A qualitative study exploring the psychosocial value of weekend camping experiences for children and adolescents with complex heart defects.

BACKGROUND: Children living with complex heart defects (CHD) are likely to have ongoing social, emotional, physical, and health concerns, and are in need of additional psychosocial support. Summer camps can provide therapeutic benefits. Little research exists regarding the value of shorter camping experiences from the perspectives of children with CHD. The aim of our study was to explore what children and adolescents with CHD considered meaningful when attending a therapeutic camping weekend in the company of peers with similar medical diagnoses. METHODS: Engaging a phenomenological approach we used participant generated photography and reflective semi-structured interviews to explore participants' lived experience and value derived from their weekend camping experiences. The study was completed with thirteen participants ranging in age from 9 to 16 years. Interviews were recorded and transcribed verbatim. Data were analysed using Van Manen's guidelines. RESULTS: Three themes reflecting the camp experiences were generated from the data. Meaningful experiences spanned three outcomes which had some overlapping influences: (i) Developing relationships and feeling accepted by peers and counsellors at camp; (ii) Enjoying and learning during the weekend; and (iii) Experiencing the natural and human-built therapeutic environmental features of camp. The camping programme features, inputs, and processes as identified by the participants in contributing to these outcomes are described. CONCLUSION: This qualitative study showed that children living with complex CHD valued the opportunity for participating in weekend camping experiences in the company of peers with similar heart defects. Findings contribute to a better understanding of what programme features and processes were considered meaningful. Given the scarcity of resources to devote to such social support activities, the findings may help professionals to plan effective interventions to maximize benefits during a shorter camping experience.

Plasma norepinephrine levels in infants and children with congestive heart failure.

To characterize the sympathetic nervous system response to congestive heart failure (CHF) in infants and children, plasma catecholamine levels were measured in 102 subjects undergoing routine cardiac catheterization (mean age 3.3 years, range 0.1 to 14.7), including 61 with left-to-right shunts. Plasma norepinephrine levels were significantly higher (p less than 0.0001) in children with CHF than in those without CHF. A highly significant association (p less than 0.0001) was found between the level of plasma norepinephrine and severity of CHF symptoms. This relation was found for CHF secondary to lesions producing a left-to-right shunt and CHF resulting from primary myocardial dysfunction. In congenital lesions with a left-to-right shunt, plasma norepinephrine levels correlated well with size of the shunt (Qp/Qs) (r = 0.75, p less than 0.001) and degree of pulmonary arterial hypertension (r = 0.70, p less than 0.001). Elevation of plasma norepinephrine concentrations in infants and children are seen with severe CHF regardless of its origin.

Relation of symptoms to contractility and defect size in infants with ventricular septal defect.

Forty-two infants with a ventricular septal defect (VSD) (21 asymptomatic and 21 symptomatic) were compared with 17 control infants to determine if symptoms of congestive heart failure (i.e., tachypnea/poor growth) were due to depressed contractility or defect size, or both. Echocardiographic indexes of defect size, left ventricular performance (shortening fraction), preload (left ventricular end-diastolic dimension), afterload (left ventricular end-systolic wall stress) and contractility (the relation between velocity of circumferential fiber shortening and wall stress) were measured. Clinical assessment included measurement of weight and respiratory rate. Pulmonary and systemic blood flow were assessed in 17 symptomatic and 3 asymptomatic patients by cardiac catheterization. Although there was no significant difference in age, the symptomatic group had significantly lower weight (5.5 +/- 2.9 vs 7.3 +/- 2.3 kg, p less than 0.05) and a higher respiratory rate (53 +/- 14 vs 43 +/- 6 breaths/min, p less than 0.05), compared with control subjects. The mean pulmonary to systemic blood flow ratio in the symptomatic group was 2.9:1. Preload indexed for body surface area was significantly higher in the groups with a VSD compared with control subjects (asymptomatic, 8.5 +/- 1.7 cm/m2; symptomatic, 9.1 +/- 1.7 cm/m2; control subjects, 6.8 +/- 1.1 cm/m2; p less than 0.05). Shortening fraction, afterload and contractility were not significantly different among all groups. A defect size greater than 0.5 cm (or defect size indexed for body surface area greater than 1.8 cm/m2) was predictive of the presence of symptoms. It is concluded that contractility is normal in infants with a VSD. Symptoms may be related to pulmonary congestion.(ABSTRACT TRUNCATED AT 250 WORDS)

Effect of digoxin on contractility and symptoms in infants with a large ventricular septal defect.

