Prenatal diagnosis and prognosis of accelerated idioventricular rhythm.

OBJECTIVES: As postnatal identification of accelerated idioventricular rhythm (AIVR) relies on specific electrocardiographic patterns, prenatal diagnosis of this condition is challenging and its true incidence is unknown. The objectives of this study were to evaluate the performance of prenatal ultrasonography in identifying intrauterine cardiocirculatory events linked to specific electrocardiographic signs of postnatal AIVR, including left or right ventricular origin, and to assess the prenatal prognosis of this arrhythmia. METHODS: We reviewed Doppler tracings from the superior vena cava/ascending aorta (SVC/Ao), ductus venosus (DV), ductus arteriosus (DA) and aortic isthmus (AoI), as well as simultaneous M-mode recordings of septal and left ventricular wall motions of fetuses diagnosed with AIVR from January 2004 to December 2014. RESULTS: Three cases of AIVR were identified among 27 912 fetuses. SVC/Ao Doppler flow recordings revealed atrioventricular dissociation (ventricular rates within 20% of atrial rates) in all three fetuses and episodes of isorhythmic atrioventricular dissociation in one, while M-mode confirmed normal left ventricular shortening fraction in all cases. Fusion beats were observed on AoI tracing in one fetus, while simultaneous recordings of AoI and DA revealed signs of right bundle branch block in one case and left bundle branch block in the other two. On DV Doppler recordings, retrograde a-waves in the presence of simultaneous atrial and ventricular contractions were observed in all three fetuses, leading to an increase in central venous pressure in all and hydrops fetalis in two cases without evidence of ventricular dysfunction. CONCLUSIONS: Echocardiographic criteria required for postnatal diagnosis of AIVR can be documented in utero using specific ultrasonographic approaches. During fetal life, AIVR may not be a benign entity. Hydrops fetalis is frequently associated with AIVR because of increase in central venous pressure related to simultaneous atrioventricular contractions; thus, the ultrasonographic investigation protocol of fetuses with unexplained hydrops fetalis should aim at ruling out AIVR and include Doppler flow recordings in SVC/Ao, DV, AoI, DA and umbilical vein. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.

Feasibility and reliability of Doppler flow recordings in the fetal aortic isthmus: a multicenter evaluation.

OBJECTIVES: To evaluate the performance of three different centers with respect to their ability to identify the fetal aortic isthmus (AoI) adequately and place a Doppler sample volume in the AoI correctly, and to address the reproducibility of the isthmic flow index (IFI) calculated from Doppler waveforms recorded in the three centers. METHODS: The three collaborating centers sent several ultrasonographic recordings taken at random over a 6-week period to the Saint-Justine Fetal Cardiology Unit (StJ-FCU). A performance quotient ((number of total readings - number of unsatisfactory results)/number of total readings) was calculated for each center by each of three judges, who were experienced fetal cardiologists, to assess the ability of each center to identify the isthmus and to place the Doppler sample volume (DSV) adequately. Intraclass correlation coefficients (ICC) were computed to quantify the variability of IFI measurements ((systolic + diastolic)/systolic flow velocity integrals). RESULTS: Fifty-five recordings were available for this study. Concerning isthmus identification, there was 100% agreement between the three judges from StJ-FCU and the performance quotients of Centers A, B and C were: 0.90, 0.95 and 1.00, respectively. For DSV positioning, agreement between the judges varied; for Judge 1 vs. Judge 2, kappa = 0.836 (95% CI, 0.651-1.000); for Judge 1 vs. Judge 3, kappa = 0.773 (95% CI, 0.557-1.000); for Judge 2 vs. Judge 3, kappa = 0.941 (95% CI, 0.805-1.000). The performance quotients of the three centers for DSV positioning were consistently lower than were those for identification of the isthmus, being 0.85, 0.76 and 0.92, respectively. The ICC between the first and second measurements of the IFI by Rater 1 was 0.96 (95% CI, 0.93-0.98, P < 0.001) and that between Raters 1 and 2 was 0.97 (95% CI, 0.95-0.99, P < 0.001). CONCLUSION: Adequate imaging of the fetal AoI can be achieved easily by a trained sonographer, while DSV positioning is challenging. The intra- and interrater variability of the IFI are low.

