Orthotopic heart transplantation in patients with univentricular physiology

Parallel advancements in surgical technique, preoperative and postoperative care, as well as a better understanding of physiology in patients with duct-dependent pulmonary or systemic circulation and a functional single ventricle, have led to superb results in staged palliation of most complex congenital heart disease (CHD) [1]. The Fontan procedure and its technical modifications have resulted in markedly improved outcomes of patients with single ventricle anatomy [2,3,4]. The improved early survival has led to an exponential increase of the proportion of Fontan patients surviving long into adolescence and young adulthood [5]. Improved early and late survival has not yet abolished late mortality secondary to myocardial failure, therefore increasing the referrals for cardiac transplantation [6]. Interstage attrition [7] is moreover expected in staged palliation towards completion of a Fontan-type circulation, while Fontan failure represents a growing indication for heart transplantation [8]. Heart transplantation has therefore become the potential "fourth stage" [9] or a possible alternative to a high-risk Fontan operation [10] in a strategy of staged palliation for single ventricle physiology. Heart transplant barely accounts for 16% of pediatric solid organ transplants [11]. The thirteenth official pediatric heart transplantation report- 2010 [11] indicates that pediatric recipients received only 12.5% of the total reported heart transplants worldwide. Congenital heart disease is not only the most common recipient diagnosis, but also the most powerful predictor of 1-year mortality after OHT. Results of orthotopic heart transplantations (OHT) for failing single ventricle physiology are mixed. Some authors advocate excellent early and mid-term survival after OHT for failing Fontan [9], while others suggest that rescue-OHT after failing Fontan seems unwarranted [10]. Moreover, OHT outcome appears to be different according to the surgical staging towards the Fontan operation and surgical technique of Fontan completion [12].The focus of this report is a complete review of the recent literature on OHT for failing single ventricles, outlining the clinical issues affecting Fontan failure, OHT listing and OHT outcome. These data are endorsed reporting our experience with OHT for failing single ventricle physiology in recent years.     

Single ventricle palliation: greater risk of complications with the Fontan procedure than with the bidirectional Glenn procedure alone

BACKGROUND: This study was performed to evaluate and compare the early, intermediate, and long-term outcomes of the bidirectional Glenn procedure and Fontan procedure in patients who live at moderately high altitude. METHODS: The outcome of each method of palliation for patients with a functionally single ventricle was retrospectively evaluated from a review of medical records. RESULTS: The bidirectional Glenn procedure was performed in 177 patients from October 1984 to June 2004. The Fontan procedure was performed in 149 patients from June 1978 to June 2004. Cardiovascular death or heart transplantation occurred in 8% of patients after the bidirectional Glenn procedure and 17% of patients after the Fontan procedure. Complications of systemic thromboembolic events, bleeding associated with anticoagulation therapy, protein losing enteropathy, and arrhythmias requiring implantation of a pacemaker, cardioversion, or radiofrequency ablation occurred in 7% of patients after the bidirectional Glenn procedure and 47% of patients after the Fontan procedure. Cardiovascular deaths and heart transplantation occurred less frequently when the Fontan procedure was performed in patients with a previous bidirectional Glenn procedure. However, the actuarial transplant-free survival and freedom from complications was not superior for a subgroup of patients who had a Fontan procedure after a bidirectional Glenn procedure in comparison to a subgroup of patients who had a bidirectional Glenn procedure alone. CONCLUSIONS: The bidirectional Glenn procedure can be used for long-term palliation of patients with a functionally single ventricle. Additional palliation with a Fontan procedure may increase the risk of stroke, protein losing enteropathy and arrhythmias without improving survival.     

Staged Single‐ventricle Palliation in 2011: Outcomes and Expectations

Outcomes for staged palliation for single‐ventricle heart disease have improved over the past two decades. As outcomes improve, parental expectations for survival and quality of life have risen accordingly. Nevertheless, the number of interventions and complications these patients must endure remain high. The final surgical destination of the single‐ventricle patient, the total cavopulmonary connection (or Fontan operation) successfully separates systemic venous and pulmonary venous blood flow but does so at great cost. Fontan patients remain at significant risk of complications despite what are perceived to be “favorable” hemodynamics. The outcomes in this population are discussed in this review, with particular attention to the history behind our current strategies as well as to recent salient studies.     

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.     

