Objective To compare outcomes of infants and children who underwent lung transplantation for genetic disorders of surfactant metabolism ( SFTPB , SFTPC , ABCA3 , and NKX2-1 ) over 2 epochs (1993-2003 ...and 2004-2015) at St Louis Children's Hospital. Study design We retrospectively reviewed clinical characteristics, mortality, and short- and long-term morbidities of infants (transplanted at <1 year; n = 28) and children (transplanted >1 year; n = 16) and compared outcomes by age at transplantation (infants vs children) and by epoch of transplantation. Results Infants underwent transplantation more frequently for surfactant protein-B deficiency, whereas children underwent transplantation more frequently for SFTPC mutations. Both infants and children underwent transplantation for ABCA3 deficiency. Compared with children, infants experienced shorter times from listing to transplantation ( P = .014), were more likely to be mechanically ventilated at the time of transplantation ( P < .0001), were less likely to develop bronchiolitis obliterans post-transplantation ( P = .021), and were more likely to have speech and motor delays ( P ≤ .0001). Despite advances in genetic diagnosis, immunosuppressive therapies, and supportive respiratory and nutritional therapies, mortality did not differ between infants and children ( P = .076) or between epochs. Kaplan-Meier analyses demonstrated that children transplanted in epoch 1 (1993-2003) were more likely to develop systemic hypertension ( P = .049) and less likely to develop post-transplantation lymphoproliferative disorder compared with children transplanted in epoch 2 (2004-2015) ( P = .051). Conclusion Post-lung transplantation morbidities and mortality remain substantial for infants and children with genetic disorders of surfactant metabolism.
Background Perioperative advances have led to significant improvements in outcomes after many complex neonatal open heart procedures. Whether similar improvements have been realized for the modified ...Blalock-Taussig shunt, the most common palliative neonatal closed-heart procedure, is not known. Methods Data were abstracted from The Society of Thoracic Surgeons Congenital Heart Surgery Database (2002 to 2009). Inclusion criteria were all neonates who received a modified Blalock-Taussig shunt with or without cardiopulmonary bypass, and with or without concomitant ligation of a patent ductus arteriosus. Discharge mortality was the primary end point. A composite morbidity end point one or more of the following: postoperative extracorporeal membrane oxygenation, low cardiac output, or unplanned reoperation. Associations with patient and procedural variables were assessed with univariable and multivariable analyses. Results The inclusion criteria were met by 1273 patients. The discharge mortality rate was 7.2%, and composite morbidity, as defined, was 13.1%. Primary diagnoses were classified as (1) those potentially amenable to biventricular repair (62%), (2) functionally univentricular hearts (22%), and (3) pulmonary atresia with intact ventricular septum (PA/IVS; 14%), and miscellaneous (2%). Discharge mortality stratified by primary diagnoses was PA/IVS (15.6%), functionally univentricular hearts (7.2%), and diagnoses potentially amenable to biventricular repair (5.1%). Need for preoperative ventilatory support, diagnosis of PA/IVS or functionally univentricular hearts, and any weight less than 3 kg, were risk factors for death. Preoperative acidosis or shock (resolved or persistent) and diagnosis of PA/IVS or functionally univentricular hearts were predictors of composite morbidity. Nearly 33% of the deaths occurred within 24 hours postoperatively, and 75% within the first 30 days. Conclusions The mortality rate after the neonatal modified Blalock-Taussig shunt remains high, particularly for infants weighing less than 3 kg and those with the diagnosis of PA/IVS.
Summary Survival in congenital heart disease has steadily improved since 1938, when Dr. Robert Gross successfully ligated for the first time a patent ductus arteriosus in a 7-year-old child. To ...continue the gains made over the past 80 years, transformative changes with broad impact are needed in management of congenital heart disease. Three-dimensional printing is an emerging technology that is fundamentally affecting patient care, research, trainee education, and interactions among medical teams, patients, and caregivers. This paper first reviews key clinical cases where the technology has affected patient care. It then discusses 3-dimensional printing in trainee education. Thereafter, the role of this technology in communication with multidisciplinary teams, patients, and caregivers is described. Finally, the paper reviews translational technologies on the horizon that promise to take this nascent field even further.
