改良Fontan术治疗内脏异位综合征的术后护理

目的总结对15例Fontan手术治疗内脏异位综合征患者的术后护理经验。方法 Fontan手术是内脏异位综合征主要的手术方式,术中均采用心外管道建立下腔静脉一肺动脉连接。术后患者易出现肺动脉高压、心律失常、蛋白丢失性肠病、持续性胸腔积液等并发症,手术风险大。通过体位护理、呼吸机气道管理和并发症护理,为患者提供良好的康复条件。结果 15例患者经精心的治疗与护理,术后早、中期结果良好,无住院死亡,1年随访死亡1例。结论有针对性的、个性化的术后护理有利于Fontan手术患者顺利康复。     

改良Fontan手术治疗功能单心室合并左异构的早中期临床结果

目的:总结分析改良Fontan手术治疗功能单心室合并左异构的早期及中期临床结果。方法:回顾性纳入中国医学科学院阜外医院自2009年1月至2016年1月完成的改良Fontan手术治疗功能单心室合并左异构患者19例,依据是否分期完成改良Fontan手术,分为一期手术组(n=9)和分期手术组(n=10),对比分析两组患者术后早期及中期的临床结果。结果:19例患者中,男性13例(68.4%),女性6例(31.6%)。分期手术组患者行第一期Glenn手术时的平均年龄为(1.87±1.56)岁;两组患儿行Fontan手术时的平均年龄为(5.09±2.01)岁。住院期间死亡1例,为分期手术组患者;平均随访时间4.2(1~9)年,随访期间无死亡患者。两组患者术前在性别、年龄、身高、体重、心室形态、房室瓣反流及上腔静脉形态、肺动脉发育和平均肺动脉压力等方面的差异均无统计学意义(P均>0.05);术中体外循环时间、阻断时间、管道建立方式及术后住院时间的组间差异也均无统计学意义(P均>0.05)。在重症监护病房停留时间、机械辅助通气时间及术后血浆用量、胸腔引流量、胸腔引流时间、腹腔积液发生率等方面,一期手术组均优于分期手术组,差异均有统计学意义(P均<0.05)。两组患者术后均出现进行性血氧饱度下降,各有1例患者经皮血氧饱和度<85%,组间差异无统计学意义(P>0.05)。结论:与分期Fontan手术相比,一期Fontan手术治疗年龄偏大的功能单心室合并左异构患者具有良好的围术期结果,两种手术策略在早期和中期疗效方面无明显差异,术后均会出现氧饱和度进行性下降的问题,需要远期随访。     

Fontan手术后早期并发症分析

Fontan手术越来越多地应用于复杂紫绀型先天性心脏病的治疗，并取得较好的临床疗效。我院自1984年1月至2001年12月共施行134例Fontan手术，作者回顾性分析术后并发症及处理的经验。     

计算流体动力学在Fontan术后肾血流预测中的应用

目的：利用计算流体动力学探讨Fontan术后肾血流动力学变化,以辅助临床实现早期监测。方法：基于医学影像数据三维重建肾动脉分析Fontan手术前后不同时刻肾血流动力学参数差异。结果：肾动脉阻力指数及压力阶差在Fontan术后3 h较术前均增高,右肾动脉在术后3 d前均为高速流动,且术后左、右肾动脉血流动力学并未呈一致性改变。结论：计算流体动力学技术结合多普勒超声检查可为临床Fontan手术患儿早期异常肾血流动力学监测提供综合全面的评估,有助于患儿围手术期管理,改善预后。     

肺动脉高压靶向药物应用于儿童Fontan术围术期1例报道并文献复习

正Fontan术是目前功能性单心室患者治疗中的首选手术方案。术前肺动脉压力和肺血管阻力对于手术的成功与否至关重要。Hosein等~([1])的一项研究表明,术前肺动脉压力过高(肺动脉平均压 15 mmHg;1 mmHg=0.133 kPa)对Fontan术早期及远期预后均存在不良影响。此外,术前较低的肺动脉阻力和良好的心功能储备被认为是Fontan术后患者循环维持稳定的最重要的前提~([2])。由于一氧化氮通路失调及血管内皮功能障碍,Fontan     

