Effect of QT interval-prolonging drugs taken in pregnancy on the neonatal QT interval.

Introduction: Acquired QT interval prolongations due to drug side effects can result in detrimental arrhythmia. Maternal use of placenta-permeable drugs may lead to fetal exposure, thus leading to an increased risk of neonatal QT prolongation and arrhythmia. Objectives: This study aimed to evaluate the influence of maternal QT-prolonging medication on the neonatal QT interval. Methods: In the prospective KUNO-Kids health study, an ongoing population-based birth cohort, we classified maternal medications according to the known risk of QT interval prolongation. Effects on the neonatal QT interval were tested by linear regression analyses, correcting for perinatal confounders (birth weight, gestational age, birth mode, and age at ECG recording). Subgroup analyses were performed for selective serotonin reuptake inhibitors, proton pump inhibitors, and antihistamine dimenhydrinate. Logistic regression analysis was performed using a QTc of 450 ms as the cut-off value. Results: A total of 2,550 pregnant women received a total of 3,990 medications, of which 315 were known to increase the risk of QT prolongation, resulting in 105 (4.1%) neonates exposed in the last month of pregnancy. Overall, the mean age of the neonates at ECG was 1.9 days and the mean QTc (Bazett) was 414 ms. Univariate (regression coefficient -2.62, p = 0.288) and multivariate (regression coefficient -3.55, p = 0.146) regression analyses showed no significant effect of fetal medication exposure on the neonatal QT interval, neither in the overall nor in the subgroup analysis. Logistic regression analysis showed no association of exposure to maternal medication with an increased risk of neonatal QT interval prolongation (OR (odds ratio) 0.34, p = 0.14). Conclusion: The currently used maternal medication results in a relevant number of fetuses exposed to QT interval-prolonging drugs. In our cohort, exposure was found to have no effect on the neonatal QT interval.

Coronary Artery Z-scores in Febrile Children with Suspected Kawasaki's Disease-The Value of Serial Echocardiography.

BACKGROUND: Progressive enlargement of the coronary artery (CA) diameters on serial echocardiography can support diagnosis of Kawasaki's disease (KD) even CA dimensions are within the normal range. METHODS: A single-center, retrospective study compared mean Z-scores of the proximal CA internal diameters in children hospitalized with non-KD febrile illnesses (FCs) with those of KD patients. RESULTS: A total of 223 patients with suspicion of KD have been admitted over a period of 16 years and data were evaluable for 176 children. Distributions for age, sex, and body surface area were similar for both groups. FC had a significantly shorter duration of hospitalization, higher levels of hemoglobin, lower levels of liver transaminases, and segmented neutrophils, respectively. The majority of FC patients (75/82, 91.5%) had normal CA Z-scores (p < 0.001) and only 3 (3.7%) had CA Z-score ≥2.5 standard deviation (SD). In KD, subjects (46/94, 49.5%) had a CA dilation (Z-score ≥2.5 SD) and the maximum CA Z-score (Zmax) was significantly higher compared with FC patients (p < 0.001). On serial echocardiograms, FC patients showed a mild decrease, whereas KD patients developed a significant increase of CA Zmax (p < 0.001). Seven KD patients had a segmental dilation of a CA which has been confirmed by cardiac catheter. In FC, no segmental dilation of any CA was documented by echocardiography. CONCLUSION: This study found that mean CA dimensions in FCs were smaller and did not increase in serial echocardiograms compared with KD patients.

Value of serial echocardiography in diagnosing Kawasaki's disease.