Outcome in infants with pulmonary atresia, intact ventricular septum, and right ventricle-dependent coronary circulation

Management of all patients with pulmonary atresia, intact ventricular septum, and right ventricle-dependent coronary circulation (n = 12) with staged surgery directed toward a Fontan palliation resulted in an 83% 5-year actuarial survival. Both deaths in the study were presumably related to coronary ischemia and occurred in the first 4 months of life.     

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。     

Impact of placing a conduit from the right ventricle to the pulmonary arteries as the first stage of further palliation in the Norwood sequence for hypoplasia of the left heart

OBJECTIVE: We describe the experience from a single institution with the Norwood sequence of palliation for hypoplasia of the left heart, emphasizing complications related to placement of a conduit from the right ventricle to the pulmonary arteries and their management. METHODS: Between November, 2002 and January, 2006, we palliated 32 patients with hypoplastic left heart syndrome or its variants by placing a conduit from the right ventricle to the pulmonary arteries. We reviewed retrospectively the charts and angiograms from these patients. RESULTS: Hospital survival after construction of the conduit was 90.6%. There were 3 interstage deaths, of which 2 were likely due severe obstruction of the conduit. Stents were implanted into the proximal or medial portions of the conduits of 3 patients. Early revision of the distal anastomosis, and shortening the conduit, was performed early postoperatively in 2 patients. So far, 24 out 26 survivors of the first stage underwent a bi-directional cavopulmonary anastomosis after a mean interval of 4.3 plus or minus 1.4 months. Of these, 3 required a semi-urgent second stage of palliation because of worsening cyanosis, with one patient dying after the second stage. Completion of the Fontan circulation by insertion of an extracardiac conduit was performed in 8 patients at the mean age of 19.8 plus or minus 2.2 months. We were able to achieve biventricular repair in 1 patient, with aortic atresia, hypoplastic arch and ventricular septal defect, 4.3 months after the initial palliative procedure. Overall survival of the whole cohort of 32 patients was 78.9%, plus or minus 7.8%, at 5 months, and 74.3%, plus or minus 8.6%, up to 25 months. CONCLUSIONS: The introduction of the conduit placed from the right ventricle to the pulmonary arteries has led to an improved outcome in the complex entity of hypoplastic left heart syndrome and its variants. Stenosis of the conduit, nonetheless, may account for significant interstage morbidity, and often requires intervention or early installation of the second stage of palliation.     

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.     

Surgical evaluation of the modified Fontan procedure.

From 1980 to 1990 152 patients underwent Fontan operation at our institution. The following patient groups were identified: 1. patients with tricuspid atresia (n = 82, 54.0%); 2. patients with single ventricle (n = 31, 20.3%); 3. patients with a wide variety of non correctable, complex cardiac malformations (n = 39, 25.7%). In 27.0% of the patients a primary Fontan operation was performed. 45.0% of the patients received a previous shunt to increase pulmonary blood flow and in 29.4% of the patients a pulmonary artery band was placed to reduce pulmonary flow. Overall mortality was not significantly different in patients with previous palliative procedures (19.4%, n = 18) as compared to 17.4% (n = 6) in patients with primary Fontan operation. Risk of death was high in the group with complex cardiac malformations (28.2%, n = 11) and in patients with single ventricle (19.4%, n = 6). Early mortality was considerably less in patients tricuspid atresia (8.5%, n = 7). Postoperatively patients with incorporation of the residual right ventricular chamber and pulmonary valve (Fontan-Bjoerk) showed a significant (p less than 0.05) lower incidence of pleural effusion as compared to patients with other modifications of the Fontan procedure. Actuarial survival rate of all patients is 83.8 +/- 3.1% (mean +/- SEM) at ten years. The modified Fontan procedures are providing an accepted surgical method for patients with otherwise non correctable cardiac malformations.     

