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Aortic arch stent placement in young children is a medical dilemma. This is attributed to the critical lack of commercially available stents that can be delivered through small sheaths and yet have the potential to be dilated to the adult-size aorta. Here in, we describe an innovative first-in-human technique that allows for overcoming the aforementioned challenges. A Palmaz Genesis XD stent was placed to treat coarctation of the aorta in two young children through small-bore sheaths.

There has been a paradigm shift in the management of patients with congenital heart disease with a move away from conventional surgical treatment in favor of a percutaneous catheter-based approach across the spectrum of valvular heart diseases. The Sapien S3 valve implantation in the pulmonary position has been previously reported using a conventional transcatheter approach in patients with pulmonary insufficiency due to an enlarged right ventricular outflow tract. In this report, we present 2 unique cases of intraoperative hybrid implantation of Sapien S3 valves in patients with complex pulmonic and tricuspid valvular disease.

Neonatal myocardial infarction due to coronary thrombus is a rare cause of acute heart failure and is associated with high morbidity and mortality. We present a rare case of a full-term newborn who developed coronary artery thrombus treated with intracoronary recombinant tissue plasminogen activator infusion while undergoing therapeutic hypothermia. Also, we describe a unique treatment strategy to support systemic circulation sparing the patient from neonatal extracorporeal membrane oxygenation and its complications. Neonatal myocardial infarction should be suspected and ruled out in sick newborns.

Percutaneous transhepatic venous access has been utilised for numerous transcatheter cardiac procedures. Traditionally, a large transhepatic tract requires the placement of permanent occlusion devices or coils. We describe a successful closure using a simple technique (Surgifoam) without the need for metal hardware placement. Immediate hemostasis was achieved. No complications were encountered.

The objective of this study was to evaluate the safety and efficacy of combining transcatheter pulmonary valve replacement (TPVR) and electrophysiology (EP) procedures. A retrospective review was undertaken to identify TPVR and EP procedures that were concomitantly performed in the cardiac catheterization laboratory at University of Iowa Stead Family Children’s Hospital from January 2011 to October 2019. Procedural and follow-up data were compared between patients who underwent TPVR and EP procedures in the same setting to those who received TPVR or EP procedure separately and that were similar in age and cardiac anatomy. A total of 8 patients underwent combined TPVR and EP procedures. One patient was excluded due to lack of adequate control, leaving seven study subjects (57% female; median age at time of procedure 16 years). The median follow-up time was 11.5 months (range 2–36 months). Patients who received combined TPVR and EP had shorter recovery times (combined: median 18.9 h; IQR 18.35–19.5 vs separate: median 27.98 h; IQR 21.42–39.25; p-value 0.031), shorter hospital length of stay (combined: median 27.5 h; IQR 26.47–31.4 vs separate: median 38.4 h; IQR 33.42–51.50; p-value 0.016), and a 51% reduction in total hospital charges (combined: median $171,640; IQR 135.43–219.22 vs separate: median $333,560 IQR 263.20–400.98; p-value 0.016). There were no significant differences in radiation dose or procedure time between the combined and control groups. The median radiation time for those who had the combination procedure was 30.5 min [IQR 29.6–47.9], and the median dose area product was 215 mGy [IQR 158–935]. In conclusion, combining TPVR and EP procedures is feasible, safe, and economically advantageous.

Transcatheter closure of ventricular septal defects is considered first-line therapy when anatomically appropriate but is often challenged by proximity to the conduction system in perimembranous defects, or irregular defect shape, especially residual defects that may remain post-operatively. Advancements in device design, however, have allowed for significant improvements in deployment techniques and overall safety. Here we describe the first use of the Lifetech Konar-multifunction™ occluder device in North America, and our specific use of this device to close complex post-operative muscular and a perimembranous-ventricular septal defects in the same patient.

