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Background: Interventional therapies for severe pulmonary arterial hypertension (PAH) can provide right ventricular (RV) decompression and preserve cardiac output. Transcatheter stent placement in a residual ductus arteriosus (PDA) is one potentially effective option in critically ill infants and young children with PAH. We sought to assess recovery of RV function by echocardiographic strain in infants and young children following PDA stenting for acute PAH., Methods: Retrospective review of patients < 2 years old who underwent PDA stenting for acute PAH. Clinical data were abstracted from the electronic medical record. RV strain (both total and free wall components) was assessed from echocardiographic images at baseline and 3, 6, and 12 months post-intervention, as well as at last echocardiogram., Results: Nine patients underwent attempted ductal stenting for PAH. The median age at intervention was 38 days and median weight 3.7 kg. One-third (3of 9) of patients had PAH associated with a congenital diaphragmatic hernia. PDA stents were successfully deployed in eight patients. Mean RV total strain was - 14.9 ± 5.6% at baseline and improved to - 23.8 ± 2.2% at 6 months post-procedure (p < 0.001). Mean free wall RV strain was - 19.5 ± 5.4% at baseline and improved to - 27.7 ± 4.1% at 6 months (p = 0.002). Five patients survived to discharge, and four patients survived 1 year post-discharge., Conclusion: PDA stenting for severe, acute PAH can improve RV function as assessed by strain echocardiography. The quantitative improvement is more prominent in the first 6 months post-procedure and stabilizes thereafter., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Current metrics used to adjust for case mix complexity in congenital cardiac catheterization are becoming outdated due to the introduction of novel procedures, innovative technologies, and expanding patient subgroups. This study aims to develop a risk adjustment methodology introducing a novel, clinically meaningful adverse event outcome and incorporating a modern understanding of risk., Methods: Data from diagnostic only and interventional cases with defined case types were collected for patients ≤18 years of age and ≥2.5 kg at all Congenital Cardiac Catheterization Project on Outcomes participating centers. The derivation data set consisted of cases performed from 2014 to 2017, and the validation data set consisted of cases performed from 2019 to 2020. Severity level 3 adverse events were stratified into 3 tiers by clinical impact (3a/b/c); the study outcome was clinically meaningful adverse events, severity level ≥3b (3bc/4/5)., Results: The derivation data set contained 15 224 cases, and the validation data set included 9462 cases. Clinically meaningful adverse event rates were 4.5% and 4.2% in the derivation and validation cohorts, respectively. The final risk adjustment model included age <30 days, Procedural Risk in Congenital Cardiac Catheterization risk category, and hemodynamic vulnerability score (C statistic, 0.70; Hosmer-Lemeshow P value, 0.83; Brier score, 0.042)., Conclusions: CHARM II (Congenital Heart Disease Adjustment for Risk Method II) risk adjustment methodology allows for equitable comparison of clinically meaningful adverse events among institutions and operators with varying patient populations and case mix complexity performing pediatric cardiac catheterization., Competing Interests: None.

Objectives: Currently, there are no prediction tools available to identify patients at risk of needing high-complexity care following cardiac catheterization for congenital heart disease. We sought to develop a method to predict the likelihood a patient will require intensive care level resources following elective cardiac catheterization., Design: Prospective single-center study capturing important patient and procedural characteristics for predicting discharge to the ICU. Characteristics significant at the 0.10 level in the derivation dataset (July 1, 2017 to December 31, 2019) were considered for inclusion in the final multivariable logistic regression model. The model was validated in the testing dataset (January 1, 2020 to December 31, 2020). The novel pre-procedure cardiac status (PCS) feature, collection started in January 2019, was assessed separately in the final model using the 2019 through 2020 dataset., Setting: Tertiary pediatric heart center., Patients: All elective cases coming from home or non-ICU who underwent a cardiac catheterization from July 2017 to December 2020., Interventions: None., Measurements and Main Results: A total of 2,192 cases were recorded in the derivation dataset, of which 11% of patients ( n = 245) were admitted to the ICU, while 64% ( n = 1,413) were admitted to a medical unit and 24% ( n = 534) were discharged home. In multivariable analysis, the following predictors were identified: 1) weight less than 5 kg and 5-9.9 kg, 2) presence of systemic illness, 3) recent cardiac intervention less than 90 days, and 4) ICU Admission Tool for Congenital Heart Catheterization case type risk categories (1-5), with C -statistics of 0.79 and 0.76 in the derivation and testing cohorts, respectively. The addition of the PCS feature fit into the final model resulted in a C -statistic of 0.79., Conclusions: The creation of a validated pre-procedural risk prediction model for ICU admission following congenital cardiac catheterization using a large volume, single-center, academic institution will improve resource allocation and prediction of capacity needs for this complex patient population., Competing Interests: The authors have disclosed that they do not have any potential conflicts of interest., (Copyright © 2022 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies.)

