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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. [ABSTRACT FROM AUTHOR]

Transcatheter pulmonary vein recanalisation is challenging. Herein, we described a novel technique facilitating the recanalisation of occluded three pulmonary veins in two small children. This technique enhances the catheter's alignment and stability during the recanalisation process. Referred to as the "anchoring guide catheter" technique, it can be applied only when the ipsilateral vein is patent.

Background: With improved survival of adults with congenital heart disease (CHD) comes a need to understand the lifelong outcomes of this population. The aim of this paper is to describe the rationale and design of Congenital Heart Disease Project to Understand Lifelong Survivor Experience (CHD PULSE), a study to determine long-term medical, neurocognitive, and psychosocial outcomes among adults with a history of intervention for CHD and to identify factors associated with those outcomes., Methods: CHD PULSE is a cross-sectional survey conducted from September 2021 to April 2023 among adults aged 18 and older with a history of at least 1 intervention for CHD at 1 of 11 participating U.S. centers in the Pediatric Cardiac Care Consortium. Participants with CHD were asked to complete a 99-question survey on a variety of topics including: demographics, surgeries, health insurance, health care, heart doctors, general health, height and weight, education and work history, reproductive health (for women only), and COVID-19. To construct a control group for the study, siblings of survey respondents were invited to complete a similar survey. Descriptive statistics for demographics, disease severity, center, and method of survey completion were computed for participants and controls. Comparisons were made between participants and non-participants to assess for response bias and between CHD participants and sibling controls to assess for baseline differences., Results: Among the 14,322 eligible participants, there were 3,133 respondents (21.9%) from 48 U.S. states with surveys returned for inclusion in the study. Sibling contact information was provided by 691 respondents, with surveys returned by 326 siblings (47.2%). The median age of participants was 32.8 years at time of survey completion, with an interquartile range of 27.2 years to 39.7 years and an overall range of 20.1 to 82.9 years. Participants were predominantly female (55.1%) and of non-Hispanic White race/ethnicity (87.1%). There were no differences between participants and non-participants regarding severity of CHD. Compared to nonparticipants, participants were more likely to be female, of older age, and be of non-Hispanic White race/ethnicity. Enrolled siblings were more likely to be female and slightly younger than participants., Conclusions: With surveys from 3,133 participants from across the U.S., CHD PULSE is poised to provide keen insights into the lifelong journey of those living with CHD, extending beyond mere survival. These insights will offer opportunities for informing strategies to enhance and improve future outcomes for this population of patients., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Transcatheter patent ductus arteriosus (PDA) closure (TCPC) utilizing transthoracic echocardiogram (TTE) as the sole imaging guide could simplify care. This single-center study compares PDA dimensions obtained from the TTE and angiogram images of patients who underwent attempted TCPC at Stead Family Children's Hospital from 10/01/2019 to 10/31/2020. Blinded investigators measured these dimensions solely for this study and had no impact on clinical care. Also, a hypothetical Piccolo device size was chosen based on the TTE dimensions and another on the angiographic dimensions, and then the correlation was analyzed. Sixty-two patients underwent TCPC attempts. TTE tends to overestimate the PDA narrowest dimension and underestimate the PDA length and aortic end dimension. Linear regression analysis revealed a weak correlation between the length and aortic diameter (R = 0.37 and 0.21, respectively). A modest correlation was observed for the smallest dimension without color Doppler (R = 0.57) and with color Doppler, which was utilized when needed (R = 0.6). Bland-Altman analysis revealed a smaller mean difference between the TTE and angiogram measurements of the narrowest diameter without color Doppler (0.4 mm) and with color Doppler (used as needed) (0.4 mm). However, the mean difference is larger for the aortic end (- 1.64 mm) and the length (- 1.73 mm). TTE accurately predicted the Piccolo device size in 43 (72%) patients and overestimated the size in 17 (28%) patients to the next size. Our findings should be verified with further studies, and additional development of protocols is needed to use TTE to guide TCPC without fluoroscopy., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Competing Interests: None.

