Assessment of atrioventricular conduction by echocardiography and magnetocardiography in normal and anti-Ro/SSA-antibody-positive pregnancies.

OBJECTIVES: The objectives of this study were, first, to evaluate the association between fetal echocardiographic atrioventricular (AV) and magnetocardiographic (fMCG) PR intervals at different gestational ages (GAs) in normal and anti-Ro/SSA-antibody-positive pregnancies; second, to determine if PR interval could be predicted by AV interval; and third, to assess the neonatal outcome of fetuses with prolonged AV and PR intervals, with the goal of developing criteria for fetal first-degree AV block (AVB-I). METHODS: This was a retrospective study of anti-Ro/SSA-antibody-positive pregnancies (cases) and controls that underwent fMCG and fetal echocardiography at the same recording session. Cardiac cycle length, GA and AV (by mitral inflow/aortic outflow Doppler) and PR (by fMCG) intervals were measured. We tested for significant differences between AV and PR intervals using generalized estimating equations to account for repeat measurements, and assessed whether PR interval could be predicted reliably by AV interval. After delivery, infants with fetal AV or PR interval Z-score ≥ 3 underwent 12-lead electrocardiography. RESULTS: Thirty-nine controls and 31 cases underwent 46 and 36 simultaneous fMCG and echocardiographic examinations, respectively; 101 controls and nine cases underwent fMCG only. AV and PR intervals increased with GA (P < 0.05 for both). Overall, AV and PR intervals were significantly different from each other (P < 0.001); this difference was not significant when compared between cases and controls (P = 0.222). PR interval could not be predicted accurately from AV interval and GA alone. Three of four cases with AV and PR interval Z-scores > + 3 had postnatal AVB-I despite treatment. The fourth fetus, which had predominately second-degree AVB and rare periods of AVB-I, progressed to third-degree AVB despite treatment with dexamethasone. CONCLUSIONS: The diagnostic threshold for AVB-I, defined by AV interval Z-score, is GA dependent. Based on the observed data, an AV interval Z-score threshold of 3 (AV interval, 151-167 ms) may be appropriate. Echocardiographic AV interval was not predictive of fMCG-PR interval. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Serial exercise performance in children with surgically corrected congenital aortic stenosis.

We examined serial exercise test performance in children with congenital aortic stenosis (AS) treated surgically compared to that of nonoperated children with mild to moderate AS. Maximal treadmill exercise data were assessed in 21 children 5.5 +/- 3.8 years after aortic valve (AO) surgery. Patients had undergone the Ross procedure (n = 6) or previous aortic valvotomy, balloon valvuloplasty, or neonatal aortic valvotomy (n = 15). Follow-up treadmill tests were conducted 3.7 +/- 2.8 years later. Data were compared to those of 19 nonoperated AS patients (mean gradient by echocardiogram <50 mmHg). These patients were exercised 3.6 +/- 3.2 years apart. Endurance time, heart rate, systemic blood pressure, and electrocardiogram were compared as repeated measures between tests and to age- and sex-matched normative data. Postsurgical children with AS had normal endurance times despite low peak heart rates on the initial test, and they maintained endurance over time. Nonoperated children with mean AO gradients <50 mmHg also had normal endurance times on the initial test but increased endurance over 3.6 years. Children with operated and nonoperated AS were able to reach or exceed normal endurance times, which may make it difficult to achieve compliance to imposed activity restrictions in this population.

Magnetocardiographic rhythm patterns at initiation and termination of fetal supraventricular tachycardia.

BACKGROUND: Using fetal magnetocardiography (fMCG), we characterize for the first time the electrophysiological patterns of initiation and termination of reentrant fetal supraventricular tachycardia (SVT), the most common form of life-threatening fetal arrhythmia. METHODS AND RESULTS: In contrast to the expectation that reentrant SVT is initiated by spontaneous premature atrial contractions (PACs) and is terminated by spontaneous block, 5 distinct patterns of initiation and 4 patterns of termination were documented, with the most common patterns of initiation involving reentrant PACs. Waveform morphology and timing, including QRS and ventriculoatrial interval, were assessed. This enabled detection of such phenomena as Wolff-Parkinson-White syndrome, QRS aberrancy, and multiple reentrant pathways that were crucial for defining the rhythm patterns. In addition, fMCG actocardiography revealed an unexpectedly strong association between fetal trunk movement and the initiation and termination of SVT, suggesting that autonomic influences play a key role. CONCLUSIONS: This study demonstrates that the patterns of initiation and termination of fetal SVT are more diverse than is generally believed and that the most common patterns of initiation involve reentrant PACs. The ability to discern such patterns can help elucidate the underlying mechanisms and guide antiarrhythmic drug therapy. fMCG provides a noninvasive means of analyzing complex tachyarrhythmia in utero, with efficacy approaching that of postnatal electrocardiographic rhythm monitoring.