Background: Adults with congenital heart disease (ACHD) are vulnerable to contrast-induced acute kidney injury (CI-AKI) after cardiac catheterization. The aim of this study was to identify risk factors for clinically significant CI-AKI and evaluate the predictive value of contrast volume to estimated glomerular filtration rate ratio (V/eGFR) for the risk of CI-AKI following catheterization in the ACHD population., Methods: ACHD patients who underwent catheterization at Boston Children's hospital between 1/2011 and 1/2017 were retrospectively analyzed. CI-AKI was defined as an increase in serum creatinine ≥0.3 mg/dL within 48 hr or ≥1.5 times baseline within 7 days of procedure. Controls without CI-AKI were matched for calendar year of catheterization with cases using a 3:1 ratio., Results: Of 453 catheterizations meeting inclusion criteria, 27 catheterizations (5.9%) were complicated by CI-AKI, with dialysis being used to manage renal dysfunction in five of these events. Older age, male gender, admission prior to catheterization, and V/eGFR ratio were found to be related to risk of CI-AKI. Patients with a V/eGFR ≥ 2.6 had a significantly higher risk of CI-AKI (OR = 6.4; 95% CI = 2.0-20.4; P = 0.002). Survival at 3 years post-catheterization, was significantly shorter for CI-AKI cases compared to controls (49% versus 97%; P < 0.001) even in those with return to baseline renal function prior to discharge (60% versus 97%, P < 0.001)., Conclusion: In ACHD patients undergoing cardiac catheterization, a higher V/eGFR ratio is a strong predictor of clinically significant CI-AKI. Development of CI-AKI is a poor prognostic indicator and is associated with decreased survival in this population., (© 2018 Wiley Periodicals, Inc.)

Background: Relief of postoperative right ventricular outflow tract (RVOT) obstruction with transcatheter pulmonary valve replacement (TPVR) results in functional improvement in the short term which we investigated at baseline (BL), early follow-up (FU), and midterm FU after TPVR., Methods: Echocardiography and cardiopulmonary exercise testing were performed at BL and at early (median 6 months) and midterm FU (median 2.5years) after TPVR., Results: Patients with RVOT obstruction (n=22, median age 17years) were studied. The max RVOT Doppler gradient fell from BL to early FU (60±24 to 26±8mm Hg, P<.001). Left ventricular (LV) end-diastolic and stroke volume increased at early FU (both P<.001) without further change, whereas LV ejection fraction improved throughout FU (P<.001). LV end-systolic and diastolic eccentricity (leftward septal displacement) improved early (both P≤.003), and end-diastolic eccentricity improved further at midterm FU (P=.02). Furthermore, whereas mitral inflow A wave velocity increased (P=.003), the LV A' velocity declined early (P=.007) without further change at midterm. RV systolic and early diastolic function was impaired at BL. Whereas RV strain improved partially at early and midterm FU (P≤.02), RV E' velocity did not improve throughout FU. Mildly impaired LV strain at BL fully recovered by midterm FU (P≤.002). Peak oxygen uptake improved at early and midterm FU (all P≤.003)., Conclusions: Patients with RVOT obstruction had biventricular systolic and diastolic dysfunction at BL. Relieving RVOT obstruction with TPVR reduced adverse ventricular and compensatory atrioventricular interaction, resulting in progressive biventricular functional improvement and remodeling at early and midterm FU., (Published by Elsevier Inc.)

Background: In congenital aortic stenosis (AS), suboptimal agreement between Doppler-derived gradients and catheterization gradients may lead to inappropriate referrals for catheterization. To address this problem, the authors investigated whether adjusting Doppler gradients for pressure recovery (PR) improved their agreement with subsequent catheterization gradients., Methods: One hundred encounters in which patients with congenital AS underwent echocardiography and subsequent catheterization were retrospectively identified. Peak instantaneous and mean transaortic Doppler gradients were recorded from an apical view. PR (mm Hg) was calculated as 4VCW(2) × (2 × EOA/AOA) × (1 - EOA/AOA), where VCW is continuous-wave peak velocity, EOA is effective orifice area (stroke volume/velocity-time integral), and AOA is aortic cross-sectional area (π × radius(2)). The PR-corrected peak Doppler gradient was calculated as peak Doppler gradient - PR. Doppler gradients were tested for correlation and agreement with the peak-to-peak systolic gradient at catheterization (cath gradient)., Results: The median age was 12.9 years (range, 0.7-24.6 years). Median AS gradients were as follows: cath, 39 mm Hg (range, 0-103 mm Hg); peak Doppler, 48 mm Hg (range, 10-94 mm Hg); mean Doppler, 25 mm Hg (range, 4-58 mm Hg); and PR-corrected peak Doppler, 35 mm Hg (range, 5-78 mm Hg). Correlation coefficients between the various Doppler and cath gradients were not significantly different. The mean difference between Doppler and cath gradients was smallest for the PR-corrected peak Doppler gradient (-4.1 ± 14.1 mm Hg), followed by the uncorrected peak Doppler gradient (9.7 ± 15.9 mm Hg) and the mean Doppler gradient (-14.6 ± 15.6 mm Hg) (P < .001). Receiver operating characteristic curve analysis for a cath gradient ≥ 35 mm Hg revealed a significantly larger area under the curve for the PR-corrected peak Doppler gradient (0.85) compared with the uncorrected peak Doppler gradient (0.80) (P = .004) and the mean Doppler gradient (0.78) (P = .001). A PR-corrected peak Doppler gradient ≥ 27 mm Hg was associated with a cath gradient ≥ 35 mm Hg with 90% sensitivity and 61% specificity., Conclusions: In congenital AS, correcting the peak Doppler gradient for PR significantly improved agreement with the subsequently measured cath gradient. This approach may improve decisions regarding referral for catheterization., (Copyright © 2015 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Sedation/anesthesia is critical to cardiac catheterization in the pediatric/congenital heart patient. We sought to identify current sedation/anesthesia practices, the serious adverse event rate related to airway, sedation, or anesthesia, and the rate of intra-procedural conversion from procedural sedation to the use of assisted ventilation or an artificial airway. Data from 13,611 patients who underwent catheterization at eight institutions were prospectively collected from 2007 to 2010. Ninety-four (0.69 %) serious sedation/airway-related adverse events occurred; events were more likely to occur in smaller patients (<4 kg, OR 4.4, 95 % CI 2.3-8.2, p < 0.001), patients with non-cardiac comorbidities (OR 1.7, 95 % CI 1.1-26, p < 0.01), and patients with low mixed venous oxygen saturation (OR 2.3, 95 % CI 1.4-3.6, p < 0.001). Nine thousand three hundred and seventy-nine (69 %) patients were initially managed with general endotracheal anesthesia, LMA, or tracheostomy, whereas 4232 (31 %) were managed with procedural sedation without an artificial airway, of which 75 (1.77 %) patients were converted to assisted ventilation/general anesthesia. Young age (<12 months, OR 5.2, 95 % CI 2.3-11.4, p < 0.001), higher-risk procedure (category 4, OR 10.1, 95 % CI 6.5-15.6, p < 0.001), and continuous pressor/inotrope requirement (OR 11.0, 95 % CI 8.6-14.0, p < 0.001) were independently associated with conversion. Cardiac catheterization in pediatric/congenital patients was associated with a low rate of serious sedation/airway-related adverse events. Smaller patients with non-cardiac comorbidities or low mixed venous oxygen saturation may be at higher risk. Patients under 1 year of age, undergoing high-risk procedures, or requiring continuous pressor/inotrope support may be at higher risk of requiring conversion from procedural sedation to assisted ventilation/general anesthesia.