Management of fetal tachyarrhythmia based on superior vena cava/aorta Doppler flow recordings.

OBJECTIVE: To evaluate a management protocol of fetal supraventricular tachycardia (SVT) based on prior identification of the underlying mechanism. DESIGN AND SETTING: Prospective study in a mother-child tertiary university centre. PATIENTS: During a consecutive 36 month period, 18 fetuses with sustained SVT underwent a superior vena cava/ascending aorta (SVC/AA) Doppler investigation in an attempt to determine the atrioventricular (AV) relation and to treat the arrhythmia according to a pre-established management protocol. MAIN OUTCOME MEASURE: Rate of conversion to sinus rhythm. RESULTS: Seven fetuses had short ventriculoatrial tachycardia, five of these with a 1:1 AV conduction suggesting re-entrant tachycardia. The first choice drug was digoxin and all were converted. One fetus had AV dissociation leading to the diagnosis of junctional ectopic tachycardia, which was resistant to digoxin and sotalol; amiodarone achieved postnatal conversion. One fetus had SVT and first or second AV block; the diagnosis was atrial ectopic tachycardia (AET), which responded to sotalol given as a drug of first choice. Seven fetuses had long ventriculoatrial tachycardia: one with sinus tachycardia (no treatment), one with permanent junctional reciprocating tachycardia (PJRT), and three with AET. The first choice drug was sotalol and all were converted. One AET was classified postnatally as PJRT. Six fetuses had intra-atrial re-entrant tachycardia: five with 2:1 AV conduction and one with variable block. The first choice drug was digoxin. Conversion was achieved in all but one, who died after birth from advanced cardiomyopathy. CONCLUSION: The electrophysiological mechanisms of fetal SVT can be clarified with SVC/AA Doppler. The proposed management protocol has so far yielded a good rate of conversion to sinus rhythm.

Echocardiographic evaluation of fetal hydrothorax: the effusion ratio as a diagnostic tool.

OBJECTIVE: Fetal hydrothorax may lead to hydrops and is associated with mortality as high as 50%. The objective of this study was to define the pathophysiology of fetal hydrothorax and its relation to hydrops. METHODS: Measurements from echocardiograms of 33 fetuses diagnosed with hydrothorax were made, and included diameters of the thorax, heart, inferior vena cava, right ventricle (RV), left ventricle (LV) and aortic and pulmonary valves. Doppler-derived velocities were measured in the aorta and pulmonary artery just above the aortic and pulmonary valves. The ratio of the area of the effusion to the area of the thorax (effusion ratio) was calculated. Variables were converted into Z-scores from regression equations based on normal data. Features of fetuses with and without hydrops were compared. RESULTS: Higher effusion ratios were noted in hydropic versus non-hydropic fetuses. Compared to a normal population, study subjects had smaller dimensions of LV, RV and aortic and pulmonary valves. They also had higher pulmonary artery peak velocities. The comparison between non-hydropic and hydropic fetuses revealed lower values for LV and pulmonary valve dimensions, and peak aortic velocity in hydropic fetuses. The severity of LV compression correlated significantly with effusion ratio. CONCLUSION: Fetal hydrothorax is accompanied by compression of the cardiac structures, resulting in altered cardiac hemodynamics. Echocardiographic assessment, including the measurement of effusion ratio, may be a useful tool in guiding fetal therapy.

Acquired right ventricular outflow tract obstruction in the recipient twin in twin-twin transfusion syndrome.

