The effects of percutaneous branch pulmonary artery interventions in biventricular congenital heart disease: study protocol for a randomized controlled Dutch multicenter interventional trial.

BACKGROUND: Branch pulmonary artery (PA) stenosis is one of the most common indications for percutaneous interventions in patients with transposition of the great arteries (TGA), tetralogy of Fallot (ToF), and truncus arteriosus (TA). However, the effects of percutaneous branch PA interventions on exercise capacity remains largely unknown. In addition, there is no consensus about the optimal timing of the intervention for asymptomatic patients according to international guidelines. This trial aims to identify the effects of percutaneous interventions for branch PA stenosis on exercise capacity in patients with TGA, ToF, and TA. In addition, it aims to assess the effects on RV function and to define early markers for RV adaptation and RV dysfunction to improve timing of these interventions. METHODS: This is a randomized multicenter interventional trial. TGA, ToF, and TA patients ≥ 8 years with a class IIa indication for percutaneous branch PA intervention according to international guidelines are eligible to participate. Patients will be randomized into the intervention group or the control group (conservative management for 6 months). All patients will undergo transthoracic echocardiography, cardiac magnetic resonance (CMR) imaging, and cardiopulmonary exercise testing at baseline, 6 months, and 2-4 years follow-up. Quality of life (QoL) questionnaires will be obtained at baseline, 2 weeks post intervention or a similar range for the control group, and 6 months follow-up. The primary outcome is exercise capacity expressed as maximum oxygen uptake (peak VO2 as percentage of predicted). A total of 56 patients (intervention group n = 28, control group n = 28) is required to demonstrate a 14% increase in maximum oxygen uptake (peak VO2 as percentage of predicted) in the interventional group compared to the control group (power 80%, overall type 1 error controlled at 5%). Secondary outcomes include various parameters for RV systolic function, RV functionality, RV remodeling, procedural success, complications, lung perfusion, and QoL. DISCUSSION: This trial will investigate the effects of percutaneous branch PA interventions on exercise capacity in patients with TGA, ToF, and TA and will identify early markers for RV adaptation and RV dysfunction to improve timing of the interventions. TRIAL REGISTRATION: ClinicalTrials.gov NCT05809310. Registered on March 15, 2023.

Cardiac Function and Serum Biomarkers throughout Staged Fontan Palliation: A Prospective Observational Study.

Fontan patients undergo multiple cardiothoracic surgeries in childhood. Following these procedures, ventricular function is temporarily decreased, and recovers over months. This is presumably related to cardiopulmonary bypass, but this is incompletely understood. Throughout the Fontan palliation, cardiac function is also affected by volume unloading. We aimed to gain insight into the biological processes related to impaired ventricular function and recovery following Fontan palliations using a panel of biomarkers. Furthermore, we described changes in ventricular function across the Fontan palliation due to volume unloading. We performed a prospective multicenter observational study in patients undergoing partial (PCPC) or total cavo-pulmonary connection (TCPC). Patients underwent assessment-including echocardiography and blood sampling-before surgery (T1), at first follow-up (T2), and 1 year after their procedures (T3). Blood samples were analyzed using a biomarker panel (OLINK CVD-III). Ninety-two biomarkers were expressed as principal components (PC) to limit multiple statistical testing. We included 32 PCPC patients aged 7.2 [5.3-10.3] months, and 28 TCPC patients aged 2.7 [2.2-3.8] years. The single ventricular longitudinal strain (SV GLS) temporarily decreased for PCPC patients at T2 (-15.1 ± 5.6 (T1) to -13.5 ± 5.2 (T2) to -17.3 ± 4.5 (T3), p < 0.047 for all differences), but not following TCPC. The serum biomarkers were expressed as 4 PCs. PC1, including biomarkers of cell-cell adhesion, was not related to any patient characteristic. PC2, including biomarkers of superoxide anion regulation, increased at T2. PC3, including biomarkers of cardiovascular development, related to the stage of Fontan palliation. PC4 was of uncertain biological or clinical significance. No PC was found that related to ventricular performance. The SV GLS was temporarily diminished following PCPC, but not following TCPC. Several biomarkers were related to post-operative stress and adaptation to the PCPC or TCPC circulation, but none were related to the outcome.

