Does the External Pericardial Lateral Tunnel Fontan Pathway Enlarge to Accommodate Somatic Growth? A Preliminary Analysis.

Objectives: An ideal Fontan pathway should be capable of adapting to changes in circulatory demands. The external pericardial lateral tunnel Fontan (EPLTF) is constructed of viable, autologous tissue and may be capable of changing in size. We investigated the ability of the EPLTF to enlarge with increasing physiologic demands of somatic growth. Methods: Retrospective review of echocardiographic images for patients with a minimum of five years follow-up after EPLTF. Serial echocardiographic measurements of the EPLTF pathway were obtained at three distinct locations: the inferior vena cava junction with the EPLTF, midsection of the EPLTF, and cross-sectional area of the EPLTF visualized in a four-chamber view. Body surface area (BSA) was calculated at the time of each echocardiographic measurement. Changes in echocardiographic measurements over time were analyzed and compared with changes in BSA. Results: A total of 332 echocardiographic studies from 38 patients were reviewed. Significant enlargement of the EPLTF pathway is observed at the inferior vena caval junction (P < .001), midsection (P < .01), and cross-sectional area (P < .001). Repeated measures correlation between pathway measurements and BSA is highly significant (P < .001). Conclusions: The EPLTF pathway enlarges over time in correlation with increasing BSA. Further research is needed to define ideal pathway size, differentiate normal physiologic growth from pathologic enlargement, and correlate changes with clinical outcomes.

Total arch replacement using frozen elephant trunk and repair of bilateral subclavian artery aneurysms in a patient with type 2 Loeys-Dietz syndrome.

Loeys-Dietz syndrome (LDS) is a rare connective tissue disorder. Vessel tortuosity and aneurysms throughout the vasculature are unique to LDS. Aortic root enlargement is ubiquitous, with most patients undergoing root replacement at some point in their lifetime. Multiple vascular procedures are required to prolong life expectancy. We describe a staged hybrid approach to a 17-year-old patient with LDS presenting with ascending aorta, arch, and bilateral subclavian artery aneurysms and prominent tortuosity. Transposition of the left vertebral and subclavian arteries onto the common carotid artery was performed. Total aortic arch replacement with frozen elephant trunk extension into the descending thoracic aorta was performed as a second stage. Bilateral subclavian artery aneurysms were excluded with the use of a four-branched graft.

Giant Aneurysm of Left Main Coronary Artery in a Patient With Prior Operation for 4-Valve Endocarditis.

Left main coronary artery aneurysm is an unusual complication of infective endocarditis. Although this type of aneurysm is often asymptomatic, rupture and thrombus formation that result in myocardial infarction are known complications; therefore, prompt recognition and surgical intervention are warranted. This report describes a patient who presented with a giant left main coronary artery aneurysm 3.5 years after being treated for 4-valve endocarditis. The management and technical aspects of this challenging case are discussed here.

Impact of transplant center volume on donor heart offer utilization rates in the United States.

BACKGROUND: We tested the hypothesis that transplant centers (TCs) with higher volumes have higher donor heart (DH) offer utilization rates. METHODS: Using the Annual Data reports of the US Organ Procurement and Transplantation Network and the Scientific Registry of Transplant Recipients (SRTR) we reviewed all adult heart transplant offers between July 1, 2016 and June 29, 2019. Unadjusted donor offer utilization rates and observed to expected (O/E) DH utilization ratios adjusted using the SRTR model were calculated for each TC for all DH offers and for the following sub-categories: DH with left ventricular ejection fraction <60%, DH >40 years, DH >500 miles from TC, "hard-to-place hearts" (defined as those offered to >50 TCs) and DH designated as increased infectious risk. Univariable linear regression was used to identify a relationship between average yearly center volume and DH utilization. RESULTS: During the study 118,841 total offers were made to 107 TCs and 8300 transplants were performed. The unadjusted utilization rate was not associated with TC volume for all donor offers (p = .517). However, among all subcategories other than DH >40 years, the unadjusted DH utilization rate was associated with TC volume (p < .05). In addition, using the adjusted SRTR O/E ratio, there was a significant impact of TC volume on utilization rate for all donor offers (for an increase TC volume of 10 transplants/year coefficient = 0.095, 95% confidence interval: 0.037-0.151, p = .001). This relationship persisted with an identifiable change for each of the subcategories (p ≤ .001). CONCLUSIONS: TC volume is significantly correlated to DH offer utilization rate.

