Transcatheter pulmonary valve replacement (TPVR) is now frequently performed in adult congenital heart disease (ACHD) patients. As the life-expectancy of the ACHD population continues to improve, more patients will require pulmonary valve intervention. This study details the short-term and mid-term clinical outcomes of patients aged 40 years and older undergoing TPVR. We performed an institutional retrospective cohort study that included patients aged 40 and older undergoing TPVR (and clinical follow up) from January 1, 2012 to January 1, 2024. Descriptive analyses, Kaplan-Meier survival analysis, and Cox proportional hazard modeling were utilized to determine outcomes and risk factors affecting survival. The study included 67 patients and median age at TPVR was 48 [43,57] years. Median hospital length of stay following TPVR was 1 [1,3] day, peri-procedural complications occurred in 5 patients and acute kidney injury occurred in 1 patient. Median duration of follow-up was 3.5 [0.1,9.7] years. There were 9 total deaths and 1, 3, and 5-year Kaplan-Meier survival after TPVR was 95%, 91%, and 82%, respectively. Moderate or worse right ventricular dysfunction was present in 22 patients before TPVR and in 20 patients after TPVR. Inpatient status prior to TPVR negatively impacted survival (HR = 24.7 [3.3, 186.1], p=0.002). In conclusion, TPVR was performed in patients ages 40 and older with favorable periprocedural and mid-term follow-up outcomes including survival, but right ventricular dysfunction did not improve and further exploration on ideal timing of TPVR in this age group is warranted.

Atrial septal defects (ASDs), comprising a significant portion of congenital cardiac anomalies, encompass a rarer and more diagnostically challenging subset known as sinus venosus ASDs (SVASDs). ASDs are more prevalent in females, and the prognosis for patients under 40 years of age is generally favorable with advancements in surgical and transcatheter interventions. However, undiagnosed ASDs in adults above 40 years old, especially females, often lead to severe complications, including pulmonary hypertension, atrial fibrillation, Eisenmenger syndrome, and a mortality rate exceeding 50%. Our detailed case study focuses on an obese 42-year-old Hispanic migrant female with chronic respiratory failure misattributed to pulmonary hypertension, resulting in the progression of complications from undiagnosed SVASD. Further investigation using contrast-enhanced transesophageal echocardiography (TEE) elucidated the correct diagnosis four years after her initial presentation. This report explores the potential factors contributing to the patient\'s delayed diagnosis and development of advanced cardiac complications of pulmonary hypertension leading to Eisenmenger syndrome that precluded her from procedural intervention. Furthermore, this report pioneers the first thorough review of case reports in adults newly diagnosed with SVASD, revealing sex-based differences in complications.

UNASSIGNED: Little is known regarding the characteristics, treatment patterns, and outcomes in patients with adult congenital heart disease (ACHD) admitted to cardiac intensive care units (CICUs).
UNASSIGNED: The authors sought to better define the contemporary epidemiology, treatment patterns, and outcomes of ACHD admissions in the CICU.
UNASSIGNED: The Critical Care Cardiology Trials Network is a multicenter network of CICUs in North America. Participating centers contributed prospective data from consecutive admissions during 2-month annual snapshots from 2017 to 2022. We analyzed characteristics and outcomes of admissions with ACHD compared with those without ACHD. Multivariable logistic regression was used to assess mortality in ACHD vs non-ACHD admissions.
UNASSIGNED: Of 23,299 CICU admissions across 42 sites, there were 441 (1.9%) ACHD admissions. Shunt lesions were most common (46.1%), followed by right-sided lesions (29.5%) and complex lesions (28.7%). ACHD admissions were younger (median age 46 vs 67 years) than non-ACHD admissions. ACHD admissions were more commonly for heart failure (21.3% vs 15.7%, P < 0.001), general medical problems (15.6% vs 6.0%, P < 0.001), and atrial arrhythmias (8.6% vs 4.9%, P < 0.001). ACHD admissions had a higher median presenting Sequential Organ Failure Assessment score (5.0 vs 3.0, P < 0.001). Total hospital stay was longer for ACHD admissions (8.2 vs 5.9 days, P < 0.01), though in-hospital mortality was not different (12.7% vs 13.6%; age- and sex-adjusted OR: 1.19 [95% CI: 0.89-1.59], P = 0.239).
UNASSIGNED: This study illustrates the unique aspects of the ACHD CICU admission. Further investigation into the best approach to manage specific ACHD-related CICU admissions, such as cardiogenic shock and acute respiratory failure, is warranted.

