Kathy J. Jenkins

Noninherited Risk Factors and Congenital Cardiovascular Defects: Current Knowledge A Scientific Statement From the American Heart Association Council on Cardiovascular Disease in the Young: Endorsed by the American Academy of Pediatrics

Prevention of congenital cardiovascular defects has been hampered by a lack of information about modifiable risk factors for abnormalities in cardiac development. Over the past decade, there have been major breakthroughs in the understanding of inherited causes of congenital heart disease, including the identification of specific genetic abnormalities for some types of malformations. Although relatively less information has been available on noninherited modifiable factors that may have an adverse effect on the fetal heart, there is a growing body of epidemiological literature on this topic. This statement summarizes the currently available literature on potential fetal exposures that might alter risk for cardiovascular defects. Information is summarized for periconceptional multivitamin or folic acid intake, which may reduce the risk of cardiac disease in the fetus, and for additional types of potential exposures that may increase the risk, including maternal illnesses, maternal therapeutic and nontherapeutic drug exposures, environmental exposures, and paternal exposures. Information is highlighted regarding definitive risk factors such as maternal rubella; phenylketonuria; pregestational diabetes; exposure to thalidomide, vitamin A cogeners, or retinoids; and indomethacin tocolysis. Caveats regarding interpretation of possible exposure-outcome relationships from case-control studies are given because this type of study has provided most of the available information. Guidelines for prospective parents that could reduce the likelihood that their child will have a major cardiac malformation are given. Issues related to pregnancy monitoring are discussed. Knowledge gaps and future sources of new information on risk factors are described.

Transcatheter Device Closure of Congenital and Postoperative Residual Ventricular Septal Defects

Background—Our purpose was to describe a 13-year experience with patients undergoing transcatheter device closure of unrepaired congenital or postoperative residual ventricular septal defects (VSDs). Methods and Results—Since 1989, 170 patients (median age, 3.9 years) have undergone catheterization for closure of 1 or more congenital (n=92) or postoperative (n=78) residual VSDs using successive generations of STARFlex-type devices. Outcomes included echocardiographic assessment of residual flow and device position, assessment of VSD shunt/severity, and adverse events. Among 168 patients in whom device implantation was performed, between 1 and 7 devices were placed per patient (median, 1), with multiple devices placed in 40%. There was a significant decrease in left-to-right shunting after device implantation (P<0.001) and significant improvement in VSD size/severity, and device position proved stable. Of 332 adverse events, 39 were related to the device and 261 were related to the catheterization; all but 5 occurred in the periprocedural period. At a median follow-up of 24 months (0 to 154 months), 14 patients had died and 18 had device(s) explanted. Conclusions—Congenital and postoperative VSD closure using STARFlex-type devices resulted in stable improvement in clinical status and decreased interventricular shunting. Although periprocedural events occurred frequently, late events caused by the device were rare. Transcatheter device closure is an effective management option for patients with complex muscular VSDs that are difficult to approach surgically and for postoperative residual VSDs.

Factors Associated With Increased Resource Utilization for Congenital Heart Disease

Objective. To identify patient, institutional, and regional factors that are associated with high resource utilization for congenital heart surgery. Methods. We used hospital discharge data from the Healthcare Cost and Utilization Project (HCUP) Kids’ Inpatient Database (KID) year 2000 (data from 27 states). Patients who had congenital heart surgery and were younger than 18 years were identified using International Classification of Diseases, Ninth Revision, Clinical Modification codes. High resource utilization admissions were defined as those in the highest decile for total hospital charges. Univariate and multivariate analyses with and without deaths were used to determine demographic and hospital predictors for cases of high resource use. Case-mix severity was approximated using Risk Adjustment for Congenital Heart Surgery risk groups. Regional and state differences were also examined. Results. Among 10569 cases of congenital heart surgery identified, median total hospital charges were

Impact of Cardiac Rehabilitation on the Exercise Function of Children With Serious Congenital Heart Disease

53828. Statewide differences in the number of high resource use admissions were present; California, Colorado, Florida, Hawaii, Pennsylvania, and Texas were more likely to have high resource use cases, and Maine and South Carolina were less likely. Subsequent analyses were performed adjusting for baseline state effects. Multivariate analyses using generalized estimating equations models revealed Risk Adjustment for Congenital Heart Surgery risk category (odds ratio [OR]: 1.66–14.1), age (OR: 3.81), prematurity (OR: 4.85), the presence of other major noncardiac structural anomalies (OR: 2.53), Medicaid insurance (OR: 1.48), and admission during a weekend (OR: 1.62) to be independent predictors of a higher odds of high cost cases. Although some institutional differences were noted in univariate analyses, gender, race, bed size, teaching and children’s hospital status, hospital ownership, and hospital volume of cardiac cases were not independently associated with greater odds of high resource utilization. Conclusions. States varied in the frequency of high resource utilization for congenital heart surgery. Patients who had greater disease complexity, younger age, prematurity, other anomalies, and Medicaid and were admitted during a weekend were more likely to result in high resource utilization. Institutions of various types did not differ in high cost admissions, regardless of children’s hospital or teaching status.

