Outcomes After Decompression of the Right Ventricle in Infants With Pulmonary Atresia With Intact Ventricular Septum Are Associated With Degree of Tricuspid Regurgitation

Petit, Christopher J.; Glatz, Andrew C.; Qureshi, Athar M.; Sachdeva, Ritu; Maskatia, Shiraz A.; Justino, Henri; Goldberg, David J.; Mozumdar, Namrita; Whiteside, Wendy; Rogers, Lindsay S.; Nicholson, George T.; McCracken, Courtney; Kelleman, Mike; Goldstein, Bryan H.

doi:10.1161/circinterventions.116.004428

Cited by 48 publications

(69 citation statements)

References 25 publications

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“…Over the study period, 103 neonates underwent RV decompression for PA‐IVS. Patient characteristics, procedural details and outcomes were previously reported . In brief, there were two procedural deaths.…”

Section: Resultsmentioning

confidence: 99%

“…We performed a case control study to assess echocardiographic parameters associated with achievement of a 2V circulation and with RV growth. This was a post hoc analysis of a previously performed study of clinical outcomes in patients with PA/IVS who underwent RV decompression in the neonatal period . The patient population consisted of all infants with PA/IVS who underwent RV decompression at less than 30 days of age between January 1, 2005 and May 1, 2015 at four large pediatric centers (Children’s Healthcare of Atlanta, Children’s Hospital of Philadelphia, Cincinnati Children’s Hospital Medical Center, and Texas Children’s Hospital).…”

Section: Methodsmentioning

confidence: 99%

“…Regular web‐based teleconferences were held during which example studies were reviewed in an attempt to generate “core laboratory” standards without necessitating the use of a single echocardiographic core laboratory. Interobserver reliability testing was performed for measurement of TV annulus diameter and TR grade as part of the initial study, and demonstrated excellent agreement between observers for both measures …”

Section: Methodsmentioning

confidence: 99%

“…Because baseline echocardiographic parameters associated with achieving a 2V circulation had been previously reported in our initial study, we did not repeat these analyses. We did generate an receiver operating characteristic (ROC) curve of baseline RV area and achievement of 2V circulation as this had not been previously performed.…”

Section: Methodsmentioning

confidence: 99%

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Echocardiographic parameters associated with biventricular circulation and right ventricular growth following right ventricular decompression in patients with pulmonary atresia and intact ventricular septum: Results from a multicenter study

Maskatia

Petit

Travers

et al. 2018

Congenital Heart Disease

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Background In patients with pulmonary atresia, intact ventricular septum (PA/IVS) following right ventricular (RV) decompression, RV size and morphology drive clinical outcome. Our objectives were to (1) identify baseline and postdecompression echocardiographic parameters associated with 2V circulation, (2) identify echocardiographic parameters associated with RV growth and (3) describe changes in measures of RV size and changes in RV loading conditions. Methods We performed a retrospective analysis of patients who underwent RV decompression for PA/IVS at four centers. We analyzed echocardiograms at baseline, postdecompression, and at follow up (closest to 1‐year or prior to Glenn circulation). Results Eighty‐one patients were included. At last follow‐up, 70 (86%) patients had 2V circulations, 7 (9%) had 1.5 ventricle circulations, and 4 (5%) had single ventricle circulations. Follow‐up echocardiograms were available in 43 (53%) patients. The majority of patients had improved RV systolic function, less tricuspid regurgitation (TR), and more left‐to‐right atrial shunting at a median of 350 days after decompression. Multivariable analysis demonstrated that larger baseline tricuspid valve (TV) z‐score (P = .017), ≥ moderate baseline TR (P = .045) and smaller baseline RV area (P < .001) were associated with larger increases in RV area. Baseline RV area ≥6 cm2/m2 had 93% sensitivity and 80% specificity for identifying patients who ultimately achieved 2V circulation. All patients with RV area ≥8 cm2/m2 at follow up achieved 2V circulation. This finding was confirmed in a validation cohort from a separate center (N = 25). Factors associated with achieving RV area ≥8 cm2/m2 included larger TV z‐score (P = .004), ≥ moderate baseline TR (P = .031), and ≥ moderate postdecompression pulmonary regurgitation (P = .002). Conclusions Patients with PA/IVS and smaller TV annuli are at risk for poor RV growth. Volume‐loading conditions signal increased capacity for growth sufficient for 2V circulation.