A methodology that would allow for comparison of charges across institutions has not been developed for catheterization in congenital heart disease. A single institution catheterization database with prospectively collected case characteristics was linked to hospital charges related and limited to an episode of care in the catheterization laboratory for fiscal years 2008-2010. Catheterization charge categories (CCC) were developed to group types of catheterization procedures using a combination of empiric data and expert consensus. A multivariable model with outcome charges was created using CCC and additional patient and procedural characteristics. In 3 fiscal years, 3,839 cases were available for analysis. Forty catheterization procedure types were categorized into 7 CCC yielding a grouper variable with an R (2) explanatory value of 72.6%. In the final CCC, the largest proportion of cases was in CCC 2 (34%), which included diagnostic cases without intervention. Biopsy cases were isolated in CCC 1 (12%), and percutaneous pulmonary valve placement alone made up CCC 7 (2%). The final model included CCC, number of interventions, and cardiac diagnosis (R (2) = 74.2%). Additionally, current financial metrics such as APR-DRG severity of illness and case mix index demonstrated a lack of correlation with CCC. We have developed a catheterization procedure type financial grouper that accounts for the diverse case population encountered in catheterization for congenital heart disease. CCC and our multivariable model could be used to understand financial characteristics of a population at a single point in time, longitudinally, and to compare populations.

Continued advancements in congenital cardiac catheterization and interventions have resulted in increased patient and procedural complexity. Anticipation of life-threatening events and required rescue measures is a critical component to preprocedural preparation. We sought to determine the incidence and nature of life-threatening adverse events in congenital and pediatric cardiac catheterization, risk factors, and resources necessary to anticipate and manage events. Data from 8905 cases performed at the 8 participating institutions of the Congenital Cardiac Catheterization Project on Outcomes were captured between 2007 and 2010 [median 1,095/site (range 133-3,802)]. The incidence of all life-threatening events was 2.1 % [95 % confidence interval (CI) 1.8-2.4 %], whereas mortality was 0.28 % (95 % CI 0.18-0.41 %). Fifty-seven life-threatening events required cardiopulmonary resuscitation, whereas 9 % required extracorporeal membrane oxygenation. Use of a risk adjustment model showed that age <1 year [odd ratio (OR) 1.9, 95 % CI 1.4-2.7, p < 0.001], hemodynamic vulnerability (OR 1.6, 95 % CI 1.1-2.3, p < 0.01), and procedure risk (category 3: OR 2.3, 95 % CI 1.3-4.1; category 4: OR 4.2, 95 % CI 2.4-7.4) were predictors of life-threatening events. Using this model, standardized life-threatening event ratios were calculated, thus showing that one institution had a life-threatening event rate greater than expected. Congenital cardiac catheterization and intervention can be performed safely with a low rate of life-threatening events and mortality; preprocedural evaluation of risk may optimize preparation of emergency rescue and bailout procedures. Risk predictors (age < 1, hemodynamic vulnerability, and procedure risk category) can enhance preprocedural patient risk stratification and planning.

