法洛四联症术后右室限制性生理临床转归的研究

目的 研究法洛四联症(tetralogy of Fallot,TOF)术后右室限制性生理临床转归,探讨TOF术后右室限制性生理发生的危险因素、评估指标和转归情况.方法 2011年10月至2012年5月在上海儿童医学中心行TOF根治术的80例患儿随机入选本次研究,通过床边心脏彩超明确30例患儿发生了右室限制性生理.统计患儿围手术期的临床资料,记录患儿最后一次超声随访的结果,比较患儿的右室流出道梗阻情况、肺主动脉梗阻情况、肺动脉分支狭窄情况和肺动脉反流情况.结果 右室限制性生理组患儿比非右室限制性生理组的年龄更小,体外循环时间、主动脉阻断时间、机械通气时间、重症监护时间和住院时间更长(P均<0.01).Logistic多元回归分析表明,术前年龄小(OR=0.48,P=0.01)、术中体外循环时间长(OR=1.7,P=0.04)是患儿发生右室限制性生理的危险因素.术后1周内,右室限制性生理组静脉血氧饱和度的上升和氧摄取分数的下降程度均小于非右室限制性生理组(P均<0.05).右室限制性生理组乳酸水平高于非右室限制性生理组(P=0.03).C-反应蛋白在术后2d内先上升,3~7d内下降,且右室限制性生理组下降的速度要慢于非右室限制性生理组(P=0.08).右室限制性生理组氨基末端脑钠肽(N-terminal pro-BNP,NT-proBNP)术后1周内显著升高, NT-proBNP≥4750pg/ml 提示患儿发生了右室限制性生理.中期随访结果表明两组患儿右室流出道梗阻、肺主动脉梗阻、肺动脉分支狭窄的发生率无明显差别,肺动脉反流程度也无明显差别(P均>0.05).结论 TOF术后发生右室限制性生理与手术年龄和体外循环时间相关,NT-proBNP可作为评估TOF术后患儿发生右室限制性生理的指标.术后早期右室限制性生理的发生在一定程度上会影响患儿转归,然而随访数据表明右室限制性生理对患儿中期转归无明显影响.     

Evaluation of patients undergoing Senning repair with two-dimensional contrast echocardiography

Summary The postoperative anatomy of the Senning interatrial diversion was defined by two-dimensional contrast echocardiography in ten patients. A modified apical four-chamber view proved most valuable, allowing for simultaneous visualization of both the systemic venous atrium and the pulmonary venous atrium. The anatomy was confirmed with two-dimensional contrast echocardiography utilizing catheters placed in the systemic venous atrium and pulmonary venous atrium at the time of surgery. In addition, in several of the patients, we were able to detect atrial or ventricular level shunts which were not clinically apparent. One patient who was judged to have a significant shunt by two-dimensional contrast echocardiography had no anatomic site found at autopsy to explain the shunting. We conclude that two-dimensional contrast echocardiography can define the postoperative anatomy following Senning repair allowing for immediate and future evaluation. Shunting at the atrial and ventricular levels can also be detected, but the method is very sensitive and difficult to quantitate or correlate clinically.     

Celiac artery flow pattern in infants with single right ventricle following the Norwood procedure with a modified Blalock-Taussig or right ventricle to pulmonary artery shunt

A potential advantage of the right ventricle to pulmonary artery versus modified Blalock?CTaussig shunt in patients undergoing the Norwood procedure is limitation of diastolic runoff from the systemic to pulmonary circulation. We evaluated mesenteric flow patterns and gastrointestinal outcomes following the Norwood procedure associated with either shunt type. Patients randomized to a right ventricle to pulmonary artery versus modified Blalock?CTaussig shunt in the Pediatric Heart Network Single Ventricle Reconstruction Trial at centers participating in this ancillary study were eligible for inclusion; those with active necrotizing enterocolitis, sepsis, or end-organ dysfunction were excluded. Celiac artery flow characteristics and gastrointestinal outcomes were collected at discharge. Forty-four patients (five centers) were included. Median age at surgery was 5?days [interquartile range (IQR)?=?4?C8?days]. Median celiac artery resistive index (an indicator of resistance to perfusion) was higher in the modified Blalock?CTaussig shunt group (n?=?19) versus the right ventricle to pulmonary artery shunt group (n?=?25) [1.00 (IQR?=?0.84?C1.14) vs. 0.82 (IQR?=?0.74?C1.00), p?=?0.02]. There was no difference in interstage weight gain, necrotizing enterocolitis, or feeding intolerance episodes between the groups. The celiac artery resistive index was higher in patients with the modified Blalock?CTaussig shunt versus the right ventricle to pulmonary artery shunt but was not associated with measured gastrointestinal outcomes.     

