Abstract Objective Existing studies of patients palliated with the Fontan operation are limited by heterogeneous patient populations and incomplete follow-up. This study aimed to describe long-term ...post-Fontan survival in a modern patient cohort. Methods All 773 patients who underwent a first Fontan operation at our institution between 1992 and 2009 were reviewed. The primary outcome was the composite endpoint of Fontan takedown, heart transplantation, or death before 2013. Results Follow-up rate was 99.2%. Survival with intact Fontan circulation was 94% at 1 year (95% confidence interval 95% CI, 92%-95%), 90% at 10 years (95% CI, 88%-92%), 85% at 15 years (95% CI, 82%-88%), and 74% at 20 years (95% CI, 67%-80%). Distinct risk factors were identified for early (≤1 year) and late composite outcomes. Independent risk factors for early outcome included prolonged pleural drainage (hazard ratio HR, 4.4; P < .001), intensive care unit stay >1 week (HR, 2.4; P < .001), Fontan before 1997 (HR, 3.3; P < .001), preoperative atrioventricular valve regurgitation (HR, 2.0; P < .001), and longer crossclamp time (HR, 1.3 per 10 minutes; P < .001). Late outcome was predicted by atrioventricular valve regurgitation prior to Fontan (HR, 2.0; P ≤ .001), and post-Fontan ICU stay >1 week (HR, 2.4; P < .001). Conclusions Long-term mortality after Fontan operation remains substantial. Risk factors for death or loss of Fontan circulation differ between the early and late postoperative periods. Long-term survival has not improved appreciably over the last decade, suggesting that alternatives to the Fontan are warranted.
Objective Small head circumferences and white matter injury in the form of periventricular leukomalacia have been observed in populations of infants with severe forms of congenital heart defects. ...This study tests the hypothesis that congenital heart defects delay in utero structural brain development. Methods Full-term infants with hypoplastic left heart syndrome or transposition of the great arteries were prospectively evaluated with preoperative brain magnetic resonance imaging. Patients with independent risk factors for abnormal brain development (shock, end-organ injury, or intrauterine growth retardation) were excluded. Outcome measures included head circumferences and the total maturation score on magnetic resonance imaging. Total maturation score is a previously validated semiquantitative anatomic scoring system used to assess whole brain maturity. The total maturation score evaluates 4 parameters of maturity: (1) myelination, (2) cortical infolding, (3) involution of glial cell migration bands, and (4) presence of germinal matrix tissue. Results The study cohort included 29 neonates with hypoplastic left heart syndrome and 13 neonates with transposition of the great arteries at a mean gestational age of 38.9 ± 1.1 weeks. Mean head circumference was 1 standard deviation below normal. The mean total maturation score for the cohort was 10.15 ± 0.94, significantly lower than reported normative data in infants without congenital heart defects, corresponding to a delay of 1 month in structural brain development. Conclusion Before surgery, term infants with hypoplastic left heart syndrome and transposition of the great arteries have brains that are smaller and structurally less mature than expected. This delay in brain development may foster susceptibility to periventricular leukomalacia in the preoperative, intraoperative, and postoperative periods.
Abstract Although survival after the Fontan operation has improved, little is known about the burden of major medical morbidities associated with the modern total cavopulmonary connection (TCPC). A ...total of 773 consecutive patients who underwent a first Fontan operation at our institution between 1992 and 2009 were retrospectively reviewed. All subjects underwent TCPC (53% lateral tunnel, 47% extracardiac conduit). Median length of follow-up was 5.3 years (IQR 1.4-11.2), and 30% had follow-up >10 years. Freedom from a composite medical morbidity outcome (protein-losing enteropathy, plastic bronchitis, serious thromboembolic event, or tachyarrhythmia) was 47% at 20 years (95% CI 38-55%). Independent risk factors for morbidity included pre-Fontan atrioventricular valve regurgitation (HR 1.7, 95% CI 1.2-2.4; p=0.001), pleural drainage > 14 days (HR 1.5, 95% CI 1.01-2.2, p=0.04), and longer cross-clamp time (HR 1.2 per 10 minutes, 95% CI 1.06-1.3; p=0.004) at the time of TCPC. Surgical era, Fontan type, and ventricular morphology were not associated with the composite outcome. Presence of Fontan-associated morbidity was associated with a 36-fold increase in the risk of subsequent Fontan takedown, heart transplantation, or death (95% CI 17– 76, p<0.001). For patients without any component of the composite outcome, freedom from Fontan failure was 98% at 20 years (95% CI 96-99%). Medical morbidities after TCPC are common and significantly reduce the longevity of the Fontan circulation. However, for those patients who remain free from the composite morbidity outcome, 20-year survival with intact Fontan circulation is encouraging.