Objectives: A previously published randomized clinical trial comparing cardiac magnetic resonance (CMR) versus routine catheterization in patients with functional single ventricle before bidirectional Glenn (BDG) operation demonstrated similar short-term post-BDG outcomes. We sought to assess late outcomes in this cohort to ascertain any long-term effects of this evaluation strategy., Methods: Retrospective review of enrolled patients through most recent follow-up was performed on all 82 patients in the original cohort, at a median age of 8.8 years., Results: Of these, 76 (93%) underwent Fontan operation; 2 died before Fontan. Baseline demographics, anatomic factors, and age at BDG did not differ between those randomized to CMR versus catheterization. Although pre-BDG CMR patients were younger at Fontan (2.4 vs 2.7 years; P = .02), baseline weight, body surface area, oxygen saturation, ventricular function, and degree of atrioventricular valve regurgitation were similar. Catheterization before Fontan (n = 76) demonstrated similar hemodynamic parameters including pulmonary vascular resistance and mean pulmonary artery, atrial, and ventricular end-diastolic pressures. CMR patients had comparable rates of transcatheter interventions (71% vs 79%; P = .6), including coil occlusion of systemic-pulmonary collaterals (66% vs 61%; P = .29). At Fontan surgery, short-term complications, hospital length of stay, and the percent meeting a 5-part definition of successful Fontan operation were not different (71% vs 55%; P = .23)., Conclusions: Pre-BDG CMR and catheterization groups had equivalent clinical and hemodynamic profiles before Fontan and similar post-Fontan outcomes at a median follow-up of 8 years after BDG. For selected patients, a pre-BDG evaluation with CMR is an acceptable alternative to catheterization., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Background: Studies have documented the importance of procedure type and hemodynamic variables on the incidence of procedure related adverse events (AE) after cardiac catheterization. However, little is known about the impact of low weight on the incidence and severity of AE., Methods: Data were prospectively collected using a multicenter registry (C3PO). Infants <1 year were divided into four weight categories: <2 kg, 2-3 kg, 3-5 kg, ≥5 kg. AE severity was classified as level 1-5 (none, minor, moderate, major, death)., Results: Eight centers submitted details on 3,679 cases (34% diagnostic) performed in infants <1 year from 2/07 to 6/10: <2 kg: 57 (1.5%), 2-3 kg: 403 (11%), 3-5 kg: 1,527 (41.5%), ≥5 kg: 1,692 (46%). AE occurred in 20% of cases (<2 kg: 28%, 2-3 kg: 25%, 3-5 kg: 23%, ≥5 kg: 16%) with 41% of all AE being level 3-5 AE. Death occurred more frequently in the <2 kg group (12%), 71% of which were interventional cases. The case-related mortality in all other weight groups was <1%. By multivariable analysis, weight <2 kg, 2-3 kg, and 3-5 kg were independent risk factors for high severity (level 3-5) AE (<2 kg: OR 2, 95%CI 1.1-3.6; 2-3 kg: OR 1.4, 95%CI 1-1.8; 3-5 kg: OR 1.3, 95%CI 1.1-1.5), with similar findings for all AE. Blood transfusions were more common in lower weight categories (<2 kg: 42%, 2-3 kg: 29%, 3-5 kg: 25%, ≥5 kg: 15%, p<0.001)., Conclusions: The risk of AE during cardiac catheterization of infants increases with lower weight. Infants who weigh less than 2 kg have a significantly higher risk of adverse events (most notably death) even after correcting for hemodynamic vulnerability and procedure type risk group., (Copyright © 2013 Wiley Periodicals, Inc.)

Background: Data examining the effect of operator years in practice and volume on adverse events (AE) after cardiac catheterization in patients with congenital heart disease is limited., Methods and Results: Data were prospectively collected using a multi-center registry (C3PO). 10,885 catheterizations performed between 02/07 and 06/10 at eight institutions were included. AE rates were risk-adjusted for hemodynamic vulnerability, procedure type risk group, and age and compared between operators with different years in practice (YIP) and volume. AE occurred in 13% of procedures. Operators with less than five YIP had higher adjusted odds of any AE (OR 1.42, 95% CI 1.14-1.77) or a high severity AE (OR 1.35, 95% CI 1.04-1.75), when compared with operators with 5 to less than 25 YIP (5 < 25), while operators with ≥25 YIP had higher odds of a high severity (but not any) AE (OR 1.39, 95% CI 1.08-1.80). Operators with <5 YIP had a higher percentage of preventable AE (out of all AE, 16% vs. 8%, P < 0.001) as well as higher odds of vascular or cardiac trauma (OR 1.81, 95% CI 1.11-2.97), or technical AE (OR 1.98, 95% CI 1.31-2.99) when compared with operators with 5 < 25 YIP. There was no consistent relationship between operator volume, and incidence of AE., Conclusions: Operators with less than 5 years in practice have higher risk-adjusted AE rates. While an important consideration in guiding and mentoring operators with fewer years in practice, it is important to emphasize that reporting adverse events does not take into account procedural efficacy., (Copyright © 2013 Wiley Periodicals, Inc.)

Background: Angioplasty and stent implantation have become accepted therapies for isolated peripheral pulmonary stenosis, and have been shown to increase vessel diameter and reduce right ventricular (RV) pressure acutely in patients with pulmonary artery (PA) stenosis. The purpose of this study was to assess long-term outcomes after primary transcatheter therapy for peripheral pulmonary stenosis., Methods and Results: We studied 69 patients who underwent primary transcatheter intervention for severe isolated peripheral pulmonary stenosis at ≤ 5 years of age. Genetic/syndromic diagnoses included Williams syndrome (n=23), non-Williams familial arteriopathy (n=12), and Alagille syndrome (n=3). At the initial PA intervention, median RV:aortic pressure ratio decreased from 1.00 to 0.88 (median decrease, 0.18; P<0.001). Patients with a higher preintervention RV:aortic pressure ratio had a greater reduction (P<0.001). During follow-up (median, 8.5 years), 10 patients died, 5 from complications of PA catheterization (all before 1998). Thirteen patients underwent surgical PA intervention, most within 1 year and along with repair of supravalvar aortic stenosis. Freedom from any PA reintervention was 38 ± 6% at 1 year and 22 ± 6% at 5 years. The median RV:aortic pressure ratio decreased from 1.0 at baseline to 0.53 at the most recent catheterization (P<0.001), and 82% of patients with available clinical follow-up were asymptomatic., Conclusions: Transcatheter therapy for infants with severe peripheral pulmonary stenosis has become safer, regardless of genetic condition. Coupled with reintervention and surgical relief in selected cases, RV:aortic pressure ratios decrease substantially and most patients are asymptomatic at late follow-up.

