Dronedarone for the Treatment of Atrial Arrhythmias in Adults With Congenital Heart Disease.

BACKGROUND: Atrial tachyarrhythmias are common and difficult to treat in adults with congenital heart disease. Dronedarone has proven effective in patients without congenital heart disease, but data are limited about its use in adults with congenital heart disease of moderate to great complexity. METHODS: A single-center, retrospective chart review of 21 adults with congenital heart disease of moderate to great complexity who were treated with dronedarone for atrial tachyarrhythmias was performed. RESULTS: The median (IQR) age at dronedarone initiation was 35 (27.5-39) years. Eleven patients (52%) were male. Ten patients (48%) had New York Heart Association class I disease, 10 (48%) had class II disease, and 1 (5%) had class III disease. Ejection fraction at initiation was greater than 55% in 11 patients (52%), 35% to 55% in 9 patients (43%), and less than 35% in 1 patient (5%). Prior treatments included ß-blockers (71%), sotalol (38%), amiodarone (24%), digoxin (24%), and catheter ablation (38%). Rhythm control was complete in 5 patients (24%), partial in 6 (29%), and inadequate in 10 (48%). Two patients (10%) experienced adverse events, including nausea in 1 (5%) and cardiac arrest in 1 (5%), which occurred 48 months after initiation of treatment. There were no deaths during the follow-up period. The median (IQR) follow-up time for patients with complete or partial rhythm control was 20 (1-54) months. CONCLUSION: Dronedarone can be effective for adult patients with congenital heart disease and atrial arrhythmias for whom more established therapies have failed, and with close monitoring it can be safely tolerated.

A Novel Approach to Diagnostic Left and Right Heart Catheterization in a Patient With a Mechanical Prosthetic Tricuspid Valve.

A 47-year-old patient was experiencing dyspnea and fatigue concerning for right ventricular hypertension and new heart failure. Because of the risks associated with catheter entrapment, prosthetic valve leaflet damage, and valve thrombosis associated with crossing a mechanical valve, a novel technique was used for diagnostic left and right heart catheterization in a patient with mechanical tricuspid valve replacement and tortuous pulmonary arteries. Using a percutaneous subxiphoid approach to avoid traversing the mechanical valve without discontinuing anticoagulation, a Volcano fractional flow reserve pressure wire (Philips Volcano) was advanced for distal measurements of pressures and saturations.

Imaging and guiding intervention for tricuspid valve disorders using 3-dimensional transesophageal echocardiography in pediatric and congenital heart disease.

In the pediatric and congenital heart disease (CHD) population, tricuspid valve (TV) disorders are complex due to the variable TV morphology, its sophisticated interaction with the right ventricle as well as associated congenital and acquired lesions. While surgery is the standard of care for TV dysfunction in this patient population, transcatheter treatment for bioprosthetic TV dysfunction has been performed successfully. Detailed and accurate anatomic assessment of the abnormal TV is essential in the preoperative/preprocedural planning. Three-dimensional transthoracic and 3D transesophageal echocardiography (3DTEE) provides added value to 2-dimensional imaging in the characterization of the TV to guide therapy and 3DTEE serves as an excellent tool for intraoperative assessment and procedural guidance of transcatheter treatment. Notwithstanding advances in imaging and therapy, the timing and indication for intervention for TV disorders in this population are not well defined. In this manuscript, we aim to review the available literature, provide our institutional experience with 3DTEE, and briefly discuss the perceived challenges and future directions in the assessment, surgical planning, and procedural guidance of (1) congenital TV malformations, (2) acquired TV dysfunction from transvenous pacing leads, or following cardiac surgeries, and (3) bioprosthetic TV dysfunction.

Continuous-Flow Ventricular Assist Device Support in Adult Congenital Heart Disease: A 15-Year, Multicenter Experience of Temporary and Durable Support.

