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Background: Isolated anterior mitral valve clefts (MVC) are rare congenital heart defects, and data are limited regarding the natural history and surgical outcomes for such isolated MVCs. Methods: We conducted a retrospective review of patients with congenital MVC who were evaluated at Mayo Clinic in Rochester, Minnesota between 1993 and 2020. Patients were separated into two cohorts: those who underwent surgical repair of the MVC and those who had not yet undergone repair. Baseline and postoperative clinical and echocardiographic data were analyzed. Results: Fourteen patients were included in the nonsurgical cohort and eight patients in the surgical cohort. Surgical repair was via primary median sternotomy (n = 6) or robot-assisted, minimally invasive (n = 2). All cleft repairs were performed by simple suture closure. Intraoperative evaluation of the clefts did not reveal additional structural factors that could account for the mitral regurgitation (MR). At latest follow-up of the surgical cohort, the median grade of MR was 1 (range 0-1), and median left ventricular ejection fraction was 65% (IQR 59%-67%), both similar to the immediate postoperative result. At latest follow-up, all patients in the nonsurgical cohort were NYHA Class 1, and median MR grade was 1. All patients were asymptomatic (NYHA Class 1). Conclusions: Our findings corroborate prior reports that MVC repair is safe and successful and is followed by a low rate of recurrent mitral valve dysfunction. Durable surgical repair of isolated, congenital MVC can be performed safely in select patients. The decision to intervene should be based on the severity of mitral regurgitation and patient symptoms rather than the presence of the MVC alone.

Patients with patent foramen ovale can manifest in a variety of ways. These presentations and their resolution are discussed in this article.

BACKGROUND: Transcatheter stent therapy is as effective as surgery in producing acute hemodynamic improvement in patients with coarctation of aorta (COA). However, left ventricular (LV) remodeling after transcatheter COA intervention has not been systematically investigated. The purpose of this retrospective cohort study was to compare remodeling of LV structure and function after transcatheter stent therapy vs surgical therapy for COA. METHODS: LV remodeling was assessed at 1, 3 and 5-years post-intervention using: LV mass index (LVMI), LV end-diastolic dimension, LV ejection fraction, LV global longitudinal strain (LVGLS), LV e’ and E/e’. RESULTS: There were 44 and 128 patients in the transcatheter and surgical groups respectively. Compared to the surgical group, the transcatheter group had less regression of LVMI (−4.6[95%CI −5.5 - −3.7] vs −7.3[95%CI −8.4 - −6.6] g/m(2), p