Background: Numerous studies have detailed outcomes of Transcatheter pulmonary valve placement (TPV) in young patients, but there is a paucity of data in older patients. We sought to evaluate short and mid-term outcomes of TPV in patients older than 40 years of age. Methods: Retrospective multicenter study of patients older than 40 years at the time of TPV from January 2010 to February 2018. Demographics, procedure details, and follow up data were collected. Results: A total 75 subjects presented for TPV with median age of 49.8 (IQ 45–60). Half of the patients 39 (52%) had evidence of NYHA class III or IV at time of presentation. A total of 65 (87%) had successful TPV. Patients with severe PR had immediate resolution of PI (except for one). Patients with PS had significant improvement in gradient from median of 25.5 to 5.5 mmHg (p

Introduction: Newer research has highlighted significant neurological impairments in children and adults with congenital heart disease (CHD). The more severe the heart defect, the higher the neurologic impairment. Pregnancies complicated by fetal or maternal CHD carry a higher risk of placental abnormalities. We studied the association between pregnancy hormonal levels and fetal head and body growth. Hypothesis: Placental dysfunction in pregnancies with fetal or maternal CHD alters pregnancy hormonal levels affecting fetal brain and somatic growth. Methods: We performed a retrospective case-control study on pregnant women (year 2010-2019) at the University of Iowa. Only women with first and/or second trimester prenatal screening (ultrasound and blood test) were included. Pregnancies with fetal chromosomal abnormalities, multiple gestation, maternal diabetes, smoking, or hypertension were excluded. Pregnancies were either healthy controls (n=36), women with CHD (MCHD; n=26), or fetus with CHD (FCHD; n=23). Pregnancy hormonal levels, ultrasound findings, and fetal/neonatal growth percentiles and/or z-scores data were analyzed. Results: Women with CHD were younger (p=0.004) with increased fetal nuchal translucency (p=0.003) compared to controls. Women in MCHD (p=0.02) and FCHD (p=0.02) group delivered earlier than controls. FCHD had significantly lower pregnancy associated plasma protein-A (PAPP-A) levels than controls (p=0.04). The groups had no difference in the second trimester fetal head circumference (HC) and femur length. FCHD group had significantly smaller HC percentile (p=0.03) and z-scores (p=0.03) at birth than controls. Both FCHD and MCHD had smaller birth weight and length compared to controls. However, only FCHD group demonstrated significantly lower HC to birth weight ratio (p=0.01). The controls had a positive correlation between human chorionic gonadotropin level and head circumference z-score at birth (r=0.34;p=0.053). Conclusions: Pregnant women with CHD have increased fetal nuchal translucency and deliver at earlier gestational age. Women with fetal CHD have smaller neonatal head and body size at birth with associated low PAPP-A level early in their pregnancy probably related to placental dysfunction.

Introduction: Transcatheter pulmonary valve implantation (TPVi) is a widely available option to treat right ventricular outflow tract (RVOT) conduits, but coronary artery compression is an absolute contraindication. Cardiac magnetic resonance (CMR) may evaluate coronary anatomy, but its utility in predicting coronary compression is not well established. Hypothesis: CMR prior to TPVi can accurately predict coronary compression risk. Methods: We analyzed all patients with a recent CMR (≤ 12 months) and attempted TPVi in an RVOT conduit at 9 centers from 2007-2016. A core lab reviewed all CMRs for the shortest orthogonal distance from a coronary artery to the conduit, the shortest distance from a coronary artery to the most stenotic area of the conduit, and subjective assessment of coronary compression risk. Receiver operating characteristic curve was used to determine optimal predictive distances. Univariate and independent associations of the distances and qualitative assessment with coronary compression were examined using logistic regression. Results: Of 231 patients (62% male, median age 19.0 years), TPVi was successful in 198 (86%); in 24 (10%) balloon testing (documented coronary compression or high risk) precluded implantation. Distance to the RV to PA conduit ≤ 2.1 mm (area under the curve [AUC] 0.70) and distance to most stenotic area ≤ 13.1 mm (AUC 0.69) predicted coronary compression (Table). Subjective assessment had the highest AUC (0.78), with 96% negative predictive value. Both distances and qualitative assessment remained independently associated with coronary compression when controlling for abnormal coronary anatomy. Conclusions: CMR can predict the risk of coronary compression during TPVi in RVOT conduits but cannot completely exclude the risk of coronary compression. CMR may assist in patient selection and counselling families prior to TPVi, although balloon testing prior to TPVi remains essential.

We report a case of a 15-year-old female who underwent combined hybrid pulmonary valve replacement and transcatheter atrial septal defect device closure, which was performed due to severe volume overload of the right side of the heart secondary to pulmonary regurgitation and atrial septal defect.

Branch pulmonary artery valve implantation has been reported in larger patients with dysfunctional right ventricular outflow tracts via routine femoral access. Here, we report treatment of severe pulmonary insufficiency with bilateral branch pulmonary artery Melody valve implantation (Medtronic, Minneapolis, Minnesota). To the best of our knowledge, this is the first report of bilateral valve implantation utilising the hybrid approach in a small-size patient.

