayden and in 1983 Wiedemann et al. delineated a syndrome consisting of partial gigantism of the hands and/or feet, nevi, hemihypertrophy, subcutaneous tumors, macrocephaly or other skull anomalies and possible accelerated growth and visceral affections. Hitherto the literature pertaining to this syndrome consists of somewhat more than 100 cases of which some, that have been described previously or subsequently under other headings, were rediagnosed as being Proteus syndromes. Of these, more than half show vascular anomalies closely resembling those observed in the Klippel-Trenaunay syndrome, but in the Proteus syndrome appear to be more haphazardly distributed over the integument. We report 3 pediatric patients with the Proteus syndrome, all showing cutaneous angiodysplasias. These patients were initially diagnosed as suffering from "severe or atypical Klippel-Trenaunay syndrome". In one of these, cardiac tumors were observed soon after birth which subsequently showed spontaneous involution and were therefore considered to be rhabdomyomas. In the Proteus syndrome cardiac pathology is rare, and cardiac tumors have not been described previously. Moreover, we observed umbilical hernia in two of our patients, a feature which has hitherto not been reported in patients with the Proteus syndrome. In all our patients a broad thoracic cage resembling a "body-builders chest", asymmetrical and disproportional macrodactyly and broad, flat feet were conspicuous. These broad, flat feet with macrodactyly and large spaces between the first and second digits were designed by the parents of one of our patient as "chimpanzee's feet". Macrodactyly, "chimp's" feet and a broad thoracic cage are considered by us to be clinical hallmarks of the Proteus syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)     

Echocardiographic study of the early repolarization syndrome. Demonstration of dynamic obstruction with isoprenaline. Apropos of 16 cases

This study provides detailed information on the echocardiographic data obtained before and during an intravenous infusion of isoprenaline in a group of 16 patients under 50 years of age presenting with early repolarization syndrome and in a control group of 16 patients of the same age-group. The stereotyped echocardiographic features of early repolarization syndrome are described. They include: in the basal state, ventricular hyperkinesia, anterior motion of the mitral valve and reduction of the left ventricular outflow tract; these data were significant when compared with the control group; under isoprenaline, dynamic obstruction of the left ventricle. The authors discuss the electrical significance of the early repolarization syndrome, the relationship between that syndrome and the borderline forms of obstructive hypertrophic cardiomyopathy and the role of catecholamines in the pathogenesis of obstructive hypertrophic cardiomyopathy.     

The remarkable right ventricle of patients with Eisenmenger syndrome

Patients with Eisenmenger syndrome do much better than patients with all other etiologies of severe pulmonary hypertension. The primary reason, and a major focus of this review, is the right ventricle, a remarkable chamber with preserved systolic function despite a lifetime of systemic level pulmonary hypertension.     

Pulmonary orifice stenosis: the medical solution. Apropos of 62 cases

Sixty two patients with moderate or severe stenosis of the pulmonary orifice (SPO) underwent balloon catheter valvuloplasty. Thirty one were aged under 5 years. The mean right ventricle-pulmonary artery trunk (RV-PAT) gradient fell from 75 (+/- 26) to 23 (+/- 10) mmHg (p less than 0.001). All dilatations except one were effective with only a few incidents and no mortality. Six (+/- 2) months later, hemodynamic evaluation of 45 patients (70%) showed stability of the RV-PAT gradient at 26 (+/- 18) as compared with 23 (+/- 11) mmHg. Ten patients required a second dilatation because of a residual gradient of greater than 35 mmHg. The result was favourable in all ten cases, the RV-PAT gradient falling from 53 (+/- 17) to 13 (+/- 8) mmHg (p less than 0.001). This series confirmed the efficacy, reproducibility and safety of this technique which led to a lowering of ventriculo-pulmonary gradient, persistent at six months, to an infra-surgical level, at the price of pulmonary incompetence (27%) which was always well tolerated in the mid-term. This valvuloplasty may be suggested for all cases of SPO with a gradient of greater than 35 mmHg.