First-stage Palliation Research Articles

Fetal Right Heart Strain in Systemic Right Ventricles and Impact on Post-surgical Outcomes.

Patients with hypoplastic left heart syndrome (HLHS) and its variants rely on the right ventricle (RV) to provide cardiac output. Diminished RV systolic function has been associated with poor clinical outcomes in this population. Echocardiographic strain has emerged as a useful method to quantify RV deformation. We aimed to describe fetal strain in the systemic RV and further investigate if there was any correlation with clinical outcomes. We conducted a retrospective, single center study evaluating strain in fetuses with systemic RV. We measured fetal RV global longitudinal strain (GLS) and segmental strain using Tomtec 2D speckle tracking software and compared these findings to controls. Fifty patients with systemic RV were included in the study group with controls matched one to one for each echocardiogram. Ten patients died after first-stage palliation. GLS was reproducible, with interobserver ICC 0.82. There was no statistically significant difference in GLS among different HLHS subtypes. Abnormal GLS did not correlate with worse clinical outcomes. GLS in systemic RVs in the 2nd and 3rd trimester did not vary significantly throughout gestation and did not correlate with clinical outcomes. Risk factors associated with poor outcome were mainly postnatal. Multi-centered studies are needed to determine if these findings hold true in a larger sample size.

Long-Term Survival After Single-Ventricle Palliation: A Swedish Nationwide Cohort Study.

Long-term survival after single-ventricle palliation and the effect of dominant ventricle morphology in large, unselected series of patients are scarcely reported. This nationwide cohort study included all children undergoing operation with single-ventricle palliation during their first year of life in Sweden between January 1994 and December 2019. Data were obtained from institutional records and assessment of underlying cardiac anomaly and dominant ventricular morphology was based on complete review of medical records, surgical reports, and echocardiographic examinations. Data on vital status and date of death were retrieved from the Swedish Cause of Death Register, allowing for complete data on survival. Among 766 included patients, 333 patients (43.5%) were classified as having left or biventricular dominance, and 432 patients (56.4%) as having right ventricular (RV) dominance (of whom 231 patients had hypoplastic left heart syndrome). Follow-up was 98.7% complete (10 patients emigrated). Mean follow-up was 11.3 years (maximum, 26.7 years). Long-term survival was significantly higher in patients with left ventricular compared with RV dominance (10-year survival: 91.0% [95% CI, 87.3%-93.6%] versus 71.1% [95% CI, 66.4%-75.2%]). RV dominance had a significant impact on outcomes after first-stage palliation but was also associated with impaired survival after completed total cavopulmonary connection. In total, 34 (4.4%) patients underwent heart transplantation. Of these 34 patients, 25 (73.5%) had predominant RV morphology. This study provides clinically relevant knowledge about the long-term prognosis in patients with different underlying cardiac anomalies undergoing single-ventricle palliation. RV dominance had a significant impact on outcomes after initial surgical treatment but was also associated with impaired survival after completed Fontan circulation. URL: https://www.clinicaltrials.gov; Unique identifier: NCT03356574.

Outcomes of single-ventricle physiology in central South Africa.

Single-ventricle physiology is a critical cardiac condition requiring early diagnosis and intervention. The objectives of this study were to report on the management and outcomes of patients diagnosed with single-ventricle physiology in central South Africa. This study was a retrospective, observational analysis of patients presenting with single-ventricle physiology at the Universitas Academic Hospital in central South Africa between November 1997 and June 2021. Patients were referred from the Free State (54%) and Northern Cape (29%) provinces and Lesotho. One hundred and fifty-four patients presented with single-ventricle physiology: 114 received interventions and 40 were not eligible for intervention. Patients presented for the first time at a median age of 34.5 days, with patients from nearby districts presenting within a few days of birth. However, patients from outlying areas presented much later. Eighty-seven patients received systemic-to-pulmonary artery shunting or pulmonary artery banding. Sixty-three patients proceeded to bidirectional Glenn procedures, and 30 patients (26%) had full palliation to Fontan. Twenty-one patients died after stage 1, six after the Glenn procedure and two after the Fontan procedure. Overall, 34 (29.8%) patients were lost to follow up. Patients in our study presented late and follow up of these patients was a challenge. The highest mortality rate occurs during the first stage of palliation. Outcomes from this study are comparable to other sub-Saharan studies.

