Incidence, Predictors, and Outcomes of Cardiac Perforation During Pediatric Cardiac Catheterization: a Retrospective Observational Study from the Congenital Cardiac Interventional Study Consortium (CCISC).

INTRODUCTION: Cardiac perforation is a rare life-threatening complication of cardiac catheterization. There is very little published literature detailing risk factors for cardiac perforation and outcomes from this complication in children. MATERIALS AND METHODS: This was a retrospective study analyzing the cardiac catheterization case registry of the Congenital Cardiovascular Interventional Study Consortium. Children aged < 18 years were included during the study period of 9 years (January 2009-December 2017). The primary outcome measures were incidence of cardiac perforation, risk factors for and outcomes of patients who experience cardiac perforation during cardiac catheterization. RESULTS: Cardiac perforation occurred in 50 patients from a total of 36,986 (0.14%). Cardiac perforation was more likely to occur in younger, smaller patients undergoing urgent/emergent and interventional procedures (p < 0.01). Cardiac peroration risk was significantly different across diagnostic and procedure categories (p < 0.01). Higher CRISP score (Area Under Curve [AUC] = 0.87), lower age, and procedure category (radiofrequency perforation of pulmonary valve, AUC = 0.84) were independent predictors of cardiac perforation. Cardiac perforation was associated with a significantly higher rate of mortality (14%), further emergency procedure (42%), ECMO (14%), and cardiac arrest (6%), p < 0.01. CONCLUSIONS: Cardiac perforation during cardiac catheterization is a life-threatening complication with a range of associated secondary complications. Higher CRISP score, lower age, and radiofrequency perforation of pulmonary valve are independent predictors.

Transjugular venous approach for Piccolo patent ductus arteriosus closure in a 1.4 kg infant.

Transfemoral venous approach is the standard method for transcatheter patent ductus arteriosus closure using the Amplatzer Piccolo patent ductus arteriosus occluder in small infants. We report a 1.4 kg infant who underwent transcatheter Piccolo patent ductus arteriosus closure. Transjugular venous approach was taken due to the unexpected finding of interrupted inferior vena cava.

Pericardiocentesis in children: 20-year experience at a tertiary children's hospital.

BACKGROUND: Pericardiocentesis is the invasive percutaneous procedure for acute and chronic excessive accumulation of pericardial fluid. There is a paucity of data on the effectiveness and safety of pericardiocentesis in children. OBJECTIVES: To evaluate the effectiveness and safety of pericardiocentesis and factors associated with acute procedural failure and adverse events. METHODS: This was a single-centered retrospective study to describe all the children aged ≤20 years who underwent pericardiocentesis. Data on demographics, etiologies of pericardial effusion, and repeat intervention at follow-up were collected. RESULTS: A total of 127 patients underwent 153 pericardiocentesis. The median age was 6.5 years (1 day-20 years) with weight of 17 kg (0.5-125). Most common etiology was post-pericardiotomy syndrome (n = 56, 44%), followed by infectious (12%), malignant (10%), and iatrogenic (9%). Pericardiocentesis was performed more commonly in the catheterisation laboratory (n = 86, 59%). Concurrent pericardial drain placement was performed in 67 patients (53%). Acute procedural success was 92% (141/153). Repeat intervention was performed in 33 patients (22%). The incidence of adverse events was 4.6% (7/153): hemopericardium requiring emergent surgery (n = 2); hemopericardium with hypotension (n = 2); seizure with anesthesia induction (n = 1); and right ventricle puncture with needle (n = 2). Pericardiocentesis at the bedside had a higher rate of acute procedural failure than that in the catheterisation lab (17 versus 1%, p < 0.01). No identifiable risk factors were associated with adverse events. CONCLUSIONS: Pericardiocentesis was life-saving in children with its high effectiveness and safety even in urgent situations. Although initial pericardiocentesis was effective, one of five patients required re-intervention for recurrent pericardial effusion.

Optimal criteria for transcatheter closure of Fontan fenestration: a single-center experience with a review of literature.

