Leading initial
J
First name
William
Last name
Gaynor

Title

Fetal growth and gestational age improve outcome predictions in neonatal heart surgery.

Year of Publication

2022

Date Published

05/2022

ISSN Number

1097-685X

Abstract

OBJECTIVE: Current risk adjustment models for congenital heart surgery do not fully incorporate multiple factors unique to neonates such as granular gestational age (GA) and birth weight (BW) z score data. This study sought to develop a Neonatal Risk Adjustment Model for congenital heart surgery to address these deficiencies.

METHODS: Cohort study of neonates undergoing cardiothoracic surgery during the neonatal period captured in the Pediatric Cardiac Critical Care Consortium database between 2014 and 2020. Candidate predictors were included in the model if they were associated with mortality in the univariate analyses. GA and BW z score were both added as multicategory variables. Mortality probabilities were predicted for different GA and BW z scores while keeping all other variables at their mean value.

RESULTS: The C statistic for the mortality model was 0.8097 (95% confidence interval, 0.7942-0.8255) with excellent calibration. Mortality prediction for a neonate at 40 weeks GA and a BW z score 0 to 1 was 3.5% versus 9.8% for the same neonate at 37 weeks GA and a BW z score -2 to -1. For preterm infants the mortality prediction at 34 to 36 weeks with a BW z score 0 to 1 was 10.6%, whereas it was 36.1% for the same infant at <32 weeks with a BW z score of -2 to -1.

CONCLUSIONS: This Neonatal Risk Adjustment Model incorporates more granular data on GA and adds the novel risk factor BW z score. These 2 factors refine mortality predictions compared with traditional risk models. It may be used to compare outcomes across centers for the neonatal population.

DOI

10.1016/j.jtcvs.2022.05.022

Alternate Title

J Thorac Cardiovasc Surg

PMID

35750509

Title

Impact of Maternal-Fetal Environment on Mortality in Children With Single Ventricle Heart Disease.

Year of Publication

2022

Number of Pages

e020299

Date Published

2022 Jan 18

ISSN Number

2047-9980

Abstract

<p>BACKGROUND Children with single ventricle heart disease have significant morbidity and mortality. The maternal-fetal environment (MFE) may adversely impact outcomes after neonatal cardiac surgery. We hypothesized that impaired MFE would be associated with an increased risk of death after stage 1 Norwood reconstruction. METHODS AND RESULTS We performed a retrospective cohort study of children with hypoplastic left heart syndrome (and anatomic variants) who underwent stage 1 Norwood reconstruction between 2008 and 2018. Impaired MFE was defined as maternal gestational hypertension, preeclampsia, gestational diabetes, and/or smoking during pregnancy. Cox proportional hazards regression models were used to investigate the association between impaired MFE and death while adjusting for confounders. Hospital length of stay was assessed with the competing risk of in-hospital death. In 273 children, the median age at stage 1 Norwood reconstruction was 4&nbsp;days (interquartile range [IQR], 3-6 days). A total of 72 children (26%) were exposed to an impaired MFE; they had more preterm births (18% versus 7%) and a greater percentage with low birth weights &lt;2.5&nbsp;kg (18% versus 4%) than those without impaired MFE. Impaired MFE was associated with a higher risk of death (hazard ratio [HR], 6.05; 95% CI, 3.59-10.21; &lt;0.001) after adjusting for age at surgery, Hispanic ethnicity, genetic syndrome, cardiac diagnosis, surgeon, and birth era. Children with impaired MFE had almost double the risk of prolonged hospital stay (HR, 1.95; 95% CI, 1.41-2.70; &lt;0.001). CONCLUSIONS Children exposed to an impaired MFE had a higher risk of death following stage 1 Norwood reconstruction. Prenatal exposures are potentially modifiable factors that can be targeted to improve outcomes after pediatric cardiac surgery.</p>

DOI

10.1161/JAHA.120.020299

Alternate Title

J Am Heart Assoc

PMID

35014861

Title

Abnormalities in the Von Willebrand-Angiopoietin Axis Contribute to Dysregulated Angiogenesis and Angiodysplasia in Children With a Glenn Circulation.

