Comparison of management strategies for neonates with symptomatic tetralogy of Fallot and weight <2.5 kg.

2021

2021 Feb 03

1097-685X

OBJECTIVE: To compare management strategies for neonates <2.5 kg with tetralogy of Fallot and symptomatic cyanosis who either undergo staged repair (SR) (initial palliation followed by later complete repair) or primary repair (PR).

METHODS: Consecutive neonates with tetralogy of Fallot and symptomatic cyanosis weighing <2.5 kg at initial intervention and between 2005 and 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. Primary outcome was mortality and secondary outcomes included component (eg, initial palliation, complete repair, or primary repair) and cumulative (SR: initial palliation followed by later complete repair) hospital and intensive care unit lengths of stay, durations of ventilation, inotrope use, cardiopulmonary bypass time, procedural complications, and reintervention. Outcomes were compared with propensity score adjustments with PR as the reference group.

RESULTS: The cohort included 76 SR (initial palliation: 53 surgical and 23 transcatheter) and 44 PR patients. The observed risk of overall mortality was similar between SR and PR groups (15.8% vs 18.2%: P = .735). The adjusted hazard of mortality remained similar between groups overall (hazard ratio, 0.59; 95% confidence interval, 0.26-1.36; P = .214), as well as during short-term (<4 months: hazard ratio, 0.37; 95% confidence interval, 0.13-1.09; P = .071) and midterm (>4 months: hazard ratio, 1.32; 95% confidence interval, 0.30-5.79; P = .717) follow-up. Reintervention in the first 18 months was common in both groups (53.2% vs 48.4%; hazard ratio, 1.69; 95% confidence interval, 0.96-2.28; P = .072). Adjusted procedural complications and neonatal morbidity burden were overall lower in the SR group. Cumulative secondary outcome burdens largely favored the PR group.

CONCLUSIONS: In this study comparing SR and PR treatment strategies for neonates with tetralogy of Fallot and symptomatic cyanosis and weight <2.5 kg, mortality and reintervention burden was highly independent of treatment strategy. Other potential advantages were observed with each approach.

10.1016/j.jtcvs.2021.01.100

J Thorac Cardiovasc Surg

33726912

Comparative Costs of Management Strategies for Neonates With Symptomatic Tetralogy of Fallot.

2022

1170-1180

2022 Mar 29

1558-3597

BACKGROUND: Recent data have demonstrated that overall mortality and adverse events are not significantly different for primary repair (PR) and staged repair (SR) approaches to management of neonates with symptomatic tetralogy of Fallot (sTOF). Cost data can be used to compare the relative value (cost for similar outcomes) of these approaches and are a potentially more sensitive measure of morbidity.

OBJECTIVES: This study sought to compare the economic costs associated with PR and SR in neonates with sTOF.

METHODS: Data from a multicenter retrospective cohort study of neonates with sTOF were merged with administrative data to compare total costs and cost per day alive over the first 18 months of life in a propensity score-adjusted analysis. A secondary analysis evaluated differences in department-level costs.

RESULTS: In total, 324 subjects from 6 centers from January 2011 to November 2017 were studied (40% PR). The 18-month cumulative mortality (P = 0.18), procedural complications (P = 0.10), hospital complications (P = 0.94), and reinterventions (P = 0.22) did not differ between PR and SR. Total 18-month costs for PR (median $179,494 [IQR: $121,760-$310,721]) were less than for SR (median: $222,799 [IQR: $167,581-$327,113]) (P < 0.001). Cost per day alive (P = 0.005) and department-level costs were also all lower for PR. In propensity score-adjusted analyses, PR was associated with lower total cost (cost ratio: 0.73; P < 0.001) and lower department-level costs.

CONCLUSIONS: In this multicenter study of neonates with sTOF, PR was associated with lower costs. Given similar overall mortality between treatment strategies, this finding suggests that PR provides superior value.

