Adrenocortical response in infants undergoing cardiac surgery with cardiopulmonary bypass and circulatory arrest.

OBJECTIVE: To detail changes in adrenocorticotropic hormone (ACTH), cortisol, and aldosterone levels following cardiac surgery and to test the hypothesis that postcardiotomy infants requiring excessively high-dose vasopressor support will demonstrate adrenal insufficiency which will be proportional to cardiopulmonary bypass (CPB)/circulatory arrest times and vasopressor requirements. DESIGN: Prospective observational pilot study. SETTING: A tertiary care pediatric cardiac intensive care unit. PATIENTS: Prospectively enrolled infants were divided into three subgroups: CPB, CPB with deep hypothermic circulatory arrest (DHCA), and control subjects. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A representative patient sample from each surgical group underwent preoperative synthetic ACTH testing. Postoperative serum samples for cortisol, ACTH, and inotrope score (IS) were collected at discrete intervals over 48 hrs along with patient demographics, surgical procedure, and CPB/DHCA times. Fifty-eight patients were classified by subgroup: 31 CPB, 22 DHCA, and 5 controls. Ten patients with DHCA, analyzed separately, received intraoperative steroids. Tested patients demonstrated preoperative adrenal competence. Cortisol peaked within 2 hrs of surgery without differences among groups. ACTH inversely correlated with bypass time in patients with DHCA (p = .03) but not with circulatory arrest time. Peak cortisol level did not correlate with simultaneous IS. Although not noted in any DHCA-steroid patients, nine patients had increased ACTH/cortisol ratios in association with elevated ISs suggesting inadequate adrenal responsiveness to endogenous ACTH. CONCLUSIONS: The majority of infants with congenital heart disease and intact hypothalamic-pituitary-adrenal axes demonstrated an appropriate adrenocortical stress response to cardiac surgery. Peak serum cortisol was unrelated to CPB/DHCA time and did not predict the level of inotrope support. However, a subset of patients with elevated ACTH/cortisol ratios seemed to have a clinical status consistent with adrenal insufficiency and may be a target group for early postoperative steroid therapy.

Implantable cardioverter defibrillator therapy for life-threatening arrhythmias in young patients.

OBJECTIVES: This study examined the indications, efficacy and outcomes of implantable cardioverter defibrillator (ICD) use in the pediatric population. BACKGROUND: ICDs are first-line therapy for adults resuscitated from sudden cardiac death (SCD) or at high risk for life-threatening ventricular arrhythmias. Use of ICDs in children and young adults is infrequent and there are few data regarding this group. METHODS: We abstracted and analyzed data for all patients in whom ICDs were implanted. RESULTS: A total of 38 devices were implanted in 27 patients. Age ranged from 6 to 26 years (mean, 14) and weight ranged from 16 to 124 kg (mean, 47). Diagnoses included long QT syndrome (9), hypertrophic cardiomyopathy [6], repaired congenital heart disease [5];, and idiopathic ventricular tachycardia/fibrillation [4]. Indications comprised resuscitated SCD [15], syncope [9], and life-threatening ventricular arrhythmia [3]. Initial device placement was infraclavicular in 13, abdominal in 13 and intrathoracic in 1. Epicardial leads were used with 5 systems. A single coil lead was used in 17. Seven patients, all previously resuscitated from SCD, experienced 88 appropriate successful discharges. There were 6 inappropriate discharges in 3 patients. Mean time to device replacement was 3.1 years (n = 11). Complications included 2 infected systems, 2 lead dislodgments, 2 lead fractures, 1 post-pericardiotomy syndrome, 1 adverse event with defibrillation threshold (DFT); testing, and 1 patient with psychiatric sequelae. No deaths occurred with implanted ICDs. CONCLUSIONS: These data demonstrate that ICDs provide safe and effective therapy in young patients. The indications for ICDs as primary preventive therapy remain uncertain.

Novel troponin T mutation in familial dilated cardiomyopathy with gender-dependant severity.