Dysrhythmias following the Mustard and Senning operation for transposition of the great arteries

The prevalence and nature of late dysrhythmias following operative repair of transposition of the great arteries were evaluated in 32 children using pre- and postoperative standard electrocardiograms and postoperative Holter monitoring. The Mustard procedure was performed in 17 patients, the Senning procedure in 15 patients. The mean age at operation was 40 +/- 35 months in the Mustard and 9 +/- 6 months in the Senning group. The mean interval between operation and Holter monitoring 7 +/- 2 years in the Mustard and 1.6 +/- 1 years in the Senning group. All patients had sinus rhythm preoperatively. Following operation, the incidence of sinus rhythm increased from 59% in the Mustard and 87% in the Senning group in the first postoperative week to 76% in the Mustard and 100% in the Senning group at the end of follow-up (mean 4.6 +/- 3.5 years). Holter monitoring revealed dysrhythmias or conduction abnormalities in 12 patients (9 Mustard, 3 Senning). A permanent pacemaker had to be inserted in 2 Mustard patients because of complete atrioventricular block and sinus bradycardia with sinus arrest, respectively. Results indicate a prevalence of late postoperative dysrhythmias of 20% in the Senning and of 59% in the Mustard group (p less than 0.05). However, the significantly longer mean follow-up period of the Mustard group precludes a direct comparison between the 2 types of repair.     

心肌致密化不全合并弥漫性冠状动脉右室瘘1例

目的：探讨心肌致密化不全合并弥漫性冠状动脉瘘的临床特点及诊断方法。方法：报告1例心肌致密化不全合并弥漫性冠状动脉右室瘘患儿,回顾其临床症状、辅助检查结果和治疗方案。在国外数据库（Pubmed、OVID和Elsevier）和国内数据库（万方和维普）中检索心肌致密化不全合并冠状动脉瘘和心肌病合并冠状动脉瘘病例的文献,检索时间为建库至2017年3月30日。总结此类病例的临床表现、诊断和治疗。结果：本文患儿为6月龄女婴,因“胃纳差伴盗汗2周”于2013年10月12日在上海交通大学医学院附属上海儿童医学中心心内科就诊。二维超声心动图显示,左心室增大伴左室致密化不全、左右冠状动脉扩张、右心室内心肌窦状间隙持续状态、二尖瓣中度返流、心室收缩功能低下等。心导管造影及冠状动脉造影检查显示,右冠状动脉及左前降支弥漫性微小右心室瘘、二尖瓣返流、左心室增大。予地高辛、速尿、安体舒通后患儿症状稍好转。国外数据库中检索到2例心肌致密化不全合并冠状动脉左室瘘的成年病例,以进行性呼吸困难和心前区疼痛为主要临床表现,心肌缺血表现更明显,诊断主要依靠二维超声心动图及心导管造影和冠状动脉造影检查,缺乏有效治疗手段;有部分心肌病合并心肌致密化不全患者接受选择性冠状动脉瘘介入封堵术的尝试。结论：心肌致密化不全合并冠状动脉瘘是一类罕见的先天性心脏发育异常疾病,婴儿临床症状主要为纳差、盗汗等心功能不全的表现,确诊主要依靠二维超声心动图以及心导管造影、冠脉造影检查,目前尚缺乏有效治疗手段,以强心、利尿、扩血管改善心功能治疗为主。     

Double-chambered right ventricle: case series

The double-chambered right ventricle is a rare congenital heart disease caused by hypertrophic anomalous muscle bands that divide the ventricular cavity, resulting in the formation of a high-pressure proximal chamber and other low-pressure distal one. Because of its evolving nature, its diagnosis is usually made during adolescence or adulthood. While pediatricians see patients whose age range is well established, currently pediatric cardiologists attend patients with cardiopathies from the fetal stage to adulthood, because they are more familiar with these malformations. We report a series of cases treated with divided right ventricle in a new public hospital (Congenital heart disease Section) of Buenos Aires province.     