10.1016/j.jacc.2021.12.036

J Am Coll Cardiol

35331412

Factors Influencing Reintervention Following Ductal Artery Stent Implantation for Ductal-Dependent Pulmonary Blood Flow: Results From the Congenital Cardiac Research Collaborative.

2021

CIRCINTERVENTIONS120010086

2021 Nov 18

1941-7632

<p><strong>BACKGROUND: </strong>Stenting of the patent ductus arteriosus (PDA) is an established palliative option for infants with ductal-dependent pulmonary blood flow. Following initial palliation, reintervention on the PDA stent is common, but risk factors have not been characterized.</p>

<p><strong>METHODS: </strong>Infants with ductal-dependent pulmonary blood flow palliated with PDA stent between 2008 and 2015 were reviewed within the Congenital Cardiac Research Collaborative. Rates and risk factors for reintervention were analyzed.</p>

<p><strong>RESULTS: </strong>Among 105 infants who underwent successful PDA stenting, 41 patients (39%) underwent a total of 53 reinterventions on the PDA stent, with all but one occurring within 6 months of the initial intervention. Stent redilation constituted the majority of reintervention (n=35; 66%) followed by additional stent placement (n=11; 21%) and surgical shunt placement (n=7; 13%). The majority of reintervention was nonurgent, and there were no deaths during the reintervention procedure. All but one reintervention occurred within 6 months of the initial procedure. On univariate analysis, risk factors for reintervention included anticipated single-ventricle physiology, lack of prior balloon pulmonary valvuloplasty, use of drug-eluting stent, and increased ductal tortuosity.</p>

<p><strong>CONCLUSIONS: </strong>In infants with ductal-dependent pulmonary blood flow palliated with PDA stent implantation, reintervention is common, can be performed safely, and is associated with both anatomic/procedural factors and anticipated final physiology.</p>

10.1161/CIRCINTERVENTIONS.120.010086

Circ Cardiovasc Interv

34789017

Comparison of Patent Ductus Arteriosus Stent and Blalock-Taussig Shunt as Palliation for Neonates with Sole Source Ductal-Dependent Pulmonary Blood Flow: Results from the Congenital Catheterization Research Collaborative.

2021

2021 Sep 15

1432-1971

<p>Patent ductus arteriosus (PDA) stenting is an accepted method for securing pulmonary blood flow in cyanotic neonates. In neonates with pulmonary atresia and single source ductal-dependent pulmonary blood flow (SSPBF), PDA stenting remains controversial. We sought to evaluate outcomes in neonates with SSPBF, comparing PDA stenting and surgical Blalock-Taussig shunt (BTS). Neonates with SSPBF who underwent PDA stenting or BTS at the four centers of the Congenital Catheterization Research Collaborative from January 2008 to December 2015 were retrospectively reviewed. Reintervention on the BTS or PDA stent prior to planned surgical repair served as the primary endpoint. Additional analyses of peri-procedural complications, interventions, and pulmonary artery growth were performed. A propensity score was utilized to adjust for differences in factors. Thirty-five patients with PDA stents and 156 patients with BTS were included. The cohorts had similar baseline characteristics, procedural complications, and mortality. Interstage reintervention rates were higher in the PDA stent cohort (48.6% vs. 15.4%, p &lt; 0.001).</p>

10.1007/s00246-021-02699-7

Pediatr Cardiol

34524483

Comparison of Management Strategies for Neonates With Symptomatic Tetralogy of Fallot.

