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Objectives: Diastolic dysfunction (DD), a key driver of long-term Fontan outcomes, may be concealed during standard haemodynamic evaluation. We sought to identify Fontan patients with occult DD using 'ventricular stress testing' with rapid volume expansion (RVE)., Methods: Cardiac catheterisation with RVE was performed routinely in Fontan patients between 11/2012 and 4/2015. Baseline and post-stress haemodynamic data were compared using t test, Mann-Whitney U test, χ(2) and Fisher's exact tests. A post-stress ventricular end diastolic pressure (EDP) threshold of 15 mm Hg defined occult DD., Results: Forty-six Fontan patients (48% female, median age 14.1 (IQR 9.1 to 21.3) years) were included. The median Fontan duration was 10.8 (IQR 5.1 to 17.8) years and dominant left ventricular morphology was present in 63% of patients. Volume expansion increased mean Fontan pressure (15.2±2.5 vs 12.4±2.2 mm Hg, p<0.001), pulmonary capillary wedge pressure (11.3±2.6 vs 7.9±2 mm Hg, p<0.001) and EDP (12.7±3.3 vs 8.5±2.1 mm Hg, p<0.001). Sixteen patients (35%) had occult DD, demonstrating higher baseline EDP (10.3±1.9 vs 7.6±1.5 mm Hg, p<0.001) and greater increase in EDP (6.3±2.4 vs 3.1±1.4 mm Hg, p<0.001) compared with patients without DD. Higher baseline EDP, lower baseline cardiac index and longer duration of Fontan circulation were associated with higher post-stress EDP. There were no complications related to RVE., Conclusions: Ventricular stress testing by RVE is feasible, safe and identifies a subgroup of Fontan patients with occult DD. Higher baseline EDP and longer duration of Fontan circulation are associated with worse diastolic function. Future work is necessary to better understand the aetiology, associations and clinical implications of occult DD in Fontan survivors., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/)

Changes in ventricular geometry are often seen in patients with right ventricular hypertension secondary to pulmonary hypertension (PH). Progressive systolic bowing of the inter-ventricular septum occurs with increasing right ventricular pressure (RVp) and can be quantified with the left ventricular end-systolic eccentricity index (LVEI). Only limited data exist in children to evaluate the relationship between the LVEI and invasive RVp. We sought to assess the correlation between the LVEI and an invasively measured peak systolic RVp to aortic pressure (pAo) ratio. Medical records of patients undergoing echocardiography within 30 days of right and left heart catheterization for evaluation of PH between February 2009 and March 2014 were retrospectively reviewed. Forty-six studies in 29 subjects (median age 3.8 years, 46 % female), with a median time from echocardiogram to catheterization of -1.0 days, were included for analysis. The mean LVEI was 1.6 ± 0.5, and mean RVp/pAo ratio was 0.68 ± 0.26. There was a significant positive correlation (r = 0.76, p < 0.001) between LVEI and RVp/pAo ratio. ROC analysis demonstrated an area under the curve = 0.91 for prediction of RVp/pAo >0.50 by the LVEI. An LVEI >1.48 had a sensitivity of 76 % and specificity of 100 % in predicting RVp/pAo >0.50, while an LVEI >1.24 had a sensitivity of 88 % and specificity of 83 %. Echocardiographically derived LVEI is strongly correlated with invasively determined RVp/pAo ratio. In combination with other noninvasive measures of RVp, LVEI may help minimize the need for invasive patient evaluation.

