多普勒超声对心内膜弹力纤维增生症左心室收缩和舒张功能的评价

本研究采用多普勒超声心动图检测技术对35例心内膜弹力纤维增生症患儿的左心室收缩和舒张功能进行了观察，结果显示心内膜弹力纤维增生症可出现心脏收缩和舒张功能的降低，表现为CI、EF、FS、E、E／A、1／3FF、1／2FF指数的降低和IRT、A增加，并且与正常对照组比较，P＜0．05，同时观察到治疗后收缩功能明显改善而舒张功能有所好转但与治疗前无显著性差异，所以我们认为心内膜弹力纤维增生症治疗时要注意收缩与舒张功能同时纠正。     

The feasibility and usefulness of contrast exercise echocardiography for the assessment of left ventricular function in master athletes.

BACKGROUND: The number of competitive master athletes (MA, over 40 years) has been rising. Since the incidence of coronary artery disease (CAD) is increasing in this population, cardiovascular pre-participation screening, including a maximum exercise test, is recommended. In this context the addition of contrast to echo could be useful because wall thickening and motion are better markers of myocardial function when the whole endocardial border (EB) is visible. OBJECTIVE: To evaluate the feasibility and usefulness of rest and exercise contrast echo for the assessment of LV wall motion in competitive master athletes with suboptimal acoustic windows. METHODS: Forty consecutive MA underwent echo and contrast echo both at rest and during exercise. Contrast-enhanced images were achieved at rest and at peak exercise after administration of SonoVue (BR1), using apical 4 and 2-chamber views divided into 6 myocardial segments (MS). The EB resolution for each segment was graded as: 0=not visible, 1=barely visible, 2=well-delineated. RESULTS: In the baseline at-rest echo 17/40 (45%) patients were graded as score 0, 22/40 (55%) were graded as score 1 and only 1 athlete has reached score 2. In at-rest echo contrast 28/40 (70%) athletes have reached score 1 and 12/40 (30%) score 2. Nobody was graded as score 0. At the same time in the baseline peak-exercise echo 12/40 (30%) athletes were graded as score 0, 24/40 (60%) athletes reached score 1 and 4/40 (10%) score 2, while using contrast at peak-exercise echo 28/40 (70%) were grated as score 1 and 12/40 (30%) reached score 2. The differences about the grading of the score between the two groups with and without contrasts, at rest and at peak exercise, are statistically significant (p<0.001). Considering the whole of the MS analyzed in the majority of the athletic population studied, we can see that at-rest echo, 64/480 MS (13.3%) were graded as 0, 156/480 MS (32.5%) as 1 and 260/480 MS (54.2%) were graded 2, while in the peak-exercise 96/480 MS (20%) were graded as 0, 235/480 MS (48.9%) as 1 and 209/480 MS (43.5%) were graded 2. On the other hand, using contrast, in at-rest echo, 480/480 MS (100%) were graded as 2 while in the peak-exercise echo 460/480 MS (95.9%) were graded as 2 and 20/480 MS (4.1%) as 1. The percentage of the MS graded as 2 reach almost the whole number of the analyzed MS with a percentage increment in the at-rest and peak-exercise echo of 85% and 120%, respectively. CONCLUSIONS: Our results show that the use of contrast echo improves the visibility of the EB in a way that the two groups of competitive athletes show at rest and after exercise a significant increment of the score 2 compatible with a better visibility of the EB. These results suggest that contrast echo, both at rest and during exercise, is a better method for EB analysis to understand the behaviour of the wall motion in subjects with suboptimal acoustic windows, and could be suitable for cardiovascular screening in master athletes.     

Cardiac fulguration, a new alternative therapy for atrioventricular nodal reentrant tachycardia.

