Restriction of congenital portosystemic shunt using the modified microvascular plug.

Congenital portosystemic shunts (CPSS) may produce a variety of severe, clinically detrimental presentations. When indicated, closure is recommended; however, if the intrahepatic portal venous system (IPVS) is underdeveloped complete closure may not be possible and may result in severe acute portal hypertension. Staged restriction of CPSS flow by both surgical and complex transcatheter interventions has been successful in augmenting development of the IPVS such that complete occlusion of the CPSS can be performed. We report use of a modified microvascular plug to restrict CPSS flow with subsequent IPVS development and safe complete occlusion of CPSS.

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.

Transcatheter Tricuspid Valve-in-Valve Implantation for the Treatment of Dysfunctional Surgical Bioprosthetic Valves: An International, Multicenter Registry Study.

BACKGROUND: Off-label use of transcatheter aortic and pulmonary valve prostheses for tricuspid valve-in-valve implantation (TVIV) within dysfunctional surgical tricuspid valve (TV) bioprostheses has been described in small reports. METHODS AND RESULTS: An international, multicenter registry was developed to collect data on TVIV cases. Patient-related factors, procedural details and outcomes, and follow-up data were analyzed. Valve-in-ring or heterotopic TV implantation procedures were not included. Data were collected on 156 patients with bioprosthetic TV dysfunction who underwent catheterization with planned TVIV. The median age was 40 years, and 71% of patients were in New York Heart Association class III or IV. Among 152 patients in whom TVIV was attempted with a Melody (n=94) or Sapien (n=58) valve, implantation was successful in 150, with few serious complications. After TVIV, both the TV inflow gradient and tricuspid regurgitation grade improved significantly. During follow-up (median, 13.3 months), 22 patients died, 5 within 30 days; all 22 patients were in New York Heart Association class III or IV, and 9 were hospitalized before TVIV. There were 10 TV reinterventions, and 3 other patients had significant recurrent TV dysfunction. At follow-up, 77% of patients were in New York Heart Association class I or II (P<0.001 versus before TVIV). Outcomes did not differ according to surgical valve size or TVIV valve type. CONCLUSIONS: TVIV with commercially available transcatheter prostheses is technically and clinically successful in patients of various ages across a wide range of valve size. Although preimplantation clinical status was associated with outcome, many patients in New York Heart Association class III or IV at baseline improved. TVIV should be considered a viable option for treatment of failing TV bioprostheses.

Transcatheter valve implantation for right atrium-to-right ventricle conduit obstruction or regurgitation after modified Björk-fontan procedure.

BACKGROUND: The most common reason for reintervention after a Björk modification of the Fontan procedure, in which the right ventricle (RV) is incorporated into the pulmonary circulation by connecting the right atrial (RA) appendage to the RV directly or with an extra-anatomic graft, is obstruction or regurgitation of the RA-RV connection. Transcatheter implantation of a valved stent is an appealing option for the treatment of RA-RV conduit dysfunction in these patients. In the present study, we assessed early and intermediate results after transcatheter valve implantation within an obstructed or regurgitant RA-RV modified Fontan pathway. METHODS: Through a retrospective multicenter registry, we collected data from 16 patients with a modified Fontan circulation who were treated with percutaneous Melody or Sapien valve implantation for dysfunction of an extra-anatomic RA-RV conduit or valve. RESULTS: All patients had successful and uncomplicated implantation of a Melody (n = 15) or Sapien 3 (n = 1) valve with hemodynamic and, in most cases, clinical improvement. During a median follow-up of 3.3 years, 3 patients died of cardiovascular causes unrelated to the procedure or the valve, and no major valve dysfunction was observed. CONCLUSION: Percutaneous transcatheter valve deployment to treat a dysfunctional RA-RV connection after a Björk modification of the Fontan procedure is a viable alternative to surgery, with low procedural risk, and appears to offer good early and intermediate results.© 2016 Wiley Periodicals, Inc.

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.

Hemodynamic response to ketamine in children with pulmonary hypertension.