Objectives: The aim of this study was to define age-stratified, procedure-specific benchmark radiation dose levels during interventional catheterization for congenital heart disease., Background: There is a paucity of published literature with regard to radiation dose levels during catheterization for congenital heart disease. Obtaining benchmark radiation data is essential for assessing the impact of quality improvement initiatives for radiation safety., Methods: Data were obtained retrospectively from 7 laboratories participating in the Congenital Cardiac Catheterization Project on Outcomes collaborative. Total air kerma, dose area product, and total fluoroscopy time were obtained for the following procedures: 1) patent ductus arteriosus closure; 2) atrial septal defect closure; 3) pulmonary valvuloplasty; 4) aortic valvuloplasty; 5) treatment of coarctation of aorta; and 6) transcatheter pulmonary valve placement., Results: Between January 2009 and July 2013, 2,713 cases were identified. Radiation dose benchmarks are presented including median, 75th percentile, and 95th percentile. Radiation doses varied widely between age groups and procedure types. Radiation exposure was lowest in patent ductus arteriosus closure and highest in transcatheter pulmonary valve placement. Total fluoroscopy time was a poor marker of radiation exposure and did not correlate well with total air kerma and dose area product., Conclusions: This study presents age-stratified radiation dose values for 6 common congenital heart interventional catheterization procedures. Fluoroscopy time alone is not an adequate measure for monitoring radiation exposure. These values will be used as baseline for measuring the effectiveness of future quality improvement activities by the Congenital Cardiac Catheterization Project on Outcomes collaborative., (Copyright © 2014 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objective: To validate a method for determination of cardiac index (CI) using real-time measurement of oxygen consumption (VO2 ) in young children undergoing cardiac catheterization., Design: Retrospective review comparing thermodilution cardiac index (TDCI) to CI calculated by the Fick equation using real-time measured VO2 (RT-VO2 ) and VO2 derived from 2 published predictive equations. Paired t-test and Bland-Altman analysis were used to compare TDCI to Fick CI. A survey to ascertain pediatric cardiac catheterization practices regarding VO2 determination was also conducted., Setting: Quaternary care children's hospital cardiac catheterization laboratory., Patients: Children <3 years old with structurally normal hearts undergoing cardiac catheterization under general anesthesia with at least one set of contemporaneous TDCI and RT-VO2 measurements., Results: Thirty-six paired measurements of TDCI and RT-VO2 were made in 27 patients over a 2-year period. Indications for catheterization included congenital diaphragmatic hernia postrepair (n = 13), heart disease post-orthotopic heart transplant (n = 13), and suspected cardiomyopathy (n = 1). Mean age was 21.5 ± 8 months; median weight was 9.9 kg (IQR 8.57, 12.2). RT-VO2 was higher than VO2 predicted by the LaFarge equation (190 ± 31 vs. 173.8 ± 12.8 mL/min/m(2), P < .001), but there was no difference between TDCI and Fick CI calculated using VO2 from any method. Bland-Altman analysis showed excellent agreement between TDCI and Fick CI using RT-VO2 and VO2 predicted by the Lundell equation; Fick CI using VO2 predicted by the LaFarge equation showed fair agreement with TDCI., Conclusions: In children <3 years with a structurally normal heart, RT-VO2 generates highly accurate determinations of Fick CI as compared with TDCI. Additionally, in this population, VO2 derived from the LaFarge and Lundell equations generates accurate Fick CI compared with TDCI. Future studies are needed to identify factors associated with inaccurate VO2 generated from these predictive equations., (© 2013 Wiley Periodicals, Inc.)

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: 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.)

Introduction: Procedural cooperation between cardiac surgeon and interventional cardiologist to facilitate interventions such as device delivery or angioplasty (hybrid procedure) has become increasingly common in the management of patients with congenital heart disease., Design: Data were prospectively collected using a multicenter registry (C3PO). Between February 2007 and December 2008, seven institutions submitted data regarding 7019 cardiac catheterization procedures. Procedural data and adverse events (AEs) of 128 hybrid procedures were evaluated., Results: There was significant variability in the number of hybrid procedures per center, ranging from one to 89 with a median of eight. A total of 60% of interventional (vs. strictly diagnostic) hybrid procedures were performed by one center. The median weight was 3.7 kg (0.7-86 kg). Single-ventricle circulation was present in 60% of the procedures. Hybrid procedures included: patent ductus arteriosus (PDA) stent placement (n = 55), vascular rehabilitation (n = 25), ventricular septal defect (VSD) device closure (n = 7), valvotomy (n = 3), and diagnostic hybrid procedures (n = 38). Sixteen AEs occurred in 15/128 (12%) procedures. These included minor or trivial AEs (n = 9), moderate AEs (n = 5), major AEs (n = 1), and catastrophic AEs (n = 1). The type of AE documented included arrhythmias (n = 6), hypoxia or hypotension (n = 3), vessel or cardiac trauma (n = 2), and other events (n = 5). Of documented AEs, 9/16 (56%) were classified as not preventable, 6/16 (38%) as possibly preventable, and 1/16 (6%) as preventable. The incidence of AE related to PDA stent placement with surgical exposure (5/50, 10%) was significantly lower when compared with PDA stent placement performed percutaneously (4/5, 80%, P= .002)., Conclusion: Hybrid procedures appear to have a low incidence of associated major AEs. PDA stent placement performed as a palliation of hypoplastic left heart syndrome (HLHS) or complex single/two ventricle patients may have a lower incidence of AEs if performed using a direct approach with surgical exposure rather than a percutaneous approach. Accurate definitions of these innovative procedures are required to facilitate prospective data collection.