Background: Inferior vena cava (IVC) size and collapsibility provide a noninvasive estimate of right heart filling pressures, an important determinant of right heart hemodynamic performance that is not measured by cardiac magnetic resonance imaging (CMRI). We hypothesized that compared with CMRI risk model alone, a combined CMRI-IVC risk model will have better correlation with disease severity and peak oxygen consumption in patients with tetralogy of Fallot (TOF). Methods: We performed a retrospective review of patients with TOF with moderate/severe pulmonary regurgitation who underwent CMRI and echocardiography. A CMRI risk model was constructed using right ventricular (RV) end-diastolic volume index, RV end-systolic volume index, RV ejection fraction, and left ventricular ejection fraction. We added IVC hemodynamic classification to the CMRI indices to create CMRI-IVC risk model, and IVC hemodynamics were modeled as a categorical variable: normal vs mild/moderately abnormal (dilated IVC or reduced collapsibility) vs severely abnormal IVC hemodynamics (dilated IVC and reduced collapsibility). We defined disease severity as atrial arrhythmias, ventricular arrhythmias, and heart failure hospitalization. Results: Of 207 patients, 131 (63%), 72 (35%), and 4 (2%) had normal, mild/moderately abnormal, and severely abnormal IVC hemodynamics, respectively. Compared with the CMRI risk model, the CMRI-IVC risk model had a better correlation with disease severity (area under the curve, 0.62; 95% confidence interval, 0.51-0.74 vs area under the curve 0.84, 95% confidence interval, 0.78-0.91, P = 0.006) and peak oxygen consumption (r = 0.35, P = 0.042 vs r = 0.43, P = 0.031, Meng test P = 0.026). Conclusions: The combined CMRI-IVC risk model had a better correlation with disease severity compared with CMRI indices alone and can potentially improve risk stratification in the population with TOF. Résumé: Contexte: Le diamètre et la collapsibilité de la veine cave inférieure (VCI) permettent d’estimer de façon non invasive les pressions de remplissage du cœur droit, un déterminant important de la capacité hémodynamique cardiaque droite que ne mesure pas l’imagerie par résonance magnétique cardiaque (IRMC). Notre hypothèse était que, comparativement au modèle de risque IRMC seul, un modèle de risque combiné IRMC-VCI présenterait une meilleure corrélation avec la gravité de la maladie et la consommation maximale d’oxygène chez les patients atteints de la tétralogie de Fallot (TF). Méthodologie: Nous avons effectué une étude rétrospective de cas de TF avec régurgitation pulmonaire modérée ou sévère où les patients ont subi un examen d’IRMC et une échocardiographie. Nous avons créé un modèle de risque IRMC intégrant l’indice du volume télédiastolique ventriculaire droit, l’indice du volume télésystolique ventriculaire droit, la fraction d’éjection ventriculaire droite et la fraction d’éjection ventriculaire gauche. Nous avons ajouté une classification hémodynamique de la VCI aux indices d’IRMC pour créer le modèle de risque IRMC-VCI, et les caractéristiques hémodynamiques de la VCI ont été modélisées en tant que variable nominale : état normal vs anomalie légère ou modérée (VCI dilatée ou collapsibilité réduite) vs anomalie sévère des caractéristiques hémodynamiques de la VCI (VCI dilatée et collapsibilité réduite). Nous avons défini la gravité de la maladie en distinguant les arythmies auriculaires, les arythmies ventriculaires et l’insuffisance cardiaque entraînant une hospitalisation. Résultats: Au sein d’un groupe de 207 patients, les caractéristiques hémodynamiques de la VCI présentaient un état normal, une anomalie légère ou modérée et une anomalie sévère dans 131 cas (63 %), 72 cas (35 %) et 4 cas (2 %), respectivement. Comparativement au modèle de risque IRMC, le modèle de risque IRMC-VCI a présenté une meilleure corrélation avec la gravité de la maladie (aire sous la courbe = 0,62 et intervalle de confiance à 95 % = 0,51-0,74 vs aire sous la courbe = 0,84 et intervalle de confiance à 95 % = 0,78-0,91, p = 0,006) et avec la consommation maximale d’oxygène (r = 0,35, p = 0,042 vs r = 0,43, p = 0,031, p = 0,026 pour le test de Meng). Conclusions: Le modèle de risque combiné IRMC-VCI a présenté une meilleure corrélation avec la gravité de la maladie comparativement aux indices d’IRMC seuls. Il pourrait améliorer la stratification du risque au sein de la population atteinte de la TF.

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.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.

There are sparse data on outcomes after expanded polytetrafluoroethylene artificial neochordae (ePTFE-AN) for tricuspid valve (TV) repair. We evaluated outcomes after TV repair with ePTFE-AN in both pediatric and adult patients.We analyzed clinical data of 87 consecutive patients who underwent ePTFE-AN implantation at the time of TV repair from 1998 to 2020. Patients were categorized into pediatric and adult groups.There were 29 pediatric (33.3%) and 58 adult (66.7%) patients. The most common etiology of tricuspid regurgitation (TR) was congenital (pediatrics: 86.2% [25 of 29]; adults: 39.7% [23 of 59]). The median number of pairs of ePTFE-AN implanted was 2 (interquartile range [IQR], 2-5 pairs) for pediatric and 3 (IQR, 2-4 pairs) for adult patients. There was no early death. Three adult patients (5.2%) required early TV reoperation, and 4 patients (1 pediatric, 3 adults) underwent late TV reintervention. Etiology of TR was congenital in 4 of the 6 adults who required TV reintervention. The 3-year cumulative risk of TV reintervention was 0.0% for pediatric and 7.3% (95% CI, 0.4%-14.2%) for adult patients. There was significant improvement in TR grade after TV repair at dismissal and at the latest echocardiographic follow-up in each group (P.001). Severe TR developed in 1 pediatric patient and 7 adult patients during follow-up, and 6 (1 pediatric, 5 adults) of them underwent TV reoperation.ePTFE-AN implantation in the TV position can be performed safely and effectively with no early death. In adult patients with congenital TR, patient selection is critical to achieve a durable outcome after TV repair.