Adverse event rates in congenital cardiac catheterization — A multi-center experience†

Objectives. The exercise capacity of children with congenital heart disease (CHD) is often depressed. This depression is thought to be attributable to (1) residual hemodynamic defects and (2) deconditioning secondary to physical inactivity. We hypothesized that this latter component would be ameliorated by a formal cardiac rehabilitation program designed specifically for children. The objective of this study was to characterize the effect of a cardiac rehabilitation program on the exercise performance of children with CHD and to define the physiologic mechanisms that might account for any improvements that are observed. Methods. Nineteen patients with CHD who were referred for exercise testing and found to have a peak oxygen consumption (VO2) and/or peak work rate <80% of predicted were enrolled in the study. Sixteen patients (11 Fontan patients, 5 with other CHD) completed the program and had postrehabilitation exercise tests, results of which were compared with the prerehabilitation studies. Results. Improvements were found in 15 of 16 patients. Peak VO2 rose from 26.4 ± 9.1 to 30.7 ± 9.2 mL/kg per min; peak work rate from 93 ± 32 to 106 ± 34 W, and the ventilatory anaerobic threshold from 14.2 ± 4.8 to 17.4 ± 4.5 mL/kg per min. The peak heart rate and peak respiratory exchange ratio did not change, suggesting that the improvements were not attributable merely to an increased effort. In contrast, the peak oxygen pulse rose significantly, from 7.6 ± 2.8 to 9.7 ± 4.1 mL/beat, an improvement that can be attributed only to an increase in stroke volume and/or oxygen extraction at peak exercise. No patient experienced rehabilitation-related complications. Conclusion. Cardiac rehabilitation can improve the exercise performance of children with CHD. This improvement is mediated by an increase in stroke volume and/or oxygen extraction during exercise. Routine use of formal cardiac rehabilitation may greatly reduce the morbidity of complex CHD.

Relationship of Surgical Approach to Neurodevelopmental Outcomes in Hypoplastic Left Heart Syndrome

Objectives: To describe case mix variation among institutions, and report adverse event rates in congenital cardiac catheterization by case type. Background: Reported adverse event rates for patients with congenital heart disease undergoing cardiac catheterization vary considerably, due to non‐comparable standards of data inclusion, and highly variable case mix. Methods: The Congenital Cardiac Catheterization Outcomes Project (C3PO) has been capturing case characteristics and adverse events (AE) for all cardiac catheterizations performed at six pediatric institutions. Validity and completeness of data were independently audited. Results: Between 2/1/07 and 4/30/08, 3855 cases (670 biopsy, 1037 diagnostic, and 2148 interventional) were recorded, median number of cases per site 480 (308 to 1526). General anesthesia was used in 70% of cases (28 to 99%), and 22% of cases (15 to 26%) were non‐electively or emergently performed. Three institutions performed a higher proportion of interventions during a case, 72 to 77% compared to 56 to 58%. The median rate of AE reported per institution was 16%, ranging from 5 to 18%. For interventional cases the median rate of AE reported per institution was 19% (7 to 25%) compared to 10% for diagnostic cases (6 to 16%). The incidence of AE was significantly higher for interventional compared to diagnostic cases (20% vs 10%, p<0.001), as was the incidence of higher severity AE (9% vs 5%, p<0.001). Adverse events in biopsy cases were uncommon. Conclusions: In this multi‐institutional cohort, the incidence of AE is higher among interventional compared to diagnostic cases, and is very low among biopsy cases. Equitable comparisons among institutions will require the development and application of risk adjustment methods.