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.

Objective: A substantial unmet medical device need exists in pediatric care. As a result, the off-label use of approved devices is routine in pediatric interventional cardiology, but the extent and nature of this practice has not been previously described. The purpose of this study, therefore, is to evaluate the prevalence and nature of off-label cardiac device use in an active pediatric interventional program in the United States., Study Design: This study is a retrospective review of all interventional cardiac procedures performed at our institution from July 1, 2005 to June 30, 2008. Diagnostic (noninterventional) catheterizations, myocardial biopsies, invasive electrophysiology studies, and studies involving investigational devices were excluded. Interventions performed were compared with the manufacturer's labeled indications for each device., Results: During this 3-year period, 473 patients (median age 4.1 years) underwent 595 transcatheter interventions. An approved device was utilized for an off-label application in 63% of patients, and in 50% of all interventions performed. The most frequent off-label procedures were stent implantations (99% off-label), balloon dilations (78% off-label), and coil embolizations (29% off-label). In contrast, the off-label use of septal and ductal occluders was relatively uncommon., Conclusions: In our routine (noninvestigational) practice of pediatric interventional cardiology, 63% of patients underwent procedures utilizing medical devices for off-label indications. These data underscore the need to enhance cardiac device review and approval processes in the United States to include pediatric applications.

Hypoplastic left heart syndrome (HLHS) with intact atrial septum (IAS) is a severe congenital cardiac malformation with little possibility of postnatal survival, unless an intra-atrial connection is created promptly after delivery. The timing of that intervention is often dictated by logistic restrictions, such as the proximity of the delivery room to the cardiac catheterization laboratory. This report details the use of a hybrid cardiac catheterization suite to expedite the delivery, transfer, and initial treatment of a fetus with HLHS/IAS. The mother underwent cesarean delivery in one hybrid room, and the newborn infant was transferred directly into an adjoining room where successful atrial septal stenting was performed without delay via a percutaneous, transhepatic approach., (Copyright 2008 Wiley-Liss, Inc.)

Objectives: The present study evaluates two transcatheter closure strategies utilized at a single center and makes recommendations for device selection when occluding the patent ductus arteriosus., Background: A variety of devices are available for transcatheter closure of the patent ductus arteriosus (PDA) but no guidelines exist to guide operator device choice., Methods: A total of 132 patients underwent attempted transcatheter PDA closure utilizing one of two consecutive closure strategies between January 2000 and June 2005. Strategy A (n = 64; January 2000-May 2003) utilized Gianturco coils only. Strategy B (n = 68; June 2003-June 2005) utilized a single Gianturco coil for the PDA with a minimal diameter

There has been a recent trend toward hybrid cardiac catheterization procedures for the treatment of patients with various forms of congenital heart disease. Hybrid procedures offer the combined advantages of outstanding imaging in a full operating room environment, allowing direct access onto the heart or the great vessels for access or procedure completion, or complementary imaging before, during, or after surgical correction when necessary. With the increase in frequency of hybrid procedures, more medical centers are contemplating the conversion of standard cardiac catheterization rooms to hybrid facilities, or de novo construction. In this report, we detail a single-center experience of conversion from a standard catheterization facility into a hybrid suite. The strategic planning, design, system integration, and the challenges inherent to this project are discussed. Many of the solutions to these challenges are likely to be applicable to other institutions planning on similar hybrid conversion or construction., ((c) 2007 Wiley-Liss, Inc.)

