Catheterization for Congenital Heart Disease Adjustment for Risk Method II.

BACKGROUND: Current metrics used to adjust for case mix complexity in congenital cardiac catheterization are becoming outdated due to the introduction of novel procedures, innovative technologies, and expanding patient subgroups. This study aims to develop a risk adjustment methodology introducing a novel, clinically meaningful adverse event outcome and incorporating a modern understanding of risk. METHODS: Data from diagnostic only and interventional cases with defined case types were collected for patients ≤18 years of age and ≥2.5 kg at all Congenital Cardiac Catheterization Project on Outcomes participating centers. The derivation data set consisted of cases performed from 2014 to 2017, and the validation data set consisted of cases performed from 2019 to 2020. Severity level 3 adverse events were stratified into 3 tiers by clinical impact (3a/b/c); the study outcome was clinically meaningful adverse events, severity level ≥3b (3bc/4/5). RESULTS: The derivation data set contained 15 224 cases, and the validation data set included 9462 cases. Clinically meaningful adverse event rates were 4.5% and 4.2% in the derivation and validation cohorts, respectively. The final risk adjustment model included age <30 days, Procedural Risk in Congenital Cardiac Catheterization risk category, and hemodynamic vulnerability score (C statistic, 0.70; Hosmer-Lemeshow P value, 0.83; Brier score, 0.042). CONCLUSIONS: CHARM II (Congenital Heart Disease Adjustment for Risk Method II) risk adjustment methodology allows for equitable comparison of clinically meaningful adverse events among institutions and operators with varying patient populations and case mix complexity performing pediatric cardiac catheterization.

Self-Expanding Transcatheter Pulmonary Valve Implant in the Right Pulmonary Artery.

Newer self-expanding transcatheter pulmonary valves (TPVs) are approved for the treatment of severe pulmonary regurgitation in patients with large right ventricular outflow tracts. We present a patient with Tetralogy of Fallot whose right ventricular outflow tract was too large for self-expanding TPV, who was treated successfully with a self-expanding TPV in the right pulmonary artery. (Level of Difficulty: Advanced.).

Update on percutaneous and perventricular device closure of congenital ventricular septal defect.

INTRODUCTION: Ventricular septal defect is the most common congenital heart defect. Surgical repair has been standard therapy for symptomatic ventricular septal defects since the 1950s. Catheter-based device closure of ventricular septal defects emerged in the 1980s and has become a safe and effective alternative in select patients. AREAS COVERED: This review focuses on patient selection and procedural techniques for device closure of ventricular septal defects, including percutaneous and hybrid perventricular approaches. The available devices used for these procedures and outcomes of their use are reviewed. EXPERT OPINION: Percutaneous and perventricular device closure of ventricular septal defects is safe and effective in select patients. However, the majority of ventricular septal defects requiring closure continue to be managed with conventional surgery. Further development and investigation of transcatheter and hybrid surgical approaches for closing ventricular septal defects is required.

Palliative stenting of the venous duct in a premature neonate with obstructed infradiaphragmatic total anomalous pulmonary venous connection.

In infracardiac, infradiaphragmatic total anomalous pulmonary venous connection, all four pulmonary veins connect to a descending vertical vein that usually drains to the portal vein or one of its tributaries. Obstruction is common, and definitive treatment is surgical repair. We present a case of late-diagnosed infradiaphragmatic total anomalous pulmonary venous connection in a premature neonate who was too high risk for surgery and underwent palliative stenting of the venous duct. We demonstrate the feasibility of a transhepatic approach when umbilical access is no longer available.

Perventricular Transcatheter Pulmonary Valve Implantation in a Symptomatic 3-Year-Old Child With Repaired Tetralogy of Fallot.

In select patients, transcatheter pulmonary valve replacement through a percutaneous approach can be challenging because of complicated anatomy or small patient size. In these patients, especially those weighing <20 kg, hybrid perventricular valve delivery may provide a preferred alternative approach. (Level of Difficulty: Intermediate.).

Acute and midterm results following perventricular device closure of muscular ventricular septal defects: A multicenter PICES investigation.

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 < 0.01). At mid-term follow-up of 19.2 months (IQR 2.3-43) 90% of pts had complete mVSD closure; none developed late heart block, increased atrioventricular (AV) valve insufficiency or ventricular dysfunction. CONCLUSIONS: Perventricular device closure of simple mVSD was associated with a high rate of procedural success, few adverse events, and short hospital LOS. Procedural adverse events were associated with the presence of concomitant complex surgery. Residual mVSD, AV valve insufficiency, or ventricular dysfunction were uncommon at mid-term follow-up. © 2017 Wiley Periodicals, Inc.

Electrocardiography in the diagnosis of right ventricular hypertrophy in children.

OBJECTIVE: Although the electrocardiogram is commonly obtained in the evaluation of patients with pulmonary hypertension, its value as a screening test for right ventricular hypertrophy or pulmonary hypertension is unclear. Therefore, we sought to determine the value of an electrocardiogram in the diagnosis of right ventricular hypertrophy using echocardiography as the gold standard. METHODS: We identified children without congenital heart disease who underwent evaluation for suspected pulmonary hypertension that included both an electrocardiogram and echocardiography within a specified time frame. RESULTS: A total of 76 echocardiography-electrocardiogram pairs for pulmonary hypertension were identified. Although there was a significant relationship between electrocardiogram and echocardiography evidence of right ventricular hypertrophy, the sensitivity of an electrocardiogram in diagnosing echocardiography-documented right ventricular hypertrophy was only 69%, and the positive predictive value was 67%. There was no relationship between electrocardiogram changes and Doppler tricuspid regurgitation gradient. CONCLUSION: Despite a statistically significant relationship between an electrocardiogram and echocardiography in the diagnosis of right ventricular hypertrophy, an electrocardiogram has limited value as a screening tool for right ventricular hypertrophy because of its relatively low sensitivity and positive predictive value.

Reactivity of the ductus arteriosus: Implications for transcatheter therapy.

We report our experience in a 13-month-old boy undergoing transcatheter coil occlusion of a patent ductus arteriosus. Constriction of the ductus arteriosus with subsequent relaxation resulted in inadvertent coil embolization. This case report and review of the literature have implications for transcatheter treatment of persistent ductus arteriosus.

Safety of endomyocardial biopsy in children.

Endomyocardial biopsy in children remains important in the evaluation of cardiomyopathy, myocarditis, and rejection following cardiac transplantation. We sought to evaluate the morbidity associated with endomyocardial biopsy on the basis of a large experience from a single institution. We reviewed records of all endomyocardial biopsies performed at our institution. Complications were defined as adverse events resulting from the biopsy requiring intervention or additional observation. We also reviewed the most recent echocardiogram from all the patients for the presence and severity of tricuspid valvar regurgitation. Between November 1986 and April 2002, we performed 1051 endomyocardial biopsies in 135 patients ranging in age from 9 days to 18 years. The internal jugular vein was the site of vascular access in 68% of the procedures. There were 10 acute procedural complications requiring intervention or additional observation. Severe tricuspid regurgitation developed in two patients who had undergone multiple biopsies after cardiac transplantation, one of whom underwent subsequent replacement of the tricuspid valve. There were no deaths or cardiac perforations. The total incidence of morbidity was 1.1%. No demographic or procedural factors were identified to be predictive of complications. In experienced hands, therefore, endomyocardial biopsy can be safely performed in children with very low morbidity.