Long-Term Incidence of Arrhythmias in Extracardiac Conduit Fontan and Comparison Between Systemic Left and Right Ventricle.

AIMS: The extracardiac conduit-Fontan (ECC) has become the preferred technique for univentricular heart palliation, but there are currently no data on the incidence of long-term arrhythmias. This study investigated the incidence of arrhythmias and relation to single ventricle morphology in the long-term follow-up in ECC. METHODS AND RESULTS: All patients with ECC performed in our Centre between 1987 and 2017 were included (minimum follow-up 5 years). Of 353 consecutive patients, 303 [57.8% males, aging 8-50 (median 20) years at last follow-up] were considered and divided into 2 groups depending on left (194 in Group 1) or right (109 in Group 2) ventricular morphology. Eighty-five (28%) experienced ≥1 arrhythmic complications, with early and late arrhythmias in 17 (5.6%) and 73 (24.1%) patients, respectively. Notably, late bradyarrhythmias occurred after 6 years in 21 (11%) patients in Group 1, and in 15 (13.8%) in Group 2 [p=0.48]. Late tachyarrhythmias occurred in 55 (18.2%) patients after 12 years: 33 (17%) in Group 1 and 22 (20.2%) patients in Group 2 [p=0.5]. Ventricular tachycardias were documented after 12.5 years in 14 (7.2%) patients of Group 1 and 15 (13.8%) of Group 2 [p=0.06] with a higher incidence in Group 2 during the follow-up [p=0.005]. CONCLUSION: ECC is related to a significant arrhythmic risk in the long-term follow-up, higher than previously reported. Bradyarrhythmias occur earlier but are less frequent than tachyarrhythmias. Interestingly, patients with systemic right ventricle have a significantly higher incidence of ventricular tachycardias, especially in very long follow-up.

Health-related quality of life in Italian children and adolescents with congenital heart diseases.

BACKGROUND: Congenital heart disease (CHD) is the most common congenital anomaly at birth, affecting approximately 1% of live births. In recent decades great medical and surgical advances have significantly increased life expectancy, shifting healthcare professionals' and researchers' interests in patients' Quality of Life (QoL). The main aims of our study were to evaluate generic and condition-specific QoL in a group of Italian children and adolescents with CHD and their parents and examine the level of agreement and directional disagreement between child/adolescent and parents reports on generic and condition-specific QoL. METHODS: A cross-sectional study was designed with CHD children and adolescents and their parents referred to the Cardiology Department of "Bambino Gesù" Children's Hospital. The PedsQL scale was used, including generic (PedsQL 4.0) and cardiac-specific modules (PedsQL 3.0) were administered to patients and caregivers. A Kruskal-Wallis test was used to compare generic and cardiac module scores between patients with different ages, CHD diagnoses, and between patients who underwent surgery interventions and/or are currently taking cardiac medications. RESULTS: 498 families were enrolled in this study. On average, patients reported a good level of generic and condition-specific QoL, as well as their mothers and fathers. Children aged between 5-7 years old reported lower generic and cardiac-specific total QoL levels than children aged 8-12 years and adolescents (13-18 years). With regard to the agreement, patient-parent agreement on condition-specific QoL ranged from 25 to 75% while on generic QoL, it ranged from 19 to 76%. The highest percentage of disagreement between parents and children was found in patients aged 5-7 years old, both for condition-specific and generic QoL rates. CONCLUSIONS: Our study contributed to the growing body of knowledge on QoL in CHD, emphasizing the need for these families to receive support from multidisciplinary standardized care, including psychological consultations and support.

Progressive involvement of cardiac conduction system in paediatric patients with Kearns-Sayre syndrome: how to predict occurrence of complete heart block and sudden cardiac death?

