Athar M. Qureshi

BACKGROUND: The Amplatzer Muscular Ventricular Septal Defect (VSD) Occluder (Abbott Structural Heart, Plymouth, MN) was approved by the US Food and Drug Administration in 2007. As a condition of approval, a postapproval study was required to further evaluate its safety and effectiveness. This study reports the clinical outcomes from the postapproval study over 5 years of follow-up. METHODS: Patients with complex congenital muscular VSDs at high risk for surgical closure were enrolled between May 2008 and February 2020 in the United States and Canada. The primary effectiveness end points were technical implant success, acute procedure shunt-closure success, and 1-year shunt-closure success. The primary safety end point was the proportion of patients with any serious adverse event within 12 months of the procedure. RESULTS: A total of 92 patients (median age, 2 years; 50 females) were enrolled in the study. Technical implant success was achieved in 93.8% of patients, and acute shunt-closure success was achieved in 78.0%. Successful shunt closure at 1-year was 94.4% and increased to >98% between 2 and 5 years. Within 12 months of the procedure, all-cause serious adverse events occurred in 47 of the 90 (52.2%) patients which included 7 device-related and 23 procedure-related serious adverse events. Between 1 and 5 years, there was 1 additional device- and procedure-related serious adverse event. CONCLUSIONS: This postapproval study demonstrates that transcatheter muscular VSD closure with the Amplatzer Muscular VSD Occluder is a safe and effective therapy for the treatment of complex congenital muscular VSDs in patients who are at high-risk for surgical closure. REGISTRATION: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT00647387.

Background: Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention, using a staged or primary repair strategy. Postprocedural mortality risk within this group is considerable. The mode of death and associated factors have been inadequately defined in early childhood mortality following treatment of sTOF. This study aims to describe the timing, cause, and associated risk factors of postprocedural death in infants with sTOF. Methods: Neonates with sTOF who had an initial intervention from 2005 to 2017 at 9 centers of the Congenital Cardiac Research Collaborative were reviewed. Among nonsurvivors, details regarding timing, cause of death, and last follow‐up were obtained. Patient characteristics between survivors and nonsurvivors were compared. Results: Of 572 patients with sTOF (230 primary repair, 342 staged repair), 52 (9%) died at a median age of 82 (25th–75th%ile: 28–246) days. Death was perioperative (≤30 days) in 52% of cases. Most deaths were cardiac (69%, n=36), with heart failure and sudden death being most common. Noncardiac causes included necrotizing enterocolitis (n=3), infectious (n=3), and intracranial hemorrhage (n=2). Risk factors for mortality included prematurity, low birth weight, genetic syndrome, race categorized as other, noncardiac anomaly, and ventilation before initial intervention ( P <0.05 for all). Among patients discharged after definitive repair, right ventricular dysfunction was more common in nonsurvivors. Sudden death is a prominent known cause of death in this subgroup. Conclusions: When death occurs after interventions for sTOF, it frequently occurs early and relates to traditional risk factors. Sudden death is a prominent cause of mortality following discharge from definitive repair.

BACKGROUND: Infants with hypoplastic left heart syndrome with severely restrictive or intact atrial septum (R/IAS) have low survival. In-utero creation of an atrial septal communication has been reported, with high complication rates. METHODS: We performed a retrospective cohort study of fetuses with hypoplastic left heart syndrome, double outlet right ventricular with mitral stenosis/atresia, or mitral valve dysplasia and R/IAS from 2012 to 2024 who underwent evaluation for fetal atrial septal intervention (FASI). Excluding those with comorbidities limiting candidacy, discharge survival and overall survival were compared between (1) those undergoing and not undergoing FASI, and (2) those undergoing technically successful FASI (TS-FASI) and all others. RESULTS: Of 60 fetuses with R/IAS, 18 were considered extremely high risk and excluded from analysis. Among the remaining 42, 25 underwent FASI and 17 did not, with no significant baseline echocardiographic differences. FASI was technically successful in 18 cases, with 13 survivors (6 post-Glenn, 6 post-Fontan, 1 post-Ross). In the 7 unsuccessful FASIs, there were 5 peri-procedural deaths and 1 fetal demise that occurred post-FASI. Technical success rates improved from 58% (first 12) to 85% (last 13). Of 20 FASI patients with fetal magnetic resonance imaging, 15 (75%) had nutmeg lung ; of the 9 with nutmeg lung and post-TS-FASI fetal magnetic resonance imaging, 8 improved. Survival to discharge and overall survival were 52% in the FASI group versus 29% in the non-FASI group ( P =0.147 and log-rank P =0.376, respectively). When comparing TS-FASI to all others, survival was higher (72.2% versus 20.8% for discharge and overall survival, P =0.001 and log-rank P <0.001, respectively). All TS-FASI survivors with catheterization data (11/13) had PVR <3 Woods units before Glenn/Fontan. CONCLUSIONS: In our cohort, fetuses with R/IAS post-TS-FASI had significantly higher discharge and overall survival rates compared with all other fetuses with R/IAS. Nutmeg lung improved in the majority of TS-FASI cases with post-FASI imaging.