Development of Superior Vena Cava to Pulmonary Vein Fistulae Following Modified Fontan Operation: Case Report of a Rare Anomaly and Embolization Therapy

A patient with double inlet single ventricle, L-transposition of the great arteries, and atrioventricular valve regurgitation developed progressive heart failure necessitating modified Fontan operation at 16 months of age. The procedure included replacement of the atrioventricular valve and pacemaker insertion. Eight years following the operation, the patient developed progressive cyanosis. Catheterization confirmed the presence of fistulae from the superior vena cava and innominate vein to the pulmonary veins as the cause of cyanosis. Coil embolization of several fistulae was performed successfully at catheterization. Contributing factors for fistula formation in Fontan patients are discussed, and therapy is reviewed.     

A Case of Plastic Bronchitis Presenting 9 Years After Fontan

A 15-year-old boy with hypoplastic left heart syndrome experienced plastic bronchitis 9 years after completion of a nonfenestrated lateral tunnel Fontan. Despite cardiac catheterization with coil embolization of collateral vessels and initiation of a pulmonary toilet regimen, including aerosolized tissue plasminogen activator, he continued to expectorate large acellular-mucinous casts. Finally, after optimization of cardiac function with the addition of carvedilol, the expectorated casts decreased in number. This report reviews pathophysiology of plastic bronchitis cast formation and therapy in the context of this late presentation after Fontan.     

Exercise Performance Following Repair of Hypoplastic Left Heart Syndrome: A Comparison with Other Types of Fontan Patients

Reports of exercise performance after Fontan surgery for hypoplastic left heart syndrome (HLHS) are lacking. We compared the exercise performance of total cavopulmonary connection type (TCPC) of Fontan subjects with HLHS (group 1, n= 7) to those not requiring a Norwood procedure having a systemic right ventricle (group 2, n= 6) or a systemic left ventricle (group 3, n= 8). The subjects underwent assessment of resting pulmonary mechanics followed by maximal exercise testing with a bicycle or treadmill protocol. ECG, oxygen consumption, and carbon dioxide production were measured continuously. There was not a significant difference seen between HLHS and the comparison groups for the following parameters: maximum heart rate, maximum oxygen consumption, respiratory exchange ratio, breathing reserve, and arterial oxygen saturation at rest or exercise. Exercise performance in the TCPC type of Fontan patients was comparable regardless of ventricular morphology or surgical approach.     

先天性心脏病肺动脉高压手术前后右心功能变化和治疗

目的 了解先天性心脏病左向右分流导致的重度肺动脉高压对右心功能的影响以及手术矫治后右心功能的恢复状态。方法 对室间隔缺损(室缺)合并重度肺动脉高压(全肺循环阻力增加)的40例患儿，用心导管的方法进行术前、术后5～7年右心功能、肺循环的血流动力学随访测定。结果 术前右心心搏指数、作功指数、心排指数显著高于术后；术前右房压、右室收缩压及舒张压、肺动脉压力和阻力均增高，缺损修补后右室舒张压恢复正常，收缩压的降低与肺动脉压力下降有关；大型室缺左向右分流重度肺动脉高压右心功能不全系继发性右室高排出量心力衰竭，且伴有舒张功能障碍。结论 治疗心力衰竭不宜首选正性心肌收缩药物；降低肺动脉压力、减少左向右分流、根治心内畸形是合理的选择；术后右室收缩压持续不能恢复至正常水平，提示继发性肺血管梗阻性病变存在。     

The Effect of Carvedilol Treatment on Chronic Heart Failure in Pediatric Patients With Dilated Cardiomyopathy: A Prospective, Randomized-Controlled Study

