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1.
Fetal Diagn Ther ; : 1-8, 2019 Oct 08.
Artigo em Inglês | MEDLINE | ID: mdl-31593939

RESUMO

OBJECTIVE: To assess the intrauterine course and outcome of fetal cardiac intervention (FCI) in fetuses with critical aortic stenosis (CAS), severe mitral regurgitation (MR), severe left atrial dilatation (LAD), and restrictive foramen ovale (RFO) or intact atrial septum. METHODS: All fetuses with a prenatal diagnosis of CAS, severe MR, severe LAD, and RFO were retrospectively collected in one tertiary center for fetal medicine over a period of 10 years. Video recordings, pre- and postnatal charts were reviewed for cardiac and extracardiac anomalies, intrauterine course, and postnatal outcome. RESULTS: Nineteen fetuses with CAS, severe MR, severe LAD, and RFO were diagnosed in the study period. In 5 cases, FCI was not considered as the parents either opted for expectative management or for termination. In the remaining 14 fetuses, 21 FCI were performed: 14 balloon valvuloplasties, 2 atrioseptostomies, and 5 fetal atrial stent insertions. Seven of 14 fetuses (50%) had fetal hydrops, 5 of 14 fetuses (36%) presented with intact atrial septum. Procedure-related death occurred in 5 fetuses after aortic valvuloplasty or concomitant atrioseptostomy but in none after fetal atrial stenting. Due to progressive hydrops, two terminations of pregnancy were performed. Among the 7 live births, 3 died in the neonatal period. The remaining 4 received single ventricle palliation, 2 following fetal aortic valvuloplasty and 2 after fetal atrial stent insertion. CONCLUSIONS: CAS with severe MR, severe LAD, and RFO has a high overall mortality even in cases undergoing intrauterine intervention. Parameters that accurately predict the intrauterine and postnatal outcome have yet to be defined.

2.
Circ Cardiovasc Imaging ; 12(9): e009047, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31500448

RESUMO

BACKGROUND: The ratios of tricuspid annular plane systolic excursion (TAPSE)/echocardiographically measured systolic pulmonary artery pressure (PASP), fractional area change/invasively measured mean pulmonary artery pressure, right ventricular (RV) area change/end-systolic area, TAPSE/pulmonary artery acceleration time, and stroke volume/end-systolic area have been proposed as surrogates of RV-arterial coupling. The relationship of these surrogates with the gold standard measure of RV-arterial coupling (invasive pressure-volume loop-derived end-systolic/arterial elastance [Ees/Ea] ratio) and RV diastolic stiffness (end-diastolic elastance) in pulmonary hypertension remains incompletely understood. We evaluated the relationship of these surrogates with invasive pressure-volume loop-derived Ees/Ea and end-diastolic elastance in pulmonary hypertension. METHODS: We performed right heart echocardiography and cardiac magnetic resonance imaging 1 day before invasive measurement of pulmonary hemodynamics and single-beat RV pressure-volume loops in 52 patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. The relationships of the proposed surrogates with Ees/Ea and end-diastolic elastance were evaluated by Spearman correlation, multivariate logistic regression, and receiver operating characteristic analyses. Associations with prognosis were evaluated by Kaplan-Meier analysis. RESULTS: TAPSE/PASP, fractional area change/mean pulmonary artery pressure, RV area change/end-systolic area, and stroke volume/end-systolic area but not TAPSE/pulmonary artery acceleration time were correlated with Ees/Ea and end-diastolic elastance. Of the surrogates, only TAPSE/PASP emerged as an independent predictor of Ees/Ea (multivariate odds ratio: 18.6; 95% CI, 0.8-96.1; P=0.08). In receiver operating characteristic analysis, a TAPSE/PASP cutoff of 0.31 mm/mm Hg (sensitivity: 87.5% and specificity: 75.9%) discriminated RV-arterial uncoupling (Ees/Ea <0.805). Patients with TAPSE/PASP <0.31 mm/mm Hg had a significantly worse prognosis than those with higher TAPSE/PASP. CONCLUSIONS: Echocardiographically determined TAPSE/PASP is a straightforward noninvasive measure of RV-arterial coupling and is affected by RV diastolic stiffness in severe pulmonary hypertension. CLINICAL TRIAL REGISTRATION: URL: https://www.clinicaltrials.gov. Unique identifier: NCT03403868.