Year of Publication

2021

Number of Pages

222-235

Date Published

2021 Mar

ISSN Number

2452-302X

Abstract

<p>Children with a bidirectional superior cavopulmonary (Glenn) circulation develop angiodysplasia and pulmonary arteriovenous malformations (AVMs). The von Willebrand factor (vWF)-angiopoietin axis plays a major role in AVM formation in multiple diseases. We observed derangements in global angiogenic signaling, vWF metabolism, angiopoietins, and in&nbsp;vitro angiogenesis in children with a Glenn circulation versus controls and within Glenn pulmonary versus systemic circulations. These findings support the novel hypothesis that abnormalities in the vWF-angiopoietin axis may dysregulate angiogenesis and contribute to Glenn pulmonary AVMs. The vWF-angiopoietin axis may be a target to correct angiogenic imbalance in Glenn patients, for whom no targeted therapy exists.</p>

DOI

10.1016/j.jacbts.2020.12.014

Alternate Title

JACC Basic Transl Sci

PMID

33778210

Title

Genetic variant burden and adverse outcomes in pediatric cardiomyopathy.

Year of Publication

2020

Date Published

2020 Aug 03

ISSN Number

1530-0447

Abstract

<p><strong>BACKGROUND: </strong>Previous genetic research in pediatric cardiomyopathy (CM) has focused on pathogenic variants for diagnostic purposes, with limited data evaluating genotype-outcome correlations. We explored whether greater genetic variant burden (pathogenic or variants of unknown significance, VUS) correlates with worse outcomes.</p>

<p><strong>METHODS: </strong>Children with dilated CM (DCM) and hypertrophic CM (HCM) who underwent multigene testing between 2010 and 2018 were included. Composite endpoint was freedom from major adverse cardiac event (MACE).</p>

<p><strong>RESULTS: </strong>Three hundred and thirty-eight subjects were included [49% DCM, median age 5.7 (interquartile range (IQR) 0.2-13.4) years, 51% HCM, median age 3.0 (IQR 0.1-12.5) years]. Pathogenic variants alone were not associated with MACE in either cohort (DCM p = 0.44; HCM p = 0.46). In DCM, VUS alone [odds ratio (OR) 4.0, 95% confidence interval (CI) 1.9-8.3] and in addition to pathogenic variants (OR 5.2, 95% CI 1.7-15.9) was associated with MACE. The presence of VUS alone or in addition to pathogenic variants were not associated with MACE in HCM (p = 0.22 and p = 0.33, respectively).</p>

<p><strong>CONCLUSION: </strong>Increased genetic variant burden (pathogenic variants and VUS) is associated with worse clinical outcomes in DCM but not HCM. Genomic variants that influence DCM onset may be distinct from those driving disease progression, highlighting the potential value of universal genetic testing to improve risk stratification.</p>

<p><strong>IMPACT: </strong>In pediatric CM, inconsistent findings historically have been shown between genotype and phenotype severity when only pathogenic variants have been considered.Increased genetic variant burden (including both pathogenic variants and VUS) is associated with worse clinical outcomes in DCM but not HCM.Genomic variants that influence CM onset may be distinct from those variants that drive disease progression and influence outcomes in phenotype-positive individuals.Incorporation of both pathogenic variants and VUS may improve risk stratification models in pediatric CM.</p>

DOI

10.1038/s41390-020-1101-5

Alternate Title

Pediatr. Res.

PMID

32746448

Title

Preoperative Clinical and Echocardiographic Factors Associated with Surgical Timing and Outcomes in Primary Repair of Common Atrioventricular Canal Defect.