Kawasaki disease (KD) is an acute vasculitis predominantly affecting the small arteries of young children. Up to 25% of untreated patients suffer from coronary artery (CA) complications. Early diagnosis and treatment is mandatory in incomplete KD to reduce the risk of coronary involvement. Between 2002 and 2018, 124 patients have been diagnosed suffering from KD at the University Children's Hospital Regensburg (KUNO). We assessed luminal diameters of both CAs normalized as Z-scores by 2D-echocardiography. A total of 94 patients were analyzed. Of them, 31 (33%) were affected by an incomplete form of KD. In 24 children (26%), serial echocardiography was necessary in order to confirm diagnosis. Mean Z-scores for the left main coronary artery (LMCA), right main coronary artery (RMCA), and left anterior descending artery increased significantly between the initial (LMCA 0.79z, RMCA 0.15z, LAD 0.49z) and second (LMCA 1.69z, RMCA 0.99z, LAD 1.69z) examination (p < 0.05).Conclusion:To confirm diagnosis of KD, it might not be necessary to detect dilation or aneurysms. Our observation suggests that patients suspected having KD should be monitored with serial echocardiography in order to detect a possible enlargement of the CA diameters, even if Z-scores are within the normal range. What is Known: • Kawasaki disease (KD) is an acute vasculitis predominantly affecting the small arteries of young children. Up to 25% of untreated patients suffer from coronary artery (CA) complications. • Due to less classic clinical criteria in patients with incomplete KD, the risk for CA pathology is even higher. What is New: • A significant progression of patients' CA Z-scores in serial echocardiographic measurements may be helpful to ensure diagnosis of KD early even if Z-scores are within the normal range. • Twenty-seven patients (90%) with incomplete KD could be diagnosed within 10 days of fever, early enough to prevent significantly higher rates of CA aneurysm.

Coronary artery z score values in adolescent elite male soccer players.

BACKGROUND: With the increased training loads at very early ages in European elite youth soccer, there is an interest to analyse coronary artery remodelling due to high-intensity exercise. DESIGN AND METHODS: Prospective echocardiographic study in 259 adolescent elite male soccer players and 48 matched controls. RESULTS: The mean age was 12.7 ± 0.63 years in soccer players and 12.6 ± 0.7 years in controls (p > 0.05). Soccer players had significant greater indexed left ventricular mass (93 ± 13 g/m2 versus 79 ± 12 g/m2, p = 0.001). Both coronary arteries origin could be identified in every participant. In soccer players, the mean diameter of the left main coronary artery was 3.67 mm (SD ± 0.59) and 2.61 mm (SD ± 0.48) for right main coronary artery. Controls showed smaller mean luminal diameter (left main coronary artery, p = 0.01; right main coronary artery, p = 0.025). In soccer players, a total of 91% (n = 196) and in controls a total of 94% (n = 45) showed left main coronary artery z scores within the normal range: -2.0 to 2.0. In right main coronary artery, a pattern of z score values distribution was comparable (soccer players 94%, n = 202 vs. controls 84%, n = 40). A subgroup of soccer players had supernormal z score values (>2.0 to 2.5) for left main coronary artery (9%, n = 19, p = 0.01) and right main coronary artery (6%, n = 10, p = 0.025), respectively. CONCLUSION: Elite soccer training in early adolescence may be a stimulus strong enough to develop increased coronary arteries diameters. In soccer players, a coronary artery z score >2.0-2.5 might reflect a physiologic response induced by multiannual high-intensity training.

Z-score values of left ventricular dimensions in adolescent elite male soccer players.

Recent studies showed contrasting findings in morphological changes due to competitive soccer in adolescent players (SP). We present a prospective study in 315 consecutive adolescent (10-14 years) male elite SP and 53 healthy matched active controls (CON). All participants underwent a complete transthoracic two-dimensional echocardiography (TTE). The mean age in SP was 12.8 ± 0.65 years compared to 12.6 ± 0.8 years in CON. For all left ventricular (LV) dimensions, mean Z-score values were higher in SP. There was a significant Z-score increase in interventricular septum diastolic diameter (2.47z vs. 1.62z, p < 0.05), left ventricular posterior wall diastolic and systolic diameter (1.15z vs. 0.47z, p < 0.05 and 1.05z vs. - 0.4z, p < 0.05). Athletes had significant greater LV mass indexed for BSA (94 ± 12 g/m2 vs. 81 ± 13 g/m2, p < 0.05). There was no significant difference in LV function or diameters. Conclusion: Our findings suggest that elite soccer training in adolescent male is a type of sport predominantly related to cardiac resistance remodeling. Adolescent SP may develop supernormal left ventricular wall dimensions (+ 2.0 to + 2.5z). If in SP Z-scores, any LV dimension above + 2.5 is measured, primary or secondary cardiomyopathies should be excluded. What is Known: • Morphological cardiac adaptation in response to exercise depends on the type, duration, and intensity of training. • Morphological and functional changes due to competitive sports (athlete's heart) occur even in pre-adolescent athletes. What is New: • Our findings point out that German elite soccer training in adolescent male (10-14 years of age) is a type of sport predominantly related to cardiac resistance remodeling. • If in an adolescent competitive soccer player any LV dimension Z-score value above + 2.5 is measured, a primary or secondary cardiomyopathy should be excluded.

