Search

Sinus node dysfunction is a common problem and adversely impacts patients who have undergone the Fontan operation. In Fontan patients with an adequate junctional escape rhythm, the benefit of atrial pacing to restore atrioventricular synchrony remains unclear. Data were collected retrospectively on all Fontan patients with junctional rhythm who underwent atrial pacing during cardiac catheterization. Hemodynamics were obtained at baseline and after atrial pacing for 5-10 min. Seven patients, mean age 10 years (3-21) were studied. The type of Fontan was extracardiac in 6 and lateral tunnel in one. Patients were paced at 10 bpm faster than their junctional rate. With pacing, there was a significant decrease in left atrial pressure from (mean ± SEM) 8.8 ± 2.6 to 5.5 ± 2.9 mmHg (p = 0.02), a significant increase in cardiac index from 2.7 ± 0.8 to 3.5 ± 1 L/min/m 2 (p = 0.01) and pulmonary blood flow from 2.1 ± 0.6 to 2.7 ± 0.7 L/min/m 2 (p = 0.001), and no significant change in pulmonary artery pressure, from 13.4 ± 2.8 to 12.4 mmHg ± 3.6 (p = 0.06) or pulmonary vascular resistance from 2.1 ± 0.86 to 3.25 ± 1.9 WU × M2 (p = 0.1). In Fontan patients with junctional rhythm, atrial pacing decreased the left atrial pressure, increased cardiac output and did not significantly change the pulmonary artery pressure. Our study supports further investigation into the utility of prophylactic atrial pacemaker implantation in Fontan patients with significant durations of JR on ambulatory monitoring., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

While rheumatic heart disease (RHD) is a treatable disease nearly eradicated in the United States, it remains the most common form of acquired heart disease in the developing world. This study used echocardiographic screening to determine the prevalence of RHD in children in American Samoa. Screening took place at a subset of local schools. Private schools were recruited and public schools underwent cluster randomization based on population density. We collected survey information and performed a limited physical examination and echocardiogram using the World Heart Federation protocol for consented school children aged 5-18 years old. Of 2200 students from two private high schools and two public primary schools, 1058 subjects consented and were screened. Overall, 133 (12.9%) children were identified as having either definite (3.5%) or borderline (9.4%) RHD. Of the patients with definitive RHD, 28 subjects had abnormal mitral valves with pathologic regurgitation, three mitral stenosis, three abnormal aortic valves with pathologic regurgitation, and seven borderline mitral and aortic valve disease. Of the subjects with borderline disease, 77 had pathologic mitral regurgitation, 12 pathologic aortic regurgitation, and 7 at least two features of mitral valve disease without pathologic regurgitation or stenosis. Rheumatic heart disease remains a major cause of morbidity and mortality worldwide. The prevalence of RHD in American Samoa (12.9%) is to date the highest reported in the world literature. Echocardiographic screening of school children is feasible, while reliance on murmur and Jones criteria is not helpful in identifying children with RHD.

Sedation/anesthesia is critical to cardiac catheterization in the pediatric/congenital heart patient. We sought to identify current sedation/anesthesia practices, the serious adverse event rate related to airway, sedation, or anesthesia, and the rate of intra-procedural conversion from procedural sedation to the use of assisted ventilation or an artificial airway. Data from 13,611 patients who underwent catheterization at eight institutions were prospectively collected from 2007 to 2010. Ninety-four (0.69 %) serious sedation/airway-related adverse events occurred; events were more likely to occur in smaller patients (<4 kg, OR 4.4, 95 % CI 2.3-8.2, p < 0.001), patients with non-cardiac comorbidities (OR 1.7, 95 % CI 1.1-26, p < 0.01), and patients with low mixed venous oxygen saturation (OR 2.3, 95 % CI 1.4-3.6, p < 0.001). Nine thousand three hundred and seventy-nine (69 %) patients were initially managed with general endotracheal anesthesia, LMA, or tracheostomy, whereas 4232 (31 %) were managed with procedural sedation without an artificial airway, of which 75 (1.77 %) patients were converted to assisted ventilation/general anesthesia. Young age (<12 months, OR 5.2, 95 % CI 2.3-11.4, p < 0.001), higher-risk procedure (category 4, OR 10.1, 95 % CI 6.5-15.6, p < 0.001), and continuous pressor/inotrope requirement (OR 11.0, 95 % CI 8.6-14.0, p < 0.001) were independently associated with conversion. Cardiac catheterization in pediatric/congenital patients was associated with a low rate of serious sedation/airway-related adverse events. Smaller patients with non-cardiac comorbidities or low mixed venous oxygen saturation may be at higher risk. Patients under 1 year of age, undergoing high-risk procedures, or requiring continuous pressor/inotrope support may be at higher risk of requiring conversion from procedural sedation to assisted ventilation/general anesthesia.