3.
Am J Obstet Gynecol ; 2019 Sep 11.
Artigo em Inglês | MEDLINE | ID: mdl-31520628

RESUMO

BACKGROUND: Most fetal deaths are unexplained. Long QT syndrome (LQTS) is a genetic disorder of cardiac ion channels. Affected individuals, including fetuses, are predisposed to sudden death. We sought to determine the risk of fetal death in familial LQTS, in which the mother or father carries LQTS genotype. In addition, we assessed whether risk differed if the LQTS genotype was inherited from the mother or father. OBJECTIVE: (s): This was a retrospective review of pregnancies in families with the 3 most common heterozygous pathogenic LQTS genotypes in KCNQ1 (LQT1), KCNH2 (LQT2) or SCN5A (LQT3), which occur in ∼1/2000 individuals. The purpose of our study was to compare pregnancy and birth outcomes in familial LQTS with the normal population and between maternal and paternal carriers of the LQTS genotype. We hypothesized that fetal death before (miscarriage) and after (stillbirths) 20 weeks gestation would be increased in familial LQTS compared to the normal population, and the parent of origin would not affect birth outcomes. STUDY DESIGN: Our study was a multicenter observational case series of 148 pregnancies from 103 families (80 mothers, 23 fathers) with familial LQTS (60 LQT1, 29 LQT2, 14 LQT3) recruited from 11 international centers with expertise in hereditary heart rhythm diseases, pediatric and/or adult electrophysiology, and high-risk pregnancies. Clinical databases from these sites were reviewed for LQTS occurring in men or women of childbearing age (18-40 years). Pregnancy outcomes (livebirth, stillbirth and miscarriage), birthweights and gestational age at delivery were compared between LQTS genotypes and between maternal vs. paternal LQTS-affected status using logistic regression analysis. RESULTS: Most offspring (80%, 118/148) were term live born, and 66% (73/110) had LQTS. Newborns of LQTS mothers were delivered earlier and, when controlling for gestational age, weighed less than newborns of LQTS fathers. Fetal arrhythmias were rarely observed, but stillbirths (fetal death > 20 weeks) were 8 times more frequent in LQTS (4% vs.∼ 0.5%) while miscarriages (fetal death ≤ 20 weeks) were 2 times that of the general population (16% vs. 8%). The likelihood of fetal death was significantly greater with maternal vs. paternal LQTS (24.4% vs. 3.4%, P=0.036). Only 10% of all fetal deaths underwent postmortem LQTS testing; 2 of 3 were positive for the family LQTS genotype. CONCLUSION: (s): This is the first report demonstrating that mothers with LQTS are at increased risk of fetal death and uncovers a previously unreported etiology of stillbirth. Our results suggest that maternal effects of LQTS channelopathy may cause placental or myometrial dysfunction conferring increased susceptibility to fetal death and growth restriction in newborn survivors, regardless of LQTS status.

4.
Fetal Diagn Ther ; : 1-9, 2019 Aug 27.
Artigo em Inglês | MEDLINE | ID: mdl-31454806

RESUMO

PURPOSE: To assess the intrauterine course, the outcome, and to establish a new prenatal echocardiographic scoring system to predict biventricular (BV) versus univentricular (UV) outcome of fetuses with severe pulmonary stenosis or atresia with intact ventricular septum (PSAIVS). METHODS: All cases of PSAIVS diagnosed prenatally over a period of 14years were retrospectively collected in 2 tertiary referral centers. RESULTS: Forty-nine fetuses with PSIVS (n = 11) or PAIVS (n = 38) were identified prenatally. Nineteen (38.8%) fetuses had additional ventriculocoronary connections (VCCs) and 21 (42.9%) fetuses had right ventricular hypoplasia. Four (8.2%) pregnancies were terminated, 2 (4.1%) ended in intrauterine fetal death, 4 (8.2%) in neonatal death, and 5 (10.2%) children died in infancy or childhood, including one case with compassionate care. Thirty-four of 44 (77.3%) fetuses with the intention-to-treat were alive at latest follow-up, 25 (73.5%) with BV, and 9 (26.5%) with UV circulation. Most significant predictive markers of UV circulation were Vmax of tricuspid regurgitation (TR) <2 m/s, right ventricle/left ventricle length ratio ≤0.6, and presence of VCC. A scoring system including these 3 markers had 100% sensitivity and 100% specificity predicting an UV outcome if more than one of these criteria was fulfilled. All 25 liveborn infants that were suitable for BV repair survived, whereas only 9 out of 14 candidates for UV repair survived. None of the 14 fetuses with predicted UV outcome would have met the inclusion criteria for fetal intervention, as 10 of them had VCC and the remaining 4 had absent TR or Vmax <2 m/s. CONCLUSION: The prognosis of prenatally diagnosed PSAIVS is good if BV circulation can be achieved, while postnatal mortality in UV circulation is high within the first 4 months of life. Postnatal outcome can be predicted prenatally with high accuracy using a simple scoring system. This information is mandatory for parental counseling and may be useful in selecting fetuses for intrauterine valvuloplasty.

