Growth of children with a functionally single ventricle following palliation at moderately increased altitude

The bidirectional Glenn and Fontan procedures are empirically performed as interim and definitive procedures in children with a functionally single ventricle. The optimal stage of palliation, nonetheless, remains unknown. During childhood, growth is a fundamental measure of response to therapy. Growth may be influenced by the degree of cyanosis, the volume load on the ventricle, and cardiac performance. Thus, the weight and stature of children with a functionally single ventricle who underwent a bidirectional Glenn procedure or a Fontan procedure were studied to determine the effect of each intervention on growth. Z scores for weight and stature were retrospectively determined prior to palliation, at yearly intervals for 4 years, and from long-term measurements until 18 years of age in all patients with at least 2 years of observation following palliation. Growth was evaluated in 54 patients with a bidirectional Glenn procedure, and 65 patients with a Fontan procedure. The Z scores for weight were improved after each method of surgical palliation. Stature, however, was improved only following the bidirectional Glenn procedure. Growth was impaired in patients who developed protein losing enteropathy. Weight improved only during the initial 2 years after the Fontan procedure in patients who had a surgical fenestration. Over the long-term, patients who underwent a Fontan procedure were more likely to have a Z score less than -2.0 for weight and stature than patients who underwent only a bidirectional Glenn procedure. Late mortality and the incidence of heart transplantation were increased in patients who experienced a decrease in their rate of growth, defined as a negative change of more than one Z score in weight or stature, following the Fontan procedure. In conclusion, at moderately increased altitude, children with a functionally single ventricle grow more appropriately following the bidirectional Glenn procedure than following the Fontan procedure. A decrease in the rate of growth is associated with a poor prognosis following the Fontan procedure.     

Outcome of single ventricle and total anomalous pulmonary venous connection

Management of patients with single-ventricle physiology is significantly affected by anomalies of pulmonary venous return at all stages, whether primary palliation, bidirectional Glenn shunt, or completion of Fontan circulation. We treated 25 patients with pulmonary venous anomalies and single ventricle by staged palliation, from June 1996 to May 2005. Visceral heterotaxy with atrial isomerism was present in 19 of them. Primary palliation with a systemic-to-pulmonary artery shunt was undertaken in 15 patients. There were 5 early deaths, of which 4 were due to obstruction of pulmonary venous return. A bidirectional Glenn shunt was constructed in 17 patients including 10 who had it as a primary palliative procedure. There were 7 early deaths after the bidirectional Glenn procedure; only one was due to pulmonary venous obstruction. Five patients attained completion of the Fontan procedure. There was one early death after the Fontan operation. Anomalous pulmonary venous return can significantly complicate the management of patients with single ventricle, with an impact on survival in early infancy. Palliation with the aim of instituting extracardiac conduit Fontan circulation allows greater latitude and more streamlined management.     

Risk factors in the Glenn bidirectional shunt as an intermediate procedure before Fontan correction

OBJECTIVE: The bidirectional Glenn shunt is the most common palliation before the Fontan repair, especially in high-risk patients. We studied the influence of certain risk factors in bidirectional Glenn results, with and without an additional source of pulmonary blood flow. METHODS: Between 1993 and 1998 twenty patients (6-53 months of age) underwent a bidirectional Glenn shunt as the intermediate repair for the Fontan procedure. Diagnoses were: 7 cases of double inlet single ventricle, 4 of tricuspid atresia, 3 of unbalanced AV septal defect, 4 of mitral atresia, 1 hypoplastic left heart syndrome and 1 TGA with hypoplasia of the right ventricle. 17 patients had undergone previous operations. Mean preoperative arterial oxygen saturation was 78.5%. In 6 patients an auxiliary source of pulmonary blood flow was added. RESULTS: Hospital mortality was 15%. In 4 patients the bidirectional Glenn failed. By univariate analysis low weight, preoperative functional status and high pulmonary pressure were factors associated with early death. In bidirectional Glenn failure only the duration of ventilatory support was significant. By multivariate analysis, preoperative functional status and pulmonary pressure were significant. Mean postoperative arterial oxygen saturation at a mean follow-up of 10 months was 84%. CONCLUSIONS: Bidirectional Glenn shunt is an effective and low-risk palliation for patients with univentricular hearts. Only low weight and high pulmonary pressure were significant in hospital mortality, and we advise the association of an additional source of pulmonary blood flow in these patients at the start. Early extubation provides correct performance of the shunt.     