A Uniform Description of Perioperative Brain MRI Findings in Infants with Severe Congenital Heart Disease: Results of a European Collaboration.

BACKGROUND AND PURPOSE: A uniform description of brain MR imaging findings in infants with severe congenital heart disease to assess risk factors, predict outcome, and compare centers is lacking. Our objective was to uniformly describe the spectrum of perioperative brain MR imaging findings in infants with congenital heart disease. MATERIALS AND METHODS: Prospective observational studies were performed at 3 European centers between 2009 and 2019. Brain MR imaging was performed preoperatively and/or postoperatively in infants with transposition of the great arteries, single-ventricle physiology, or left ventricular outflow tract obstruction undergoing cardiac surgery within the first 6 weeks of life. Brain injury was assessed on T1, T2, DWI, SWI, and MRV. A subsample of images was assessed jointly to reach a consensus. RESULTS: A total of 348 MR imaging scans (180 preoperatively, 168 postoperatively, 146 pre- and postoperatively) were obtained in 202 infants. Preoperative, new postoperative, and cumulative postoperative white matter injury was identified in 25%, 30%, and 36%; arterial ischemic stroke, in 6%, 10%, and 14%; hypoxic-ischemic watershed injury in 2%, 1%, and 1%; intraparenchymal cerebral hemorrhage, in 0%, 4%, and 5%; cerebellar hemorrhage, in 6%, 2%, and 6%; intraventricular hemorrhage, in 14%, 6%, and 13%; subdural hemorrhage, in 29%, 17%, and 29%; and cerebral sinovenous thrombosis, in 0%, 10%, and 10%, respectively. CONCLUSIONS: A broad spectrum of perioperative brain MR imaging findings was found in infants with severe congenital heart disease. We propose an MR imaging protocol including T1-, T2-, diffusion-, and susceptibility-weighted imaging, and MRV to identify ischemic, hemorrhagic, and thrombotic lesions observed in this patient group.

Automatic extraction of the intracranial volume in fetal and neonatal MR scans using convolutional neural networks.

MR images of infants and fetuses allow non-invasive analysis of the brain. Quantitative analysis of brain development requires automatic brain tissue segmentation that is typically preceded by segmentation of the intracranial volume (ICV). Fast changes in the size and morphology of the developing brain, motion artifacts, and large variation in the field of view make ICV segmentation a challenging task. We propose an automatic method for segmentation of the ICV in fetal and neonatal MRI scans. The method was developed and tested with a diverse set of scans regarding image acquisition parameters (i.e. field strength, image acquisition plane, image resolution), infant age (23-45 weeks post menstrual age), and pathology (posthaemorrhagic ventricular dilatation, stroke, asphyxia, and Down syndrome). The results demonstrate that the method achieves accurate segmentation with a Dice coefficient (DC) ranging from 0.98 to 0.99 in neonatal and fetal scans regardless of image acquisition parameters or patient characteristics. Hence, the algorithm provides a generic tool for segmentation of the ICV that may be used as a preprocessing step for brain tissue segmentation in fetal and neonatal brain MR scans.

Validation of aortic valve 4D flow analysis and myocardial deformation by cardiovascular magnetic resonance in patients after the arterial switch operation.