Fenestration closure is considered to remove the persistent right-to-left shunt after the Fontan operation. However, the criteria for effective transcatheter closure of fenestration to avoid both acute and chronic Fontan failure have not been clarified. The objective of this study was to describe the hemodynamic data with test occlusion of the Fontan fenestration for patients who underwent a closure and those who did not, along with subsequent development of Fontan-associated diseases (FAD) at follow-up. This was a retrospective study conducted to assess the outcome of Fontan fenestration closure at Children's Hospital of Michigan over 27 years (1993-2019). The inclusion criteria were patients undergoing cardiac catheterization indicated for Fontan fenestration closure. Data were compared between the two groups: closure and non-closure patients. Baseline characteristics and hemodynamic variables with the fenestration occlusion test were analyzed. The primary outcome was the development of composite events of death/transplant, deteriorated New York Heart Association class symptoms, or FAD. Among the 38 patients who were brought to the catheterization laboratory, 33 received fenestration closure and 5 did not. On a median follow-up of 3.4 years (range, 1 month-12.6 years), the incidence of primary adverse outcomes was 13% (5/38). The incidence of primary outcome was significantly higher in the non-closure group (60% vs. 6%, p < 0.01). The non-closure group had a higher incidence of moderate or severe atrioventricular valve regurgitation, New York Heart Association class III symptoms, use of angiotensin-converting enzyme inhibitor/angiotensin receptor blocker, furosemide use, and sildenafil use. The multivariable logistic regression model showed that the hemodynamic variables associated with the non-closure group were mean left atrial pressure (odds ratio 1.74, p < 0.05) and change of mean Fontan pressure at the balloon occlusion (odds ratio 2.2, p < 0.05). The judgment of fenestration closure appeared appropriate in our cohort. Fontan fenestration closure may not be advisable in cases with a high baseline left atrial pressure or a significant increase in Fontan pressure on balloon occlusion testing.

Left Transjugular Venous Approach for Transcatheter Pulmonary Valve Implantation using Sapien Valve.

Transfemoral and right transjugular approach has been described for transcatheter pulmonary valve implantation (TPVI). However, the use of left transjugular approach through the left superior vena cava to coronary sinus has not been well described. We present a 22-year-old male with Tetralogy of Fallot status post transannular patch, who underwent successful TPVI using the Sapien valve, preceded by pre-stenting of native right ventricular outflow tract. All the transcatheter intervention was performed through the left superior vena cava to coronary sinus.

Femoral Artery Thrombosis After Pediatric Cardiac Catheterization.

Femoral artery thrombosis (FAT) is an adverse event post-catheterization. We decided to study the incidence and risk factors of FAT before and after initiation of a pulse loss monitoring program. All cardiac catheterization cases performed using femoral arterial vascular access were retrospectively reviewed. The study period was divided into two eras: before (era 1), and after the initiation of the protocol (era 2). The primary outcome was FAT requiring therapy. Univariable and multivariable logistic regression models were used to identify factors associated with FAT. Receiver operating characteristic curve were generated to predict FAT. Our cohort consisted of 2,388 cases including 467 (19.6%) infants weighing < 10 kg. The overall incidence of FAT was 1.7% (n = 41). The incidence of FAT was 3.5 times higher in era 2 (p < 0.001). Multivariable model showed that era, weight, sheath exchange, and coarctation intervention were significantly associated with FAT. Infants younger than 7.7 months and less than 6.7 kg were found to be at highest risk for FAT. Our study shows that the incidence of FAT may be underestimated without a monitoring program. Small infants and coarctation interventions requiring larger sheaths or sheath exchanges are at highest risk for FAT.

Membranous septal aneurysm causing severe right ventricular outflow obstruction in an adult with trisomy 18.

Membranous ventricular septal aneurysm is a known entity but rarely causes severe right ventricular outflow obstruction. We report a 40-year-old female with trisomy 18 who developed severe right ventricular outflow obstruction caused by an enormous membranous septal aneurysm associated with unrepaired inlet ventricular septal defect with perimembranous extension.

Single center experience of pediatric percutaneous balloon pericardiotomy.