The effect of digoxin on contractility and symptoms in infants with a large ventricular septal defect (VSD) is controversial. Nineteen infants with symptoms of congestive heart failure due to a VSD were studied with load-independent indexes during 4 study periods: (1) before any medication; (2) while on chronic diuretics; (3) while on both diuretics and digoxin; and (4) while on diuretics alone, to determine if digoxin: (a) increases "contractility" when added to diuretic therapy; and (b) improves symptoms. Symptoms, signs (heart and respiratory rates, and weight gain), shortening fraction, preload (left ventricular end-diastolic dimension), afterload (left ventricular end-systolic wall stress) and contractility were measured at each period. The difference between the measured and predicted velocities of circumferential fiber shortening for the measured left ventricular end-systolic wall stress served as an index of contractility. Eighteen infants also underwent catheterization. Mean pulmonary-to-systemic blood flow ratio was 3:1. When digoxin was added to diuretics, contractility index was significantly greater than in control subjects (0.13 +/- 0.15 vs 0.0 +/- 0.12 circ/s, p = 0.04). When patients were again on diuretics alone (after discontinuation of digoxin), contractility index was no longer different. Symptoms and signs were not significantly improved by either diuretics or digoxin. It is concluded that in infants with a large left-to-right VSD shunt and receiving digoxin and diuretics, contractility index was significantly greater than in control subjects. However, neither diuretics alone nor in combination with digoxin improved symptoms significantly.

Normal fetal cardiac dimensions obtained by perpendicular imaging.

This study provides normal fetal cardiac dimensional data in a large patient group over a wide range of gestational ages. Perpendicular imaging decreased lateral resolution error, resulting in normal values with narrower confidence limits than in prior studies.

Murine monoclonal CD3 antibody (OKT3)-based early rejection prophylaxis in pediatric heart transplantation.

The purpose of this study was to review our experience with the use of OKT3 (a murine monoclonal CD3 antibody) used as immune prophylaxis for pediatric heart transplant recipients. Orthotopic heart transplantation was performed in 18 pediatric patients, 8 girls and 10 boys, ranging in age from 17 days to 17 years. OKT3 therapy was initiated intraoperatively at a dose of approximately 0.2 mg/kg and was administered at a dose of approximately 0.1 to 0.2 mg/kg/day for a period of 11.5 +/- 2.5 days. Daily average OKT3 levels were 1132 +/- 469 ng/ml. Side effects that occurred during OKT3 therapy were fever (59%), diarrhea (24%), headaches (24%), vomiting (18%), encephalopathy (12%), pulmonary edema (6%), and rash (6%). Infections occurred in 24% of patients, all within 6 months of transplantation. In the first year after transplantation, patients experienced 3.4 +/- 2.4 episodes of mild rejection and 1.0 +/- 0.8 episodes of moderate rejection. No patient experienced severe rejection. Five of the surviving 14 patients (36%) have been weaned from chronic steroid therapy, and 42% are being maintained on alternate-day prednisone at a dose of 0.06 +/- 0.02 mg/kg/day. Coronary artery disease developed in three patients; two of whom died. Actuarial survival was 83% at 1 year and 73% at 2 years. This report shows that OKT3 prophylaxis in pediatric heart transplantation can be used with acceptable short-term adverse side effects and overall survival.

When is echocardiography unreliable in patients undergoing catheterization for pediatric cardiovascular disease?

