Pulmonary blood flow in children with univentricular heart and unilateral diaphragmatic paralysis.

OBJECTIVES: Spontaneous breathing has an important effect on pulmonary arterial blood flow in patients with Glenn/Fontan circulation. Unilateral diaphragmatic paralysis (DP) is a frequent complication after heart surgery in congenital heart disease. The aim of this study was to investigate the influence of unilateral DP on blood flow distribution in the pulmonary arteries with Glenn/Fontan circulation. METHODS: Magnetic resonance phase-contrast imaging was used to evaluate stroke volume index (SVI) in the left and right pulmonary arteries in patients with Glenn/Fontan circulation with unilateral DP. Data for 18 patients with univentricular heart and unilateral DP were analysed, 8 in the Glenn stage and 10 in the Fontan stage. Ten patients had right-sided DP, and 8 had left-sided DP. A diaphragmatic plication was performed in 7 patients. The control group consisted of 36 patients with Glenn (n = 16)/Fontan (n = 20) circulation without DP. RESULTS: In both left- and right-sided DP, the SVI to the ipsilateral side was significantly lower than in controls [2.81 (1.45-4.50) ml/m2 left vs 11.97 (7.36-16.37) ml/m2 in controls, P < 0.0002; 8.2 (4.49-12.64) ml/m2 with right vs 12.64 (9.66-16.61) ml/m2 in controls; P = 0.0284]. The SVI to the contralateral side showed a slight but non-significant increase in the presence of unilateral DP. CONCLUSIONS: Unilateral DP in patients with Glenn/Fontan circulation has a negative impact on pulmonary arterial SVI on the side of the paralysis.

Stenting of Native Right Ventricular Outflow Tract Obstructions in Symptomatic Infants.

OBJECTIVE: To assess feasibility, safety and effectiveness of right ventricular outflow tract (RVOT) stenting in symptomatic young infants. METHODS: Multicentre evaluation of 35 patients intended to undergo RVOT stenting in 11 pediatric cardiac centres from 2009 to August 2011. RESULTS: Median age and weight at the time of first stent implantation were 8 weeks and 3.3 kg, with 40% of patients <3 kg. A total of 19 patients had suffered from hypoxemic spells, 8 patients were ventilated, 6 on inotropic support and 5 on prostaglandin infusion. Severe concomitant malformations were present in 11 patients, and acute infections in 2. Stenting of the RVOT was successfully performed in 33 patients, improving oxygen saturation from a median of 77 to 90% 2 days after intervention. Besides the 2 patients in whom RVOT stenting was not successful for technical reasons, there were no procedural complications. In 17 of 33 patients, 1-3 reinterventions were performed during follow-up, less than half of those were reinterventions in the RVOT. A total of 27 patients have undergone successful surgical repair 4-162 (median 19.5) weeks after initial RVOT stent implantation, 2 patients are still waiting. There were no perioperative deaths. CONCLUSIONS: Stenting of the RVOT provides a safe and effective management strategy for initial palliation in symptomatic young infants, including those patients not suitable or at higher risk for surgical therapy.

Telemonitoring with implantable electronic devices in young patients with congenital heart diseases.

AIMS: In paediatric patients with a pacemaker (PM) or an implantable cardioverter defibrillator (ICD) device, interrogation during in-clinic visits is usually required to obtain information on arrhythmias or system failures. An automated telemonitoring system tracking patient- and system-related parameters provides this information on a daily basis and might assist patient management. METHODS AND RESULTS: This retrospective analysis evaluates telemetric data obtained from 48 devices implanted in 45 patients (median age, 12.4 years; range, 5 weeks to 37.6 years) using an automated system [Home Monitoring (HM)] over the last 5 years. Regular transmissions were received on 72% of all days. The event messages and monitoring data of 34 (71%) devices induced system revisions, electrophysiological studies including ablation, programming changes, medication changes, and alterations in sporting activity. Approximately 47% of event messages and 50% of emergency messages arrived within 4 weeks of implantation or latest outpatient visit; 113 (17%) emergency messages reported acute changes in lead parameters or tachycardia, which required medical intervention. In four ICD patients, delivered shocks were unreported and only detected by HM. CONCLUSIONS: An automated telemonitoring system reporting patient- and system-related parameters, generated within the patient's home surrounding, can improve the safety and quality of PM and ICD therapy, especially in children, by allowing early detection of system failure and changes in arrhythmic events.

Vascular interventions in young patients undergoing transvenous pacemaker revision.