Patients with tetralogy of Fallot (TOF) have abnormal right ventricular (RV) afterload because of residual or recurrent outflow tract obstruction, often with abnormal pulmonary artery (PA) vascular function. The purpose of this study was to determine if RV afterload was independently associated with death and/or heart transplant in patients with TOF. This is a retrospective study of TOF patients that underwent cardiac catheterization for clinical indications at Mayo clinic between 1990 and 2015. Invasively measured RV systolic pressure (RVSP) was used to define RV afterload. To explore clinical utility for echocardiographic estimates of invasive data, correlations between invasive and Doppler-derived indices of RV afterload were examined. Among 266 patients with TOF (age 35 ± 14 years, TOF-pulmonary atresia 117 [44%]), RVSP was 72 ± 28 mm Hg, PA systolic pressure 45 ± 19 mm Hg, mean PA pressure 27 ± 10 mm Hg, pulmonary vascular resistance 4.2 ± 3.1 WU, and PA wedge pressure 14 ± 5 mm Hg. Over a mean follow up of 12.9 years, there were 35 deaths and 4 heart transplants. Invasively measured RVSP (hazard ratio 1.25, 95% confidence interval 1.12 to 1.37; p0.001) and TOF-pulmonary atresia (hazard ratio 1.18, 95% confidence interval 1.08 to 1.41; p = 0.023) were independent risk factors for death and/or transplant. Doppler-derived RVSP was well-correlated with invasive RVSP (r = 0.92, p0.001), and was also independently associated with the combined end point. RVSP, a composite measure of RV afterload, is independently prognostic in patients with TOF, and can be reliably assessed using Doppler echocardiography. Further study is required to test whether interventions to reduce RVSP can improve outcomes in patients with TOF.

To review our experience with balloon testing prior to atrial septal defect (ASD) closure in adults with left ventricular (LV) diastolic dysfunction.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.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).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 LVEDP15 mmHg. None of those with a baseline LVEDP15 mmHg had a LAP/PAWP15 mmHg during balloon testing compared to 92% of patients with a baseline LVEDP ≥15 mmHg.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.

Hypoplastic left heart syndrome (HLHS) with intact atrial septum (HLHS-IAS) carries a high risk of mortality and affects about 6% of all patients with HLHS. Fetal interventions, postnatal transcatheter interventions, and postnatal surgical resection have all been used, but the mortality risk continues to be high in this subgroup of patients. We describe a novel, sequential approach to manage HLHS-IAS and progressive fetal hydrops. A 28-year-old, gravida 4 para 2 mother was referred to Mayo Clinic for fetal HLHS. Fetal echocardiography at 28 weeks of gestation demonstrated HLHS-IAS with progressive fetal hydrops. The atrial septum was thick and muscular with no interatrial communication. Ultrasound-guided fetal atrial septostomy was performed with successful creation of a small atrial communication. However, fetal echocardiogram at 33 weeks of gestation showed recurrence of a pleural effusion and restriction of the atrial septum. We proceeded with an Ex uteroIntrapartum Treatment (EXIT) delivery and open atrial septectomy. This was performed successfully, and the infant was stabilized in the intensive care unit. The infant required venoarterial extracorporeal membrane oxygenator support on day of life 1. The patient later developed hemorrhagic complications, leading to his demise on day of life 9. This is the first reported case of an EXIT procedure and open atrial septectomy performed without cardiopulmonary bypass for an open-heart operation and provides a promising alternative strategy for the management of HLHS-IAS in select cases.