Likelihood of Aortic Valve Preservation During Repair of Acute Type A Aortic Dissection.

BACKGROUND: We sought risk factors for replacement of the aortic valve with or without the root (AVR/root) in the setting of acute type A aortic dissection (ATAD) repair. METHODS: All ATAD repairs at our institution from January 2005 to June 2018 were reviewed. Baseline characteristics were recorded. For patients with aortic valve preservation, we documented the degree of aortic insufficiency (AI) postoperatively and on subsequent echocardiograms when available. Logistic regression was used to determine the association between preoperative characteristics and the odds ratio of AVR/root. RESULTS: 206 patients underwent repair of ATAD. Thirty-four were excluded for no documented AI grading. Forty-six underwent AVR/root during repair of the ATAD (including 40 root replacements). Of 126 that did not undergo AVR/root, 42 (33.33%) had follow-up echocardiograms at a median of 68 months postoperatively, 2 required reintervention for valve insufficiency. An increase in the degree of AI, bicuspid valve morphology, size of the aortic root, and connective tissue disorder was significantly associated with increased risk of AVR/root. Of 130 patients without connective tissue disorder, bicuspid aortic valve, aortic root aneurysm or intimal root tear, the rate of valve preservation was 65/65 (100%), 25/29 (86.2%), and 22/40 (55%) for those presenting with mild, moderate, and severe AI, respectively. CONCLUSION: The degree of preoperative AI, bicuspid valve morphology, size of the aortic root, and connective tissue disorder significantly correlate with the failure of aortic valve preservation in patients with ATAD. The vast majority of tricuspid valves in patients without connective tissue disorder or aortic root pathology can be salvaged.

Likelihood of Aortic Valve Preservation During Repair of Acute Type A Aortic Dissection.

BACKGROUND: We sought risk factors for replacement of the aortic valve with or without the root (AVR/root) in the setting of acute type A aortic dissection (ATAD) repair. METHODS: All ATAD repairs at our institution from January 2005 to June 2018 were reviewed. Baseline characteristics were recorded. For patients with aortic valve preservation we documented the degree of aortic insufficiency (AI) postoperatively and on subsequent echocardiograms when available. Logistic regression was used to determine the association between preoperative characteristics and the odds ratio of AVR/root. RESULTS: A total of 206 patients underwent repair of ATAD. Thirty-four were excluded for no documented AI grading. Forty-six underwent AVR/root during repair of the ATAD (including 40 root replacements). Of 126 that did not undergo AVR/root, 42 (33.33%) had follow-up echocardiograms at a median of 68 months postoperatively and 2 required reintervention for valve insufficiency. Increase in degree of AI, bicuspid valve morphology, size of the aortic root, and connective tissue disorder were significantly associated with increased risk of AVR/root. Of 130 patients without connective tissue disorder, bicuspid aortic valve, aortic root aneurysm, or intimal root tear, the rate of valve preservation was 65/65 (100%), 25/29 (86.2%), and 22/40 (55%) for those presenting with mild, moderate, and severe AI, respectively. DISCUSSION: Degree of preoperative AI, bicuspid valve morphology, size of the aortic root, and connective tissue disorder significantly correlate with failure of aortic valve preservation in patients with ATAD. The vast majority of tricuspid valves in patients without connective tissue disorder or aortic root pathology can be salvaged.

Increased sensitivity to ischemic interval of donor hearts with diminished left ventricular function.

BACKGROUND: Previous studies have demonstrated that carefully selected donor hearts (DHs) with poor left ventricular ejection fraction (EF) may be transplanted with long-term survival equivalent to hearts with normal function. The purpose of this study is to facilitate their selection. METHODS: Using the United Network for Organ Sharing database, we reviewed all adult heart transplants between January 2000 and March 2016. Regression models were developed to estimate hazard ratios with 95% confidence intervals of post-transplant 1-year mortality and failure of EF to recover at 1 year for DHs with EF ≥50%, EF 40%-49.9%, and EF 30%-30.9%. RESULTS: During the study period, 31,979 DHs were transplanted. Compared with DHs with left ventricular ejection fraction ≥50%, DHs with reduced EF were younger and had slightly lower body mass index. There were no differences in the mechanism of death between groups and no differences in recipient characteristics, except for a higher incidence of African American recipients of hearts with an EF of 40%-49.9%. Of the variables analyzed, only a 1-hour increase in ischemia time had different hazard ratios for 1-year mortality between groups, with increasing hazard as EF diminished. It was also the only variable that predicted failure of recovery of normal EF and that was in the lowest EF group. CONCLUSIONS: The impact of DH traits associated with adverse outcomes after heart transplantation that we studied are similar between DHs with EF <50% and those with EF ≥50%. However, limiting ischemic time may be even more important for DHs with diminished left ventricular function, particularly at the low end of the EF spectrum.