BACKGROUND: Aortic regurgitation (AR) and subclinical left ventricular (LV) dysfunction expressed by myocardial deformation imaging are common in patients with transposition of the great arteries after the arterial switch operation (ASO). Echocardiographic evaluation is often hampered by reduced acoustic window settings. Cardiovascular magnetic resonance (CMR) imaging provides a robust alternative as it allows for comprehensive assessment of degree of AR and LV function. The purpose of this study is to validate CMR based 4-dimensional flow quantification (4D flow) for degree of AR and feature tracking strain measurements for LV deformation assessment in ASO patients. METHODS: A total of 81 ASO patients (median 20.6 years, IQR 13.5-28.4) underwent CMR for 4D and 2D flow analysis. CMR global longitudinal strain (GLS) feature tracking was compared to echocardiographic (echo) speckle tracking. Agreements between and within tests were expressed as intra-class correlation coefficients (ICC). RESULTS: Eleven ASO patients (13.6%) showed AR > 5% by 4D flow, with good correlation to 2D flow assessment (ICC = 0.85). 4D flow stroke volume of the aortic valve demonstrated good agreement to 2D stroke volume over the mitral valve (internal validation, ICC = 0.85) and multi-slice planimetric LV stroke volume (external validation, ICC = 0.95). 2D flow stroke volume showed slightly less, though still good agreement with 4D flow (ICC = 0.78) and planimetric LV stroke volume (ICC = 0.87). GLS by CMR was normal (- 18.8 ± 4.4%) and demonstrated good agreement with GLS and segmental analysis by echocardiographic speckle tracking (GLS = - 17.3 ± 3.1%, ICC of 0.80). CONCLUSIONS: Aortic 4D flow and CMR feature tracking GLS analysis demonstrate good to excellent agreement with 2D flow assessment and echocardiographic speckle tracking, respectively, and can therefore reliably be used for an integrated and comprehensive CMR analysis of aortic valve competence and LV deformation analysis in ASO patients.

Brain and CSF Volumes in Fetuses and Neonates with Antenatal Diagnosis of Critical Congenital Heart Disease: A Longitudinal MRI Study.

BACKGROUND AND PURPOSE: Fetuses and neonates with critical congenital heart disease are at risk of delayed brain development and neurodevelopmental impairments. Our aim was to investigate the association between fetal and neonatal brain volumes and neonatal brain injury in a longitudinally scanned cohort with an antenatal diagnosis of critical congenital heart disease and to relate fetal and neonatal brain volumes to postmenstrual age and type of congenital heart disease. MATERIALS AND METHODS: This was a prospective, longitudinal study including 61 neonates with critical congenital heart disease undergoing surgery with cardiopulmonary bypass <30 days after birth and MR imaging of the brain; antenatally (33 weeks postmenstrual age), neonatal preoperatively (first week), and postoperatively (7 days postoperatively). Twenty-six had 3 MR imaging scans; 61 had at least 1 fetal and/or neonatal MR imaging scan. Volumes (cubic centimeters) were calculated for total brain volume, unmyelinated white matter, cortical gray matter, cerebellum, extracerebral CSF, and ventricular CSF. MR images were reviewed for ischemic brain injury. RESULTS: Total fetal brain volume, cortical gray matter, and unmyelinated white matter positively correlated with preoperative neonatal total brain volume, cortical gray matter, and unmyelinated white matter (r = 0.5-0.58); fetal ventricular CSF and extracerebral CSF correlated with neonatal ventricular CSF and extracerebral CSF (r = 0.64 and 0.82). Fetal cortical gray matter, unmyelinated white matter, and the cerebellum were negatively correlated with neonatal ischemic injury (r = -0.46 to -0.41); fetal extracerebral CSF and ventricular CSF were positively correlated with neonatal ischemic injury (r = 0.40 and 0.23). Unmyelinated white matter:total brain volume ratio decreased with increasing postmenstrual age, with a parallel increase of cortical gray matter:total brain volume and cerebellum:total brain volume. Fetal ventricular CSF:intracranial volume and extracerebral CSF:intracranial volume ratios decreased with increasing postmenstrual age; however, neonatal ventricular CSF:intracranial volume and extracerebral CSF:intracranial volume ratios increased with postmenstrual age. CONCLUSIONS: This study reveals that fetal brain volumes relate to neonatal brain volumes in critical congenital heart disease, with a negative correlation between fetal brain volumes and neonatal ischemic injury. Fetal brain imaging has the potential to provide early neurologic biomarkers.

Predictors and Outcome of Pericardial Effusion After Hematopoietic Stem Cell Transplantation in Children.

