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.     

Twisted atrioventricular valves in double inlet left ventricle

Twisted atrioventricular connections usually occur in hearts with biventricular artioventricular connections. Here, we describe a case of twisted atrioventricular valves associated with double inlet left ventricle and discordant ventriculo-arterial connections. Color Doppler echocardiography, and cine magnetic resonance imaging, clearly demonstrated that the right atrioventricular valve was located anterior and superior to the left atrioventricular valve, and that the axes of the two atrioventricular valves crossed each other within the dominant left ventricle.     

Considerations in Critical-Care and Anesthetic Management of Adult Patients Living With Fontan Circulation

The Fontan procedure is a staged palliation for various complex congenital cardiac lesions, including tricuspid atresia, pulmonary atresia, hypoplastic left heart syndrome, and double-inlet left ventricle, all of which involve a functional single-ventricle physiology. The complexity of the patients’ original anatomy combined with the anatomic and physiologic consequences of the Fontan circulation creates challenges. Teens and adults living with Fontan palliation will need perioperative support for noncardiac surgery, peripartum management for labour and delivery, interventions related to their structural heart disease, electrophysiology procedures, pacemakers, cardioversions, cardiac surgery, transplantation, and advanced mechanical support. This review focuses on the anesthetic and intensive care unit (ICU) management of these patients during their perioperative journey, with an emphasis on the continuity of preintervention planning, referral pathways, and postintervention ICU management. Requests for recipes and doses of medications are frequent; however, as in normal anesthesia and ICU practice, the method of anesthesia and dosing are dependent on the presenting medical/surgical conditions and the underlying anatomy and physiologic reserve. A patient with Fontan palliation in their early 20s attending school full-time with a cavopulmonary connection is likely to have more reserve than a patient in their late 40s with an atriopulmonary Fontan at home waiting for a heart transplant. Each case will require an anesthetic and critical care plan tailored to the situation. The critical care environment is a natural extension of the anesthetic management of a patient, with complex considerations for a patient with Fontan palliation.     

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。     

Bidirectional cavopulmonary anastomosis in congenital heart disease. Functional and clinical outcome.

The bidirectional cavopulmonary anastomosis is a surgical procedure suitable for patients with cyanotic congenital heart disease and univentricular physiology. This operation is able to increase the effective pulmonary blood flow without any additional load on the cardiac work and without any further distortion on the pulmonary artery branches. The cavopulmonary anastomosis can represent the first stage for patients destined for Fontan repair or a definitive palliative operation in high risk Fontan candidates. In order to test the hypothesis of a definitive palliation by cavopulmonary anastomosis in this kind of patients, we evaluated the hemodynamic data before and after this surgical approach and compared these data with their clinical and functional outcome. We evaluated 74 patients submitted to bidirectional cavopulmonary anastomosis by either hemodynamic or functional evaluation. End-diastolic and end-systolic ventricular volumes were significantly reduced by bidirectional cavopulmonary anastomosis (p less than 0.0005). Despite these data and a normal ambulatory ECG, spirometry and echocardiographic analysis, the stress test showed discouraging results. In fact, mean work time and peak heart rate were significantly different from normal values showing an impaired functional capacity of these children. In conclusion we think that bidirectional cavopulmonary anastomosis can not be considered an adequate definitive palliation but it represents a very good stage to preserve the pulmonary arteries and to prepare the systemic ventricle towards the Fontan repair.     

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.     

Reopening of persistent left superior caval vein after bidirectional cavopulmonary connections

Persistent left superior vena cava (SVC) is a not uncommon finding in patients with congenital heart disease. This anatomical variant must be recognised before doing a Glenn anastomosis, bidirectional cavopulmonary connection or a Fontan-type procedure. Following these procedures, reopening of a left SVC leading to clinical cyanosis can occur. Five cases are described in whom persisting left SVCs were excluded before performing a bidirectional cavopulmonary connection or Fontan procedure but (re-)opened after surgery, leading to cyanosis either by reducing effective pulmonary blood flow (bidirectional cavopulmonary connection) or by an obligatory right to left shunt (Fontan). These observations suggest that, embryologically, the lumen of the left SVC obliterates rather than disappears. Balloon occlusion angiography of the innominate vein before cavopulmonary connections or Fontan procedures might improve detection of these collateral vessels.