5.
Pediatr Nephrol ; 34(12): 2591-2600, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31440827

RESUMO

BACKGROUND: Primary hyperoxaluria (PH) is characterized by progressive chronic kidney disease (CKD) and systemic oxalate deposition. Myocardial dysfunction might be present early in the course of the disease. However, this hypothesis has not yet been tested in the PH population. Therefore, we aimed to determine whether strain imaging using two-dimensional speckle tracking echocardiography (2D-STE) might detect subclinical myocardial disease in otherwise asymptomatic PH patients. METHODS: Prospective study of pediatric and adolescent PH patients with preserved LV ejection fraction (LV EF) and without renal replacement therapy. Subjects underwent conventional echocardiography and 2D-STE. Global (GLS) and segmental peak systolic LV longitudinal strain (LS) measurements were obtained. Data were compared with age- and gender-matched controls, and Z-scores were calculated as appropriate. RESULTS: Fifteen PH patients (age 14.1 ± 5.9 years; 13/15 in CKD stages 1-2) were studied. Although LV EF was preserved (63 ± 6%) in patients, GLS was significantly impaired (GLS - 17.1 ± 2.2% vs - 22.4 ± 1.9%, p < 0.001). This was mainly due to decreased LS values in the apical segments (p < 0.05). Echocardiographic indices of ventricular wall thickness were significantly increased in patients compared to controls (all p < 0.03). GLS correlated significantly with Z-scores of diastolic interventricular wall thickness (r = - 0.57, p = 0.025) and moderately with serum creatinine levels (r = 0.53, p = 0.044). No correlation was found between GLS and blood pressure measurements. CONCLUSIONS: Subclinical myocardial disease is already present early in the course of disease in PH patients with preserved LV EF and some degree of renal dysfunction, but without overt systemic oxalosis. Current recommendations to screen only PH patients with advanced CKD for cardiac disease should be revised accordingly.

6.
J Am Soc Echocardiogr ; 32(8): 1036-1045.e9, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31147145

RESUMO

BACKGROUND: Evaluation of left atrial (LA) size and function is important in congenital and acquired pediatric cardiac disease. Real-time three-dimensional echocardiography (3DE) offers noninvasive assessment of cardiac volumes and phasic function independent of geometric assumptions. The aim of this prospective multicenter study was to establish pediatric reference values for LA 3DE volumes and phasic function based on a large cohort of healthy children. METHODS: LA data sets of 432 subjects (0 days-222 months) were analyzed prospectively using a vendor-independent software. LA volumes (maximal [Vmax], minimal [Vmin], and before atrial contraction) as well as phasic function (active and passive emptying fraction [EF]) were assessed. For volumes, sex-specific reference values, percentiles, and z-scores were calculated by the LMS method of Cole and Green. RESULTS: Absolute volumes increased with age and body surface area. Active EF and relative duration of atrial emptying tended to increase with increasing R-R intervals, while passive EF decreased. Reproducibility of volumes was very good (intra- and interobserver variability for Vmax and Vmin (mean bias ± SD, 0.1 ± 0.9 mL and 0.7 ± 2.8 mL). Volumes were well correlated with cardiac magnetic resonance measurements showing known underestimation of volumes by 3DE (mean bias ± SD, Vmax -14.2 ± 14 mL; Vmin -11.5 ± 10 mL). CONCLUSIONS: Pediatric LA volumes and phasic function indices were reproducibly measured by 3DE. The provided pediatric reference values can be the basis for evaluation of the LA by 3DE and contribute to detection of LA dysfunction and follow-up of patients with congenital heart diseases.