Pulmonary artery coarctation repair in univentricular heart: indications and optimal timing.

To analyse surgical outcomes of pulmonary artery coarctation in univentricular hearts, focusing on surgical indications and optimal timing. We retrospectively reviewed 49 patients with pulmonary artery coarctation in univentricular hearts treated at our institution between 1993 and 2022. Twenty-eight patients were diagnosed before first-stage palliation. Of these, 14 underwent systemic-pulmonary shunt only as first-stage palliation (Group 1), and 14 underwent systemic-pulmonary shunt plus surgical pulmonary artery plasty as first-stage palliation (Group 2). Twenty-one patients diagnosed after first-stage palliation underwent surgical pulmonary artery plasty at the time of bidirectional Glenn procedure (Group 3). Follow-up period after initial palliation was 6±8 years. The Fontan procedure was successful in 35 patients (71%) aged 28±26 months (range 18-139). Freedom from interstage death (Group 1, 53%; Group 2, 85%; Group 3, 93%) and interstage reintervention (Group 1, 50%; Group 2, 75%; Group 3, 73%) rates were significantly lower in Group 1 (p = 0.01). Five and four patients in Group 1 and Group 3, respectively, needed additional shunts before the bidirectional Glenn procedure. In Group 1, one patient with a non-confluent pulmonary artery achieved hemi-lung Fontan circulation. In Group 2, one patient suffering with a non-confluent pulmonary artery could not achieve Fontan circulation, whereas another patient with pulmonary venous obstruction achieved hemi-lung Fontan circulation. Surgical pulmonary artery plasty performed at first-stage palliation improved outcomes of pulmonary artery coarctation in univentricular hearts, particularly when pulmonary artery coarctation had already progressed during the neonatal period or early infancy.

Hybrid Norwood for Hypoplastic Left Heart Syndrome With Rare Aortic Arch Anatomy.

We describe the hybrid Norwood as first-stage palliation for a patient with hypoplastic left heart syndrome, right aortic arch, right descending aorta, bilateral ductus arteriosus, and left innominate artery arising from the left ductus.

Impact of Neighborhood Socioeconomic Status on Outcomes Following First-Stage Palliation of Single Ventricle Heart Disease.

Background The impact of neighborhood socioeconomic status (SES) on outcomes following first-stage palliation of single ventricle heart disease remains incompletely characterized. Methods and Results This was a single-center, retrospective review of consecutive patients who underwent the Norwood procedure from January 1, 1997 to November 11, 2017. Outcomes of interest included in-hospital (early) mortality or transplant, postoperative hospital length-of-stay, inpatient cost, and postdischarge (late) mortality or transplant. The primary exposurewas neighborhood SES, assessed using a composite score derived from 6 US census-block group measures related to wealth, income, education, and occupation. Associations between SES and outcomes were assessed using logistic regression, generalized linear, or Cox proportional hazards models, adjusting for baseline patient-related risk factors. Of 478 patients, there were 62 (13.0%) early deaths or transplants. Among 416 transplant-free survivors at hospital discharge, median postoperative hospital length-of-stay and cost were 24 (interquartile range, 15-43) days and $295 000 (interquartile range, $193 000-$563 000), respectively. There were 97 (23.3%) late deaths or transplants. On multivariable analysis, patients in the lowest SES tertile had greater risk of early mortality or transplant (odds ratio [OR], 4.3 [95% CI, 2.0-9.4; P<0.001]), had longer hospitalizations (coefficient 0.4 [95% CI, 0.2-0.5; P<0.001]), incurred higher costs (coefficient 0.5 [95% CI, 0.3-0.7; P<0.001]), and had greater risk of late mortality or transplant (hazard ratio, 2.2 [95% CI, 1.3-3.7; P=0.004]), compared with those in the highest tertile. The risk of late mortality was partially attenuated with successful completion of home monitoring programs. Conclusions Lower neighborhood SES is associated with worse transplant-free survival following the Norwood operation. This risk persists throughout the first decade of life and may be mitigated with successful completion of interstage surveillance programs.