Year of Publication

2019

Date Published

2019 May 07

ISSN Number

1432-1971

Abstract

<p>In complete atrioventricular canal defect (CAVC), there are limited data on preoperative clinical and echocardiographic predictors of operative timing and postoperative outcomes. A retrospective, single-center analysis of all patients who underwent primary biventricular repair of CAVC between 2006 and 2015 was performed. Associated cardiac anomalies (tetralogy of Fallot, double outlet right ventricle) and arch operation were excluded. Echocardiographic findings on first postnatal echocardiogram were correlated with surgical timing and postoperative outcomes using bivariate descriptive statistics and multivariable logistic regression. 153 subjects (40% male, 84% Down syndrome) underwent primary CAVC repair at a median age of 3.3 (IQR 2.5-4.2) months. Median postoperative length of stay (LOS) was 7 (IQR 5-15) days. Eight patients (5%) died postoperatively and 24 (16%) required reoperation within 1&nbsp;year. On multivariable analysis, small aortic isthmus (z score &lt; -&nbsp;2) was associated with early primary repair at &lt; 3&nbsp;months (OR 2.75, 95% CI 1.283-5.91) and need for early reoperation (OR 3.79, 95% CI 1.27-11.34). Preoperative ventricular dysfunction was associated with higher postoperative mortality (OR 7.71, 95% CI 1.76-33.69). Other factors associated with mortality and longer postoperative LOS were prematurity (OR 5.30, 95% CI 1.24-22.47 and OR 5.50, 95% CI 2.07-14.59, respectively) and lower weight at surgery (OR 0.17, 95% CI 0.04-0.75 and OR 0.55, 95% CI 0.35-0.85, respectively). Notably, preoperative atrioventricular valve regurgitation and Down syndrome were not associated with surgical timing, postoperative outcomes or reoperation, and there were no echocardiographic characteristics associated with late reoperation beyond 1 year after repair. Key preoperative echocardiographic parameters helped predict operative timing and postoperative outcomes in infants undergoing primary CAVC repair. Aortic isthmus z score &lt; -&nbsp;2&nbsp; was associated with early surgical repair and need for reoperation, while preoperative ventricular dysfunction was associated with increased mortality. These echocardiographic findings may help risk-stratified patients undergoing CAVC repair and improve preoperative counseling and surgical planning.</p>

DOI

10.1007/s00246-019-02116-0

Alternate Title

Pediatr Cardiol

PMID

31065759

Title

Cumulative Effect of Preoperative Risk Factors on Mortality after Pediatric Heart Transplantation.

Year of Publication

2018

Date Published

2018 Apr 20

ISSN Number

1552-6259

Abstract

<p><strong>BACKGROUND: </strong>Risk assessment in heart transplantation is critical for candidate selection, but current models inadequately assess individual risk of postoperative mortality. We sought to identify risk factors and develop a scoring system to predict mortality following heart transplantation in children.</p>

<p><strong>METHODS: </strong>The records of patients undergoing heart transplantation at our institution from 2010 - 2016 were reviewed. Clinical characteristics were recorded and compared between survivors and non-survivors. Using Cox proportional hazard modeling, a risk factor score was developed using factors associated with postoperative mortality.</p>

<p><strong>RESULTS: </strong>Seventy-four patients underwent heart transplantation at a mean age of 8.8 ± 6.6 years. Congenital heart disease was the most common indication, comprising 48.6% of the cohort. Overall mortality was 18.9%, with 10/14 dying ≤30 days of operation or during initial postoperative admission (early mortality). The following preoperative factors were associated with overall mortality: single ventricle congenital heart disease (HR 3.2, p = 0.042), biVAD (HR 4.8, p = 0.043), history of ≥4 sternotomies (HR 3.9, p = 0.023), panel reactive antibody &gt; 10% (HR 4.4, p = 0.013), any previous surgery at an outside institution (HR 3.2, p = 0.038), and pulmonary vein disease (HR 4.7, p = 0.045). Each risk factor was assigned a point value, based on similar magnitude of the hazard ratios. A score of ≥4 predicted mortality with 57% sensitivity and 90% specificity.</p>

<p><strong>CONCLUSIONS: </strong>In this single-center pediatric cohort, post-heart transplantation mortality could be predicted using patient-specific risk factors. The cumulative effect of these risk factors predicted mortality with high specificity.</p>

DOI

10.1016/j.athoracsur.2018.03.044

Alternate Title

Ann. Thorac. Surg.

PMID

29684375

Title

Surgical and Catheter-Based Reinterventions Are Common in Long-Term Survivors of the Fontan Operation.