7.
J Perinat Med ; 47(3): 354-364, 2019 Apr 24.
Artigo em Inglês | MEDLINE | ID: mdl-30676006

RESUMO

Objective To assess the spectrum of associated anomalies, the intrauterine course, postnatal outcome and management of fetuses with double outlet right ventricle (DORV). Methods All cases of DORV diagnosed prenatally over a period of 8 years were retrospectively collected in a single tertiary referral center. All additional prenatal findings were assessed and correlated with the outcome. The accuracy of prenatal diagnosis was assessed. Results Forty-six cases of DORV were diagnosed prenatally. The mean gestational age at first diagnosis was 21+4 weeks (range, 13-37). A correct prenatal diagnosis of DORV was made in 96.3% of the cases. If the relation of the great arteries, the position of the ventricular septal defect (VSD) and additional cardiac anomalies are taken into account, the prenatal diagnosis was correct in 92.6% of the cases. One case was postnatally classified as transposition of the great arteries with subpulmonary VSD and was excluded from further analysis. A total of 41 (91.1%) fetuses with DORV had major additional cardiac anomalies, 30 (66.7%) had extracardiac anomalies and 13 (28.9%) had chromosomal or syndromal anomalies. Due to their complex additional anomalies, five (11.1%) of our 45 fetuses had multiple malformations and were highly suspicious for non-chromosomal genetic syndromes, although molecular diagnosis could not be provided. Disorders of laterality occurred in 10 (22.2%) fetuses. There were 17 terminations of pregnancy (37.8%), two (4.4%) intrauterine and seven (15.6%) postnatal deaths. Nineteen of 22 (86.4%) live-born children with an intention to treat were alive at last follow-up. The mean follow-up among survivors was 32 months (range, 2-72). Of 21 children who had already undergone postnatal surgery, eight (38.1%) achieved biventricular repair and 13 (61.9%) received univentricular palliation. One recently born child is still waiting for surgery. All children predicted prenatally to need a single ventricle palliation, and all children predicted to achieve biventricular repair, ultimately received the predicted type of surgery. After surgery, 14 of 18 (77.8%) children were healthy without any impairment. Conclusion DORV is a rare and often complex cardiac anomaly that can be diagnosed prenatally with high precision. DORV is frequently associated with major additional anomalies, leading to a high intrauterine and postnatal loss rate due to terminations or declined postnatal therapy. Without additional anomalies, the prognosis is good, although approximately 60% of children will have single ventricle palliation.


Assuntos
Dupla Via de Saída do Ventrículo Direito/diagnóstico por imagem , Dupla Via de Saída do Ventrículo Direito/epidemiologia , Dupla Via de Saída do Ventrículo Direito/cirurgia , Ecocardiografia , Feminino , Alemanha/epidemiologia , Humanos , Recém-Nascido , Gravidez , Estudos Retrospectivos , Ultrassonografia Pré-Natal
8.
Ultraschall Med ; 2019 Jan 07.
Artigo em Inglês | MEDLINE | ID: mdl-30616264

RESUMO

PURPOSE: To assess the intrauterine course, associated conditions and postnatal outcome of fetuses with pulmonary atresia with ventricular septal defect (PAVSD). METHODS: All cases of PAVSD diagnosed prenatally over a period of 10 years with a minimum follow-up of 6.5 years were retrospectively collected in 3 tertiary referral centers. RESULTS: 50 cases of PAVSD were diagnosed prenatally. 44.0 % of fetuses had isolated PAVSD, 4.0 % had associated cardiac anomalies, 10.0 % had extra-cardiac anomalies, 38.0 % had chromosomal anomalies, 4.0 % had non-chromosomal syndromes. Among the 32 liveborn children, 56.3 % had reverse flow in the patent arterial duct, 25.0 % had major aortopulmonary collateral arteries (MAPCAs) with ductal agenesis and 18.7 % had a double supply. 17 pregnancies were terminated (34.0 %), there was 1 intrauterine fetal death (2.0 %), 1 neonatal death (2.0 %), and 6 deaths (12.0 %) in infancy. 25 of 30 (83.3 %) liveborn children with an intention to treat were alive at the latest follow-up. The mean follow-up among survivors was 10.0 years (range 6.5 - 15.1). 56.0 % of infants underwent staged repair, 44.0 % had one-stage complete repair. After exclusion of infants with additional chromosomal or syndromal anomalies, 88.9 % were healthy, and 11.1 % had mild limitations. The presence of MAPCAs did not differ significantly between survivors and non-survivors (p = 0.360), between one-stage or staged repair (p = 0.656) and healthy and impaired infants (p = 0.319). CONCLUSION: The prognosis in cases without chromosomal or syndromal anomalies is good. MAPCAs did not influence prognosis or postoperative health. The incidence of repeat interventions due to recurrent stenoses is significantly higher after staged compared with single-stage repair.

