Applying Practice Analysis to Develop a New Test Content Outline for the Pediatric Cardiology Certification Examination.

The evolving breadth and complexity of the contemporary pediatric cardiology specialty requires regular, systematic analysis of the practice to ensure that training and certification requirements address the demands of real-world clinical experience. We report the process of the American Board of Pediatrics (ABP) for conducting such a practice analysis and revising the test content outline (TCO) for the pediatric cardiology subspecialty certification exam. A panel of 15 pediatric cardiologists conducted seven 2-h virtual meetings, during which they identified 37 unique tasks that represent the work a pediatric cardiologist may reasonably expect to perform within the first 5 years after training. These tasks were grouped into nine performance domains, similar to the entrustable professional activities (EPA), previously endorsed by the ABP in collaboration with the pediatric cardiology education community, and which represent the critical activities of the profession. The panel then enumerated the knowledge, skills, and abilities necessary to perform each task. These deliberations resulted in two work products: a practice analysis document (PAD) and subspecialty board TCO based on testable knowledge, skills, and abilities. Survey assessments of the panel's work were then distributed to pediatric cardiology fellowship program directors and to practicing pediatric cardiologists for their input, which largely aligned with the panel's recommendations. Survey responses were considered in the final revisions of the PAD and TCO. This approach to practice analysis proved to be an efficient process for describing the work performed by today's pediatric cardiologists and the knowledge, skills, and abilities needed to competently perform that work.

mRNA Coronavirus Disease 2019 Vaccine-Associated Myopericarditis in Adolescents: A Survey Study.

In this survey study of institutions across the US, marked variability in evaluation, treatment, and follow-up of adolescents 12 through 18 years of age with mRNA coronavirus disease 2019 (COVID-19) vaccine-associated myopericarditis was noted. Only one adolescent with life-threatening complications was reported, with no deaths at any of the participating institutions.

Covered Stents in the Management of Aortic Coarctation and Right Ventricular Outflow Tract Obstruction.

PURPOSE OF REVIEW: To review the use of covered stents in the treatment of coarctation of the aorta (CoA) and right ventricle to pulmonary artery (RV-PA) conduit obstruction. RECENT FINDINGS: The only commercially available covered stent approved for treatment of CoA and dysfunctional RV-PA conduits is the covered Cheatham-Platinum stent (CCPS). Early outcomes have demonstrated its safety and have suggested its efficacy in treating or preventing aortic wall injury (AWI) or conduit disruption. A recent study of CCPS use for CoA reported a progressive risk of stent fracture over time and a risk of AWI despite the purported protection that the CCPS provides. The use of other covered stents has been reported, but large, systematic studies are lacking. CCPS use may reduce but does not eliminate the risk of conduit disruption or AWI. Structural limitations of the CCPS may predispose it to stent fracture. Access to a broad range of covered stents continues to be an unmet need in the field of congenital interventional cardiology.

Radiation dose reduction for 3D angiography images in pediatric and congenital cardiology.

OBJECTIVES: The purpose of this study was to investigate the influence of simulated reduced-dose three-dimensional angiography (3DA) on the accuracy and precision of linear measurements derived from 3DA datasets. BACKGROUND: Three-dimensional angiography is performed during X-ray guided interventional procedures to aid diagnosis and inform treatment strategies for children and adults with congenital heart disease. However, 3DA contributes substantially to patient radiation dose and may lead to an increased radiation-induced cancer risk. METHODS: Reduced-dose patient 3DA images were simulated by adding quantum noise to the 2D projection angiograms, then reconstructing the projection angiograms into the 3DA dataset. Dose reduction in the range 33-72% was simulated. Five observers performed 46 vessel diameter measurements along prespecified axes within 23 vessel segments from 11 patient 3DA datasets. Statistical tests were performed to assess the influence of radiation dose reduction on the accuracy and precision of vessel diameter measurements. RESULTS: Vessel diameter measurements were in the range 5.9- 22.7 mm. Considering all vessel segments and observers, the influence of dose level on the accuracy of diameter measurements was in the range 0.02 - 0.15 mm (p .05-.8). Interobserver variability increased modestly with vessel diameter, but was not influence by dose level (p = .52). The statistical test for observer recall bias was negative (p = .51). CONCLUSIONS: Simulated dose reduction up to 72% did not affect the accuracy or precision of the diameter measurements acquired from 3DA images. These findings may embolden 3DA radiation dose reduction for pediatric and congenital heart disease patients.

