Angiographic tool to detect pulmonary arteriovenous malformations in single ventricle physiology.

OBJECTIVE: Individuals with single ventricle physiology who are palliated with superior cavopulmonary anastomosis (Glenn surgery) may develop pulmonary arteriovenous malformations. The traditional tools for pulmonary arteriovenous malformation diagnosis are often of limited diagnostic utility in this patient population. We sought to measure the pulmonary capillary transit time to determine its value as a tool to identify pulmonary arteriovenous malformations in patients with single ventricle physiology. METHODS: We defined the angiographic pulmonary capillary transit time as the number of cardiac cycles required for transit of contrast from the distal pulmonary arteries to the pulmonary veins. Patients were retrospectively recruited from a single quaternary North American paediatric centre, and angiographic and clinical data were reviewed. Pulmonary capillary transit time was calculated in 20 control patients and compared to 20 single ventricle patients at the pre-Glenn, Glenn, and Fontan surgical stages (which were compared with a linear-mixed model). Correlation (Pearson) between pulmonary capillary transit time and haemodynamic and injection parameters was assessed using angiograms from 84 Glenn patients. Five independent observers calculated pulmonary capillary transit time to measure reproducibility (intraclass correlation coefficient). RESULTS: Mean pulmonary capillary transit time was 3.3 cardiac cycles in the control population, and 3.5, 2.4, and 3.5 in the pre-Glenn, Glenn, and Fontan stages, respectively. Pulmonary capillary transit time in the Glenn population did not correlate with injection conditions. Intraclass correlation coefficient was 0.87. CONCLUSIONS: Pulmonary angiography can be used to calculate the pulmonary capillary transit time, which is reproducible between observers. Pulmonary capillary transit time accelerates in the Glenn stage, correlating with absence of direct hepatopulmonary venous flow.

Angiographic Tool to Detect Pulmonary Arteriovenous Malformations in Single Ventricle Physiology.

Background: Individuals with single ventricle physiology who are palliated with superior cavopulmonary anastomosis (Glenn surgery) may develop pulmonary arteriovenous malformations (PAVMs). The traditional tools for PAVM diagnosis are often of limited diagnostic utility in this patient population. We sought to measure the pulmonary capillary transit time (PCTT) to determine its value as a tool to identify PAVMs in patients with single ventricle physiology. Methods: We defined the angiographic PCTT as the number of cardiac cycles required for transit of contrast from the distal pulmonary arteries to the pulmonary veins. Patients were retrospectively recruited from a single quaternary North American pediatric center, and angiographic and clinical data was reviewed. PCTT was calculated in 20 control patients and compared to 20 single ventricle patients at the pre-Glenn, Glenn, and Fontan surgical stages (which were compared with a linear-mixed model). Correlation (Pearson) between PCTT and hemodynamic and injection parameters was assessed using 84 Glenn angiograms. Five independent observers calculated PCTT to measure reproducibility (intra-class correlation coefficient). Results: Mean PCTT was 3.3 cardiac cycles in the control population, and 3.5, 2.4, and 3.5 in the pre-Glenn, Glenn, and Fontan stages, respectively. PCTT in the Glenn population did not correlate with injection conditions. Intraclass correlation coefficient was 0.87. Conclusions: Pulmonary angiography can be used to calculate the pulmonary capillary transit time, which is reproducible between observers. PCTT accelerates in the Glenn stage, correlating with absence of direct hepatopulmonary venous flow.

Catheterization for Congenital Heart Disease Adjustment for Risk Method II.

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.

Transcatheter Device Therapy and the Integration of Advanced Imaging in Congenital Heart Disease.

Transcatheter device intervention is now offered as first line therapy for many congenital heart defects (CHD) which were traditionally treated with cardiac surgery. While off-label use of devices is common and appropriate, a growing number of devices are now specifically designed and approved for use in CHD. Advanced imaging is now an integral part of interventional procedures including pre-procedure planning, intra-procedural guidance, and post-procedure monitoring. There is robust societal and industrial support for research and development of CHD-specific devices, and the regulatory framework at the national and international level is patient friendly. It is against this backdrop that we review transcatheter implantable devices for CHD, the role and integration of advanced imaging, and explore the current regulatory framework for device approval.

Role of Cross-Sectional Imaging in Pediatric Interventional Cardiac Catheterization.