Impact of Extracardiac Anomalies on Mortality and Morbidity in Staged Single Ventricle Palliation

This study was intended to determine the impact of extracardiac anomalies on outcomes in patients with functional single ventricle who underwent staged palliation. We reviewed medical records of patients who underwent first-stage palliation at our center between 2001 and 2020. The prevalence and type of extracardiac anomalies were evaluated, and their impact on outcomes during staged palliation was analyzed. Among 602 patients who underwent first-stage palliation, 81 (14%) patients had associated with extracardiac anomalies. They were more frequently associated with prematurity (p=0.03) and low birth weight below 2.5 kg (p<0.01). Mortality between first-stage palliation and stage II was similar in patients with and without extracardiac anomalies (24.7 vs. 17.1%, p=0.10). However, mortality between stage II and stage III was significantly higher in patients with extracardiac anomalies compared to those without (22.2 vs. 12.5 %, p=0.02). Mortality after stage III was also higher in patients with extracardiac anomalies compared to those without (4.9 vs. 1.5%, p=0.04). In the sub-group analysis of 81 patients with extracardiac anomalies, renal anomalies were identified as a significant risk factor for mortality (p=0.03, HR: 2.44). The incidence of extracardiac anomalies in this study was 14 %, and patients with extracardiac anomalies were highly associated with prematurity and low birth weight. Presence of extracardiac anomalies was associated with higher mortality between stage II and stage III palliation and after stage III phase, but not before stage II. Among extracardiac anomalies, renal anomalies were identified as a risk factor for mortality.

Hybrid First-stage Palliation and Other Strategies to Achieve Biventricular Repair in High-Risk Neonates With Complex Heart Anomalies and Aortic Arch Obstruction.

Medical and surgical advances have allowed single-stage total repair in neonates born with complex congenital heart anomalies and aortic arch obstruction. Nonetheless, total repair might be too complex or high risk in certain neonates with demographic, clinical or morphologic risk factors. Alternative management strategies might offer these neonates better outcomes with superior anatomic repair, shorter hospitalization, reduced morbidity, and improved survival. Alternative initial surgical strategies might include aortic arch repair and pulmonary artery band with or without cardiopulmonary bypass, extracardiac repair only and pulmonary artery band, Norwood operation, and hybrid first-stage palliation; all deferring complex biventricular intra-cardiac repair to later stage. The strategy choice should be personalized to each patient, taking into consideration the morphologic and clinical state, and the existent goals of care.

Trends in the Hospital Care of Neonates With Hypoplastic Left Heart Syndrome in the United States

BackgroundSurgical outcomes of first-stage palliation for hypoplastic left heart syndrome (HLHS) are vastly improved from prior eras and are well described, but rates and determinants of nonsurgical management are understudied, particularly on the national scale. MethodsThe Pediatric Health Information System database was used to identify all index neonatal HLHS admissions between 2015 and 2021 in the United States. Surgical palliation included cardiac surgery and transplantation during the index admission. Multivariable binary logistic regression was used to identify factors independently predictive of nonsurgical management, represented by odds ratio <1.0. ResultsIn total, 3902 HLHS neonatal admissions to 48 tertiary care centers occurred. Eighteen percent (n = 710) of neonates did not receive surgical palliation. Ninety-seven percent of nonsurgical patients died in the hospital or were discharged to comfort care, whereas 84% of surgically palliated neonates survived to discharge. Nonsurgical patients had greater rates of prematurity, birth weight <2.5 kg, female sex, and chromosomal abnormality (all P < .01). Factors independently associated with nonoperative management included admission to a low-volume surgical program (odds ratio, 0.2 [95% CI, 0.15-0.26]), birth weight <2.5 kg (0.30 [0.24-0.37]), chromosomal abnormality (0.55 [0.43-0.71]), and non-White race/ethnicity (0.68 [0.56-0.82]). ConclusionsIn the contemporary era of neonatal HLHS management, nearly one-fifth of neonates do not undergo surgical palliation in the United States. Additional work can outline the precise reasons underlying such high rates of nonoperative management in hopes of ensuring equitable care for all children with HLHS.

HLHS: Complex Anatomic Surgical Considerations During Treatment Pathway.

The Norwood operation as first-stage palliation for hypoplastic left heart syndrome has been standardized in most centers. However, in the presence of anomalies of aortic arch anatomy or position, or other anatomical variants, important modifications of the procedure may become necessary. This report summarizes surgical techniques to address these challenges.