Thyroidal response following iodine excess for cardiac catheterisation and intervention in early infancy.

BACKGROUND: Cardiac catheterisation requiring the use of contrast medium is increasingly utilised in infants with congenital heart disease. Thyroid function in infants is potentially vulnerable to relatively high doses of iodine. METHODS: Single-centre prospective study of 21 patients (10 neonates, 11 infants) exposed to iodine during cardiac catheterisation. Median age was 30days (1-180), median body weight 3.3kg (1.6-7.0). Serum-levels of thyroid-stimulating hormone (TSH), free triiodothyronine (fT3) and free thyroxine (fT4) were measured at baseline before, and at three different times points after intervention. RESULTS: Baseline thyroidal levels were within normal range in all patients. After applying contrast media of median 6.8ml/kg (range 4.5-14.9), we observed a wide range of TSH values: median 5.01µg/l (range 0.59-37.73; reference 0.72-11.0) three to five days after catheterisation. Five of the 10 neonates demonstrated transient TSH elevation consistent with latent hypothyroidism, and a sixth a transient drop in all hormone levels typical of non-thyroidal illness syndrome. However, circulating thyroid hormones remained within normal range, except for two borderline-low fT4 values within the first week after catheterisation that normalised without any medical intervention. CONCLUSION: Systemic iodine exposure during cardiac catherterisation seems to be clinically well tolerated in early infancy. However, exposure to iodine has demonstrable but apparently reversible effects on thyroid hormones during a potentially important developmental period. The implications of this are unclear, but warrant further investigation in larger cohorts.

High-dose flecainide with low-dose ß-blocker therapy in catecholaminergic polymorphic ventricular tachycardia: A case report and review of the literature.

BACKGROUND: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is characterized by recurrent syncopes and sudden cardiac death triggered by sympathetic activation in young individuals without structural heart disease and a normal baseline electrocardiogram. There is reason to question whether the current expert consensus treatment recommendation, maximal tolerated ß-blockade alone or in combination with low-dose flecainide, is the optimal antiarrhythmic treatment strategy in CPVT, as high doses of ß-blockers may eventually lead to adverse side effects and ß-blocker discontinuation. Indeed, ß-blocker non-compliance accounts for around 5% of sudden cardiac deaths in CPVT patients. CASE REPORT: Differing from the current recommendation, we present the first report of a CPVT patient successfully treated with high-dose flecainide and minimal ß-blockade. This combination resulted in complete suppression of ventricular arrhythmias during exercise stress tests and Holter monitoring and was well tolerated without any side effects. We review the current literature on ß-blocker non-compliance-related sudden cardiac death in CPVT, summarize the in vitro and in vivo data on flecainide therapy in CPVT, and discuss the rationale of our antiarrhythmic approach..

Surgical thrombectomy of two left ventricular thrombi in a child with acute myocarditis.

Myocarditis is a potentially life-threatening disease. Although ventricular thrombus formation in myocarditis is rare, it carries the risk of serious complications. We report on a 10-year-old previously healthy girl presenting with 2 large left ventricular thrombi in acute lymphocytic acute myocarditis. No coagulation disorder was found. Her clinical course and mobile thrombi characteristics prompted us to carry out an urgent surgical thrombectomy rather than primary anticoagulation therapy. The patient has recovered well without clinical signs of thromboembolism.

Follow-up of a child with congenital chloride diarrhoea caused by a novel mutation.

UNLABELLED: Congenital chloride diarrhoea (CLD) is a rare autosomal recessive disease with chronic secretory diarrhoea and a need for lifelong salt replacement therapy. We describe a male newborn of consanguineous parents with CLD. Postnatally, frequent watery diarrhoea and electrolyte disturbances were noted from the day 8 of his life. At molecular level, a homozygous mutation was detected in the solute carrier family 26 member A3 gene (SLC26A3), confirming the clinical diagnosis of CLD. CONCLUSION: The relatively late onset of persistent clinical and laboratory signs may demonstrate a new clinical course of CLD. These findings support the need of a tight follow-up and monitoring if such a diagnosis is considered.