Management of congenital heart disease (CHD) has recently increased utilization of cross-sectional imaging to plan percutaneous interventions. Cardiac computed tomography (CT) and cardiac magnetic resonance (CMR) imaging have become indispensable tools for pre-procedural planning prior to intervention in the pediatric cardiac catheterization lab. In this article, we review several common indications for referral and the impact of cross-sectional imaging on procedural planning, success, and patient surveillance.

Presumptive Neonatal Multisystem Inflammatory Syndrome in Children Associated with Coronavirus Disease 2019.

OBJECTIVE: The study aimed to alert the neonatal community to the possibility of multisystem inflammatory syndrome in children (MIS-C) like disease in critically ill neonates born to mothers with coronavirus disease 2019 (COVID-19). STUDY DESIGN: Diagnosis of MIS-C like disease was pursued after echocardiography showed severely depressed ventricular function and pathological coronary artery dilation in the setting of medically refractory multisystem organ failure and maternal COVID-19 infection. The neonate did not respond to standard medical therapy, and there was no alternative disease that could explain the clinical course. High index of clinical suspicion coupled with low risk of intravenous immunoglobulin (IVIG) prompted us to pursue IVIG administration even though the neonate did not meet classic criteria for MIS-C. RESULT: Following treatment with IVIG, there was rapid clinical improvement. Ventricular function improved within 15 hours and coronary artery dilation resolved in 8 days. There was no recurrence of disease during follow-up. CONCLUSION: COVID-19 associated MIS-C like disease has not been well described in neonates. As typical features may be conspicuously absent, a high index of suspicion is warranted in critically ill neonates born to mothers with COVID-19. Echocardiography may provide critical diagnostic information and narrow the differential diagnosis. KEY POINTS: · COVID-19 associated MIS-C can present in neonates.. · Echocardiography is helpful in raising suspicion for MIS-C in neonates.. · Consider MIS-C in the differential diagnosis of ill neonates born to mothers with COVID-19..

Variation in the outer diameter of vascular sheaths commonly used in infant cardiac catheterization.

OBJECTIVE: To compare the outer diameter (OD) of conventional and radial sheaths from different manufacturers for sizes typically used in infant catheterization. BACKGROUND: The smallest sheath size is recommended to minimize risk of arterial injury in infants. However, sheath size refers to its internal diameter (ID), but it is the larger OD that determines risk. The OD varies between manufacturers and is frequently not known to the end user. METHODS: Three authors measured the OD of 3, 3.3, 4, 5, and 6 French (Fr) sheaths from different manufacturers using a Mitutoyo digital micrometer. Continuous variables are reported as mean ± SD. The midshaft and proximal-shaft sheath measurements were compared using an independent samples t test. Agreement between author measurements was tested using intra- and inter-rater reliability analysis. Manufacturer sheath OD measurements were obtained. Statistical significance was set at p < .05. RESULTS: There was no difference in the measured sheath diameters at the midpoint and proximally (p < .05). The intra-rater and inter-rater reliability were excellent (Intraclass correlation coefficient 1.0). Among conventional sheaths the OD varied between manufacturers, Terumo sheaths had the smallest and Galt sheaths had the largest OD. Radial sheaths had the smallest OD (1 Fr smaller) when compared to similar sized conventional sheaths. For instance, the OD of 4 Fr radial sheath (1.610 ± 0.006 mm) is essentially the same as the OD of the conventional 3 Fr (1.644 ± 0.016 mm) and 3.3 Fr (1.635 ± 0.005 mm) sheaths. CONCLUSIONS: Our study shows variation in the OD of sheaths used in infant catheterization. The radial sheaths offer the smallest OD across sizes.

Novel direct approach for placement of permanent transvenous pacing leads after Fontan procedure.

The need for transvenous pacing (patients who have exhausted epicardial options) after a Fontan-type operation has been recognized. Many novel strategies have been proposed, but currently, all of them require additional maneuvers or rerouting of the leads to the pacemaker pocket. In this report, we describe a novel direct approach to transvenous pacing after a Fontan-type operation from a standard, prepectoral approach.

Modified Stumper technique for acute postoperative bifurcation stenosis causing right ventricular failure after Ross procedure.

In this report, we describe a 15-year-old patient who underwent a Ross procedure for a regurgitant bicuspid aortic valve and ascending aortic dilation. After the operation was over, he could not be separated from cardiopulmonary bypass and was noted to have isolated right ventricular failure. This report takes the reader through the diagnostic evaluation, highlights the importance of invasive assessment in the immediate postoperative period, and discusses successful transcatheter intervention in the acute postoperative setting.