Influence of intraoperative residual lesions and timing of extracorporeal membrane oxygenation on outcomes following first-stage palliation of single-ventricle heart disease

BackgroundData regarding the influence of intraoperative residual lesions on extracorporeal membrane oxygenation (ECMO) following the Norwood procedure are limited. Moreover, the significance of postoperative ECMO timing on in-hospital outcomes remains incompletely characterized. MethodsThis was a single-center, retrospective review of consecutive patients who underwent the Norwood operation from January 1997 to November 2017. Patients with at least minor residual lesions based on the intraoperative postcardiopulmonary bypass echocardiogram were identified. The association between residual lesions and postoperative ECMO was assessed with logistic regression, adjusting for age, weight, prematurity, various preoperative system-specific and procedural risk factors, shunt type, and era. Among patients receiving ECMO, associations between late ECMO (≥3 days post-Norwood) and in-hospital mortality or transplant, postoperative hospital length-of-stay, and cost of hospitalization were evaluated using logistic regression or generalized linear models with a gamma distribution and logarithmic link. ResultsAmong 500 patients, 78 (15.6%) received ECMO postoperatively. On multivariable analysis, the presence of at least minor residual lesions (odds ratio, 4.4; 95% CI, 2.1-9.3; P < .001) was associated with postoperative ECMO. In the ECMO subpopulation, there were 44 (56.4%) deaths or transplants. Late ECMO was associated with increased risk of in-hospital mortality or transplant (adjusted odds ratio, 6.2; 95% CI, 1.5-26.0), longer postoperative hospital length of stay (regression coefficient, 0.7; 95% CI, 0.3-1.1), and greater cost (regression coefficient, 0.6; 95%, CI 0.4-0.7), versus early ECMO (all P values < .05). ConclusionsThe presence of even minor intraoperative residua significantly increases the risk of ECMO following the Norwood operation. Among patients receiving ECMO postoperatively, early institution of ECMO is associated with lower mortality and resource utilization.

Utilizing technology to expand home monitoring to high-risk infants with CHD.

Infants born with single ventricle physiology that require an aorto-pulmonary shunt are at high risk for sudden cardiac death, particularly during the interstage period between the first-stage palliation and the second-stage palliation. Home monitoring programs have decreased interstage mortality in the hypoplastic left heart syndrome population prompting programs to expand the home monitoring program to other high-risk populations. At our mid-sized program, we implemented the Locus Health home monitoring platform first in the hypoplastic left heart syndrome population, then expanding to the single ventricle shunt population. Interstage mortality for the hypoplastic left heart syndrome population after initiation of the home monitoring program went from 18% prior to 2009 to 7% as of the end of 2020 (n = 99), with 2.8% mortality from 2013 to 2020 and 0% mortality since initiation of the Locus program in 2017. Caregiver surveys done prior to discharge and then 3 weeks later were used to document caregiver experience using the digital home monitoring program. Caregivers reported overall positive experience with the digital application, with 91.8% stating that they felt confident taking care of their baby at home. Transitioning the home monitoring program from a traditional binder to an iPad with the Locus Health application allowed us to expand the program, utilize the electronic medical record, bill for the service, and demonstrate positive experiences for caregivers. Overall engagement and adherence with the program by caregivers were 50.94 and 45.45%, with a total of 112 patient episodes. Reimbursement from private insurance providers was 22% of the billed amount for 2020.

Patent ductus arteriosus stenting versus modified Blalock-Taussig shunt for palliation of duct-dependent pulmonary blood flow lesions.

Patent ductus arteriosus (PDA) stenting is an alternative to modified Blalock-Taussig shunt (MBTS) as first-stage palliation of duct-dependent lesions. The superiority of one approach over the other is still controversial. Our objective was to compare PDAstent versusMBTS for palliation in regard to safety, efficacy, and efficiency. From 2010 to 2021, 134 patients had first-stage palliation with either PDAstent (n = 83) or MBTS (n = 51). Twenty-seven patients failed the primary treatment and were converted to the other group. The study endpoints were hospital outcomes, interstage reintervention, and concomitant procedures at the second-stage palliation. Patients with PDAstent were significantly younger. The prevalence of antegrade pulmonary blood flow (PBF) was higher in patients who had MBTS and graft thrombosis was higher in the PDAstent. Hospital stay was significantly longer in patients who had MBTS. Predictors of prolonged mechanical ventilation were low-weight, MBTS, and conversion. Intensive careunit stay significantly increased with conversion, low-weight, and antegrade PBF. The interstage intervention was required more frequently in PDA-stent group. Predictors of reintervention were conversion and pulmonary atresia with the intact interventricular septum. Pulmonary artery plasty was required more frequently during the second-stage palliation in PDA-stent group. PDAstent is an alternative to MBTS for first-stage palliation. It is associated with shorter hospital stays and avoidance of surgery at the expense of a high rate of stent thrombosis and interstage reintervention. Conversion increased the risk of the procedure. More studies are needed to determine factors that affect PDA-stent outcomes and patient selection criteria.

