Early Outcomes From a Multicenter Transcatheter Self-Expanding Pulmonary Valve Replacement Registry.

BACKGROUND: Transcatheter pulmonary valve replacement (TPVR) with the self-expanding Harmony valve (Medtronic) is an emerging treatment for patients with native or surgically repaired right ventricular outflow tract (RVOT) pulmonary regurgitation (PR). Limited data are available since U.S. Food and Drug Administration approval in 2021. OBJECTIVES: In this study, the authors sought to evaluate the safety and short-term effectiveness of self-expanding TPVR in a real-world experience. METHODS: This was a multicenter registry study of consecutive patients with native RVOT PR who underwent TPVR through April 30, 2022, at 11 U.S. CENTERS: The primary outcome was a composite of hemodynamic dysfunction (PR greater than mild and RVOT mean gradient >30 mm Hg) and RVOT reintervention. RESULTS: A total of 243 patients underwent TPVR at a median age of 31 years (Q1-Q3: 19-45 years). Cardiac diagnoses were tetralogy of Fallot (71%), valvular pulmonary stenosis (21%), and other (8%). Acute technical success was achieved in all but 1 case. Procedural serious adverse events occurred in 4% of cases, with no device embolization or death. Hospital length of stay was 1 day in 86% of patients. Ventricular arrhythmia prompting treatment occurred in 19% of cases. At a median follow-up of 13 months (Q1-Q3: 8-19 months), 98% of patients had acceptable hemodynamic function. Estimated freedom from the composite clinical outcome was 99% at 1 year and 96% at 2 years. Freedom from TPVR-related endocarditis was 98% at 1 year. Five patients died from COVID-19 (n = 1), unknown causes (n = 2), and bloodstream infection (n = 2). CONCLUSIONS: In this large multicenter real-world experience, short-term clinical and hemodynamic outcomes of self-expanding TPVR therapy were excellent. Ongoing follow-up of this cohort will provide important insights into long-term outcomes.

Ventricular septal defect Doppler peak gradient underestimates right ventricular systolic pressure in the presence of right bundle branch block.

Estimation of the right ventricular systolic pressure (RVSP) using echocardiographically derived maximal instantaneous gradient across the ventricular septal defect is a well-utilized tool, which can potentially underestimate the RVSP in patients with conduction abnormalities as such its utility in these patients is questionable.

ASSURED clinical study: New GORE® CARDIOFORM ASD occluder for transcatheter closure of atrial septal defect.

OBJECTIVE: Assess clinical performance of a new device for transcatheter closure of atrial septal defect (ASD). BACKGROUND: Previously-approved ASD Closure devices have known limitations. Device erosion has been associated with the AMPLATZER® septal occluder in patients with retro-aortic rim deficiency (<5 mm), while defects ≥18 mm are too large for the GORE® CARDIOFORM septal occluder. The GORE® CARDIOFORM ASD occluder (GCA), a hybrid of the approved devices, was designed to expand the eligible ASD population. METHODS: One-hundred and twenty-five ASD patients were enrolled in a prospective, multicenter registry. Descriptive clinical endpoints included: technical implant success, closure success, serious adverse events (SAE), clinically significant new arrhythmia, and wire frame fracture. Procedural outcomes and adverse events were adjudicated by an Echocardiography Core Lab and Independent Data Review Board, respectively. RESULTS: Median subject age was 12.3 years (range 2.9-84.7), with 72% of patients ≤18 years old. Median ASD stop-flow diameter was 17.0 mm (8.0-30.0), with 43% ≥18 mm. Deficient retro-aortic rim occurred in 57% of subjects, and 30% had both diameter ≥ 18 mm and deficient rim. Technical Implant Success was achieved in 120/125 (96%), though three devices were removed within 24 hr. At the scheduled 6-month evaluation, 112/117 returned for evaluation. All 112 had closure success. SAE occurred in 6/125 (4.8%) and 6/125 (4.8%) had clinically significant new arrhythmia. Wire frame fractures occurred in 37/104 (36%), without any associated clinical sequelae, residual shunt, or device instability. CONCLUSION: The GCA performed effectively and safely in this initial cohort, and led to FDA approval based on this data.

Frequency of Ventricular Arrhythmias and Other Rhythm Abnormalities in Children and Young Adults With the Marfan Syndrome.