OBJECTIVES: The goal of this study was to determine the prevalence and evolution of acquired right ventricular outflow tract obstruction (RVOTO) in the recipient twin in twin-twin transfusion syndrome (TTTS). BACKGROUND: Twin-twin transfusion syndrome complicates 4% to 26% of diamniotic monochorionic twin gestations and is associated with high fetal morbidity and mortality. Cardiac dysfunction and biventricular hypertrophy may develop in the recipient twin with the potential for RVOTO. METHODS: This was a retrospective review of a two-center experience of TTTS to describe the prevalence and evolution of acquired RVOTO in the recipient twin. Right ventricular outflow tract obstruction was diagnosed or excluded by fetal or postnatal echocardiography or clinical assessment. RESULTS: Of 73 twin pregnancies with TTTS identified between 1994 to 1998, a total of seven (9.6%) were complicated by RVOTO in the recipient twin: two subvalvar/muscular, four valvar and one combined. Of 44 pregnancies with fetal echo, six had in utero RVOTO with antegrade flow diagnosed at gestational ages ranging from 19 to 27 weeks. In utero progression occurred in four cases over a period of four to eight weeks, with the development of RVOT atresia by delivery. Postnatal progression of RVOTO occurred in two cases, one of which required pulmonary balloon valvuloplasty at age two years. Postnatal regression of subvalvar RVOTO occurred in two cases in early infancy. Death related directly or indirectly to the RVOTO occurred in all four patients who developed complete RVOT obliteration. CONCLUSIONS: Right ventricular outflow tract obstruction may occur in the recipient twin of at least 9% of pregnancies complicated by TTTS. Right ventricular outflow tract obstruction progression is common in utero and may worsen neonatal outcome.

Superiority of 3-dimensional versus 2-dimensional echocardiography for left ventricular volume assessment in small piglet hearts.

To evaluate the accuracy of 3-dimensional (3D) echocardiography in the estimation of left ventricular (LV) volume in vivo, we studied 15 newborn piglets ranging in weight from 2.6 to 11.8 kg. Measurements of beating LV volumes by 3D echocardiograms were compared with measurements by conductance catheter and transthoracic 2-dimensional (2D) echocardiograms with the use of Simpson's rule. The results of both 3D and 2D echocardiograms correlated strongly with the actual volume (r = 0.98 and 0.95 for LV end-diastolic volume, and 0.998 and 0.95 for LV end-systolic volume, respectively). However, the standard error of estimate (SEE) for 2D echocardiography was larger than for 3D. The SEE values for LV end-diastolic volume for 2D and 3D echocardiograms were 2.30 mL and 1.85 mL, respectively, and 1.52 mL and 0.5 mL for LV end-systolic volume. We conclude that 3D echocardiography not only accurately measures LV volume and systolic function in a newborn heart, it is more precise than measurements from 2D echocardiography in the assessment of small beating hearts.

Echocardiographic predictors of outcome in newborns with congenital diaphragmatic hernia.

OBJECTIVE: Despite new treatments, congenital diaphragmatic hernia (CDH) still has high mortality. The aim of this study was to identify echocardiographic predictors of outcome in newborns with an isolated CDH. METHODS: We reviewed medical charts and echocardiograms of 40 newborns who presented with CDH in the first 24 hours of life, from 1992 to 1996. We compared the cardiac valves and great arteries diameters, left-ventricular volume and mass, Apgar scores, and modified McGoon index (the combined diameter of hilar pulmonary arteries, indexed to the descending aorta) of survivors and nonsurvivors. We performed Student's t test and multiple logistic regression analysis between the 2 groups. RESULTS: Fourteen patients died 1 to 33 days after birth (median: 3 days), including 8 from progressive hypoxemia without operation; 26 have survived up to 5 years (median: 2 years) after successful operations. Nonsurvivors had significantly smaller diameters of right and left hilar pulmonary arteries, more frequent right-sided CDH, and lower mean Apgar scores at 1 and 5 minutes. The most significant prognostic factor was the modified McGoon index. A modified McGoon index

Dilated cardiomyopathy as a first sign of nutritional vitamin D deficiency rickets in infancy.

A five-month-old boy presented with severe dilated cardiomyopathy, requiring intravenous inotropes as part of the initial management. He was found to have hypocalcemia due to vitamin D deficiency rickets. His cardiac function recovered completely after six months of vitamin D supplementation.