BACKGROUND: Percutaneous balloon pericardiotomy is a percutaneous procedure that creates a window in the parietal pericardium by balloon dilation. The use of percutaneous balloon pericardiotomy has not been reported well in children. OBJECTIVES: The objective of this study was to describe the single centre experience of percutaneous balloon pericardiotomy in children. METHODS: This was a retrospective study to describe all the children aged <20 years undergoing percutaneous balloon pericardiotomy during an 18-year period (2001-2019). Patient characteristics, technical and ultimate procedural success, and repeat interventions were collected. RESULTS: A total of 13 percutaneous balloon pericardiotomy's were performed in 11 children at the median age of 12 years (range 1.8-19). The etiologies of pericardial effusion were post-pericardiotomy syndrome (n = 4), restrictive cardiomyopathy (n = 1), autoimmune diseases (n = 3), malignancy (n = 2), and idiopathic (n = 1). Two patients received two percutaneous balloon pericardiotomy. The technical success of percutaneous balloon pericardiotomy was 100% with no acute adverse events (balloon rupture or local bleeding). Five (45%) required re-intervention and ultimately three required a surgical pericardial window 6 to 35 days after the percutaneous balloon pericardiotomy. As a result, ultimate procedural success rate was 73% (8/11). CONCLUSION: Percutaneous balloon pericardiotomy was performed safely with high technical success in children. Percutaneous balloon pericardiotomy may be considered for recurrent and persistent pericardial effusion, before considering a surgical pericardial window.

Amplatzer Piccolo Occluder clinical trial for percutaneous closure of the patent ductus arteriosus in patients ≥700 grams.

OBJECTIVES: Characterize the safety and effectiveness of the Amplatzer Piccolo Occluder for patent ductus arteriosus (PDA) closure. BACKGROUND: The presence of a hemodynamically significant PDA has been associated with an increased risk of morbidity and mortality in children born premature. METHODS: This was a single arm, prospective, multicenter, non-randomized study to evaluate the Amplatzer Piccolo Occluder to treat PDA in patients ≥700 g. From June 2017 to February 2019, 200 patients were enrolled at nine centers, with 100 patients weighing ≤2 kg. Primary effectiveness endpoint was the rate of PDA closure at 6-month follow-up. Primary safety endpoint was the rate of major complications through 6 months. Secondary endpoint was rate of significant pulmonary or aortic obstruction through 6 months' follow-up. RESULTS: The implant success rate was 95.5% (191/200) overall and 99% in patients ≤2 kg (99/100). The primary effectiveness endpoint was achieved in 99.4% of implanted patients. Four patients experienced a primary safety endpoint event (2 transfusions, 1 hemolysis, and 1 aortic obstruction). There were no branch pulmonary artery obstructions. Five patients, all ≤2 kg, were noted to have worsening of tricuspid regurgitation (TR) after the procedure. None of the TR incidences manifested clinically. The Amplatzer Piccolo Occluder received FDA approval in January 2019 and became the first device approved for PDA closure in patients ≥700 g. CONCLUSIONS: This study supports the safety and effectiveness of the Amplatzer Piccolo Occluder, particularly in patients between 700 g and 2 kg where there is currently a significant unmet need in the United States. ClinicalTrials.gov identifier: NCT03055858.

Hot topics in interventional cardiology: Proceedings from the society for cardiovascular angiography and interventions 2020 think tank.

The society for cardiovascular angiography and interventions (SCAI) think tank is a collaborative venture that brings together interventional cardiologists, administrative partners, and select members of the cardiovascular industry community for high-level field-wide discussions. The 2020 think tank was organized into four parallel sessions reflective of the field of interventional cardiology: (a) coronary intervention, (b) endovascular medicine, (c) structural heart disease, and (d) congenital heart disease (CHD). Each session was moderated by a senior content expert and co-moderated by a member of SCAI's emerging leader mentorship program. This document presents the proceedings to the wider cardiovascular community in order to enhance participation in this discussion, create additional dialogue from a broader base, and thereby aid SCAI and the industry community in developing specific action items to move these areas forward.