Objective: We sought to determine the relationship between relative value units (RVUs) and intended measures of work in catheterization for congenital heart disease., Methods: RVU was determined by matching RVU values to Current Procedural Terminology codes generated for cases performed at a single institution. Differences in median case duration, radiation exposure, adverse events, and RVU values by risk category and cases were assessed. Interventional case types were ranked from lowest to highest median RVU value, and correlations with case duration, radiation dose, and a cases-predicted probability of an adverse event were quantified with the Spearman rank correlation coefficient., Results: Between January 2008 and December 2010, 3557 of 4011 cases were identified with an RVU and risk category designation, of which 2982 were assigned a case type. Median RVU values, radiation dose, and case duration increased with procedure risk category. Although all diagnostic cases had similar RVU values (median 10), adverse event rates ranged from 6% to 21% by age group (P < .001). Median RVU values ranged from 9 to 54 with the lowest in diagnostic and biopsy cases and increasing with isolated and then multiple interventions. Among interventional cases, no correlation existed between ranked RVU value and case duration, radiation dose, or adverse event probability (P = .13, P = .62, and P = .43, respectively)., Conclusions: Time, skill, and stress inherent to performing catheterization procedures for congenital heart disease are not captured by measurement of RVU alone.

Objectives: This study sought to develop a method to adjust for case mix complexity in catheterization for congenital heart disease to allow equitable comparisons of adverse event (AE) rates., Background: The C3PO (Congenital Cardiac Catheterization Project on Outcomes) has been prospectively collecting data using a Web-based data entry tool on all catheterization cases at 8 pediatric institutions since 2007., Methods: A multivariable logistic regression model with high-severity AE outcome was built using a random sample of 75% of cases in the multicenter cohort; the models were assessed in the remaining 25%. Model discrimination was assessed by the C-statistic and calibration with Hosmer-Lemeshow test. The final models were used to calculate standardized AE ratios., Results: Between August 2007 and December 2009, 9,362 cases were recorded at 8 pediatric institutions of which high-severity events occurred in 454 cases (5%). Assessment of empirical data yielded 4 independent indicators of hemodynamic vulnerability. Final multivariable models included procedure type risk category (odds ratios [OR] for category: 2 = 2.4, 3 = 4.9, 4 = 7.6, all p < 0.001), number of hemodynamic indicators (OR for 1 indicator = 1.5, ≥2 = 1.8, p = 0.005 and p < 0.001), and age <1 year (OR: 1.3, p = 0.04), C-statistic 0.737, and Hosmer-Lemeshow test p = 0.74. Models performed well in the validation dataset, C-statistic 0.734. Institutional event rates ranged from 1.91% to 7.37% and standardized AE ratios ranged from 0.61 to 1.41., Conclusions: Using CHARM (Catheterization for Congenital Heart Disease Adjustment for Risk Method) to adjust for case mix complexity should allow comparisons of AE among institutions performing catheterization for congenital heart disease., (Copyright © 2011 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Cardiac catheterization is routinely performed in patients with single ventricle before bidirectional Glenn operation (BDG). There is interest in noninvasive evaluation alone before BDG, but concern for echocardiography successfully imaging the relevant anatomy persists. We evaluated the accuracy of echocardiographic imaging of vascular anatomy., Methods and Results: Diagnostic images of 130 patients who had echocardiography and catheterization before BDG were reviewed; diameters of the pulmonary arteries (PAs) and aortic arch were measured, and stenoses were recorded. Patient and procedural factors associated with echocardiographic imaging were analyzed. Median age at echocardiography was 4 months; the most common diagnosis was hypoplastic left heart syndrome (55%). The left PA was imaged by echocardiography in 83 patients (64%), with 4 of 21 stenoses (19%) diagnosed by catheterization identified; similarly, the right PA was imaged in 81 (62%), and 3 of 17 stenoses (18%) were identified. The distal aortic arch was visualized in 104 (80%), with successful identification of 21 of 27 (78%) of coarctations diagnosed by catheterization. Complete vascular echocardiography (visualization of PAs and aortic arch) occurred in 43% and was not obtained more frequently with sedation., Conclusions: In a large cohort of patients presenting for BDG, evaluation by echocardiography frequently failed to image the PAs and missed the majority of PA stenoses. Sedation did not appear to improve the performance of echocardiography for evaluation of the PAs. Echocardiography cannot be relied on as the sole investigation before BDG.

Background: The Congenital Cardiac Catheterization Project on Outcomes (C3PO) was established to develop outcome assessment methods for pediatric catheterization., Methods and Results: Six sites have been recording demographic, procedural and immediate outcome data on all cases, using a web-based system since February 2007. A sample of data was independently audited for validity and data completeness. In 2006, participants categorized 84 procedure types into 6 categories by anticipated risk of an adverse event (AE). Consensus and empirical methods were used to determine final procedure risk categories, based on the outcomes: any AE (level 1 to 5); AE level 3, 4, or 5; and death or life-threatening event (level 4 or 5). The final models were then evaluated for validity in a prospectively collected data set between May 2008 and December 31, 2009. Between February 2007 and April 2008, 3756 cases were recorded, 558 (14.9%) with any AE; 226 (6.0%) level 3, 4, or 5; and 73 (1.9%) level 4 or 5. General estimating equations models using 6 consensus-based risk categories were moderately predictive of AE occurrence (c-statistics: 0.644, 0.664, and 0.707). The participant panel made adjustments based on the collected empirical data supported by clinical judgment. These decisions yielded 4 procedure risk categories; the final models had improved discrimination, with c-statistics of 0.699, 0.725, and 0.765. Similar discrimination was observed in the performance data set (n=7043), with c-statistics of 0.672, 0.708, and 0.721., Conclusions: Procedure-type risk categories are associated with different complication rates in our data set and could be an important variable in risk adjustment models for pediatric catheterization., (© 2011 American Heart Association, Inc.)

