Total cavopulmonary connection with off-pump technique

The aim of this study was to evaluate an off-pump technique for total cavopulmonary connection. Between May 2000 and June 2004, 16 patients underwent total cavopulmonary connection without cardiopulmonary bypass. There were 8 males and 8 females, with a mean age of 9.8 +/- 6.2 years and a mean weight of 27.7 +/- 14.9 kg. Preoperative percutaneous oxygen saturation was 75% +/- 6%, and pulmonary arterial pressure was 12.6 +/- 2.7 mm Hg. Postoperative outcomes were retrospectively compared with a group of 17 patients who underwent total cavopulmonary connection with an extracardiac conduit under cardiopulmonary bypass. Two patients died early postoperatively. Chylothorax occurred in 2 patients and 3 underwent reoperation for fenestration between the extracardiac conduit and the right atrium in the early postoperative period. All survivors were acyanotic. On discharge from hospital, the percutaneous oxygen saturation breathing air was 95% +/- 3%. Patients in the off-pump group demonstrated significantly shorter extubation time and intensive care unit stay. This connection technique is easy to perform and helpful in the early management of patients with a functional univentricular heart. However, much remains to be learned about this unique physiologic system.     

Total cavopulmonary connection in a bedridden patient with Sotos syndrome

Sotos syndrome is an overgrowth disorder of unknown etiology associated with a high incidence of congenital heart defects. Of 60 patients with Sotos syndrome treated in our hospital, 6 had congenital heart defects. We describe a case of successful total cavopulmonary connection at 30 months of age in a patient having pulmonary atresia with intact ventricular septum and a patent ductus arteriosus who had walking disability. The postoperative course was uneventful.     

全腔静脉肺动脉连接术治疗复杂先天性心脏病

目的总结全腔静脉肺动脉连接术(TCPC)的经验和体会。方法6例复杂先天性心脏病患者均在全麻低温体外循环下手术。上腔静脉与右肺动脉吻合完毕后,2例采用右心房内隧道法、4例采用心房外管道完成TCPC手术(合并左位上腔静脉的2例同时行左侧G lenn手术)。结果1例三尖瓣闭锁患者术后2d死亡,5例治愈者血氧饱和度由术前的0.75±0.03提高至术后的0.93±0.02,生活质量改善。结论TCPC矫治复杂先心病,能明显改善患者的缺氧症状;术中肺动脉直接测压是能否行TCPC的重要依据。     

心外管道全腔静脉肺动脉连接术疗效分析

目的 :分析心外管道全腔静脉肺动脉连接手术的治疗效果。方法 :1990年 1月至 2 0 0 0年 1月施行心内全腔静脉肺动脉连接术 34例 ,心外管道全腔静脉肺动脉连接术 14例 ,对其围术期多种疗效指标进行分析。结果 :与心内全腔静脉肺动脉连接术相比 ,心外管道全腔静脉肺动脉连接术后多巴胺用量减少 ,中心静脉压降低 ,体外循环时间、胸腔引流管留置时间及监护天数均明显缩短。手术死亡率7 1%。结论 :心外管道全腔静脉肺动脉连接术有利于改善术后心脏功能 ,疗效优于心内全腔静脉肺动脉连接术。     

Surgical considerations of extracardiac total cavopulmonary connection

Extracardiac total cavopulmonary connection has been proposed as a rational alternative to the lateral intra-atrial tunnel for complex congenital cyanotic heart diseases undergoing univentricular repair. In five patients, aged 4 1/2 years to 27 years, an extracardiac lateral conduit was used for total cavopulmonary connection at our Institute. Extracardiac lateral conduits were constructed in all the five patients using polytetrafluoroethylene tube grafts. One child required reoperation because of graft thrombosis and an aortic homograft was used in him on the second occasion. Aortic cross clamp was completely avoided in all including reoperation. The results showed no early or late mortality. One child developed persistent hypoxemia and progressive hepatomegaly. Reoperation on 5th post-operative day revealed graft thrombosis with technically unrestricted anastomosis which was replaced by an aortic homograft. He also required embolectomy for inferior vena cava and hepatic veins thrombosis. Post-operative follow-up (range 3 months to 14 months) revealed all patients in functional class I and in normal sinus rhythm. Doppler echocardiography, magnetic resonance imaging and angiocardiography revealed unrestricted anastomoses and unobstructed pulmonary blood flow across the conduit. The technical advantages and haemodynamic benefits of this operation are encouraging. However, the lone incidence of graft thrombosis raises concern about the routine usage of synthetic grafts in extracardiac total pulmonary connection. Other inherent weakness appears to be the lack of growth potential of the synthetic tube.     

Interventional treatment of lateral tunnel dehiscence in a total cavopulmonary connection using a balloon expandable covered stent.

In this paper we present a patient with dehiscence of an intra-atrial tunnel previously constructed during a total cavopulmonary connection procedure. We describe the use of a custom made covered stent to seal off the dehisced segment, and abolish the intra-cardiac shunting. We believe this is the first account of such a procedure being undertaken.     