Background Acute kidney injury (AKI) is common in infants after cardiopulmonary bypass and is associated with poor outcomes. Peritoneal dialysis improves outcomes in adults with AKI after bypass, but ...pediatric data are limited. This retrospective case-matched study was conducted to determine if the practice of peritoneal dialysis catheter (PDC) placement during congenital heart surgery is associated with improved clinical outcomes in infants at high risk for AKI. Methods Forty-two infants undergoing congenital heart surgery with planned PDC placement (PDC+) were age-matched to infants undergoing similar surgery without PDC placement (PDC−). Demographic, baseline and outcome data were compared. Our primary outcome was negative fluid balance on postoperative days 1 to 3. Secondary outcomes included time to negative fluid balance, time to extubation, frequency of electrolyte corrective medications, inotrope scores, and other clinical outcomes. Results Baseline data did not differ between groups. The PDC+ group had a higher percentage of negative fluid balance on postoperative days 1 and 2 (57% vs 33%, P = .04; 85% vs 61%, P = .01). The PDC+ group had shorter time to negative fluid balance (16 vs 32 hours, P < .0001), earlier extubation (80 vs 104 hours, P = .02), improved inotrope scores ( P = .04), and fewer electrolyte imbalances requiring correction ( P = .03). PDC-related complications were rare. Conclusions PDC use is safe and associated with earlier negative fluid balance and improved clinical outcomes in infants at high risk for AKI. Routine PDC use should be considered for infants undergoing cardiopulmonary bypass. Further prospective studies are essential to prove causative effects of PDC placement in this population.
Objectives Modified ultrafiltration (MUF) has been shown to decrease the postcardiac surgery inflammatory response and to improve respiratory function and cardiac performance in pediatric patients; ...however, this approach has not been well established in adults. The present study hypothesized that MUF could decrease the postsurgical inflammatory response, leading to improved respiratory and cardiac function in adults undergoing coronary artery bypass grafting. Methods Sixty patients undergoing coronary artery bypass grafting were randomized to the MUF or control group (n = 30 each). MUF was performed for 15 minutes at the end of bypass. The following data were recorded at the beginning of anesthesia, end of bypass, end of experimental treatment, and 24 and 48 hours after surgery: alveolar-arterial oxygen gradient, red blood cell units transfused, chest tube drainage, hemodynamic parameters, and cytokine levels (interleukin-6, P-selectin, intercellular adhesion molecule, and soluble tumor necrosis factor receptor). Results The MUF group displayed less chest tube drainage than the control group after 48 hours (598 ± 123 mL vs 848.0 ± 455 mL; P = .04) and less red blood cell transfusions (0.6 ± 0.6 units/patient vs 1.6 ± 1.1 units/patient; P = .03). Hematocrit level was higher in the MUF group than in the control group at the end of bypass (37.8% ± 1.1% vs 34.1% ± 1.1%; P < .05), but the levels were comparable at 48 hours. Similar values for interleukin-6 and P-selectin were observed at all stages. Plasma levels of intercellular adhesion molecule were higher in the MUF group than in the control group, particularly in the first sampling after experimental treatment ( P = .01). Plasma levels of soluble tumor necrosis factor receptor were higher in the MUF group than in the control group at 48 hours. Hemodynamic and oxygen transport parameters were similar in both groups throughout the observation period. There were no differences in other clinical outcomes. Conclusions Use of MUF was associated with increased inflammatory response, reduced blood loss, and less blood transfusions in adults undergoing coronary artery bypass grafting.
