Parents of Very Young Children with Congenital Heart Defects Report Good Quality of Life for Their Children and Families Regardless of Defect Severity.

The purpose of this study was to investigate parent reports of quality of life for their very young children with congenital heart defects (CHD) and to compare their scores to previously published data. Parents of children 1-3 years old with CHD or innocent heart murmurs completed the Pediatric Quality of Life Inventory (PedsQL) core, cardiac, and family impact modules. Multivariable regression analyses assessed the impact of age, sex, family income, and CHD treatment history (study group) on PedsQL scores. Correlations between family impact and core/cardiac modules were examined. PedsQL scores were compared to healthy norms. 140 parents of young children participated within four study groups: CHD no treatment (n = 44), CHD treatment without bypass (n = 26), CHD treatment with bypass (n = 42) ,and innocent heart murmurs (n = 28). Male sex was associated with higher core (F = 4.16, p = 0.04, σ2 = .03) and cardiac quality of life (F = 4.41, p = .04, σ2 = 0.04). Higher family income was associated with higher family quality of life (F = 8.89, p < .01, σ2 = 0.13). Parents of children with innocent heart murmurs and children with CHD not requiring treatment had higher core quality of life compared to young healthy children. Cardiac-related quality of life scores were associated with family impact (r = 0.68) and core module (r = 0.63) quality of life scores. Parents of very young children with CHD report good quality of life for their children and families. Quality of life exceeds in children with innocent murmurs or CHD not requiring repair. Parents report a lower quality of life among girls, and lower family quality of life is associated with lower family income.

Presentation, diagnosis, and medical management of heart failure in children: Canadian Cardiovascular Society guidelines

Pediatric heart failure (HF) is an important cause of morbidity and mortality in childhood. This article presents guidelines for the recognition, diagnosis, and early medical management of HF in infancy, childhood, and adolescence. The guidelines are intended to assist practitioners in office-based or emergency room practice, who encounter children with undiagnosed heart disease and symptoms of possible HF, rather than those who have already received surgical palliation. The guidelines have been developed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology, and are accompanied by practical Recommendations for their application in the clinical setting, supplemented by online material. This work does not include Recommendations for advanced management involving ventricular assist devices, or other device therapies.

Left pulmonary artery sling: difference in monochorionic monoamniotic twin presentation.

Pulmonary artery sling (PAS) is a rare congenital vascular anomaly where the left pulmonary artery (LPA) originates from the right pulmonary artery and passes between the trachea and esophagus. Limited literature has been published about PAS in twins. This report demonstrates two different outcomes in mono-mono twins presenting with PAS. Patients with PAS can experience sequelae including wheezing, stridor, atelectasis, or emphysema. Vascular rings occur when the anomalous LPA encircles the airway and esophagus, causing direct compression and respiratory distress. In the case of LPA sling, the sling does not encircle the airway and esophagus but acts as a cradle so the airway which is adjacent to the vessel is not directly compressed. Unfortunately, patients with LPA sling may become severely ill due to airway narrowing even when there is no compression of the adjacent airway. This is the more common presentation in PAS and was seen in Twin A whereas direct airway compression was seen in Twin B. PAS usually presents in patients during their first year of life and high clinical suspicion is important for diagnosis. As this case report demonstrates, presentations and outcomes can be variable in mono-mono twins with LPS and as a result, appropriate investigations should be offered with monitoring of respiratory compromise.

Fetal tachyarrhythmia with 1:1 atrioventricular conduction. Adenosine infusion in the umbilical vein as a diagnostic test.

This is the report of a case of fetal tachyarrhythmia with 1:1 atrioventricular conduction detected by pre-natal echocardiography in a fetus at 25-weeks gestation. Adenosine infusion via cordocentesis was performed as a diagnostic test to differentiate between atrioventricular nodal reentrant supraventricular tachyarrhythmia and atrial flutter. After infusion, transient 2:1 atrioventricular dissociation was obtained and the diagnosis of atrial flutter was made. Transplacental therapy with digoxin and amiodarone was then successfully used.

