Viktor Tomek

Isolated Atrioventricular Block in the Fetus A Retrospective, Multinational, Multicenter Study of 175 Patients

Background— Isolated complete atrioventricular block in the fetus is a rare but potentially lethal condition in which the effect of steroid treatment on outcome is unclear. The objective of this work was to study risk factors associated with death and the influence of steroid treatment on outcome.nnMethods and Results— We studied 175 fetuses diagnosed with second- or third-degree atrioventricular block (2000–2007) retrospectively in a multinational, multicenter setting. In 80% of 162 pregnancies with documented antibody status, atrioventricular block was associated with maternal anti-Ro/SSA antibodies. Sixty-seven cases (38%) were treated with fluorinated corticosteroids for a median of 10 weeks (1–21 weeks). Ninety-one percent were alive at birth, and survival in the neonatal period was 93%, similar in steroid-treated and untreated fetuses, regardless of degree of block and/or presence of anti-Ro/SSA. Variables associated with death were gestational age <20 weeks, ventricular rate ≤50 bpm, fetal hydrops, and impaired left ventricular function at diagnosis. The presence of ≥1 of these variables was associated with a 10-fold increase in mortality before birth and a 6-fold increase in the neonatal period independently of treatment. Except for a lower gestational age at diagnosis in treated than untreated (23.4±2.9 versus 24.9±4.9 weeks; P =0.02), risk factors were distributed equally between treatment groups. Two-thirds of survivors had a pacemaker by 1 year of age; 8 children developed cardiomyopathy.nnConclusions— Risk factors associated with a poor outcome were gestation <20 weeks, ventricular rate ≤50 bpm, hydrops, and impaired left ventricular function. No significant effect of treatment with fluorinated corticosteroids was seen.nn# Clinical Perspective {#article-title-22}Background— Isolated complete atrioventricular block in the fetus is a rare but potentially lethal condition in which the effect of steroid treatment on outcome is unclear. The objective of this work was to study risk factors associated with death and the influence of steroid treatment on outcome. Methods and Results— We studied 175 fetuses diagnosed with second- or third-degree atrioventricular block (2000–2007) retrospectively in a multinational, multicenter setting. In 80% of 162 pregnancies with documented antibody status, atrioventricular block was associated with maternal anti-Ro/SSA antibodies. Sixty-seven cases (38%) were treated with fluorinated corticosteroids for a median of 10 weeks (1–21 weeks). Ninety-one percent were alive at birth, and survival in the neonatal period was 93%, similar in steroid-treated and untreated fetuses, regardless of degree of block and/or presence of anti-Ro/SSA. Variables associated with death were gestational age <20 weeks, ventricular rate ⩽50 bpm, fetal hydrops, and impaired left ventricular function at diagnosis. The presence of ≥1 of these variables was associated with a 10-fold increase in mortality before birth and a 6-fold increase in the neonatal period independently of treatment. Except for a lower gestational age at diagnosis in treated than untreated (23.4±2.9 versus 24.9±4.9 weeks; P=0.02), risk factors were distributed equally between treatment groups. Two-thirds of survivors had a pacemaker by 1 year of age; 8 children developed cardiomyopathy. Conclusions— Risk factors associated with a poor outcome were gestation <20 weeks, ventricular rate ⩽50 bpm, hydrops, and impaired left ventricular function. No significant effect of treatment with fluorinated corticosteroids was seen.

Successful Permanent Resynchronization for Failing Right Ventricle After Repair of Tetralogy of Fallot

After repair of tetralogy of Fallot with significantly decreased exercise tolerance, a 17-year-old symptomatic boy underwent successful permanent resynchronization of a failing right ventricle (RV). The patient had surgical repair including pulmonary valvulotomy, infundibulectomy, and pericardial right ventricular outflow tract (RVOT) plasty at 1 year of age. At 3 years of age, he underwent resection of RVOT aneurysm, pulmonary valvuloplasty, and patch enlargement of the left pulmonary artery. Finally, a balloon pulmonary valvuloplasty of a mild residual pulmonary valve stenosis was performed at 17 years of age, resulting in a gradient reduction from 18 to 4 mm Hg and mild (grade I) pulmonary and tricuspid regurgitation. However, the patient continued to show significant RV dilation and systolic/diastolic dysfunction with signs of major RV dyssynchrony (right bundle-branch block; QRS duration of 200 ms and paradoxical interventricular septal …