Primary Transplantation for Congenital Heart Disease in the Neonatal Period: Long-term Outcomes.

BACKGROUND: Primary transplantation was developed in the 1980s as an alternative therapy to palliative reconstruction of uncorrectable congenital heart disease. Although transplantation achieved more favorable results, its utilization has been limited by the availability of donor organs. This review examines the long-term outcomes of heart transplantation in neonates at our institution. METHODS: The institutional pediatric heart transplant database was queried for all neonatal heart transplants performed between 1985 and 2017. Follow-up was obtained from medical records and an annually administered questionnaire. Overall survival and time to development of complications were estimated using the Kaplan Meier method. Univariate and multivariate analyses were performed to identify independent predictors of survival. RESULTS: Heart transplantation was performed in 104 neonates. Median age was 17 days. Hypoplastic left heart syndrome (classic or variant) was the primary diagnosis in 77.8% of patients. Survival at 10 years and 25 years was 73.9% and 55.8%, respectively. At 20 years, freedom from allograft vasculopathy and lymphoproliferative disease was 72.0% and 81.9%, respectively. Freedom from re-transplantation was 81.4% at 20 years. Eight patients (7.6%) developed end-stage renal disease. By multivariate analysis, lower glomerular filtration rate and allograft vasculopathy were the only significant predictors of death. CONCLUSIONS: Neonatal heart transplantation remains a durable therapy with very acceptable long-term survival. Children transplanted in the newborn period have the potential to reach adulthood with minimal need for reintervention.

Interaction Between Ischemic Time and Donor Age on Adult Heart Transplant Outcomes in the Modern Era.

BACKGROUND: We examined the effect of cold ischemic interval on modern outcomes to determine whether advances in patient management have made an impact. METHODS: Using the United Network of Organ Sharing database, we reviewed adult heart transplants between January 2000 and March 2016. We divided donor age into terciles: younger than 18 years, 18 to 33 years, and 34 years and older. Within each tercile, transplants were divided by cold ischemic interval of less than 4 hours, 4 to 6 hours, and more than 6 hours. Survival curves were compared between cold ischemic interval categories within each tercile. Covariate-adjusted and donor age-stratified Cox proportional hazards regression models were used to estimate overall mortality and graft failure hazards ratios. RESULTS: Of 29,192 transplants, no significant differences between cold ischemic interval groups in survival or graft failure were apparent in the group aged younger than 18. For donors older than 18, significant differences were found for survival and graft failure with cold ischemic interval exceeding 4 hours in both univariate and multivariate analysis, and survival functions at different ischemic intervals continued to diverge beyond 1 year. The interaction effect between donor age and cold ischemic interval on overall mortality was not significant when analyzed as continuous variables, however younger donor age appeared to attenuate increase in overall mortality with longer cold ischemic intervals. CONCLUSIONS: Despite advances in perioperative management during the past 30 years, for donors older than 18 years, cold ischemic interval exceeding 4 hours is associated with gradual but significantly diminished survival that persists well beyond the perioperative period. Comparison to historical data suggests that advances in management have somewhat attenuated the hazard associated with longer ischemic times.

Long-term transplant outcomes of donor hearts with left ventricular dysfunction.