Partnership Between Japan and the United States for Early Development of Pediatric Medical Devices　- Harmonization By Doing for Children.

BACKGROUND: The Harmonization By Doing (HBD) program was established in 2003 as a partnership among stakeholders of academia, industry and regulatory agencies in Japan and the United States, with a primary focus on streamlining processes of global medical device development for cardiovascular medical devices. While HBD has traditionally focused on development of devices intended to treat conditions prevalent in adults, in 2016, HBD established the "HBD-for-Children" program, which focuses on the development of pediatric devices as the development of medical devices for pediatric use lags behind that of medical devices for adults in both countries.Methods and Results:Activities of the program have included: (1) conducting a survey with industry to better understand the challenges that constrain the development of pediatric medical devices; (2) categorizing pediatric medical devices into five categories based on global availability and exploring concrete solutions for the early application and regulatory approval in both geographies; and (3) facilitating global clinical trials of pediatric medical devices in both countries. CONCLUSIONS: The establishment of the HBD-for-Children program is significant because it represents a global initiative for the introduction of pediatric medical devices for patients in a timely manner. Through the program, academia, industry and regulatory agencies can work together to facilitate innovative pediatric device development from a multi-stakeholder perspective. This activity could also encourage industry partners to pursue the development of pediatric medical devices.

Saccular "Cauliflower" aneurysm of coarctation of aorta: a hybrid management.

Aneurysm formation around the site of coarctation of aortic arch is a well-recognised complication of untreated coarctation and is associated with an increased risk of aortic rupture and mortality. We present a rare case in a teenage girl with the combination of significant aortic arch coarctation, a "cauliflower-like" saccular aneurysm, and stenosis at the origin of the left subclavian artery. She was successfully managed with a hybrid approach, which is a combination of an endovascular surgical repair (a bypass graft placement from left carotid artery to subclavian artery by a vascular surgeon) and a transcatheter covered stent placement across the stenosis and aneurysm. This case highlights the successful role of a hybrid approach in patient's who present with a combination of coarctation of the aorta and aortic arch aneurysms. This approach avoids the conventional surgical aortoplasty, which carries a higher mortality and morbidity risk in teenage patients.

Recurrent infective endocarditis in a patient after a second Melody valve placement.

A 16-year-old Caucasian male was diagnosed with recurrent infective endocarditis associated with his second Melody valve placed inside a previously treated infected Melody valve. The replacement of Melody valve after the first infective endocarditis episode might increase the risk of the second episode of infective endocarditis.

Validation and refinement of the catheterization RISk score for pediatrics (CRISP score): An analysis from the congenital cardiac interventional study consortium.

OBJECTIVES: To externally validate the CRISP score, and determine if refinements might improve clinical utility. BACKGROUND: The CRISP score estimates risk of serious adverse events (SAEs) for pediatric catheterization. METHODS: Pediatric (age < 18) procedures reported to the Congenital Cardiovascular Interventional Study Consortium registry from 05/08 to 09/17 (n = 29,830, 27 centers) were divided into a development dataset of 14,784 earlier procedures, and a validation dataset of 15,046 more recent procedures. The development dataset was used to refit the original CRISP model, and to develop a revised(r) CRISP score, consisting of entirely pre-procedurally collected data. The validation dataset was then used to compare model fit and risk prediction between CRISP, rCRISP and two existing risk scores using Akaike's (AIC), Schwarz's (BIC) Bayes Information Criteria, -log Likelihood (N2LL), area under the receiver operator curve and chi-square goodness-of-fit statistic (across 5 risk categories). RESULTS: Overall 4.31% of patients experienced at least one SAE with frequency increasing from 1.08% in CRISP category 1 to 27.34% in category 5. Both CRISP and rCRISP (entirely pre-procedural) predicted risk of SAEs well, with observed to predicted ratios ranging from 0.71 to 1.18 across the 5 risk categories. Compared to the original CRISP score, rCRISP demonstrated less optimal model fit (higher AIC, BIC, and N2LL) but similar risk prediction (C-statistic = 0.71 vs. 0.70; chi-squared statistic = 6.77 vs. 6.85). CONCLUSION: The CRISP score accurately predicts procedural risk. With minor modifications, the revised version (rCRISP) performed well with arguably greater clinical utility as an entirely preprocedural risk model.