Flecainide versus digoxin for fetal supraventricular tachycardia: Comparison of two drug treatment protocols

BACKGROUNDnThe optimal treatment for fetal supraventricular tachycardia (SVT) with 1:1 atrioventricular relationship is unclear.nnnOBJECTIVEnWe compared the effectiveness of transplacental treatment protocols used in 2 centers.nnnMETHODSnPharmacologic treatment was used in 84 fetuses. Maternal oral flecainide was the primary therapy in center 1 (n = 34) and intravenous maternal digoxin in center 2 (n = 50). SVT mechanism was classified by mechanical ventriculoatrial (VA) time intervals as short VA or long VA. Treatment success was defined as conversion to sinus rhythm (SR), or rate control, defined as >15% rate reduction.nnnRESULTSnShort VA interval occurred in 67 fetuses (80%) and long VA in 17 (20%). Hydrops was present 28 of 84 (33%). For short VA SVT, conversion to SR was 29 of 42 (69%) for digoxin and 24 of 25 (96%) for flecainide (P = .01). For long VA SVT, conversion to SR and rate control was 4 of 8 (50%) and 0 of 8, respectively, for digoxin, and 6 of 9 (67%) and 2 of 9 (cumulative 89%) for flecainide (P = .13). In nonhydropic fetuses, digoxin was successful in 23 of 29 (79%) and flecainide in 26 of 27 (96%) (P = .10). In hydrops, digoxin was successful in 8 of 21 (38%), flecainide alone in 6 of 7 (86%, P = .07 vs digoxin), and flecainide ± amiodarone in 7 of 7 (100%) (P = .01). Intrauterine or neonatal death occurred in 9 of 21 hydropic fetuses treated with digoxin (43%), compared to 0 of 7 (P = .06) treated with flecainide.nnnCONCLUSIONSnFlecainide was more effective than digoxin, especially when hydrops was present. No adverse fetal outcomes were attributed to flecainide.

Isolated Atrioventricular Block in the Fetus

Background— Isolated complete atrioventricular block in the fetus is a rare but potentially lethal condition in which the effect of steroid treatment on outcome is unclear. The objective of this work was to study risk factors associated with death and the influence of steroid treatment on outcome.nnMethods and Results— We studied 175 fetuses diagnosed with second- or third-degree atrioventricular block (2000–2007) retrospectively in a multinational, multicenter setting. In 80% of 162 pregnancies with documented antibody status, atrioventricular block was associated with maternal anti-Ro/SSA antibodies. Sixty-seven cases (38%) were treated with fluorinated corticosteroids for a median of 10 weeks (1–21 weeks). Ninety-one percent were alive at birth, and survival in the neonatal period was 93%, similar in steroid-treated and untreated fetuses, regardless of degree of block and/or presence of anti-Ro/SSA. Variables associated with death were gestational age <20 weeks, ventricular rate ≤50 bpm, fetal hydrops, and impaired left ventricular function at diagnosis. The presence of ≥1 of these variables was associated with a 10-fold increase in mortality before birth and a 6-fold increase in the neonatal period independently of treatment. Except for a lower gestational age at diagnosis in treated than untreated (23.4±2.9 versus 24.9±4.9 weeks; P =0.02), risk factors were distributed equally between treatment groups. Two-thirds of survivors had a pacemaker by 1 year of age; 8 children developed cardiomyopathy.nnConclusions— Risk factors associated with a poor outcome were gestation <20 weeks, ventricular rate ≤50 bpm, hydrops, and impaired left ventricular function. No significant effect of treatment with fluorinated corticosteroids was seen.nn# Clinical Perspective {#article-title-22}Background— Isolated complete atrioventricular block in the fetus is a rare but potentially lethal condition in which the effect of steroid treatment on outcome is unclear. The objective of this work was to study risk factors associated with death and the influence of steroid treatment on outcome. Methods and Results— We studied 175 fetuses diagnosed with second- or third-degree atrioventricular block (2000–2007) retrospectively in a multinational, multicenter setting. In 80% of 162 pregnancies with documented antibody status, atrioventricular block was associated with maternal anti-Ro/SSA antibodies. Sixty-seven cases (38%) were treated with fluorinated corticosteroids for a median of 10 weeks (1–21 weeks). Ninety-one percent were alive at birth, and survival in the neonatal period was 93%, similar in steroid-treated and untreated fetuses, regardless of degree of block and/or presence of anti-Ro/SSA. Variables associated with death were gestational age <20 weeks, ventricular rate ⩽50 bpm, fetal hydrops, and impaired left ventricular function at diagnosis. The presence of ≥1 of these variables was associated with a 10-fold increase in mortality before birth and a 6-fold increase in the neonatal period independently of treatment. Except for a lower gestational age at diagnosis in treated than untreated (23.4±2.9 versus 24.9±4.9 weeks; P=0.02), risk factors were distributed equally between treatment groups. Two-thirds of survivors had a pacemaker by 1 year of age; 8 children developed cardiomyopathy. Conclusions— Risk factors associated with a poor outcome were gestation <20 weeks, ventricular rate ⩽50 bpm, hydrops, and impaired left ventricular function. No significant effect of treatment with fluorinated corticosteroids was seen.