9.
Orphanet J Rare Dis ; 13(1): 120, 2018 Jul 19.
Artigo em Inglês | MEDLINE | ID: mdl-30025539

RESUMO

BACKGROUND: Mitochondrial acyl-CoA dehydrogenase family member 9 (ACAD9) is essential for the assembly of mitochondrial respiratory chain complex I. Disease causing biallelic variants in ACAD9 have been reported in individuals presenting with lactic acidosis and cardiomyopathy. RESULTS: We describe the genetic, clinical and biochemical findings in a cohort of 70 patients, of whom 29 previously unpublished. We found 34 known and 18 previously unreported variants in ACAD9. No patients harbored biallelic loss of function mutations, indicating that this combination is unlikely to be compatible with life. Causal pathogenic variants were distributed throughout the entire gene, and there was no obvious genotype-phenotype correlation. Most of the patients presented in the first year of life. For this subgroup the survival was poor (50% not surviving the first 2 years) comparing to patients with a later presentation (more than 90% surviving 10 years). The most common clinical findings were cardiomyopathy (85%), muscular weakness (75%) and exercise intolerance (72%). Interestingly, severe intellectual deficits were only reported in one patient and severe developmental delays in four patients. More than 70% of the patients were able to perform the same activities of daily living when compared to peers. CONCLUSIONS: Our data show that riboflavin treatment improves complex I activity in the majority of patient-derived fibroblasts tested. This effect was also reported for most of the treated patients and is mirrored in the survival data. In the patient group with disease-onset below 1 year of age, we observed a statistically-significant better survival for patients treated with riboflavin.

10.
J Am Soc Echocardiogr ; 31(9): 1070-1071, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30025647
11.
J Am Soc Echocardiogr ; 31(9): 1050-1063, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29908725

RESUMO

BACKGROUND: Functional assessment of the right ventricle using real-time three-dimensional echocardiography (RT3DE) has fundamental relevance in young patients with congenital heart disease. Reference values for the pediatric population are scarce. This multicenter study was designed to (1) validate new evaluation software for RT3DE and (2) establish pediatric reference values. METHODS: For validation, right ventricular (RV) end-diastolic volume (EDV) and end-systolic volume (ESV) were determined from real-time three-dimensional echocardiographic data sets of 38 subjects (n = 17 healthy individuals and n = 21 patients with congenital heart disease) using new dedicated evaluation software (RV-Function 2.0) and compared with cardiac magnetic resonance investigations of the same patient cohort. In a prospective multicenter design, 360 real-time three-dimensional echocardiographic data sets of healthy children (172 girls) were analyzed. To create reference centiles, the cohort was subdivided into group I (children <7 years of age, n = 136 [female and male]), group II (girls 7-18 years of age, n = 106), and group III (boys 7-18 years of age, n = 118). RESULTS: Using RT3DE, RV volumes were slightly higher than using cardiac magnetic resonance (EDV, 0.8 ± 5.8% [limits of agreement, -10.8% to 12.5%; r = 0.993]; ESV, 2.0 ± 13.1% [limits of agreement, -24.2% to 28.2%; r = 0.989). Reproducibility was promising (intraobserver variability, 3.9 ± 11.4% for EDV and -1.7 ± 13.4% for ESV [intraclass correlation coefficient range, 0.94-0.98]; interobserver variability, 1.9 ± 11.8% for EDV and -0.3 ± 22.8% for ESV [intraclass correlation coefficient range, 0.85-0.96]). Regarding functional parameters, no significant gender differences were found among children in group I. In contrast, children in groups II and III differed in RV volumes, dimensional parameters, and tricuspid annular plane systolic excursion (P < .005); the children did not differ in deformation parameters. Feasibility was 90%. CONCLUSIONS: RT3DE yields accurate and reproducible RV volumes. The calculated percentile curves may facilitate the clinical use of RT3DE to analyze RV function in children.

12.
Circ Arrhythm Electrophysiol ; 11(4): e005797, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29654130

RESUMO

BACKGROUND: Long-QT syndrome (LQTS), an inherited cardiac repolarization disorder, is an important cause of fetal and neonatal mortality. Detecting LQTS prenatally is challenging. A fetal heart rate (FHR) less than third percentile for gestational age is specific for LQTS, but the sensitivity is only ≈50%. Left ventricular isovolumetric relaxation time (LVIRT) was evaluated as a potential diagnostic marker for fetal LQTS. METHODS AND RESULTS: LV isovolumetric contraction time, LV ejection time, LVIRT, cycle length, and FHR were measured using pulsed Doppler waveforms in fetuses. Time intervals were expressed as percentages of cycle length, and the LV myocardial performance index was calculated. Single measurements were stratified by gestational age and compared between LQTS fetuses and controls. Receiver-operator curves were performed for FHR and normalized LVIRT (N-LVIRT). A linear mixed-effect model including multiple measurements was used to analyze trends in FHR, N-LVIRT, and LV myocardial performance index. There were 33 LQTS fetuses and 469 controls included. In LQTS fetuses, the LVIRT was prolonged in all gestational age groups (P<0.001), as was the N-LVIRT. The best cutoff to diagnose LQTS was N-LVIRT ≥11.3 at ≤20 weeks (92% sensitivity, 70% specificity). Simultaneous analysis of N-LVIRT and FHR improved the sensitivity and specificity for LQTS (area under the curve=0.96; 95% confidence interval, 0.82-1.00 at 21-30 weeks). N-LVIRT, LV myocardial performance index, and FHR trends differed significantly between LQTS fetuses and controls through gestation. CONCLUSIONS: The LVIRT is prolonged in LQTS fetuses. Findings of a prolonged N-LVIRT and sinus bradycardia can improve the prenatal detection of fetal LQTS.