Keywords: persistent left superior vena cava; cavopulmonary connection; Fontan procedure; congenital heart disease     

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.     

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.     

Parallel arterial trunks with discordant atrioventricular and concordant ventriculo-arterial connections in mirror-imaged arrangement

Cardiac malformations are very rare in which the great arteries arise from their appropriate ventricles, but are abnormally related to each other. Here, we present a patient with mirror-imaged atrial arrangement and a left-sided heart who has parallel arterial trunks with discordant atrioventricular and concordant ventriculo-arterial connections. The ventricles were related in supero-inferior fashion, the left ventricle being anterior and superior, this being an additional feature which, to the best of our knowledge, has not previously been described in this setting.     

Amplatzer occlusion of accessory ventriculopulmonary connections

Background: Children with complex congenital heart disease often require staged palliation to regulate systemic and pulmonary blood flow. Accessory sources of pulmonary blood flow including aortopulmonary collaterals, aortopulmonary shunts, and ventriculopulmonary connections following Glenn or Fontan palliation can be associated with elevated central venous pressures and persistent pleural drainage. Occlusion of accessory ventriculopulmonary connections in this setting has traditionally been accomplished surgically. Objective: To review the efficacy of Amplatzer devices in transcatheter occlusion of accessory ventriculopulmonary connections in children with complex congenital heart disease. Methods: Patients were identified and their records retrospectively reviewed for indication, procedural details, and clinical efficacy and outcome. Results: Between December 2004 and March 2008, seven patients underwent occlusion of accessory ventriculopulmonary connections using an Amplatzer Septal Occluder (3), an Amplatzer Duct Occluder (3), or an Amplatzer Vascular Plug (1). Underlying single ventricle physiology was present in six of these patients. The site of occlusion was the right ventricle to pulmonary artery (Sano) conduit in two patients, the native main pulmonary artery in three patients, the pulmonary valve in one patient, and a left ventricle to pulmonary artery homograft in one patient with biventricular physiology. There were no complications associated with these procedures. Conclusions: Amplatzer occlusion devices provide a safe and effective means of eliminating accessory ventriculopulmonary connections in children who have undergone surgical palliation of congenital heart disease. © 2008 Wiley‐Liss, Inc.     

Five decades of the Fontan operation: A systematic review of international reports on outcomes after univentricular palliation

Almost fifty years after its first clinical application, the modified Fontan operation is among the most frequently performed procedures in congenital heart disease surgery in children today. The objective of this review is to systematically summarize the international evolution of outcomes in regard to morbidity and mortality of patients with Fontan palliation. All studies published over the past five decades with more than 100 Fontan patients included were screened. In eligible studies, information concerning preoperative patients' characteristics, Fontan modifications employed, early mortality, long‐term survival and frequency of relevant complications was extracted. Ultimately, thirty‐one studies published by the largest surgical centers with an overall number of 9390 patients were included in this review. The extracardiac total cavopulmonary anastomosis is the most frequently used Fontan modification. Hemodynamic data demonstrate a rigorous overall adherence to suggested Fontan selection criteria. The analysis showed a clear trend toward improved early and long‐term survival over the time period covered. Although inconsistently reported, severe complications such as arrhythmias, thromboembolic events and protein‐losing enteropathy as well as reoperations and reinterventions were frequent. In conclusion, patients palliated for complex univentricular heart malformations nowadays benefit from the experience and technical developments of the past decades and have a significantly improved long‐term prognosis. However, important issues concerning postoperative long‐term morbidity and mortality are still unsolved and clear intrinsic limitations of the Fontan circulation are becoming evident as the population of Fontan patients ages.     