Impaired exercise parameters in pediatric heart transplant recipients: comparison of biatrial and bicaval techniques.

The exercise performance of pediatric heart transplant recipients and the effects of bicaval anastomosis were studied in 19 children using a Bruce protocol. Although all children had decreased exercise capacity and heart rates when compared with normals, the bicaval anastomosis patients had similar endurance and peak heart rates as the standard biatrial group.

Management strategy for fetal tachycardia.

OBJECTIVE: To develop a management strategy for fetal tachycardia. METHODS: Forty-four fetuses (20-40 weeks' gestation) with nonsinus tachycardia were divided into three groups based on duration of tachycardia and degree of heart failure. Fetuses with intermittent tachycardia were treated expectantly. Fetuses with sustained tachycardia were treated with transplacental antiarrhythmic agents alone if heart failure was mild to moderate, and with direct intramuscular therapy if heart failure was severe. Degree of heart failure was determined by echocardiographic variables of ventricular function, atrioventricular valve insufficiency, and hydrops. Fetal well-being and response to treatment were evaluated by daily heart rate surveillance and frequent fetal echocardiograms and ultrasounds. RESULTS: Fifteen fetuses with intermittent tachycardia (n = 15, group 1) did not progress to sustained tachycardia or heart failure. Fetuses with sustained tachycardia and mild-to-moderate heart failure (n = 14, group 2) were cardioverted or rate controlled with transplacental agents (n = 9); three term fetuses were delivered electively without treatment and two progressed to severe heart failure and were treated in group 3. Seventeen fetuses (15 initially, two progressing) with severe heart failure were cardioverted (in 0. 25-21 days; mean 4.3 days) with fetal intramuscular plus transplacental antiarrhythmic therapy (group 3). Overall, 43 of 44 fetuses were delivered at 32 to 41 (mean 37) weeks with minimal morbidity and a mortality rate of 2.2% (95% confidence interval 0. 06%, 12.0%). CONCLUSION: Perinatal mortality and morbidity were low after following a management strategy based on duration of tachycardia, degree of heart failure, and biophysical profile combined with vigilant ongoing fetal surveillance.

Transmural atrial pacing in patients with postoperative congenital heart disease.

INTRODUCTION: Some patients with postoperative congenital heart disease require permanent cardiac pacing, but the use of transvenous or epicardial pacing leads may be limited by type of cardiac malformation, venous connections, body size, or fibrosis. Transmural atrial pacing may provide an alternative in difficult patients, but to date has been described in only a few articles with small patient numbers, and data from lead performance are lacking. METHODS AND RESULTS: Records were reviewed in 18 consecutive patients (4 months to 21 years old) with postoperative congenital heart disease receiving transmural atrial pacing leads from July 1994 to December 1996. Implantation materials and techniques were described. Lead sensing and capture thresholds obtained acutely and during short-term follow-up (mean: 11.0 months) were evaluated, and comparisons were made between patients with postoperative Fontan anatomy and non-Fontan patients, and between patients receiving steroid-eluting and nonsteroid leads. Overall, the median acute sensing and capture thresholds of transmural leads were 4.1 m V and 0.7 V at 0.5 msec, respectively. Median follow-up thresholds were 2.8 m V and 0.8 V, respectively. Performance of leads in Fontan patients was similar to those in non-Fontan patients. Steroid-eluting leads had a chronic capture threshold of 0.6 V versus 0.9 V for nonsteroid leads (P = 0.038). CONCLUSION: Transmural atrial pacing leads were successfully implanted in patients with diverse ages and types of postoperative congenital heart disease. Lead performance was acceptable both acutely and during the first year of follow-up.

Abnormal systemic venous Doppler flow patterns in atrial tachycardia in infants.

Congestive heart failure due to atrial tachycardia in the neonate may be caused in part by altered preload and changes in diastolic flow characteristics. During atrial tachycardia, venous flow reversal is present. Onset of flow reversal is associated with a ventriculoatrial interval. Decreased tricuspid valve inflow mean velocity and time velocity integral, decreased diastolic duration, and delayed diastolic onset were present during tachycardia when compared with sinus rhythm.

The medical therapy of cardioinhibitory syncope in pediatric patients.

