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Functional health status in children and adolescents after Fontan: comparison of generic and disease-specific assessments
- Brian W. McCrindle, Victor Zak, Victoria L. Pemberton, Linda M. Lambert, Victoria L. Vetter, Wyman W. Lai, Karen Uzark, Renee Margossian, Andrew M. Atz, Amanda Cook, Jane W. Newburger
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- Journal:
- Cardiology in the Young / Volume 24 / Issue 3 / June 2014
- Published online by Cambridge University Press:
- 10 June 2013, pp. 469-477
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Purpose: The aim of this study was to compare associations between generic versus disease-specific functional health status assessments and patient and clinical characteristics for patients with severe congenital heart disease. Methods: This was a cross-sectional observational study involving 325 single ventricle patients, aged 10–18 years, after Fontan procedure. Enrolled patients underwent a medical history review, laboratory testing, and assessment of the functional health status by completion of the generic Child Report Child Health Questionnaire and the disease-specific Congenital Heart Adolescent and Teenage questionnaire. Correlated conceptually equivalent domains from both questionnaires were identified and their associations with patient and clinical variables were compared. Results: From the generic assessment, patients perceived marginally lower physical functioning (p = 0.05) but greater freedom from bodily pain compared with a normal population (p < 0.001). The equivalent physical functioning/limitations domain of the generic instrument, compared with the disease-specific instrument, had similar associations (higher multi-variable model R2) with medical history variables (R2 = 0.14 versus R2 = 0.12, respectively) and stronger associations with exercise testing variables (R2 = 0.22 versus R2 = 0.06). Similarly, the corresponding freedom from bodily pain/symptoms domains from both questionnaires showed a greater association for the generic instrument with medical history variables (R2 = 0.15 versus R2 = 0.09, respectively) and non-cardiac conditions (R2 = 0.13 versus R2 = 0.06). The associations of each questionnaire with echocardiographic results, cardiac magnetic resonance imaging results, and serum brain natriuretic peptide levels were uniformly weak (R2 range <0.01 to 0.04). Conclusions: Assessment of the physical functional health status using generic and disease-specific instruments yields few differences with regard to associations between conceptually similar domains and patient and clinical characteristics for adolescents after Fontan procedure.
Ventricular tachycardia in infants with structurally normal heart: a benign disorder
- Mark D. Levin, Paul Stephens, Ronn E. Tanel, Victoria L. Vetter, Larry A. Rhodes
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- Journal:
- Cardiology in the Young / Volume 20 / Issue 6 / December 2010
- Published online by Cambridge University Press:
- 20 August 2010, pp. 641-647
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We evaluated the presentation, treatment, and outcome of infants who present with ventricular tachycardia in the first year of life. Seventy-six infants were admitted to our institution with a diagnosis of ventricular tachycardia between January, 1987 and May, 2006. Forty-five infants were excluded from the study because of additional confounding diagnoses including accelerated idioventricular rhythm, Wolff–Parkinson–White syndrome, supraventricular tachycardia with aberrancy, long QT syndrome, cardiac rhabdomyoma, myocarditis, congenital lesions, or incomplete data. The remaining 31 included infants who had a median age at presentation of 1 day, with a range from 1 to 255 days, and a mean ventricular tachycardia rate of 213 beats per minute, with a range from 171 to 280, at presentation. The infants were treated chronically with propranolol (38.7%), amiodarone (12.9%), mexiletine (3.2%), propranolol and mexiletine (9.7%), or propranolol and procainamide (6.5%). The median duration of treatment was 13 months, with a range from 3 to 105 months. Ventricular tachycardia resolved spontaneously in all infants. No patient died, or received catheter ablation or device therapy. Median age at last ventricular tachycardia was 59 days, with a range from 1 to 836 days. Mean follow-up was 45 months, with a range from 5 to 164 months, with a mean ventricular tachycardia-free period of 40 months. Infants with asymptomatic ventricular tachycardia, a structurally normal heart, and no additional electrophysiological diagnosis all had spontaneous resolution of tachycardia. Furthermore, log-rank analysis of the time to ventricular tachycardia resolution showed no difference between children who received chronic outpatient anti-arrhythmic treatment and those who had no such therapy. While indications for therapy cannot be determined from this study, lack of symptoms or myocardial dysfunction suggests that therapy may not be necessary.