Elevated ventricular filling pressures and long-term survival in adults post-Fontan.

OBJECTIVE: To assess the association between elevated ventricular-end diastolic pressures (VEDP) and pulmonary artery wedge pressure (PAWP) on long-term survival in adult Fontan patients. BACKGROUND: The impact of ventricular filling pressures on long-term survival in adults post-Fontan palliation is unknown. METHODS: We included 148 adult Fontan patients (age ≥ 18 years) without atrioventricular valve prosthesis or pulmonary vein stenosis undergoing arterial and venous catheterization between December 1999 and November 2017. VEDP was defined as ≥12 mmHg and PAWP as >12 mmHg based on optimal cut-offs for prediction of mortality on receiver-operator curves (AUC 0.63 and 0.66, respectively). RESULTS: Mean age was 31.3 ± 9.2 years and 48.6% of patients were females. Most common congenital defects were tricuspid atresia (36.4%) and double-inlet left ventricle (28.3%); 59.5% patients had atriopulmonary Fontan connections. Mean VEDP was 11.5 ± 4.7 mmHg and PAWP 10.6 ± 4.5 mmHg (correlation coefficient .76). During a follow-up of 6.0 ± 4.8 years (median 5.4, IQR 1.4-9.4), there were 45 deaths (30.4%). Overall survival was lower in patients with VEDP ≥ 12 compared to those with VEDP < 12 mmHg (p = .02). Similarly, survival was lower in patients with PAWP>12 compared to patients with PAWP ≤ 12 mmHg (p < .0001). In the multivariate model, PAWP was an independent predictor of death (HR 1.1 per mmHg, 95% CI 1.02-1.15, p = .009) whereas VEDP was not (HR 1.1 per mmHg, 95% CI 1.0-1.13; p = .08). CONCLUSION: PAWP but not VEDP was independently associated with long-term overall mortality in adult Fontan patients. Perhaps PAWP rather than VEDP should be used in the risk stratification of these patients.

Temporary balloon occlusion of atrial septal defects in suspected or documented left ventricular diastolic dysfunction: Hemodynamic and clinical findings.

OBJECTIVE: To review our experience with balloon testing prior to atrial septal defect (ASD) closure in adults with left ventricular (LV) diastolic dysfunction. BACKGROUND: ASD closure in patients with LV diastolic dysfunction may precipitate LV failure. Temporary ASD occlusion has been used in this scenario but data are limited. METHODS: Retrospective review of 27 patients age ≥ 50 years undergoing temporary ASD balloon occlusion between 2000 and 2018 for suspected LV diastolic dysfunction or elevated LV end-diastolic pressure (LVEDP). RESULTS: Median age was 72 years (IQR 66.7; 75.2). Atrial fibrillation was seen in 58% of patients, hypertension in 58%, and coronary artery disease in 26%; 52% were females. Median ASD size was 13 mm (10; 18) and Qp/Qs 1.8 (1.6; 2.2). Median LVEDP was 14 mmHg (12; 22); pulmonary artery wedge pressure (PAWP) 12 mmHg (9; 16.5) and left atrial pressure (LAP) 13.5 mmHg (8; 16.3). After a median of 5 min (3; 10) of balloon occlusion, patients with baseline LVEDP ≥15 mmHg had more significant increases in LVEDP (9 [6; 12] vs. 2 mmHg [0.5; 5]; p = 0.03) and LAP/PAWP (10.5 [8.3; 16.3] vs. 1.5 mmHg [-1.5; 3]; p = 0.0003) than those with baseline LVEDP <15 mmHg. None of those with a baseline LVEDP <15 mmHg had a LAP/PAWP >15 mmHg during balloon testing compared to 92% of patients with a baseline LVEDP ≥15 mmHg. CONCLUSION: LVEDP might be used to predict LAP post-ASD closure. Comorbidities typically associated with LV diastolic dysfunction are common in these patients and should be considered in their management.

Variation in Anticoagulation Practices in the Congenital Cardiac Catheterization Lab: Results of a Multinational PICES Survey.