Patients with single-ventricle circulation presenting for Fontan completion routinely undergo cardiac catheterization despite ongoing debate concerning its additive value. Increasing interest in noninvasive preoperative evaluation alone led the authors to analyze the utility of routine pre-Fontan catheterization and to determine whether a subset of patients could avoid this invasive procedure. Patients younger than 5 years referred for pre-Fontan evaluation were retrospectively reviewed. Medical records and catheter angiograms were examined, and catheterizations were categorized as "additive" based on predetermined criteria. Associations between precatheterization variables, catheterization findings, and short-term postoperative outcomes were evaluated. Cardiac catheterization was clinically nonadditive for 89 of 175 patients undergoing pre-Fontan evaluation (51%). There were no robust precatheterization predictors of a nonadditive catheterization. Echocardiography did not fully demonstrate the relevant anatomy of 115 patients (66%), most frequently due to inadequate visualization of the pulmonary arteries, and 22 patients had additive catheterizations due to new diagnostic findings alone. Interventions at catheterization were frequent and deemed "important" for 64 patients (37%). Catheterization hemodynamic data were not associated with early postoperative outcomes. Minor catheterization complications occurred for 51 patients (29%) and major complications for 4 patients (2%). Although at least 50% of the patients presenting for Fontan completion may be able to avoid routine catheterization safely, an echocardiography-based imaging strategy alone is insufficient to allow proper identification of those who could be evaluated noninvasively. A more comprehensive imaging strategy not based solely on echocardiography should be considered.

Objectives: To describe case mix variation among institutions, and report adverse event rates in congenital cardiac catheterization by case type., Background: Reported adverse event rates for patients with congenital heart disease undergoing cardiac catheterization vary considerably, due to non-comparable standards of data inclusion, and highly variable case mix., Methods: The Congenital Cardiac Catheterization Outcomes Project (C3PO) has been capturing case characteristics and adverse events (AE) for all cardiac catheterizations performed at six pediatric institutions. Validity and completeness of data were independently audited., Results: Between 2/1/07 and 4/30/08, 3855 cases (670 biopsy, 1037 diagnostic, and 2148 interventional) were recorded, median number of cases per site 480 (308 to 1526). General anesthesia was used in 70% of cases (28 to 99%), and 22% of cases (15 to 26%) were non-electively or emergently performed. Three institutions performed a higher proportion of interventions during a case, 72 to 77% compared to 56 to 58%. The median rate of AE reported per institution was 16%, ranging from 5 to 18%. For interventional cases the median rate of AE reported per institution was 19% (7 to 25%) compared to 10% for diagnostic cases (6 to 16%). The incidence of AE was significantly higher for interventional compared to diagnostic cases (20% vs 10%, p<0.001), as was the incidence of higher severity AE (9% vs 5%, p<0.001). Adverse events in biopsy cases were uncommon., Conclusions: In this multi-institutional cohort, the incidence of AE is higher among interventional compared to diagnostic cases, and is very low among biopsy cases. Equitable comparisons among institutions will require the development and application of risk adjustment methods.

Background: In pediatrics, contrast-related AE such as allergic reactions, seizures, and nephropathy have been reported to occur after cardiac catheterization, but their incidence remains unknown., Objective: We sought to report adverse event (AE) rates attributed to contrast administration in a pediatric cardiac catheterization lab and identify characteristics related to higher doses., Methods: A single institution prospective cardiac catheterization AE database identified AE in children <18 years old exposed to contrast. All AE were reviewed and classified by relationship to contrast. Medical records for the 50 cases who received highest contrast doses were retrospectively reviewed for AE. Patient and procedural characteristics were compared in the top quartile of contrast dose versus remaining cases., Results: Over 3 years, 2,321 consecutive cases required median 3.9 cm(3)/kg [IQR: 2.0, 6.0] of contrast. Patients receiving high dose contrast (top quartile) were more likely to be <1 year (51% vs. 24%), weigh <10 kg (66% vs. 29%), have complex 2 ventricle disease (56% vs. 35%), be in a high procedure type risk group (57% vs. 26%), and undergo procedures >2 h (67% vs. 28%), all P < 0.001. Only 2 of 2,321 cases (0.09%, 95% CI 0.01-0.31%) had AE possibly related to contrast. These events were an acute neurological change and transient nephropathy. In 50 cases receiving the most contrast, no AE were attributed to contrast., Conclusion: A large volume pediatric cardiac catheterization lab administered >or= 6 cm(3)/kg of contrast in a quarter of cases; however, AE related to contrast exposure were exceedingly rare.