This study aimed to evaluate the clinical efficacy and safety of carvedilol for pediatric patients with chronic heart failure caused by dilated cardiomyopathy. Seven pediatric medical centers participated in this prospective study. Pediatric patients (n = 89) were randomly divided into an experimental group (carvedilol treatment) and a control group (conventional treatment). The analysis excluded 12 patients lost during the follow-up period. Carvedilol was added to the therapy of the experimental group after at least 1 month of basic treatment with digoxin, an angiotensin-converting-enzyme inhibitor, and diuretics. The control patients received the same basic treatment but did not receive carvedilol. The initial dose of carvedilol was 0.1 (mg/kg day), and the dose was doubled every 2 weeks until the maximum tolerated dose or 0.8 (mg/kg day) was achieved. The tolerated dose was maintained for 6 months. The Ross scales and echocardiographic parameters including left ventricular diastolic diameter, left ventricular systolic diameter, left ventricular ejection fraction, left ventricular fractional shortening, and serous brain natriuretic peptide (BNP) concentration, as well as clinical progress were compared between the two groups. The Ross scales decreased by 11.94 % in the experimental group, which was more than in the control group (2.81 %). In addition, changes to other echocardiographic parameters in the experimental group also were superior to those in the control group. The serous BNP concentration in the experimental group decreased by 30.1 %, which also was more than the decrease (22.2 %) observed in the control group. Clinical improvement was demonstrated by 40 %, no change by 35 %, and clinical deterioration by 25 % of the patients in the experimental group, and by respectively 37.8, 27, and 35.2 % of the patients in the control group. These differences were not statistically significant. Only one patient demonstrated a severe adverse event, severe pulmonary infection, and complete atrioventricular block. None of the other carvedilol-treated patients experienced drug-related side effects. Carvedilol can be safely used for treatment of chronic heart dysfunction in pediatric patients with dilated cardiomyopathy. The results in this study showed an apparent improvement in the cardiac function of these patients. Additional clinical studies are required to determine the most favorable dosing levels and regimens of carvedilol before its safety and efficacy for the pediatric population can be determined conclusively.     

Plastic bronchitis. A case report and review of the literature

Plastic bronchitis is a rare complication in which bronchial casts of lymphatic origin develop in the tracheobronchial tree and cause airway obstruction. The main feature is expectoration of bronchial casts. This condition usually occurs in the postoperative period after the Fontan operation for congenital heart disease. Treatment modalities include mucolytics and chest physiotherapy, and in the most severe cases bronchoscopy to remove the casts and aerosolized urokinase or r-TPA. We describe a 12-year-old boy with pulmonary atresia with intact ventricular septum and severely hypoplastic right ventricle who underwent a modified Fontan operation at the age of 7 years. At the age of 12 years the patient started to present frequent episodes of coughing, dyspnea, and desaturation followed by difficult emission of white bronchial casts. An extracardiac Fontan conversion was performed to improve hemodynamics and symptoms. During the postoperative period frequent episodes of airway obstruction required bronchoscopy and finally improved with administration of aerosolized urokinase.     

全腔静脉-肺动脉连接术Ⅰ期或分期生理矫治复杂先天性心脏病

目的 总结全腔静脉-肺动脉连接术(TCPC)生理矫治复杂先天性心脏病的疗效,探讨选择Ⅰ期或分期TCPC手术的策略.方法 2003年12月至2009年11月,为88例患儿施行了TCPC术.男58例,女30例.低风险病例进行Ⅰ期TCPC术,高风险病例进行分期TCPC术.Ⅰ期手术组41例,平均年龄(8.4±4.5)岁;分期手术组47例,平均年龄(9.0±4.2)岁.Ⅰ期TCPC组中27例采用外管道,14例肺动脉直接下拉与下腔静脉吻合.Ⅱ期TCPC组中42例采用外管道,3例采用心房内通道,2例肺动脉直接与下腔静脉吻合.结果 早期死亡6例,病死率6.8%.Ⅰ期TCPC组死亡5例(4例死于重度低心排征,1例死于呼吸衰竭),病死率12.2%;Ⅱ期TCPC组死亡1例(死于开胸时大出血),病死率2.1%,二组差异无统计学意义(P=0.054).二组的体外循环时间、辅助通气时间、胸管停留时间、住监护室时间、术后住院大数差异无统计学意义.65例随访4个月到6.5年.1例于术后8个月死于严重的房室瓣反流、心力衰竭;1例术后3个月出现感染性心内膜炎,死于多器官衰竭.结论 Ⅰ期和分期TCPC都能达到满意的手术效果.分期TCPC的效果似较Ⅰ期TCPC好.对存在两个或两个以上危险因素者,应选择分期手术.低风险患儿也应尽可能进行分期TCPC手术.     