Pericardial Effusion (PE) is a potentially life-threatening complication of Hematopoietic Cell Transplantation (HCT). Our study aim was to identify incidence, risk factors, response to treatment, and outcome of PE after pediatric HCT. All patients after HCT at our tertiary center between 2005 and 2010 were included. Endpoints were PE development and overall survival. We analyzed patient factors, HCT details, and complications and used Cox proportional hazard regression modeling to identify predictors for PE. Twelve out of 129 patients (9.3%) developed PE. Multivariate analysis demonstrated that young age at HCT was a predictor for PE: expressed per year increase in age HR = 0.66 (95% CI 0.46-0.95, p = 0.03). PE had no impact on overall mortality of HCT. Mild respiratory symptoms and vomiting were presenting symptoms for PE. Discontinuation of calcineurin inhibitors-with or without pericardiocentesis-was the only effective treatment for PE, in contrast to diuretics or increased immunosuppression. Seven of 12 PE patients had pericardiocentesis, which was safe and effective in all. Pericardial effusion is not rare after HCT, and young age is the only significant risk factor. Calcineurin inhibitor toxicity appears to be the primary cause of PE after HCT, and discontinuation is effective in the reduction of PE. Pericardiocentesis for PE is a safe and effective procedure. Pericardial effusion did not have an impact on survival after HCT.

Imaging of pediatric great vessel stents: Computed tomography or magnetic resonance imaging?

BACKGROUND: Complications might occur after great vessel stent implantation in children. Therefore follow-up using imaging is warranted. PURPOSE: To determine the optimal imaging modality for the assessment of stents used to treat great vessel obstructions in children. MATERIAL AND METHODS: Five different large vessel stents were evaluated in an in-vitro setting. All stents were expanded to the maximal vendor recommended diameter (20mm; n = 4 or 10mm; n = 1), placed in an anthropomorphic chest phantom and imaged with a 256-slice CT-scanner. MRI images were acquired at 1.5T using a multi-slice T2-weighted turbo spin echo, an RF-spoiled three-dimensional T1-weighted Fast Field Echo and a balanced turbo field echo 3D sequence. Two blinded observers assessed stent lumen visibility (measured diameter/true diameter *100%) in the center and at the outlets of the stent. Reproducibility of diameter measurements was evaluated using the intraclass correlation coefficient for reliability and 95% limits of agreement for agreement analysis. RESULTS: Median stent lumen visibility was 88 (IQR 86-90)% with CT for all stents at both the center and outlets. With MRI, the T2-weighted turbo spin echo sequence was preferred which resulted in 82 (78-84%) stent lumen visibility. Interobserver reliability and agreement was good for both CT (ICC 0.997, mean difference -0.51 [-1.07-0.05] mm) and MRI measurements (ICC 0.951, mean difference -0.05 [-2.52 --2.41] mm). CONCLUSION: Good in-stent lumen visibility was achievable in this in-vitro study with both CT and MRI in different great vessel stents. Overall reliability was good with clinical acceptable limits of agreement for both CT and MRI. However, common conditions such as in-stent stenosis and associated aneurysms were not tested in this in-vitro study, limiting the value of the in-vitro study.

Regression and Complications of z-score-Based Giant Aneurysms in a Dutch Cohort of Kawasaki Disease Patients.

Kawasaki disease (KD) is a pediatric vasculitis. Its main complication is the development of coronary artery aneurysms (CAA), with giant CAA at the end of the spectrum. We evaluated regression and event-free rates in a non-Asian cohort of patients with giant CAA using the current z-scores adjusted for body surface area instead of absolute diameters. KD patients with giant CAA (z-score ≥10) visiting our outpatient clinic between January 1999 and September 2015 were included. Patient characteristics and clinical details were extracted from medical records. Regression was defined as all coronary arteries having a z-score of ≤3. A major adverse event was defined as cardiac death, myocardial infarction, cardiogenic shock, or any coronary intervention. Regression-free and event-free rates were calculated using the Kaplan-Meier method. We included 52 patients with giant CAA of which 45 had been monitored since the acute phase. The 1-, 2-, and 5-year regression-free rates were 0.86, 0.78, and 0.65, respectively. The 5-year, 10-year, and 15-year event-free rates were 0.79, 0.75, and 0.65, respectively. Four children, whose CAA would not have been classified as 'giant' based on absolute diameters instead of z-scores, had experienced an event during follow-up. CONCLUSION: We found a high percentage of children in whom the lumen of giant CAA completely normalized. Four children not classified as 'giant' based on absolute diameters with z-scores of ≥10 experienced a cardiac event. Hence, the use of z-scores seems to be justified.