Native atrial septal restriction after Fontan palliation successfully treated with transcatheter Diabolo stent.

A 6-year-old male child born with hypoplastic left heart syndrome (HLHS) was palliated with an extracardiac nonfenestrated Fontan procedure (18-mm Gore-Tex tube graft). He developed low-pressure (mean Fontan pressure 10 mmHg) protein-losing enteropathy 6 months after Fontan palliation. After initially responding to medical therapy and transcatheter pulmonary artery stent implantation, he developed medically refractory protein-losing enteropathy. At this time, his transthoracic echocardiogram showed new restriction across his native atrial septum with an 8 mmHg mean gradient. Cardiac catheterization now showed high-pressure (mean Fontan pressure 18-20 mmHg) protein-losing enteropathy and a new 6 mmHg mean gradient across the atrial septum. To avoid cardiopulmonary bypass, he underwent successful transcatheter relief of atrial septal restriction and creation of a fenestration with rapid clinical and biochemical improvement of his protein-losing enteropathy.

Modified Technique to Create Diabolo Stent Configuration.

Diabolo stent configuration aids in stent positioning, stability, and creating a controlled defect with a predetermined size. A number of techniques to create the diabolo configuration have been previously described. The indications for creating a controlled "defect" are rapidly growing and include the Fontan circulation, patients with severe end-stage pulmonary hypertension, restrictive atrial communication in the setting of hypoplastic right or left heart syndrome, and diastolic left heart failure. We describe an alternative technique using a prefabricated readily available tool (gooseneck snare) to create a diabolo stent configuration. The chosen balloon expandable stent is mounted on a 5-mm gooseneck snare centered on an angioplasty catheter larger than 5 mm diameter. When deployed, the snare restricts the central waist to 5 mm and both ends expand to a larger diameter creating a dumbbell/diabolo configuration. A total of six diablo stent configurations were successfully implanted in four patients with failing Fontan physiology; five to create a transcatheter fenestration and one to relieve atrial septal restriction. Data expressed as median and IQR. Their weight was 24.8 kg (19.6-46.95), and age years was 9.2 (6.28-13.23). There were no complications and a consistent diabolo configuration with a 5-mm central waist was created in all patients. The snare serves as a sterile, preconfigured, radiopaque, readily available tool of adequate length and strength, to create consistent diabolo stent configuration without any modifications. This technique is a simple, reproducible, and easy to learn.

CRISP: Catheterization RISk score for Pediatrics: A Report from the Congenital Cardiac Interventional Study Consortium (CCISC).

OBJECTIVES: We sought to develop a scoring system that predicts the risk of serious adverse events (SAE's) for individual pediatric patients undergoing cardiac catheterization procedures. BACKGROUND: Systematic assessment of risk of SAE in pediatric catheterization can be challenging in view of a wide variation in procedure and patient complexity as well as rapidly evolving technology. METHODS: A 10 component scoring system was originally developed based on expert consensus and review of the existing literature. Data from an international multi-institutional catheterization registry (CCISC) between 2008 and 2013 were used to validate this scoring system. In addition we used multivariate methods to further refine the original risk score to improve its predictive power of SAE's. RESULTS: Univariate analysis confirmed the strong correlation of each of the 10 components of the original risk score with SAE attributed to a pediatric cardiac catheterization (P < 0.001 for all variables). Multivariate analysis resulted in a modified risk score (CRISP) that corresponds to an increase in value of area under a receiver operating characteristic curve (AUC) from 0.715 to 0.741. CONCLUSION: The CRISP score predicts risk of occurrence of an SAE for individual patients undergoing pediatric cardiac catheterization procedures.

CRISP: catheterization RISk score for pediatrics: a report from the congenital cardiac interventional study consortium (CCISC)

OBJECTIVES:We sought to develop a scoring system that predicts the risk of serious adverse events (SAE's) for individual pediatric patients undergoing cardiac catheterization procedures. BACKGROUND: Systematic assessment of risk of SAE in pediatric catheterization can be challenging in view of a wide variation in procedure and patient complexity as well as rapidly evolving technology. METHODS: A 10 component scoring system was originally developed based on expert consensus and review of the existing literature. Data from an international multi-institutional catheterization registry (CCISC) between 2008 and 2013 were used to validate this scoring system. In addition we used multivariate methods to further refine the original risk score to improve its predictive power of SAE's. RESULTS: Univariate analysis confirmed the strong correlation of each of the 10 components of the original risk score with SAE attributed to a pediatric cardiac catheterization (P < 0.001 for all variables). Multivariate analysis resulted in a modified risk score (CRISP) that corresponds to an increase in value of area under a receiver operating characteristic curve (AUC) from 0.715 to 0.741. CONCLUSION: The CRISP score predicts risk of occurrence of an SAE for individual patients undergoing pediatric cardiac catheterization procedures.