Somatic Growth after Fontan and Mustard Palliation

Objective. Patients with complex congenital heart disease frequently develop early growth failure; however, the long‐term outcome for growth after surgery for single ventricle or anatomic right ventricle as systemic ventricle is not clear. This study was designed to determine long‐term growth in patients following the Fontan and Mustard operations. Method. We retrospectively reviewed the growth parameters of children who had previously undergone the Fontan (n = 80) or Mustard (n = 66) palliation at the Riley Hospital for Children, Indiana. Results. Both the Fontan and Mustard groups had normal height and weight at birth. At the time of their Fontan or Mustard palliation, there was a significant retardation in weight (Z‐score: ?0.98 and ?1.79, respectively) and height (Z‐score: ?0.96 and ?1.03, respectively). Both cohorts postoperatively demonstrated significant catch‐up in their weights. Although the Mustard group normalized their heights, the Fontan patients continued to demonstrate short statures in long‐term follow‐up. Conclusion. Children with single ventricles and those with palliated d‐loop transposition of the great arteries suffer somatic growth delay prior to definitive surgery, despite being of normal size at birth. Catch‐up growth in weight occurs after the Fontan and Mustard operations. In the Mustard population, height also normalizes, whereas in patients with univentricular circulation, height remains abnormally low.     

Surgical management of severe aortic outflow obstruction in lesions other than the hypoplastic left heart syndrome: use of a pulmonary artery to aorta anastomosis.

Between December 1985 and April 1990, 50 infants with a variety of congenital cardiac lesions other than the hypoplastic left heart syndrome underwent surgical relief of aortic outflow obstruction by creation of a pulmonary artery to aorta anastomosis. The patients were grouped anatomically by ventriculoarterial alignment. Nineteen had normally aligned great arteries (group I); 25 had transposition of the great arteries, all with a univentricular heart of left ventricular morphology (group II); and 6 had a double-outlet right ventricle (group III). All patients had either aortic stenosis with atresia, subaortic stenosis or a restrictive ventricular septal defect. Sixteen had normal arch anatomy; 34 had arch anomalies consisting of arch hypoplasia (n = 17), coarctation (n = 11), interruption of the arch (n = 4) and complex arch anomalies (n = 2). Surgery was performed at a median age of 10 days (range 2 to 184). Of the 50 infants, 33 survived. No significant difference in early survival (30 days) was noted among the groups of varying ventriculoarterial alignment (68% group I, 72% group II, 83% group III) (p greater than 0.05). Overall actuarial survival was 63% at 18 months. Analysis of actuarial survival by arch anatomy, although not statistically significant, revealed a trend toward better survival at 18 months postoperatively in infants with normal arch anatomy (81%) than in infants with arch anomalies (54%). Of the 33 survivors, 26 have proceeded to the next surgical stage, including the Fontan procedure in 8, superior cavopulmonary anastomosis in 13 and biventricular repair in 5.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pulsatile Glenn as long‐term palliation for single ventricle physiology patients

Objective: There are limited studies analyzing pulsatile Glenn as a long‐term pallia‐ tion strategy for single ventricle patients. This study sought to determine their out‐ comes at a single institution.
Design: A retrospective review was performed.
Setting: Study performed at a single pediatric hospital.
Patients: All single ventricle patients who underwent pulsatile Glenn from 1995 to 2016 were included.
Outcome measures: Pulsatile Glenn failure was defined as takedown, transplant, or death. Further palliation was defined as Fontan, 1.5, or biventricular repair. Risk fac‐ tors were assessed by Cox multivariable competing risk analyses.
Results: Seventy‐eight patients underwent pulsatile Glenn at age 9 months (inter‐ quartile range, 5‐14). In total, 28% had heterotaxy, 18% had a genetic syndrome, and 24% had an abnormal inferior vena cava. There were 3 (4%) perioperative mortalities. Further palliation was performed in 41 (53%) patients with a median time‐to‐pallia‐ tion of 4 years (interquartile range, 3‐5). Pulsatile Glenn failure occurred in 10 (13%) patients with 8 total mortalities. Five‐ and 10‐year transplant‐free survival were 91% and 84%, respectively. At a median follow‐up of 6 years (interquartile range, 2‐8), 27 patients (35%) remained with PG (age 7 years [interquartile range, 3‐11], oxygen sat‐ uration 83% ± 4%). Preoperative moderate‐severe atrioventricular valve regurgita‐ tion (AVVR) (hazard ratio 7.77; 95% confidence interval 1.80‐33.43; P =.005) and higher pulmonary vascular resistance (hazard ratio 2.59; 95% confidence interval 1.08‐6.15; P =.031) were predictors of pulsatile Glenn failure after adjusting for co‐ variates. Reaching further palliation was less likely in patients with preoperative moderate‐severe AVVR (hazard ratio 0.22, 95% confidence interval 0.08‐0.59; P =.002).
Conclusion: Pulsatile Glenn can be an effective tool to be used in challenging circum‐ stances, these patients can have a favorable long‐term prognosis without reducing their suitability for further palliation.     