Three-dimensional rotational angiography in children with an aortic coarctation.

BACKGROUND: Children with aortic coarctations (CoA) are increasingly percutaneously treated. Good visualisation of the CoA is mandatory and can be obtained with three-dimensional rotational angiography (3DRA). This study aims to compare the diagnostic and therapeutic additional value of 3DRA with conventional biplane angiography (CA) in children with a CoA. METHODS: Patients undergoing percutaneous treatment of CoA with balloon angioplasty (BA) or stent between 2003 and 2015, were retrospectively reviewed on success rate, complications, radiation and technical settings. Diagnostic quality of CA and 3DRA and additional value of 3DRA were scored. RESULTS: In total, 134 patients underwent 183 catheterisations, 121 CA and 62 3DRA-guided. Median age was 0.52 years in the BA group and 11.19 years in the stent group. 3DRA was superior to CA in displaying the left ventricle (p = 0.008), ascending aorta (p < 0.001), aortic arch (p = 0.005) and coronary arteries (p < 0.001). In the BA group, 3DRA had a significantly higher success rate than CA (100.0 % versus 68.9 %, p = 0.016). All stent interventions were successful. Complication rates did not differ significantly. The median total dose area product did not significantly differ between CA and 3DRA in the BA (27.88 µGym2/kg versus 15.81 µGym2/kg, p = 0.275) or stent group (37.34 µGym2/kg versus 45.24 µGym2/kg, p = 0.090). 3DRA was of additional value in 96.8 % of the interventions. CONCLUSIONS: 3DRA is superior to CA in diagnostic quality and not associated with increased radiation exposure. It provides high additional value in guiding CoA related interventions.

KinCor, a national registry for paediatric patients with congenital and other types of heart disease in the Netherlands: aims, design and interim results.

OBJECTIVE: Studies in children with heart disease have been hampered by a lack of easily identifiable patient groups. Currently, there are few prospective population-based registries covering the entire spectrum of heart disease in children. KinCor is a Dutch national registry for children with heart diseases. This paper presents the aims, design and interim results of the KinCor project. METHODS: All children presenting at a Dutch university medical centre with a diagnosis of heart disease from 2012 onwards were eligible for registration in the KinCor database. Data entry is through a web-based portal. Entry codes have been synchronised with the European Paediatric Cardiac Coding system, allowing coupling with similar databases for adults, such as CONCOR. RESULTS: Between June 2012 and July 2015, 8421 patients were registered (76 % of those eligible). Median age of the patients was 9.8 years, 44.7 % were female; 6782 patients had morphological congenital heart disease. The most prevalent morphological congenital heart defects were ventricular septal defects (18 %), Tetralogy of Fallot (10 %) and transposition of great arteries (9 %). For 42 % of the patients additional diagnoses were registered. Sixty percent of patients had undergone at least one intervention (catheter intervention or surgery). CONCLUSION: The KinCor database has developed into a large registry of data of children with all types of heart disease and continues to grow. This database will provide the opportunity for epidemiological research projects on congenital and other types of heart disease in children. Entry codes are shared with the CONCOR database, which may provide a unique dataset.

Treatment of infantile haemangiomas with atenolol: comparison with a historical propranolol group.