Heart failure (HF) is common in adult congenital heart disease (ACHD) patients; however, use of continuous-flow ventricular assist devices (CF-VADs) remains rare. We reviewed outcomes of patients with congenital heart disease greater than or equal to 18 years of age at the time of CF-VAD implant at the affiliated pediatric and adult institutions between 2006 and 2020. In total, 18 ACHD patients (15 with great anatomical complexity) received 21 CF-VADs. Six patients (median age 34 years) received seven percutaneous CF-VADs with a median duration of support of 20 days (3-44 days) with all patients survived to hospital discharge and two patients were bridged to durable CF-VADs. Fourteen patients (median age 38 years) received durable CF-VADs. Thirteen patients (93%) survived to hospital discharge and the median duration of support was 25.8 months (6.4-52.1 months). Estimated survival on durable CF-VAD at 1, 3, and 5 years was 84%, 72%, and 36%, respectively. Three patients were successfully bridged to transplantation. Device-related complications include cerebrovascular accident (n = 5), driveline infection (n = 3), device infection requiring chronic antibiotic therapy (n = 4), gastrointestinal bleeding (n = 6), and presumed pump thrombosis (n = 5). These results show percutaneous and durable CF-VADs can support ACHD patients with advanced HF.

A Multisite Retrospective Review of Direct Oral Anticoagulants Compared to Warfarin in Adult Fontan Patients.

PURPOSE: Direct oral anticoagulants (DOACs) are not recommended in adult Fontan patients (Level of Evidence C). We hypothesized that DOACs are comparable to warfarin and do not increase thrombotic and embolic complications (TEs) or clinically significant bleeds. METHODS: We reviewed the medical records of adult Fontan patients on DOACs or warfarin at three major medical centers. We identified 130 patients: 48 on DOACs and 107 on warfarin. In total, they were treated for 810 months on DOACs and 5637 months on warfarin. RESULTS: The incidence of TEs in patients on DOACs compared to those on warfarin was not increased in a statistically significant way (hazard ratio [HR] 1.7 and p value 0.431). Similarly, the incidence of nonmajor and major bleeds in patients on DOACs compared to those on warfarin was also not increased in a statistically significant way (HR for nonmajor bleeds in DOAC patients was 2.8 with a p value of 0.167 and the HR for major bleeds was 2.0 with a p value 0.267). In multivariate analysis, congestive heart failure (CHF) was a risk factor for TEs across both groups (odds ratio [OR] = 4.8, 95% confidence interval [CI] = 1.3-17.6) and bleed history was a risk factor for clinically significant bleeds (OR = 6.8, 95% CI = 2.7-17.2). CONCLUSION: In this small, retrospective multicenter study, the use of DOACs did not increase the risk of TEs or clinically significant bleeds compared to warfarin in a statistically significant way.

Transcatheter pulmonic valve implantation in adult patients with prior congenital heart surgery.

BACKGROUND: Transcatheter pulmonary valve replacement (TPVR) is now an established modality for pulmonary valve replacement in suitable candidates. We aim to describe our experience with TPVR in adults. METHODS: This is a descriptive study of all TPVR performed in adults with congenital heart disease at a single institution from 2010-2020. All adult patients (defined as 18 years old or older at TPVR) were included. Time-to-event outcomes were described using Kaplan-Meier estimates with 95% confidence intervals (CIs). RESULTS: Out of a total of 200 patients that had undergone TPVR, 81 patients (57% male) met the inclusion criteria, with a median age and weight of 26 years (IQR 21-37) and 71.0 kg (IQR 54.6-89.0), respectively. In the cohort, 45 (56%) patients had tetralogy of Fallot. While 53 (65%) patients received a Melody valve, a Sapien valve (S3 in 20, XT in eight) was implanted in the rest. Pre-stenting was performed in 49 (52%) patients. One patient died of severe heart failure a year following TPVR. One patient had a second TPVR performed 2.2 years following initial TPVR for severe pulmonary regurgitation. Valve survival at 2.2 years was 94% (95% CI: 87-100%). Four patients developed endocarditis. Endocarditis-free survival was 89% (95% CI: 80-100%) at three years. CONCLUSIONS: Our experience suggests favorable results of TPVR in adults with congenital heart disease. Additional research would be warranted with a focus on total valve longevity and patient reported outcomes, in order to improve the understanding of TPVR in this population and further refine this technology.

Platypnea-Orthodeoxia Syndrome Caused by an Intracardiac Shunt.