Ten patients underwent endocardial catheter ablation of the atrioventricular junction for atrioventricular nodal reentrant tachycardias. Unipolar cathodic discharges at the distal electrode were administered against an external plate. Bipolar His and atrial deflections showed a mean of 0.15 mv and 0.5 mv respectively. Mean total energy used per patient was 195 J (range: 50-750), with a mean number of ablating discharges of 2.0 per patient, (range: 1-5). Complete atrioventricular block was achieved, but conduction reappeared in all except one patient, after a mean interval of 19.9 min. Electrophysiological evaluation was assessed 3-8 days after ablation. Sustained atrioventricular nodal reentrant tachycardias were no longer inducible in any patient. Retrograde conduction was abolished in six, and was slow and decremental in four. First-degree atrioventricular block, with intranodal delay was diagnosed in six, with an AH interval that ranged from 240 to 130 ms. Mean cycle length for appearance of Wenckebach atrioventricular block was 390 ms after ablation. One patient developed complete atrioventricular block after two discharges of 50 J, another required a repeat ablation for recurrence of intranodal tachycardia and also developed complete anterograde block in a new session of ablation with a 150 J discharge. In these two patients permanent pacing was needed. Eight patients were cured after a mean follow-up of 20 months. Less energy and fewer discharges should be administered to abolish functional dissociation of the atrioventricular node, without complete interruption of anterograde conduction.     

Right diaphragmatic paralysis following endocardial cryothermal ablation of inappropriate sinus tachycardia.

Inappropriate sinus tachycardia (IST) is a rare disorder amenable to catheter ablation when refractory to medical therapy. Radiofrequency (RF) catheter modification/ablation of the sinus node (SN) is the usual approach, although it can be complicated by right phrenic nerve paralysis. We describe a patient with IST, who had symptomatic recurrences despite previous acutely successful RF SN modifications, including the use of electroanatomical mapping/navigation system. We decided to try transvenous cryothermal modification of the SN. We used 2 min applications at -85 degrees C at sites of the earliest atrial activation guided by activation mapping during isoprenaline infusion. Every application was preceded by high output stimulation to reveal phrenic nerve proximity. During the last application, heart rate slowly and persistently fell below 85 bpm despite isoprenaline infusion, but right diaphragmatic paralysis developed. At 6 months follow-up, the patient was asymptomatic and the diaphragmatic paralysis had partially resolved. This is the first report, we believe, of successful SN modification for IST by endocardial cryoablation, although this case also demonstrates the considerable risk of right phrenic nerve paralysis even with this ablation energy.     

Limited efficacy of nintedanib for idiopathic pleuroparenchymal fibroelastosis

BackgroundThe antifibrotic agent nintedanib has been reported to effectively prevent the decline in forced vital capacity (FVC) in a broad range of interstitial lung diseases. However, the efficacy of nintedanib against idiopathic pleuroparenchymal fibroelastosis (iPPFE) remains unclear.MethodsWe retrospectively examined patients with idiopathic PPFE or idiopathic pulmonary fibrosis (IPF) who received nintedanib for more than 6 months. We evaluated annual changes in %FVC, radiological PPFE lesions, and body weight before and during nintedanib treatment. To investigate radiological PPFE lesions, we examined the fibrosis score, which was defined as the mean percentage of the high attenuation area in the whole lung parenchyma using three axial computed tomography images.ResultsOverall, 15 patients with iPPFE and 27 patients with IPF were included in the present study. In patients with IPF, the annual rate of decline in %FVC was significantly lower during nintedanib treatment than that before treatment (?2.01%/year [?7.64 to 3.21] versus ?7.64%/year [?10.8 to ?4.44], p = 0.031). Meanwhile, in patients with iPPFE, the annual rate of decline in %FVC during nintedanib treatment was higher than that before treatment (?18.0%/year [?21.6 to ?12.7] versus ?9.40%/year [?12.3 to ?8.23], p = 0.109). In addition, nintedanib treatment failed to inhibit the annual rate of increase in fibrosis score in patients with iPPFE (6.53/year [1.18–15.3] during treatment versus 2.70/year [0.27–12.2] before treatment, p = 0.175).ConclusionsNintedanib efficacy may be limited in patients with iPPFE.     

Prenatal sonographic monitoring of progressive cardiac damages caused by anti‐Ro antibodies: A case report

Maternal autoantibodies to Ro/SSA are often linked to congenital heart block and rarely associated with structural defects. We describe the case of a fetus with anti‐Ro‐mediated second‐degree block at 19 weeks, which progressed to a complete block, fibroelastosis, atrioventricular valve insufficiency, and semilunar valve stenosis/insufficiency at 20, 22, 24, and 26 weeks, respectively, although the fetus received transplacental anti‐arrhythmic drugs. The 2150‐g fetus was vaginally delivered at 35 weeks. An external pacemaker was inserted immediately after birth and replaced with permanent pacemaker at the age of 3 months. The newborn has had a good outcome with well‐controlled heart rate.     