Propranolol, a lipophilic non-selective beta-blocker, has proven to be effective in the treatment of infantile haemangioma (IH). However, several side effects have been reported. Atenolol, a hydrophilic selective beta-1 blocker, could be an alternative and associated with fewer side effects. Thirty consecutive patients with IH were treated with atenolol between June 2010 and May 2011. The therapeutic effect was judged by clinical assessment and quantified by using a visual analogue scale (VAS) and the Haemangioma Activity Score (HAS). Side effects were also evaluated. The atenolol cohort was compared with a previously described cohort of 28 patients treated with propranolol between July 2008 and December 2009. Clinical involution was present in 90% (27/30) of the IH patients treated with atenolol. Mild side effects occurred in 40% (12/30) of these patients and severe side effects occurred in 3% (1/30). Compared with the previously described cohort treated with propranolol, mild side effects occurred in 50% (14/28) and severe side effects in 25% (7/28) of the patients (p=0.04). Quantitative improvement of the IH in the atenolol group (n=27) showed no significant difference in either the VAS score or the HAS compared to the propranolol group (n=24). This study indicates that atenolol is effective in the treatment of IH. Compared with a historical control group treated with propranolol, the effects of atenolol seem to be similar and less frequently associated with severe side effects. Randomised clinical trials are necessary to evaluate the efficacy and safety of atenolol treatment in IH.

Sudden cardiac death after repair of anomalous origin of left coronary artery from right sinus of Valsalva with an interarterial course : Case report and review of the literature.

Anomalous aortic origin of the coronary artery from the opposite sinus with interarterial course (AAOCA) is a rare condition with a high risk of sudden cardiac death (SCD) during or after strenuous exertion. SCD after repair of this anomaly is extremely rare. Here we present a 15-year-old athlete who collapsed on the basketball court in whom an anomalous origin of the left coronary artery from the right sinus of Valsalva with interarterial course (ALCA) was diagnosed. In spite of extensive pre-sport participation testing, SCD occurred shortly after surgical correction. We reviewed the literature to establish an evidence-based recommendation to aid physicians in conducting the optimal pre-sport participation management for the prevention of SCD in patients with a surgically corrected AAOCA/ALCA, especially for those who participate in strenuous exercise. Review of the literature (60 articles with 325 patients) reveals that post-surgical, pre-sport participation testing varies greatly but that mortality after surgical repair is extremely low (1.5 %). In conclusion, SCD can still rarely occur after repair of AAOCA despite extensive pre-sport participation testing. This should raise awareness among physicians treating these patients and raises the question whether or not return-to-play guidelines need to be revised.

Improving surgical outcome following the Norwood procedure.

BACKGROUND: The Norwood procedure consists of three palliative operations, performed in neonates with hypoplastic left heart syndrome. Especially the first stage (Norwood I) is associated with the highest mortality rates in paediatric cardiac surgery (up to 25%). During surgery, the aorta is reconstructed and a systemic-to-pulmonary shunt is applied. Originally the modified Blalock-Taussig shunt was used, but recently the right-ventricle-to-pulmonary-artery shunt is increasingly being employed. We reviewed the results of our operative strategy, where an individualised choice of shunt is made. Furthermore, attempts to reduce interstage mortality (between Norwood I and II) were assessed. METHODS: All neonates who underwent Norwood stage I palliation from August 2004 until November 2010 were included in this retrospective analysis. Mortality rates and management strategies were compared. RESULTS: Thirty-six patients were available for analysis. Overall 30-day mortality was 5.6% (2 patients) and interstage mortality after discharge was 14% (5 patients). In 2006, a novel clinical protocol was introduced, aimed at reduction of mortality during the interstage period. This resulted in reduction of interstage mortality from 23% to 9% (3 of 13 infants, versus 2 of 23), with a cumulative survival of 82% (maximum follow-up 4 years). CONCLUSION: Early surgical results following the Norwood procedure using an individualised shunt choice are favourable.

[Long term effects of Kawasaki disease]. / Langetermijneffecten van de ziekte van Kawasaki.

Kawasaki Disease (KD) is an acute, self-limiting, vasculitis typically occurring in children under the age of five. Less than 5% of children with KD develop coronary aneurysms and require follow-up by a (paediatric) cardiologist. The majority of patients do not receive follow-up care. However, recent data suggest that the inflammation associated with KD has the potential to affect the entire cardiovascular system. Patients with a history of KD may have an increased risk of long-term cardiovascular sequelae. Therefore KD should be considered a cardiovascular risk factor.