2021

1093-1106

2021 Mar 02

1558-3597

<p><strong>BACKGROUND: </strong>Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention.</p>

<p><strong>OBJECTIVES: </strong>This study sought to perform a balanced multicenter comparison of staged repair (SR) (initial palliation [IP] and subsequent complete repair [CR]) versus primary repair (PR) treatment strategies.</p>

<p><strong>METHODS: </strong>Consecutive neonates with sTOF who underwent IP or PR at&nbsp;≤30&nbsp;days of age from 2005 to 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. The primary outcome was death. Secondary outcomes included component (IP, CR, PR) and cumulative (SR): hospital and intensive care unit lengths of stay; durations of cardiopulmonary bypass, anesthesia, ventilation, and inotrope use; and complication and reintervention rates. Outcomes were compared using propensity score adjustment.</p>

<p><strong>RESULTS: </strong>The cohort consisted of 342 patients who underwent SR (IP: surgical, n&nbsp;=&nbsp;256; transcatheter, n&nbsp;=&nbsp;86) and 230 patients who underwent PR. Pre-procedural ventilation, prematurity, DiGeorge syndrome, and pulmonary atresia were more common in the SR group (p&nbsp;≤0.01). The observed risk of death was not different between the groups (10.2% vs 7.4%; p&nbsp;=&nbsp;0.25) at median 4.3 years. After adjustment, the hazard of death remained similar between groups (hazard ratio: 0.82; 95% confidence interval: 0.49 to 1.38; p&nbsp;=&nbsp;0.456), but it favored SR during early follow-up (&lt;4&nbsp;months; p&nbsp;=&nbsp;0.041). Secondary outcomes favored the SR group in component analysis, whereas they largely favored PR in cumulative analysis. Reintervention risk was higher in the SR group (p&nbsp;=&nbsp;0.002).</p>

<p><strong>CONCLUSIONS: </strong>In this multicenter comparison of SR or PR for management of neonates with sTOF, adjusted for patient-related factors, early mortality and neonatal morbidity were lower in the SR group, but cumulative morbidity and reinterventions favored the PR group, findings suggesting potential benefits to each strategy.</p>

10.1016/j.jacc.2020.12.048

J Am Coll Cardiol

33632484

Impact of Treatment Strategy on Outcomes in Isolated Pulmonary Artery of Ductal Origin.

2021

2021 Jan 04

1432-1971

<p>Isolated pulmonary artery (PA) of ductal origin (IPADO) is a rare cardiac defect which requires surgical repair, with or without preceding palliation. We sought to determine the impact of treatment strategy on outcomes. Retrospective study of consecutive patients with IPADO that underwent staged or primary repair from 1/05 to 9/16 at 6 Congenital Cardiac Research Collaborative centers. Patients with single ventricle physiology, major aortopulmonary collaterals, or bilateral IPADO were excluded. Primary outcome was isolated PA z-score at late follow-up. Secondary outcomes included PA symmetry index (isolated:confluent PA diameter) and reintervention burden. Propensity score adjustment was used to account for baseline differences. Of 60 patients in the study cohort, 26 (43%) underwent staged and 34 (57%) primary repair. The staged and primary repair groups differed in weight at diagnosis and presence of other heart disease but not in baseline PA dimensions. Staged patients underwent ductal stent (n = 16) or surgical shunt (n = 10) placement followed by repair at 210 vs. 21 days in the primary repair group (p &lt; 0.001). At median follow-up of 4.5 years post-repair, after adjustment, isolated PA z-score (- 0.74 [- 1.75, - 0.26] vs. - 1.95 [- 2.91, - 1.59], p = 0.012) and PA symmetry index (0.81 [0.49, 1.0] vs. 0.55 [0.48, 0.69], p = 0.042) significantly favored the staged repair group. Freedom from PA reintervention was not different between groups (adjusted HR 0.78 [0.41, 1.48]; p = 0.445). A staged approach to repair of IPADO is associated with superior isolated PA size and symmetry at late follow-up. Consideration should be given to initial palliation in IPADO patients, when feasible.</p>

10.1007/s00246-020-02511-y

Pediatr Cardiol

33394118

Use of carotid and axillary artery approach for stenting the patent ductus arteriosus in infants with ductal-dependent pulmonary blood flow: A multicenter study from the congenital catheterization research collaborative.