BACKGROUND: The safety of ketamine in children with pulmonary hypertension has been debated because of conflicting results of prior studies in which changes in mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR) have been widely variable. The goal of this prospective study was to quantitate the effects of ketamine on pulmonary hemodynamics in a cohort of children with pulmonary hypertension under conditions in which variables such as airway/ventilatory management, FiO(2), and use of vasodilating anesthetics were controlled. METHODS: The IRB approved this study of 34 children undergoing cardiac catheterization for pulmonary hypertension studies. Following anesthetic induction with sevoflurane and tracheal intubation facilitated by the administration of rocuronium 0.7-1 mg·kg(-1) iv, sevoflurane was discontinued and anesthesia was maintained with midazolam 0.1 mg·kg(-1) iv (or 0.5 mg·kg(-1) po preoperatively) and remifentanil iv infusion 0.5-0.7 mcg·kg(-1) ·min(-1). Ventilation was mechanically controlled to maintain PaCO(2) 35-40 mmHg. When endtidal sevoflurane was 0% and FiO(2) was 0.21, baseline heart rate (HR), mean arterial pressure (MAP), mPAP, right atrial pressure (RAP), pulmonary artery occlusion pressure (PAOP), right ventricular end-diastolic pressure (RVEDP), cardiac output, and arterial blood gases were measured, and indexed systemic vascular resistance (SVRI), indexed pulmonary vascular resistance (PVRI), and cardiac index (CI) were calculated. Each child then received a bolus of ketamine 2 mg·kg(-1) infused over 2 min. Measurements and calculations were repeated 2 min after the conclusion of the infusion. RESULTS: The mean (95% CI) increase in mPAP following ketamine was 2 mmHg (0.2, 3.7), which was statistically significant but clinically insignificant. PVRI and PVRI/SVRI did not change significantly. Hemodynamic changes did not differ among subjects with differing severity of pulmonary hypertension or between subjects chronically treated with pulmonary vasodilators or not. CONCLUSION: Ketamine is associated with minimal, clinically insignificant hemodynamic changes in sedated, mechanically ventilated children with pulmonary hypertension.

Diagnostic Utility of Three-Dimensional Rotational Angiography in Congenital Cardiac Catheterization.

We evaluated the diagnostic utility of the three modalities of three-dimensional rotational angiography (3DRA): rotational angiography (RA), multiplanar reformat (MPR) and three-dimensional angiographic reconstruction (3D-R) in pediatric cardiac catheterization. The 3DRA studies were classified by anatomy of interest based on our injection protocol: pulmonary arteries (PA), aorta, cavopulmonary connection (CPC), and others. Retrospective review of 3DRA images by two reviewers for each modality was conducted with grading as inferior, similar, or superior in comparison with the diagnostic quality of fixed-plane angiography (FPA). The percentages of grades for each modality were averaged. Weighted kappa statistic was used to evaluate inter-rater reliability. In total, 114 3DRA studies were performed on 87 patients between August 2010 and March 2012. Median age was 2.7 years (1 day-48.4 years) and median weight 12.1 kg (3.6-106.5 kg). For RA: 79.4 % of the studies were of diagnostic quality and 52.2 % were superior; 3D-R: 82 % were of diagnostic quality and 65.8 % were superior; and MPR: 83.5 % were of diagnostic quality and 63 % were superior. Overall 3DRA technologies (RA, 3D-R, MPR) were of diagnostic quality or better in 111/114 (97.4 %) studies and 103/114 (90.4 %) were judged superior. Most common reasons for inferior grading were limited opacification and metallic artifact. In pediatric cardiac catheterization, 3DRA imaging was of diagnostic quality and frequently provided additional clinically relevant data when compared to FPA.

Use of rotational angiography in assessing relationship of the airway to vasculature during cardiac catheterization.

OBJECTIVES: We are the first to describe the use of three-dimensional rotational angiography (3DRA) in creating multiplanar reconstruction (MPR) and volume rendering, in the catheterization suite, of airways at risk for compression by adjacent cardiac structures. BACKGROUND: 3DRA has emerged as a promising tool for improved visualization of cardiac and vascular structures in congenital heart disease. METHODS: This is a retrospective review of all available cases at our institution in which MPR from 3DRA was used to assess airways in relation to surrounding cardiovascular structures. RESULTS: Eight cases were reviewed from January 1, 2011 to November 30, 2013. Seven children had complex biventricular anatomy, including repaired truncus arteriosus, repaired absent pulmonary valve, repaired double outlet right ventricle, and vascular rings. One child had double inlet left ventricle and had undergone a hybrid procedure (stenting of the patent ductus arteriosus and banding of bilateral pulmonary arteries) before the Glenn procedure. Six of these cases involved distortion or stenosis of the pulmonary conduit or branch pulmonary arteries. In all cases, the trachea and the main bronchi were clearly visualized using MPR. Management was affected by the visualization of the airways during the catheterization procedure in seven of eight cases. Four cases had intraprocedural bronchoscopy that confirmed airway findings seen by MPR. In one case, computed tomography confirmed left bronchial compression seen by MPR. CONCLUSIONS: 3DRA can visualize airway anatomy and its relationship to the vasculature accurately. This has significant implications for preinterventional planning, intraprocedural management, as well as postprocedural recovery.

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.

Pulmonary Artery Stent Implantation.