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.

Objective: To identify risk factors for pediatric mechanical mitral valve replacement (mMVR) to improve management in this challenging population. Methods: From 1993 to 2019, 93 children underwent 119 mMVR operations (median age, 8.8 years [interquartile range [IQR]: 2.1-13.3], 54.6% females) at our institution. Twenty-six (21.8%) patients underwent mMVR at ≤2 years and 93 (78.2%) patients underwent mMVR at >2 years. Median follow-up duration was 7.6 years [IQR: 3.2-12.4]. Results: Early mortality was 9.7%, but decreased with time and was 0% in the most recent era (13.9% from 1993 to 2000, 7.3% from 2001 to 2010, 0% from 2011 to 2019, P = .04). It was higher in patients ≤2 years compared to patients >2 years (26.9% vs 2.2%, P < .01). On multivariable analysis for mitral valve reoperation, valve size 2 years and those with a prosthesis ≥23 mm. Median time to reoperation was 7 years (IQR: 4.5-9.1) in patients >2 years and 3.5 years (IQR: 0.6-7.1) in patients ≤2 years ( P = .0511), but was similar between prosthesis sizes ( P = .6). During follow-up period (median 7.6 years [IQR: 3.2-12.4], stroke occurred in 10%, prosthetic valve thrombosis requiring reoperation in 4%, endocarditis in 3%, and bleeding in 1%. Conclusion: Early and late outcomes of mMVR in children are improved when performed at age >2 years and with prosthesis size ≥23 mm. These factors should be considered in the timing of mMVR.

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.

1. Simrit K. Warring, MS* 2. Richmond Castillo, MS* 3. Kelsey Klaas, MD† 4. Nathaniel Taggart, MD‡ 5. Philip R. Fischer, MD† 1. *Mayo Clinic School of Medicine, 2. †Department of General Pediatric and Adolescent Medicine, and 3. ‡Department of Pediatric Cardiology, Mayo Clinic, Rochester, MN A 14-month-old boy is referred to our clinic after his parents noted that he had stretchy skin and looked thinner than his siblings did at his age. The patient was born to a 32-year-old gravida 3 para 3 woman in Saudi Arabia. The pregnancy was complicated by maternal hypertension and gestational diabetes. He was born by cesarean delivery at term gestation due to maternal hypertension. There was no premature onset of labor, bleeding, or leakage of amniotic fluid. The mother reports that he seemed fine in the newborn nursery. He had no difficulty breathing; however, he had asymptomatic bilateral inguinal hernias and a minor diaphragmatic hernia, all of which were repaired when he was 2 weeks of age without complications. At the time of his surgery, a heart murmur was found, and echocardiography revealed that he had a dilated ascending aorta. There were no vision or hearing concerns. He did not have any feeding or developmental problems. The patient’s family history reveals that the patient’s maternal grandfather and father are first cousins. The patient has 2 first cousins who had surgical correction of vascular abnormalities. A maternal grandmother had multiple valve operations as an adult. There is no history of aortic dissection or rupture. On physical examination his height is 31.6 in (80.2 cm) (81st percentile) and weight is 22.7 lb (10.3 kg) (57th percentile). The patient’s blood pressure is 125/35 mm Hg, heart rate is 115 beats/min, and temperature is 97.9°F (36.6°C). The patient is in no apparent distress and is breathing comfortably. A normal precordial impulse is present, with normal S1 …

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 (V o 2). 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 V o 2 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 V o 2 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 V o 2 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 V o 2 correlated with SVI (r = 0.30, P o 2 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 V o 2 and chronotropic index improved. Conclusions Overall, there was a temporal decline in peak V o 2 that correlated with decline in Doppler SVI. In the patients who had Fontan conversion operation, there was a temporal improvement in peak V o 2 that correlated with improvement in chronotropic index.