Pulmonary vein stenosis continues to pose significant challenges in children, frequently requiring repeated cardiac catheterisation procedures. This report describes a successful application of a "triple kissing balloon" technique to treat complex pulmonary vein stenosis in two patients, all accomplished with the use of a single 6-French sheath.

Background: Although the Avalon Elite bi-caval dual lumen catheter for veno-venous extracorporeal membranous oxygenation (ECMO) has many advantages, it requires precise positioning and dislodgement is common. Case presentation: A 2-year-old male was placed on ECMO due to respiratory failure utilizing a 20 Fr Avalon Elite bi-caval dual lumen catheter (AEC). The AEC migrated twice with unsuccessful repositioning using the classic manual manipulations. The AEC was successfully repositioned on the two occasions using a novel method by direct access of the ECMO inflow tube using a combination of catheter and guide wire. Conclusions: A migrated AEC could be successfully repositioned with simple direct access of the inflow tube. This technique was successfully utilized twice at the bedside in an infant without needing additional venous access., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Safety and acute outcomes for patients who need catheterization shortly after congenital cardiac surgery has been established; literature on mid-term outcomes is lacking. We sought to evaluate the mid-term outcomes of patients who undergo early postoperative cardiac catheterization. This is a retrospective cohort study of patients who underwent cardiac catheterization within 6 weeks of congenital cardiac surgery with longitudinal follow-up and assessment of mid-term outcomes. Multivariable analysis was utilized to relate variables of interest to outcomes. 99 patients underwent cardiac catheterizations within 6 weeks of cardiac surgery between January 2008 and September 2016. Forty-six (45.5%) interventional procedures were performed at a median age of 41 days (IQR 21-192) and a median weight of 3.9 kg (3.3-6.6). During a median follow-up duration of 4.24 years (1.6-5.6) in study survivors, 61% of patients remained free from the primary endpoint (death and/or transplant). Sixty-nine patients (69.7%) underwent an unplanned surgical or catheter procedure. Renal failure at catheterization (OR 280.5, p 0.0199), inotropic medication at catheterization (OR 14.8, p 0.002), and younger age were all significantly associated with meeting the primary endpoint. Patients requiring surgical intervention as an initial additional intervention underwent more unplanned re-interventions, while patients who survived to hospital discharge demonstrated favorable mortality, though with frequent need for re-intervention. In patients requiring early postoperative cardiac catheterization, renal failure, younger age, and need for inotropic support at catheterization are significantly associated with meeting the primary endpoint., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

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.

Background: Definitive closure of the patent ductus arteriosus (PDA) is associated with significant changes in the loading conditions of the left ventricle (LV), which may lead to cardiovascular and respiratory instability. The objective of the study was to evaluate targeted neonatal echocardiography (TnECHO) characteristics and the clinical course of preterm infants ≤2 kg undergoing percutaneous PDA closure., Methods: Retrospective cohort study of prospectively acquired pre- and post-closure TnECHOs to assess hemodynamic changes. Cardiorespiratory parameters in the first 24 h following PDA closure were also evaluated., Results: Fifty patients were included with a mean age of 30.6 ± 9.6 days and weight of 1188 ± 280 g. LV global longitudinal strain decreased from -20.6 ± 2.6 to -14.9 ± 2.9% (p < 0.001) after 1 h. There was a decrease in LV volume loading, left ventricular output, LV systolic and diastolic parameters. Cardiorespiratory instability occurred in 24 (48%) [oxygenation failure in 44%] but systolic hypotension and/or need for cardiovascular medications was only seen in 6 (12%). Patients with instability had worse baseline respiratory severity score and lower post-closure early diastolic strain rates., Conclusions: Percutaneous PDA closure leads to a reduction in echocardiography markers of LV systolic/diastolic function. Post-closure cardiorespiratory instability is characterized primarily by oxygenation failure and may relate to impaired diastolic performance., Impact: Percutaneous patent ductus arteriosus closure leads to a reduction in echocardiography markers of left ventricular volume loading, cardiac output, and left ventricular systolic/diastolic function. Post-procedural cardiorespiratory instability is characterized primarily by oxygenation failure. Post-procedural cardiorespiratory instability may relate to impaired diastolic performance., (© 2022. The Author(s), under exclusive licence to the International Pediatric Research Foundation, Inc.)