Pediatric and Congenital Interventional Cardiology is the practice of catheter-based techniques that improve cardiac physiology and circulation through the treatment of heart disease in children and adults with congenital or acquired heart defects. Over the last decade, and since last published training guidelines for pediatric cardiac catheterization and interventional cardiology were published in 2005 [1] the field of Pediatric and Congenital Cardiac Catheterization has evolved into a predominantly interventional discipline. As there is no sub-specialty certification for interventional cardiac catheterization in pediatrics, the Congenital Heart Disease Committee of the Society of Cardiovascular Angiography and Interventions has put together this consensus statement for advanced training in pediatric and congenital interventional cardiac catheterization. The statement puts forth recommendations for program infrastructure in terms of teaching, personnel, equipment, facilities, conferences, patient volume and trainee assessment. This is meant to set a standard for training programs as well as giving applicants a basis on which to judge and compare programs., (© 2014 Wiley Periodicals, Inc.)

Background: Shear stress-induced platelet dysfunction (PD) is prevalent among adults with aortic stenosis. Our aim was to determine whether abnormal platelet function was associated with specific congenital cardiac lesions in children., Methods: The charts of 407 children who had undergone cardiopulmonary bypass and had preoperative platelet function analysis were evaluated. Patients were assigned to 1 of 11 different lesion categories. Platelet dysfunction (PD) was defined as prolonged closure time (CT) as measured with a platelet function analyzer. Odds ratio (OR) estimates for prolonged CT were calculated for each lesion category. Mean CTs were compared with Tukey-Kramer separated means testing. Analysis of variance modeling was used to determine association between hematocrit value and CT., Results: CT in patients with ventricular septal defects (VSD) and right ventricular outflow tract obstruction (RVOTO) lesions was prolonged. OR analysis found that patients with VSDs (OR, 2.46) or RVOTO (OR, 2.88) had at least a 95% probability of an abnormal CT. In contrast, patients with atrial septal defect (ASD), bidirectional Glenn procedure (BDG), and pulmonary insufficiency (PI) had a reduced probability of a prolonged CT (p < 0.05). A similar pattern was seen in parametric analysis comparing mean CTs across lesion categories. A lower preoperative hematocrit value was associated with prolonged CTs across all lesion types (p < 0.05)., Conclusions: PD was common in children with congenital cardiac lesions involving systolic flow abnormalities and was uncommon among children with lesions having diastolic abnormalities. Lower preoperative hematocrit values were associated with prolonged CTs, suggesting subclinical bleeding secondary to excessive platelet shearing., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Continued advancements in congenital cardiac catheterization and interventions have resulted in increased patient and procedural complexity. Anticipation of life-threatening events and required rescue measures is a critical component to preprocedural preparation. We sought to determine the incidence and nature of life-threatening adverse events in congenital and pediatric cardiac catheterization, risk factors, and resources necessary to anticipate and manage events. Data from 8905 cases performed at the 8 participating institutions of the Congenital Cardiac Catheterization Project on Outcomes were captured between 2007 and 2010 [median 1,095/site (range 133-3,802)]. The incidence of all life-threatening events was 2.1 % [95 % confidence interval (CI) 1.8-2.4 %], whereas mortality was 0.28 % (95 % CI 0.18-0.41 %). Fifty-seven life-threatening events required cardiopulmonary resuscitation, whereas 9 % required extracorporeal membrane oxygenation. Use of a risk adjustment model showed that age <1 year [odd ratio (OR) 1.9, 95 % CI 1.4-2.7, p < 0.001], hemodynamic vulnerability (OR 1.6, 95 % CI 1.1-2.3, p < 0.01), and procedure risk (category 3: OR 2.3, 95 % CI 1.3-4.1; category 4: OR 4.2, 95 % CI 2.4-7.4) were predictors of life-threatening events. Using this model, standardized life-threatening event ratios were calculated, thus showing that one institution had a life-threatening event rate greater than expected. Congenital cardiac catheterization and intervention can be performed safely with a low rate of life-threatening events and mortality; preprocedural evaluation of risk may optimize preparation of emergency rescue and bailout procedures. Risk predictors (age < 1, hemodynamic vulnerability, and procedure risk category) can enhance preprocedural patient risk stratification and planning.