Timing of reintervention influences survival and resource utilization following first-stage palliation of single ventricle heart disease

ObjectiveOutcomes after first-stage palliation of single-ventricle heart disease are influenced by many factors, including the presence of residual lesions requiring reintervention. However, there is a dearth of information regarding the optimal timing of reintervention. We assessed if earlier reintervention would be favorably associated with in-hospital outcomes among patients requiring unplanned reinterventions after the Norwood operation. MethodsThis was a single-center, retrospective review of all patients who underwent the Norwood procedure from January 1997 to November 2017 and required a predischarge unplanned surgical or transcatheter reintervention on 1 or more subcomponent areas repaired at the index operation. Outcomes of interest included in-hospital mortality or transplant, postoperative hospital length of stay, and inpatient cost. Associations between timing of reintervention and outcomes were assessed using logistic regression (mortality or transplant) or generalized linear models (postoperative hospital length of stay and cost), adjusting for baseline patient-related and procedural factors. ResultsOf 500 patients who underwent the Norwood operation, 92 (18.4%) required an unplanned reintervention. Median time to reintervention was 12 days (interquartile range, 5-35 days). There were 31 (33.7%) deaths or transplants, median postoperative hospital length of stay was 49 days (interquartile range, 32-87 days), and median cost was $328,000 (interquartile range, $204,000-$464,000). On multivariable analysis, each 5-day increase in time to reintervention increased the odds of mortality or transplant by 20% (odds ratio, 1.2; 95% confidence interval, 1.1-1.3; P = .004). Longer time to reintervention was also significantly associated with greater postoperative hospital length of stay (P < .001) and higher cost (P < .001). ConclusionsFor patients requiring predischarge unplanned reinterventions after the Norwood operation, earlier reintervention is associated with improved in-hospital transplant-free survival and resource use.

Rapid Two-Stage Norwood Procedure Using an Auto-Pericardial Patch Fixed with an Arch-Shaped Mold

While the classical 1-stage Norwood procedure is still performed, there are several types of “hybrid” procedures for the management of hypoplastic left heart syndrome. These hybrid approaches consist of bilateral pulmonary artery banding with ductal stenting or prostaglandin infusion as the first-stage palliation, followed by a second-stage Norwood procedure or comprehensive stage II procedure. Since 2012, we have adopted a rapid 2-stage Norwood procedure as a routine strategy, where bilateral pulmonary artery banding is performed within 5 days of age with balloon atrial septectomy, if needed, before the development of hemodynamic instability. The second-stage Norwood procedure is performed within 1 month of age. The arterial duct is kept open by continuing prostaglandin administration. In addition, an important improvement of our Norwood procedure is the use of an auto-pericardial patch fixed on an arch-shaped metal mold. The pericardium is wrapped around the lesser curvature of the mold and treated with 0.6% glutaraldehyde for 15 min. This makes it easier to imagine the final shape of the arch and helps to enlarge the retroaortic space significantly, which could reduce the risk of bronchus or central pulmonary artery stenosis and facilitate hemostasis. These developments in strategy and procedure could improve our surgical results. While the classical 1-stage Norwood procedure is still performed, there are several types of “hybrid” procedures for the management of hypoplastic left heart syndrome. These hybrid approaches consist of bilateral pulmonary artery banding with ductal stenting or prostaglandin infusion as the first-stage palliation, followed by a second-stage Norwood procedure or comprehensive stage II procedure. Since 2012, we have adopted a rapid 2-stage Norwood procedure as a routine strategy, where bilateral pulmonary artery banding is performed within 5 days of age with balloon atrial septectomy, if needed, before the development of hemodynamic instability. The second-stage Norwood procedure is performed within 1 month of age. The arterial duct is kept open by continuing prostaglandin administration. In addition, an important improvement of our Norwood procedure is the use of an auto-pericardial patch fixed on an arch-shaped metal mold. The pericardium is wrapped around the lesser curvature of the mold and treated with 0.6% glutaraldehyde for 15 min. This makes it easier to imagine the final shape of the arch and helps to enlarge the retroaortic space significantly, which could reduce the risk of bronchus or central pulmonary artery stenosis and facilitate hemostasis. These developments in strategy and procedure could improve our surgical results.