[Diagnostic image (281). A girl with fever, a sore throat and a rash]. / Diagnose in beeld (281). Een meisje met koorts, keelpijn en huiduitslag.

Scarlet fever was diagnosed in a girl with high fever, a sore throat and exanthema marginatum.

Potential additional indicators for pacemaker requirement in isolated congenital atrioventricular block.

Low heart rate is the predominantly used indication for pacemaker intervention in patients with isolated congenital atrioventricular block (CAVB). The aim of this study was to compare the difference in heart rates recorded with ECG and Holter monitoring between paced (PM) and nonpaced (NPM) patients with isolated CAVB before pacemaker implantation to identify additional predictors for future PM need. Retrospective evaluation of atrial and ventricular rates (electrocardiography) and minimal and maximal (Holter) heart rates in 129 CAVB patients prior to PM implantation (n = 93) was performed, and results are expressed in V adjusted for age and sex. The average V score for the atrial rate was 0.51 (n = 50) in the PM group and 0.60 (n = 22) in the NPM group (not-significant). The average z score for the ventricular (average) rate was -0.91 (n = 83) in the PM group and -0.93 (n = 33) in the NPM group (not-significant). Minimal heart rate was -0.94 (n = 61) in the PM group and -0.86 (n = 25) in the NPM group (not significant). Maximal heart rate was -0.96 (n = 61) in the PM group and -0.95 (n = 26) in the NPM group (not significant). Initial recordings of the average heart rate and the minimal and maximal heart rate recorded during Holter monitoring do not seem to predict future pacemaker need in patients with CAVB. Studies with exercise stress tests are needed to confirm these findings.

Treatment of fetal heart block with maternal steroid therapy: case report and review of the literature.

The presence of maternal autoantibodies to SS-A/Ro and/or SS-B/La is associated with the development of fetal heart block. There are data suggesting that maternal treatment with steroids might reverse heart block. We report on a pregnancy in a mother with secondary Sjögren syndrome and systemic lupus erythematosus with presence of autoantibodies to SS-A/Ro and SS-B/La, which was complicated by the development of incomplete fetal heart block. Oral dexamethasone treatment could not prevent progression to complete heart block and was associated with a number of complications.A review of the literature revealed 19 studies (including ours) in which 93 cases of fetal heart block were treated with maternal steroid therapy. Complete heart block proved irreversible in all cases; and of 13 fetuses with incomplete heart block which received maternal steroid therapy, three had a reduction in their degree of block and one reverted to sinus rhythm. Maternal steroid therapy, initiated early in pregnancy and potentially preventing the onset of heart block, did not decrease the incidence of heart block in nine studies with 43 cases. Furthermore, the literature review revealed numerous serious side effects of maternal steroid administration during pregnancy. Data on these potential side effects are lacking in the 28 studies discussed in this review. Maternal dexamethasone therapy to prevent or treat fetal heart block remains, in our opinion, a questionable intervention and can as yet not be recommended in the clinical situation.

[Congenital complete atrioventricular block in children: pathogenesis and clinical outcomes]. / Het congenitale complete atrioventriculaire blok bij kinderen: pathogenese en kliniek.

Congenital complete atrioventricular block (CCAVB) is induced by the placental transmission of maternal anti-SS-A/Ro and anti-SS-B/La antibodies during the second trimester of pregnancy where they cause inflammatory injury to the foetal heart. Anti-SS-A/Ro and anti-SS-B/La antibodies can be detected in most mothers of children with CCAVB. However, the chance of an antibody-positive woman giving birth to a child with CCAVB is 1-5% and the chance of this recurring is 16%. In addition to the maternal antibodies, foetal and environmental factors may also play a role in the pathogenesis. CCAVB is associated with high morbidity and mortality during the foetal and neonatal period. Pacemaker implantation is indicated in approximately 60% of these children, after the development of symptoms related to bradycardia, although the timing of this is controversial. The effectiveness of therapeutic intervention in the uterus has yet to be determined. A conservative approach is advisable with respect to the use of corticosteroids.