Background: Intracardiac echocardiography (ICE) has been used during transcatheter closure of simple atrial level shunts, but its use in more complex shunt lesions has not been well described.Metho...

In pigs, the deep location of the common carotid artery and overlying sternomastoideus muscle in the neck has led to the recommendation for a surgical cutdown for common carotid access, as opposed to minimally invasive techniques for vascular access. We sought to determine if direct percutaneous common carotid artery access in piglets is attainable. Seventeen piglets were anesthetized and intubated. Under two-dimensional and color flow Doppler ultrasound guidance, a 21 gauge needle was utilized to access the right common carotid artery. Following arterial puncture, the Seldinger technique was applied to place a 4 or 5 French introducer. Upon completion of cardiac catheterization with intracoronary stem cell infusion the introducer was removed and manual pressure was applied to prevent hematoma development. Successful access with an introducer was achieved in all 17 piglets. The average weight was 8.5 ± 1.7 kg. One piglet developed a hematoma with hemorrhaging from the catheterization site and was euthanized. This piglet was given bivalirudin for the procedure. After this incident, subsequent piglets were not given anticoagulation and no other complications occurred. Ultrasound guided percutaneous common carotid artery access in piglets is attainable in a safe, reliable, and reproducible manner when performed by microvascular experts.

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

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

Background 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.

Background Premature coronary artery disease ( CAD ) is common in patients with coarctation of aorta ( COA ), but there are limited data about any direct relationship (or lack thereof) between COA and CAD . We hypothesized that atherosclerotic cardiovascular disease risk factors, rather than COA diagnosis, was the primary determinant of CAD occurrence in patients with COA . Methods and Results This is a retrospective study of 654 COA patients and a control group of 876 patients with valvular pulmonic stenosis and tetralogy of Fallot to determine prevalence and independent risk factors for CAD . There was no evidence of a difference in the unadjusted CAD prevalence between the COA and control groups (7.8% versus 6.3%, P =0.247), but premature CAD was more common in COA patients (4.4% versus 1.8%, P =0.002). In the analysis of a propensity‐matched cohort of 126 COA and 126 control patients, there was no evidence of a difference in overall CAD prevalence (6.3% versus 5.6% versus P =0.742) and premature CAD prevalence (4.8% versus 3.2%, P =0.518). The multivariable risk factors for CAD were hypertension (odds ratio [ OR ] 2.14; 95% CI 1.36–3.38), hyperlipidemia ( OR 3.33; 95% CI 2.02–5.47), diabetes mellitus ( OR 1.98; 95% CI 1.31–3.61), male sex ( OR 2.05; 95% CI 1.33–3.17), and older age per year ( OR 1.06; 95% CI 1.04–1.07). Conclusions After adjusting for atherosclerotic cardiovascular disease risk factors, we did not find evidence of a difference in CAD risk between the patients with COA and other patients with congenital heart disease.

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.The impact of ventricular filling pressures on long-term survival in adults post-Fontan palliation is unknown.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 as12 mmHg based on optimal cut-offs for prediction of mortality on receiver-operator curves (AUC 0.63 and 0.66, respectively).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 PAWP12 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).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.

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.

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

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.

Objectives This study aimed to describe the safety and short-term efficacy of the Covered Cheatham-Platinum stent (CCPS) in treating or preventing aortic wall injury (AWI) in patients with coarctation of the aorta (CoA). Background The COAST II trial (Covered Cheatham-Platinum Stents for Prevention or Treatment of Aortic Wall Injury Associated with Coarctation of the Aorta Trial) is a multicenter, single-arm trial using the CCPS for the treatment and/or prevention of AWI in patients with CoA and pre-existing AWI or increased risk of AWI. Methods Patients were enrolled if they had a history of CoA with pre-existing AWI (Treatment group) or with increased risk of AWI (Prevention group). Pre/post-implant hemodynamics and angiography were reported. A core laboratory performed standardized review of all angiograms. One-month follow-up was reported. Results A total of 158 patients (male = 65%; median age 19 years) underwent placement of CCPS. Eighty-three patients had pre-existing AWI. The average ascending-to-descending aorta systolic gradient improved from 27 ± 20 mm Hg to 4 ± 6 mm Hg. Complete coverage of pre-existing AWI was achieved in 66 of 71 patients (93%) with AWI who received a single CCPS. Ultimately, complete coverage of AWI was achieved in 76 of 83 patients (92%); 7 patients had minor endoleaks that did not require repeat intervention. Four patients experienced important access site vascular injury. There were no acute AWI, repeat interventions, or deaths. Conclusions The CCPS can effectively treat and potentially prevent AWI associated with CoA. Access site arterial injury is the most common important complication. Longer-term follow-up is necessary to define mid- and late-term outcomes.