The complex nature of congenital heart disease (CHD) has hindered the establishment of management standards for peri-catheterization anticoagulation. We sought to describe anticoagulation practice variability among providers performing cardiac catheterization in children and adults with CHD. A web-based survey ( http://www.surveymonkey.com ) was distributed to pediatric and congenital interventional cardiologists. Respondents were queried on their training, practice setting, years in practice, and case volume. Clinical questions focused on general anticoagulation strategies and on five common clinical scenarios: two diagnostic (biventricular circulation, single ventricle physiology) and three interventional cardiac catheterizations (atrial septal defect closure, pulmonary artery stenting in Fontan circulation, stent placement for coarctation of aorta). Seventy-seven pediatric and congenital interventional cardiologists responded to the survey (81% in the United States). Twenty-six (36%) worked in a public medical institution; 57% worked in a free-standing children's hospital. Twenty-six percent had been in practice for < 5 years and 32% for > 15 years; 75% completed additional training in interventional congenital cardiology. The median number of cases performed was 200/year (IQR 110); median number of interventional cases was 100/year (IQR 100). Responses to general queries and specific clinical scenarios suggested significant variation in anticoagulation practices, including monitoring of anticoagulation during catheterization, protamine use, and outpatient anticoagulation after catheterization. Practices not only varied between providers but also between different clinical scenarios. Practice patterns did not correlate with provider experience or case volume. Management of anticoagulation in the congenital cardiac catheterization lab varies from operator to operator. Our study may provide some initial insight and context for discussion regarding anticoagulation in a field of increasingly heterogeneous interventional techniques and patient substrates. Future studies would be helpful to better define "best practices" for peri-procedural thromboprophylaxis in patients with congenital heart disease.

Exercise Capacity After Repair of Ebstein Anomaly in Adults.

Repair of Ebstein anomaly has evolved over the last decade, and timing of repair remains variable. There have been no studies of exercise or functional capacity in patients who have had tricuspid valve surgery for Ebstein anomaly in adulthood. We aimed to compare exercise capacity before and after tricuspid valve repair or replacement for Ebstein anomaly in adults at Mayo Clinic. We performed a retrospective chart review of all patients with Ebstein anomaly who underwent tricuspid valve surgery at Mayo Clinic between June 2007 and January 2015. We compared pre- and postoperative echocardiograms, exercise tests, and clinic visits. Tricuspid valve surgery was done for 322 patients, and 32 patients met criteria of native tricuspid valve repair or replacement at age 18 or older and had maximal pre- and postoperative exercise tests. Nineteen patients had valve repair, and 13 had valve replacement. Surgery for Ebstein anomaly resulted in significant reduction in tricuspid regurgitation and right ventricular size. There was a significant improvement in NYHA functional class after surgery; however, there was no significant improvement in functional aerobic capacity (FAC), metabolic equivalents (METs), exercise time, or [Formula: see text] after surgery. Patients who had an atrial shunt closed during surgery had improved minimum blood oxygen saturations during exercise, though no improvement in exercise capacity. In our cohort, patients who had tricuspid valve repair or replacement for Ebstein anomaly reported an improvement in functional capacity; however, this did not reflect improvement in measured exercise capacity, despite excellent surgical results by echocardiography.

Mechanism for temporal changes in exercise capacity after Fontan palliation: Role of Doppler echocardiography.

BACKGROUND: The objective was to better understand Doppler hemodynamics and exercise capacity in patients with Fontan palliation by delineating the hemodynamic mechanism for temporal changes in their peak oxygen consumption (V̇o2). METHODS: We performed a retrospective review of adult Fontan patients with systemic left ventricle (LV) who underwent serial transthoracic echocardiograms (TTE) and cardiopulmonary exercise tests (CPET) at Mayo Clinic in 2000-2015. TTE and CPET data were used (1) to determine agreement between V̇o2 and Doppler-derived LV function indices (eg, stroke volume index [SVI] and cardiac index [CI]) and (2) to determine agreement between temporal changes in peak V̇o2 and LV function indices. RESULTS: Seventy-five patients (44 men; 59%) underwent 191 pairs of TTE and CPET. At baseline, mean age was 24±3 years, peak V̇o2 was 22.9±4.1 mL/kg/min (63±11 percent predicted), SVI was 43±15 mL/m2, and CI was 2.9±0.9 L/min/m2. Peak V̇o2 correlated with SVI (r=0.30, P<.001) and with CI (r=0.45, P<.001) in the 153 pairs of TTE and CPET in patients without cirrhosis. Temporal changes in percent predicted peak V̇o2 correlated with changes in SVI (r=0.48, P=.005) and CI (r=0.49, P=.004) among the 33 patients without interventions during the study. In the 19 patients with Fontan conversion, percent predicted peak V̇o2 and chronotropic index improved. CONCLUSIONS: Overall, there was a temporal decline in peak V̇o2 that correlated with decline in Doppler SVI. In the patients who had Fontan conversion operation, there was a temporal improvement in peak V̇o2 that correlated with improvement in chronotropic index.