Objectives Arch branching has never been shown to influence recoarctation after extended end-to-end anastomosis via thoracotomy, yet in each study bovine arch identification is grossly underreported. This study aims to (1) assess chart review reliability in bovine arch identification; (2) determine recoarctation risk with a bovine arch; and (3) explore an anatomic explanation for recurrent arch obstruction based on arch anatomy. Patients A total of 49 consecutive patients underwent thoracotomy with extended end-to-end aortic coarctation repair at a single institution (2007-2012). Methods Echocardiograms from these patients were reviewed for arch anatomy and compared with the echocardiographic reports. Recurrent arch obstruction was defined as an echocardiographic gradient across the repair of 20 mm Hg or greater. For cases with angiographic images (n = 17), a scaled clamping distance between the left subclavian artery and the maximal proximal clamp location on orthogonal projections was then calculated across arch anatomies. Results Chart review identified 6.1% (3/49) of patients with a bovine arch compared with 28.6% (14/49) on targeted image review. A total of 28.6% (4/14) of patients with a bovine arch had a follow-up gradient of 20 mm Hg or greater. Only 5.7% (2/35) of patients with normal arch branching had a follow-up gradient of 20 mm Hg or greater. The mean clamping index was significantly diminished in patients with bovine arch anatomy. Conclusions Arch anatomy often goes undocumented on preoperative imaging, yet children undergoing extended end-to-end repair with bovine arch anatomy are at a significantly increased risk of recoarctation. This may be due to a reduced clampable distance to facilitate repair. These results should be considered in the preoperative assessment, parental counseling, and surgical approach for children with discrete aortic coarctation.

In cases where femoral access is untenable for secundum atrial septal defect closure, the internal jugular vein can be used instead. We report a case of atrial septal defect closure in a patient with significant vascular thrombosis, requiring the use of the left internal jugular vein for access. To the best of our knowledge, this is the first report that documents the use of the left internal jugular vein for atrial septal defect closure in a patient with situs solitus anatomy.

Background The arterial switch operation is standard of care for infants born with dextrotransposition of the great arteries. Supravalvar pulmonary stenosis is a common complication that may require reintervention—balloon angioplasty, stenting, or surgical augmentation. A subset of patients requires more than one reintervention. Methods We performed a retrospective review of patients who underwent the arterial switch operation for dextrotransposition of the great arteries at a single institution between August 1990 and January 2014. Anatomic, perioperative and follow-up data were collected. Reinterventions were stratified in a site-specific manner. Statistical analysis was performed using IBM SPSS version 21 (IBM Corp, Armonk, NY). Results Of the 103 patients who met inclusion criteria, 28% (29) required reintervention for supravalvar pulmonary stenosis; 41% of those receiving primary reintervention required an additional 21 reinterventions. Balloon angioplasty of the main pulmonary artery and left pulmonary artery was associated with the need for multiple reinterventions (odds ratio 4.9, p = 0.051, and odds ratio 5.1, p = 0.029, respectively). Freedom from future reintervention at the main pulmonary artery and left pulmonary artery was significantly shorter after balloon angioplasty relative to alternative reintervention options (hazard ratio 10, p = 0.005, and hazard ratio 3.2, p = 0.02, respectively). Balloon angioplasty of the right pulmonary artery was not associated with an increased risk of reintervention ( p = 0.42). Conclusions Supravalvar pulmonary stenosis after the arterial switch operation for dextrotransposition of the great arteries is common and more than one reintervention are required in a subset of patients. The benefit of balloon angioplasty of the main pulmonary artery and left pulmonary artery was shown to be temporary. Attempting balloon angioplasties at these locations remain reasonable, although families should be counseled about the increased incidence of, and decreased time to, subsequent reintervention that is associated with this treatment option.

Supravalvular aortic stenosis as an early complication of transposition of the great artery repair is rare with few cases reported. Furthermore, transcatheter intervention is uncommon as surgical re-intervention has been traditionally done. We describe two cases of supravalvular aortic stenosis at the anastomotic site as an early complication of the arterial switch operation. Both patients underwent balloon angioplasty of the supravalvular aorta with improvement in postangioplasty gradients and angiographic appearance. Both patients at short-term follow-up had persistent improved gradient without need for further intervention.

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.