Echocardiographic evaluation of univentricular physiology and cavopulmonary shunts

Since the surgical palliation of a univentricular congenital heart defect was first described in the early 1970s, thousands of lives were saved by this groundbreaking operation. Although initially described for the palliation of tricuspid valve atresia, the Fontan operation is now utilized for many other univentricular heart defects involving either hypoplastic right or left ventricles, and the number of babies who survive the surgery and the postsurgical hospitalization continues to grow. Echocardiography constitutes the mainstay follow‐up imaging modality for patients with a Fontan system. In order to obtain all the needed information from the echocardiographic studies, close familiarity with the native congenital heart defects, the build of the Fontan systems, and the potential long‐term complications is required. Detailed studies provide valuable information on the various components of the Fontan circulation such as the function of the single ventricle and the valves, flow in the Glenn and Fontan pathways, and possible sources of cyanosis and shunts. Three‐dimensional and transesophageal echocardiogram studies can assist in delineating the Fontan anatomy and to evaluate smaller intracardiac structures.     

Bidirectional superior cavopulmonary anastomosis: how young is too young?

OBJECTIVE--To define the lowest age at which the bidirectional superior cavopulmonary anastomosis can safely be used in infants with complex congenital heart defects. DESIGN--A retrospective analysis of clinical, echocardiographic, haemodynamic, and angiographic data in four consecutive patients undergoing bidirectional superior cavopulmonary anastomosis below the age of 2 months. PATIENTS--Between November 1990 and September 1993, four infants less than 8 weeks of age (3, 4, 6, and 7 weeks) underwent bidirectional superior cavopulmonary anastomosis as a primary palliation for complex heart disease. The indication for early surgical intervention was progression of cyanosis (n = 3) and high pulmonary blood flow causing heart failure (n = 1). In two infants with tricuspid atresia, surgery was performed through a right thoracotomy without the use of cardiopulmonary bypass. In one infant with double inlet left ventricle and discordant ventriculoarterial connection, cavopulmonary anastomosis was combined with an arterial switch procedure. The final infant had double inlet left ventricle with pulmonary atresia; the central pulmonary arteries were virtually discontinuous and each branch was supplied by a separate arterial duct. The central pulmonary arteries were reconstructed using the subaortic innominate vein. Temporary prostacyclin infusion was used in three patients in the immediate postoperative period. RESULTS--Early postoperative extubation (5, 7, and 48 h) was successful in three patients. The youngest child required ligation of the ductus arteriosus four days later because of severe upper body oedema. The postoperative course was complicated by prolonged effusions in two patients. All were alive and well 14-48 months postoperatively, with satisfactory systemic saturations (80-87%) and haemodynamic indices. CONCLUSIONS--This limited experience challenges the widely held belief that the bidirectional superior cavopulmonary anastomosis cannot be used as a primary palliation for complex heart disease in early infancy. A wider experience is required to determine the safety and indications for this approach.     

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.     

Atresia of the left atrioventricular valve with patency of the aorta: anatomico-functional analysis of 23 patients.

We analyzed the findings in 23 patients with atresia of the left atrioventricular valve and a patent aorta seen in the period from January 1980 to July 1989. Having divided the cases according to the anatomical findings, we made a subsequent analysis of the clinical and surgical results with the aim of establishing the management most likely to diminish risks, still high, in treatment of this complex anomaly. From the anatomical viewpoint, three variants were observed. In the first, made up of 15 cases, there was absence of the left atrioventricular connexion. The characteristic finding in the second group, with five cases, was an imperforate left atrioventricular valve in the setting of concordant atrioventricular connexions. The third group, of these cases, was dominated by the presence of isomerism of the atrial appendages, both appendages being of left morphology in one case, and of right morphology in the other two. Further anatomical variation was then found in each group. Nine of the 15 with absence of the left atrioventricular connexion had the right atrium connected to a dominant left ventricle in presence of a rudimentary and incomplete right ventricle associated with discordant ventriculo-arterial connexions, all of them being in usual atrial arrangement and three with pulmonary stenosis. The remaining six in this first variant had the right atrium connected to a dominant right ventricle. In the five patients with imperforate left atrioventricular valves, two had discordant and three had concordant ventriculo-arterial connexions. In the three cases with isomerism, two had absence of the left atrioventricular connexion, with a dominant right ventricle. The last patient had an imperforate left atrioventricular valve and a discordant ventriculo-arterial connexion. From the functional viewpoint, there were 14 patients (10 with absence of an atrioventricular connexion, four with imperforate atrioventricular valve) with congestive heart failure and nine patients (five from the first, one from second, and three from the third variant) with hypoxia. Long-term follow-up (median 16.4 months--varying from 1 to 41 months--in the group with congestion and 27.7 months--varying from 12 to 57 months--in those with hypoxia) showed favorable clinical evolution in 11 (91%). We conclude that an anatomico-functional division can point towards the most appropriate management in this complex anomaly.     