13.
J Am Soc Echocardiogr ; 31(6): 702-711.e13, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29605473

RESUMO

BACKGROUND: Real-time three-dimensional echocardiography (RT3DE) is a promising method for accurate assessment of left ventricular (LV) volumes and function, however, pediatric reference values are scarce. The aim of the study was to establish pediatric percentiles in a large population and to compare the inherent influence of different evaluation software on the resulting measurements. METHODS: In a multicenter prospective-design study, 497 healthy children (ages 1 day to 219 months) underwent RT3DE imaging of the LV (ie33, Philips, Andover, MA). Volume analysis was performed using QLab 9.0 (Philips) and TomTec 4DLV2.7 (vendor-independent; testing high (TomTec75) and low (TomTec30) contour-finding activity). Reference percentiles were computed using Cole's LMS method. In 22 subjects, cardiovascular magnetic resonance imaging (CMR) was used as the reference. RESULTS: A total of 370/497 (74.4%) of the subjects provided adequate data sets. LV volumes had a significant association with age, body size, and gender; therefore, sex-specific percentiles were indexed to body surface area. Intra- and interobserver variability for both workstations was good (relative bias ± SD for end-diastolic volume [EDV] in %: intraobserver: QLab = -0.8 ± 2.4; TomTec30 = -0.7 ± 7.2; TomTec75 = -1.9 ± 6.7; interobserver: QLab = 2.4 ± 7.5; TomTec30 = 1.2 ± 5.1; TomTec75 = 1.3 ± 4.5). Intervendor agreement between QLab and TomTec30 showed larger bias and wider limits of agreement (bias: QLab vs TomTec30: end-systolic volume [ESV] = 0.8% ± 23.6%; EDV = -2.2% ± 17.0%) with notable individual differences in small children. QLab and TomTec underestimated CMR values, with the highest agreement between CMR and QLab. CONCLUSIONS: RT3DE allows reproducible noninvasive assessment of LV volumes and function. However, intertechnique variability is relevant. Therefore, our software-specific percentiles, based on a large pediatric population, serve as a reference for both commonly used quantification programs.

15.
Eur J Pediatr ; 176(9): 1217-1226, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-28730319

RESUMO

Postoperative junctional ectopic tachycardia (JET) is a frequent complication after pediatric cardiac surgery. Current recommendations on how and when to treat JET are inconsistent. We evaluated the management strategies of postoperative JET in German-speaking countries. We sent an online survey to 30 centers of pediatric cardiology that perform surgery for congenital heart defects in Germany (24), Austria (4), and Switzerland (2). The survey asked 18 questions about how and in what treatment sequence postoperative JET was managed. All 30 centers completed the survey (100% return rate). There was general agreement that the management of JET is based on administration of antiarrhythmic drugs, body surface cooling, and temporary pacing. Many centers presented treatment algorithms based on published literature, all centers named amiodarone as the first drug of choice. Significant disagreement was found concerning the timing and sequential order of additional therapeutic measures and particularly about the dosing of amiodarone and the role of R-wave synchronized atrial pacing. CONCLUSION: This survey reveals that from center to center, the treatment of postoperative JET may vary substantially. Future work should focus on those treatment modalities where a high rate of variation is found. Such studies may be of value to achieve commonly adopted treatment recommendations. What is known: • Treatment of postoperative junctional ectopic tachycardia is predominantly based on administration of antiarrhythmic drugs, therapeutic cooling, and temporary pacing. • Amiodarone is the antiarrhythmic drug of choice in this context. What is new: • Dosing and duration of administration of amiodarone differ relevantly from center to center. • The sequential order of drug administration, therapeutic cooling, and pacing is not consistent.