Objective: We sought to develop a method to adjust for case mix diversity and allow comparison of adverse outcome rates among practitioners in pediatric and congenital cardiac catheterization., Patients and Methods: A single institutional database that captured demographic and procedural data was used to identify patient and procedural characteristics associated with adverse events (AE) and any high severity event classified as preventable or possibly preventable (P/PP). Diagnostic and procedural risk groups were created and indicators of hemodynamic vulnerability were defined. Expected event rates by the practitioners adjusting for case mix were calculated. Standardized adverse event ratios (SAER), defined as the observed rate divided by the expected rate for each practitioner were calculated with 95% confidence intervals., Results: The database included 1727 hemodynamic (30%) and interventional (70%) cases performed by seven practitioners in 18 months. During 147 cases, at least one P/PP AE occurred; among the seven practitioners observed, event rates ranged from 3.2 to 14.2%. In multivariable analysis, risk factors for all P/PP events included highest procedure risk group (odds ratio [OR] 2.1 for group 2, and 2.8 for group 3, relative to group 1, P = .001 and P < .001, respectively) and weight less than 4 kg (OR 2.8, P < .001). High severity P/PP events occurred in 67 cases with rates ranging from 2.0 to 6.6% by the practitioners. For these events, risk factors included: highest procedure risk group (OR 4.5 for group 2, and 4.9 for group 3, both P < .001) and an indicator of hemodynamic vulnerability (OR 1.8, P = .026). For the seven practitioners, the SAER ranged from 0.41 to 1.32 for any P/PP AE and from 0.69 to 1.44 for P/PP high severity events. In this cohort, we did not identify any statistically significant performance differences., Conclusion: Despite wide variations in case mix complexity in pediatric and congenital cardiac catheterization, this study demonstrates a method for risk adjustment which allows equitable comparisons among practitioners at a single institution.

We sought to characterize the frequency, severity, and attributability of adverse events (AE) in congenital cardiac catheterization and identify patient and procedural characteristics associated with AE. Risk for any AE was associated with age < 1 year (odds ratio [OR] 2.3), interventional cases (OR 2.2), increasing procedure type risk group (OR 1, 2.0, 2.5), and indicator of hemodynamic vulnerability (OR 1.6), all p < 0.001. Each of these and weight were also associated with increased risk for high severity events, except for young age. Our understanding of populations at risk for adverse outcomes in congenital cardiac catheterization cases can be enhanced through the development and refinement of variables that categorize procedure types and patient characteristics by similar risk for adverse outcomes.

Background: Routine preoperative catheterization is standard practice in patients with single-ventricle physiology before bidirectional Glenn anastomosis. Because catheterization is invasive and exposes patients to ionizing radiation, cardiac magnetic resonance (CMR) may be a safe and effective alternative., Methods and Results: We conducted a prospective, randomized, single-center clinical trial comparing catheterization with CMR in patients considered for bidirectional Glenn operation from February 2003 to June 2006. End points were frequency of adverse events of the preoperative evaluation and a composite score of clinically successful surgery. Of 92 eligible patients, 82 were enrolled on the basis of screening echocardiogram, fulfillment of inclusion criteria, and informed consent. Patients were randomized to catheterization (n=41) or CMR (n=41). There were no baseline differences between groups. Four treatment crossovers occurred, 3 to catheterization and 1 to CMR. Catheter interventions were performed in 17 patients (41%). Catheterization resulted in more minor adverse events (78% versus 5%; P<0.001), longer preoperative hospital stays (median, 2 versus 1 day; P<0.001), and higher hospital charges ($34 477 versus $14 921; P<0.001). There was 1 major adverse event in the CMR group (P=1.0). The operative course and frequency of postoperative complications were similar between the 2 groups. The proportion of patients who had a successful bidirectional Glenn operation was similar (71% versus 83%; P=0.3). At the 3-month follow-up, there were no differences in clinical status, oxygen saturation, or frequency of reinterventions., Conclusions: CMR is a safe, effective, and less costly alternative to routine catheterization in the evaluation of selected patients before bidirectional Glenn operation. Further studies are necessary to determine whether there are long-term benefits from transcatheter interventions in these patients.

Background: The presence of an intact or highly restrictive atrial septum (I/HRAS) has long been recognized as a predictor of poor outcome among patients with hypoplastic left heart syndrome (HLHS), although the rarity of this condition has precluded conclusive study. The purpose of this review is to summarize recent surgical outcomes for these patients at our center and to identify predictors., Methods: We retrospectively identified all neonates with a diagnosis of HLHS and I/HRAS who underwent stage I palliation at Children's Hospital Boston between January 2001 and December 2006. Chart review enabled analysis of patient and procedural variables., Results: All 32 patients underwent left atrial decompression in utero or postnatally before surgery. Fourteen patients (44%) underwent fetal intervention, either atrial septoplasty (n = 9) or aortic valvuloplasty (n = 5). Twenty-nine of the 32 patients had postnatal left atrial hypertension and underwent transcatheter atrial septoplasty as neonates before surgery; 3 did not require postnatal atrial septoplasty after successful fetal atrial septoplasty. After stage I, hospital survival was 69% (22 of 32). Need for shunt revision (p = 0.02) and for extracorporeal membrane oxygenation use (p < 0.001) were associated with hospital mortality. Survival at 6 months was 69% for patients who had fetal intervention, and 38% for those who were treated only postnatally (p = 0.2)., Conclusions: Surgical outcome for patients with HLHS and I/HRAS continues to improve. Prenatal decompression of the left atrium may be associated with greater hospital survival. Proposed effects of fetal intervention on lung pathology and longer-term survival are subjects for future study in this unique group of patients.