Pulmonary Right Ventricular Resynchronization in Congenital Heart DiseaseCLINICAL PERSPECTIVE: Acute Improvement in Right Ventricular Mechanics and Contraction Efficiency

Background— Electromechanical discoordination may contribute to long-term pulmonary right ventricular (RV) dysfunction in patients after surgery for congenital heart disease. We sought to evaluate changes in RV function after temporary RV cardiac resynchronization therapy. Methods and Results— Twenty-five patients aged median 12.0 years after repair of tetralogy of Fallot and similar lesions were studied echocardiographically (n=23) and by cardiac catheterization (n=5) after primary repair (n=4) or after surgical RV revalvulation for significant pulmonary regurgitation (n=21). Temporary RV cardiac resynchronization therapy was applied in the presence of complete right bundle branch block by atrial-synchronized RV free wall pacing in complete fusion with spontaneous ventricular depolarization using temporary electrodes. The q-RV interval at the RV free wall pacing site (mean 77.2% of baseline QRS duration) confirmed pacing from a late activated RV area. RV cardiac resynchronization therapy carried significant decrease in QRS duration (P<0.001) along with elimination of the right bundle branch block QRS morphology, increase in RV filling time (P=0.002), pulmonary artery velocity time integral (P=0.006), and RV maximum +dP/dt (P<0.001), and decrease in RV index of myocardial performance (P=0.006). RV mechanical synchrony improved: septal-to-lateral RV mechanical delay decreased (P<0.001) and signs of RV dyssynchrony pattern were significantly abolished. RV systolic stretch fraction reflecting the ratio of myocardial stretching and contraction during systole diminished (P=0.001). Conclusions— In patients with congenital heart disease and right bundle branch block, RV cardiac resynchronization therapy carried multiple positive effects on RV mechanics, synchrony, and contraction efficiency.

Prolonged repolarization in atrial septal defect: An example of mechanoelectrical feedback due to right ventricular volume overload.

BACKGROUNDnProlonged corrected QT (QTc) intervals are frequently observed in children before atrial septal defect (ASD) closure in our department.nnnOBJECTIVEnThe aim of the study was to assess the effect of long-term right ventricular volume overload on repolarization.nnnMETHODSnQRS, QT, and JT intervals were measured manually in leads II and V5 of a standard 12-lead electrocardiogram in 45 children with an isolated ASD a day before and at least 6 months after ASD closure. QT intervals were corrected for heart rate using the Bazett, Fridericia, Framingham, and Hodges formulas. Each QTc interval calculated using the Bazett formula was compared to sex- and age-matched normal values.nnnRESULTSnIndividual QTc intervals shortened significantly (P < .001) using all correction formulas, whereas the QRS duration did not change. The prevalence of prolonged QTc interval decreased from 22.2% to 2.2% after shunt closure (P = .007).nnnCONCLUSIONnThe QTc interval shortens significantly after the closure of a hemodynamically relevant ASD in childhood. This phenomenon is independent of the used QT correction formula and may reflect a mechanoelectrical feedback associated with right ventricular volume overload.