Exertional Oscillatory Ventilation During Cardiopulmonary Exercise Test in Fontan Patients with Total Cavopulmonary Connection

Exertional oscillatory ventilation (EOV) has been noted during cardiopulmonary exercise testing (CPX) in patients with heart failure. EOV is a predictor of poor prognosis in adult patients with heart failure. The objective of this study was to clarify the incidence and influence of EOV in Fontan patients. Symptom-limited CPX was performed in 36 Fontan patients at 12.3 ± 4.3 (6.5–24.4) years of age or 5.9 ± 2.0 (3.0–11.2) years after total cavopulmonary connection (TCPC). Mean age at the time of TCPC was 6.3 ± 3.3. All 36 TCPC patients were classified as New York Heart Association classification I or II. They also underwent cardiac catheterization subsequently. EOV was defined as cyclic fluctuations in minute ventilation at rest that persist during effort lasting ≥60% of the exercise duration, with an amplitude ≥15% of the average resting value. EOV was noted in 21 of 36 Fontan patients (58%) with good clinical status. Univariable analysis between Fontan patients with and those without EOV showed significant differences in age at TCPC (p < 0.05), age at CPX (p < 0.02), weight at CPX (p < 0.02), follow-up duration between TCPC and CPX (p < 0.04), ventricular morphology (p < 0.05), and metabolic equivalents (p < 0.05) and peak minute oxygen uptake (VO₂) per body weight (p < 0.05). Multivariable analysis showed that EOV was significantly related to peak VO₂ per kilogram. In conclusion, EOV was frequently noted during exercise in Fontan patients with good clinical status. EOV during exercise seems to be related to higher peak VO₂ per kilogram and younger age at TCPC, which is a contrary result to those for adult patients with chronic heart failure. EOV is a remarkable phenomenon during exercise to compensate for impaired cardiopulmonary function in Fontan patients.     

全腔静脉-肺动脉连接术Ⅰ期或分期生理矫治复杂先天性心脏病

目的 总结全腔静脉-肺动脉连接术(TCPC)生理矫治复杂先天性心脏病的疗效,探讨选择Ⅰ期或分期TCPC手术的策略.方法 2003年12月至2009年11月,为88例患儿施行了TCPC术.男58例,女30例.低风险病例进行Ⅰ期TCPC术,高风险病例进行分期TCPC术.Ⅰ期手术组41例,平均年龄(8.4±4.5)岁;分期手术组47例,平均年龄(9.0±4.2)岁.Ⅰ期TCPC组中27例采用外管道,14例肺动脉直接下拉与下腔静脉吻合.Ⅱ期TCPC组中42例采用外管道,3例采用心房内通道,2例肺动脉直接与下腔静脉吻合.结果 早期死亡6例,病死率6.8%.Ⅰ期TCPC组死亡5例(4例死于重度低心排征,1例死于呼吸衰竭),病死率12.2%;Ⅱ期TCPC组死亡1例(死于开胸时大出血),病死率2.1%,二组差异无统计学意义(P=0.054).二组的体外循环时间、辅助通气时间、胸管停留时间、住监护室时间、术后住院大数差异无统计学意义.65例随访4个月到6.5年.1例于术后8个月死于严重的房室瓣反流、心力衰竭;1例术后3个月出现感染性心内膜炎,死于多器官衰竭.结论 Ⅰ期和分期TCPC都能达到满意的手术效果.分期TCPC的效果似较Ⅰ期TCPC好.对存在两个或两个以上危险因素者,应选择分期手术.低风险患儿也应尽可能进行分期TCPC手术.     