Transcatheter fenestration of a total cavopulmonary connection baffle

Transcatheter creation of a de novo fenestration of a total cavopulmonary connection baffle has not been previously reported from India. We present our experience with such a procedure in a 4-year-old child with recurrent pleural effusions in the early postoperative period.     

一期全腔静脉-肺动脉连接术治疗功能性单心室患者的中期疗效

目的探讨全腔静脉肺-动脉连接术(total cavopulmonary connection,TCPC)治疗功能性单心室患者的术后临床疗效。方法回顾性总结广东省人民医院2008年1月1日至2016年12月31日一期TCPC治疗功能性单心室患者50例的临床资料。其中女17例(34%),男33例(66%);手术年龄(10.5±7.6)岁,体质量(25.3±13.2)kg。所有患者术前均有活动后发绀、气促等临床表现。术前动脉血气示饱和度为80.1%±7.5%,术前血红蛋白浓度为(176.9±29.3)g/L,术前红细胞压积为0.52%±0.09%,术前肺动脉指数为(345.5±174.0)mm^2/m^2,术前肺动脉压力为(14.4±4.3)mmHg(1 mmHg=0.133 kPa)。随访截至2016年12月31日。结果全组患者住院期间病死率为10%,术后5年、8年生存率均为86%。COX多因素回归分析未发现与术后死亡相关的危险因素。随访期间10例患者需再次干预治疗,主要并发症包括反复胸腔积液、心包积液、房室瓣反流、蛋白丢失性肠病。术后5年、8年未干预率为82%,71%。结论一期TCPC是治疗功能性单心室达到生理性矫治的有效手术方案,但术前应严格把握适应证,具备条件的患者可行一期手术,对高危患者仍需行分期手术降低手术风险,减少死亡及中远期并发症。     

Outcomes of total cavopulmonary connection for single ventricle palliation

Background

The aim of this study was to review the early and mid-term outcomes of the total cavopulmonary connection (TCPC) procedure and evaluate risk factors for prolonged pleural effusions.

Methods

The clinical records of 82 consecutive patients, who underwent a TCPC operation between January 2008 and December 2013, were reviewed for incidence of prolonged pleural effusions, duration of ventilation time and pleural drainage, length of intensive care unit (ICU) stay, and early and mid-term morbidity and mortality.

Results

The median age at surgery was 3.0 years. The main single ventricle diagnoses included 18 cases of a double-inlet single ventricle, 17 cases of heterotaxy, 16 cases of tricuspid atresia, 4 cases of mitral atresia, 12 cases of unbalanced complete atrioventricular canal (CAVC), 5 cases of double-outlet right ventricle (DORV) combined with ventricular septal defect (VSD) and pulmonary stenosis (PS), 4 cases of transposition of the great arteries (TGA) combined with VSD and PS, 4 cases of corrected transposition of great arteries (cTGA) combined VSD and PS, and 2 cases of criss-cross heart. Preoperative mean pulmonary artery pressure (mPAP) was 13.66±2.21 mmHg with 23.2% (n=19) higher than 15 mmHg. A total of 61 (74.4%) patients underwent a fenestration. The perioperative mortality was 4.9%. The median duration of pleural effusion was 10 days (range, 3−80 days), and prolonged pleural effusions occurred in 16 (19.5%) patients. Multivariable analysis revealed that mPAP >15 mmHg was independently associated with prolonged pleural effusions (OR, 8.33; 95% CI, 2.33−29.74; P=0.001), and creation of a fenestration was associated with decreased odds of effusion (OR, 0.21; 95% CI, 0.06−0.74; P=0.015). Five-year estimated Kaplan-Meier survival of two-stage TCPC was significantly higher than that of one-stage group(96.7% vs. 79.7%, P=0.023). Patients with heterotaxy or obstructed totally anomalous pulmonary venous connection (TAPVC) had significantly worse mid-term survival.

Conclusions

Staged TCPC improved the early and mid-term survival of patients with a single ventricle. mPAP >15 mmHg was independently associated with prolonged pleural effusions and a fenestration significantly associated with a lower odds of effusion.     

Pulmonary and caval flow dynamics after total cavopulmonary connection

OBJECTIVE: To assess flow dynamics after total cavopulmonary connection (TCPC). DESIGN: Cross-sectional study. SETTING: Aarhus University Hospital. PATIENTS: Seven patients (mean age 9 (4-18) years) who had previously undergone a lateral tunnel TCPC mean 2 (0. 3-5) years earlier. INTERVENTIONS: Pressure recordings (cardiac catheterisation), flow volume, and temporal changes of flow in the lateral tunnel, superior vena cava, and right and left pulmonary arteries (magnetic resonance velocity mapping). RESULTS: Superior vena cava flow was similar to lateral tunnel flow (1.7 (0.6-1.9) v 1. 3 (0.9-2.4) l/min*m2) (NS), and right pulmonary artery flow was higher than left pulmonary artery flow (1.7 (0.6-4.3) v 1.1 (0.8-2. 5) l/min*m2, p < 0.05). The flow pulsatility index was highest in the lateral tunnel (2.0 (1.1-8.5)), lowest in the superior vena cava (0.8 (0.5-2.4)), and intermediate in the left and right pulmonary arteries (1.6 (0.9-2.0) and 1.2 (0.4-1.9), respectively). Flow and pressure waveforms were biphasic with maxima in atrial systole and late ventricular systole. CONCLUSIONS: Following a standard lateral tunnel TCPC, flow returning via the superior vena cava is not lower than flow returning via the inferior vena cava as otherwise seen in healthy subjects; flow distribution to the pulmonary arteries is optimal; and some pulsatility is preserved primarily in the lateral tunnel and the corresponding pulmonary artery. This study provides in vivo data for future in vitro and computer model studies.     

