15-Year Analysis of Surgical Approaches and Outcomes for Coarctation in 132 Neonates and Infants.

A variety of surgical approaches exist to treat aortic coarctation in neonates and infants. Our institutional approach is designed to match the surgical approach to the individual anatomy of the patient. The objective of this study is to evaluate operative characteristics and outcomes of all neonates and infants who underwent surgical repair of coarctation of the aorta or hypoplastic aortic arch at University of Florida from 2006 to 2021, inclusive, either in isolation or with concomitant repair of atrial septal defect (ASD) and/or ventricular septal defect (VSD). A retrospective review was performed of 132 patients aged 0-1 year who underwent surgical repair of aortic coarctation or hypoplastic aortic arch between 2006 and 2021, inclusive, either in isolation or with concomitant repair of ASD and/or VSD. Patients were divided into two groups based on the surgical approach: Group 1 = Median Sternotomy and Group 2 = Left Lateral Thoracotomy. Continuous variables are presented as median (minimum-maximum); categorical variables are presented as N (%). The most common operative technique in Group 1 was end-to-side reconstruction with ligation of the aortic isthmus. The most common operative technique in Group 2 was extended end-to-end repair. Operative Mortality was one patient (1/132 = 0.76%). Transcatheter intervention for recurrent coarctation was performed in seven patients (7/132 = 5.3%). Surgical re-intervention for recurrent coarctation was performed in three patients (3/132 = 2.3%). From these data, one can conclude that a strategy of matching the surgical approach to the anatomy of neonates and infants who underwent surgical repair of aortic coarctation or hypoplastic aortic arch, either in isolation or with concomitant repair of ASD and/or VSD, is associated with less than 1% Operative Mortality and less than 3% recurrent coarctation requiring reoperation.

A single-institutional experience with 36 children less than 5 kilograms supported with the Berlin Heart: Comparison of congenital versus acquired heart disease.

OBJECTIVES: We reviewed outcomes in all 36 consecutive children <5 kg supported with the Berlin Heart pulsatile ventricular assist device at the University of Florida, comparing those with acquired heart disease (n = 8) to those with congenital heart disease (CHD) (n = 28). METHODS: The primary outcome was mortality. The Kaplan-Meier method and log-rank tests were used to assess group differences in long-term survival after ventricular assist device insertion. T-tests using estimated survival proportions were used to compare groups at specific time points. RESULTS: Of 82 patients supported with the Berlin Heart at our institution, 49 (49/82 = 59.76%) weighed <10 kg and 36 (36/82 = 43.90%) weighed <5 kg. Of 36 patients <5 kg, 26 (26/36 = 72.22%) were successfully bridged to transplantation. (The duration of support with ventricular assist device for these 36 patients <5 kg was [days]: median = 109, range = 4-305.) Eight out of 36 patients <5 kg had acquired heart disease, and all eight [8/8 = 100%] were successfully bridged to transplantation. (The duration of support with ventricular assist device for these 8 patients <5 kg with acquired heart disease was [days]: median = 50, range = 9-130.) Twenty-eight of 36 patients <5 kg had congenital heart disease. Eighteen of these 28 [64.3%] were successfully bridged to transplantation. (The duration of support with ventricular assist device for these 28 patients <5 kg with congenital heart disease was [days]: median = 136, range = 4-305.) For all 36 patients who weighed <5 kg: 1-year survival estimate after ventricular assist device insertion = 62.7% (95% confidence interval = 48.5-81.2%) and 5-year survival estimate after ventricular assist device insertion = 58.5% (95% confidence interval = 43.8-78.3%). One-year survival after ventricular assist device insertion = 87.5% (95% confidence interval = 67.3-99.9%) in acquired heart disease and 55.6% (95% confidence interval = 39.5-78.2%) in CHD, P = 0.036. Five-year survival after ventricular assist device insertion = 87.5% (95% confidence interval = 67.3-99.9%) in acquired heart disease and 48.6% (95% confidence interval = 31.6-74.8%) in CHD, P = 0.014. CONCLUSION: Pulsatile ventricular assist device facilitates bridge to transplantation in neonates and infants weighing <5 kg; however, survival after ventricular assist device insertion in these small patients is less in those with CHD in comparison to those with acquired heart disease.

Support with Single Ventricle-Ventricular Assist Device (sVAD) in Patients with Functionally Univentricular Circulation Prior to Fontan Operation.