Mutations in sarcomeric proteins can lead to either hypertrophic or dilated cardiomyopathy depending on their effects on the structural and functional properties of the contractile unit of the heart. Mutations in cardiac troponin T, which binds the calcium-responsive troponin complex to alpha-tropomyosin, have been shown to result in cardiac hypertrophy or cardiac dilatation and heart failure, depending on the nature of the specific mutation. In this study, we report the identification of a novel cardiac troponin T mutation (A171S) leading to dilated cardiomyopathy and sudden cardiac death. In contrast to prior described mutations, the A171S mutation results in a significant gender difference in the severity of the observed phenotype with adult males (over 20 years of age) demonstrating more severe ventricular dilatation [left ventricular end diastolic dimension (LVEDD) 7.1 vs. 5.1cm; P=0.01, t test] and left ventricular dysfunction [left ventricular shortening fraction (LVSF) 21 vs. 34%; P=0.04, t test] than adult females. The described mutation substitutes a hydrophilic amino acid for a hydrophobic one in a highly conserved domain involved in the interaction between troponin T and alpha-tropomyosin. Interestingly, four previously described mutations within 12 amino acids of A171 lead to a hypertrophic phenotype, suggesting that further characterization of the functional consequences of the A171S mutation may lead to a better understanding of the pathophysiology of DCM and of the functional differences between HCM- and DCM-causing mutations in cardiac troponin T.

Transtelephonic electrocardiographic monitors for evaluation of children and adolescents with suspected arrhythmias.

OBJECTIVE: Patient-activated transtelephonic electrocardiographic event monitors (TTMs) are often used for the evaluation of children and adolescents with suspected arrhythmias. Since their introduction 25 years ago, there has been little inquiry quantifying the usefulness of TTMs for pediatric patients. The objective of this study was to measure the utility of TTMs for children and adolescents with symptoms of a possible cardiac rhythm disturbance. METHODS: Medical records of all patients who received TTMs from C.S. Mott Children's Hospital Electrocardiography Laboratory between February 1, 1993, and October 31, 2000, were reviewed. Patients with pacemakers, known arrhythmias, or age older than 18 years were excluded. Indications for monitoring included palpitations with or without other symptoms (N = 420), chest discomfort (N = 43), and presyncope or syncope (N = 32). RESULTS: A total of 495 studies (patient mean age: 10.2 +/- 4.3 years; range: 0.1-17.9 years; 48% male) met inclusion criteria. Monitoring was performed for 1 to 1021 consecutive days (mean: 103 +/- 97). Fifty-two percent (N = 257) of patients failed to transmit an electrocardiogram while experiencing symptoms. Fewer boys transmitted electrocardiograms (N = 100/238). Of 238 symptomatic patients, 15% (N = 35; mean age: 11.4 +/- 4.7 years; range: 0.1-17.4 years; 51% male) had supraventricular tachycardia (SVT). No other significant arrhythmia that may warrant treatment was identified. All patients with SVT had palpitations. No patients with isolated chest discomfort, presyncope, or syncope had SVT (N = 75). SVT was documented more frequently in patients with postevent (N = 35/464) than loop recorders (N = 0/31). Of those with SVT, 71% (N = 25) and 91% (N = 33) transmitted events within 4 and 16 weeks, respectively. Follow-up for 1 to 108 months (mean: 32 +/- 25; median: 26) in 53% (243 of 460) of patients without SVT uncovered a 3% (N = 7) rate of subsequent SVT detection. The overall sensitivity of the TTM test was 83% (35 of 42) for detection of SVT. The sensitivity of studies theoretically limited to 4 and 16 weeks would be 60% (25 of 42) and 79% (33 of 42), respectively. The negative predictive value of the TTM study was 99% in our patient population. The negative predictive value of tests theoretically limited to 4 and 16 weeks would be 96% and 98%, respectively. TTM studies of 2 weeks' duration were most cost-effective in terms of total diagnostic yield. In contrast, studies of 4 weeks' duration were most cost-efficient for SVT detection. CONCLUSIONS: TTMs are useful for the evaluation of children and adolescents with palpitations but not with isolated chest pain, syncope, or presyncope. In this study, girls were more likely to transmit events. The sensitivity of TTMs for detection of SVT was 83%. The negative predictive value of the TTM test was 99%. Monitoring for longer than 16 weeks did not increase test sensitivity. Studies of 4 weeks' duration proved most cost-effective for SVT detection.