Risk factors for mortality after cardiovascular surgery in patients with functional single ventricle and right isomerism.

Cardiac malformations are a major component of heterotaxy syndrome that results in significant mortality and morbidity, especially in patients with a single ventricle and right isomerism (RI). The goal of this study was to evaluate the mortality after cardiovascular surgery in patients with a functional single ventricle and RI over a long follow-up period (∼40 years) and to determine the predicted risk factors for mortality. We performed a retrospective review of the medical records of 129 consecutive patients with functional single ventricle and RI who underwent pulmonary flow control operations at Mt. Fuji Shizuoka Children's Hospital between 1979 and 2020. To evaluate mortality rates, the patients were divided into 2 groups (era 1: 1979-1999 and era 2: 2000-2020) based on the date of the first-stage palliation. The estimated survival rate at 10 years was 36.4% in era 1 and 57.8% in era 2. The estimated survival rate improved significantly (P = 0.0268) between the 2 eras. The rate of Fontan procedure completion was also significantly better in the current era (P = 0.0392; 22/59 in era 1 and 38/66 in era 2). In the multivariable analysis, the date of the first-stage palliation was the only predictor of mortality. The mortality rate after cardiovascular surgery in patients with a functional single ventricle and RI has improved over the past 20 years; however, it still remains high. This improvement may be attributed to our current surgical strategy and clinical management; however, further investigations are needed to prove this observation.

Outcomes of single ventricle palliation in infants with heterotaxy syndrome.

Heterotaxy is a known risk factor for morbidity and mortality in single ventricle palliation. In this study, we examined our experience with this challenging group of patients. Records of patients born between 2001 and 2019 with heterotaxy, who needed staged single ventricle palliation were retrospectively analysed. A total of 53 patients were included in this study. Thirty-five (66%) patients had a right ventricular dominance, common atrioventricular septal defect was present in 37 (70%) patients. Anomalous pulmonary venous drainage was present in 29 (55%) patients. Forty-six (86%) patients underwent first-stage palliation. Forty-one (77.3%) patients received a bidirectional cavopulmonary connection. Thirty-one (58%) patients received total cavopulmonary connection (TCPC). Overall survival rate was 92% at 1 month, 74% at 1 year and 68% at 10 years. Twelve (22.6%) patients died before second palliation stage. Four (10%) patients died before TCPC. No patient died after TCPC. Independent risk factors for mortality in the multivariate COX regression were a presence of restrictive pulmonary blood flow (HR 3.23; 95% CI 1.02-10.2; P = 0.05) and greater than mild atrioventricular valve regurgitation (HR 3.57; 95% CI 1.27-10.0; P = 0.02). Mortality and morbidity in patients with single ventricle and heterotaxy are high. Restrictive pulmonary blood flow needing early modulation and greater than mild atrioventricular valve regurgitation at presentation are independent risk factors for mortality. Total anomalous pulmonary venous connection was not identified as a risk factor in the current era.

Pulsatile ventricular assist device as a bridge to transplant for the early high-risk single-ventricle physiology

BackgroundThe use of ventricular assist devices (VADs) as a bridge-to-transplant in patients with single-ventricle physiology post–stage one palliation has been associated with poor outcomes. We describe our center's successful experience in the use of paracorporeal pulsatile VADs in the palliation of high-risk single ventricle physiology before or after the first stage of palliation with an impetus on pre-palliation implant. MethodsThis is a single-center retrospective review of univentricular patients implanted with the Berlin Heart EXCOR VAD. Our center's approach includes early implantation of the Berlin Heart EXCOR with common atrial cannulation, a cardiac index between 3.5 and 5 L/min/m2, and a bivalirudin-based anticoagulation regimen. Patient-related data were collected postimplant at week 1 and months 1, 2, and 3. Post-transplant data, including neurological outcomes, were collected. ResultsNine patients were supported. Survival to discharge post-transplant was 83% (5/6) in patients bridged-to-transplant and 33% (1/3) in patients bridged-to-decision. Six patients had no previous palliation. Median hospital stay before implantation was 111 days for nonsurvivors versus 20 days for survivors. The need for extracorporeal membrane oxygenation and cardiopulmonary resuscitation in nonsurvivors versus survivors was 1 in 3 versus 1 in 6 and 2 in 3 versus 1 in 6, respectively. There were no major central nervous system complications except for 1 significant hemorrhagic event. The pediatric overall performance category score on follow-up was normal to mild disability in 83% of survivors. Limitations include hemolysis and intermittent periods of infection and/or inflammation. ConclusionsThe use of pulsatile paracorporeal VADs is a feasible option as a bridge-to-transplant in the peri–stage one high-risk single ventricle.