Cardiac transplantation after the Fontan or Glenn procedure

OBJECTIVES: The purpose of this study was to review the clinical course and outcome of cardiac transplantation after a failed Glenn or Fontan procedure. BACKGROUND: Late complications of the Glenn or Fontan procedure, including ventricular failure, cyanosis, protein-losing enteropathy, thromboembolism, and dysrhythmias often lead to significant morbidity and mortality. If other therapies are ineffective, cardiac transplantation is the only therapeutic recourse. Transplantation in this unique population presents significant challenges in the operative and perioperative periods. METHODS: The anatomic diagnoses, previous operations, clinical status, and indications for transplantation were characterized in patients transplanted after a Glenn or Fontan procedure. Outcomes after transplantation, including postoperative complications and mortality, were reviewed. Comparisons were made between survivors and nonsurvivors. RESULTS: Primary orthotopic cardiac transplantation was performed in 35 patients (mean age 15.7 +/- 8.5 years) with a mean follow-up of 54 +/- 46 months. A total of 11 patients had undergone a Glenn shunt and 24 patients a Fontan procedure. Indications for transplantation were a combination of causes including ventricular dysfunction, failed Fontan physiology, and/or cyanosis. Ten patients died 相似文献   

Is it necessary to routinely fenestrate an extracardiac fontan?

OBJECTIVES: This study was conducted to assess the need for, and use of, fenestration of an extracardiac conduit Fontan. BACKGROUND: Fenestration of a Fontan connection has been proposed as a means of improving outcomes of single ventricle palliation. The benefit of fenestration is likely to be greatest in the early postoperative period when patients may experience increased pulmonary vascular resistance and decreased ventricular function due to the effects of cardiopulmonary bypass, aortic cross-clamping and positive pressure ventilation. However, there are potential drawbacks to fenestration. The utility of fenestration with extracardiac Fontan operation has not been determined. METHODS: Since 1992, 81 patients have undergone a modification of the Fontan procedure in which an extracardiac inferior cavopulmonary conduit is used in combination with a previously staged bidirectional Glenn anastomosis. We conducted a retrospective review of these patients. RESULTS: Fenestration was performed selectively in 32 patients (39%), including only 2 of the last 38 (5%). In seven patients, a fenestration was placed or clipped in the early postoperative period without cardiopulmonary bypass. There were two operative deaths. Prolonged (>2 weeks) pleural drainage occurred in 13 patients, 8 with fenestration and 5 without. In addition to undergoing earlier Fontan in our experience, patients who had a fenestration placed had significantly higher preoperative pulmonary vascular resistance, significantly higher common atrial pressure after Fontan and significantly lower post-Fontan systemic arterial oxygen saturation. Fontan pressure did not differ between nonfenestrated and fenestrated patients. At follow-up ranging to five years, there were two late deaths and no patients developed protein losing enteropathy. CONCLUSIONS: Fenestration is not necessary in most Fontan patients when an extracardiac conduit technique is performed as described in this article, and therefore, should not be performed routinely with the extracardiac conduit Fontan. The need for fenestration should be assessed after cardiopulmonary bypass when hemodynamics can be evaluated accurately. Fenestration can be placed and revised easily without bypass and with minimal intervention in patients with an extracardiac conduit Fontan.     