The lateral plane delivers higher dose than the frontal plane in biplane cardiac catheterization systems.

The objective of the study is to compare radiation dose between the frontal and lateral planes in a biplane cardiac catheterization laboratory. Tube angulation progressively increases patient and operator radiation dose in single-plane cardiac catheterization laboratories. This retrospective study captured biplane radiation dose in a pediatric cardiac catheterization laboratory between April 2010 and January 2014. Raw and time-indexed fluoroscopic, cineangiographic and total (fluoroscopic + cineangiographic) air kerma (AK, mGy) and kerma area product (PKA, µGym(2)/Kg) for each plane were compared. Data for 716 patients were analyzed: 408 (56.98 %) were male, the median age was 4.86 years, and the median weight was 17.35 kg. Although median beam-on time (minutes) was 4.2 times greater in the frontal plane, there was no difference in raw median total PKA between the two planes. However, when indexed to beam-on time, the lateral plane had a higher median-indexed fluoroscopic (0.75 vs. 1.70), cineangiographic (16.03 vs. 24.92), and total (1.43 vs. 5.15) PKA (p < 0.0001). The median time-indexed total PKA in the lateral plane is 3.6 times the frontal plane. This is the first report showing that the lateral plane delivers a higher dose than the frontal plane per unit time. Operators should consciously reduce the lateral plane beam-on time and incorporate this practice in radiation reduction protocols.

Hemodynamic assessment with interventional support should be routine for primary electrophysiology procedures after atrial switch procedure.

OBJECTIVES: We sought to review our current philosophy that all primary invasive electrophysiologic (EP) studies in patients with atrial switch procedures (ASPs) should undergo hemodynamic evaluation and have interventional expertise available. BACKGROUND: Patients who have undergone an ASP for dextro-transposition of the great arteries have a high incidence of both hemodynamic and EP sequelae. We present our data to support the combined assessment approach for these patients. METHODS: Hemodynamic evaluation and interventions performed concurrently during a primary invasive EP procedure in patients with ASP were reviewed. RESULTS: A total of 18 patients underwent concurrent EP invasive procedure and cardiac catheterization. The median age was 31 (14-43 years) with the majority being male (67%). Patients underwent a total of 30 concurrent primary invasive EP procedure and cardiac catheterization; 14 (47%) of the catheterization procedure were interventional. Some of the catheterization procedures required more than one intervention with total of 19 separate interventions. There were nine (47%) unexpected interventions. The majority of patients (n = 14, 77.8%) had one or more abnormal hemodynamic finding including baffle obstruction (n = 13, 72%), elevated filling pressures (n = 3, 17%), and secondary pulmonary hypertension (n = 3, 17%). Non-EP-related interventional procedures included systemic or pulmonary venous baffle stenting for significant obstruction (n = 7). EP-related interventions included transbaffle puncture for ablation of left-sided reentry circuits (n = 5), closure of previously undiagnosed baffle leaks prior to pacemaker/implantable cardioverter defibrillator (ICD) placement to prevent paradoxical embolism (n = 3), superior baffle stenting to facilitate pacemaker/ICD lead placement (n = 2), and retrieval of retained transvenous pacemaker/ICD lead (n = 2). CONCLUSION: Due to the frequency of abnormal hemodynamics or interventional needs, strong consideration for routine concurrent hemodynamic assessment and availability of interventional expertise is warranted during primary invasive EP procedures in patients post ASP.

Prograde transcatheter aortic arch intervention in patients with single-ventricle physiology: a word of caution.