Assuntos
Amiodarona/administração & dosagem , Antiarrítmicos/administração & dosagem , Estimulação Cardíaca Artificial/métodos , Crioterapia/métodos , Taquicardia Ectópica de Junção/terapia , Áustria , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Criança , Alemanha , Pesquisas sobre Serviços de Saúde , Humanos , Lactente , Complicações Pós-Operatórias/terapia , Suíça , Taquicardia Ectópica de Junção/prevenção & controle
16.
J Clin Monit Comput ; 31(2): 353-360, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26886899

RESUMO

To compare stroke volumes (SV) in small hearts assessed by real-time three-dimensional echocardiography (3DE) with SV measured by transpulmonary thermodilution (TPTD) and continuous pulse contour analysis (PC) under various hemodynamic conditions. In thirteen anesthetized piglets (range 3.6-7.1 kg) SV were measured by 3DE, TPTD and PC at baseline and during phenylephrine and esmolol administration. 3DE and TPTD measurements were done successively while SV calculated by PC was documented at the time of 3DE. 3DE and TPTD showed a good correlation (r2 = 0.74) and a bias of -1.3 ml (limits of agreement -4.1 to 1.5 ml). While TPTD measured higher SV than 3DE, both methods tracked SV changes with a concordance rate of 91 %. PC and 3DE showed a lower correlation coefficient of r2 = 0.57 and a bias of -2.1 ml (limits of agreement -5.9 to 1.8 ml). Inter- and intra-observer variability of SV measured by 3DE was good with a mean bias <5 %. SV3DE showed a small variance and tracked acute small changes in SV in acceptable concordance with TPTD. PC measured SV with a higher variance and mean difference compared to 3DE. In an experimental setting 3DE has the possibility to offer non-invasive assessments of ventricular volumes volume changes. To determine whether 3DE could be used for SV assessment in a clinical routine our results need confirmation in a clinical setting.


Assuntos
Ecocardiografia Tridimensional/métodos , Coração/fisiologia , Volume Sistólico , Termodiluição/métodos , Animais , Feminino , Frequência Cardíaca , Hemodinâmica , Modelos Animais , Variações Dependentes do Observador , Pediatria , Fenilefrina/administração & dosagem , Propanolaminas/administração & dosagem , Reprodutibilidade dos Testes , Suínos , Sístole , Fatores de Tempo , Disfunção Ventricular Esquerda , Função Ventricular Esquerda
17.
Ultraschall Med ; 38(4): 427-436, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27248797

RESUMO

Purpose To assess the spectrum of associated anomalies, the intrauterine course, the outcome and possible prognostic markers in prenatally diagnosed Ebstein's anomaly (EA). Materials and Methods All cases of EA diagnosed over a period of 13 years with a minimum follow-up of 1 year were retrospectively collected in 4 tertiary referral centers in Germany. Results In the study period 76 cases of EA were prenatally diagnosed. The mean gestational age at diagnosis was 25.0 weeks (range: 13 - 35). 41 (53.9 %) cases were isolated and 35 (46.1 %) had other cardiac and/or extracardiac anomalies. 19 (25.0 %) pregnant women opted for termination of pregnancy, intrauterine fetal death occurred in 7 cases (9.2 %), neonatal death in 14 cases (18.4 %), death in infancy or childhood in 9 cases (11.8 %) and 27 children (35.5 %) were alive at the last follow-up. After exclusion of terminations, the only parameter inversely correlated with intrauterine survival was hydrops fetalis. Prognostic parameters significantly associated with postnatal non-survival were an abnormal Celermajer index (right atrium/heart ratio > 0.7), cardiomegaly (cardiothoracic circumference ratio > 0.5), absence of antegrade flow over the pulmonary valve and earlier diagnosis in pregnancy. Conclusion Prenatally diagnosed EA has a high morbidity and mortality with the highest loss rate in the intrauterine and neonatal period. In our study, hydrops fetalis was the only parameter significantly associated with intrauterine demise, while other prenatal markers were only significantly associated with postnatal mortality.

18.
Ultraschall Med ; 38(2): 206-211, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26085459

RESUMO

Purpose The rare finding of a levoatrial cardinal vein (LACV) represents a pulmonary-systemic connection providing an alternative egress from the left atrium for pulmonary venous blood in fetal cardiac malformations with severe left heart obstruction and intact atrial septum (IAS). The purpose of the study was the description of the various sonographic and Doppler findings, the peripartal management and neonatal outcome of this rare cardiovascular anomaly. Materials and Methods Retrospective review of 53 967 echocardiograms in our fetal database between 2002 and 2013 for cases with an LACV. The various sonographic findings of the LACV, the associated cardiac findings and the perinatal management were assessed. Results The presence of a decompressing LACV was documented in 5/27 (18.5 %) of all fetuses with hypoplastic left heart syndrome and intact atrial septum. All five fetuses were diagnosed with an LACV originating from the left atrium and draining either into the innominate vein (two cases), the azygos vein (one case), or directly into the vena cava superior (two cases). Elevated pressure in the pulmonary veins was present in the three cases with obstruction of the LACV. Two pregnancies were terminated, two neonates received compassionate care and one neonate died despite atrioseptectomy. Conclusion LACV in the presence of HLHS and IAS is a rare condition with various forms of pathway and degree of obstruction and generally has an unfavorable prognosis. The presence of obstruction can be diagnosed by spectral Doppler of the LACV. Therefore, in cases of HLHS and IAS, careful sonographic evaluation for aberrant vessels is mandatory.