Does First-Trimester Screening Modify the Natural History of Congenital Heart Disease?

Background: The study analyzed the impact of first-trimester screening on the spectrum of congenital heart defects (CHDs) later in pregnancy and on the outcome of fetuses and children born alive with a CHD. Methods: The spectrum of CHDs, associated comorbidities, and outcome of fetuses, either diagnosed with a CHD in the first trimester (Group I, 127 fetuses) or only in the second-trimester screening (Group II, 344 fetuses), were analyzed retrospectively between 2007 and 2013. Second-trimester fetuses diagnosed with a CHD between 2007 and 2013 were also compared with Group III (532 fetuses diagnosed with a CHD in the second trimester from 1996 to 2001, the period before first-trimester screening was introduced). Results: The spectrum of CHDs diagnosed in the first and second trimesters in the same time period differed significantly, with a greater number of comorbidities (P<0.0001), CHDs with univentricular outcome (P<0.0001), intrauterine deaths (P=0.01), and terminations of pregnancy (P<0.0001) in Group I compared with Group II. In Group III, significantly more cases of CHDs with univentricular outcome (P<0.0001), intrauterine demise (P=0.036), and early termination (P<0.0001) were identified compared with fetuses diagnosed with CHDs in the second trimester between 2007 and 2013. The spectrum of CHDs seen in the second-trimester groups differed after first-trimester screening was implemented. Conclusions: First-trimester screening had a significant impact on the spectrum of CHDs and the outcomes of pregnancies with CHDs diagnosed in the second trimester. Early detection of severe forms of CHDs and significant comorbidities resulted in an increased pregnancy termination rate in the first trimester.

Does First-Trimester Screening Modify the Natural History of Congenital Heart Disease?Clinical Perspective: Analysis of Outcome of Regional Cardiac Screening at 2 Different Time Periods

Background: The study analyzed the impact of first-trimester screening on the spectrum of congenital heart defects (CHDs) later in pregnancy and on the outcome of fetuses and children born alive with a CHD. Methods: The spectrum of CHDs, associated comorbidities, and outcome of fetuses, either diagnosed with a CHD in the first trimester (Group I, 127 fetuses) or only in the second-trimester screening (Group II, 344 fetuses), were analyzed retrospectively between 2007 and 2013. Second-trimester fetuses diagnosed with a CHD between 2007 and 2013 were also compared with Group III (532 fetuses diagnosed with a CHD in the second trimester from 1996 to 2001, the period before first-trimester screening was introduced). Results: The spectrum of CHDs diagnosed in the first and second trimesters in the same time period differed significantly, with a greater number of comorbidities (P<0.0001), CHDs with univentricular outcome (P<0.0001), intrauterine deaths (P=0.01), and terminations of pregnancy (P<0.0001) in Group I compared with Group II. In Group III, significantly more cases of CHDs with univentricular outcome (P<0.0001), intrauterine demise (P=0.036), and early termination (P<0.0001) were identified compared with fetuses diagnosed with CHDs in the second trimester between 2007 and 2013. The spectrum of CHDs seen in the second-trimester groups differed after first-trimester screening was implemented. Conclusions: First-trimester screening had a significant impact on the spectrum of CHDs and the outcomes of pregnancies with CHDs diagnosed in the second trimester. Early detection of severe forms of CHDs and significant comorbidities resulted in an increased pregnancy termination rate in the first trimester.

Hemodynamics and Exercise Tolerance after Senning Operation for Transposition of Great Arteries and Its Limiting Factors: A Longitudinal Study