Background A Potts shunt has been proposed as effective palliative therapy in children with severe pulmonary hypertension (PH) who have suprasystemic right ventricular pressures. Methods A ...retrospective single-center study was performed to assess outcomes in 5 children who underwent a Potts shunt for severe PH. Results All 5 children were in World Health Organization functional class IV. Only 3 children were classified as having idiopathic pulmonary arterial PH. Preoperatively, 4 children were receiving intravenous prostacyclins, and 3 were placed on intravenous inotropes for acute right-side heart failure. Three children were potential lung transplant candidates. All but 1 child had evidence for suprasystemic right heart pressures immediately before their operation. All 5 children survived the procedure without significant complications. Four of the 5 children were successfully discharged from the hospital and have had sustained clinical improvement with follow-up ranging from approximately 5 to 16 months. The child who did not have suprasystemic right-side heart pressures before the operation did not benefit from the Potts shunt. Conclusions The Potts shunt can be an effective palliation for children with severe PH. Our results further suggest that (1) a Potts shunt should be considered early in a child’s clinical course, before right ventricular deterioration develops; (2) a Potts shunt should be considered in any child with severe, intractable PH regardless of etiology; (3) one might consider a Potts shunt in lieu of intravenous prostacyclins; and (4) a Potts shunt should be considered before lung transplantation and does not preclude future transplantation candidacy.
Objectives The Single Ventricle Reconstruction trial randomized 555 subjects with a single right ventricle undergoing the Norwood procedure at 15 North American centers to receive either a modified ...Blalock-Taussig shunt or right ventricle-to-pulmonary artery shunt. Results demonstrated a rate of death or cardiac transplantation by 12 months postrandomization of 36% for the modified Blalock-Taussig shunt and 26% for the right ventricle-to-pulmonary artery shunt, consistent with other publications. Despite this high mortality rate, little is known about the circumstances surrounding these deaths. Methods There were 164 deaths within 12 months postrandomization. A committee adjudicated all deaths for cause and recorded the timing, location, and other factors for each event. Results The most common cause of death was cardiovascular (42%), followed by unknown cause (24%) and multisystem organ failure (7%). The median age at death for subjects dying during the 12 months was 1.6 months (interquartile range, 0.6 to 3.7 months), with the highest number of deaths occurring during hospitalization related to the Norwood procedure. The most common location of death was at a Single Ventricle Reconstruction trial hospital (74%), followed by home (13%). There were 29 sudden, unexpected deaths (18%), although in retrospect, 12 were preceded by a prodrome. Conclusions In infants with a single right ventricle undergoing staged repair, the majority of deaths within 12 months of the procedure are due to cardiovascular causes, occur in a hospital, and within the first few months of life. Increased understanding of the circumstances surrounding the deaths of these single ventricle patients may reduce the high mortality rate.
Background Treatment of congenital heart disease may include placement of a right ventricle to pulmonary artery conduit that requires future surgical replacement. We sought to identify ...surgeon-modifiable factors associated with durability (defined as freedom from surgical replacement or explantation) of the initial conduit in children less than 2 years of age at initial insertion. Methods Since 2002, 429 infants were discharged from 24 Congenital Heart Surgeons' Society member institutions after initial conduit insertion. Parametric hazard analysis identified factors associated with conduit durability while adjusting for patient characteristics, the institution where the conduit was inserted, and time-dependent interval procedures performed after conduit insertion but before replacement/explantation. Results In all, 138 conduit replacements (32%) and 3 explantations (1%) were performed. Conduit durability at a median follow-up of 6.0 years (range, 0.1 to 11.7) was 63%. After adjusting for interval procedures and institution, placement of a conduit with smaller z-score was associated with earlier replacement/explantation ( p = 0.002). Moreover, conduit durability was substantially reduced with aortic allografts ( p = 0.002) and pulmonary allografts ( p = 0.03) compared with bovine jugular venous valved conduits (JVVC). The JVVC were 12 mm to 22 mm in diameter at insertion (compared with 6 mm to 20 mm for allografts); therefore, a parametric propensity-adjusted analysis of patients with aortic or pulmonary allografts versus JVVC with diameter of 12 mm or greater was performed, which verified the superior durability of JVVC. Conclusions Pulmonary conduit type and z-score are associated with late conduit durability independent of the effects of institution and subsequent interval procedures. Surgeons can improve long-term conduit durability by judiciously oversizing, and by selecting a JVVC.
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