Left Innominate Vein–Pulmonary Artery Shunt with Glenn Anastomosis in a Fontan Candidate with Central Pulmonary Artery Stenosis

Heart failure developed 9 years after Fontan takedown with systemic–pulmonary artery shunt in a 12-year-old girl with pulmonary atresia, intact ventricular septum, and obstruction in the proximal pulmonary artery. Surgical scar after multiple operations complicated direct repair of the pulmonary artery, and thus she was not eligible for definitive palliation. Left innominate vein–to–left pulmonary artery shunt using an expanded polytetrafluoroethylene conduit in association with Glenn anastomosis functionally established an unobstructive superior cavopulmonary connection without direct repair of the central pulmonary artery, later facilitating one and a half ventricle repair. Use of an extraanatomical shunt may functionally relieve central pulmonary artery obstruction in candidates for Fontan-like circulation.     

Anomalous Origin of One Pulmonary Artery from the Innominate Artery: A Report of Two Cases

Two children with an anomalous origin of one pulmonary artery from the innominate artery are reported. One was a 15-month-old boy presenting with respiratory distress. He had a right aortic arch and his left pulmonary artery originated from the innominate artery. The other was a 1-month-old girl presenting with congestive heart failure. She had a left aortic arch and her right pulmonary artery originated from the innominate artery. An understanding of the embryological pathogenesis of these anomalies has significant therapeutic implications.     

可调式房间隔造口在改良Fontan术中的应用

目的 探讨可调式房间隔造口在改良Ｆｏｎｔａｎ术中的应用方法和临床意义。方法 ９例复杂先天性心脏病患儿,８例行全腔静脉肺动脉连接术、１例行右心房主肺动脉连接术。术中在心房内隧道补片或房间隔中作荷包缝合式可调式房间隔造口。１例术中结扎造口线关闭造口,４例永久性开放造口,４例将可调式装置引出胸外供术后调节。结果 全组无手术死亡和严重并发症。４例可调式装置引出胸外者,均在术后早期右心房压力下降后,予以调节     

不同剂量野百合碱诱导SD幼鼠右室阻力负荷性心力衰竭模型的比较

目的采用不同剂量野百合碱(monocrota line,MCT)诱导SD幼鼠右心衰竭模型,探讨适合的右心衰竭模型制作方法。方法清洁级SD雄性幼鼠125只,随机分为G0、G1、G2、G3、G4组,每组各25只SD幼鼠。G1～G4组幼鼠分别一次性腹腔注射MCT 30、40、50、60 mg/kg,诱导制作右心衰竭动物模型;G0组为对照组,给予生理盐水腹腔注射。腹腔注射后每2周1次,采用彩色多普勒超声仪测SD幼鼠右心室横径、三尖瓣反流速度、肺动脉压、射血分数(EF值)、缩短分数(FS值),共检测6周,4次;随后处死SD幼鼠,取心脏、肺组织病理切片对比观察。结果 G0组SD幼鼠无死亡,G1、G2、G3组SD幼鼠的死亡率远远低于G4组。各组SD幼鼠右心室横径逐渐增大,G1～G4组较G0组更明显(P<0.01);G0组SD幼鼠各时期的三尖瓣反流速度、肺动脉压无明显改变,而G1～G4组逐渐增高(P<0.01)。G1组SD幼鼠肺动脉仅出现轻度新生内膜病变,心脏组织病理切片无明显改变;G2组SD幼鼠肺动脉可见较明显新生内膜病变,心脏组织病理切片可见明显炎性细胞浸润;G3、G4组SD幼鼠肺组织病理切片可见中小动脉中膜明显增厚和小动脉肌化,部分肺组织远端小动脉闭塞,肺泡内可见心衰细胞,右室心肌细胞肥大伴纤维组织增生。结论 50 mg/kg MCT可成功诱导建立肺动脉高压致右心衰竭模型,其病理变化可更好模拟重度肺动脉高压致右心衰竭,且实验动物有较高的生存率。     