AIMS: The aims of this study are to evaluate the progressive involvement of the cardiac conduction system in the Kearn-Sayre syndrome (KSS) and to establish criteria for the prevention of episodes of syncope or sudden cardiac death. METHODS AND RESULTS: This is a prospective monocentric study including KSS patients, with diagnosis based on clinical manifestations, muscle biopsy, and genetic tests, before the age of 18. All patients underwent cardiac screening examination with 12-lead electrocardiogram (ECG), 24-h Holter monitoring, and pacemaker (PM) interrogation twice a year. Fifteen patients (nine males, mean age 16.6 ± 3.9 years) with a sporadic KSS were recruited. All subjects manifested at least one of the intraventricular conduction defects (IVDs): 1 right bundle branch block (RBBB), 2 left anterior fascicular block (LAFB), 11 a bi-fascicular block (RBBB + LAFB), and 1 left posterior fascicular block. Most children with bi-fascicular block developed LAFB before the RBBB (P = 0.0049). In six patients, IVD degenerated into atrioventricular block (AVB). Endocavitary PM was implanted in 11 patients (6 with AVB and 5 with a bi-fascicular block), while an implantable cardioverter-defibrillator only in one patient with a non-sustained ventricular tachycardia. Four died at mean age of 14.7 ± 2.6 years, but none of them suddenly. CONCLUSION: Even a 'simple' ECG can predict the arrhythmic risk and the occurrence of catastrophic events in young patients with KSS. Left anterior fascicular block precedes RBBB in determining the bi-fascicular block and this can predict an inexorable progression of the conduction defects even in a short time. Pacemaker implantation may be indicated in these patients since the first bi-fascicular block manifestation.

Impact of complex congenital heart disease on the prevalence of arterial hypertension after aortic coarctation repair.

OBJECTIVES: This study was designed to evaluate the difference in the prevalence of long-term arterial hypertension among patients with corrected aortic coarctation according to the existence of associated cardiac congenital lesions. METHODS: We identified 235 patients who had undergone surgery for aortic coarctation and classified them into 2 groups: patients with isolated coarctation of the aorta (CoA) and patients with aortic coarctation associated with complex congenital heart disease. Data were retrospectively analysed. RESULTS: There were 148 subjects with isolated CoA and 87 with complex CoA (CoA-c). Patients were defined as hypertensive if they required antihypertensive treatment and/or when blood pressure was above 95th percentile. Patients with isolated aortic coarctation were significantly younger than patients with CoA-c (P < 0.001) and a markedly higher prevalence of arterial hypertension (44% vs 24%) was documented in the isolated coarctation group. The difference in the prevalence of hypertension in the 2 groups was still significant after correcting for differences in age (P < 0.001), confirming that the prevalence of arterial hypertension in patients with CoA-c was half of that of patients with isolated CoA. CONCLUSIONS: We conclude that complex congenital heart disease in patients who have undergone surgical correction for aortic coarctation results in a significantly lower prevalence of late-onset hypertension. Reduced systemic flow and pressure before surgery in patients with CoA-c might be associated with a lower rate of arterial hypertension.

Dendritic cells modification during sublingual immunotherapy in children with allergic symptoms to house dust mites.

BACKGROUND: the importance of dendritic cells (DCs) in the initiation of the Th2-mediated inflammatory response to allergens is well known and more recently it has been proposed that DCs have a pivotal role in maintaining tolerance to allergens. The aim of this study was to investigate whether the success of sublingual immunotherapy (SLIT) in allergic asthma is mediated by the induction of changes of DCs functions. METHODS: ten children with allergic asthma sensitive to house dust mite were studied before and after 12 months of SLIT. Immature DCs were derived from peripheral blood monocytes cultured for 6 days in presence of interleukin (IL)-4 and GM-CSF and stimulated with lipopolysaccharide for the last 24 hours to induce maturation. RESULTS: after 12 months of SLIT, mature DCs derived from SLIT-treated patients showed a statistically significant defect of CD86 up-regulation, an increase of IL-10, and a reduction of IL-12 production. CONCLUSION: SLIT induces changes in DCs functions that might be responsible for an impairment of T cell activation or drive T cells towards a regulatory activity, thus restoring immune tolerance to allergens.