Improved early results with cavopulmonary connections

BACKGROUND: We describe the recent results in a large cohort of patients with functionally single ventricle who underwent various modifications of cavopulmonary connections. METHODS: Using the database at our institution, we identified all children who underwent cavopulmonary connection operations between June 1995 and June 1997. Demographic data, surgical history, and perioperative course were reviewed. RESULTS: We performed 130 consecutive operations in 113 patients. The procedures included superior cavopulmonary connections in the form of the HemiFontan procedure in 45 instances, and bidirectional Glenn procedures in 11, and bilateral superior cavopulmonary connections in 7. The median age of these patients was 7.0 months. We completed Fontan operations using a fenestrated lateral tunnel on 47 occasions, and using an extracardiac conduit 9 times, 5 of which were fenestrated. A lateral tunnel without fenestration was constructed in one patient. The median age for these procedures was 19.5 months. In the remaining 10 instances, we revised Fontan procedures at a median age of 8 years. Diagnoses included hypoplastic left heart syndrome in 43 patients, double outlet right ventricle in 22, heterotaxy in 13, tricuspid atresia in 13, and a miscellaneous group accounting for the other 22. One death (0.7%) occurred within 30 days of surgery. Clinical seizures occurred in 7 children (5.3%), 6 had no residual neurologic deficits. Atrial pacing was needed in 14 children (10.7%) because of transient junctional rhythm, and 2 received treatment for supraventricular tachycardia. Pleural effusions were diagnosed radiographically after 31 of 130 (24%) procedures. Diuretic therapy resolved the effusion in 21 of these, with only 6 children requiring thoracostomy catheter drainage, and 4 undergoing thoracentesis alone. The median length of stay on the intensive care unit was 2 days, with a range from 1 to 30 days, and median stay in hospital was 6 days, with a range from 3 to 58 days. CONCLUSION: Mortality and perioperative morbidity after cavopulmonary connections have decreased dramatically in the current era. The long-term results of staged reconstruction for functionally single ventricle, nonetheless, await ongoing study.     

Treatment of elevated pulmonary artery pressure in a child after Glenn procedure: transcatheter closure of pulmonary artery banding with subsequent sildenafil therapy

An additional source of pulmonary blood flow in a patient with bidirectional Glenn procedure (BGD) may cause elevation of mean pulmonary artery pressure (MPAP), precluding safe completion of the Fontan operation. We present a case of single ventricle physiology after pulmonary artery banding (PAB) and Glenn procedure. At the age of six years, cardiac catheterisation revealed in the patient elevated MPAP (22 mm Hg). The PAB was closed through the right internal jugular vein with an Amplatzer Atrial Septal Occluder. After the procedure, MPAP remained at a similar level. Sildenafil oral therapy was applied for six months. Subsequent heart catheterisation confirmed complete closure of PAB and decrease of MPAP to 10 mm Hg. The abovementioned complex treatment of elevated MPAP pressure in a child after Glenn therapy allowed safe completion of the Fontan operation.     

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.     

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.     

Single-stage versus two-stage modified Fontan procedure

We compared surgical outcomes of the single-stage and two-stage modified Fontan procedures to clarify clinical superiority. Of 28 children undergoing a modified Fontan procedure from October 1995 to October 2005, 15 had a 1-stage and 13 had a 2-stage operation. In the 2-stage group, pulmonary artery growth was evaluated before and after the first stage. Operative mortality was 26.6% in the 1-stage group and 0% in the 2-stage group. The benefits of a previous bidirectional Glenn shunt were decreased cyanosis and volume overload, but there was no significant difference in pulmonary artery growth reflected in pulmonary artery indices before and after the bidirectional Glenn procedure. Older children underwent a 2-stage modified Fontan procedure and had better outcomes in terms of lower mortality, improved oxygen saturation, decreased volume load, and less deterioration of atrioventricular valve regurgitation.     

Discontinuous Pulmonary Arteries Do Not Preclude Good Fontan Outcomes

Objective. Discontinuous pulmonary arteries are believed to portend poor outcomes for a single ventricle palliation leading to Fontan's operation. This is a single institutional review of patients with single ventricle and discontinuous pulmonary arteries who underwent pulmonary artery centralization as part of staged surgical palliation. Design. The study is a retrospective case series. Patients. From November 1997 to December 2005, 12 centralization procedures were performed on 12 single ventricle patients with discontinuous pulmonary arteries. The diagnoses at surgery were as follows: heterotaxy 67%, pulmonary atresia 75%, a single morphologic right ventricle 58%, a single morphologic left ventricle 33%, and functional single ventricle with atrial situs inversus 8%. Outcome Measures. The outcome was assessed by hospital survival, actuarial survival, and New York Heart Association (NYHA) classification at follow-up. Results. The overall actuarial survival following centralization is 100% (95% confidence interval = 0.698 to 1). Seventy-five percent of the patients have undergone a Fontan procedure. Median McGoon ratio pre-Fontan = 1.65 (range: 1–2.1). Median follow-up after Fontan = 4.4 years (range: 1.2–9 years). Overall actuarial survival following Fontan is 100% (95% confidence interval = 0.428–0.911). Following the Fontan, there have been no thromboembolic complications, protein-losing enteropathy, nor Fontan takedowns. One hundred percent of the Fontan patients are NYHA class I. Conclusions. This experience indicates that a resuscitative strategy for discontinuous pulmonary arteries can result in good outcomes after the Fontan procedure. The presence of discontinuous pulmonary arteries in patients with single ventricle physiology should not preclude a management strategy with the goal of Fontan candidacy.     