Assuntos
Vasos Coronários/diagnóstico por imagem , Septos Cardíacos/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Ultrassonografia Pré-Natal , Feminino , Humanos , Gravidez , Segundo Trimestre da Gravidez , Terceiro Trimestre da Gravidez , Valores de Referência
19.
Pediatr Cardiol ; 38(2): 215-227, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-27847970

RESUMO

In August 2010, the Nit-Occlud® Lê (EUREVECO) became available for transcatheter coil occlusion of ventricular septal defects (VSDs). Retrospective European Registry for VSD Closure using the Nit-Occlud® Lê-VSD-Coil; analysis of the feasibility, results, safety and follow-up of VSD-closure over a 3-year period in 18 European centers. In 102 of 111 patients (female 66), successful VSD closure was performed (mean age 8.2 years, mean weight 28.82 kg), 81 perimembranous VSDs (48 with aneurysm), 30 muscular VSDs, mean procedure time was 121.1 min, and mean fluoroscopy time was 26.3 min. Short- and midterm term follow-up was possible in 100/102 patients, there was 1 embolization and 1 explantation after 24 months. Immediate complete closure occurred in 49 of 101 patients (48.5%), trivial residual shunt was present in 51 (50.0%), closure rate was 95% after 6 months and 97% after 1 year. Out of the 102 patients, there were 2 severe complications (1.8%) (1 severe hemolysis, 1 embolization) and 8 moderate/transient (=7.2%) including 1 transient AV block. During a mean follow-up period of 31.3 months (range 24-48) and a total follow-up time of 224.75 patient years, no further problems occurred. VSD closure with the Nit-Occlud® Lê VSD coil is feasible and safe with a minimal risk of severe side effects. The long-term effects and safety require further clinical follow-up studies.


Assuntos
Cateterismo Cardíaco/métodos , Fluoroscopia , Comunicação Interventricular/terapia , Hemólise , Dispositivo para Oclusão Septal , Adolescente , Criança , Pré-Escolar , Europa (Continente) , Feminino , Comunicação Interventricular/classificação , Humanos , Lactente , Masculino , Duração da Cirurgia , Sistema de Registros , Estudos Retrospectivos , Resultado do Tratamento
20.
PLoS One ; 11(10): e0165397, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27776179

RESUMO

BACKGROUND: Pressure-volume loops (PVL) provide vital information regarding ventricular performance and pathophysiology in cardiac disease. Unfortunately, acquisition of PVL by conductance technology is not feasible in neonates and small children due to the available human catheter size and resulting invasiveness. The aim of the study was to validate the accuracy of PVL in small hearts using volume data obtained by real-time three-dimensional echocardiography (3DE) and simultaneously acquired pressure data. METHODS: In 17 piglets (weight range: 3.6-8.0 kg) left ventricular PVL were generated by 3DE and simultaneous recordings of ventricular pressure using a mini pressure wire (PVL3D). PVL3D were compared to conductance catheter measurements (PVLCond) under various hemodynamic conditions (baseline, alpha-adrenergic stimulation with phenylephrine, beta-adrenoreceptor-blockage using esmolol). In order to validate the accuracy of 3D volumetric data, cardiac magnetic resonance imaging (CMR) was performed in another 8 piglets. RESULTS: Correlation between CMR- and 3DE-derived volumes was good (enddiastolic volume: mean bias -0.03ml ±1.34ml). Computation of PVL3D in small hearts was feasible and comparable to results obtained by conductance technology. Bland-Altman analysis showed a low bias between PVL3D and PVLCond. Systolic and diastolic parameters were closely associated (Intraclass-Correlation Coefficient for: systolic myocardial elastance 0.95, arterial elastance 0.93, diastolic relaxation constant tau 0.90, indexed end-diastolic volume 0.98). Hemodynamic changes under different conditions were well detected by both methods (ICC 0.82 to 0.98). Inter- and intra-observer coefficients of variation were below 5% for all parameters. CONCLUSIONS: PVL3D generated from 3DE combined with mini pressure wire represent a novel, feasible and reliable method to assess different hemodynamic conditions of cardiac function in hearts comparable to neonate and infant size. This methodology may be integrated into clinical practice and cardiac catheterization programs and has the capability to contribute to clinical decision making even in small hearts.


Assuntos
Ecocardiografia/métodos , Ventrículos do Coração , Animais , Imagem por Ressonância Magnética , Pressão , Suínos
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