Technologic advances in echocardiography (e.g., better spatial resolution, Doppler, and color flow mapping) have improved our ability to demonstrate anatomy and physiology in previously problematic conditions, precluding catheterization and angiography in some instances. However, diagnostic catheterization remains necessary in other instances. The aims of this study were to determine whether echocardiography alone was sufficient to delineate the anatomic and flow abnormalities in patients subsequently selected to undergo catheterization and, if not, under what circumstances was echocardiography unable to establish the definitive diagnosis. Echocardiograms of 252 infants and children who underwent catheterization during a 14-month interval were analyzed retrospectively to determine whether the echographic assessment was nondiscrepant (group 1) or discrepant (group 2) with the catheterization assessment. Any deviation in the complete accurate assessment constituted a discrepancy; identification of more than one discrepancy in a single patient was possible. Independent variables included patient's age, weight, operative status, use of color flow mapping, echocardiograph operator, and interval between echocardiogram and catheterization. To determine whether the discrepancies were clinically significant, data from patients in group 2 were reviewed independently by three cardiologists to determine whether patient management would have changed given the added data provided by catheterization. Echocardiographic evaluations were discrepant in 155 instances. In 54 of 155 instances (35%), discrepancies were judged to be clinically significant (group 3). Twenty-three of 54 cases (43%) involved extracardiac lesions (i.e., aortic arch, pulmonary arterial, bronchial collateral, and pulmonary venous anomalies), 20 of 54 (37%) involved pressure gradients, 7 of 54 (13%) involved intracardiac lesions, and 4 of 54 (7%) involved coronary arterial lesions.(ABSTRACT TRUNCATED AT 250 WORDS)

[Congenital diseases of the aorta]. / Enfermedades congénitas de la aorta.

Congenital diseases of the aorta may be divided into three main groups. The most common group includes diseases which obstruct blood flow to the distal circulatory system. The second category includes those diseases which obstruct either the trachea or the esophagus. A third category of congenital diseases of the aorta includes abnormalities of the mechanical composition of the aorta. The major clinical manifestations included in each of these groups will be discussed.

Heart murmurs in pediatric patients: when do you refer?

Many normal children have heart murmurs, but most children do not have heart disease. An appropriate history and a properly conducted physical examination can identify children at increased risk for significant heart disease. Pathologic causes of systolic murmurs include atrial and ventricular septal defects, pulmonary or aortic outflow tract abnormalities, and patent ductus arteriosus. An atrial septal defect is often confused with a functional murmur, but the conditions can usually be differentiated based on specific physical findings. Characteristics of pathologic murmurs include a sound level of grade 3 or louder, a diastolic murmur or an increase in intensity when the patient is standing. Most children with any of these findings should be referred to a pediatric cardiologist.

Interactive telecardiology for the evaluation of heart murmurs in children.

OBJECTIVE: To study the accuracy, patient satisfaction, and cost of telecardiographic evaluations of pediatric patients. MATERIALS AND METHODS: Patients referred to a rural pediatric cardiology outreach clinic were examined in person by a pediatric cardiologist. A second pediatric cardiologist who had no knowledge of the findings of face-to-face examination reevaluated the same patients utilizing a 768-Kbps telemedicine system. Any additional testing was performed by personnel who had no knowledge of the face-to-face evaluation. The main outcome measures included the final cardiac diagnosis, frequency of additional tests such as electrocardiography, (ECG) echocardiography (ECHO), and patient satisfaction. RESULTS: The diagnosis was agreed upon in 19 of the 21 patients studied. Two patients with small ventricular septal defects were missed during the telemedicine evaluation. The utilization rates of additional studies for both the face-to-face cardiologist and the telemedicine cardiologist were not significantly different. Patient satisfaction with the telemedicine encounter was good. CONCLUSIONS: Telemedicine appears to be effective and useful for the cardiac evaluation of pediatric patients. In spite of high data-transfer rates, differences between telemedicine and face-to-face patient encounters were observed.

Fatal obliterative coronary vasculitis in Kawasaki disease.

We report a unique case of Kawasaki disease with late sudden death from obliteration of the lumen of the full length of the left anterior descending coronary artery. Sequential echocardiograms showed early uniform coronary dilatation that resolved before sudden death. The implications of obliterative "healing" of coronary ectasia are unknown.