Pulmonary artery stent implantation has become integral in the treatment of pulmonary artery stenosis and is probably the most efficacious therapy for these lesions. Advancements in technology involving stent design and the equipment used for stent delivery have made this procedure much safer and more effective. Strategies to mitigate and successfully treat adverse events related to pulmonary artery stent implantation are reasonably well-established. Pulmonary artery stent implantation remains one of the most complex and technically challenging of congenital cardiac interventions.

Cryo-balloon angioplasty for pulmonary vein stenosis in pediatric patients.

This study sought to determine the safety and effectiveness of cryo-balloon angioplasty (CbA) for pulmonary vein stenosis (PVS) in pediatric patients. Current therapy options for PVS are less than satisfactory due to recurrent progressive restenosis and neointimal proliferation. Catheterization database, hospital records, imaging studies, and pathologic specimens were reviewed for procedural-related and outcomes data in all patients who underwent pulmonary vein (PV) CbA using the Boston Scientific PolarCath Peripheral Dilation System between August 2006 and June 2009. Thirteen patients (19 PVs; median age 13 months [range 3.5 months to 18.5 years] and weight 7.9 kg [range 3.8 to 47.7]) underwent CbA. Mean PVS diameter after CbA increased from 2.19 (± 0.6) to 3.77 (± 1.1) mm (p < 0.001). Mean gradient decreased from 14 (± 7.4) to 4.89 (± 3.2) mm Hg (p < 0.001). Mean stenosis-to-normal vein diameter ratio increased from 0.52 (± 0.15) to 0.89 (± 0.33) (p < 0.001). Eight patients underwent repeat catheterization a mean of 5.6 months (± 3.66) later. Improved PVS diameter was maintained in 2 PVs. Four veins had restenosis but maintained diameters greater than that before initial CbA. In 11 PVs, the diameter decreased from 4.28 (± 1.14) to 2.53 (± 0.9) mm (p = 0.001). Mean gradient increased from 3.55 (± 3.0) to 14.63 (± 9.6) mm Hg (p = 0.011). All vessels underwent repeat intervention with acute relief of PVS. Stroke occurred within 24 h of CbA in 1 patient. CbA of PVS is safe and results in acute relief of stenosis. However, CbA appears minimally effective as the sole therapy in maintaining long-term relief of PVS.

Clinical manifestations and long-term follow-up in pediatric patients living at altitude with isolated pulmonary artery of ductal origin.

This study's aim was to define the clinical manifestations and long-term outcome of pediatric patients living at altitude with isolated pulmonary artery (PA) of ductal origin (IPADO). This was a retrospective cohort study of 17 consecutive cases of IPADO at a single center. All patients lived at modest altitude (median 2050 m [range 1700 m to 3050 m]). Fifteen children (88%) were symptomatic at presentation. High-altitude pulmonary edema was present in 2 patients (12%) at diagnosis, and only 1 patient had episodes of hemoptysis during follow-up. Fourteen patients (82%) demonstrated evidence of pulmonary arterial hypertension (PAH). Among 14 patients with PAH, 11 patients had surgical interventions. PAH resolved in 5 of 11 patients (45%) undergoing surgical rehabilitation. One patient died during follow-up, and 7 patients are receiving oral vasodilator therapies due to residual PAH; 14 patients remained asymptomatic. Our study showed that early intervention in patients with IPADO at modest altitude can potentially rehabilitate the isolated PA and reverse PAH. Whether surgery is indicated for patients with this disorder in the absence of PAH is unknown.

Hybrid Transcarotid Exclusion of Postoperative Ascending Aortic Dissection in an Infant.

Aortic dissection is very rare in pediatric patients, and associated risk factors include congenital heart disease, previous cardiac surgery, and vasculopathies. Acute postoperative aortic dissection in pediatric patients can be life-threatening. We performed a novel hybrid transcarotid covered stent exclusion of a postoperative ascending aortic dissection in an infant. (Level of Difficulty: Advanced.).

Left ventricular diastolic dysfunction in bronchopulmonary dysplasia.

We report 2 infants with severe bronchopulmonary dysplasia in whom left ventricular diastolic dysfunction contributed to clinical abnormalities, including pulmonary hypertension and recurrent pulmonary edema. We speculate that close monitoring for left ventricular diastolic dysfunction may assist with clinical management and improve outcomes of infants with severe bronchopulmonary dysplasia.

Outcomes of Transcatheter Tricuspid Valve-in-Valve Implantation in Patients With Ebstein Anomaly.