Somatic growth failure after the Fontan operation
- Mitchell I. Cohen, David M. Bush, Robert J. Ferry, Jr, Thomas L. Spray, Thomas Moshang, Jr, Gil Wernovsky, Victoria L. Vetter
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- Journal:
- Cardiology in the Young / Volume 10 / Issue 5 / September 2000
- Published online by Cambridge University Press:
- 19 August 2008, pp. 447-457
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Our study was designed to characterize the patterns of growth, in the medium term, of children with functionally univentricular hearts managed with a hemi-Fontan procedure in infancy, followed by a modified Fontan operation in early childhood. Failure of growth is common in patients with congenital cardiac malformations, and may be related to congestive heart failure and hypoxia. Repair of simple lesions appears to reverse the retardation in growth. Palliation of the functionally single ventricular physiology with a staged Fontan operation reduces the adverse effects of hypoxemia and prolonged ventricular volume overload. The impact of this approach on somatic growth is unknown. Retrospectively, we reviewed the parameters of growth of all children with functionally univentricular hearts followed primarily at our institution who had completed a staged construction of the Fontan circulation between January 1990 and December 1995. Measurements were available on all children prior to surgery, and annually for three years following the Fontan operation. Data was obtained on siblings and parents for comparative purposes. The criterions of eligibility for inclusion were satisfied by 65 patients. The mean Z score for weight was-1·5± 1·2 at the time of the hemi-Fontan operation. Weight improved by the time of completion of the Fontan circulation (−0·91 ±0·99), and for the first two years following the Fontan operation, but never normalized. The mean Z scores for height at the hemi-Fontan and Fontan operations were −0·67 ±1·1 and −0·89±1·2 respectively. At most recent follow-up, with a mean age of 6·1 ± 1·3 years, and a mean time from the Fontan operation of 4·4±1·4 years, the mean Z score for height was −1·15 ±1·2, and was significantly less than comparable Z scores for parents and siblings. In our experience, children with functionally univentricular hearts who have been palliated with a Fontan operation are significantly underweight and shorter than the general population and their siblings
Report from the Task Force of the European Society of Cardiology for the interpretation of the neonatal electrocardiogram
- Peter J. Schwartz, Arthur Garson, Thomas Paul, Marco Stramba-Badiale, Victoria L. Vetter, Elisabeth Villain, Christopher Wren
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- Journal:
- Cardiology in the Young / Volume 12 / Issue 6 / December 2002
- Published online by Cambridge University Press:
- 15 August 2006, pp. 592-608
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The prevention of unexpected cardiac events in the young remains elusive. Among them, some are lethal. Early identification of life-threatening arrhythmogenic disorders which often manifest in infancy, childhood or even later, may allow initiation of effective preventive therapies. A large prospective study has indicated that some infants with prolongation of the QT interval in the first week of life died suddenly, and would have previously been labelled as victims of the sudden infant death syndrome. Furthermore, in infants with this diagnosis, post-mortem molecular screening has revealed the presence of the long QT syndrome. As an evolution of this background, some European countries have begun to consider the possibility of introducing in their National Health Services the performance of an electrocardiogram during the first month of life in all newborns, as part of a programme for cardiovascular screening. Most adult cardiologists, however, have no or minimal experience with electrocardiograms recorded in infants. Accordingly, the European Society of Cardiology has instituted a Task Force with the objective of creating guidelines for the interpretation of the neonatal electrocardiogram, focusing on the most clinically relevant abnormalities and on the ensuing options for management and referral. The main objective of the present document is to provide adult cardiologists with a practical approach to neonatal electrocardiography, and paediatricians and neonatologists with a tool that should facilitate medical interaction on cardiologic issues. There are important differences between neonatal and adult electrocardiograms. When a cardiologist examines the electrocardiogram of an apparently normal and healthy infant, the focus has to be on distinguishing between patterns that should cause no alarm, and those that require action or additional investigations. To provide clues for this distinction has been the main objective of the members of this Task Force. Whenever possible or appropriate, we have also suggested steps in management.