Prevalence and outcome of thrombotic and embolic complications in adults after Fontan operation.

There are limited studies of thrombotic and embolic complications (TEC) in the adult Fontan population. The purpose of the study was to determine the prevalence, risk factors, and outcomes of TECs in this population. METHODS: Retrospective review of adults with a previous Fontan operation, with follow-up at Mayo Clinic, 1994-2014. Systemic TEC was defined as intracardiac thrombus, ischemic stroke, or systemic arterial embolus. Nonsystemic TEC was defined as Fontan conduit/right atrial thrombus or pulmonary embolus. RESULTS: We identified 387 patients with a mean (SD) age of 28 (7) years and a mean follow-up of 8 (2) years. An atriopulmonary connection (APC) was done for 286 patients (74%). Atrial arrhythmias were present in 278 (72%). There were 121 TECs (systemic n=36, nonsystemic n=85) in 98 patients (25%). Risk factors for systemic TEC were atrial arrhythmia (hazard ratio 2.28, P=.001) and APC (hazard ratio 1.98, P=.02); nonsystemic TEC also had similar risk factors. All 98 patients received warfarin. Warfarin was discontinued in 10 of 98 because of bleeding, and 8 of these 10 subsequently had a second TEC. Among the 82 patients who had follow-up imaging, 16 (20%) had resolution of thrombus. In total, 24 of 98 patients had a second TEC, most of whom had inadequate anticoagulation. CONCLUSIONS: Thrombotic and embolic complication was not uncommon; risk factors for TEC were APC and atrial arrhythmias. Most patients were treated successfully with warfarin alone. A second TEC occurred in most patients whose anticoagulation was discontinued because of bleeding events.

Novel delivery technique for atrial septal defect closure in young children utilizing the GORE® CARDIOFORM® septal occluder.

OBJECTIVES: To develop a novel delivery technique to overcome vascular size limitations for device closure of a secundum ASD in the young patient. BACKGROUND: The efficacy, clinical utility, and safety of transcatheter closure in comparison to surgical closure are well established; however, access vessel size remains a potential limitation to device selection in the young patient. METHODS: A retrospective medical record review of all cases of secundum ASD closure between April 2015 and April 2016 utilizing a novel delivery method described herein at two separate academic institutions. The device is disassembled allowing the delivery sheath to serve as the introducer. It is advanced to the RA with re-introduction of the device allowing for deployment in the standard fashion. RESULTS: Overall, 10 patients underwent secundum ASD closure via this novel delivery technique. There were six females (60%) and the average age at time of the procedure was 4.2 years ± 1.6 years (range 2-6 years). The majority of patients had an isolated secundum ASD (70%) with the primary indication for closure being right ventricular volume overload (90%). All patients had successful placement of a GORE® CARDIOFORM® Septal Occluder (GCSO) with no or trivial residual shunt. No patients had vascular complications related to the procedure. CONCLUSIONS: We describe a novel technique for ASD closure using the GCSO delivery sheath as the access sheath, which reduces the vascular access size requirement by 25%, thus addressing one common limitation for percutaneous device closure of a secundum ASD in young patients. © 2016 Wiley Periodicals, Inc.

Long-term follow-up after PFO device closure: Outcomes and Complications in a Single-center Experience.