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.

Transcatheter intervention on the pulmonary vein often requires performing transseptal puncture, which is a risky procedure. We describe a rare association of a partially unroofed coronary sinus with pulmonary vein stenosis causing desaturation in a young infant where the presence of the unroofed coronary sinus allowed for a novel interventional approach to the stenotic left pulmonary vein, avoiding the high risk of transseptal puncture.

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.

Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Background Until recently, a large right ventricle outflow tract interfered with the feasibility of standard transcatheter pulmonary valve replacement (PVR). We are describing our experience using a hybrid approach for PVR using a left anterior thoracotomy approach to allow for plication of the main pulmonary artery followed by a transcatheter PVR using a Sapien S3 valve. Methods and Results This is a single-center, retrospective review of patients who were evaluated to be appropriate for a hybrid PVR approach. The patients' demographics, procedure details, and follow-up data were collected. Between May 2018 and April 2021, a total of 11 patients presented for hybrid transcatheter PVR. The median age and weight were 24 years (interquartile range, 19-43 years) and 81.8 kg (interquartile range, 69-91 kg), respectively. Nine out of 11 patients received a transcatheter PVR after main pulmonary artery plication. There were no procedurally related deaths. One major complication was encountered in which the valve was malpositioned requiring successful surgical PVR. Minor complications included acute kidney injury (n=1) and a broken rib (n=1). The median length of stay was 4 days (interquartile range, 2-4 days), with median follow-up of 7 months (interquartile range, 3-18 months). A well-functioning pulmonary valve was observed in all patients at the last follow-up. Conclusions A hybrid approach using left anterior thoracotomy with pulmonary artery plication followed by transcatheter Sapien S3 PVR provides a less-invasive option for patients with an enlarged right ventricular outflow tract. Preliminary results demonstrated this to be a safe option with good short-term outcomes.

Background Prolonged exposure to a hemodynamically significant patent ductus arteriosus (PDA) is associated with major morbidity, particularly in infants born at <27 weeks' gestation. High-frequency jet ventilation (HFJV) is a standard of care at our center. There are no data about transcatheter PDA closure while on HFJV. The aim of this study was to assess the feasibility, safety, and outcomes of HFJV during transcatheter PDA closure. Methods and Results This is a retrospective cohort study of premature infants undergoing transcatheter device closure on HFJV. The primary outcome was successful device placement. Secondary outcomes included procedure time, fluoroscopy time and dose, time off unit, device complications, need for escalation in respiratory support, and 7-day survival. Subgroup comparative evaluation of patients managed with HFJV versus a small cohort of patients managed with conventional mechanical ventilation was performed. Thirty-eight patients were included in the study. Median age and median weight at PDA device closure for the HFJV cohort were 32 days (interquartile range, 25.25-42.0 days) and 1115 g (interquartile range, 885-1310 g), respectively. There was successful device placement in 100% of patients. There were no device complications noted. The time off unit and the procedure time were not significantly different between the HFJV group and the conventional ventilation group. Infants managed by HFJV had shorter median fluoroscopy times (4.5 versus 6.1 minutes; P <0.05) and no increased risk of adverse respiratory outcomes. Conclusions Transcatheter PDA closure in premature infants on HFJV is a safe and effective approach that does not compromise device placement success rate and does not lead to secondary complications.

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.

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.

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.

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.