Background: Studies have documented the importance of procedure type and hemodynamic variables on the incidence of procedure related adverse events (AE) after cardiac catheterization. However, little is known about the impact of low weight on the incidence and severity of AE., Methods: Data were prospectively collected using a multicenter registry (C3PO). Infants <1 year were divided into four weight categories: <2 kg, 2-3 kg, 3-5 kg, ≥5 kg. AE severity was classified as level 1-5 (none, minor, moderate, major, death)., Results: Eight centers submitted details on 3,679 cases (34% diagnostic) performed in infants <1 year from 2/07 to 6/10: <2 kg: 57 (1.5%), 2-3 kg: 403 (11%), 3-5 kg: 1,527 (41.5%), ≥5 kg: 1,692 (46%). AE occurred in 20% of cases (<2 kg: 28%, 2-3 kg: 25%, 3-5 kg: 23%, ≥5 kg: 16%) with 41% of all AE being level 3-5 AE. Death occurred more frequently in the <2 kg group (12%), 71% of which were interventional cases. The case-related mortality in all other weight groups was <1%. By multivariable analysis, weight <2 kg, 2-3 kg, and 3-5 kg were independent risk factors for high severity (level 3-5) AE (<2 kg: OR 2, 95%CI 1.1-3.6; 2-3 kg: OR 1.4, 95%CI 1-1.8; 3-5 kg: OR 1.3, 95%CI 1.1-1.5), with similar findings for all AE. Blood transfusions were more common in lower weight categories (<2 kg: 42%, 2-3 kg: 29%, 3-5 kg: 25%, ≥5 kg: 15%, p<0.001)., Conclusions: The risk of AE during cardiac catheterization of infants increases with lower weight. Infants who weigh less than 2 kg have a significantly higher risk of adverse events (most notably death) even after correcting for hemodynamic vulnerability and procedure type risk group., (Copyright © 2013 Wiley Periodicals, Inc.)

Objectives: This study sought to develop a method to adjust for case mix complexity in catheterization for congenital heart disease to allow equitable comparisons of adverse event (AE) rates., Background: The C3PO (Congenital Cardiac Catheterization Project on Outcomes) has been prospectively collecting data using a Web-based data entry tool on all catheterization cases at 8 pediatric institutions since 2007., Methods: A multivariable logistic regression model with high-severity AE outcome was built using a random sample of 75% of cases in the multicenter cohort; the models were assessed in the remaining 25%. Model discrimination was assessed by the C-statistic and calibration with Hosmer-Lemeshow test. The final models were used to calculate standardized AE ratios., Results: Between August 2007 and December 2009, 9,362 cases were recorded at 8 pediatric institutions of which high-severity events occurred in 454 cases (5%). Assessment of empirical data yielded 4 independent indicators of hemodynamic vulnerability. Final multivariable models included procedure type risk category (odds ratios [OR] for category: 2 = 2.4, 3 = 4.9, 4 = 7.6, all p < 0.001), number of hemodynamic indicators (OR for 1 indicator = 1.5, ≥2 = 1.8, p = 0.005 and p < 0.001), and age <1 year (OR: 1.3, p = 0.04), C-statistic 0.737, and Hosmer-Lemeshow test p = 0.74. Models performed well in the validation dataset, C-statistic 0.734. Institutional event rates ranged from 1.91% to 7.37% and standardized AE ratios ranged from 0.61 to 1.41., Conclusions: Using CHARM (Catheterization for Congenital Heart Disease Adjustment for Risk Method) to adjust for case mix complexity should allow comparisons of AE among institutions performing catheterization for congenital heart disease., (Copyright © 2011 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Significant morbidities, including angina, symptomatic heart failure, and myocardial infarction, have been reported after coronary artery fistula (CAF) closure; however, predictors that may be associated with adverse outcomes have not been established. The goal of this investigation is to describe the long-term outcomes witnessed in patients with either treated or untreated CAF at our institution and to investigate whether certain features predicted adverse outcomes., Methods and Results: The records and angiograms of patients with CAF who underwent a diagnostic cardiac catheterization at Children's Hospital Boston from 1959 through 2008 were reviewed. Of 76 patients identified, 20% were associated with additional congenital heart disease. Forty-four underwent transcatheter closure, 20 underwent surgical repair, and no intervention was performed in the remaining 12 subjects. Three patients who had initially undergone surgical closure had a second intervention, 1 underwent repeat surgery, and 2 underwent transcatheter closure. One patient who had undergone transcatheter closure underwent a second transcatheter closure for residual fistula. Major complications, including myocardial infarction, angina with coronary thrombosis, and symptomatic cardiomyopathy, occurred in 11 (15%) patients. The sole angiographic feature that was predictive of adverse outcome was drainage of the CAF into the coronary sinus (P<0.001). Clinical predictors associated with adverse outcomes included older age at diagnosis (P<0.001), tobacco use (P=0.006), diabetes (P=0.05), systemic hypertension (P<0.001), and hyperlipidemia (P<0.001)., Conclusions: Long-term complications of CAF closure may include coronary thrombosis, myocardial infarction, and cardiomyopathy. CAF that drain into the coronary sinus are at particularly high-risk of long-term morbidities after closure, and strategies including long-term anticoagulation should be considered.