Carvedilol as therapy in pediatric heart failure: an initial multicenter experience

OBJECTIVE: The objective was to determine the dosing, efficacy, and side effects of the nonselective beta-blocker carvedilol for the management of heart failure in children. STUDY DESIGN: Carvedilol use in addition to standard medical therapy for pediatric heart failure was reviewed at 6 centers. RESULTS: Children with dilated cardiomyopathy (80%) and congenital heart disease (20%), age 3 months to 19 years (n = 46), were treated with carvedilol. The average initial dose was 0.08 mg/kg, uptitrated over a mean of 11.3 weeks to an average maintenance dose of 0.46 mg/kg. After 3 months on carvedilol, there were improvements in modified New York Heart Association class in 67% of patients (P =.0005, chi2 analysis) and improvement in mean shortening fraction from 16.2% to 19.0% (P =.005, paired t test). Side effects, mainly dizziness, hypotension, and headache, occurred in 54% of patients but were well tolerated. Adverse outcomes (death, cardiac transplantation, and ventricular-assist device placement) occurred in 30% of patients. CONCLUSIONS: Carvedilol as an adjunct to standard therapy for pediatric heart failure improves symptoms and left ventricular function. Side effects are common but well tolerated. Further prospective study is required to determine the effect of carvedilol on survival and to clearly define its role in pediatric heart failure therapy.     

复杂性先天性心脏病的介入治疗

目的探讨先天性心脏病(简称先心病)介入治疗在复杂性先心病中的应用.方法应用Rashkind球囊导管对59例复杂性先心病患儿行球囊房隔造口术(BAS);对2例右室发育不良综合征患儿进行静态球囊房隔扩张造口术;对1例在大动脉转位术后出现肺动脉吻合口狭窄的完全性大动脉转位(TGA)的患儿,采用球囊扩张导管进行扩张;对15例法洛四联症(TOF)患儿进行经皮球囊肺动脉瓣成形术(PBPV)、球囊血管成形术;对18例合并主动脉至肺动脉侧支血管的患儿以及5例B-T分流术的患儿在外科根治术前采用弹簧圈进行侧支血管和分流管道的堵塞术;对1例Fontan手术后的先心病患儿采用Amplatzer房间隔缺损(简称房缺)堵塞装置进行窗孔堵塞术.结果46例TGA患儿BAS后动脉血氧饱和度(SaO2)由0.57±0.17上升至0.76±0.13(t=14.58, P<0.01),左右心房压差﹤2 mmHg(1 mmHg=0.133 kPa),术后房缺5～20 mm;10例肺动脉闭锁伴室间隔完整的患儿BAS后SaO2变化不大(t=1.57, P﹥0.05),左右心房压差﹤2 mmHg;TOF患儿经PBPV及球囊血管成形术后SaO2平均上升15%,肺动脉瓣狭窄解除;18例主动脉至肺动脉侧支血管堵塞术,14例效果良好被完全堵塞;5例B-T分流管道堵塞术,全部封堵成功;1例Fontan手术后窗孔堵塞术,获完全封堵.结论复杂性先心病应用介入治疗和外科手术相配合,可获得更好的疗效.     

Diverse multi‐organ histopathologic changes in a failed Fontan patient

We report multi‐organ histopathological changes in a patient with protein‐losing enteropathy (PLE) over 12 years after Fontan operation. A 14‐year‐old boy with right isomerism heart and single ventricle had undergone Fontan procedure at 19 months of age, and PLE was diagnosed at 28 months. He had several episodes of intestinal bleeding and pre‐renal failure with elevated creatine, and eventually died of pneumonia. The intrapulmonary small arteries showed medial and intimal thickening resembling pulmonary hypertension. No major ulcerative lesions were found in the small or large intestines. Dilated lymph ducts, one of the characteristic features of PLE, were not seen in mucosal and submucosal areas. Liver cirrhosis was obvious despite little increase in liver enzymes. Histological changes in bilateral kidneys were subtle despite repeated episodes of renal failure. Thus, there may be significant discrepancies between clinical manifestations and multi‐organ histological changes in failed Fontan patients.     