Use of adenosine for diagnosis and treatment of tachyarrhythmias in pediatric patients.

This report reviews our experience with the use of adenosine for diagnosis and treatment of narrow QRS complex tachyarrhythmias in children. All electrocardiograms obtained since the introduction of adenosine for clinical use at one pediatric tertiary care institution during an 18-month period were reviewed, and those patients receiving adenosine were included for study. Of the 24 patients who received adenosine, the median age was 4 years; four neonates were included. Adenosine produced atrioventricular block in 21 (88%) of 24 patients. It terminated the tachyarrhythmia in 11 patients and produced atrioventricular block but did not terminate the tachyarrhythmia in 10 patients. The mechanism of the arrhythmia was known in three patients before adenosine administration. Adenosine was useful in establishing the mechanism of the tachyarrhythmia in 17 of the remaining 18 patients but was not useful in one patient, in whom the arrhythmia was successfully terminated because a good-quality electrocardiogram was not obtained during adenosine administration. Therefore the mechanism of the supraventricular tachycardia was ultimately determined for all patients in whom adenosine successfully produced atrioventricular block and had acceptable electrocardiographic tracings. Side effects were limited and transient. We conclude that adenosine was a safe and effective agent for the pharmacologic treatment of narrow QRS complex tachyarrhythmias in our patients, including those less than 1 year of age. If proper electrocardiographic recordings are performed during adenosine administration, it is also helpful in establishing the cause of the tachyarrhythmia.

Spontaneous closure of isolated secundum atrial septal defects in infants: an echocardiographic study.

The incidence and timing of spontaneous closure of isolated secundum atrial septal defects are unknown. Twenty-nine consecutive infants under 12 months of age with clinical evidence of significant left-to-right shunting through atrial septal defects were evaluated by M-mode and two-dimensional echocardiography (2DE). All had right ventricular hypertrophy by ECG. Right ventricular, right atrial, and left atrial areas were measured and right ventricular and left atrial dimensions were obtained in all patients. Spontaneous closure of the defect in four infants was suggested by normalization of clinical examination and ECG and was documented by 2DE and M-mode echocardiography at 15 to 31 months of age. All of the remaining patients, who had suitable 2DE echocardiograms and who did not spontaneously close their defects, had enlarged right ventricular and right atrial areas. Left atrial areas were normal in the four who closed spontaneously and were large in all but three who did not close. The mean diameter of the defect was similar for all patients. The 14% incidence reported here may underestimate the true incidence of spontaneous closure, since seven patients had surgical closure, all prior to 30 months of age, the oldest age at which spontaneous closure was documented. An atrial septal flap, found in all four patients who closed spontaneously but only in 4 of the 16 patients who did not close spontaneously, may contribute in some way to spontaneous closure. Since spontaneous closure may occur up to 30 months, it seems clear that surgical closure should not be performed before that age unless medical management has failed to control symptoms.

Pinhole radionuclide ventriculography in small infants.

Radionuclide equilibrium gated ventriculograms were performed in 23 infants before and after surgery for congenital heart disease using standard parallel hole general purpose collimation followed by a pinhole collimation technique to magnify images and improve spatial resolution. A second group of nine infants had pinhole collimated gated ventriculography during cardiac catheterization for comparison with cineangiographic ejection fraction. In group I, pinhole collimation gave improved ventricular and atrial separation over general purpose parallel collimation for determination of left ventricular ejection fraction (42 of 42 studies calculable vs 37 of 42) and for right ventricular ejection fraction (36 of 37 studies calculable vs 20 of 37). In patients with transposition, pinhole technique allowed right ventricular ejection determination in nine of nine studies but in only one of nine was right ventricular ejection fraction possible by standard parallel collimation. In group II, the correlation between left ventricular ejection fraction by cineangiography and pinhole radionuclide ventriculography was excellent (r = 0.95). The correlation for right ventricular ejection fraction between cineangiography and pinhole radionuclide ventriculography was 0.82. Pinhole radionuclide ventriculography is a useful, practical clinical tool that can be used to assess ventricular function in small infants in an intensive care or outpatient setting.