[Fetal supraventricular tachyarrhythmias. Experience of a fetal cardiology referral center]. / Taquiarritmias supraventriculares no feto. Experiência de uma unidade de referência em cardiologia fetal.

PURPOSE: To describe the presentation, diagnosis and treatment of fetal supraventricular tachyarrhythmias in a series of fetuses followed in a tertiary Fetal Cardiology Center. METHODS: Twenty-five fetuses with diagnosis of supraventricular tachyarrhytmia were reported from January 1989 to October 1997, among 3117 pregnant women referred for fetal cardiac evaluation. RESULTS: There were 17 fetuses with the diagnosis of supraventricular tachycardia (SVT) and 8 patients with atrial flutter (AF). Gestational age ranged from 26 to 40 weeks. Twelve patients were hydropic at presentation (6 with SVT and 6AF). Four fetuses with SVT showed structural abnormalities (two with Ebsten's anomaly and two with VSD). All patients were admitted to the Fetal Cardiology Unit for monitoring and treatment. Among 17 fetuses with SVT, twelve showed good response to digoxin administration, but this drug was not useful in any of the patients with flutter. In two patients with SVT and in six with AF, the pregnancy was interrupted to perform post-natal cardioversion. The mortality rate was 3/17 in the SVT group (including 2 patients with ebstein's anomaly and 0/8 in the flutter group) CONCLUSION: Fetal supraventricular tachyarrithmias are rare in the general population. Nevertheless, the fetus may present with severe heart failure and death. Considering the satisfactory therapeutic response, accurate diagnosis and early treatment of these conditions are extremely important.

[Prenatal restriction of the foramen ovale]. / Restrição pré-natal do forame oval.

The parallel arrangement of the fetal intracardiac circulation requires unrestricted flow of blood through the foramen ovale in the atrial septum. Restriction to this flow is a significant cardiac abnormality, with potentially serious sequelae in post-natal life. A wide spectrum of abnormalities has been associated to the restriction of flow through the foramen ovale, including enlargement of right atrium, hypertrophy of right ventricle, enlargement of tricuspid valve annulus, hypoplastic syndrome of left heart, non-immune hydrops fetalis and supraventricular tachycardias. We report the diagnosis and follow-up of a fetus identified in the prenatal period, in whom the restriction of the foramen ovale originated severe tricuspid regurgitation and severe right ventricular hypocontractility, despite the morphologically normal tricuspid valve. After delivery, there was complete resolution of the clinical findings. The paramount importance of complete assessment of interatrial flow in high risk fetuses, allowing for the early detection and intervention, is emphasized.

[Intrauterine and perinatal management of complete atrioventricular block in the fetus]. / Manejo intra-uterino e perinatal do bloqueio atrioventricular total no feto.

PURPOSE: To report the experience of one of the most severe fetal rhythm disturbances, the complete atrioventricular block. METHODS: Descriptive study of 14 fetuses diagnosed and monitored at the Fetal Cardiology Unit, from January, 1991 to August, 1995. RESULTS: Fourteen cases of complete AV block were identified during the study period, with post-conceptional age between 22 and 38 weeks at the moment of diagnosis. Twelve cases were referred to the Fetal Cardiology Unit because of low heart rate during obstetric examination. Eight fetuses showed complete AV block without evidence of structural heart disease; maternal collagen tissue disease was diagnosed in three of these cases. Four neonates were discharged from the hospital, two of them with a permanent pacemaker, one with complete AV block but no pacemaker and the fourth with sinus rhythm. The remaining seven neonates presented with severe heart failure leading to death despite treatment. CONCLUSION: Complete AV block is a rare fetal condition, but carries a high morbidity and mortality. A several treatment techniques have been suggested to improve monitoring and survival of these patients, particularly in utero.