一期Fontan手术治疗复杂紫绀型先天性心脏病21例

目的：总结一期房坦（ Fontan）手术治疗复杂紫绀型先天性心脏病的临床经验和疗效。方法2007-06～2012-12该院心血管外科对21例复杂紫绀型先天性心脏病患者施行了一期Fontan单心室手术,同期行房室瓣整形术8例。12例采取心内管道或自体心包内隧道连接,8例采取心外管道连接,1例采取不用管道材料肺动脉直接下拉吻合法。5例保留房间隔缺损或行心房板障开窗。结果20例手术获得成功,1例术后当天因低心排出量综合征死亡。1例房室瓣成形术后随访至25个月出现房室瓣重度反流,行机械瓣置换。术前经皮血氧饱和度为67％～91％,术后经皮血氧饱和度为88％～95％。术后随访（7～52个月）所有患者心功能Ⅰ～Ⅱ级。结论一期Fontan手术治疗复杂紫绀型先天性心脏病安全有效,可以避免多次手术创伤,节约医疗资源,但应注意把握手术适应证。     

Fontan循环

Fontan手术为全腔静脉一肺动脉连接术,是Glenn术后的二期手术方式.常用方法为使用心内隧道或心外管道将腔静脉血流完全导入肺动脉,使功能性单心室患儿获得生存机会.目前Fontan手术的手术指征较"经典"十大标准大为扩大,对于尚难直接Fontan手术时推荐分期手术,手术方式的改进提高了Fontan技术.手术的一些并发症影响了术后的转归,同时,对Fontan手术尚存一些争议.     

Fontan循环

Fontan手术为全腔静脉一肺动脉连接术,是Glenn术后的二期手术方式。常用方法为使用心内隧道或心外管道将腔静脉血流完全导入肺动脉,使功能性单心室患儿获得生存机会。目前Fontan手术的手术指征较“经典”十大标准大为扩大,对于尚难直接Fontan手术时推荐分期手术,手术方式的改进提高了Fontan技术。手术的一些并发症影响了术后的转归,同时,对Fontan手术尚存一些争议。     

复杂先天性心脏病患者58例行改良Fontan术后的护理

目的总结复杂先天性心脏病患者行改良Fontan术后的护理问题及措施。方法 2013年1月到2014年12月58例复杂先天性心脏病患者在广东省人民医院接受改良Fontan手术。手术均在体外循环下进行,采用胸部正中切口,58例患者均接受肺动脉与下腔静脉直接连接的改良Fontan手术。术后采取特殊体位:即患儿上半身抬高30°~40°,下半身抬高15°~30°,应用正性肌力维护心功能;充分镇静、镇痛、降低肺血管阻力及严密监测血流动力学各项指标。结果术后死亡1例,死亡原因是术后出现重度低心排血量综合征并发多器官功能衰竭。住重症监护病房时间16~336 h,呼吸机辅助时间1~168 h,胸腔引流时间2~94 d,其中14例需重新放置胸腔引流管,胸液检查结果提示乳糜胸。57例经处理后好转治愈出院。结论改良Fontan术后患者的护理有其特殊性,术后严密监护,减少术后并发症,是保证患者存活的基础。     

婴幼儿复杂先天性心脏病的外科治疗——Fontan手术及术式改进

Fontan手术于1968年问世，为三尖瓣闭锁的外科治疗谱写了新的篇章。随着术式的不断改进，Fontan手术目前已成为许多复杂心脏畸形的治疗措施。     

Protein‐Losing Enteropathy after Fontan Operation

Protein‐losing enteropathy (PLE) is a poorly understood and enigmatic disease process affecting patients with single ventricle after Fontan operation. In those afflicted, PLE after Fontan operation results in significant morbidity and mortality. The pathophysiology of the disease is unknown; however, a proposed mechanism incorporates a combination of phenomena including: (1) altered hemodynamics, specifically low cardiac output; (2) increased mesenteric vascular resistance; (3) systemic inflammation; and (4) altered enterocyte basal membrane glycosaminoglycan make‐up. A paradigm for the clinical management of PLE after Fontan operation is proposed.     