Objectives: This study sought to review the outcomes of 3 generations of closure devices for secundum atrial septal defects (ASDs) at a single institution., Background: Transcatheter closure of ASDs is now increasingly performed with devices that have been modified over time to improve performance., Methods: A review of prospective clinical trials of Clamshell (C.R. Bard Inc., Murray Hill, New Jersey), CardioSEAL (NMT Medical Inc., Boston, Massachusetts), and STARFlex (NMT Medical Inc.) closure devices for simple ASDs was performed. The entry criteria for these trials were age > or =2 years, isolated secundum ASD, evidence of right ventricular volume overload, and maximum stretched diameter varying from 20 to 25 mm. Successful outcomes were defined as no more than small residual leak (< or =2 mm) with absence of a severe complication or the need for an additional device or surgery., Results: A total of 72 Clamshell, 30 CardioSEAL, and 42 STARFlex devices were placed in uncomplicated ASDs. Each device modification resulted in improved closure rates, with the STARFlex device achieving a 93% complete closure rate with a device/stretched diameter ratio significantly smaller than with the other devices (p = 0.001). Severe complications occurred in 5 Clamshell, 1 CardioSEAL, and 0 STARFlex cases. Successful outcomes increased for each generation of device (79%, 93%, and 98% respectively, p = 0.009). There have been no long-term complications for either the CardioSEAL or the STARFlex devices., Conclusions: Modifications in 3 generations of devices have resulted in improved results for percutaneous ASD closure.

Background: Our purpose was to describe a 13-year experience with patients undergoing transcatheter device closure of unrepaired congenital or postoperative residual ventricular septal defects (VSDs)., Methods and Results: Since 1989, 170 patients (median age, 3.9 years) have undergone catheterization for closure of 1 or more congenital (n=92) or postoperative (n=78) residual VSDs using successive generations of STARFlex-type devices. Outcomes included echocardiographic assessment of residual flow and device position, assessment of VSD shunt/severity, and adverse events. Among 168 patients in whom device implantation was performed, between 1 and 7 devices were placed per patient (median, 1), with multiple devices placed in 40%. There was a significant decrease in left-to-right shunting after device implantation (P<0.001) and significant improvement in VSD size/severity, and device position proved stable. Of 332 adverse events, 39 were related to the device and 261 were related to the catheterization; all but 5 occurred in the periprocedural period. At a median follow-up of 24 months (0 to 154 months), 14 patients had died and 18 had device(s) explanted., Conclusions: Congenital and postoperative VSD closure using STARFlex-type devices resulted in stable improvement in clinical status and decreased interventricular shunting. Although periprocedural events occurred frequently, late events caused by the device were rare. Transcatheter device closure is an effective management option for patients with complex muscular VSDs that are difficult to approach surgically and for postoperative residual VSDs.

Objectives: We sought to characterize the outcomes of routine catheterization prior to superior cavopulmonary anastomosis and to determine if some patients were unlikely to benefit from catheterization and thus might be evaluated preoperatively with noninvasive methods alone., Background: Congenital heart disease patients with single ventricle physiology undergo routine echocardiography and cardiac catheterization prior to superior cavopulmonary anastomosis to determine anatomic and hemodynamic suitability for this procedure., Methods: We performed a retrospective review of all infants (n = 114) evaluated for potential superior cavopulmonary anastomosis at our institution from January 1997 to June 2000., Results: Patients' median age was 5.5 months. Full echocardiograms were obtained in 79 patients (69%). At catheterization a total of 41 interventions were performed in 35 patients (31%). Twenty-seven patients (24%) were transfused, 18 patients (17%) required cardiac intensive care unit admission, and median length of stay following catheterization was 1 day (range 0 to 22). Complications occurred in 28 patients (25%), most transient. Of 51 patients who had complete echocardiograms without indication for catheterization, none subsequently had significant interventions and only 2 had new findings at catheterization. Three candidates were excluded from operation; all 111 others underwent successful procedures and survived to hospital discharge., Conclusions: Interventions were frequent at catheterization prior to superior cavopulmonary anastomosis, but transient complications, transfusion, intensive care unit admission, and prolonged hospital length of stay were common. For patients in whom no issues indicating need for catheterization are identified by echocardiogram, routine catheterization rarely results in new information or intervention. These patients may be more safely evaluated preoperatively using exclusively noninvasive techniques.

To explore whether transcatheter intracardiac devices increase risk for sudden death, we analyzed patient and device-related variables with a case-control design. Sudden death was defined as unexpected death with abrupt onset and rapid deterioration. Cases included all patients known to have died suddenly after device closure at our institution, using 3 large databases. Controls (2:1), matched on year of procedure, were chosen randomly from the same databases. Data were obtained from medical record review. Of 777 patients who received implants between February 1989 and February 1999, 9 were known to have died suddenly (1.2%). In 27 cases and controls, diverse defects were occluded: atrial (n = 16), ventricular (n = 4), Fontan fenestration (n = 4), or other (n = 3). Cases were more likely to have had a history of serious arrhythmias (p = 0.008), severe valve regurgitation (p = 0.03), > or =1 cardiac surgery (p = 0.009), and multiple devices (p = 0.03). Cases were somewhat more likely to have pulmonary hypertension (p = 0.09), ventricular dysfunction (p = 0.09), and nonatrial septal defects (p = 0.10). Patients were less likely to have device arm fractures (p = 0.05). The following were not related to sudden death: age, weight, gender, low systemic cardiac index, end-diastolic pressure, major noncardiac disease, serious adverse events, maximum device size, malposition, and residual leak. This study suggests that sudden death was more likely to occur in device-implanted patients who had severe underlying cardiac disease and/or multiple devices. No other device-related variables were associated with sudden death. [ABSTRACT FROM AUTHOR]