Year of Publication

2017

Date Published

2017 Sep

ISSN Number

1941-7632

Abstract

<p><strong>BACKGROUND: </strong>There are limited follow-up studies examining surgical and catheter-based reinterventions in long-term survivors of the Fontan operation.</p>

<p><strong>METHODS AND RESULTS: </strong>All 773 patients who underwent Fontan at our institution between 1992 and 2009 were retrospectively reviewed. Current information regarding post-Fontan intervention was available for 70%. By 20 years after Fontan, 65% of patients had experienced either surgical or transcatheter intervention. The median time to first reintervention was 9.8 years. Freedom from reoperation was 69% at 15 years and 63% at 20 years. The most common operations were pacemaker placement and Fontan revision. Risk factors for pacemaker placement included systemic left ventricle (hazard ratio [HR], 2.2; P=0.006) and lateral tunnel Fontan (HR, 4.3; P=0.001). Freedom from interventional catheterization was 53% at 15 years and 50% at 20 years. The most common procedures performed were fenestration closure and pulmonary artery intervention. Catheter intervention for anatomic indications was associated with Fontan after 2002 (HR, 2.1; P=0.007), Norwood operation (HR, 2.3; P=0.001), and longer cardiopulmonary bypass time (HR, 1.1 per 10 minutes; P=0.001). Catheter intervention for physiological indications was associated with prolonged post-Fontan pleural drainage (HR, 4.0; P&lt;0.001) and hypoplastic left heart syndrome (HR, 2.0; P=0.01).</p>

<p><strong>CONCLUSIONS: </strong>In this study of Fontan survivors, two thirds of patients required surgical or catheter-based reintervention by 20 years. Families should be counseled that the Fontan is typically not the final stage of single-ventricle palliation.</p>

DOI

10.1161/CIRCINTERVENTIONS.116.004924

Alternate Title

Circ Cardiovasc Interv

PMID

28851719

Title

Parents' Preferences Regarding Public Reporting of Outcomes in Congenital Heart Surgery.

Year of Publication

2018

Number of Pages

606-11

Date Published

2018 Feb

ISSN Number

1552-6259

Abstract

<p><strong>BACKGROUND: </strong>Calls for public reporting of outcomes in congenital heart surgery have led to several different reporting schemes, including a star rating system and benchmark procedure-specific mortality data tables. Important unanswered questions remain about the optimal format and content of public reporting of congenital heart surgery outcomes.</p>

<p><strong>METHODS: </strong>In conjunction with three parent advocacy groups, we developed a questionnaire to gauge parents' attitudes regarding the format and content of an "optimal" public reporting scheme. Parents were solicited for participation through email lists of members of parent advocacy groups and from a cohort of parents whose children had undergone an STS benchmark procedure of the Society of Thoracic Surgeons at the Children's Hospital of Philadelphia after January 1,&nbsp;2007.</p>

<p><strong>RESULTS: </strong>The 1,297 responses received provided complete data for analysis. Nearly all the participants were mothers of children with congenital heart disease, and most were white. About half of the children were diagnosed prenatally, and 63% underwent initial repair of the defect in the neonatal period. Parents identified survival statistics, surgeon-specific experience, and complication rates as most important. Presented with three display formats for mortality rates, most parents (89%) identified a numeric procedure-based approach as the best format, and more than half identified the hospital star rating system as the worst format.</p>

<p><strong>CONCLUSIONS: </strong>Parents of children with congenital heart disease identify survival statistics, surgeon-specific experience, and complication rates as the most important outcome measures to report publicly. Additionally, parents preferred mortality data to be presented in a&nbsp;procedure-specific format using a numeric procedure-based approach, as opposed to the star rating system.</p>

DOI

10.1016/j.athoracsur.2017.05.043

Alternate Title

Ann. Thorac. Surg.

PMID

28826991

Title

Systemic Atrioventricular Valve Excision and Ventricular Assist Devices in Pediatric Patients.