Typical left ventricular assist devices are often ineffective for the failing Fontan circulation. We report the first successful use of a total artificial heart as a bridge to transplant in a patient ...who had previously undergone a Fontan operation.
Objective Hypoxic-ischemic white mater brain injury commonly occurs in neonates with hypoplastic left heart syndrome (HLHS). Approximately one half of HLHS survivors will exhibit neurobehavioral ...symptoms believed to be associated with this injury, although the exact timing of the injury is unknown. Methods Neonates with HLHS were recruited for pre- and postoperative monitoring of cerebral oxygen saturation, cerebral oxygen extraction fraction, and cerebral blood flow using 2 noninvasive optical-based techniques: diffuse optical spectroscopy and diffuse correlation spectroscopy. Anatomic magnetic resonance imaging was performed before and approximately 1 week after surgery to quantify the extent and timing of the acquired white matter injury. The risk factors for developing new or worsened white matter injury were assessed using uni- and multivariate logistic regression. Results A total of 37 neonates with HLHS were studied. On univariate analysis, neonates who developed a large volume of new, or worsened, postoperative white matter injury had a significantly longer time to surgery ( P = .0003). In a multivariate model, a longer time between birth and surgery, delayed sternal closure, and greater preoperative cerebral blood flow were predictors of postoperative white matter injury. Additionally, a longer time to surgery and greater preoperative cerebral blood flow on the morning of surgery correlated with lower cerebral oxygen saturation ( P = .03 and P = .05, respectively) and greater oxygen extraction fraction ( P = .05 for both). Conclusions A longer time to surgery was associated with new postoperative white matter injury in otherwise healthy neonates with HLHS. The results suggest that earlier Norwood palliation might decrease the likelihood of acquiring postoperative white matter injury.
Background Circulatory failure necessitating cardiac transplantation will ultimately develop in many patients with functional single-ventricle physiology. Interest in the use of mechanical ...circulatory support (MCS) in this population is growing. Methods This was a retrospective case series of patients with functional single-ventricle physiology who underwent MCS with a ventricular assist device or a total artificial heart as a bridge to cardiac transplantation between January 2006 and December 2014. Baseline demographics, intraoperative data, postoperative complications, and outcome data were collected from the medical record. Results MCS was used in 5 patients: HeartWare ventricular assist device (HeartWare International, Framingham, MA) in 1 patient, SynCardia total artificial heart (SynCardia Systems, Tucson, AZ) in 1, Thoratec Paracorporeal ventricular assist device (Thoratec Corp, Pleasanton, CA) in 1, and the Berlin Heart EXCOR (Berlin Heart Inc, The Woodlands, TX) in 2. The mean age at MCS was 12 ± 8 years. There were 2 early deaths at 12 and 28 days after MCS: 1 patient died of multiorgan system failure and 1 of neurologic injury. Overall, neurologic complications occurred in 3 patients (60%), and 1 patient (20%) required renal replacement therapy. Three patients (60%) underwent successful cardiac transplantation. The median time on the waiting list was 59 days (interquartile range, 18 to 126 days), and the median duration of MCS was 60 days (interquartile range, 28 to 93 days). At the time of transplant, all 3 patients were ambulatory, without the need for mechanical ventilation, and end-organ dysfunction had resolved. The 3 patients who received transplants were discharged from the hospital and were alive at an average follow-up of 9 ± 14 months. Conclusions MCS can be successfully used as a bridge to transplantation in patients with a failing single-ventricle circulation. Use of MCS can allow for resolution of end-organ dysfunction and rehabilitation, leading to improved outcomes in this difficult population.
Background Despite recent improvements in survival of patients with hypoplastic left heart syndrome, those with severe atrial level restriction continue to face higher rates of mortality. We sought ...to assess the impact of prenatal diagnosis and immediate intervention on the outcome of this high-risk population. Methods We performed a retrospective review of patients with hypoplastic left heart syndrome and restriction at the atrial level born between 1997 and 2006. Patients with an intact atrial septum or an interatrial communication less than 2 mm by color Doppler flow mapping on initial postnatal study, or evidence of obstruction to left atrial egress on fetal echocardiogram, were included. Patients were subdivided into two groups based on severity of obstruction. Results Thirty-eight patients met inclusion criteria; 16 had the most severe form of anatomic obstruction (group 1), and 22 had a lesser degree of obstruction (group 2). Thirty-day cumulative survival for all was 68%: 38% for group 1 and 94% for group 2 ( p = 0.001). Twenty-six of the 38 patients (68%) were prenatally diagnosed. Prenatal diagnosis did not improve initial hospital survival or cumulative survival for either group. Eight patients had progression of atrial level restriction from the first fetal study to the first postnatal study. Seven patients prenatally diagnosed with severe restriction underwent planned cesarean section followed by immediate surgical or catheter-based intervention, with only 2 survivors (28%). Conclusions Hypoplastic left heart syndrome with an intact atrial septum is a highly lethal condition despite prenatal diagnosis and immediate intervention at birth. Fetal intervention should be considered for these high-risk fetuses.
