Rationale and Design of CHD PULSE: Congenital Heart Disease Project to Understand Lifelong Survivor Experience.

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 one intervention for CHD at one 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 3133 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 non-participants, 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 3133 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.

Bilateral Harmony™ valve placement in branch pulmonary arteries.

While newer self-expanding pulmonic valves were primarily designed for larger right ventricular outflow tracks, there are instances where even larger anatomies cannot accommodate these devices. In this report, we describe the successful implantation of two Harmony™ valves in bilateral branch pulmonary arteries after exhausting other options.

Transcatheter Patent Ductus Arteriosus Closure in Premature Infants: Comparison of Echocardiogram and Angiogram Measurements.

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.

Utility of simultaneous triple balloon inflation technique through a single 6-French sheath in treating pulmonary vein stenosis.

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.

Bedside repositioning of a migrated avalon ECMO cannula in an infant: Novel technique.

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.

Left atrial appendage occlusion using hydrogel coils in a child.

Left atrial appendage occlusion in young children has not been reported before. Herein, we describe a successful occlusion using hydrogel coils in a toddler. The decision to occlude the appendage was made to mitigate the potential risk of systemic thromboembolism, given the child's unusual anatomy.

Hybrid approach for harmony transcatheter pulmonary valve replacement.

The Harmony™ Transcatheter Pulmonary Valve (Medtronic) was recently approved by the Food and Drug Administration for transcatheter pulmonary valve replacement in native right ventricular outflow tracts. Despite this milestone, some patients have main pulmonary arteries that are severely dilated and continue to require surgical pulmonary valve replacement. The hybrid approach combines surgical creation of a landing zone, transcatheter valve deployment, and suture stabilization of the implanted valve. In this case series, we report the first use of a hybrid approach for Harmony™ transcatheter pulmonary valve replacement. Two cases are reported with varying approaches for surgical creation of a landing zone followed by successful placement of a Harmony™ valve.

Left ventricular function before and after percutaneous patent ductus arteriosus closure in preterm infants.

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.

Medium-Term Outcomes in Pediatric Patients Undergoing Cardiac Catheterization Early After Congenital Cardiac Surgery.

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.

A novel approach to place an adult-size stent to treat coarctation of the aorta using small introducers: "Nijres technique".

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.

Partially unroofed coronary sinus in an infant with pulmonary vein stenosis: mixed blessing.

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.

Gelatin sponge to close a tract after large-bore transhepatic access: tips for success.

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 Safety and Efficacy of Transcatheter Pulmonary Valve Replacement Combined with Electrophysiology Procedures.

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.

The road less travelled: percutaneous closure of a secundum atrial septal defect through the left internal jugular vein.

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 utilisation of lifetech multifunction™ occluder device for closure of perimembranous and muscular ventricular septal defects: first use in North America.

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.

Acute and medium term results of balloon expandable stent placement in the transverse arch-a multicenter pediatric interventional cardiology early career society study.

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.

Combined hybrid pulmonary valve placement and atrial septal defect closure: case report and literature review.

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.

Treatment of severe pulmonary insufficiency with bilateral branch pulmonary artery Melody valve implantation.

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.

Cost analysis of combining congenital cardiac catheterization and electrophysiology procedures in an outpatient setting.

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.