A small percentage of pediatric patients with neurally mediated syncope will have an asystolic response during upright tilt table testing. The purpose of this study is to evaluate the incidence of asystole during tilt table testing, and to assess the outcome of medical management of such patients. Of 398 patients undergoing evaluation for recurrent syncope between January 1989 and 1994, 18 (4.5%) experienced asystole lasting > or = 5 seconds during baseline tilt test. Patients had experienced a mean of four episodes of syncope, with a mean age at the time of tilt test of 11.1 +/- 4.0 years. The median duration of asystole was 10 seconds (range 5-40 s). Treatment was individualized to increased fluids and salt intake (3 patients), metoprolol (8 patients), pseudoephedrine (4 patients), disopyramide (1 patient), or combination therapy with fludrohydrocortisone (2 patients). During a median duration of follow-up of 31 months, no additional syncope was experienced by 78% of patients. Recurrent syncope in 4 patients was associated with either noncompliance or discontinuation of therapy in 3 patients; in 1 patient, increasing the dose of metoprolol was effective in preventing recurrences. We conclude that young patients with recurrent syncope and asystole during tilt test may be safely and effectively managed with pharmacological therapy, without resorting to pacemaker implantation.

Survival following blunt chest impact-induced cardiac arrest during sports activities in young athletes.

Blunt chest impact-induced cardiac arrest on the athletic field (commotio cordis) is not necessarily fatal. The 3 survivors reported here emphasize the importance of recognizing this syndrome so that emergency resuscitative measures are more likely to be implemented promptly, and such catastrophes avoided.

Fetal supraventricular tachycardia complicated by hydrops fetalis: a role for direct fetal intramuscular therapy.

Maternally administered digoxin for the treatment of fetal supraventricular tachycardia (SVT) complicated by hydrops fetalis may be ineffective secondary to poor transplacental drug transfer. We present our experience with eight pregnancies treated with transplacental therapy or combined maternal and direct fetal intramuscular therapy. Response to treatment following maternal intravenous administration (MIV) of digoxin or a combination of fetal intramuscular (FIM) digoxin and MIV is described for eight hydropic fetuses during nine successful pharmacologic conversions. The MIV digoxin was administered using standard loading and maintenance protocols. FIM was administered at a dose of 88 micrograms/kg q 12-24 hours, to a maximum of three injections in the fetal buttock. Time to onset of the first two hours of sinus rhythm (TO2 degrees), time to onset > 90% sinus rhythm (TO > 90%), and time to resolution of hydrops fetalis (HF) were noted. The mean heart rate was 257 +/- 36 beats/minute and the mean gestational age was 29 +/- 4.8 weeks. Fetal SVT was due to a reentrant mechanism in all cases. For the three fetuses that underwent successful cardioversion following MIV digoxin (all required additional maternal antiarrhythmic drugs), TO2 degrees was 145 +/- 114 hours, TO > 90% was 176 +/- 55 hours, and HF resolved in 41 +/- 37 days. Initial combined FIM and MIV therapy in four fetuses resulted in a TO2 degrees of 5.5 +/- 4 hours, TO > 90% of 22 +/- 14 hours, and resolution of HF in 25 +/- 21 days. For the two failed cardioversions with transplacental treatment alone (one fetus had recurrent SVT with hydrops after initial successful cardioversion with MIV), TO2 degrees was 203 +/- 180 hours and TO > 90% was 313 +/- 270 hours. Once FIM was begun in these fetuses, TO2 degrees was 17 +/- 7 hours and TO > 90% was 60 +/- 13 hours; HF resolved in 45 days in one fetus, whereas the other fetus never had resolution of hydrops despite 100 days of antiarrhythmic therapy. Direct fetal intramuscular injection of digoxin combined with transplacental therapy appears to shorten the time to initial conversion of SVT and to sustain sinus rhythm in the fetus with SVT complicated by hydrops fetalis.

Fetal tachycardia: mechanisms and predictors of hydrops fetalis.