Objectives and Background: Coarctation of the aorta represents 5-8% of all congenital heart disease. Although balloon expandable stents provide an established treatment option for native or recurrent coarctation, outcomes from transverse arch (TAO) stenting, including resolution of hypertension have not been well studied. This study aims to evaluate immediate and midterm results of TAO stenting in a multi-center retrospective cohort., Methods: TAO stenting was defined as stent placement traversing any head and neck vessel, with the primary intention of treating narrowing in the transverse aorta. Procedural details, complications and medications were assessed immediately post procedure, at 6 month follow-up and at most recent follow-up., Results: Fifty-seven subjects, 12 (21%) native, and 45 (79%) surgically repaired aortic arches, from seven centers were included. Median age was 14 years (4 days-42 years), median weight 54 kg (1.1-141 kg). After intervention, the median directly measured arch gradient decreased from 20 mmHg (0-57 mmHg) to 0 mmHg (0-23 mmHg) (p < .001). The narrowest arch diameter increased from 9 mm (1.4-16 mm) to 14 mm (2.9-25 mm) (p < .001), with a median increase of 4.9 mm (1.1-10.1 mm). One or more arch branches were covered by the stent in 55 patients (96%). There were no serious adverse events. Two patients warranted stent repositioning following migration during deployment. There were no late complications. There were 8 reinterventions, 7 planned, and 1 unplanned (6 catheterizations, 2 surgeries). Antihypertensive management was continued in 19 (40%) at a median follow-up of 3.2 years (0.4-7.3 years)., Conclusions: TAO stenting can be useful in selected patients for resolution of stenosis with minimal complications. This subset of patients are likely to continue on antihypertensive medications despite resolution of stenosis., (© 2020 Wiley Periodicals LLC.)

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.

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.

Background: Traditional approaches to pediatric cardiac catheterization have relied on femoral venous access. Upper- extremity venous access may enable cardiac catheterization procedures to be performed safely for diagnostic and interventional catheterizations. The objective of this multicenter study was to demonstrate the feasibility and safety of upper-extremity venous access in a pediatric cardiac catheterization laboratory., Methods: A retrospective chart review of all patients who underwent cardiac catheterization via upper-extremity vascular access was performed., Results: Eighty-two cardiac catheterizations were attempted via upper-extremity vein on 72 patients. Successful access was obtained in 75 catheterizations (91%) in 67 patients. Median age at catheterization was 18.79 years (interquartile range [IQR], 13.02-32.75 years; n = 75) with a median weight of 59.4 kg (IQR, 43.3-76.5 kg; n = 75). The youngest patient was 4.1 months old, weighing 4.3 kg. Local anesthesia or light sedation was utilized in 46 procedures (61%). Diagnostic right heart catheterization was the most common procedure (n = 65; 87%), with intervention performed via the upper extremity in 8 cases (11%). Median fluoroscopy time was 10.02 min (IQR, 2.87-36.26 min; n = 75), with dose area product/kg of 3.765 μGy•m²/kg (IQR, 0.74-34.12 μGy•m²/kg; n = 64). Median sheath duration time was 48 min (IQR, 19.5-147 min; n = 57) and median total procedure time was 116 min (IQR, 80.5-299 min; n = 65). Median length of stay for outpatient procedures was 5.37 hr (IQR, 4.25-6.92 hr; n = 27). There were no procedural complications., Conclusion: Upper-extremity venous access is a useful, feasible, and safe modality for cardiac catheterization in the pediatric cardiac catheterization laboratory.

Background: Patients with congenital heart disease require multiple procedures over their lifetime. These procedures increase cost and time commitment. Previous studies in the field of medicine have shown that combining procedures is an effective method to reduce cost and time. There has been no such study to evaluate the cost and efficiency of combining pediatric cardiac procedures., Objective: The objective of this study was to compare the cost and time commitment of combined cardiac catheterization (cath) and electrophysiology (EP) outpatient procedures against separate cath and EP procedure., Methods: Outpatient combination procedures performed in the pediatric cardiac cath lab from 2013 to 2016 were matched to a control population of two or three similar single outpatient procedures from 2009 to 2016 for patients of similar age and cardiac anatomy. Procedure duration, recovery duration, length of stay, equipment charges, physician charges, all other hospital charges, and total admission charges were analyzed. The two groups were compared using an unpaired t-test., Results: A total of 92 subjects, 32 study subjects and 60 control subjects, were included in this study. Study group procedures had a significantly shorter recovery duration (P = 0.04) and length of stay (P = 0.01). Study group procedure duration trended shorter on average but statistically insignificant (P = 0.20). The total median savings for patients undergoing combined procedures in the study group was $13,181 (interquartile range $423.8-$26710)., Conclusions: Combining cath and EP outpatient procedures reduces the time commitment and provides some economic advantage., (© 2018 Wiley Periodicals, Inc.)