Some patients with functionally univentricular circulation develop cardiac failure refractory to maximal management and are supported with a ventricular assist device (VAD). The purpose of this manuscript is to summarize our previous publications related to single ventricle-ventricular assist device (sVAD) support in patients with functionally univentricular circulation and to describe our current institutional approach at University of Florida to sVAD support in neonates, infants, and children prior to Fontan. Our programmatic philosophy at University of Florida is to strive to identify the minority of neonates with functionally univentricular circulation who are extremely high-risk prior to initiating staged palliation and to stabilize these neonates with primary preemptive sVAD in preparation for cardiac transplantation; our rationale for this approach is related to the challenges associated with failed staged palliation and subsequent bail-out sVAD support and transplantation. A subset of extremely high-risk neonates and infants with functionally univentricular ductal-dependent circulation undergo primary preemptive sVAD insertion and subsequent cardiac transplantation. Support with VAD clearly facilitates survival on the waiting list during prolonged wait times and optimizes outcomes after Norwood (Stage 1) by providing an alternative pathway for extremely high-risk patients. Therefore, the selective utilization of sVAD in extremely high-risk neonates facilitates improved outcomes for all patients with functionally univentricular ductal-dependent circulation. At University of Florida, our programmatic approach to utilizing sVAD support as a bridge to transplantation in the minority of neonates with functionally univentricular circulation who are extremely high-risk for staged palliation is associated with Operative Mortality after Norwood (Stage 1) Operation of 2.9% (2/68) and a one-year survival of 91.1% (82/90) for all neonates presenting with hypoplastic left heart syndrome (HLHS) or HLHS-related malformation with functionally univentricular ductal-dependent systemic circulation. Meanwhile, at University of Florida, for all 82 consecutive neonates, infants, and children supported with pulsatile paracorporeal VAD: Kaplan-Meier survival estimated one year after VAD insertion = 73.3% (95% confidence interval [CI] = 64.1-83.8%), and Kaplan-Meier survival estimated five years after VAD insertion = 68.3% (95% CI = 58.4-79.8%). For all 48 consecutive neonates, infants, and children at University of Florida with biventricular circulation supported with pulsatile paracorporeal VAD: Kaplan-Meier survival estimated one year after VAD insertion = 82.7% (95% CI = 72.4-94.4%), and Kaplan-Meier survival estimated five years after VAD insertion = 79.7% (95% CI = 68.6-92.6%). For all 34 consecutive neonates, infants, and children at University of Florida with functionally univentricular circulation supported with pulsatile paracorporeal sVAD: Kaplan-Meier survival estimated one year after VAD insertion = 59.7% (95% CI = 44.9-79.5%), and Kaplan-Meier survival estimated five years after VAD insertion = 50.5% (95% CI = 35.0-73.0%). These Kaplan-Meier survival estimates for patients supported with pulsatile paracorporeal VAD are better in patients with biventricular circulation in comparison to patients with functionally univentricular circulation both one year after VAD insertion (P=0.026) and five years after VAD insertion (P=0.010). Although outcomes after VAD support in functionally univentricular patients are worse than in patients with biventricular circulation, sVAD provides a reasonable chance for survival. Ongoing research is necessary to improve the outcomes of these challenging patients, with the goal of developing strategies where outcomes after sVAD support in functionally univentricular patients are equivalent to the outcomes achieved after VAD support in patients with biventricular circulation.

Lessons learned from linking two complementary databases: the Society of Thoracic Surgeons Congenital Heart Surgery Database and The Vermont Oxford Network Expanded Database.

The Society of Thoracic Surgeons Congenital Heart Surgery Database and the Vermont Oxford Network Expanded Database are both large, international, well-established quality and outcomes databases with high penetration in their respective fields of congenital heart surgery and neonatology. Previous studies have shown the value of combining large databases for research purposes. Our aim was to examine the feasibility and value of combining these databases on a local level.We included patients from both databases, cared for at our centre and born from 2015-2020, who had cardiac surgery as neonates or during the birth hospitalisation. We examined the number of patients from each database and overlap between the two. We compared cardiac diagnoses, surgeries performed, pre-operative factors, mortality, and length of stay between databases.Of the 255 patients meeting criteria, 209 (81.9%) had records in both databases. The most common diagnoses in both were hypoplastic left heart syndrome, coarctation, and transposition of the great arteries. Surgical data were incompletely recorded in Vermont Oxford. Gestational age, birth weight, multiple gestation, mortality, and length of stay did not differ significantly between the databases, while the percentage of patients with an extracardiac malformation or genetic syndrome recorded was higher in the Society for Thoracic Surgeons group.Larger-scale matching and comparison studies using these databases are feasible and desirable; for some variables, a record with data from both databases may be more complete. Specific attention should be given to inclusion criteria, reconciling different schema of diagnoses, and formulating questions relying on each database's relative strengths.

Effect of prenatal diagnosis on hospital costs in complete transposition of the great arteries.