Evaluation of Fontan liver disease: Correlation of transjugular liver biopsy with magnetic resonance and hemodynamics

Introduction: Liver fibrosis and cirrhosis are late complications in Fontan palliation. Liver biopsy is the gold standard. The goal of this study is to correlate transjugular liver biopsy (TJLB) in the setting of Fontan palliation with noninvasive testing and hemodynamics.
Methods: Between August 2014 and July 2017, 49 Fontan patients underwent TJLB. All the patients had hemodynamic evaluation, 28 patients had MRE (magnetic reso‐ nance elastography) and 40 patients had cardiopulmonary exercise test. Histologic liver fibrosis was quantitated using traditional histologic scoring systems and a modi‐ fied Ishak congestive hepatic fibrosis score.
Results: Median age 17.8 years, median time since Fontan 15.2 years. Primary diagnosis and Fontan type were variables, but predominantly LV morphology (30/49), lateral tun‐ nel Fontan (29/49), originally fenestrated (37/49), and 11/49 had a pacemaker. Histologic fibrosis correlated with MRE (R = 0.62, P ≤ .001). Histologic fibrosis and MRE correlated with Fontan pressure (R = 0.38, P = .008 & R = 0.59, P ≤ .001). Morphology of the single ventricle did not correlate with liver fibrosis. The presence of a fenestration resulted in a higher cardiac index (P = .026) but did not resulted in lower liver fibrosis (P = .64).
Conclusion: Noninvasive tests, such as MRE, may be suitable for longitudinal follow‐up in patients with single ventricle physiology. Our data suggest that there is reasonable cor‐ relation of MRE liver stiffness with biopsy scoring systems and Fontan pressures. We demonstrated the feasibility of TJLB in the setting of Fontan palliation and demonstrated its correlation with noninvasive measures particularly MRE. We recommend selective use of TJLB when MRE score is >5 KPa or when there are other clinical signs of cirrhosis.     

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.     

Regression of Pulmonary Arteriovenous Malformations after Transcatheter Reconnection of the Pulmonary Arteries in Patients with Unidirectional Fontan

Objective. Pulmonary arteriovenous malformations (PAVM) develop in patients with a lung deprived of hepatic blood flow. Unidirectional Fontan patients have superior vena cava (SVC) blood directed to the left or right pulmonary artery (PA), and inferior vena cava (IVC) and hepatic blood baffled to the contra‐lateral PA. Cyanosis often develops due to PAVM in the lung deprived of hepatic blood. We report experiences with transcatheter PA reconnection in patients with unidirectional Fontan and PAVM. Design. Patients with unidirectional Fontan, cyanosis, and PAVM underwent transcatheter PA reconnection. Following simultaneous injection of contrast into PAs, a transseptal needle was used to puncture from one PA to the other. A balloon‐mounted, uncovered stent was positioned bridging the pulmonary arteries. The procedure provided a nonrestrictive, permanent communication between the PAs. Results were retrospectively reviewed. Catheterization data and oxygen saturations before and after procedure were analyzed. Results. Six patients with a median age of 14 years (range 13–35 years) were identified. All patients had functional single ventricle with unidirectional Fontan and PAVM in the lung supplied solely by SVC flow. The reconnection procedure was performed successfully without complications in all cases. The median follow‐up time for all 6 patients was 12.8 months (range 5.8–19.3 months). Median oxygen saturation was 0.86 (range 0.81–0.92) prior to the procedure, and 0.95 (range 0.92–0.98) on follow‐up, with a median increase of 9% (range 8–11%). Conclusion. Patients with unidirectional Fontan and PAVM demonstrate increased oxygen saturations following reconnection of PAs, suggesting regression of PAVM. This procedure can be performed safely using uncovered stents, and it is effective in improving systemic oxygen saturations.     