2019

2019 Dec 09

1522-726X

<p><strong>BACKGROUND: </strong>Carotid artery (CA) and axillary artery (AA) access are increasingly used for transcatheter stenting of the patent ductus arteriosus (PDA), although reports are limited.</p>

<p><strong>METHODS: </strong>The Congenital Catheterization Research Collaborative (CCRC) reviewed multicenter data from infants who underwent PDA stenting via the CA or AA approach from 2008 to 2017, and compared outcomes to those of infants undergoing PDA stenting via the femoral artery (FA) approach. Post-procedure ultrasound (US) imaging was reviewed.</p>

<p><strong>RESULTS: </strong>Forty-nine infants underwent PDA stenting from the CA (n = 43) or AA (n = 6) approach, compared with 55 infants who underwent PDA stenting from the FA approach. The PDA was the sole pulmonary blood flow (PBF) source in 61% of infants in the CA/AA cohort, compared with 33% of the FA cohort (p &lt; .01). Ductal tortuosity for CA/AA cohort was Type I (straight) in 10 (20%), Type II (one turn) in 17 (35%), and Type III (multiple turns) in 22 (45%) infants and reflected a greater degree of tortuosity when compared to the FA cohort (p &lt; .01). In 17 infants with CA/AA approach, the "flip technique" was used, and was associated with shorter procedure times for highly tortuous PDA (Type III) patients. Rates of procedural complications were similar across access sites. Most common complications were access site injury (thrombus or bleeding) and stent malposition. No complications were specifically related to the "flip technique."</p>

<p><strong>CONCLUSIONS: </strong>Use of CA and AA approach for PDA stenting was found to be more commonly employed in sole source PBF and highly tortuous PDAs. Procedural modifications such as the "flip technique" may lead to shorter procedure times. CA and AA approaches are associated with a similar burden of procedural or late complications. Post-procedural surveillance of the CA and AA is suggested, given the incidence of vascular findings on US.</p>

10.1002/ccd.28631

Catheter Cardiovasc Interv

31815357

Comparison of Outcomes at Time of Superior Cavopulmonary Connection Between Single Ventricle Patients With Ductal-Dependent Pulmonary Blood Flow Initially Palliated With Either Blalock-Taussig Shunt or Ductus Arteriosus Stent

2019

e008110

2019 Oct

1941-7632

<p><strong>BACKGROUND: </strong>Patients with single ventricle anatomy and ductal-dependent pulmonary blood flow may be initially palliated with either modified Blalock-Taussig shunt (BTS) or ductus arteriosus stent (DAS). Comparisons of outcomes during the interstage period and at the time of superior cavopulmonary connection (SCPC) are lacking and may differ between palliation strategies.</p>

<p><strong>METHODS: </strong>Infants with single ventricle anatomy and ductal-dependent pulmonary blood flow palliated with either DAS or BTS from 2008 to 2015 were reviewed across 4 centers. Interstage outcomes, and for those who had SCPC, anatomy, hemodynamics, and perioperative clinical outcomes were compared. Thirty-five patients with DAS and 136 patients with BTS were included.</p>

<p><strong>RESULTS: </strong>At initial palliation, demographic, clinical variables, and pulmonary artery size were similar. Interstage death, transplant, or unplanned reintervention to treat cyanosis occurred in 25.7% of DAS and 35.8% of BTS, =0.27. Reintervention was more common with DAS (48.6% versus 2.2%; &lt;0.001). Twenty-three DAS patients and 111 BTS patients underwent SCPC. Preoperative hemodynamics and overall pulmonary atresia growth were similar, although right pulmonary artery growth was better with DAS (change in -score: 1.57 versus 0.65, =0.026). SCPC intraoperative and postoperative courses were similar.</p>

<p><strong>CONCLUSIONS: </strong>In patients with single-ventricle anatomy and ductal-dependent pulmonary blood flow, interstage outcomes, hemodynamics before SCPC, and acute postoperative outcomes were similar. Overall reintervention was more common in the DAS group, driven by more frequent planned reintervention. Unplanned reintervention, death, and transplant were similar. Both groups demonstrated good pulmonary atresia growth. DAS is a reasonable initial palliative alternative to BTS in select patients.</p>

10.1161/CIRCINTERVENTIONS.119.008110

Circ Cardiovasc Interv

31607156

Comprehensive comparative outcomes in children with congenital heart disease: The rationale for the Congenital Catheterization Research Collaborative.