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Patients with congenital heart disease now live well into adulthood because of advances in surgical techniques, improvements in medical management, and the development of novel therapeutic agents. As patients grow older into adults with congenital heart disease, many require catheter-based interventions for the treatment of residual defects, sequelae of their initial repair or palliation, or acquired heart disease. The past 3 decades have witnessed an exponential growth in both the type and number of transcatheter interventions in patients with congenital heart disease. With improvements in medical technology and device design, including the use of devices designed for the treatment of acquired valve stenosis or regurgitation, patients who previously would have required open-heart surgery for various conditions can now undergo percutaneous cardiac catheter-based procedures. Many of these procedures are complex and occur in complex patients who are best served by a multidisciplinary team. This review aims to highlight some of the currently available transcatheter interventional procedures for adults with congenital heart disease, the clinical outcomes of each intervention, and any special considerations so that the reader may better understand both the procedure and patients with adult congenital heart disease.

UNASSIGNED: Patients with a patent foramen ovale (PFO) who undergo percutaneous PFO closure are at a greater risk of developing atrial fibrillation (AF) compared with patients whose PFOs are managed medically. Postclosure AF appears to be well tolerated if treated but may increase the risk for stroke. Postclosure AF is reported to occur in 3.7% to 7.4% of patients; however, incidence across devices remains uncertain. This study aims to evaluate the frequency of postclosure AF, atrial flutter, and arrhythmias in 6 PFO closure devices.
UNASSIGNED: Four hundred forty-five patients underwent percutaneous PFO closure with appropriate follow-up between 2001 and 2021. The procedure was performed using Abbott Amplatzer PFO, Amplatzer ASD, Amplatzer Cribriform, NMT CardioSEAL, Gore Helex, or Gore Cardioform devices. Incidence of AF, atrial flutter, and arrhythmias were assessed by electrocardiogram within 6 months from closure. Multivariate logistic regression evaluated potential predictors of postclosure AF or atrial flutter.
UNASSIGNED: Postclosure AF or atrial flutter occurred in 30 patients (6.7%) within 6 months, and its incidence was significantly different across devices. Gore Cardioform had the greatest frequency of postclosure AF or atrial flutter events (16.8%) compared with other devices. The Gore Cardioform device, larger device sizes, and male sex were associated with greater risk of postclosure AF or atrial flutter.
UNASSIGNED: Postclosure AF or atrial flutter was more likely to occur in the Gore Cardioform device, in males, and in patients who underwent PFO closure with larger devices. Although it is more effective for complete closure, the Gore Cardioform device was shown to be an independent predictor of postclosure AF or atrial flutter.

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UNASSIGNED: Although surgical repair was the traditional first-line treatment for native coarctation of the aorta (CoA), balloon angioplasty (BA) and stenting are now increasingly being performed. We aimed to determine the practice patterns and acute outcomes of transcatheter interventions for native coarctation in the largest multicenter registry for congenital catheterization.
UNASSIGNED: CoA interventions from the IMPACT (IMproving Pediatric and Adult Congenital Treatment) National Cardiovascular Data Registry were analyzed. The procedure choice and acute outcomes were compared among patients with no prior interventions on the aortic isthmus (native CoA). Procedural success was defined as no major adverse events (MAEs) and a final peak gradient of <20 mm Hg and optimal outcome as no MAEs and a final gradient of <10 mm Hg.
UNASSIGNED: Over the 8-year study period, 5928 CoA procedures were performed, of which 1187 were performed in patients with native CoA. In this group, stenting was performed in more then half of children aged >1 year and >90% of those aged >8 years. Procedural success was achieved in >90% of stenting procedures but in only 69% of BAs. Stent implantation was associated with a higher likelihood of optimal gradient (<10 mm Hg) after adjustment for age and baseline characteristics. MAEs were most common in children aged <1 year (14%), occurred in 2% to 2.5% of those aged 1 to 18 years and in 6.6% of adults (P < .001), and were more likely after BA than after stenting (odds ratio, 0.5; 95% CI, 0.28-0.9; unadjusted P = .02).
UNASSIGNED: Catheter interventions for native coarctation are performed safely in older children and adults, with a high degree of immediate procedural success, particularly with stenting.

Severe systemic right ventricular failure with tricuspid regurgitation is associated with poor prognosis. Here, we report a case of 49-year-old patient who experienced severe systemic right ventricular failure following atrial switch. We chose the surgical strategy for this challenging case using comprehensive four-dimensional imaging. The patient underwent tricuspid valve repair and cardiac resynchronization therapy and recovered with improved cardiac function and regulated tricuspid valve regurgitation.