Graphical abstract, Highlights • Large isolated ventricular septal defect resulting from blunt trauma can be associated with high morbidity and mortality. • High index of suspicion and rapid diagnosis with echocardiography are needed for proper management of isolated ventricular septal defect following blunt trauma. • Surgical patch repair or percutaneous VSD closure, when appropriate, are the mainstay of interventions to avoid late morbidity and mortality associated with large unrepaired VSD.

The increased workload on single right ventricle (sRV) leads to increased risk of Fontan failure due to ventricular dysfunction. Conventional heart failure therapy has mixed results. This has led to increased interest in seeking alternative therapeutic strategies to improve ventricular function

Background Stenosis of the venous connections and conduits is a well‐known late complication of the Fontan procedure. Currently, data on the outcomes of percutaneous intervention for the treatment of extra‐ or intracardiac conduits and lateral tunnel baffles obstruction are limited. In an attempt to better define the nature and severity of the stenosis and the results of catheter interventional management, we reviewed Fontan patients with obstructed extra‐ or intracardiac conduits and lateral tunnel baffles. Methods Retrospective review of all Fontan patients who had cardiac catheterization from January 2002 to October 2018 was performed. Hemodynamic and angiographic data that assessed extra‐ or intracardiac conduit, or lateral tunnel baffle obstruction/stenosis were evaluated. Results Twenty patients underwent catheter intervention because of conduit stenosis, including calcified homografts, stenotic Gore‐Tex conduits and obstructed lateral tunnels. Six other patients had Fontan obstruction but were referred for surgical revision. After stenting, there was a significant reduction in the connection gradient [2.0 mm Hg (IQR 2; 3) vs 0 mm Hg (IQR 0; 1), P

The purpose of this study was to define the risk for adults with congenital heart disease who underwent cardiac catheterization and to propose a precatheterization risk scoring system. Data were prospectively collected using a multicenter registry of the Congenital Cardiovascular Interventional Study Consortium. The occurrence of serious adverse events (SAE) was correlated with 12 predefined variables. Catheterization RISk in Adult patients (CRISA) score was derived using multivariate logistic regression with backward elimination model selection method. The CRISA score was compared with the American Society of Anesthesiology score and a consensus-derived, 20-point risk score based on their ability to predict SAE. From June 2008 to September 2017, 300 adjudicated SAE's occurred in 7317 catheterization procedures (overall SAE rate 4.1%) performed in adults over 18 years of age at 27 contributing centers. Nine of the 12 tested variables were ultimately included in the CRISA score. CRISA score positively correlated with risk of SAE, and was superior to American Society of Anesthesiology and the 20-point risk score in predicting SAE. Minimal (CRISA score 0 to 2), low (3 to 7), moderate (8 to 10) and high (≥11) risk categories were identified, corresponding to 0.5%, 3.2%, 7.9%, and 16.7% risk of SAE, respectfully. In conclusion, the CRISA score reliably predicts risk of SAE in adults with congenital heart disease who underwent cardiac catheterization and may be useful for preprocedural risk assessment.