OBJECTIVE: Despite small single-center reports demonstrating acceptable outcomes using donor hearts with left ventricular dysfunction, 19% of potential donor hearts are currently unused exclusively because of left ventricular dysfunction. We investigated modern long-term survival of transplanted donor hearts with left ventricular dysfunction using a large, diverse cohort. METHODS: Using the United Network for Organ Sharing database, we reviewed all adult heart transplants between January 2000 and March 2016. Baseline and postoperative characteristics and Kaplan-Meier survival curves were compared. A covariates-adjusted Cox regression model was developed to estimate post-transplant mortality. To address observed variation in patient profile across donor ejection fraction, a propensity score was built using Cox predictors as covariates in a generalized multiple linear regression model. All the variables in the original Cox model were included. For each recipient, a predicted donor ejection fraction was generated and exported as a new balancing score that was used in a subsequent Cox model. Cubic spline analysis suggested that at most 3 and perhaps no ejection fraction categories were appropriate. Therefore, in 1 Cox model we added donor ejection fraction as a grouped variable (using the spline-directed categories) and in the other as a continuous variable. RESULTS: A total of 31,712 donor hearts were transplanted during the study period. A total of 742 donor hearts were excluded for no recorded left ventricular ejection fraction, and 20 donor hearts were excluded for left ventricular ejection fraction less than 20%. Donor hearts with reduced left ventricular ejection fraction were from younger donors, more commonly male donors, and donors with lower body mass index than normal donor hearts. Recipients of donor hearts with reduced left ventricular ejection fraction were more likely to be on mechanical ventilation. Kaplan-Meier curves revealed no significant differences in recipient survival up to 15 years of follow-up (P = .694 log-rank test). Cox regression analysis showed that after adjustment for propensity variation, transplant year, and region, ejection fraction had no statistically significant impact on mortality when analyzed as a categoric or continuous variable. Left ventricular ejection fraction at approximately 1 year after transplantation was normal for all groups. CONCLUSIONS: Carefully selected donor hearts with even markedly diminished left ventricular ejection fraction can be transplanted with long-term survival equivalent to normal donor hearts and therefore should not be excluded from consideration on the basis of depressed left ventricular ejection fraction alone. Functional recovery of even the most impaired donor hearts in this study suggests that studies of left ventricular function in the setting of brain death should be interpreted cautiously.

Subaortic Stenosis Resection in Children: Emphasis on Recurrence and the Fate of the Aortic Valve.

BACKGROUND: Recurrence after surgical resection of discrete subvalvar aortic stenosis in children often requires repeat operation. Risk factors for recurrence are poorly understood. We sought to determine potential risk factors for recurrence and postoperative comorbidities in the long term. METHODS: Retrospective chart review was performed on all pediatric patients who underwent surgical resection of discrete subaortic stenosis at our institution. Demographics, perioperative findings, and clinical data were analyzed for predisposing factors. RESULTS: From 1991 to 2015, a total of 104 patients underwent primary surgical resection of discrete subaortic stenosis. There were no postoperative deaths. Three (2.9%) patients required pacemaker implantation. Nine (8.4%) patients required repeat resection for recurrence of subaortic membrane over a median follow-up of 8.5 years (interquartile range: 5.9-13.5 years). Actuarial freedom from repeat resection was 100%, 94%, and 82% at one, five, and ten years, respectively. Repeat resection occurred more frequently in patients with genetic disease (37.5% vs 10.7%; P = .033) and preoperative mitral regurgitation (MR; 25% vs 1.2%; P < .001). Postoperative aortic insufficiency (AI) that was moderate or worse was associated with older age at the time of first resection (relative risk [RR]: 1.54, P < .05), moderate or severe preoperative AI (RR: 1.84, P = .002), and repeat resection of subaortic stenosis (RR: 1.90, P < .001). CONCLUSION: The majority of children who undergo surgical resection of subaortic stenosis will not experience recurrence in childhood and those who do require repeat resection may have a higher incidence of genetic disease and preoperative MR. Postoperative AI is associated with repeat resection, older age at the time of surgery, and degree of preoperative AI.

Role of Negative Pressure Wound Care and Hyperbaric Oxygen Therapy for Sternal Wound Infections After Pediatric Cardiac Surgery.

BACKGROUND: Sternal wound infections after pediatric cardiac surgery are uncommon but can be morbid. METHODS: We describe an institutional protocol for complicated sternal wounds utilizing hyperbaric oxygen therapy (HBO) and negative pressure wound therapy (NPWT). PARTICIPANTS: A retrospective chart review (2001-2013) of 4,028 pediatric cardiac operations in 3,264 patients less than 18 years of age. RESULTS: Fifty-three patients (1.62%; 53/3,264) were diagnosed with clinical sternal wound infections. There were 27 (50.9%) males and 26 (49.1%) females. Thirty-seven (69.8%) patients received antibiotics and/or debridement; sixteen (30.2%) patients had more complicated infections requiring NPWT and/or HBO therapy. The time to heal for wounds treated with HBO and HBO + NPWT was a mean of 43.75 (±24.27) days (range: 21-98 days; median: 35 days). Among all infected patients, the time from diagnosis of the infection to resolution of the infection for all survivors was 7 to 98 days (mean: 26.41 days; median: 21 days). Forty-eight (90.6%) patients completely healed their wounds, and 45 (84.9%) patients are currently alive. Thirty-eight patients had a cyanotic cardiac diagnosis and 15 had an acyanotic cardiac diagnosis. The most common bacteria isolated from the blood or wound cultures was Staphylococcus aureus. Six of 53 patients died. Causes of death are as follows: three from respiratory failure, one from sepsis, one from hypoxic ischemic encephalopathy, and one from exsanguination leading to cardiac arrest Conclusions: Complicated sternal wound infections after pediatric cardiac surgery refractory to antibiotic therapy and/or debridement can be successfully treated with NPWT and/or HBO therapy.