心外管道全腔肺动脉连接术在复杂先心病中的应用

目的：总结心外管道全腔肺动脉连接（total cavopulmonary connection,TCPC）术治疗复杂先天性心脏病的临床经验。方法：2002年1月～2011年5月,采用外管道TCPC术矫治复杂先天性心脏病47例（病种包括三尖瓣闭锁、单心室、肺动脉闭锁、右心室发育不良、右室双出口）患者,手术年龄（8±4）岁。一期手术25例,分期手术22例。结果：术后早期（术后1月）死亡4例（8%）。死亡原因：多脏器功能衰竭、心脏骤停、感染、蛋白丢失性肠病和低心排。术后早期并发症发生率为17%,主要为肺水肿3例、胸腔积液2例、心律失常1例、心包积液1例、蛋白丢失性肠病和低心排1例。术后均随访,随访时间9～87月。远期再住院率为9%（4/43）,再次手术7%（3/43）。2例再次出现胸腔积液,2例因自行停服肠溶阿司匹林后出现外管道堵塞再次手术,1例于术后出现低心排死亡。其余患者恢复良好。结论：对于适应证明确的复杂先天性心脏病患者行心外管道TCPC手术治疗,近期临床效果满意。     

成人功能性单心室的一期Fontan手术治疗

目的:总结和分析成人功能性单心室Fontan手术治疗的早、中期结果。方法: 自2001年11月~2010年11月,共15例成人功能性单心室患者行一期Fontan手术治疗,术前心功能NYHA Ⅱ级4例,NYHA Ⅲ级11例;手术适应证为肺动脉发育良好,肺动脉平均压≤15 mmHg;手术方法为心外管道法Fontan手术,6例行人工血管和右心房开窗吻合,随访3月～6年。结果: 围术期死亡2例,术后平均拔除气管插管时间11 h（6～576 h）,平均住院时间13 d（7～44 d）,平均中心静脉压13 mmHg（7～16 mmHg）,平均动脉血氧饱和度96％（87%～99％）;12例患者心功能得到明显改善,4例心功能NYHA Ⅰ级,8例心功能NYHA Ⅱ级,1例心功能仍为NYHA Ⅲ级。结论: 一期心外管道法Fontan手术治疗成人功能性单心室手术早期和中期疗效良好。     

Fontan术后应用波生坦对中远期预后的影响

目的 Fontan术是一种常用的姑息性术式,他能够提高一些复杂先心患者的生存期限,但术后出现心肌功能障碍,心律失常,血栓事件,蛋白丢失性肠病和运动耐量的降低等一系列并发症的发生率也居高不下。一些研究认为引起运动耐量下降、心肌功能障碍的原因可能是肺动脉压力和肺血管阻力的升高,而血浆内皮素-1已经被证实与肺血管阻力增加直接相关,且是Fontan循环衰竭的危险因素。这就提示我们在Fontan术后使用内皮素受体拮抗剂(波生坦)可能会改善他们的预后。方法 符合条件的受试者将被随机分为治疗组和安慰剂组。波生坦治疗的方案与肺动脉高压靶向药物治疗的方案一样,在手术后的第2年,所有患者均在我院行心脏CT检查,并检测心功能的NYHA分级、6MWT肝功能(ALT)。结果 2年后揭盲,两组均无死亡患者。但是在次要观察指标上两次显示出了一定差异,其中试验组在心功能和6MWT方面显著优于对照组,差异有统计学意义。术后2年复查心脏CT,发现两组患者均有研究对象存在肺部下野细小肺动-静脉瘘生成的现象,但是都未观察到粗大肺动-静脉瘘,试验组发生率(20%)虽然少于对照组(50%),但是差异无统计学意义。肝功能ALT两组病例差异无统计学意义,初步确定了波生坦在本研究中的安全性。结论 波生坦能够改善Fontan术后患者纽约心功能状况及增加6分钟步行距离,但没有观察到他能够在其他方面带来更多的获益。     