Objectives This study sought to determine whether pulmonary artery intervention in patients with unilateral proximal pulmonary artery stenosis (PAS) improves exercise capacity, abnormal ventilatory response to exercise, and symptoms. Background Stenosis of the branch pulmonary arteries results in pulmonary blood flow maldistribution (PBFM). The resulting ventilation–perfusion mismatch is associated with an increased ventilatory response to exercise and decreased exercise capacity. It is unclear if technical success in relieving branch PAS translates to clinical improvement in exercise capacity and ventilatory response. Methods Twenty patients with biventricular circulation and a minimum 10% PBFM who underwent transcatheter relief of PAS were enrolled in a multi-institutional prospective cohort study. Pre- and post-procedure assessment of the degree of PBFM, exercise capacity, ventilatory response to exercise, and subjective assessment of breathlessness were collected and analyzed. Results Technical success was achieved in all patients with significant angiographic improvement in minimal lumen diameter (p = 0.001) and peak gradient (p = 0.001). Median PBFM improved (19.5% [range 12.0% to 31.0%] before vs. 7.0% [range 0% to 33.0%] after; p = 0.003). Exercise capacity was low at baseline and improved significantly post-intervention; percent predicted peak oxygen consumption improved from 70% (range 45% to 96%) to 83% (range 47% to 121%) (p = 0.02). Percent predicted oxygen pulse improved (p = 0.02). Ventilatory response to exercise improved; ventilatory equivalent of carbon dioxide slope post-intervention decreased to 29.3 versus 32.5 pre-intervention (p = 0.01). Subjective assessment of dyspnea improved. Five patients with minimal improvement in PBFM also showed minimal improvement in exercise parameters. Conclusions Successful relief of unilateral branch PAS results in significant improvements in exercise capacity, ventilatory efficiency, and symptoms.

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 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.

Case Report: An otherwise healthy 14-month-old presented for a second opinion related to “stretchy skin” and concerns for a vascular abnormality. The patient had previously undergone hernia repair without complication. The patient’s maternal grandfather and father were first cousins. Two of the patient’s cousins had surgery for an unspecified vascular abnormality. A maternal grandmother had cardiac valve surgery later in life. Physical exam revealed mild blepharophimosis, periorbital fullness, down-slanting palpebral fissures, mild malar hypoplasia, and a somewhat beaked nose. He had hyperextensibility and excessive wrinkling of the hands, and slight redundancy of the skin on his face and lower sacrum. A …

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. © 2015 Wiley Periodicals, Inc.

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. © 2015 Wiley Periodicals, Inc.

Background/objectives Although echocardiographic-Doppler cardiac index (CI) assessment is widely used to guide heart failure management in patients with biventricular circulation, this application has not been studied in the Fontan population. The objective of this study was to: (1) determine the correlation between Doppler and cardiac catheterization CI calculation; (2) determine the association between Doppler CI and the occurrence of Fontan failure. Methods Retrospective review of adult Fontan patients followed at Mayo Clinic Adult Congenital Heart Disease program, 1994–2015. Inclusion criteria were: systemic left ventricle and echocardiogram and cardiac catheterization performed within the same week. Fontan failure was defined as a composite of all-cause mortality, heart transplantation listing, and palliative care. Results 59 patients (age 29±6years; men 32[54%]) underwent 97 studies. Of the 59, 41[69%] had atriopulmonary Fontan and 12 (20%) had cirrhosis. Compared to patients without cirrhosis, patients with cirrhosis had higher Doppler CI (3.6±0.6 vs 2.8±0.4L/min∗m 2 , p =0.039); Fick CI (3.3 [2.5–3.7] vs 2.4 [1.6–3.1] L/min/m 2 , p =0.028); lower systemic vascular resistance (20±3 vs 25±4 WU∗m 2 , p =0.04). There was a positive correlation between Doppler and Fick CI ( r =0.52; p 2 were associated with Fontan failure (odds ratio [OR] 1.58, p =0.046) and (OR 1.43, p =0.051) respectively. Conclusions Doppler CI assessment in feasible in a selected group of Fontan patients and it is predictive of clinical outcomes. The application of this concept in systemic right ventricles deserves further research.