OBJECTIVES: To describe medium- to long-term follow-up of patients undergoing device closure of patent foramen ovale (PFO) for stroke or transient ischemic attack (TIA). BACKGROUND: Transcatheter device closure of PFO continues to be used for secondary prevention of cryptogenic stroke or TIA, but data are lacking regarding long-term outcomes. METHODS: We reviewed the Mayo Clinic Rochester PFO database for patients who underwent device closure of PFO for secondary prevention of stroke/TIA from December 2001 to June 2012. Demographic, clinical, procedural, and follow-up data were reviewed. Recurrence rate of stroke/TIA were calculated. Data were analyzed to identify risk factors of recurrent neurologic events. RESULTS: Seven hundred and thirty patients (mean age 53 years; 40% female) were included in our study. Most patients (72%) had cryptogenic stroke as their qualifying event for PFO closure. There were no procedure-related deaths or strokes. The most commonly used device was the Amplatzer Septal Occluder (82%). Median follow-up was 6 years. A 3.9% of patients had documented residual right-to-left shunting at last echocardiogram. Recurrent stroke and/or TIA were reported in 45 patients, (6.3% of patients with follow-up data). Patients who experienced recurrent stroke/TIA were older and had a higher rate of diabetes and hypertension than those who did not. The presence of residual shunt did not correlate with risk of recurrent events. CONCLUSIONS: Device closure of PFO can be performed safely with very good long-term resolution of atrial shunting. Recurrent neurologic events after PFO closure may reflect additional comorbid risk factors unrelated to the potential for paradoxical embolism. © 2016 Wiley Periodicals, Inc.

Intravascular and hybrid intraoperative stent placement for baffle obstruction in transposition of the great arteries after atrial switch.

OBJECTIVES: To report our experience with intravascular and hybrid intra-operative stent placement for baffle obstruction in patients with complete transposition of the great arteries (TGA) after the atrial switch (Mustard/Senning) operation. BACKGROUND: Venous baffle obstruction is a challenging complication after atrial switch operation in patients with TGA. Traditional treatment options include intravascular stenting or surgery. METHODS: A retrospective analysis of Mayo Clinic's electronic medical record was completed to identify consecutive pediatric and adult patients with TGA after atrial switch who underwent baffle stent implantation from 1994 to 2015. RESULTS: Overall, 64 patients were referred for cardiac catheterization, in whom 47 (73%) were noted to have hemodynamic and angiographic evidence of baffle obstruction. A total of 20 patients mean age 33 (range: 8-46) years old underwent stent implantation of baffle stenosis at a mean of 33 (range: 7.5-45) years after initial atrial switch operation (Mustard, n = 19; Senning, n = 1). Overall, 27 baffles were stented in 20 patients via the following approaches: intravascular (17); hybrid surgical (3); staged intravascular & hybrid (2). Sites of stent placement were: superior vena cava (SVC) (13); inferior vena cava (IVC) (9); pulmonary venous baffle (5). Three patients had stent placement in the SVC and IVC baffles during the same procedure. Procedural adverse events occurred in 2/22 cases (9%) including creation of unintentional baffle leak (n = 1) and stent migration (n = 1). There was no procedure-related mortality. At follow-up (median 2, range 0.02-10 years), significantly improved NYHA class and mean Doppler baffle gradient were demonstrated (P < 0.05). Mild baffle re-stenosis (mean Doppler gradient; 2-3 mmHg) occurred in two patients who have not required re-intervention. Trivial baffle leak was noted in four patients. Baffle re-intervention was only occurred in one patient. One patient with pre-procedural Class IV symptoms died 3.5 months after stent implantation. CONCLUSIONS: This study is the largest reported experience of intravascular and intra-operative hybrid stent placement for patients with TGA after atrial switch. Transcatheter and minimally invasive hybrid intraoperative surgical stent placement are an effective strategy for relief of systemic and pulmonary venous baffle obstruction. © 2016 Wiley Periodicals, Inc.

Acute and midterm results following perventricular device closure of muscular ventricular septal defects: A multicenter PICES investigation.

OBJECTIVES: To describe acute and mid-term results of hybrid perventricular device closure of muscular ventricular septal defects (mVSDs). BACKGROUND: Perventricular device closure of mVSDs can mitigate technical limitations of percutaneous closure and need for cardiopulmonary bypass or ventriculotomy with a surgical approach. METHODS: This is a multicenter retrospective cohort study of patients undergoing hybrid perventricular mVSD device closure from 1/2004 to 1/2014. Procedural details, adverse events, outcomes, and follow-up data were collected. Patients were divided into two groups: (1) simple (mVSD closure alone) and (2) complex (mVSD closure with concomitant cardiac surgery). RESULTS: Forty-seven patients (60% female) underwent perventricular mVSD device closure at a median age of 5.2 months (IQR 1.8-8.9) and weight of 5.1 kg (IQR 4.0-6.9). Procedural success was 91% [100% (n = 22) simple and 84% (n = 21/25) complex]. Adverse events occurred in 19% (9/47) [9% (2/22) simple and 28% (7/25) complex]. Hospital length of stay (LOS) was shorter in the simple vs. complex group (4 vs. 14 days, P < 0.01). At mid-term follow-up of 19.2 months (IQR 2.3-43) 90% of pts had complete mVSD closure; none developed late heart block, increased atrioventricular (AV) valve insufficiency or ventricular dysfunction. CONCLUSIONS: Perventricular device closure of simple mVSD was associated with a high rate of procedural success, few adverse events, and short hospital LOS. Procedural adverse events were associated with the presence of concomitant complex surgery. Residual mVSD, AV valve insufficiency, or ventricular dysfunction were uncommon at mid-term follow-up. © 2017 Wiley Periodicals, Inc.