Tissue plasminogen activator for a left atrial thrombus after Senning repair.

OBJECTIVE: To avoid the surgical removal of an obstructive thrombus in a Senning baffle by the administration of recombinant tissue-type plasminogen activator. SETTING: A pediatric intensive care unit in a children's university hospital. PATIENTS: A 3-yr-old male was diagnosed with a large left atrial thrombus 2 wks after Senning repair for D-transposition of the great arteries. The child presented with massive chylous pleural, pericardial effusions, and cardiac tamponade, secondary to partial obstruction of the pulmonary venous channel. INTERVENTION: Thrombolysis with recombinant tissue-type plasminogen activator was instituted. RESULTS: We observed a resolution of the thrombus in <48 hrs. Minor local bleeding was the only noted side effect. No signs of systemic thromboembolization were detected. CONCLUSION: Early thrombolysis with recombinant tissue-type plasminogen activator could be considered a possible alternative to surgical thrombectomy in selected postoperative pediatric cases, although there may be a potential risk of serious bleeding.     

Noonan syndrome with double-chambered right ventricle

The association of Noonan syndrome and a double-chambered right ventricle has not been reported previously in the medical literature. We report two patients with Noonan syndrome associated with pulmonary valve stenosis and double-chambered right ventricle due to anomalous hypertrophied muscle bundles. Pulmonary valve stenosis was operated on. However, hypertrophied muscle bundles were not resected, as they caused no obstruction to blood passage. Postoperative follow-up examinations revealed no symptoms. In conclusion, in those with Noonan syndrome there may be anomalous muscle bundles in the right ventricular cavity. It may not be necessary to resect them if they do not obstruct the right ventricular outflow tract.     

The right ventricle in Fabry disease

AIM: Left ventricular (LV) hypertrophy is a common feature in Fabry disease-related progressive infiltrative hypertrophic cardiomyopathy and affects both men and women, but at different ages. To date, however, little is known about the role of right ventricular (RV) function in Fabry disease. Therefore, this study aimed to investigate the extent of RV involvement in patients with Fabry disease. METHODS: Echocardiographic examination of the right and left ventricle was carried out in 129 patients (80 women and 49 men) with Fabry disease. RESULTS: RV hypertrophy was present in 46 patients (35.7%). Of these patients, 13 showed signs of severely depressed right systolic function (tricuspid annulus movement < 10 mm and a prolonged RV pre-ejection period/pulmonary ejection time ratio) and six patients showed additional severe depression of parameters of diastolic function (pseudo-normal or restrictive RV filling pattems). Those patients with RV hypertrophy and severely compromised systolic and diastolic function had the highest LV masses (92 +/- 11.7 g/m(2.7)). CONCLUSION: RV involvement is common in Fabry disease and ultimately progresses to severe systolic and diastolic RV dysfunction. These findings might explain why patients with preserved LV function can develop clinical features such as reduced exercise capacity, organomegaly and lymphoedema.     

The right ventricle in Fabry disease

Aim: Left ventricular (LV) hypertrophy is a common feature in Fabry disease-related progressive infiltrative hypertrophic cardiomyopathy and affects both men and women, but at different ages. To date, however, little is known about the role of right ventricular (RV) function in Fabry disease. Therefore, this study aimed to investigate the extent of RV involvement in patients with Fabry disease. Methods: Echocardiographic examination of the right and left ventricle was carried out in 129 patients (80 women and 49 men) with Fabry disease. Results: RV hypertrophy was present in 46 patients (35.7%). Of these patients, 13 showed signs of severely depressed right systolic function (tricuspid annulus movement <10 mm and a prolonged RV pre-ejection period/pulmonary ejection time ratio) and six patients showed additional severe depression of parameters of diastolic function (pseudo-normal or restrictive RV filling patterns). Those patients with RV hypertrophy and severely compromised systolic and diastolic function had the highest LV masses (92±11.7 g/m^2.7).
Conclusion: RV involvement is common in Fabry disease and ultimately progresses to severe systolic and diastolic RV dysfunction. These findings might explain why patients with preserved LV function can develop clinical features such as reduced exercise capacity, organomegaly and lymphoedema.     