Abnormal distribution of pulmonary blood flow after the Glenn shunt or Fontan procedure: risk of development of arteriovenous fistulae

Since the Fontan procedure results in low pulsatile pulmonary blood flow similar to that seen in patients with a Glenn shunt, it may also be associated with abnormal distribution of flow to the lower lung lobes and with the development of pulmonary arteriovenous fistulae (PAVF). In 12 patients 0.8 to 4.5 years after Fontan procedure and in 20 patients 0.2 to 18 years after receipt of Glenn shunts we assessed ventilation (with 133Xe) and perfusion (after a peripheral injection of 99mTc-macroaggregated albumin) to compare upper to lower lobe distribution of blood flow with that in a control group. The presence of PAVF was assessed by radionuclide activity in kidneys and the brain and by a two-dimensional echocardiographic contrast study. A decreased upper/lower lobe perfusion ratio was noted in 13 of 20 patients with Glenn shunts (65%) and correlated with the time after surgery (p less than .05). Despite the shorter follow-up period, two of 12 (16%) patients who had undergone the Fontan procedure also had a decreased upper/lower lobe perfusion ratio, and one of these developed right heart failure. Brain and kidney radionuclide counts above control values were observed in all patients with Glenn shunts and in 11 of 12 patients who had the Fontan operation. However, in only five of 20 (25%) patients with Glenn shunts were PAVF confirmed by the two-dimensional echocardiographic contrast study. Three of the five patients with PAVF had Glenn shunts of long duration.(ABSTRACT TRUNCATED AT 250 WORDS)     

The Role of Iron Deficiency in Protein‐losing Enteropathy Following the Fontan Procedure

We describe the case of an adult who developed protein‐losing enteropathy 21 years after having undergone a Fontan procedure. Investigations revealed iron deficiency anemia which was treated with intravenous iron sucrose leading to resolution of both the anemia as well as symptoms of protein‐losing enteropathy.     

The bidirectional Glenn operation in 100 cases with complex congenital heat diseases: factors influencing surgical results]

OBJECTIVE: The bidirectional Glenn shunt is a well established surgical technique in children with complex congenital heart disease. The present study is a retrospective analysis of patients undergoing this operation in order to assess the influence of different variables in the outcome. PATIENTS AND METHOD: From December 1990 to June 2000, 100 patients received a bidirectional Glenn shunt.Two groups were defined, based on the outcome. Group A (n = 15, unsuccessfully result) including death and need to reoperate, and Group B (n = 85, patients with good outcome). RESULTS: The mortality was 8%. Eight patients were reoperated at follow-up. The survivors were followed for a mean of 3.5 years. Mean pulmonary artery pressure 7 mmHg was a factor associated with poor clinical progress. Other variables (age less than 1 year, excessive pulmonary blood flow, double Glenn operation, significant anatomic anomalies, and arrythmias), were also associated with outcome. Significant variations were observed in the time of by-pass or the need for aortic clamp in cases with simultaneous operative repair of pulmonary branch stenosis. Actuarial survival rate, most more than 1 year was 92%, and freedom from reoperation at 3 years was 90%. CONCLUSIONS: The bidirectional Glenn shunt is an excellent palliation in patients with functionally single ventricle. Mean pulmonary artery pressure was the most important variable related with the outcome. We are encouraged to continue with tendency to perform bidirectional Glenn shunt preferably early, avoiding, whenever possible, previous palliative surgery.     

Arrhythmias after the Fontan procedure.