Intra-atrial reentrant tachycardia after palliation of congenital heart disease: Characterization of multiple macroreentrant circuits using fluoroscopically based three-dimensional endocardial mapping

OBJECTIVE. Two strategies for surgical management are used for infants with hypoplastic left heart syndrome (HLHS), primary heart transplantation and the Norwood procedure. We sought to determine how these 2 surgical approaches influence neurodevelopmental outcomes at school age. METHODS. A multicenter, cross-sectional study of neurodevelopmental outcomes among school-aged children (>8 years of age) with HLHS was undertaken between July 2003 and September 2004. Four centers enrolled 48 subjects, of whom 47 completed neuropsychologic testing. Twenty-six subjects (55%) had undergone the Norwood procedure and 21 (45%) had undergone transplantation, with an intention-to-treat analysis. The mean age at testing was 12.4 ± 2.5 years. Evaluations included the Wechsler Abbreviated Scale of Intelligence, Clinical Evaluation of Language Fundamentals, Wechsler Individual Achievement Test, and Beery-Buktenica Developmental Test of Visual-Motor Integration. RESULTS. The mean neurocognitive test results were significantly below population normative values. The mean full-scale IQ for the entire cohort was 86 ± 14. In a multivariate model, there was no association of surgical strategy with any measure of developmental outcome. A longer hospital stay, however, was associated significantly with lower verbal, performance, and full-scale IQ scores. Aortic valve atresia was associated with lower math achievement test scores. CONCLUSIONS. Neurodevelopmental deficits are prevalent among school-aged children with HLHS, regardless of surgical approach. Complications that result in prolonged hospitalization at the time of the initial operation are associated with neurodevelopmental status at school age.

Clinical Outcome of Fenestrated Fontan Patients After Closure The First 10 Years

Catheter Mapping of IART. introduction: The anatomic substrate of intra‐atrial reentrant tachycardia (IART) following congenital heart surgery is poorly understood, but is presumed to be different than common atrial flutter.

Databases for assessing the outcomes of the treatment of patients with congenital and paediatric cardiac disease - the perspective of cardiology

BackgroundThe late clinical status of Fontan patients after fenestration closure is unknown. Data are now available on all patients who underwent closure from 1989 to 1999. Methods and ResultsAll patients who underwent catheter closure of a Fontan fenestration were enrolled in either the Clamshell (1989 to 1994) or CardioSEAL (1996 to 1999) regulatory trials. Physiological values obtained at catheterization helped assess the hemodynamic effects of fenestration occlusion. In addition to survival, outcomes assessed included O2 saturations, medication use, significant clinical findings (eg, heart failure, protein-losing enteropathy, or new arrhythmias), and somatic growth. Of 181 patients who underwent closure, 27 had additional significant leaks. The remaining 154 patients constituted the study group. Median time from closure to latest follow-up was 3.4 years (range 0.4 to 10.3 years). Fenestration closure increased O2 saturation 9.4% on average (P <0.001). The numbers of patients receiving digoxin or diuretics decreased at the most recent follow-up compared with baseline (P <0.001), but use of antiarrhythmic agents increased marginally (P =0.05). Height and weight percentiles rose (medians of 2 and 4, respectively;P <0.001). Clinical decompensation during follow-up of 154 patients was rare (4.5%), with 2 deaths, 3 Fontan revisions, and 1 patient each with protein-losing enteropathy and ascites. No other patient developed chronic congestive symptoms; 21 patients developed new arrhythmias, and 2 had a stroke or transient ischemic attack. ConclusionsFenestration closure in Fontan patients was followed by improved oxygenation, reduced need for anticongestive medication, and improved somatic growth at latest follow-up. Death (1.3%) or chronic decompensation (3.2%) was rare.

Survival analysis and risk factors for mortality in transplantation and staged surgery for hypoplastic left heart syndrome.

This review includes a brief discussion, from the perspective of cardiac surgeons, of the rationale for creation and maintenance of multi-institutional databases of outcomes of congenital heart surgery, together with a history of the evolution of such databases, a description of the current state of the art, and a discussion of areas for improvement and future expansion of the concept. Five fundamental areas are reviewed: nomenclature, mechanism of data collection and storage, mechanisms for the evaluation and comparison of the complexity of operations and stratification of risk, mechanisms to ensure the completeness and accuracy of the data, and mechanisms for expansion of the current capabilities of databases to include comparison and sharing of data between medical subspecialties. This review briefly describes several European and North American initiatives related to databases for pediatric and congenital cardiac surgery the Congenital Database of The European Association for Cardio-Thoracic Surgery, the Congenital Database of The Society of Thoracic Surgeons, the Pediatric Cardiac Care Consortium, and the Central Cardiac Audit Database in the United Kingdom. Potential means of approaching the ultimate goal of acquisition of long-term follow-up data, and input of this data over the life of the patient, are also considered.