单心室合并完全性肺静脉异位引流的外科治疗

目的:探讨单心室合并完全性肺静脉异位引流(TAPVC)的外科治疗效果。方法:2009-05至2011-09,我科共收治单心室合并TAPVC患儿11例,年龄(30.1±21.4)个月,体重(11.0±3.2)kg。例2行改良B-T分流术,例1、3～6、8、9、11行一侧或双侧双向腔肺分流术,例7、10行一期改良Fontan术。例1、6～9、11同期行TAPVC矫治术。结果:例6、10围术期死亡(18.2%,2/11),死亡原因分别为中枢神经系统感染和多器官功能衰竭。存活的患儿随访1～29个月,例4死亡,余患儿氧饱和度改善,未发现残余肺静脉梗阻,例5行二期改良Fontan术并顺利出院。结论:单心室合并TAPVC患儿仍具有较高早期死亡率,术前明确诊断TAPVC并制定合理的个体化治疗方案是提高手术疗效的关键。     

Percutaneous catheter aspiration thrombectomy for the occluded stents of pulmonary artery in children with single ventricle physiology after fontan surgery

Successful surgical palliation with the Fontan procedure allows survival into adulthood for many patients born with single ventricle (SV) physiology, but the limited studies reported incidence of perioperative and long‐term complications including thromboembolic events. Chronic pulmonary embolism is a common complication in patients with Fontan circulation, and may have serious consequences. Percutaneous intervention may be less invasive option for such a high‐risk population than surgery is. We described two patients who developed complete thrombosis of the left pulmonary artery following catheter placement of a stent in this vessel shortly after Fontan surgery. Percutaneous catheter aspiration thrombectomy was successfully performed. Percutaneous catheter aspiration thrombectomy may be considered as a viable option in acute thrombus in children with SV physiology after Fontan surgery. © 2014 Wiley Periodicals, Inc.     

The influence of different strategies on clinical outcome in patients undergoing total cavopulmonary connection.

BACKGROUND: We report on results of a prospective clinical trial designed to demonstrate the influence of various strategies in "Total Cavopulmonary Connection" (TCPC) for palliative therapy of patients with "single ventricle" physiology. METHODS: From 1989 to 1997, a total of 47 patients (mean age 4.8 +/- 3.6 years) underwent definitive TCPC at our unit. 31 patients (66%) underwent one-stage TCPC, in 16 patients (34%) we performed a two-stage modified Fontan operation; 21 patients had central fenestration (4 mm). Inhalative NO therapy in the immediate postoperative period was adopted in 1993. RESULTS: Overall 5-year survival was 76.4%, after two-stage TCPC 87.5%, and 81.3% in patients undergoing fenestrated procedures. Two of three patients survived perioperative Fontan take-down. We lost 11 patients (nine early and two late deaths): three patients died primarily because of neurologic dysfunction and eight patients because of cardiac failures. Under perioperative NO therapy there was no early death. After a mean follow-up of 35.9 +/- 23.3 months, 76% of all patients were in NYHA I and 21 % in NYHA I-II. 89.7% had sinus rhythm. 42% of our patients suffered from temporary pleuropericardial effusions. CONCLUSIONS: Definitive palliation with TCPC achieves acceptable clinical results. Two-stage repair, fenestration, and postoperative inhalative NO therapy - each have a positive influence on early and long-term survival.     

Course of tricuspid atresia in the Fontan era

Tricuspid atresia is an uncommon form of congenital heart disease and long-term survival was rare before the Fontan era. It was thought that the long-term survival of patients with tricuspid atresia would be improved by the introduction of the Fontan procedure and its subsequent modifications. This study reviews the clinical course of 84 patients with tricuspid atresia identified in the first year of life in the Fontan era. Prior palliative operations, their results and their ultimate application for the Fontan procedure were considered. Eleven patients died before surgical intervention and 5 did not undergo catheterization or echocardiographic confirmation before death. Five children underwent the Fontan procedure without prior palliation and 1 child does not require palliation at the present time. Sixty-seven patients (80%) had surgical procedures before evaluation for the suitability of a Fontan operation. Thirty-four patients had a second surgical palliation and 9 patients had a third palliation. The surgical mortalities for the first, second and third palliative surgery were 17.9, 17.6 and 0%, respectively. Thirty-two patients (38%) underwent the Fontan procedure and 2 deaths occurred (6%). An estimate of the probability of surviving for 1 year was 64% (95% confidence limits 54 to 74%) and that of 8 years was 55% (95% confidence limits 44 to 66%).     

Atrioventricular Valve Failure in Fontan Palliation

Background

Atrioventricular valve failure (moderate or greater regurgitation, or valve operation) is a risk factor for adverse outcomes in patients undergoing Fontan palliation.