Double-chambered right ventricle mimicking asymmetric septal hypertrophy

Double-chambered right ventricle (DCRV) is an uncommon congenital heart disease. Anomalous muscle bundles in the right ventricle divide the right ventricle into two portions and cause intracavitary obstruction of the right ventricle. We report a patient with DCRV mimicking asymmetric septal hypertrophy. We did clinical, echocardiographic and angiographic evaluation. Surgical intervention was successfully managed.     

Double-outlet right ventricle with intact ventricular septum

An infant with double-outlet right ventricle (DORV) with intact ventricular septum (VS) is described. Hypoplastic left ventricle and mitral valve atresia were also associated. To the authors' knowledge, this appears to be the first case in which this rare form has been demonstrated with magnetic resonance imaging (MRI). The patient required balloon atrial septostomy (BAS) three times until 4 months of age because of progressive cyanosis. In such a form of DORV, surgical atrial septectomy should be performed instead of repeating BAS because the tendency to functional closure of the restrictive secundum arterial septal defect caused by incompetent foramen ovale seems to be prominent.     

Double outflow right ventricle with pulmonary stenosis

Indian Journal of Pediatrics -     

Unguarded tricuspid orifice with two-chambered right ventricle

Summary Catheterization and anatomical data are presented of an infant with cyanosis due to a most unusual variant of unguarded tricuspid valve orifice in association with a two-chambered right ventricle. The inlet portion of the right ventricle ended blindly and was completely separated from the outlet portion by a muscular wall. The outlet chamber, which supported the pulmonary trunk, received its blood from the left ventricle through an outlet foramen. The condition is considered to be related to imperforate Ebstein's anomaly.     

Gadolinium-enhanced magnetic resonance angiography of right ventricle to pulmonary artery shunts following Norwood 1 palliation in infants

The classic Norwood I palliation for the treatment of hypoplastic left-heart disease includes an atrial septectomy, neoaorta creation, and a systemic-to-pulmonary artery shunt. The Norwood I palliation has been modified by the use of a right ventricle-to-pulmonary artery shunt instead of a traditional systemic-to-pulmonary artery shunt. Right ventricle-to-pulmonary artery shunts were non-invasively imaged in four infants using gadolinium-enhanced magnetic resonance angiography. Diagnostic MR angiograms were performed in all infants throughout the length of the shunt and were useful in the detection of shunt patency, stenosis, and aneurysm. Associated surgical conduits and shunts were also evaluated. All findings were verified by subsequent conventional angiography or surgery.     

Atrioventricular septal defect: Quantitative anatomy of the right ventricle

Summary In 19 human fetal and newborn hearts with atrioventricular septal defect (AVSD), not associated with other anomalies, the two ventricles were studied morphometrically. A total of 17 different parameters were studied: nine in the right ventricle and eight in the left. In the right ventricle we analyzed ventricular wall thickness, length of right ventricular inflow and outflow tracts, and volume of right ventricular inflow and outflow tracts. The data for these parameters were compared with the patterns of normality published previously, and the volumetric data were compared with patterns of normality published previously by us. The ventricular inflow tract was shorter than the outflow tract, the difference being especially significant in the left ventricle. The length of the diaphragmatic wall of the heart in both the right and left ventricle was equal to the sum of the length of the inflow tract and the thickness of the ventricular wall at the apex.     

Thrombosis of the right ventricle in 2 infants with myocardiopathy

We report two cases of left ventricular thrombosis in infants with myocardiopathy. Patients were aged ten and twelve months respectively. Two-dimensional echocardiography, performed because of the development of heart failure, evidenced an echogenic image within the left ventricle and significant dilatation of the left ventricular chamber. One patient developed a peripheral arterial thrombosis that resolved under anticoagulant therapy. The intracardiac thrombus disappeared under anticoagulant therapy after one month in one patient and six months in the other. Pathophysiology of the left ventricular thrombosis is discussed; the dilatation of the left ventricle apparently played a significant role. We emphasize the value of early initiation of preventive anticoagulant therapy in patients with myocardiopathy.