Patients with the Marfan syndrome (MFS) are at risk for sudden death. The contribution of arrhythmias is unclear. This study examines the prevalence of arrhythmias in children with the MFS and their relation to clinical and/or echocardiographic factors. Data from the Pediatric Heart Network randomized trial of atenolol versus losartan in MFS were analyzed (6 months to 25 years old, aortic root diameter z-score > 3.0, no previous aortic surgery and/or dissection). Baseline 24-hour ambulatory electrocardiographic monitoring was performed. Significant ventricular ectopy (VE) and supraventricular ectopy (SVE) were defined as ≥10 VE or SVE/hour, or the presence of high-grade ectopy. Three-year composite clinical outcome of death, aortic dissection, or aortic root replacement was analyzed. There were 274 analyzable monitors on unique patients from 11 centers. Twenty subjects (7%) had significant VE, 13 (5%) significant SVE; of these, 2 (1%) had both. None had sustained ventricular or supraventricular tachycardia. VE was independently associated with increasing number of major Ghent criteria (odds ratio [OR] = 2.13/each additional criterion, p = 0.03) and greater left ventricular end-diastolic dimension z-score (OR = 1.47/each 1 unit increase in z-score, p = 0.01). SVE was independently associated with greater aortic sinotubular junction diameter z-score (OR = 1.56/each 1 unit increase in z-score, p = 0.03). The composite clinical outcome (14 events) was not related to VE or SVE (p ≥ 0.3), but was independently related to heart rate variability (higher triangular index). In conclusion, in this cohort, VE and SVE were rare. VE was related to larger BSA-adjusted left ventricular size. Routine ambulatory electrocardiographic monitoring may be useful for risk stratification in select MFS patients.

A multicenter review of ablation in the aortic cusps in young people.

BACKGROUND: Ablation within the aortic cusp is safe and effective in adults. There are little data on aortic cusp ablation in the pediatric literature. We investigated the safety and efficacy of aortic cusp ablation in young patients. METHODS: A retrospective, descriptive study of aortic cusp ablation in five pediatric electrophysiology centers from 2008 to 2014 was performed. All patients <21 years of age who underwent ablation in the aortic cusps were included. Factors analyzed included patient demographics, procedural details, outcomes, and complications. RESULTS: Thirteen patients met inclusion criteria (median age 16 years [range 10-20.5] and median body surface area 1.58 m2 [range 1.12-2.33]). Substrates for ablation included: nine premature ventricular contractions or sustained ventricular tachycardia (69%), two concealed anteroseptal accessory pathways (APs) (15%), one Wolff-Parkinson-White with an anteroseptal AP (8%), and one ectopic atrial tachycardia (8%). Three-dimensional electroanatomic mapping in combination with fluoroscopy was used in 12/13 (92%) patients. Standard 4-mm-tip radiofrequency (RF) current was used in 11/13 (85%) and low-power irrigated-tip RF in 2/13 (15%). Angiography was used in 13/13 and intracardiac echocardiography was additionally utilized in 3/13 (23%). Ablation locations included: eight noncoronary (62%), three left (23%), and two right (15%) cusps. Ablation was acutely successful in all patients. At median follow-up of 20 months, there was one recurrence of PVCs (8%). There were no ablation-related complications and no valvular injuries observed. CONCLUSION: Arrhythmias originating from the coronary cusps in this series were successfully and safely ablated in young people without injury to the coronary arteries or the aortic valve.

SCAI/CCAS/SPA Expert Consensus Statement for Anesthesia and Sedation Practice: Recommendations for Patients Undergoing Diagnostic and Therapeutic Procedures in the Pediatric and Congenital Cardiac Catheterization Laboratory.

Current practice of sedation and anesthesia for patients undergoing pediatric and congenital cardiac catheterization laboratory (PCCCL) procedures is known to vary among institutions, a multi-society expert panel with representatives from the Congenital Heart Disease Council of the Society for Cardiovascular Angiography and Interventions, the Society for Pediatric Anesthesia and the Congenital Cardiac Anesthesia Society was convened to evaluate the types of sedation and personnel necessary for procedures performed in the PCCCL. The goal of this panel was to provide practitioners and institutions performing these procedures with guidance consistent with national standards and to provide clinicians and institutions with consensus-based recommendations and the supporting references to encourage their application in quality improvement programs. Recommendations can neither encompass all clinical circumstances nor replace the judgment of individual clinicians in the management of each patient. The science of medicine is rooted in evidence, and the art of medicine is based on the application of this evidence to the individual patient. This expert consensus statement has adhered to these principles for optimal management of patients requiring sedation and anesthesia. What follows are recommendations for patient monitoring in the PCCCL regardless of whether minimal or no sedation is being used or general anesthesia is being provided by an anesthesiologist.

Malattached septum primum and deficient septal rim predict unsuccessful transcatheter closure of atrial communications.