OBJECTIVE: We sought to review and analyze the hemodynamic derangements during prograde transcatheter aortic intervention (PTAI) in single ventricle patients. BACKGROUND: Although PTAI for postsurgical recurrent coarctation in single ventricle patients has been described; hemodynamic instability during the intervention is variably reported. METHODS: Pre-, intra-, and postprocedural records and outcomes of patients with SVP undergoing PTAI for post-Norwood aortic coarctation were retrospectively reviewed. The full disclosure waveform review was used to further categorize hemodynamic derangements during the intervention. RESULTS: A total of 26 PTAIs were performed in 11 patients between October 2007 and December 2013. The median age and weight was 4.2 (2.3-43) months and 5.3 (3.2-15.7) kg. PTAI included balloon angioplasty (BA) in 73% of procedures (n = 19) and stent implantation (SI) in 27% (n = 7). Hemodynamic derangement was more severe in the SI group compared with the BA group. Two of seven (29%) of the SI group required cardiopulmonary resuscitation. CONCLUSIONS: Hemodynamic instability during PTAI is common in patients with SVP and more profound during SI. These findings have important implications for informed consent, anesthetic considerations, inotropic support, additional central venous access, and extracorporeal support/surgical backup.

Standardizing radiation dose reporting in the pediatric cardiac catheterization laboratory-a multicenter study by the CCISC (Congenital Cardiovascular Interventional Study Consortium).

OBJECTIVES: We examine normalized air Kerma area product (PKA ) by body weight (PKA /BW) as a reference value of radiation dose and benchmark PKA /BW in pediatric laboratories using a multicenter registry database. BACKGROUND: Reduction of radiation dose is an important quality improvement task in pediatric cardiac catheterization laboratories. Physicians need to agree on a standard method of reporting radiation dose that would allow comparisons to be made between operators and institutions. METHODS: This was a multicenter observational study of radiation dose in pediatric laboratories. Patient demographic, procedural and radiation data including fluoroscopic time and PKA (µGy m(2) ) were analyzed. PKA /BW was obtained by indexing PKA to body weight. RESULTS: A total of 8,267 pediatric catheterization procedures (age <18 years) were included from 16 institutions. The procedures consisted of diagnostic (n = 2,827), transplant right ventricular (RV) biopsy (n = 1,172), and interventional catheterizations (n = 4268). PKA correlated with body weight better than with age and best correlated with weight-fluoroscopic time product. PKA /BW showed consistent values across pediatric ages. Interventional catheterizations had the highest PKA /BW (50th, 75th, and 90th percentiles: 72, 151, and 281 µGy m(2) /kg), followed by diagnostic (59, 105, and 175 µGy m(2) /kg) and transplant RV biopsy (27, 79, and 114 µGy m(2) /kg). CONCLUSION: PKA /BW appeared to be the most reliable standard to report radiation dose across all procedure types and patient age. We recommend PKA /BW to be used as the standard unit in documenting radiation usage in pediatric laboratories and can be used to evaluate strategies to lower radiation dosage in pediatric patients undergoing cardiac catheterizations. © 2014 Wiley Periodicals, Inc.

Standardizing Radiation Dose Reporting in thePediatric Cardiac Catheterization Laboratory—AMulticenter Study by the CCISC (CongenitalCardiovascular Interventional Study Consortium)

We examine normalized air Kerma area product (PKA) by body weight(PKA/BW) as a reference value of radiation dose and benchmark PKA/BW in pediatriclaboratories using a multicenter registry database. Background: Reduction of radiationdose is an important quality improvement task in pediatric cardiac catheterizationlaboratories. Physicians need to agree on a standard method of reportingradiation dose that would allow comparisons to be made between operators andinstitutions. Methods: This was a multicenter observational study of radiation dosein pediatric laboratories. Patient demographic, procedural and radiation data includingfluoroscopic time and PKA (mGy m2) were analyzed. PKA/BW was obtained byindexing PKA to body weight. Results: A total of 8,267 pediatric catheterization procedures(age <18 years) were included from 16 institutions. The procedures consistedof diagnostic (n52,827), transplant right ventricular (RV) biopsy (n51,172),and interventional catheterizations (n54268). PKA correlated with body weight betterthan with age and best correlated with weight–fluoroscopic time product. PKA/BWshowed consistent values across pediatric ages. Interventional catheterizations hadthe highest PKA/BW (50th, 75th, and 90th percentiles: 72, 151, and 281 lGy m2/kg),followed by diagnostic (59, 105, and 175 lGy m2/kg) and transplant RV biopsy (27,79, and 114 lGy m2/kg). Conclusion: PKA/BW appeared to be the most reliablestandard to report radiation dose across all procedure types and patient age. We recommend PKA/BW to be used as the standard unit in documenting radiationusage in pediatric laboratories and can be used to evaluate strategies to lower radiationdosage in pediatric patients undergoing cardiac catheterizations.