Results of the combined U.S. multicenter postapproval study of the Nit-Occlud PDA device for percutaneous closure of patent ductus arteriosus.

OBJECTIVES: To report the results of the Nit-Occlud PDA prospective postapproval study (PAS) along with a comparison to the results of the pivotal and continued access trials. BACKGROUND: The Nit-Occlud PDA (PFM Medical, Cologne, Germany), a nitinol coil patent ductus arteriosus (PDA) occluder, was approved by the Food and Drug Administration in 2013. METHODS: The PAS enrolled a total of 184 subjects greater than 6 months of age, weighing at least 5 kg, with PDAs less than 4 mm by angiography at 11 centers. Patients were followed prospectively at 2 months, 12 months, and 24 months postprocedure. These outcomes were compared to the 357 subjects enrolled in the pivotal and continued access protocols. Efficacy and safety data were reported. RESULTS: Among 184 subjects enrolled for the PAS between 2014 and 2017, 180 (97.8%) had successful device implantation. After 12 months, 98.7% (150/152) had trivial or no residual shunt by echocardiography and two subjects had only small residual shunts. There were three device embolizations that were all retrieved by snare without clinical consequence. Together with the pivotal and continued access study, 97.4% (449/461) had complete echocardiographic closure at 12 months in 541 enrolled subjects. The composite success was 94.4%. There were no mortalities and no serious device-related adverse events. CONCLUSIONS: The Nit-Occlud PDA is a safe and effective device for closure of a small to moderate sized PDA. There were no serious device-related adverse events in a large cohort of three clinical trials.

Catheter-directed therapy for acute pulmonary embolism in children.

BACKGROUND: Acute pulmonary embolism is a life-threatening condition and rarely occurs in children. In adults, catheter-directed therapy emerges as a potentially safer and effective therapeutic option. However, there is a paucity of data on the safety and efficacy of catheter-directed therapy for pulmonary embolism in children. We report a single-centred experience of catheter-directed therapy for acute pulmonary embolism in children. METHODS: This is a retrospective study of children who had no CHD and underwent catheter-directed therapy at Detroit Medical Center during a 12-year period from 2005 to 2017. Demographic and clinical data associated with pulmonary embolism were collected along with the outcome. RESULTS: A total of nine patients of median age 16 years with the range from 12 to 20 received catheter-directed therapy for sub-massive (n = 6) and massive pulmonary embolism (n = 3). Among nine patients, one patient received Angiojet thrombectomy and balloon angioplasty, whereas eight patients received catheter-directed thrombolysis using tissue plasminogen activator through infusion catheters (n = 3) or EkoSonic ultrasound-accelerated thrombolysis system (n = 5). In four out of five patients treated with EkoSonic, significant clinical improvement was noticed within 24 hours. Among seven patients who survived, two patients had minor gastrointestinal bleeding with median hospital stay of 8 days with the range from 5 to 24 days, and two patients with massive pulmonary embolism died possibly due to delayed institution of catheter-directed therapy. CONCLUSION: Catheter-directed therapy with/without EkoSonic is an emerging alternative therapy for sub-massive and massive pulmonary embolism in children. A timely institution of catheter-directed therapy appeared important to improve the outcome.

Bilateral branch pulmonary artery valve implantation in repaired tetralogy of fallot.