OBJECTIVE--To study the determinants and outcome of arrhythmias after the Fontan type operation. DESIGN--Retrospective analysis of data in patients operated on between 1972 and 1986 (follow up 5-19 years (mean 12 years)). PATIENTS--All 60 patients undergoing a Fontan type procedure at the National Heart Hospital, London, during the study period (mean age (SD) 12.3 (6.8) years). RESULTS--Postoperative arrhythmias occurred in 34 patients (57%), and 11 (58%) of 19 early postoperative deaths (within seven days) were related to arrhythmias. Early arrhythmias occurred in 19 (32%) patients of whom 11 (58%) died. All patients with early atrial fibrillation and His bundle tachycardia died and only preoperative atrial fibrillation recurred early. There was a higher incidence of early arrhythmias, which were less well tolerated, in double inlet single ventricle patients (9/19) than in those with tricuspid atresia (8/37). There were no other preoperative determinants of early arrhythmias or deaths from early arrhythmia. Late (after seven days) arrhythmias occurred in 15 (37% of hospital survivors). They had higher right atrial (RA) pressures both early and late after operation and had lower ventricular ejection fractions late after operation. Of those with atrial arrhythmias 86% had RA obstruction and 57% had an RA thrombus or pulmonary embolism at presentation; this was also confirmed in two patients in whom late sudden deaths occurred. Atrial fibrillation early after reoperation for RA obstruction was fatal. The actuarial arrhythmia free survival for hospital survivors was 60% at 10 years. CONCLUSIONS--Early postoperative arrhythmias were poorly tolerated, particularly atrial fibrillation and His bundle tachycardia. Previous atrial fibrillation was a relative contraindication to this procedure. Late postoperative arrhythmias were associated with higher RA pressures measured both early and late after operation and worse late ventricular function. Late arrhythmias may be the first manifestation of RA obstruction, which must be sought. RA thrombus was common in patients with atrial arrhythmias and should be treated early with anticoagulants.     

Fontan procedure--indication and clinical results

Twenty-six patients with tricuspid atresia (15), univentricular heart (7), and single ventricle (4) underwent 27 Fontan or modified Fontan procedures between 1975 and 1981. The age of the patients varied between 4 and 26 years. Twenty patients had had a total of 33 palliative operations prior to correction. The original Fontan procedure was performed in 10 patients from 1975 to 1977. According to the various anatomical findings modifications of the Fontan procedure, such as direct anastomosis or implantation of a valveless conduit, were introduced in 1977. Early mortality among all the patients was 22% (6 patients died). Three deaths occurred in the initial period 1975 to 1977. Among the last 20 patients (1978 to 1981) there were 3 early deaths. Three patients with single ventricle survived, one died due to pulmonary failure. There were 2 late deaths (sepsis, sudden cardiac death). Postoperative cardiac catheterization performed in 17 patients revealed excellent results in 13 patients; the remaining 4 displayed diminished arterial oxygen saturation, three of them had Glenn palliation prior to corrective surgery. Postoperative right atrial mean pressure varied from 10 to 23 mmHg. The left ventricular parameters were within the normal range.     

Postoperative and long‐term outcomes in children with Trisomy 21 and single ventricle palliation

Objective: Patients with Trisomy 21 (T21) and single ventricle (SV) physiology present unique challenges compared to euploidic counterparts. This study reports postoperative and long‐term outcomes in patients with T21 and SV palliation.
Design: This retrospective cohort study from the Pediatric Cardiac Care Consortium (PCCC) included patients with T21 (<21 years old) that underwent surgical palliation for SV between 1982 and 2008 and control patients without known genetic anom‐ aly following Fontan palliation for similar diagnoses. Kaplan‐Meier survival plots were created based on death events obtained from the PCCC and by linkage with the National Death Index (NDI) and the Organ Procurement and Transplantation Network (OPTN) through 2014 for patients with adequate identifiers.
Results: We identified 118 children with T21 who underwent initial surgical SV pallia‐ tion. Among 90 (75.6%) patients surviving their first surgery, 66 (73.3%) underwent Glenn anastomosis and 25 (27.8%) completed Fontan palliation with in‐hospital sur‐ vival of 80.3% and 76.0%, respectively. Fifty‐three patients had sufficient identifiers for PCCC‐NDI‐OPTN linkage. Ten‐year survival, conditioned on discharge alive after the Fontan procedure, was 66.7% compared to 92.2% for 51 controls without genetic anomaly (P = .001). Median age at death for T21 patients following initial surgical SV palliation was 2.69 years (IQR 1.34‐7.12) with most deaths (89.2%) attributed to the underlying congenital heart disease (CHD).
Conclusions: Children with T21 and SV are at high risk for procedural and long‐term mortality related to their genetic condition and underlying CHD. Nevertheless, a se‐ lect group of patients can successfully complete Glenn or Fontan palliation, reaching satisfactory long‐term survival.