2019

341-349

2019 May

1747-0803

<p>Clinical research in the treatment of patients with congenital heart disease (CHD) is limited by the wide variety of CHD manifestations and therapeutic options as well as the generally low incidence of CHD. The availability of comprehensive, contemporary outcomes studies is therefore limited. This inadequacy may result in a lack of data-driven medical decision making. In 2013, clinician scientists at two centers began a research collaboration, the Congenital Catheterization Research Collaborative (CCRC). Over time, the CCRC has grown to include nine cardiac centers from across the United States, with a common data coordinating center. The CCRC seeks to generate high-quality, contemporary, statistically robust, and generalizable outcomes research which can help address important clinical questions in the treatment of CHD. To date, the CCRC has reported on multicenter outcomes in: neonates with congenital aortic stenosis, infants undergoing right ventricular decompression for pulmonary atresia and intact ventricular septum, and infants with ductal-dependent pulmonary blood flow. The CCRC has been successful at leveraging large multicenter cohorts of patients in a contemporary period to perform comparative studies. In the future, the CCRC plans to continue to perform hypothesis-driven retrospective and prospective observational studies of CHD populations where controversy exists or where novel interventions or therapies have emerged. Quality improvement efforts including lesion-specific registry development may be an additional potential future target.</p>

10.1111/chd.12737

Congenit Heart Dis

31183955

Differences in Cost of Care by Palliation Strategy for Infants With Ductal-Dependent Pulmonary Blood Flow.

2019

e007232

2019 Apr

1941-7632

<p><strong>BACKGROUND: </strong>In infants with ductal-dependent pulmonary blood flow, initial palliation with patent ductus arteriosus (PDA) stent or modified Blalock-Taussig (BT) shunt have comparable mortality but discrepant length of stay, procedural complication rates and reintervention burdens, which may influence cost. The relative economic impact of these palliation strategies is unknown.</p>

<p><strong>METHODS AND RESULTS: </strong>Retrospective study of infants with ductal-dependent pulmonary blood flow palliated with PDA stent (n=104) or BT shunt (n=251) from 2008 to 2015 at 4 centers of the Congenital Catheterization Research Collaborative. Inflation-adjusted inpatient hospital costs were calculated for first year of life using Pediatric Health Information System data. Costs derived from outpatient catheterizations not in Pediatric Health Information System were imputed. Costs were compared using propensity score-adjusted multivariable models, to account for baseline differences between groups. After propensity score adjustment, first year of life costs were significantly lower in PDA stent ($215 825 [190 644-244 333]) than BT shunt ($249 855 [230 693-270 609]) patients ( P=0.05). After addition of imputed costs, first year of life costs were not significantly different between PDA stent ($226 403 [200 274-255 941]) and BT shunt ($252 072 [232 955-272 759]) groups ( P=0.15). Patient characteristics associated with higher costs included: younger gestational age, genetic syndrome, noncardiac diagnoses, procedural complications, extracorporeal membrane oxygenation, duration of ventilation, intensive care unit and hospital length of stay and reintervention ( P≤0.02 for all).</p>

<p><strong>CONCLUSIONS: </strong>In this first multicenter comparative cost study of PDA stent or BT shunt as palliation for infants with ductal-dependent pulmonary blood flow, adjusted for baseline differences, PDA stent was associated with lower to equivalent costs over the first year of life. Combined with previous evidence suggesting clinical noninferiority, these findings suggest that PDA stent provides competitive health care value.</p>

10.1161/CIRCINTERVENTIONS.118.007232

Circ Cardiovasc Interv

30998390