Recurrent Protein‐losing Enteropathy and Tricuspid Valve Insufficiency in a Transplanted Heart: A Causal Relationship?

This case report describes a toddler who developed a protein‐losing enteropathy (PLE) 4 years after orthotopic heart transplantation (OHT). He was born with a hypoplastic left heart syndrome for which he underwent a successful Norwood procedure, a Hemi–Fontan palliation, and a Fontan palliation at 18 months of age. Fifteen months following the Fontan operation, he developed a PLE and Fontan failure requiring OHT. Four years after OHT, he developed a severe tricuspid regurgitation and a PLE. His PLE improved after tricuspid valve replacement. It is now 2 years since his tricuspid valve replacement and he remains clinically free of ascites and peripheral edema with a normal serum albumin level. His prosthetic tricuspid valve is functioning normally.     

Results of transcatheter Fontan fenestration to treat protein losing enteropathy.

Transcatheter fenestration to create an interatrial communication has been used to treat patients with protein losing enteropathy (PLE) after Fontan operation. No systematic data have been reported assessing the results of this procedure. Our institutional database was queried to identify patients after Fontan operation who had transcatheter fenestration to treat PLE. Clinical notes, laboratory data, echocardiograms, and cardiac catheterization data were reviewed. From 1995 to 2005, 16 transcatheter fenestration procedures were performed in seven patients. Median age at fenestration was 18 years (range 13-41 years). Median duration of follow-up was 3.6 years (range 0.2-10.4 years). Techniques for fenestration included blade/balloon septostomy, stent placement, Amplatzer-fenestrated ASD device, and balloon dilation of previous stent. Size of the fenestration created was 5.2 +/- 1.1 mm. Systemic venous pressure remained unchanged after fenestration. Cardiac index increased significantly. Reduction of ascites and edema was noted after 9 of the 16 procedures. Ten of 16 (63%) of fenestrations spontaneously occluded. Three patients are free of ascites although recurrence of PLE occurred in all. One patient with a patent fenestration continues to have ascites. Two patients had Fontan takedown. One patient had conversion to a fenestrated extracardiac conduit Fontan and died postoperatively. The results of transcatheter Fontan fenestration are often disappointing. Maintaining fenestration patency is difficult. Even after "successful" fenestration, resolution of PLE may be incomplete and recurrences have occurred in all. Early consideration should be given to Fontan takedown or cardiac transplant in severely symptomatic patients with PLE who do not respond to fenestration. Transcatheter fenestration may be a bridge to a definitive procedure.     

Clinical Outcomes and Improved Survival in Patients With Protein-Losing Enteropathy After the Fontan Operation

Background

Patients with protein-losing enteropathy (PLE) following the Fontan operation have a reported 50% mortality at 5 years after diagnosis.

Objectives

The aim of this study was to review outcomes in patients with PLE following the Fontan operation.

Methods

From 1992 to 2010, 42 patients (55% male) with PLE following the Fontan operation were identified from clinical databases at the Mayo Clinic. Data were collected retrospectively.