Collaborative Management of Missile Injury to Right Ventricle and Subsequent Pulmonary Embolization.

Pulmonary embolization of a missile is a rare phenomenon. Localization after embolization can be confounding, and there is no consensus on management. This report describes a case of a gunshot wound to the chest with preoperative and initial intraoperative imaging localizing the bullet to the right ventricle but a negative intraoperative exploration of the right-sided cardiac chambers. Intraoperative fluoroscopy allowed for immediate localization of the bullet to the hilum of the left lung, with subsequent endovascular retrieval.

Effect of left ventricular dysfunction on utilization of donor hearts.

BACKGROUND: In this study we investigated modern, non-utilization rates of potential cardiac donors with left ventricular dysfunction (LVD) to clarify this phenomenon's magnitude and the impact of recent studies suggesting these organs can be safely transplanted. METHODS: Using the United Network for Organ Sharing transplant database, we reviewed all donors evaluated between January 1, 2007 and June 30, 2014. Exclusion criteria included lack of consent and age <13 or >59 years. The number of hearts not transplanted due to non-cardiac causes, structural disease, "other" (previous cardiac surgery, donation after cardiac death, etc.) and isolated LVD was determined and a covariates-adjusted Poisson regression model with robust standard errors was developed to estimate non-utilization relative risk (RR) with 95% confidence interval (CI) for LVD. Heart disposition for potential donor hearts was determined separately for 2 previous eras (1990 to 1999 and 2000 to 2006), and trends were evaluated. RESULTS: There were 60,789 donors assessed. Of the 44,829 organs meeting the inclusion criteria, 15,654 (34.92%) were transplanted and 29,175 (65.08%) were not. Of the non-utilized hearts, 15,512 (34.60%) were declined for non-cardiac reasons, 1,051 (2.34%) for structural disease, 4,073 (9.09%) for "other" and 8,539 (19.05%) exclusively for LVD. Of this last category, 4,950 (11.04%) lacked documented evidence of LVD. Covariates-adjusted RR for non-utilization showed that, for every 10% increase in LV ejection fraction, the risk of non-utilization decreased by 20% (RR = 0.80, 95% CI 0.79 to 0.81). Analysis of era-effect demonstrated significantly decreased overall utilization of donor hearts, with increases in the number of hearts not transplanted across all categories over time (p < 0.001). CONCLUSIONS: Roughly 20% of potential cardiac donors are excluded due to LVD. This figure has not been impacted by recent studies indicating that these hearts may be used safely. More complete data are required to understand why 11.04% of hearts that met inclusion criteria were refused for "poor function" without documented evidence.

Heart transplantation in children for end-stage congenital heart disease.

Heart transplantation (HT) as primary therapy for children with congenital heart disease (CHD) has become unusual. With improved early results of reconstructive surgery, the population of children and adults surviving with CHD is expanding. End-stage CHD related to myocardial dysfunction or circulation failure after prior surgery is becoming more common as an indication for HT. This heterogeneous group of CHD recipients referred for HT presents unique decision-making, technical, and physiologic challenges. Historically, a diagnosis of CHD has been a major risk factor for early mortality after HT. Rescue HT, especially in the setting of failing Fontan physiology, has the worst outcome. Early referral (before end-organ damage), proper selection, and optimization of recipients, as well as meticulous intra- and postoperative management are crucial to improving early outcomes of HT in this population. Beyond the early post-HT period, children with end-stage CHD experience long-term survival comparable to most other non-CHD recipients.

Left thoracotomy: an ideal approach for mitral valve replacement in patient with severe chest wall deformity.

We report a 65-year-old woman with chronic diastolic congestive heart failure, pulmonary hypertension, and severe mitral valve regurgitation. She was not a candidate for percutaneous mitral valve repair and was turned down for an open heart operation by 2 institutions based on her severe pectus excavatum deformity. A left posterior lateral thoracotomy approach provided excellent exposure for central cannulation and replacement of the mitral valve.

Blake drains: a novel method of chest drainage after extracardiac fontan operation with autologous pericardium.