Gene transfer in utero biologically engineers a patent ductus arteriosus in lambs by arresting fibronectin-dependent neointimal formation.

Closure of the ductus arteriosus requires prenatal formation of intimal cushions, which occlude the vessel lumen at birth. Survival of newborns with severe congenital heart defects, however, depends on ductal patency. We used a gene transfer approach to create a patent ductus arteriosus by targeting the fibronectin-dependent smooth muscle cell migration required for intimal cushion formation. Fetal lamb ductus arteriosus was transfected in utero with hemagglutinating virus of Japan liposomes containing plasmid encoding 'decoy' RNA to sequester the fibronectin mRNA binding protein. Fibronectin translation was inhibited and intimal cushion formation was prevented. We thus established the essential role of fibronectin-dependent smooth muscle cell migration in intimal cushion formation in the intact animal and the feasibility of incorporating biological engineering in the management of congenital heart disease.

Factors affecting the prognosis of Ebstein's anomaly during fetal life.

BACKGROUND: The echocardiographic criteria that have been used to evaluate severity of Ebstein's anomaly in utero are the same as those applied after birth. OBJECTIVE: The objective of this study was to establish prognostic criteria that take into account the peculiarities of the fetal hemodynamics. METHOD: The video recordings of eight fetuses with Ebstein's anomaly were retrospectively reviewed. RESULTS: The following indexes had no prognostic significance either on fetal or neonatal outcome: the ratio of functional tricuspid opening over the diameter of the annulus, the degree of displacement of the tricuspid valve opening, and the degree of tricuspid regurgitation. The index of severity (based on the surfaces of right atrium + atrialized right ventricle) and the cardiothoracic ratio had a significant impact only on neonatal survival. The smallest fossa ovalis were found in two fetuses who had hydrops. Fetuses who reached term without problems had higher left ventricular outputs. A positive linear correlation was found between the z score of the left ventricular output and the size of the fossa ovalis (r = 0.81, p < 0.05). CONCLUSION: The prognosis of Ebstein's anomaly during fetal life is not influenced by criteria described for postnatal life and may be related to factors that control the volume load of the left ventricle.

Risk factors for venous obstruction in children with transvenous pacing leads.

To determine the incidence and risk factors for venous obstruction in children with transvenous pacing leads, 63 children were evaluated clinically and echocardiographically. Patients with abnormal clinical and/or echocardiographic findings were further investigated by venography. Thirteen patients (21%) had evidence of venous obstruction. Venography in 11 (2 refused) showed that severity of obstruction (as defined by percentage of luminal narrowing) was complete (100%) in 3, severe (> 90%) in 4, and moderate (60%-90%) in 5 (1 patient having 2 sites of obstruction). Risk factors for obstruction in 55 patients with single implantation procedures (10 with obstruction; 18%) were sought. Total cross-sectional area of lead(s) was indexed to body surface area at implantation (INDEX). Patients with obstruction had a higher mean INDEX (7.6 +/- 1.6 mm2/m2) than patients without obstruction (4.9 +/- 2.0 mm2/m2); P < 0.0002). Receiver-operator characteristic curves showed an INDEX > 6.6 mm2/m2 to best predict obstruction, with a sensitivity of 90% and specificity of 84%. Since pacing is lifelong, sizing of transvenous leads to the child is important to prevent obstruction and preserve venous access.

Reducing the deleterious effects of intrauterine CO2 during fetoscopic surgery.