Pulmonary atresia with ventricular septal defect and tetralogy of Fallot: transannular path augmentation versus systemic to pulmonary artery shunt for first-stage palliation.

Pulmonary atresia with ventricular septal defect and severe tetralogy of Fallot require a palliative procedure for pulmonary artery rehabilitation. For first-stage palliation, two main surgical options are still debated: right ventricle to pulmonary artery connection and modified Blalock-Taussig shunt. We compared the clinical outcomes of the two procedures. From 1995 to 2018, 88 patients needed palliation (pulmonary atresia with ventricular septal defect n = 47; tetralogy of Fallot n = 41). Among these patients, 70 modified Blalock-Taussig shunt and 18 transannular path augmentation were performed before 6 months of age. Using a 1:1 propensity score match analysis, 20 patients were included in the analysis. The primary outcome was in-hospital mortality and pulmonary artery growth. After matching, the pre-operative Nakata was smaller in transannular path augmentation 54 ± 24 mm2/m2 than modified Blalock-Taussig shunt 109 ± 31 mm2/m2 (p < 0.001). The age and weight were similar (p = 0.31 and p = 0.9, respectively). There was no difference in in-hospital mortality (p = 0.3). The Nakata index before biventricular repair and delta Nakata were smaller in modified Blalock-Taussig shunt group (206 ± 80 mm2/m2, 75 ± 103 mm2/m2) than transannular path augmentation (365 ± 170 mm2/m2, 214 ± 165 mm2/m2; p = 0.03; p < 0.001). Median time to biventricular repair was similar (p = 0.46). The rate of interstage reintervention was similar (p = 0.63). The transannular path augmentation is better for the rehabilitation of the native pulmonary artery. Despite a smaller pulmonary artery, right ventricle to pulmonary artery connection is equivalent to modified Blalock-Taussig shunt for rate of biventricular repair and time to biventricular repair.

Impact of prior diaphragm plication on subsequent stages of single ventricle palliation

BackgroundPhrenic nerve injury is a known cause of morbidity after single ventricle palliation. Previous studies have shown that hemidiaphragm plication improves short-term outcomes. The effect of plication on the outcomes of subsequent stages of single ventricle palliation is unknown. MethodsFrom 1997 to 2015, 1146 patients underwent surgical management of single ventricle physiology at our institution. We reviewed the records of 30 patients who had undergone diaphragm plication for phrenic nerve injury before Fontan completion. Each patient was compared with 2 propensity-matched controls identified from patients who underwent the Glenn or Fontan procedure during the same period without diaphragm plication. Propensity matching was achieved for each test subject using the nearest neighbor algorithm. Data are presented as the median and quartiles or numbers and percentages. ResultsThe cohort included 18 boys (60%). Of the 30 patients, 19 (63%) had undergone plication after first-stage palliation. Of these, 13 have undergone completion Fontan, 5 were awaiting Fontan at the last follow-up, and 1 had died. An additional 11 patients had undergone plication after Glenn and proceeded to Fontan completion. Thus, 24 patients with diaphragm plication have undergone Fontan completion. No difference was found in pulmonary pressure or resistance between the plicated patients and their propensity-matched controls. Both groups had comparable chest tube output and hospital lengths of stay. Equal proportions of patients in both groups required pulmonary vasodilator therapy and/or supplemental oxygen at hospital discharge. ConclusionsPrior diaphragm plication does not adversely affect Fontan completion in children with single ventricle physiology. The hospital course during subsequent stages of palliation for plicated patients was no different than that of matched controls.