Cardiac Catheterization Is Necessary Before Bidirectional Glenn and Fontan Procedures in Single Ventricle Physiology

There are significant mortality and morbidity in bidirectional Glenn (stage II) and Fontan (stage III) procedures for congenital heart diseases with single ventricle physiology. In order to minimize the mortality and morbidity, the presence or absence of hemodynamic and anatomical abnormalities, such as poor ventricular function, coarctation of the aorta, pulmonary artery distortion, high pulmonary resistance, and abnormal collateral vessels, should be evaluated. Echocardiography and magnetic resonance imaging (MRI) can visualize ventricular size and coarctation of the aorta but may fail to visualize pulmonary artery distortion. Furthermore, cardiac catheterization is needed to measure pulmonary resistance. If the pulmonary resistance is >3 Wood units·m² before stage II, the risk for the bidirectional Glenn operation may be high. Abnormal aortopulmonary collateral vessels may develop after the stage II procedure and echocardiography and MRI may not visualize these vessels. Coil embolization may be required to treat these vessels. In conclusion, cardiac catheterization is required to evaluate abnormalities, which may be treated by interventional catheterization, and to reduce mortality and morbidity of stage II and III procedures.     

功能性单心室Ⅱ期全腔静脉-肺动脉连接术后的监护管理

目的 探讨分期全腔静脉-肺动脉连接术(total cava-pulmonany correction, TCPC)治疗功能性单心室的疗效.方法 自2002年12月至2007年9月为18例既不适宜进行双心室解剖矫治、也不适宜Ⅰ期生理矫治的复杂先天性心脏病患儿施行了分期TCPC术.男13例,女5例.年龄4.0～16.0岁(Md=8.0岁).体重11.0～52.0kg(Md=19.5kg).所有患儿在Ⅰ期和Ⅱ期手术前均行心脏超声和心导管检查确诊.全组患儿入ICU后采取上身抬高30°,下身抬高15°体位.机械辅助呼吸期间避免使用高PEEP,尽早撤离呼吸机.观察头面部、四肢水肿情况,根据上、下腔静脉压力调整补液方案.术后根据肺动脉压力增高程度应用各种选择性肺血管扩张药物降低肺动脉压力.无明显活动性出血者,术后早期使用小剂量肝素(0.05～0.1mg·kg~(-1)·h~(-1))抗凝治疗.出ICU后改为口服华法令或阿斯匹林抗凝治疗.结果 术后早期存活16例,死亡2例,病死率11.1%.死亡原因为重度低心排出量综合征.全组辅助呼吸时间为9～157h,平均:(27.0±35.0)h,Md=17.5h.术后住院天数为11～86d(Md=17d).术后并发症有胸内出血2例,乳糜胸1例.存活病例术后经皮血氧饱和度(98.0±3.9)%,较术前数值(82.0±6.4)%显著上升(P＜0.01).结论 根据TCPC术后病理生理的改变,制定合理的治疗方案,对于提高TCPC术后成功率和减少术后并发症发生率有重要的作用.     

Enalapril for severe heart failure in infancy.

Eight infants aged between 4 days and 12 weeks with severe heart failure that was refractory to optimal conventional treatment with diuretics were treated with enalapril. The starting dose was 0.1 mg/kg/day, increasing according to response to 0.12-0.43 mg/kg/day. One infant with severe myocarditis did not tolerate enalapril because of hypotension and later died of intractable heart failure. Six of the remaining patients had congenital systemic to pulmonary shunts and one had a simple aortic coarctation. Two weeks after starting enalapril the clinical features of heart failure had improved in all the infants, the mean (SEM) plasma sodium concentration had increased from 129 (2.4) to 136 (1.1) mmol/l and plasma urea concentration had fallen from 7.0 (0.85) to 2.9 (0.85) mmol/l. These data suggest that enalapril is a potentially useful treatment for severe heart failure in infancy.