We sought to describe the acute results and short- to medium-term durability of transcatheter tricuspid valve-in-valve (TVIV) implantation within surgical bioprostheses among patients with Ebstein anomaly (EA). Cases were identified from a voluntary, multicenter, international registry of 29 institutions that perform TVIV. Demographic, clinical, procedural, and follow-up data were analyzed. Eighty-one patients with EA underwent TVIV from 2008 to 2016. Thirty-four patients (42%) were New York Heart Association (NYHA) class 3/4 at time of TVIV. The most common indication for TVIV was the presence of moderate or severe tricuspid regurgitation (40%). Most patients received a Melody valve (64%). TVIV was ultimately successful in all patients, and there was no procedural mortality. Four patients (5%) developed acute valve thrombosis, 4 patients (5%) developed endocarditis, and 9 patients (11%) developed valve dysfunction not related to thrombosis or endocarditis. Eight patients (10%) underwent reintervention (2 transcatheter, 6 surgical) due to thrombosis (3), endocarditis (2), other valve dysfunction (2), and patient-prosthesis mismatch without valve dysfunction (1). Among 69 patients who were alive without reintervention at latest follow-up, 96% of those with NYHA status reported were class 1/2, a significant improvement from baseline (62% NYHA class 1/2, p <0.001). In conclusion, transcatheter TVIV offers a low-risk, minimally invasive alternative to surgical tricuspid valve re-replacement in patients with EA and a failing tricuspid valve bioprosthesis.

Feasibility and Safety of Using a Fused Echocardiography/Fluoroscopy Imaging System in Patients with Congenital Heart Disease.

BACKGROUND: Fused real-time three-dimensional transesophageal echocardiography and fluoroscopy has been used in adult patients during percutaneous mitral valve and aortic valve procedures. The use of fused echocardiographic/x-ray fluoroscopic imaging (FEX) in pediatric patients undergoing congenital heart disease catheterization has not been evaluated for feasibility and safety. The aims of this study were to assess the feasibility and safety of FEX for interventional guidance and to perform a comparison of atrial septal defect (ASD) device closure using this technology with traditional guidance methods. METHODS: Prospective evaluation of FEX in congenital cardiac interventions was conducted. A subset of patients with ASD closures were compared with patients with historical ASD closures with and without FEX. The interventionalist and echocardiographer rated the anatomic quality of the fusion imaging as (1) excellent, (2) good, or (3) poor. In addition, the utility of FEX procedural guidance was graded as (1) superior, (2) no added benefit, or (3) inferior to that of standard guidance by fluoroscopy and transesophageal echocardiography. RESULTS: FEX was successfully used in 26 procedures on 25 patients with congenital heart disease from January 2013 to February 2015. The median age was 9 years (range, 3-26 years), and the median weight was 29 kg (range, 16-77 kg). Twenty-six procedures were performed, including ASD closure, Fontan fenestration closure, and transcatheter valve placement in the tricuspid valve position. There was reduced fluoroscopy time and radiation dose in patients with ASDs who underwent imaging using this new technology (P < .001 and P < .03, respectively). There were no statistically significant differences in procedural times between the two groups. Anatomic definition was rated as excellent in 20 of 26 procedures, with the remaining six rated was good. Twenty-one of 26 procedures were graded as superior (81%), and five of 26 (19%) were graded as providing no added benefit. There were no complications in any of the procedures. CONCLUSIONS: In this early experience, FEX is feasible and safe in patients undergoing congenital heart disease catheterization and provides useful guidance in the majority of interventional procedures. There were relative reductions in fluoroscopy time and radiation dose with the use of FEX for ASD closure.

Successful Treatment of Myocardial Infarction in an Infant With Kawasaki Disease.

Although early treatment with intravenous immunoglobulin reduces the risk of coronary artery aneurysms, in refractory cases of Kawasaki disease, myocardial infarction can result from thrombosis of coronary artery aneurysms. Early recognition of myocardial infarction from Kawasaki disease myocarditis can reduce morbidity and mortality. This report describes successful treatment of myocardial infarction from coronary thrombosis in an infant with Kawasaki disease using intravenous tissue plasminogen activator and abciximab.

Multimodality 3-dimensional image integration for congenital cardiac catheterization.

Cardiac catheterization procedures for patients with congenital and structural heart disease are becoming more complex. New imaging strategies involving integration of 3-dimensional images from rotational angiography, magnetic resonance imaging (MRI), computerized tomography (CT), and transesophageal echocardiography (TEE) are employed to facilitate these procedures. We discuss the current use of these new 3D imaging technologies and their advantages and challenges when used to guide complex diagnostic and interventional catheterization procedures in patients with congenital heart disease.

Aortoesophageal fistula and coarctation of the aorta in a 15-year-old child.

Delayed presentation of coarctation of the aorta can be associated with severe complications. A challenging case of aortoenteric fistula secondary to an aneurysm from coartation of the aorta presented with massive gastrointestinal hemorrhage. The clinical management of this patient and a review of the literature are presented.