Results

Mean age at PLE diagnosis was 18.9 ± 11.0 years. Initial Fontan operation was performed at 10.1 ± 10.8 years of age. Mean time from Fontan operation to PLE diagnosis was 8.4 ± 14.2 years. Survival was 88% at 5 years. Decreased survival was seen in patients with high Fontan pressure (mean >15 mm Hg; p = 0.04), decreased ventricular function (ejection fraction <55%; p = 0.03), and New York Heart Association functional class >2 at diagnosis (p = 0.04). Patients who died had higher pulmonary vascular resistance (3.8 ± 1.6 Wood units [WU] vs. 2.1 ± 1.1 WU; p = 0.017), lower cardiac index (1.6 ± 0.4 l/min/m² vs. 2.7 ± 0.7 l/min/m²; p < 0.0001), and lower mixed venous saturation (53% vs. 66%; p = 0.01), compared with survivors. Factors were assessed at the time of PLE diagnosis. Treatments used more frequently in survivors with PLE included spironolactone (21 [68%]), octreotide (7 [21%]), sildenafil (6 [19%]), fenestration creation (15 [48%]), and relief of Fontan obstruction (7 [23%]).

Conclusions

PLE remains difficult to treat; however, in the current era, survival has improved with advances in treatment. Further study is needed to better understand the mechanism of disease and ideal treatment strategy.     

Reversal of protein losing enteropathy with prednisone in adults with modified Fontan operations: long term palliation or bridge to cardiac transplantation?

Protein losing enteropathy (PLE), defined as severe loss of serum protein into the intestine, occurs in 4-13% of patients after the Fontan procedure and carries a dismal prognosis with a five year survival between 46% and 59%. Chronically raised systemic venous pressure is thought to be responsible for the development of PLE in these patients, with perhaps superimposed immunological or inflammatory factors. The success rate of contemporary medical, transcatheter, and surgical treatments attempting to reduce systemic venous pressure ranges from 19% to 40%. Prednisone treatment for PLE has been tried, with variable success rates reported in children. The effect of prednisone in adult patients with PLE after the Fontan procedure is largely unknown. Two cases of PLE in adults (a 39 year old woman and a 25 year old man) after modified Fontan procedure who responded dramatically to oral prednisone treatment are reported, suggesting that a trial of this "non-invasive" treatment should be considered as long term palliation or bridge to cardiac transplantation.     

Recurrent Exacerbations of Protein-losing Enteropathy after Initiation of Growth Hormone Therapy in a Fontan Patient Controlled with Spironolactone

Protein-losing enteropathy (PLE) is a rare, but serious complication in single ventricle patients after Fontan palliation, and is associated with a 5-year mortality of 46%. We describe a patient with PLE after Fontan palliation who achieved remission with high-dose spironolactone (an aldosterone antagonist), but had three exacerbations each temporally correlated with the use of growth hormone (an aldosterone agonist). Because of the opposing mechanisms of action of these two medications, caution might be indicated when using growth hormone for patients with PLE who are successfully treated with spironolactone.     

Protein‐losing Enteropathy after the Fontan Operation: Associations and Predictors of Clinical Outcome

Objective. We sought to define what clinical parameters were related to the ultimate outcome in Fontan patients who had developed protein‐losing enteropathy (PLE). Background. PLE is a serious complication of the Fontan operation. Several preoperative and perioperative findings are associated with later PLE. However, there is limited information regarding postoperative abnormalities contributing to or influencing outcome in PLE. Methods. We evaluated 44 consecutive Fontan patients with PLE. A matched control group of Fontan patients without PLE was used for comparison (matched‐pair analyses). Kaplan–Meier and Cox proportional hazard methods were used for survival analyses. Results. Median age was 18 years (range 4–48 years). Short‐axis and apical fractional area change (ΔFA) were less in PLE patients than in controls (50 vs. 57% [P < .0001] and 49 vs. 54% [P = .01]). Five and 10‐year actuarial survival rates for the PLE group were 49 ± 9% and 30 ± 11%. Deceleration time <120 milliseconds (hazard ratio [HR] = 9.2, P = .04), New York Heart Association classification III or IV (HR = 4.0, P = .01), and lower serum albumin (HR = 0.30, P = .04) were independent predictors of mortality in those with PLE. Conclusion. Mild reduction in ΔFA was the only late echocardiographic finding associated with development of PLE. However, short deceleration time, poor New York Heart Association (NYHA) class, and low serum albumin identify a group of patients at the greatest risk for death. Presence of these findings in a PLE patient should lead to aggressive management strategies and may warrant early consideration of transplantation.     