OBJECTIVE: Prenatal diagnosis of congenital heart disease (CHD) is associated with improved clinical outcomes, yet its impact on the cost of hospitalization is not well described. We hypothesized that prenatal diagnosis of complete transposition of the great arteries (d-TGA) results in lower total hospital costs compared with postnatal diagnosis. METHODS: Retrospective analysis of infants with d-TGA repaired at our center from July 2006 to 2014. Total charges from initial hospitalization until discharge were converted to costs using the cost-to-charge ratio and then converted into 2016 dollars using the consumer price index. A direct cost comparison from the hospital perspective was performed between groups. A secondary analysis included the cost of prenatal diagnosis. RESULTS: Thirty-three infants with d-TGA were identified; 8 with and 25 without prenatal diagnosis. There was no difference in baseline characteristics. Mean direct cost of hospitalization was higher in infants without prenatal diagnosis ($108 014 ± $51 305 vs $88 305 ± $22 896, P = .31). On secondary analysis, the cost of prenatal diagnosis was negligible compared with total hospital cost. CONCLUSIONS: Total cost of initial hospitalization was higher for infants without prenatal diagnosis of d-TGA. Prenatal diagnosis not only improves clinical outcomes but may also be cost saving in the current era of increasing health care costs.

A Single-Institutional Experience with 36 Children Smaller Than 5 Kilograms Supported with the Berlin Heart Ventricular Assist Device (VAD) over 12 Years: Comparison of Patients with Biventricular versus Functionally Univentricular Circulation.

OBJECTIVES: We reviewed outcomes in all 36 consecutive children <5 kg supported with the Berlin Heart pulsatile ventricular assist device (VAD) at the University of Florida, comparing those with univentricular circulation (n = 23) to those with biventricular circulation (n = 13). METHODS: The primary outcome was mortality. Kaplan-Meier methods and log-rank tests were used to assess group differences in long-term survival after VAD insertion. T-tests using estimated survival proportions and standard errors were used to compare groups at specific time points. RESULTS: Of all 82 patients ever supported with Berlin Heart at our institution, 49 (49/82 = 59.76%) weighed <10 kg and 36 (36/82 = 43.90%) weighed <5 kg. Of these 36 patients who weighed <5 kg, 26 (26/36 = 72.22%) were successfully bridged to transplantation. Of these 36 patients who weighed <5 kg, 13 (13/36 = 36.1%) had biventricular circulation and were supported with 12 biventricular assist devices (BiVADs) and 1 left ventricular assist device (LVAD) (Age [days]: median = 67, range = 17-212; Weight [kilograms]: median = 4.1, range = 3.1-4.9), while 23 (23/36 = 63.9%) had univentricular circulation and were supported with 23 single ventricle-ventricular assist devices (sVADs) (Age [days]: median = 25, range = 4-215; Weight [kilograms]: median = 3.4, range = 2.4-4.9). Of 13 biventricular patients who weighed <5 kg, 12 (12/23 = 92.3%) were successfully bridged to cardiac transplantation. Of 23 functionally univentricular patients who weighed <5 kg, 14 (14/23 = 60.87%) were successfully bridged to cardiac transplantation. For all 36 patients who weighed <5 kg: 1-year survival estimate after VAD insertion = 62.7% (95% confidence interval [CI] = 48.5%-81.2%) and 5-year survival estimate after VAD insertion = 58.5% (95% CI = 43.8%-78.3%). One-year survival after VAD insertion: 84.6% (95% CI = 67.1%-99.9%) in biventricular patients and 49.7% (95% CI = 32.3%-76.4%) in univentricular patients, P = 0.018. Three-year survival after VAD insertion: 84.6% (95% CI = 67.1%-99.9%) in biventricular patients and 41.4% (95% CI = 23.6%-72.5%) in univentricular patients, P = 0.005. CONCLUSION: Pulsatile VAD facilitates bridge to transplantation in neonates and infants weighing <5 kg; however, survival after VAD insertion in these small patients is less in those with univentricular circulation in comparison to those with biventricular circulation.

George Daicoff: A Pioneering Surgeon and Humanitarian of The Southern Thoracic Surgical Association.

George Daicoff was a true pioneer who excelled in patient care, research, teaching, and advocacy; he performed cardiac surgery on thousands. He trained at Mayo Clinic under the tutelage of John Kirklin in 1966. He served on the faculty at University of Florida from 1967 to 1977 and became Chief of Thoracic and Cardiovascular Surgery at University of Florida. He then served as Chief of Cardiovascular Surgery at All Children's Hospital from 1977 to 1998. In 1972, he was one of 13 founding members of the Congenital Heart Surgeons' Society (CHSS), and in 1973, he was one of 10 surgeons to attend the first meeting of CHSS. Dr Daicoff won the 1970 Southern Thoracic Surgical Association President's Award for best scientific paper, and the 1996 Southern Thoracic Surgical Association Osler Abbot Award. In 2015, the Southern Thoracic Surgical Association Congenital Heart Surgery President's Award was named the George Daicoff Award.