合并双孔二尖瓣的心内膜垫缺损外科手术28例

目的 回顾分析28例合并双孔二尖瓣的心内膜垫缺损病儿外科手术效果.方法 1996年10月至2007年11月共860例心内膜垫缺损病儿行矫治手术,28例合并双孔二尖瓣畸形(3.26%),其中完全型心内膜垫缺损11例(组Ⅰ),部分型心内膜垫缺损17例(组Ⅱ).将两组病儿术前、术后有关资料进行对比,包括二尖瓣关闭不全程度以及瓣膜外科处理方法等,并对外科疗效进行分析.结果 术后早期死亡4例,均为组Ⅰ病儿,死于术后严重肺部感染3例,术后低心排1例.2例病儿通过二次手术或尸检证实有二尖瓣严重关闭不全或狭窄.随访过程无中、远期死亡.两组二尖瓣瓣膜外科处理方法无差异,随访3～89个月,平均33个月,组Ⅱ术后远期随访二尖瓣关闭不全程度较组Ⅰ严重,但差异无统计学意义.结论 双孔二尖瓣的存在增加完全型心内膜垫缺损手术风险,影响部分型心内膜垫缺损远期疗效.     

心内膜弹力纤维增生症合并肺炎患儿的护理

目的：总结35例心内膜弹力纤维增生症合并肺炎患儿的护理特点,以利于提高患儿的存活率。方法：分析我科35例心内膜弹力纤维增生症合并肺炎患儿行控制心力衰竭,维持心脏功能,改善通气功能,保证营养供给,防止感染,作好药物治疗的护理和心理护理。结果：35例患儿经积极治疗和精心护理,心力衰竭得到有效控制,病情明显好转出院。结论：积极控制心力衰竭,改善通气功能,严密观察病情（护理）和出院指导是心内膜弹力纤维增生症合并肺炎患儿缩短住院日数,取得较好疗效的保证。     

原发性心内膜弹力纤维增生症75例远期疗效

目的 总结分析心内膜弹力纤维增生症(EFE)患儿治疗后的临床效果及转归,分析不同治疗方法在改善预后中的作用.方法 回顾性分析1984年8月至2006年6月曾在首都医科大学附属北京安贞医院小儿脏科住院的75例特发性婴幼儿EFE临床及随访资料.结果 本组男40例,女35例,发病年龄20 d～2岁8个月,出院后继续门诊规律治疗及随访69例,随访率92%,门诊随访时间6个月～23年(平均5.7年).随访过程中6例(8.7%)死亡.治愈率46.4%(32/69),好转率40.6%(28/69),总治愈好转率87.0%.全组治疗2年左室射血分数(EF)(55.86%±2.85%)恢复正常,治疗后1、3、5、10年左室EF正常率分别为42.6%(26/61)、64.4%(29/45)、70.7%(29/41)和84.6%(22/26).治疗3年胸部X线心胸比例(C/T)(0.50±0.01)恢复正常.治疗3年左室舒张末径(LVDD)平均值未恢复正常,治疗后1、3、5、10年正常率分别为0%(0/61)、13.3%(6/45)、53.7%(22/41)和84.6%(22/26).EFE患儿首诊评分<22分37例(糖皮质激素组),治疗1年左室EF平均值恢复正常(EF值58.44%±5.10%),治疗2年胸部X线C/T平均值恢复正常(0.50±0.00).EFE患儿首诊评分≥22分29例(糖皮质激素+环磷酰胺组),治疗3年左室EF平均值恢复正常(57.33%±3.43%),治疗3年C/T、LVDD平均值未恢复正常.加用IVIG使应用环磷酰胺治疗病例的百分比下降,选择年龄相当左室EF≤40%的IVIG组21例及非IVIG组19例,临床需加用环磷酰胺的病例分别为7例(33.3%)及11例(57.9%).EFE心内膜厚度恢复正常较慢,平均4年(1～8年).治疗后1、3、5、8年心内膜恢复正常率分别为9.8%(6/61)、22.2%(10/45)、51.2%(21/41)及100%(29/29).结论 对EFE患儿长期规范不间断治疗至痊愈的远期效果良好.重症及难治性EFE需加强免疫治疗,维持治疗时间亦较长.临床治愈的患儿停药后仍应限制活动量并定时复查,及时发现心功能的变化并给予及时治疗可改善远期效果.