Introduction A 24-year-old female presented for percutaneous pulmonary valve placement. She was born with tetralogy of Fallot and had initial palliation with a Blalock–Taussig shunt followed by complete repair at age of 4 years including placement of a homograft conduit in the right ventricle to pulmonary artery position. She had developed severe obstruction in the conduit. Procedure Angiography showed a heavily calcified conduit with moderate insufficiency. During pre-dilation, a total of six balloons ruptured due to heavy conduit calcification prior to reaching desired inflation diameter. Subsequently, double balloon technique was attempted using two 9-mm Conquest balloons. One of the conquest balloons was then replaced by 16-mm Atlas balloon and conduit dilation was performed. At full inflation, the Conquest balloon ruptured. The deflated ruptured Conquest balloon was kept in the conduit and the Atlas balloon was exchanged for 18 and then 20-mm Atlas balloons and both were used to dilate the conduit. The deflated ruptured Conquest balloon protected the Atlas balloons and conduit angioplasty proceeded successfully without further rupture of any balloon. Subsequently, four stents were placed in the conduit followed by delivery of Melody Valve using a 20-mm Ensemble system with excellent results. Conclusion This case illustrates a novel method of using the body of a ruptured balloon to protect subsequent balloons from rupture due to heavy conduit calcification. This method requires the presence of two venous access lines but might save time, effort, and cost from repeated balloon ruptures. © 2015 Wiley Periodicals, Inc.

Background— Transcatheter pulmonary valve replacement (TPVR) is an established therapy for dysfunctional right ventricular (RV) outflow tract conduits. TPVR in patients with congenitally corrected transposition of the great arteries, subpulmonary left ventricle, and left ventricular outflow tract (LVOT) conduit dysfunction has not been studied. Unique anatomic and physiological aspects of this population may contribute to distinct risks and outcomes. Methods and Results— Across 10 US centers, 27 patients with a dysfunctional LVOT conduit were evaluated in the catheterization laboratory between December 2008 and August 2015 with the intent to perform TPVR. TPVR was successful in 23 patients (85%). Five serious adverse events occurred in 4 cases (15%), including pulmonary hemorrhage, hypotension requiring vasoactive support, conduit disruption requiring covered stent (n=2), and acute RV dysfunction with flash pulmonary edema. After TPVR, the LVOT peak systolic ejection gradient decreased from median of 35 to 17 mm Hg ( P P =0.02) and higher post-TPVR RV sphericity index (median 0.88 versus 0.52; P =0.004). Post-TPVR, there were 2 late deaths because of RV failure and 1 cardiac transplantation because of progressive RV dysfunction and tricuspid regurgitation. Conclusions— TPVR in dysfunctional LVOT conduits is feasible but associated with an important rate of TPV nonimplantation and procedural serious adverse events. Worsening systemic RV function and tricuspid regurgitation may develop after LVOT TPVR.

OBJECTIVES We sought to evaluate outcomes of stent implantation (SI) for recurrent coarctation of the aorta (RC) following the Norwood operation. BACKGROUND RC is common following the Norwood operation. Balloon angioplasty (BA) is standard treatment but may result in unsatisfactory relief of RC. SI may improve RC, but outcome data are limited. METHODS We performed a multi-center retrospective study of patients who underwent SI for RC between the Norwood operation and Fontan completion. Outcomes were examined, including procedural success, serious adverse events (SAE), and freedom from re-intervention. A core laboratory was utilized to review angiograms. Coarctation Index (CI) was calculated before and after SI. Paired t-test and Wilcoxon signed-rank test were used to compare pre- and post-SI variables. RESULTS Thirty-three patients at 8 centers underwent SI for RC at a median age of 5 months (IQR 4.1, 13.3) and weight of 5.9 kg (5.2, 8.6). Aortic arch gradient improved from 20 (15, 24) to 0 (0, 2) mmHg following SI (P

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.

An infant diagnosed by fetal echocardiography with hypoplastic left heart syndrome and suspected severe restriction of the foramen ovale developed postdelivery peripheral oxygen desaturation as low as 30% soon after transfer from the neonatal nursery to the cardiac intensive care unit. Supplemental oxygen was administered via nasal cannula and the infant underwent emergent cardiac catheterization soon after birth. The catheterization revealed cor triatriatum and an obstructed vertical vein with aberrant pulmonary venous drainage. This anatomy provided significant challenges for the anesthesia and cardiology teams. The difficulties in anesthetic, diagnostic and interventional management and decision-making in this complex anatomical diagnosis are discussed and the literature is reviewed.