Background Preoperative brain injury is common in neonates with complex congenital heart disease. Increasing evidence suggests a complex interaction of prenatal and postnatal risk factors for ...development of brain white matter injury, called periventricular leukomalacia (PVL), in neonates with complex congenital heart disease. To date, there remains a limited understanding of the risk factors contributing to preoperative PVL in hypoplastic left heart syndrome (HLHS). Methods Neonates with HLHS or HLHS variants from 3 prospective magnetic resonance imaging studies (2003-2010) were selected for this cohort. Preoperative brain magnetic resonance imaging was performed the morning of the surgery. Stepwise multilogistic regression of patient characteristics, mode of delivery (cesarean section vs vaginal), time of diagnosis (prenatal vs postnatal), HLHS subtypes, brain total maturation score, time to surgery, individual averaged daily preoperative blood gases, and complete blood cell count values was used to determine significant associations. Results A total of 57 neonates with HLHS were born at 38.7 ± 2.3 weeks; 86% (49/57) had a prenatal diagnosis, with 31% (18/57) delivered by cesarean section. HLHS with aortic atresia (AA) was common in this cohort, 71% (41/57). Preoperative PVL was identified in 19% (11/57). Male patients with AA ( P = .004) were at higher risk for PVL. Lower total brain maturation score was also identified as a strong predictor for preoperative PVL ( P = .005). Conclusions In neonates with HLHS, nonmodifiable patient-related factors, including male sex with AA (lack of antegrade blood flow) and lower total brain maturation score, placed neonates at the greatest risk for preoperative white matter injury.
Background The prevalence of perioperative stroke in infants undergoing operations for congenital heart disease has not been well described. The objectives of this study were to determine the ...prevalence of stroke as assessed by postoperative brain magnetic resonance imaging (MRI), characterize the neuroanatomic features of focal ischemic injury, and identify risk factors for its development. Methods Brain MRI was performed in 122 infants 3 to 14 days after cardiac operation with cardiopulmonary bypass, with or without deep hypothermic circulatory arrest. Preoperative, intraoperative, and postoperative data were collected. Risk factors were tested by logistic regression for univariate and multivariate associations with stroke. Results Stroke was identified in 12 of 122 patients (10%). Strokes were preoperative in 6 patients and possibly intraoperative or postoperative in the other 6 patients, and were clinically silent except in 1 patient who had clinical seizures. Arterial-occlusive and watershed infarcts were identified with equal distribution in both hemispheres. Multivariate analysis identified lower birth weight, preoperative intubation, lower intraoperative hematocrit, and higher blood pressure at admission to the cardiac intensive care unit postoperatively as significant factors associated with stroke. Prematurity, younger age at operation, duration of cardiopulmonary bypass, and use of deep hypothermic circulatory arrest were not significantly associated with stroke. Conclusions The prevalence of stroke in infants undergoing operations for congenital heart disease was 10%, half of which occurred preoperatively. Most were clinically silent and undetected without neuroimaging. Mechanisms included thromboembolism and hypoperfusion, with patient-specific, procedure-specific, and postoperative contributions to increased risk.
Objective Risk factors for poor outcome with congenital complete heart block include prematurity, low birth weight, hydrops, low ventricular rates, and congenital heart disease. In this group, ...medical therapy is often ineffective, pacing is technically challenging, and mortality exceeds 80%. The purpose of this study is to assess outcomes of patients with congenital complete heart block who were paced in the first 24 hours after birth owing to the presence of known risk factors. Methods We performed a retrospective review of patients with congenital complete heart block paced in the first 24 hours after birth at our institution between November 1, 1995, and July 31, 2007. Results Thirteen patients were identified, 4 of whom had heterotaxy syndrome. Eleven patients had temporary epicardial pacing wires placed; 2 received permanent pacemakers as the initial mode of pacing. There were 7 deaths (54% mortality) at a mean age of 19.9 ± 19 days. Among 7 patients with structural heart disease, there was 1 survivor. Among 6 patients with structurally normal hearts, there were 5 survivors ( P = .025). Patients with temporary wires who survived to permanent pacemaker implantation (6/11) used their temporary leads for 33.8 ± 18.3 days. Conclusions In the severely affected fetus with congenital complete heart block and significant structural heart disease, outcomes remain poor; however, neonates with congenital complete heart block and structurally normal hearts who are monitored antenatally and delivered in a planned fashion at an institution capable of early pacing can have favorable outcomes. The use of temporary pacing wires is an option in the management of these patients.
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