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., (Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

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 < 0.0001). The median CI improved from 0.54 (0.43, 0.62) to 0.97 (0.89, 1.06) following SI (P < 0.0001). There were no procedural deaths but SAEs occurred in 12 (36%) patients. During a median follow-up duration of 29.7 months (6.8, 48.0), freedom from death or heart transplant was 82%, and from re-intervention was 45%, with median time to re-intervention of 20.1 months (11.4, 40.3)., Conclusions: SI for treatment of RC in patients after the Norwood operation provides excellent acute relief of obstruction. Intraprocedural hemodynamic instability is common and re-intervention is frequent at mid-term follow-up., (© 2017 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 <0.001); pulmonary insufficiency was trivial/none in all but 1 patient, where it was mild. Worsening of systemic RV dysfunction or tricuspid regurgitation was seen in 12 patients (57%) and was associated with a significantly lower post-TPVR LVOT peak systolic ejection gradient (median 17 versus 21 mm Hg; 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., (© 2017 American Heart Association, Inc.)

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.

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.

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.)

Persistent primitive hepatic venous plexus is an anomaly of the systemic venous return characterised by postnatal persistence of the foetal intrahepatic venous drainage. Scimitar syndrome is a condition that consists of partial anomalous pulmonary venous return of the right pulmonary venous drainage into the systemic veins, associated with pulmonary artery hypoplasia with the underdeveloped right lung, pulmonary sequestration, and cardiac malposition. Both conditions are rare and together have been rarely described in the literature. We report the first case of this combination of lesions imaged by cardiac magnetic resonance imaging with a three-dimensional reconstruction and reviewed the literature to characterise this uncommon combination.

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.

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., (© 2014 Wiley Periodicals, Inc.)

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., (© 2014 Wiley Periodicals, Inc.)

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., (Copyright © 2012 Wiley Periodicals, Inc., a Wiley company.)

The risk factors for superior vena cava (SVC) obstruction after pediatric orthotopic heart transplantation (OHT) have not been identified. This study tested the hypothesis that pretransplant superior cavopulmonary anastomosis (CPA) predisposes patients to SVC obstruction. A retrospective review of the Pediatric Cardiac Care Consortium registry from 1982 through 2007 was performed. Previous CPA, other cardiac surgeries, gender, age at transplantation, and weight at transplantation were assessed for the risk of developing SVC obstruction. Death, subsequent OHT, or reoperation involving the SVC were treated as competing risks. Of the 894 pediatric OHT patients identified, 3.1% (n = 28) developed SVC obstruction during median follow-up of 1.0 year (range: 0 to 19.5 years). Among patients who developed SVC obstruction, 32% (n = 9) had pretransplant CPA. SVC surgery before OHT was associated with posttransplant development of SVC obstruction (p <0.001) after adjustment for gender, age, and weight at OHT and year of OHT. Patients with previous CPA had increased risk for SVC obstruction compared with patients with no history of previous cardiac surgery (hazard ratio 10.6, 95% confidence interval: 3.5 to 31.7) and to patients with history of non-CPA cardiac surgery (hazard ratio 4.7, 95% confidence interval: 1.8 to 12.5). In conclusion, previous CPA is a significant risk factor for the development of post-heart transplant SVC obstruction., (Published by Elsevier 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., (© 2013 John Wiley & Sons A/S.)