OBJECTIVES: This study had three objectives: 1) to determine the electrophysiologic mechanisms of fetal supraventricular tachycardia at presentation and postnatally; 2) to identify the clinical and electrophysiologic predictors of hydrops fetalis; and 3) to describe the medium-term follow-up (1 to 7 years) of patients with fetal supraventricular tachycardia. BACKGROUND: Fetal supraventricular tachycardia causes significant fetal and neonatal morbidity and mortality. Prenatal analysis and postnatal confirmation of fetal supraventricular tachycardia mechanisms have been limited. METHODS: Supraventricular tachycardia mechanisms were evaluated by prenatal Doppler/M-mode echocardiography, immediate neonatal surface electrocardiography and postnatal transesophageal electrophysiologic procedures in 30 consecutive patients presenting with fetal supraventricular tachycardia (17 managed prenatally, 13 first managed postnatally). RESULTS: The fetal supraventricular tachycardia mechanism was 1:1 atrioventricular conduction in 22 patients and supraventricular tachycardia with atrioventricular block (atrial flutter) in 8. At the postnatal transesophageal electrophysiologic procedure, tachycardia was induced in 27 of 30 patients; atrioventricular reentrant tachycardia in 25 (93%) of 27 and intraatrial reentrant tachycardia in only 2 (7%) of 27. Hydrops was present in 12 of 30 fetuses. Sustained supraventricular tachycardia (> 12 h) and lower gestation at presentation correlated with hydrops (p < 0.02, p < 0.05), but mechanism of tachycardia and heart rate did not. Gestational age at delivery was significantly greater in those who received intrauterine management (39 +/- 1.3 vs. 37 +/- 2.9 weeks, p = 0.04) despite earlier presentation (32.6 vs. 37.1 weeks). Cesarean section deliveries were reduced in the same group (3 of 17 vs. 11 of 13, p = 0.0006). CONCLUSIONS: Atrioventricular reentrant tachycardia was the predominant mechanism of supraventricular tachycardia in the fetus. There was a high association of supraventricular tachycardia with atrioventricular block in utero and accessory atrioventricular connections. Outcome at 1 to 7 years was excellent regardless of severity of illness at clinical presentation.

Comparison of transesophageal and intracardiac electrophysiologic studies in characterization of supraventricular tachycardia in pediatric patients.

OBJECTIVES: This study sought to determine the accuracy of transesophageal electrophysiologic studies in diagnosing and characterizing various mechanisms of supraventricular tachycardia in pediatric patients. BACKGROUND: Transesophageal electrophysiologic studies are a relatively noninvasive means of characterizing supraventricular tachycardia. Although widely used, to our knowledge no data exist that directly compare information obtained from transesophageal electrophysiologic studies with that from intracardiac electrophysiologic studies. METHODS: We reviewed the records of 57 pediatric patients undergoing both transesophageal and intracardiac electrophysiologic studies at our institution. The results of these studies were compared with respect to mechanism of tachycardia, localization of accessory atrioventricular (AV) connections (if present) and characterization of anterograde accessory connection conduction properties. RESULTS: Tachycardia mechanisms were concordant in 56 of 57 patients: orthodromic reciprocating tachycardia in 43, antidromic reciprocating tachycardia in 1, both orthodromic and antidromic tachycardia in 2, AV node reentrant tachycardia in 5, atrial reentrant tachycardia in 4 and ectopic atrial tachycardia in 2. Of 29 patients with orthodromic reciprocating tachycardia using a concealed accessory connection, transesophageal study predicted the accessory connection site through changes induced by transient bundle branch block in 12. By the Bland-Altman method in 14 patients with pre-excitation, the anterograde accessory connection effective refractory period determined by transesophageal study compared favorably with that determined by intracardiac study (mean difference 5.0 ms, limits of agreement -55 and 65 ms). CONCLUSIONS: Transesophageal electrophysiologic studies are a highly accurate means of diagnosing and characterizing various mechanisms of supraventricular tachycardia in pediatric patients.

Sonographic diagnosis of congenital heart disease: comparison between the four-chamber view and multiple cardiac views.

OBJECTIVE: To report our experience in the detection of congenital heart disease using both the four-chamber view of the heart as part of the standard obstetric ultrasound examination and multiple cardiac views as part of the detailed targeted examination. METHODS: All admissions to Children's Memorial Hospital of Northwestern University Medical Center with the diagnosis of congenital heart disease between June 1988 and April 1992 were identified (N = 1947). These admissions were matched to deliveries (N = 19,321) that occurred at Prentice Women's Hospital during the same period; of these, 10,004 had at least one obstetric ultrasound examination. All fetuses were scanned either with the standard obstetric ultrasound type of examination, featuring only the four-chamber view of the heart, or by the detailed targeted imaging type of study, featuring multiple cardiac views. The type of examination performed was based on the specific request of the attending obstetrician or gynecologist. RESULTS: Thirty-three neonates who had at least one obstetric ultrasound examination were treated for congenital heart disease. An additional five pregnancies were terminated secondary to serious fetal heart defects. When only the four-chamber view was visualized, 11 of 33 fetuses (33.3%) with confirmed congenital heart disease were detected. CONCLUSIONS: Assessment of the outflow tracts is crucial for detection of many forms of congenital heart disease. However, before this is accepted as the standard of care, both the obstetric and radiologic communities should develop their skills in cardiac imaging. Only then can this sophisticated type of cardiac examination be offered to pregnant women.