AIMS: Transvenous lead implantation for pacemaker (PM) or implantable cardioverter defibrillators systems in children and young patients with congenital heart disease is widely practiced. With longer implantation times, the risk for vascular occlusion increases. Transvenous angioplasty may be used to maintain venous patency for system revision in the future. Retrospective analysis of the interventional techniques employed in our young patients undergoing PM revision may identify its clinical benefit. METHODS AND RESULTS: Between May 2005 and August 2010, 28 procedures to maintain central venous patency were performed in 24 patients. Median age was 14.3 years (range, 3.6-29.5 years). The median lead age at time of intervention was 6.8 years (range, 8 days-21.5 years). Balloon dilation of the stenotic vessels was performed with and without prior lead removal. Revascularization with stent implantation was successfully attempted in all clinically relevant obstructions. All PM systems retained full function or were upgraded as planned. There were no major complications and acute surgical referral was not required. CONCLUSION: Angioplasty techniques may be used to maintain and increase the longevity of transvenous pacing pathways in young patients.

Lead removal in young patients in view of lifelong pacing.

AIMS: In young patients with or without a congenital heart disease, transvenous leads for pacemakers or implantable cardioverter defibrillators can cause later vascular obstruction or infection. Removal of non-functional leads is controversial as it bears the risk of vascular disrupture and embolizations. We report the data of a single centre for paediatric cardiology on efficiency and safety of transvenous lead removal. METHODS AND RESULTS: Between May 2005 and August 2009 in 22 patients with a mean age of 12.9 years (range: 3.6-29.5 years) removal of 28 transvenous leads (mean lead age: 5.1 years) was attempted. The main indications for removal were vascular obstruction, increased threshold, and lead dislocation. Commercially available retraction tools were used, if necessary. Twenty-five leads (89%) were retrieved with clinical success, of which 22 (79%) were removed with complete procedural success. In three leads the lead tips were retained, while three leads could not be retrieved. No major complications occurred. Additional interventions such as recanalization, balloon dilation, or stent implantation were performed as indicated. Procedure and X-ray times could be correlated to the implant age of the leads. CONCLUSION: Using non-electrical techniques, transvenous lead removal can be performed with a success rate of 89% in young patients. In the case of vessel obstructions, lead replacement combined with revascularization should be performed early, as the older the lead, the more prolonged and more hazardous the extraction procedure becomes. The use of new leads and precautionary implantation techniques may facilitate later lead removal.

Transvenous cardiac resynchronization therapy in complex congenital heart diseases: dextrocardia with transposition of the great arteries after Mustard operation.

Cardiac resynchronization therapy revealed first promising results in patients with a congenital heart disease and a systemic right ventricle. Contrast-enhanced magnetic resonance imaging showed accessibility of the coronary sinus in an 18-year-old male patient with mirror dextrocardia, d-transposition of the great arteries and ventricular septal defect (VSD) after Mustard operation and VSD patch closure. In literatures, transvenous lead placement is discussed in this anatomical setting, with opposed position of the ventricular leads and reliable lead characteristics.

New trends in chromosomal investigation in children with cardiovascular malformations.

We investigated a group of 376 children, seen over a period of 7 years with different types of congenital cardiovascular defects, to assess the presence of chromosomal aberrations. The diagnostic approach, achieved in 3 consecutive steps, revealed conventional chromosomal aberrations in 30 of the patients (8%) excluding trisomies 13, 18, 21. Fluorescence in situ hybridisation for microdeletions showed 51 microdeletions (15%), with 43 patients having deletions of 22q11.2, 7 patients with deletion of 7q11.23, and 1 patient with deletion of 4p16.3. In 23 patients with additional clinical abnormalities, we carried out a subtelomeric screening. This revealed, in two cases (9%), different subtelomeric aberrations, namely deletions of 1p and of 1q. Thus, subtelomeric screening proved to be a very valuable as a new diagnostic approach. Our approach to genetic investigation in three phases makes it possible to detect a high rate of pathologic karyotypes in patients with congenital cardiovascular malformations, thus guaranteeing more effective genetic counselling of the families, and a more precise prognosis for the patient.

[A doctor's reflections on the breast ornament of the Artemis Ephesia]. / Gedanken eines Mediziniers zum Brustschmuck der Artemis von Ephesos.

Up to now, the discussion concerning the icongraphical identification of the mysterious ornamental dress of the breasts of the Ephesian Artemis a new approach of interpretation has been sought, a medically-diagnostic examination of this very likely polymastic form. Basing on the opinion of Christian apologists, the Ephesia to be a sample of pathologically morphological deformation with the pagan anthropomorphous idea of deities, the process of development of iconographic symbols, to be exact, the "macromasty" and the "pubic triangle", has been traced, beginning with the palaeolithic idols, coming futhermore to the old Anatolian mother deity and finally the Ephesia. In the Artemision, votive gifts where found, breastshaped and trigonal forms, inter alia made of amber. They represent please or the gratefulness of women with gynaecological disorders, directed to the goddess of fertility and childbirth. The question arises, whether we find here the reason for the connection between the amazons, having a unilateral amastia, and the Ephesia. The above-mentioned ornamental dress of the breasts may, besides other indicators and missing mamilla and areola, be token of the pathomorphological finding of polymastia glandularis, so that the main reason of those who deny a "multimammia Ephesia", can be proved wrong. It should be mentioned that the polymastic attribute of the deity may also be proved through parallel cases, shown by phenomenology of religion.