Long term negative pressure ventilation: Rescue for the failing fontan?

Current treatment strategies for single ventricle patients include non-intervention strategy, surgical palliation or primary transplantation. Surgical palliation includes a staged operative course culminating in the Fontan operation. With progress in surgical techniques, the survival has been improving. However, almost all of these Fontan patients will demonstrate pathophysiologic changes that ultimately constitute "Fontan failure physiology". This article reviews the pathophysiologic changes, current approach to management of these patients and proposes a novel way of reversing some of the pathophysiologic changes by utilization of negative pressure ventilation.     

Intermediate-term results of the Senning or Mustard procedures combined with the Rastelli operation for patients with discordant atrioventricular connections associated with discordant ventriculoarterial connections or double outlet right ventricle

BACKGROUND: In patients with discordant atrioventricular and ventriculoarterial connections, anatomic repair restores the morphologically left ventricle to its role in supporting the systemic circulation. In this study, we have evaluated the outcomes in the intermediate term for this complex surgical procedure. METHODS: Between December 1984 and October 2003, 4 patients underwent an atrial switch operation concomitantly with a Rastelli operation, and 2 patients underwent an atrial switch operation and a patch-plasty of the pulmonary outflow tract for anatomic repair at a mean age of 3.3 plus or minus 2.1 years. All patients had intracardiac rerouting, connecting the morphologically left ventricle to the aorta. RESULTS: There were no hospital deaths. In 5 patients, reoperation was needed, either for baffle complications, exchange of the conduit, repair of a residual ventricular septal defect, or relief of obstruction within the left ventricular outflow tract. Death occurred in 1 patient, from cardiac failure 6 months after correction. Mean follow-up time was 6.5 plus or minus 6.4 years, with a range from 6 months to 17 years. At follow-up, 1 patient presented with moderate tricuspid insufficiency, and 1 patient with mild obstruction of the pulmonary venous pathway. The remaining 3 patients showed good left and right ventricular function, and no, or mild tricuspid and mitral insufficiency. CONCLUSIONS: Anatomic repair can be performed with low hospital mortality. Restoration of the morphologically left ventricle into the systemic circulation in patients with discordant atrioventricular and ventriculoarterial connections is a demanding approach, associated with various reoperations over time. Despite this, the approach seems to be an appropriate solution for selected patients, since the majority of the patients show good left and right ventricular function, and no, or mild tricuspid and mitral insufficiency up to 17 years after correction.     

Fontan Circulation: Success or Failure?

Fontan surgery represents a milestone in the evolution of congenital heart disease management. It achieved the seemingly improbable (ie, restoration of a noncyanotic state by entirely bypassing the subpulmonary ventricle). In so doing, it has allowed a generation of children who may have otherwise succumbed to their severe congenital heart defect to survive to adulthood. Perfect univentricular physiology is, however, an elusive goal. The Fontan circulation inherently represents a hemodynamic compromise that results in a catalog of potential multiorgan complications. In this review, we explore current knowledge regarding pathophysiology of the failing Fontan, its varied clinical manifestations, and potential therapeutic options. Failure of the Fontan circulation is broadly divided into 3 overlapping categories: ventricular dysfunction, systemic complications of Fontan physiology, and chronic Fontan failure. As long as the Fontan operation continues to serve as the paradigm of care for patients with univentricular hearts, efforts must be directed toward supporting this dynamic circulation that progressively declines in efficiency with age. Continued research in therapies is needed for univentricular dysfunction and systemic complications of Fontan palliation, including potential uses of mechanical support as a bridge to transplantation or as a neosubpulmonary ventricle. Fontan patients remain a major challenge to the medical and surgical community as a whole. Multicentre and multidisciplinary efforts to improve the density and depth of experiences might lead to a better appreciation for, and management of, Fontan failure and its ramifications.     

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.