Usefulness of metoprolol for unexplained syncope and positive response to tilt testing in young persons.

The efficacy of intravenous metoprolol in preventing symptoms during a repeat tilt test was compared with the outcome of chronic oral treatment in 21 patients (14 female, 7 male), age 8 to 20 years (mean 13 +/- 3) with unexplained syncope (> or = 1 episode) and a positive response to tilt testing. A positive response was defined as the development of either syncope or presyncope. During the initial tilt test, a positive response occurred during baseline (14 patients) or isoproterenol (0.03 to 0.1 microgram/kg/min) infusion (7 patients) with a cardioinhibitory (1 patient), vasodepressor (5 patients) or mixed (15 patients) pattern. Metoprolol (0.1 to 0.2 mg/kg) was administered intravenously. During the repeat tilt test, response was negative in 18 patients, including 11 of 14 patients with a positive response in the baseline and 7 of 7 patients with a positive response during isoproterenol infusion. Metoprolol (0.8 to 2.8 mg/kg/day) was administered orally to 15 patients for an average of 10 months. Symptoms were absent (7 patients) or improved (2 patients); metoprolol was discontinued because of adverse effects (3 patients) or recurrence of symptoms (3 patients). In 7 of 12 patients with a negative response and 2 of 3 patients with a positive response after intravenous metoprolol, oral administration of metoprolol prevented or improved symptoms without adverse effects. Many young patients (60%) with recurrent syncope obtained symptomatic improvement from chronic oral metoprolol treatment without adverse effects; repeat tilt testing after intravenous metoprolol did not appear to offer any additional information than would have been obtained from a trial of chronic oral treatment.

Reproducibility of a symptomatic response to upright tilt in young patients with unexplained syncope.

Serial upright tilt testing has been advocated as a possible therapeutic end point in treating patients with recurrent syncope, but the reproducibility of such testing has not been well-established in the absence of therapy. In 21 patients with recurrent syncope and a symptomatic response to upright tilt testing, tilt was repeated following a 25- to 30-minute recovery. Syncope or presyncope was reproduced in 14 of 21 patients on repeat tilt; 4 of 21 patients experienced milder symptoms, and 3 of 21 patients remained entirely asymptomatic on repeat tilt. The pattern of physiologic response was different during initial and repeat tilt in 7 of the 21 patients. Blood pressure and heart rate (before and during symptoms) were similar for the group on initial and repeat tilt. The abnormal physiologic response and associated symptoms elicited during upright tilt testing for unexplained syncope can be reproduced on immediate repeat tilt testing in most patients. However, the frequently attenuated response during repeat testing may limit its use as a therapeutic end point in individual patients.

Supraventricular tachycardia mechanisms and their age distribution in pediatric patients.

To better define the natural history of supraventricular tachycardia (SVT) in young patients, age distribution of SVT mechanisms was examined in 137 infants, children and adolescents. Patients with a history of cardiac surgery or neuromuscular diseases were excluded. An electrophysiologic study was performed in each patient: transesophageal (110 patients) or transvenous (14 patients) or both (13 patients). Mechanisms were classified as SVT using accessory atrioventricular (AV) connection (SVT using accessory connection, including orthodromic and antidromic reciprocating tachycardia), primary atrial tachycardia (including chaotic, automatic and reentrant atrial tachycardia), and tachycardia due to reentry within the AV node. SVT using accessory connection occurred in 100 of 137 patients (73%) and was the most prevalent mechanism. Primary atrial tachycardia and reentry within the AV node were present in 19 of 137 (14%) and 18 of 137 (13%) patients, respectively. Using a multinomial logit model, relative probabilities for tachycardia mechanisms for 5 age groups--prenatal, less than 1, 1 to 5, 6 to 10 and greater than 10 years--were determined. Primary atrial tachycardia (11 to 16%) and SVT using accessory connection (58 to 84%) appeared throughout infancy, childhood and adolescence. On the other hand, tachycardia due to reentry within the AV node (0 to 31%) rarely appeared before age 2 years. Mechanisms of SVT appear to have age-dependent distributions. SVT using accessory connection is the most common mechanism in young patients. We speculate that the propensity to tachycardia due to reentry within the AV node occurs during postnatal development.