Platypnea-orthodeoxia syndrome, a rare condition characterized by posture-related dyspnea, is usually caused by an intracardiac shunt, hepatopulmonary syndrome, or shunting resulting from severe pulmonary disease. We report the case of a 33-year-old woman who presented with increasing dyspnea and oxygen desaturation when she sat up or arose. Our diagnosis was platypnea-orthodeoxia syndrome. A lead of a previously implanted pacemaker exacerbated a severe tricuspid regurgitant jet that was directed toward the patient's intra-atrial septum. Percutaneous closure of a small secundum atrial septal defect eliminated right-to-left shunting and substantially improved the patient's functional status. In addition to this case, we discuss this unusual condition.

Guidewire pacing during transcatheter aortic valve implantation in a patient with complex congenital heart disease.

We present a case of temporary guidewire pacing in a patient with Fontan anatomy during transcatheter aortic valve implantation. Temporary pacing was successfully achieved utilizing this method without complications. There is an increasing population of patients with complex congenital heart disease and expanding variety of transcatheter interventions. Due to limitations in vascular access and surgical anatomies, guidewire pacing may have a wide array of potential applications in pediatrics and the congenital heart disease population.

Cardiopulmonary Resuscitation in the Pediatric Cardiac Catheterization Laboratory: A Report From the American Heart Association's Get With the Guidelines-Resuscitation Registry.

OBJECTIVES: Hospitalized children with underlying heart disease are at high risk for cardiac arrest, particularly when they undergo invasive catheterization procedures for diagnostic and therapeutic interventions. Outcomes for children experiencing cardiac arrest in the cardiac catheterization laboratory remain under-reported with few studies reporting survival beyond the catheterization laboratory. We aim to describe survival outcomes after cardiac arrest in the cardiac catheterization laboratory while identifying risk factors associated with hospital mortality after these events. DESIGN: Retrospective observational study of data from a multicenter cardiac arrest registry from November 2005 to November 2016. Cardiac arrest in the cardiac catheterization laboratory was defined as the need for chest compressions greater than or equal to 1 minute in the cardiac catheterization laboratory. Primary outcome was survival to discharge. Variables analyzed using generalized estimating equations for association with survival included age, illness category (surgical cardiac, medical cardiac), preexisting conditions, pharmacologic interventions, and event duration. SETTING: American Heart Association's Get With the Guidelines-Resuscitation registry of in-hospital cardiac arrest. PATIENTS: Consecutive patients less than 18 years old experiencing an index (i.e., first) cardiac arrest event reported to the Get With the Guidelines-Resuscitation. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 203 patients met definition of index cardiac arrest in the cardiac catheterization laboratory composed primarily of surgical and medical cardiac patients (54% and 41%, respectively). Children less than 1 year old comprised the majority of patients, 58% (117/203). Overall survival to hospital discharge was 69% (141/203). No differences in survival were observed between surgical and medical cardiac patients (p = 0.15). The majority of deaths (69%, 43/62) occurred in patients less than 1 year old. Bradycardia (with pulse) followed by pulseless electrical activity/asystole were the most common first documented rhythms observed (50% and 27%, respectively). Preexisting metabolic/electrolyte abnormalities (p = 0.02), need for vasoactive infusions (p = 0.03) prior to arrest, and use of calcium products (p = 0.005) were found to be significantly associated with lower rates of survival to discharge on multivariable regression. CONCLUSIONS: The majority of children experiencing cardiac arrest in the cardiac catheterization laboratory in this large multicenter registry analysis survived to hospital discharge, with no observable difference in outcomes between surgical and medical cardiac patients. Future investigations that focus on stratifying medical complexity in addition to procedural characteristics at the time of catheterization are needed to better identify risks for mortality after cardiac arrest in the cardiac catheterization laboratory.

Percutaneous atrial septal occluders: Are we there yet?

In a randomized, controlled trial, the Occlutech Figulla Flex II occluder was shown to be noninferior to the Amplatzer Septal Occluder. Both devices provided greater than 90% efficacy, with limited residual shunting and major complications. Although the risk of overall device erosions was low, larger and longer-term studies are needed to distinguish the differences between devices.