The fetoscopic approach to fetal intervention is a promising minimally invasive technique for correcting congenital anomalies in utero. However, expansion of the amniotic cavity with CO2 to visualize the fetus causes fetal hypercarbia and acidosis. We assessed whether maternal hyperventilation during intrauterine CO2 insufflation could attenuate the fetal hypercarbic acidosis. Seven fetal lambs of 105 +/- 2 days (mean +/- SEM) gestation (term = 145 days) were instrumented with a carotid arterial catheter in utero. After 7 +/- 1 days of recovery, fetoscopic exposure was obtained with intrauterine insufflation of CO2 at 10 mmHg of intraamniotic pressure. After 30 min, the ewe was hyperventilated at a mean respiratory rate of 23/min for 30 min under continuous insufflation. The uterus was then deflated and following 1 hr of stabilization, and the same protocol of CO2 pneumometrium was repeated. Fetal and maternal arterial blood was sampled at baseline and at 15 min intervals. Fetal PaCO2 increased during 30 min of CO2 insufflation (50.8 +/- 2.8 vs. 72.3 +/- 5.0 mmHg, P < 0.01); however, this change was reversed (to 51.5 +/- 3.0 mmHg, P < 0.01) by 30 min of maternal hyperventilation. The fetus developed acidosis after 30 min of CO2 pneumometrium (pH 7.350 +/- 0.012 vs. 7.236 +/- 0.026, P < 0.01); this was also reversed (to 7.366 +/- 0.019, P < 0.01) by maternal hyperventilation. These results were reproducible during the second CO2 insufflation challenge. Fetal hypercarbic acidosis during fetoscopy with CO2 insufflation is reduced by maternal hyperventilation.

Cardiovascular responses to dynamic submaximal exercise in children previously treated with anthracycline.

This study assessed the long-term (5-year) outcome of pediatric low-dose anthracycline therapy on the circulatory response to moderate exercise. Thirteen patients (13 +/- 4 years old) and 15 age-matched control subjects completed a maximal cycle ergometer protocol as well as two 5-minute cycling tests at 33% and 66% maximal oxygen uptake (V(O2)max) for determination of cardiac index (carbon dioxide rebreathing). V(O2)max was lower in patients than in control subjects (1.3 +/- 0.5 L/min vs 2.3 +/- 0.6 L/min) (p< 0.05). Smaller relative increases in cardiac index for similar increases in relative exercise intensities were found in patients (33% V(O2)max, 73% vs 116%; 66% V(O2)max, 115% vs 192%), as a result of smaller increases in stroke index from rest (33% V(O2)max, 33% vs 54%; 66% V(O2)max, 33% vs 69%; p< 0.05). Similarly, despite normal resting systolic function, patients exhibited a lower stroke index and higher heart rate for any given value of oxygen uptake (milliliters per minute per square meter). Children who had survived cancer exhibited stroke index impairment during exercise similar in intensity to that of recreational activities or play, attesting to a limited inotropic reserve.

Short-term effect of monocuspid valves on pulmonary insufficiency and clinical outcome after surgical repair of tetralogy of Fallot.

In the surgical repair of tetralogy of Fallot, monocuspid valves are sometimes inserted within a transannular patch to prevent pulmonary insufficiency. To determine whether this monocuspid valve prevents short-term postoperative pulmonary insufficiency and improves clinical outcome, we reviewed clinical data and preoperative and postoperative echocardiographic variables from 61 patients who underwent one of three different procedures for repair of tetralogy of Fallot between August 1992 and March 1994. We compared features from 24 patients who had undergone transannular patch repair with a monocuspid valve (patch-valve) with those from 17 patients who had undergone patch repair without a monocuspid valve (patch) and 20 patients who had undergone repair without a transannular patch (no patch). We used the ratio of pulmonary valve insufficiency jet width to pulmonary artery diameter, as measured by color-flow Doppler flowmetry, as an index of severity of pulmonary insufficiency. Moderate to severe pulmonary insufficiency was arbitrarily defined as a ratio of at least 0.50. We found no significant differences in ratios among the patch-valve group (0.73 +/- 0.25, mean +/- standard deviation), the patch group (0.79 +/- 0.20), and the no patch group (0.59 +/- 0.23). The percentages of patients with moderate to severe pulmonary insufficiency did not differ among the three groups (patch-valve 80%, patch 90%, no patch 64%). Clinical data (including mortality, number of reoperations, intensive care unit and hospital lengths of stay, and postoperative hemodynamics) were similar in the three groups. We conclude that insertion of a monocuspid valve in repair of tetralogy of Fallot does not prevent short-term postoperative pulmonary insufficiency and does not improve immediate postoperative outcome for these patients.