Transcatheter fenestration of autologous pericardial extracardiac Fontan via the transhepatic approach

We describe a case of a patient with hypoplastic left heart syndrome (HLHS) who developed protein-losing enteropathy (PLE) following autologous pericardial extracardiac Fontan (APEF) operation with successful resolution of PLE following transcatheter fenestration via the transhepatic approach.     

Echocardiographic characteristics in Fontan patients before the onset of protein‐losing enteropathy or plastic bronchitis

Background

It was this study's objective to evaluate the echocardiographic characteristics and flow patterns in abdominal arteries of Fontan patients before the onset of protein‐losing enteropathy (PLE) or plastic bronchitis (PB).

Design

In this retrospective cohort investigation, we examined 170 Fontan patients from 32 different centers who had undergone echocardiographic and Doppler ultrasound examinations between June 2006 and May 2013. Follow‐up questionnaires were completed by 105 patients a median of 5.3 (1.5–8.5) years later to evaluate whether one of the complications had occurred since the examinations.

Results

A total of 91 patients never developed PLE or PB (“non‐PLE/PB”); they were compared to 14 affected patients. Eight of the 14 patients had already been diagnosed with “present PLE/PB” when examined. Six “future PLE/PB” patients developed those complications later on and were identified on follow‐up. The “future PLE/PB” patients presented significantly slower diastolic flow velocities in the celiac artery (0.1 (0.1–0.5) m/s vs 0.3 (0.1–1.0) m/s (P = .04) and in the superior mesenteric artery (0.0 (0.0–0.2) m/s vs 0.2 (0.0–0.6) m/s, P = .02) than the “non‐PLE/PB” group. Median resistance indices in the celiac artery were significantly higher (0.9 (0.8–0.9) m/s vs 0.8 (0.6–0.9) m/s, (P = .01)) even before the onset of PLE or PB.

Conclusion

An elevated flow resistance in the celiac artery may prevail in Fontan patients before the clinical manifestation of PLE or PB.     

Plastic Bronchitis and Protein-Losing Enteropathy in the Fontan Patient: Evolving Understanding and Emerging Therapies

Plastic bronchitis (PB) and protein-losing enteropathy (PLE) are rare but potentially devastating complications of the Fontan circulation. PB occurs in ～4% of Fontan patients, typically presents within 2 to 3 years of Fontan completion with chronic cough, wheezing, fever, or acute asphyxiation, and is characterised by proteinaceous airway casts that are expectorated or found on bronchoscopy. PLE develops in 4% to 13% of patients, usually within 5 to 10 years post Fontan, and manifests with edema, ascites, hypoalbuminemia, lymphopenia, hypogammaglobulinemia, and elevated fecal alpha-1 antitrypsin 1. These disorders have similar pathophysiology involving disruption of the lymphatic system resulting from elevated central venous pressure combined with elevated lymphatic production and inflammation, resulting in lymphatic drainage into low-pressure circuits such as the airways (PB) and duodenum (PLE). Our understanding of these disorders has greatly improved over the past decade as a result of advances in imaging of the lymphatic system through magnetic resonance lymphangiography and early success with lymphatic interventions including lymphatic embolisation, thoracic duct embolisation, and percutaneous thoracic duct decompression. Both PB and PLE require a multidisciplinary approach that addresses and optimises residual hemodynamic lesions through catheter-based intervention, lowers central venous pressure through medical therapy, minimises symptoms, and targets abnormal lymphatic perfusion when symptoms persist. This review summarises the pathophysiology of these disorders and the current evidence base regarding management, proposes treatment algorithms, and identifies future research opportunities. Key considerations regarding the development of a lymphatic intervention program are also highlighted.