Objectives: Most perimembranous ventricular septal defects (pmVSD) that are still patent in adult life are small, hemodynamically and clinically unimportant, and do not require any intervention. However, surgery in adulthood for those that need to be closed carries significant morbidity. A trans-catheter technique for closing pmVSDs has been developed, and this paper describes our initial experience using the Amplatzer membranous septal occluder (AMSO)., Patients/methods: Twelve patients, 9 female and 3 male, median age 34.5 years (range: 21-67) underwent catheterization for attempted pmVSD closure. Ten of the defects were native and 2 were post-operative residual defects. Transcatheter VSD closure was performed as previously described, under general anesthesia and with trans-esophageal echocardiographic (TEE) monitoring. Patients had a moderate to large left to right shunt (mean Qp/Qs = 2.0+/-0.4) with mild left heart volume overload and near normal pulmonary pressure. All 10 native pmVSDs were closed successfully, 9 with AMSO and one with an Amplatzer muscular VSD occluder, after failure to implant the AMSO. There was one post procedural complication--self-limiting retroperitoneal bleeding. Three patients had a residual leak. Attempted VSD closure in the 2 patients with post surgery residual shunt was unsuccessful., Conclusion: We conclude that transcatheter mVSD closure with the AMSO is an efficient and safe alternative to surgery in carefully selected adult patients with native pmVSDs.

Elevated pulmonary vascular resistance (PVR) continues to pose challenges in heart transplantation, but does the common belief that implanting a larger organ when the PVR is high actually affect outcomes? The purpose of this study is to assess post-transplantation outcomes in recipients with increased PVR in relation to donor organ size. The United Network for Organ Sharing (UNOS) database was used to identify patients ages 0-18 years at time of listing who underwent transplantation between 2010 and 2019 and for whom cardiac catheterization and donor-recipient weight data was available. Patients were divided by listing PVR into <3, 3-6, and >6 Wood units. Donor-Recipient weight ratio was categorized as undersized (≤0.80), mid-size (0.81-1.2), and oversized (>1.2). Subgroup analysis was done with an additional supersized group (>2.0). 1491 patients met study criteria. Median age [years (IQR)] 10 (3-15) and 45% female. 4% of heart transplantation cases used undersized, 45% used mid-size, and 51% used oversized organs. More patients with PVR>6 were transplanted with an oversized organ compared to patients with PVR<3 [59% (148/252) vs 48% (430/894), p=0.003). There was no difference in survival between the organ size groups regardless of PVR; this includes patients with PVR>6 at listing who were transplanted with an oversized organ versus those transplanted with an undersized (p=0.359) or mid-sized (p=0.956) organ. In subgroup analysis, even in patients transplanted with a supersized organ, there was no survival difference noted regardless of PVR. Despite a persistent practice pattern to transplant high PVR patients with oversized organs, there remains no difference in post-transplantation survival between these patients and those transplanted with smaller organs. Therefore, transplants in patients with high PVR should not be delayed by waiting for larger organs. [ABSTRACT FROM AUTHOR]

The purpose of this study is to investigate whether performing cardiac catheterization (cath) in pediatric lung transplantation (LTx) candidates without a diagnosis of pulmonary hypertension (PHTN) impacts listing outcomes. The UNOS Registry was queried to identify patients (0-18yo) without a diagnosis of PHTN who were listed for LTx from 2000-2019. Patients were divided into those who underwent cath during waitlist (Cath) and those who did not (No Cath). Negative listing outcome was defined as death on waitlist or removal from waitlist due to deterioration of condition. All other outcomes were considered positive. 1,732 pediatric LTx candidates without a diagnosis of PHTN were identified. These patients were divided into Cath (n=431) and No Cath (n=1,301). Cath patients most common diagnosis was cystic fibrosis (n=163; 38%), were younger (p<0.001) and were generally healthier [less frequently on ECMO (p=0.032), mechanical ventilation (p=0.004), and less renal dysfunction (p=0.04)] than No Cath patients. As illustrated in Figure 1 , on Kaplan-Meier analysis, positive listing outcome was more frequent in the PVR≤6 group compared to the PVR>6 (p=0.05) and the No Cath groups (p=0.04). PVR>6 patients had similar listing outcomes to No Cath patients (p=0.36). In CF patients who reached a waitlist outcome within one year, positive listing outcomes at one year was 88% for the PVR≤6 group compared to 67% in the PVR>6 (p=0.05). Pediatric LTx candidates without a diagnosis of PHTN who either did not undergo cath or had a PVR >6 had inferior waitlist outcomes compared to those with a PVR≤6. However, 75% of candidates were not catheterized, so right heart catheterization should strongly be considered for pediatric lung transplant recipients in order to better manage waitlist prioritization and improve overall listing outcomes. [ABSTRACT FROM AUTHOR]