Exercise Capacity Before and After Stent Placement for Coarctation of the Aorta: A Single-Center Case Series.

Over the last 15 years, stent placement has become a viable and safe alternative to surgical repair for native and recurrent coarctation of the aorta. Although patients with coarctation of the aorta often have lower exercise tolerance, there is no published data regarding the effect of coarctation stenting on exercise capacity. We aimed to determine the effect of coarctation stent placement on exercise capacity. We conducted a retrospective chart review of all patients who underwent stent placement for native or recurrent coarctation of the aorta at our institution from January 2000 to October 2012. We identified those patients who underwent exercise testing before and after stent placement. We compared measures of exercise capacity pre- and post-stent placement using paired t tests. Among 48 patients who underwent coarctation stenting, 7 (mean age = 40, range 21-65 years) were found to have isolated coarctation of the aorta and had the necessary pre- and post-stent exercise data. Only one patient had native coarctation of the aorta. The peak-to-peak systolic gradient improved by a mean of 26 mmHg (p < 0.02) after stent placement. Functional aerobic capacity (FAC) improved from a mean of 69-83% after stent placement (p < 0.03). Metabolic equivalents (METs) also improved significantly from a mean of 8.7 to 10.2 (p < 0.03). In our cohort, stent placement for relief of recurrent coarctation of the aorta resulted in a significant improvement in exercise capacity. This improvement may not be as significant in patients with milder coarctation of the aorta or those with complicating morbidities. Larger studies with more long-term follow-up are needed to better quantify this improvement and to define any lasting effect of this intervention on exercise tolerance.

Transcatheter tricuspid valve-in-valve in patients with transvalvular device leads.

OBJECTIVE: To describe the effects of percutaneously implanted valve-in-valve in the tricuspid position for patients with pre-existing transvalvular device leads. METHODS: In this case series, we describe implantation of the Melody valve and SAPIEN XT valve within dysfunctional bioprosthetic tricuspid valves in three patients with transvalvular device leads. RESULTS: In all cases, the valve was successfully deployed and device lead function remained unchanged. In 1/3 cases with 6-month follow-up, device lead parameters remain unchanged and transcatheter valve-in-valve function remains satisfactory. CONCLUSIONS: Transcatheter tricuspid valve-in-valve is feasible in patients with pre-existing transvalvular devices leads. Further study is required to determine the long-term clinical implications of this treatment approach.

AngioJet™ thrombolysis of SVC thrombosis after orthotopic heart transplantation: A case report.

SVC obstruction is a rare acute complication after bicaval cardiac transplantation and can be catastrophic for the patient. Herein, we report the case of a child who developed SVC thrombosis causing acute SVC syndrome nine days after heart transplantation. Thrombolysis was successfully performed using the AngioJet™ system and subsequent balloon angioplasty with re-establishment of flow. This procedure allowed for revascularization without repeat sternotomy or stent implantation. The patient is well without recurrence at last follow-up.

Transseptal puncture to facilitate device closure of "long-tunnel" patent foramen ovale.