Year of Publication

2018

Number of Pages

170-174

Date Published

2018 Jan

ISSN Number

1552-6259

Abstract

<p><strong>BACKGROUND: </strong>Continuous-flow ventricular assist devices (CF VADs) designed for adults are increasingly used in pediatric patients. However, there is greater risk of device inflow obstruction as a result of size and anatomy.</p>

<p><strong>METHODS: </strong>We reviewed all cases of systemic atrioventricular valve (AVV) excision with HeartWare HVAD (HeartWare, Framingham, MA) implantation in the systemic ventricle performed at our institution from November 2015 to May&nbsp;2016.</p>

<p><strong>RESULTS: </strong>AVV excision with CF VAD implantation was undertaken in 3 patients. Patient 1 was palliated in infancy, resulting in biventricular physiology with a systemic right ventricle, and presented at age 15 years with worsening ventricular dysfunction. After CF VAD implantation in the systemic ventricle and discharge to home, tricuspid valve obstruction to VAD inflow developed, and the patient and underwent tricuspid valve excision on postoperative day 52. Patients 2 and 3 were aged younger than 4 years, with a body surface area of 0.62 m(2) and 0.58 m(2), respectively, and had undergone Fontan palliation, with subsequent systemic ventricular dysfunction and AVV regurgitation. In both Fontan patients, the CF VAD was implanted in the right atrium with simultaneous excision of the AVV. None have had evidence of elevated atrial pressures or recalcitrant pulmonary edema. At a mean follow-up of 359 days (range, 304 to 422 days), there have been no concerns for inflow obstruction or low flow.</p>

<p><strong>CONCLUSIONS: </strong>CF VAD implantation with AVV excision can successfully support complex pediatric patients in a wide range of size and anatomy (small chambers, systemic right ventricles). This technique may allow for CF VAD implantation in patients previously deemed too small for such support.</p>

DOI

10.1016/j.athoracsur.2017.05.038

PMID

28821330

Title

Long-term survival after the Fontan operation: Twenty years of experience at a single center.

Year of Publication

2017

Date Published

2017 Mar 06

ISSN Number

1097-685X

Abstract

<p><strong>OBJECTIVE: </strong>Existing studies of patients palliated with the Fontan operation are limited by heterogeneous patient populations and incomplete follow-up. This study aimed to describe long-term post-Fontan survival in a modern patient cohort.</p>

<p><strong>METHODS: </strong>All 773 patients who underwent a first Fontan operation at our institution between 1992 and 2009 were reviewed. The primary outcome was the composite endpoint of Fontan takedown, heart transplantation, or death before 2013.</p>

<p><strong>RESULTS: </strong>Follow-up rate was 99.2%. Survival with intact Fontan circulation was 94% at 1&nbsp;year (95% confidence interval [95% CI], 92%-95%), 90% at 10&nbsp;years (95% CI, 88%-92%), 85% at 15&nbsp;years (95% CI, 82%-88%), and 74% at 20&nbsp;years (95% CI, 67%-80%). Distinct risk factors were identified for early (≤1&nbsp;year) and late composite outcomes. Independent risk factors for early outcome included prolonged pleural drainage (hazard ratio [HR], 4.4; P&nbsp;&lt;&nbsp;.001), intensive care unit stay&nbsp;&gt;1&nbsp;week (HR, 2.4; P&nbsp;&lt;&nbsp;.001), Fontan before 1997 (HR, 3.3; P&nbsp;&lt;&nbsp;.001), preoperative atrioventricular valve regurgitation (HR, 2.0; P&nbsp;&lt;&nbsp;.001), and longer crossclamp time (HR, 1.3 per 10&nbsp;minutes; P&nbsp;&lt;&nbsp;.001). Late outcome was predicted by atrioventricular valve regurgitation prior to Fontan (HR, 2.0; P&nbsp;≤&nbsp;.001), and post-Fontan ICU stay&nbsp;&gt;1&nbsp;week (HR, 2.4; P&nbsp;&lt;&nbsp;.001).</p>

<p><strong>CONCLUSIONS: </strong>Long-term mortality after Fontan operation remains substantial. Risk factors for death or loss of Fontan circulation differ between the early and late postoperative periods. Long-term survival has not improved appreciably over the last decade, suggesting that alternatives to the Fontan are warranted.</p>

DOI

10.1016/j.jtcvs.2017.01.056

Alternate Title

J. Thorac. Cardiovasc. Surg.

PMID

28341469

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