OBJECTIVES: Assess impact of malattached septum primum (MASP) on transcatheter closure of interatrial communications. BACKGROUND: Large defect size and deficient rims have been described as predictors for complications and unsuccessful device placement in closure of interatrial communications. MASP is an underappreciated morphologic atrial septal variation whose prevalence in isolated atrial communications and its influence on percutaneous device closure has not been systematically assessed. METHODS: We retrospectively evaluated echocardiographic data of 328 patients scheduled for percutaneous secundum atrial septal defect (ASD) and patent foramen ovale (PFO) closure between January 2006 and January 2013. In ASD patients, defect size was measured by balloon stretch diameter while the length of the surrounding rims was measured in standard transthoracic and transesophageal views. Furthermore, the distance between septum primum and septum secundum as it attaches to the left atrial roof was evaluated in both, ASD and PFO patients. Septal anatomy of patients with procedural failure was compared to the anatomy of patients in whom the procedure was successful. RESULTS: Transcatheter defect closure was successful in 131 (92.3%) of 142 ASD patients (32.3 years ± 24.7 years) and in all 186 PFO patients (49.6 years ± 16.2 years). Thirteen (4%) patients were found to have MASP (1.6 mm-13 mm). Smaller retroaortic rim, inferior rim, and the presence of a MASP were independently associated with procedural failure in ASD patients (P = 0.02, P = 0.03, and P = 0.003, respectively). CONCLUSION: Smaller retroaortic and inferior rims, as well as MASP are independent risk factors for unsuccessful transcatheter ASD closure.

Peripheral pulmonary artery stenosis masquerading as pulmonary hypertension: a diagnostic and therapeutic challenge.

Isolated peripheral pulmonary artery stenosis presenting in adulthood is an uncommon cause of right ventricular hypertension and may be misdiagnosed as primary or thromboembolic pulmonary hypertension. Treatment consists of balloon angioplasty and stenting of the affected vessels, although the procedure can be complicated by reperfusion pulmonary edema.

Outcomes of mitral regurgitation associated with large ventricular septal defect and a normal mitral valve apparatus: does intact atrial septum have an impact?

The hemodynamics of isolated large ventricular septal defect with an intact atrial septum results in greater mitral valve (MV) annular dilation and an increased incidence of mitral regurgitation (MR). MR will resolve in most patients after surgical VSD closure alone without MV repair.

Percutaneous transhepatic venous access for catheter ablation procedures in patients with interruption of the inferior vena cava.

BACKGROUND: Catheter ablation of left-sided atrial arrhythmias generally is performed using a transfemoral venous approach through the inferior vena cava (IVC). In this report, we assessed the feasibility of a percutaneous transhepatic approach to ablation of left-sided atrial arrhythmias in 2 patients with interruption of the IVC. METHODS AND RESULTS: Patient 1 had atrial flutter in the setting of complex congenital heart disease and prior Fontan for univentricular physiology and a single atrium. Patient 2 had atrial fibrillation. Percutaneous hepatic vein access was obtained with ultrasound and fluoroscopic guidance. Transseptal catheterization was performed in patient 2. After the procedure, the hepatic tract in patient 1 was cauterized using a bipolar radiofrequency catheter, and an Amplatzer vascular plug was used in patient 2 to obtain hemostasis. Percutaneous hepatic vein access was achieved without complications. After electroanatomical mapping, a linear lesion was placed between the single atrioventricular valve and the confluence of the hepatic veins in patient 1; this terminated the flutter, and bidirectional block was achieved. In patient 2, the pulmonary veins were electrically isolated using an extraostial approach, isolating the ipsilateral veins in pairs. Additionally, ablation of right-side atrial flutter was achieved by obtaining bidirectional block across a linear lesion between the tricuspid valve and confluence of the hepatic veins. Hemostasis of the transhepatic tract was attained in both patients. CONCLUSIONS: In patients with interrupted IVCs, a percutaneous transhepatic approach is a feasible alternative for performing catheter ablation of complex left-sided arrhythmias.

Transcatheter closure of recurrent postmyocardial infarction ventricular septal defect facilitated by percutaneous left ventricle access.

We report a case of a 68-year-old woman with a basal postmyocardial infarction ventricular septal defect that recurred 4 months after initial surgical repair. Successful transcatheter closure using an Amplatzer Muscular VSD Occluder was facilitated by direct percutaneous left ventricular access.

Transcatheter repair of perivalvular regurgitation.

Hemodynamically significant perivalvular regurgitation affects about 1.5% of all prosthetic valve implants. Reoperation carries increased risk--especially in the setting of concomitant comorbidities of left ventricular dysfunction, prior coronary artery bypass grafts, renal dysfunction, and advanced age. Transcatheter closure of the perivalvular regurgitant channel using a variety of occluders has been available for several years; however, recent improvements in technology and technique have made this therapy more effective and available to a wider group of patients. This article describes the recent advances and the state of the art of this therapy.