BACKGROUND: Patent foramen ovale (PFO) is common and may predispose to paradoxical embolism. Transcatheter device closure of PFO can be challenging in certain cases of "long-tunnel" PFO morphology. We report our experience with device closure of long-tunnel PFO using transseptal puncture. METHODS: We retrospectively reviewed all cases of PFO device closure using transseptal puncture at the Mayo Clinic from January 1, 2010 to September 30, 2013. We arbitrarily defined a PFO tunnel configuration as a tunnel length ≥ 12 mm as observed in intracardiac echocardiography (ICE) at the time of device closure. RESULTS: Twelve patients (mean age 40.8 [range 15-67] years; 7 males [58%]) underwent PFO device closure with transseptal puncture. The most common indication for PFO closure was previous stroke (n = 7, 58%). Median tunnel length measured by ICE was 15 mm (12-20 mm). GORE® HELEX® Septal Occluder was used for closure in all but one patient. The only significant procedural complication was a minor perforation of the left atrial wall during attempted septal puncture, which resulted in a trivial pericardial effusion that resolved without intervention. At latest follow-up (mean 543 days, [range 170-1162]) one patient had a residual shunt and subsequently died due to complications during surgical PFO closure. No recurrent strokes or TIAs were reported. CONCLUSION: Though not without risk, transseptal puncture can be a valuable tool for facilitating device closure of long-tunnel type PFOs. GORE® HELEX® Septal Occluder may be an effective option for facilitating device closure for patients with long-tunnel type PFO.

Transcatheter closure of postmyocardial infarction, iatrogenic, and postoperative ventricular septal defects: The Mayo Clinic experience.

OBJECTIVES: To determine event-free survival after transcatheter closure of ventricular septal defect (VSD), and to identify predictors of adverse events (AE) in post myocardial infarction VSD (post-MI VSD) subgroup. BACKGROUND: There are limited data on mid-term follow-up after transcatheter VSD closure. METHODS: Retrospective review of 27 cases of transcatheter VSD closure (post-MI = 18 and non-ischemic = 9) performed from 1999 to 2013. We defined AE as death, device embolization, hemolysis requiring blood transfusion, heart block and reintervention. RESULTS: In the post-MI VSD subgroup, mean age and follow-up was 69 ± 11 and 7.3 ± 7 years, respectively. AE occurred in 8 (44%) patients (death-3, device embolization-1, hemolysis-1, surgical VSD closure-2, reintervention-1). Event-free survival was 56% at 1 month and 5 years, and all AE occurred in the periprocedural period. Cardiogenic shock (HR: 3.21, CI: 1.82-5.41, P = 0.002), and VSD closure in acute phase (HR: 2.14, CI: 1.12-4.31, P = 0.004) were independent predictors of AE. In the non-ischemic VSD subgroup, mean age and follow-up was 49 ± 15 and 8.7 ± 8 years, respectively. AE occurred in 3 (33%) patients (late death-1, surgical VSD closure-2). For the entire cohort, freedom from death was 89% and 85% at 1 month and 5 years, and event-free survival was 70% and 61% at 1 month and 5 years. CONCLUSIONS: Transcatheter closure of post-MI VSD carries a moderate risk of periprocedural complications but low event rates afterwards. By comparison, device closure of non-ischemic VSD has lower periprocedural morbidity but some patients continued to experience AE during follow-up.

Radiation reduction in pediatric and adult congenital patients during cardiac catheterization.

OBJECTIVES: Our objective was to determine if technical changes combined with radiation safety initiatives reduced the radiation dose delivered to patients during congenital catheterization. BACKGROUND: Use of ionizing radiation is necessary during cardiac catheterization. Minimizing radiation dose, while maintaining clinically useful image quality, is an important safety issue. In our congenital heart center intentional practice changes, including technical changes and provider awareness initiatives, were implemented to decrease radiation dose. METHODS: Data were retrospectively collected for all procedures involving children and adults with congenital heart disease (CHD) undergoing catheterization over 45 months. Cases were divided into three categories including: noninterventional (NI), simple intervention (SI), and complex intervention (CI). The change in dose was modeled as log of cumulative air kerma (Ka,r ). The change in Ka,r was evaluated for each procedural category as well as changes occurring as a function of age and weight. RESULTS: Considering all procedures (n = 1,082), Ka,r decreased by 61%. In the NI group (n = 481), Ka,r decreased by 71%. In the SI group (n = 424), Ka,r decreased by 74%. The Ka,r for the 10-17 year old group (n = 125) and those ≥18 years (n = 709) decreased 74 and 67%, respectively. The Ka,r decreased 72 and 66% for those 20-60 kg and ≥60 kg, respectively. Groups not showing significant change in Ka,r included CI, age ≤9 years, and weight ≤20 kg. CONCLUSIONS: Through technical changes and provider awareness initiatives, our institution dramatically reduced the radiation dose in the majority of pediatric and adult CHD patients undergoing cardiac catheterization.