Objectives: The purpose of this longitudinal study was to assess hemodynamics and exercise capacity in patients after Senning operation and to determine its limiting factors. nMethods: 87 long-term survivors of the Senning operation for transposition of great arteries were recruited for a longitudinal follow-up study consisting of two cross-sectional evaluations at a mean of 12.6 and 22.6 years after surgery, respectively. The protocol included a questionnaire, echocardiography, radionuclide angiocardiography, magnetic resonance imaging and cardiopulmonary exercise test. Decreased exercise capacity was defined as maximum oxygen uptake <-2 Z-scores. nResults: Hemodynamic variables (right ventricular function and degree of tricuspid regurgitation) and chronotropic competence did not change significantly with time. Decreased exercise tolerance was present in 55.6 and 49.3% of patients at the first and second evaluation, respectively (NS) and had multi-variable correlation with lower right ventricular end-diastolic volume (P=0.039, OR 0.963) and increased ventilation/carbon dioxide production slope (P=0.040, OR 1.293). None of other hemodynamic variables correlated with decreased functional capacity. nConclusions: Long-term survivors of the Senning operation have stable hemodynamics and exercise capacity on longitudinal follow-up. Decreased exercise tolerance is found in approximately half of the patients and is significantly associated with lower ventilation efficiency. This data may point to the usefulness of targeted respiratory rehabilitation in this patient group.

Isolated Atrioventricular Block in the FetusClinical Perspective: A Retrospective, Multinational, Multicenter Study of 175 Patients

Background— Isolated complete atrioventricular block in the fetus is a rare but potentially lethal condition in which the effect of steroid treatment on outcome is unclear. The objective of this work was to study risk factors associated with death and the influence of steroid treatment on outcome.nnMethods and Results— We studied 175 fetuses diagnosed with second- or third-degree atrioventricular block (2000–2007) retrospectively in a multinational, multicenter setting. In 80% of 162 pregnancies with documented antibody status, atrioventricular block was associated with maternal anti-Ro/SSA antibodies. Sixty-seven cases (38%) were treated with fluorinated corticosteroids for a median of 10 weeks (1–21 weeks). Ninety-one percent were alive at birth, and survival in the neonatal period was 93%, similar in steroid-treated and untreated fetuses, regardless of degree of block and/or presence of anti-Ro/SSA. Variables associated with death were gestational age <20 weeks, ventricular rate ≤50 bpm, fetal hydrops, and impaired left ventricular function at diagnosis. The presence of ≥1 of these variables was associated with a 10-fold increase in mortality before birth and a 6-fold increase in the neonatal period independently of treatment. Except for a lower gestational age at diagnosis in treated than untreated (23.4±2.9 versus 24.9±4.9 weeks; P =0.02), risk factors were distributed equally between treatment groups. Two-thirds of survivors had a pacemaker by 1 year of age; 8 children developed cardiomyopathy.nnConclusions— Risk factors associated with a poor outcome were gestation <20 weeks, ventricular rate ≤50 bpm, hydrops, and impaired left ventricular function. No significant effect of treatment with fluorinated corticosteroids was seen.nn# Clinical Perspective {#article-title-22}Background— Isolated complete atrioventricular block in the fetus is a rare but potentially lethal condition in which the effect of steroid treatment on outcome is unclear. The objective of this work was to study risk factors associated with death and the influence of steroid treatment on outcome. Methods and Results— We studied 175 fetuses diagnosed with second- or third-degree atrioventricular block (2000–2007) retrospectively in a multinational, multicenter setting. In 80% of 162 pregnancies with documented antibody status, atrioventricular block was associated with maternal anti-Ro/SSA antibodies. Sixty-seven cases (38%) were treated with fluorinated corticosteroids for a median of 10 weeks (1–21 weeks). Ninety-one percent were alive at birth, and survival in the neonatal period was 93%, similar in steroid-treated and untreated fetuses, regardless of degree of block and/or presence of anti-Ro/SSA. Variables associated with death were gestational age <20 weeks, ventricular rate ⩽50 bpm, fetal hydrops, and impaired left ventricular function at diagnosis. The presence of ≥1 of these variables was associated with a 10-fold increase in mortality before birth and a 6-fold increase in the neonatal period independently of treatment. Except for a lower gestational age at diagnosis in treated than untreated (23.4±2.9 versus 24.9±4.9 weeks; P=0.02), risk factors were distributed equally between treatment groups. Two-thirds of survivors had a pacemaker by 1 year of age; 8 children developed cardiomyopathy. Conclusions— Risk factors associated with a poor outcome were gestation <20 weeks, ventricular rate ⩽50 bpm, hydrops, and impaired left ventricular function. No significant effect of treatment with fluorinated corticosteroids was seen.