BACKGROUND: Transcatheter, bilateral branch pulmonary artery (PA) valve implantation is a novel treatment for patients with severe pulmonary insufficiency and oversized right ventricle (RV) outflow tract. There is scarce data on efficacy and safety of this approach. METHODS: This was a retrospective study of 8 patients with repaired tetralogy of fallot (TOF) who underwent bilateral branch PA valve implantation. Demographics, echocardiography, cardiac catheterization, and axial imaging data were reviewed. Variables were compared by a paired sample t-test. RESULTS: All patients were adult sized (weight 43-99 kg) with oversized RV outflow tract not suitable for conventional transcatheter pulmonary valve implantation. Staged bare metal PA stenting followed by valve implantation (interval 3-5 months) was technically successful in 7 patients with one stent embolization. In another patient, proximal stent migration prevented placement of bilateral pulmonary valve stents. There were a total of 14 valved branch PA stents placed (Melody valve n = 9, Sapien XT n = 2, Sapien 3 n = 3). In the 7 patients undergoing successful branch pulmonary valve placement, at median follow up of 10 months (range 3 months to 6 years), 13 (93%) valves had none/trivial insufficiency on echocardiography. Prevalve and postvalve implantation cardiac magnetic resonance imaging in five patients showed significant reduction of indexed RV end-diastolic volume (152 ± 27 to 105 ± 15 mL/m2 , P < .001). CONCLUSIONS: Transcatheter, bilateral branch PA valve implantation was technically feasible with satisfactory efficacy and safety in patients with repaired TOF, severe pulmonary insufficiency, and oversized RV outflow tracts. Elimination of pulmonary insufficiency with this method resulted in reduced RV end-diastolic volume. This approach can be offered as an alternative to surgery, particularly in patients considered high risk for standard surgical placement and who are not candidates for the newer self-expanding valve prosthesis for placement in RV outflow tracts larger than 30 mm diameter.

Transcatheter therapy of anomalous systemic venous drainage.

Anomalous drainage of the right superior caval vein into the left atrium is a rare congenital anomaly that causes cyanosis and occult infection owing to right-to-left shunting. Transcatheter management of this anomaly is unique and rarely reported. We report a 32-year-old man with a history of brain abscess, who was diagnosed with an anomalous right superior caval vein draining to the left atrium; right upper pulmonary vein and right middle pulmonary vein draining into the inferior portion of the right superior caval vein; and a left superior caval vein draining into the right atrium through the coronary sinus without a bridging vein. Pre-procedural planning was guided by three-dimensional printed model. The right superior caval vein was occluded with a 16-mm Amplatzer muscular Ventricular Septal Defect occluder inferior to the azygous vein, but superior to the entries of right upper and middle pulmonary veins. This diverted the right superior caval vein flow to the inferior caval vein system through the azygos vein in a retrograde manner and allowed the right upper pulmonary vein and right middle pulmonary vein flow to drain into the left atrium normally, achieving exclusion of right-to-left shunting and allowing normal drainage of pulmonary veins into the left atrium. At the 6-month follow-up, his saturation improved from 93 to 97% with no symptoms of superior caval vein syndrome.

Parental anxiety among children undergoing cardiac catheterisation.

BACKGROUND: Parents may experience anxiety and stress when their children undergo cardiac catheterisation. The goal of this study was to assess the level of anxiety in parents of children undergoing cardiac catheterisation and to identify factors that were associated with level of anxiety. METHODS: This was a cross-sectional survey of parents of children who underwent cardiac catheterisation. Anxiety levels were measured using a validated self-report questionnaire - State-Trait Anxiety Inventory, which generates state anxiety scores on the current state of anxiety and trait anxiety scores on the stable aspects of anxiety proneness. One sample t-test was used to compare the data with normative data. Multiple linear regression was used to assess the factors associated with the state score. RESULTS: A total of 113 parents completed the survey. The mean age of parents was 34.0±7.7 years and the mean age of children undergoing catheterisation was 6.7±5.7 years. Compared with normative data, mean state score was significantly higher in our cohort (p<0.05) despite no difference in the trait score. Final multivariate model showed that the state score was significantly associated with child age group (<1 year [coefficient ß 7.2] and 10-18 years [6.3], compared to 1 to <10 years of age [reference]) and history of previous catheterisation (-5.2) (p<0.05). CONCLUSIONS: Concurrent state anxiety level was high among parents of children undergoing cardiac catheterisation, whereas trait anxiety level was not. Higher anxiety was experienced by parents of infants and adolescents without a history of previous catheterisation.

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.