Ambulatory 24-hour electrocardiographic monitoring following total cavopulmonary connection.

A total of 72 patients (53 males, 19 females) in the age range 1-22 years (mean 6.4 years) who underwent univentricular repair between April 1990 and May 1997 at our institute were followed up from six months to seven years (mean 3.6 years). Twenty-four hours ambulatory electrocardiographic Holter monitoring was done in all the patients. Thirty-four out of 40 patients (85%) who underwent fenestrated total cavopulmonary connection and 25 out of 32 patients (78%) who underwent non-fenestrated total cavopulmonary connection had normal Holter recording; thus 59 out of 72 (82%) patients had normal findings. Among the fenestrated total cavopulmonary connections, three patients each had atrial tachycardia and sinus bradycardia. In the non-fenestrated group, two patients had atrial tachycardia, three had supraventricular tachycardia, one sinus bradycardia and one had intermittent ventricular tachycardia. No correlation was found between age at surgery, pre-operative morphology, cross clamp time or cardiopulmonary bypass time with post-operative arrhythmia. To conclude, post-operative arrhythmias following total cavopulmonary connection were not related to age at surgery, pre-operative morphology, cross clamp or cardiopulmonary bypass time. However, a larger patient pool and longer follow-up is required for evaluation of any definitive correlation.     

B-type natriuretic peptide levels in patients with functionally univentricular hearts after total cavopulmonary connection

AIMS: To assess plasma B-type natriuretic peptide (BNP) levels in patients with univentricular hearts late after volume unloading by total cavopulmonary connection (TCPC). METHODS: Plasma BNP was measured by sandwich immunoassay in 67 patients after a modified Fontan procedure. BNP levels were compared with age and sex-specific normal values, clinical and echocardiographic data, and results of exercise testing. RESULTS: BNP had a wide range of 5-290 pg/ml, but was normal in 81% of patients, and median BNP was only 13 pg/ml. There was no difference between males and females, between patients with left or right ventricular morphology, and no correlation was found between BNP and age, post-operative follow-up period, maximum exercise capability, peak oxygen uptake, or blood oxygen saturation. New York Heart Association class I patients had lower BNP than class II patients (p=0.04). Plasma BNP concentration was positively correlated to the number of specific sequelae (r=0.59, p<0.001), and to severity of atrioventricular regurgitation (r=0.38, p=0.002). 5/13 patients with elevated BNP died during follow-up. CONCLUSION: BNP plasma concentration was normal in the majority of patients up to 15 years after TCPC. Elevated and increasing BNP levels were associated with increased morbidity and late mortality.     

Changes in pulmonary artery size before and after total cavopulmonary connection

Objective—To assess changes in size of the central pulmonary arteries following a total cavopulmonary connection (TCPC).
Design—A retrospective analysis of the angiographic diameters of the central pulmonary arteries, expressed as z scores, in infancy before the TCPC and 3.5 (0.9) years (mean (SD)) later. Analysis of the relation between the pulmonary arteriolar resistance and the z scores at follow up.
Setting—Tertiary referral centre.
Patients—32 patients who had TCPC from February 1990 to July 1993.
Results—The patients were divided into two groups (n = 16) depending on their preoperative flow ratio: group I, Qp/Qs  1; group II, Qp/Qs > 1. At the initial study in infancy the mean z scores in group I were −6.0 for the right pulmonary artery (RPA) and −9.6 for the left pulmonary artery (LPA); in group II the respective values were −2.7 and −3.0. Before the TCPC the values increased to 0.5 (RPA) and −0.5 (LPA) in group I, and to 8.8 (RPA) and 8.2 (LPA) in group II. At follow up the z scores decreased to −2.4 (RPA) and −4.9 (LPA) in group I, and to 2.2 (RPA) and −0.7 (LPA) in group II. The changes in pulmonary artery diameters were significant for both groups (p < 0.02). Following the TCPC, no significant difference in pulmonary arteriolar resistance index was found between patients with relatively small pulmonary arteries (z score RPA+LPA  0) and those with relatively large pulmonary arteries (z score RPA+LPA > 0).
Conclusions—Creation of a TCPC results in a significant reduction in size of the central pulmonary arteries. At a mean interval of 3.5 years following the TCPC, however, there was no significant difference in pulmonary arteriolar resistance index between patients with smaller and larger central pulmonary arteries.

Keywords: total cavopulmonary connection;  congenital heart disease;  pulmonary artery size;  pulmonary arteriolar resistance