Branch pulmonary artery stenting in children by using premounted stents: Can we benefit from slenderization?

Children with branch pulmonary artery stenosis represent a challenging cohort for stenting Premounted stent implantation provides immediate obstruction relief and hemodynamic improvement in infants Planned stent intervention is required to account for somatic growth and next stage palliation in children Continued slenderization of adult coronary and peripheral interventions, stent technology, and delivery systems may allow for more durable solutions in light of patient size.

Percutaneous pulmonary valve implantation: It's not like the aortic valve.

The Edwards Sapien S3 demonstrated clinical and technical feasibility in this cohort undergoing percutaneous pulmonary valve implantation. Traversing the tricuspid apparatus with an unsheathed delivery system continues to pose a risk for tricuspid valve injury. Future design innovations need to accommodate for large outflow tracts without adjacent aortic or coronary compression and allow for safe device delivery with minimal trauma to the tricuspid apparatus.

Use of Ultrasound-Accelerated, Catheter-Directed Local Thrombolysis for Venous and Arterial Occlusions in a Pediatric Hospital.

OBJECTIVES: Acute vascular thrombosis is associated with significant morbidity and mortality in children. Traditional therapies with angioplasty and manual aspiration thrombectomy are described in the pediatric population; however, data regarding the use of ultrasound-assisted catheter-directed thrombolysis in a pediatric hospital are lacking. METHODS: All patients treated at our center with ultrasound-assisted catheter-directed thrombolysis from 2014-2018 were reviewed. RESULTS: Ten patients with systemic venous (n = 5), pulmonary artery (n = 4), and systemic arterial (n = 1) were treated (20 total vessels), including 2 adults post Fontan palliation. The median age was 11.2 years (range, 9 months to 34.2 years) and median weight was 46.6 kg (range, 6.81-01.6 kg). Therapy was not completed in 1 patient. Significant improvement in clot burden/symptomatic improvement was seen in 8 patients (no improvement seen in 1 patient with acute and chronic thrombosis). There were no major bleeding or neurologic complications related to therapy. CONCLUSIONS: The use of ultrasound-assisted catheter-directed thrombolysis in a pediatric center is a valuable modality for treating acute thrombosis with an acceptable safety profile.

Novel oral anticoagulant use in adult Fontan patients: A single center experience.

OBJECTIVE: Adult Fontan patients are at increased risk for thrombosis and thromboembolic complications leading to increased morbidity and mortality. Most are prescribed antiplatelet or anticoagulant therapy for thromboprophylaxis; novel oral anticoagulants (NOACs) are uncommonly used given lack of data on their use in this population and generalized concerns regarding Fontan patients' abnormal coagulation. We report the largest single-center experience with the use of NOACs for treatment and prophylaxis of thrombosis and thromboembolism in adult Fontan patients. RESULTS: A retrospective chart review identified 21 patients (11 female, 10 male), median age 33 years (18-50) at first initiation, who were prescribed a NOAC on 27 different occasions. The main indications for anticoagulation were arrhythmia (N = 12), thrombosis (N = 8), and persistent right to left shunts (N = 2); one patient was initially on anticoagulation for arrhythmia but restarted for thrombosis. The most common indications for initiation of a NOAC over warfarin were patient/provider preference (N = 11), labile international normalized ratio (INR) (N = 5), initiation of therapy elsewhere (N = 3), and history of poor clinical follow-up (N = 2). Over a cumulative 316 months of patient therapy, one new thrombotic event was noted. No major or nonmajor bleeding events occurred, and 10 patients experienced minor bleeding that did not require the cessation of therapy. One patient died from multiorgan system failure following an unwitnessed, out of hospital arrest. At present, 10 patients remain on NOAC therapy in the setting of ongoing arrhythmia (N = 4), history of stroke (N = 2), history of pulmonary embolism (N = 2), history of deep vein thrombosis (N = 1), and history of right ventricle thrombus (N = 1). CONCLUSIONS: While our study is limited by size, our results suggest that NOACs may be a non-inferior alternative to traditional anticoagulation and that further study is warranted.