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Section: Resultsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

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Echocardiographic parameters associated with biventricular circulation and right ventricular growth following right ventricular decompression in patients with pulmonary atresia and intact ventricular septum: Results from a multicenter study

Maskatia

Petit

Travers

et al. 2018

Congenital Heart Disease

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“…Previous reports on PA-IVS had largely been limited to single-center, small cohort studies, and many of the larger series were hindered by a broad span of inclusion dates (some spanning multiple decades of institutional experience), or very small cohorts where more contemporary approaches were employed but statistical power was wanting ( Figure 1). 17 Additional analyses led to an enhanced understanding of the importance of nuanced technical variables on outcomes. We evaluated a host of echocardiographic and hemodynamic factors and identified that preintervention tricuspid regurgitation was associated with a host of important clinical end points following right ventricle decompression in neonates with PA-IVS.…”

Section: His Torymentioning

confidence: 99%

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

Petit

Qureshi

Glatz

et al. 2019

Congenital Heart Disease

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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 Individual University affiliate Hospital affiliate Membership Abstract Presentation at Society for Cardiovascular Angiography and Interventions 2018 Comparison of ductal 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 39Abstract Presentation at American Heart Association 2018Differences in somatic growth and mode of feeding by palliation strategy for infants with ductal-dependent pulmonary blood flow: results from the congenital catheterization research collaborative 18Abstract Presentation at American Heart Association 2018Comparison of outcomes at time of superior cavopulmonary connection between single ventricle patients with ductal-dependent pulmonary blood flow initially palliated with either Blalock-Taussig

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Classification scheme for ductal morphology in cyanotic patients with ductal dependent pulmonary blood flow and association with outcomes of patent ductus arteriosus stenting

Qureshi

Goldstein

Glatz

et al. 2019

Cathet Cardio Intervent

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Objectives To devise a classification scheme for ductal morphology in patients with ductal dependent pulmonary blood flow (PBF) that can be used to assess outcomes. Background The impact of ductal morphology on outcomes following patent ductus arteriosus (PDA) stenting is not well defined. Methods Patients <1 year of age who underwent PDA stenting for ductal dependent PBF at the four centers comprising the Congenital Catheterization Research Collaborative (CCRC) were included. A classification scheme for PDA morphology was devised based on a tortuosity index (TI)—Type I (straight), Type II (one turn), and Type III (multiple turns). A subtype classification was used based upon the ductal origin. Results One hundred and five patients underwent PDA stenting. TI was Type I in 58, Type II in 24, and Type III in 23 PDAs, respectively. There was a significant association between ductal origin and vascular access site (p < 0.001). Procedure times and need for >1 stent did not differ based on TI. Greater TI was associated with pulmonary artery (PA) jailing (p = 0.003). Twelve (11.4%) patients underwent unplanned reintervention, more commonly with greater TI (p = 0.022) and PA jailing (p < 0.001). At the time of subsequent surgical repair/palliative staging, PA arterioplasty was performed in 32 patients, more commonly when a PA was jailed (p = 0.048). PA jailing did not affect PA size at follow up. Conclusions The proposed qualitative and quantitative PDA morphology classification scheme may be helpful in anticipating outcomes in patients with ductal dependent PBF undergoing PDA stenting.

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Outcomes After Decompression of the Right Ventricle in Infants With Pulmonary Atresia With Intact Ventricular Septum Are Associated With Degree of Tricuspid Regurgitation

Cited by 48 publications

References 25 publications

Echocardiographic parameters associated with biventricular circulation and right ventricular growth following right ventricular decompression in patients with pulmonary atresia and intact ventricular septum: Results from a multicenter study

Echocardiographic parameters associated with biventricular circulation and right ventricular growth following right ventricular decompression in patients with pulmonary atresia and intact ventricular septum: Results from a multicenter study

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

Classification scheme for ductal morphology in cyanotic patients with ductal dependent pulmonary blood flow and association with outcomes of patent ductus arteriosus stenting

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