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.

Objective To determine whether superior vena cava (SVC) stent implantation is superior to balloon angioplasty for relieving SVC stenosis. Background SVC stent and balloon dilation have been used as treatment for SVC stenosis. Although safe and effective, outcome data comparing the two methods are limited. Methods A Pediatric Cardiac Care Consortium review identified SVC stenosis. Patients who required SVC intervention were divided into two subgroups—balloon dilation (Group A) and stent implantation (Group B). Logistic regression and the log-rank test were used to test the need for re-intervention within 6 months after the initial procedure. Results SVC intervention was performed on 210/637 patients with SVC stenosis (33%). There were 108/210 (51%) patients with balloon dilation (Group A) and 102/210 (49%) with stent implantation (Group B). Re-intervention within 6 months of the initial intervention was more common in Group A compared to Group B [Group A = 31/40 (77.5%); Group B = 5/22 (22.7%)]. The odds-ratio for re-intervention within 6 months of the initial procedure for balloon vs. stent, is 7.3 [95% CI: (2.91, 22.3), P < 0.0001]. In addition, during the first 6 months after an intervention for SVC stenosis the proportion of patients with stent implantation that remained free of re-intervention was significantly higher than after balloon angioplasty (log-rank test, P < 0.0001). Neither age nor weight was significantly associated with the need for re-intervention. Conclusions SVC stent implantation is more effective than angioplasty in relief of SVC obstruction. Weight and age are not risk factors for early re-intervention. © 2012 Wiley Periodicals, Inc.

PE can occur following HCT. However, the incidence, etiology, risk factors, and treatment remain unclear. We performed a retrospective study evaluating 355 pediatric recipients of HCT treated at a single institution between January 2005 and August 2010. No cases of PE were identified in the autologous HCT (auto-HCT) recipients (0/43), while 19% (57/296) of allogeneic HCT (allo-HCT) developed PE. Among the 57 PE patients, 40 (70%) were males; the median age at transplantation was 6.6 yr (0.1-17.3 yr). Thirty-six patients (63%) had significant PE with 23 patients (40%) treated by pericardiocentesis, and 19 (33%) experiencing recurrent PE. OS rates for patients who developed PE were 84% at 100 days and 65% at three yr after HCT. Risk factors associated with PE on multivariate analysis included myeloablative conditioning (p = 0.01), delayed neutrophil engraftment (p < 0.01), and CMV + serostatus of the recipient (p = 0.03). Recipients with non-malignant diseases were significantly less likely to die after development of PE (p = 0.02 and 0.004 when comparing with standard and high-risk diseases, respectively). In summary, PE is a common and significant complication of pediatric allo-HCT. Prospective studies are needed to better determine the etiology and optimal method of PE treatment after HCT.

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.

A 24-year-old female presented for percutaneous pulmonary valve placement. She was born with tetralogy of Fallot and had initial palliation with a Blalock-Taussig shunt followed by complete repair at age of 4 years including placement of a homograft conduit in the right ventricle to pulmonary artery position. She had developed severe obstruction in the conduit.Angiography showed a heavily calcified conduit with moderate insufficiency. During pre-dilation, a total of six balloons ruptured due to heavy conduit calcification prior to reaching desired inflation diameter. Subsequently, double balloon technique was attempted using two 9-mm Conquest balloons. One of the conquest balloons was then replaced by 16-mm Atlas balloon and conduit dilation was performed. At full inflation, the Conquest balloon ruptured. The deflated ruptured Conquest balloon was kept in the conduit and the Atlas balloon was exchanged for 18 and then 20-mm Atlas balloons and both were used to dilate the conduit. The deflated ruptured Conquest balloon protected the Atlas balloons and conduit angioplasty proceeded successfully without further rupture of any balloon. Subsequently, four stents were placed in the conduit followed by delivery of Melody Valve using a 20-mm Ensemble system with excellent results.This case illustrates a novel method of using the body of a ruptured balloon to protect subsequent balloons from rupture due to heavy conduit calcification. This method requires the presence of two venous access lines but might save time, effort, and cost from repeated balloon ruptures.

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.

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.

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.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.Hemodynamic evaluation and interventions performed concurrently during a primary invasive EP procedure in patients with ASP were reviewed.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).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.