BACKGROUND: Pleural effusions after Fontan palliation remain a cause of increased length of stay, cost, and morbidity. We report our experience with Blake drains (BDs) and the outpatient pediatric pleural drain pathway after Fontan operation. METHODS: A retrospective chart review was performed on all patients who underwent extracardiac lateral tunnel (ECLT) Fontan operation with pedicled autologous pericardium. Patients with prolonged pleural drainage were analyzed for predisposing factors. RESULTS: From March 1995 to December 2009, 162 patients (92 male, 56.8%) underwent ECLT Fontan operation. The median age at the time of Fontan operation was 30.9 months; the median weight was 12.9 kg. The median hospital stay was 4 days, and the median pleural drain requirement was 13 days. Prolonged pleural effusions occurred in 59 patients (36.4%), with prolonged cardiopulmonary bypass time identified as the only significant risk factor (p=0.04). Sixty patients (37%) were readmitted within 30 days of operation, with effusion requiring additional pleural drainage (n=41, 68.3%), infection (n=8, 13.3%), or a combination of the two (n=3, 5%) being the most common reason. There were two early deaths, neither of which was associated with BD malfunction. The BD clinical pathway for ECLT Fontan operation reduced our institutional cost to about $38,000 per patient, which represents a significant savings compared with traditional management with extended hospital stay after Fontan operation. CONCLUSIONS: Silicone BDs are safe and effective after ECLT Fontan operation. Hospital length of stay and cost can be significantly decreased when these drains are used with appropriate family involvement and close outpatient surveillance.

Late outcome after repair of mitral valve rupture during balloon atrial septostomy in a neonate.

Balloon atrial septostomy is ordinarily a safe palliative procedure for cyanotic congenital heart disease; however, if echocardiographic guidance is unavailable and fluoroscopy is used, distortions in the cardiac anatomy can invalidate the usual landmarks. Herein, we report iatrogenic mitral papillary muscle rupture during balloon atrial septostomy in a 4-day-old male neonate with total anomalous connection of the pulmonary veins. The anomalous connection and severe mitral regurgitation were emergently corrected, and the patient grew and developed normally. At age 24 years, he had only mild residual mitral regurgitation and was in New York Heart Association functional class I.In addition to describing the surgical treatment and positive late outcome of a rare complication, we highlight the importance of accurately evaluating balloon catheter location during atrial septostomy, especially in patients with a small left atrium.

In situ pericardial extracardiac lateral tunnel Fontan operation: fifteen-year experience.

BACKGROUND: The study purpose is to evaluate the long-term outcome of the in situ pericardial extracardiac lateral tunnel Fontan operation. METHODS: From June 1994 to August 2009, 160 patients with single ventricle (boys, n = 96, 60%, median age = 39 months, mean weight 15.5 kg) underwent the pedicled pericardial extracardiac lateral tunnel operation. Patients' charts were reviewed for perioperative and long-term follow-up data, outcome, and mortality. The potential growth of these tunnels was evaluated. RESULTS: The main diagnoses included tricuspid atresia (n = 44, 27%); double-outlet right ventricle (n = 29, 18%), and hypoplastic left heart syndrome (n = 26, 16%). The mean follow-up was 6.5 ± 3.7 years (range: 0.1-15 years). There were 2 (1.3%) operative and 6 (3.7%) late deaths. Actuarial survival at 14 years was 93%. Early complications included prolonged effusions (n = 35, 22%), chylothorax (n = 5, 3.1%), readmissions (n = 35, 22%), cerebrovascular accidents (n = 8, 5%), contralateral phrenic nerve palsy (n = 1, 0.8%), and transient arrhythmias (n = 5, 3.1%). No pacemaker was needed. Late complications included tunnel stenosis (n = 3, 1.8%) managed with balloon dilatation and stenting in 2 patients and surgical revision in 1; tunnel thrombosis (n = 2, 1.2%) causing death in both patients; and protein losing-enteropathy (n = 4, 2.5%). Follow-up echocardiography of 10 patients showed laminar flow, no turbulence/gradient at the inferior vena cava and mid-tunnel levels. The diameter indexed to body surface area showed growth, reduction, or no change depending on flow demands. CONCLUSIONS: The construction of the extracardiac lateral tunnel Fontan conduit using viable pedicled pericardium is a relatively simple, durable, and safe operation. Long-term follow-up confirms low morbidity and mortality. Fenestration is unnecessary in most patients. This viable tunnel adapts to physiologic flow demands.