Palliation Plus Ventricular Assist Device Insertion in 15 Neonates and Infants With Functionally Univentricular Circulation.

BACKGROUND: We report 15 high-risk neonates and infants with functionally univentricular circulation stabilized with initial surgical palliation plus ventricular assist device (VAD) insertion (PALLIATION+VAD) in preparation for transplantation. METHODS: Fifteen functionally univentricular patients with ductal-dependent systemic circulation (8 hypoplastic left heart syndrome, 1 hypoplastic left heart syndrome-related malformation: 7 neonates, 2 infants) or ductal-dependent pulmonary circulation (6 hypoplastic right heart syndrome: 5 neonates, 1 infant) presented with anatomical and/or physiological features associated with increased risk for conventional univentricular palliation (large coronary sinusoids with ventricular-dependent coronary circulation, severe systemic atrioventricular valvar regurgitation, cardiogenic shock, or restrictive atrial septum). PALLIATION+VAD for patients with ductal-dependent systemic circulation was: VAD insertion plus application of bilateral pulmonary bands, stent placement in the arterial duct, and atrial septectomy, if needed. PALLIATION+VAD for patients with ductal-dependent pulmonary circulation was: VAD insertion plus stent placement in the arterial duct or systemic-to-pulmonary artery shunt with pulmonary arterioplasty, if needed. RESULTS: At PALLIATION+VAD, median age was 20 days (range, 4-143 days) and median weight was 3.47 kg (range, 2.43-4.86 kg). Ten patients (67%) survived and 5 patients (33%) died. All ten survivors are at home doing well after successful transplantation. Only 2 of 10 survivors (20%) required intubation >10 days after PALLIATION+VAD. Median length of VAD support for all 15 patients was 138 days (range, 56-226 days). CONCLUSIONS: High-risk neonates with functionally univentricular hearts who are suboptimal candidates for conventional palliation can be successfully stabilized with pulsatile VAD insertion along with initial palliation while awaiting cardiac transplantation; these patients may be extubated, enterally nourished, and optimized for transplantation while on VAD.

A Comprehensive Approach to the Management of Patients With HLHS and Related Malformations: An Analysis of 83 Patients (2015-2021).

Background: Some patients with hypoplastic left heart syndrome (HLHS) and HLHS-related malformations with ductal-dependent systemic circulation are extremely high-risk for Norwood palliation. We report our comprehensive approach to the management of these patients designed to maximize survival and optimize the utilization of donor hearts. Methods: We reviewed our entire current single center experience with 83 neonates and infants with HLHS and HLHS-related malformations (2015-2021). Standard-risk patients (n = 62) underwent initial Norwood (Stage 1) palliation. High-risk patients with risk factors other than major cardiac risk factors (n = 9) underwent initial Hybrid Stage 1 palliation, consisting of application of bilateral pulmonary bands, stent placement in the patent arterial duct, and atrial septectomy if needed. High-risk patients with major cardiac risk factors (n = 9) were bridged to transplantation with initial combined Hybrid Stage 1 palliation and pulsatile ventricular assist device (VAD) insertion (HYBRID + VAD). Three patients were bridged to transplantation with prostaglandin. Results: Overall survival at 1 year = 90.4% (75/83). Operative Mortality for standard-risk patients undergoing initial Norwood (Stage 1) Operation was 2/62 (3.2%). Of 60 survivors: 57 underwent Glenn, 2 underwent biventricular repair, and 1 underwent cardiac transplantation. Operative Mortality for high-risk patients with risk factors other than major cardiac risk factors undergoing initial Hybrid Stage 1 palliation without VAD was 0/9: 4 underwent transplantation, 1 awaits transplantation, 3 underwent Comprehensive Stage 2 (with 1 death), and 1 underwent biventricular repair. Of 9 HYBRID + VAD patients, 6 (67%) underwent successful cardiac transplantation and are alive today and 3 (33%) died while awaiting transplantation on VAD. Median length of VAD support was 134 days (mean = 134, range = 56-226). Conclusion: A comprehensive approach to the management of patients with HLHS or HLHS-related malformations is associated with Operative Mortality after Norwood of 2/62 = 3.2% and a one-year survival of 75/83 = 90.4%. A subset of 9/83 patients (11%) were stabilized with HYBRID + VAD while awaiting transplantation. VAD facilitates survival on the waiting list during prolonged wait times.