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Biomedical subjects

Bettina F Cuneo

Publications and source records attributed to Bettina F Cuneo.

12 recordsLinked to original sources

Fetal cardiac repolarization abnormalities.

Abnormal cardiac repolarization renders the heart susceptible to lethal ventricular tachyarrhythmias, increasing the risk of sudden cardiac death in all ages; however, little is known about the incidence and etiology of T-wave abnormalities in utero. In this study, magnetocardiography was used to better define fetal T-wave characteristics, including the QT interval in the normal fetus, and to characterize T-wave abnormalities in the fetus with arrhythmia. The QT interval and T-wave alternans were assessed from magnetocardiographic recordings obtained at 14 to 39 weeks' gestation from 120 fetuses. Of these fetuses, 78 were from uncomplicated pregnancies and 42 had various forms of fetal arrhythmia (supraventricular tachycardia in 14, congenital atrioventricular block in 17, long QT syndrome with Torsades de pointes in 1, ventricular tachycardia in 2, sinus bradycardia in 4, and bradycardia due to blocked premature atrial contractions in 4). Although the corrected QT interval in normal sinus rhythm was accurately described by Bazett's formula, the corrected QT interval in fetal arrhythmia exhibited a systematic deviation at heart rate extremes. The dependence of the QT interval on the RR interval in arrhythmia was approximately described by QT alpha RR0.8. T-wave alternans was detected in 7 fetuses with arrhythmia, often in association with QT prolongation, suboptimal outcome, or fetal demise. The results of our study have demonstrated that QT-interval abnormalities exist and can be detected in fetal patients. The potential importance of T-wave assessment in the fetus with cardiac arrhythmia was evidenced by the high incidence of marked QT prolongation and T-wave alternans in the fetuses with suboptimal outcomes.

Arrhythmias, Cardiac↗

Double atrial septum with persistent interatrial space: echocardiographic features of a rare atrial septal malformation.

We describe the echocardiographic features of 4 new cases of a rare atrial septal malformation consisting a double atrial septum with a midline chamber between the left and right atrium. Half of the cases had major left-sided obstructive lesions. Transthoracic, transesophageal, intracardiac, and fetal echocardiographic features of this anomaly are demonstrated. Previous descriptions, embryologic speculations, and clinical considerations are discussed.

Adolescent↗

Outcome of fetal cardiac defects.

PURPOSE OF REVIEW: The present review summarizes the prenatal and postnatal outcome of cardiac anomalies and sustained arrhythmias diagnosed during fetal life. RECENT FINDINGS: The outcome of sustained fetal arrhythmias has improved with greater experience using Sotalol for atrial flutter, and digoxin and amiodarone for 1: 1 reciprocating tachycardia. The outcome of fetal conduction system disease secondary to maternal Sjögren's antibodies has noticeably improved with transplacental dexamethasone. The postnatal prognosis for atrioventricular block associated with left atrial isomerism, however, remains very bleak. Assessing the effect of prenatal diagnosis on the outcome of structural defects remains difficult because of the high number of extracardiac anomalies and chromosome defects in this group. Preoperative survival is improved, but postoperative mortality has not improved. One clear advantage of prenatal diagnosis is the selection of fetuses that may benefit from in-utero intervention to limit the progression of their disease or lessen its severity at birth. SUMMARY: For the most part, postoperative mortality of structural cardiac defects is not significantly improved by prenatal diagnosis. In-utero management of tachyarrhythmias and conduction system disease has improved postnatal outcome, except for the fetus with atrioventricular block and left atrial isomerism.

Arrhythmias, Cardiac↗

Conduction system disease in fetuses evaluated for irregular cardiac rhythm.

OBJECTIVES: To determine the prevalence of 1st and 2nd degree AV block in fetuses with an irregular cardiac rhythm, and to summarize outcome of these pregnancies. BACKGROUND: The diagnosis of irregular cardiac rhythm or 'skipped beats' includes isolated ectopy that resolves spontaneously. Recently, Doppler measurements of the 'mechanical' PR interval have been shown to identify AV conduction disease prenatally. Prenatal therapy of these conduction abnormalities may limit the progression to more advanced disease either in utero or after birth. METHODS: A retrospective review was performed of fetuses evaluated between 1996 and 2004 with the findings of irregular cardiac rhythm. 1st or 2nd degree AV block was diagnosed on Doppler and M-mode recordings, and confirmed using either fetal magnetocardiography (fMCG) or postnatal 12-lead ECG. Dexamethasone was administered to 4 mothers with abnormal fetal AV conduction in the setting of anti-Ro/anti-La antibodies. RESULTS: Of 702 fetuses initially referred for arrhythmia, 306 had an irregular rhythm. Eight (2.6%) had intermittent 1st or 2nd degree AV block confirmed by fMCG and/or postnatal 12-lead ECG. AV block was presumed idiopathic in 2, associated with congenital long QT syndrome in 2 or with clinically unsuspected maternal anti-Ro or anti-La antibodies in 4. During the intrauterine period there was no progression to complete AV block and all were born alive at 34-40 weeks of gestation. CONCLUSION: A small but clinically significant population of fetuses with irregular rhythm will have 1st or 2nd degree AV block. Transplacental therapy may limit the intrauterine progression to more advanced disease.

Antibodies↗

Ultrasonic biophysical measurements in the normal human fetus for optimal design of the monolithic fetal pacemaker.

Ultrasound measurements, including xiphoid-to-pericardial distance and deployment angle, were made on human fetuses as a function of gestational age for the purpose of assessing the likelihood of 3 failure modes of a monolithic fetal pacemaker, including primary positioning failure due to device length and secondary dislodgement failure due to somatic growth. The small variation of the measurements over the gestational age range relevant to device implantation for the major indications of the device (for complete heart block complicated by hydrops and for bradycardia risk after fetal surgery or intrauterine intervention) predicts a small likelihood of these failure modes.

Echocardiography↗

Novel approach to the newborn with hypoplastic left heart syndrome and intact atrial septum.

A prenatally diagnosed fetus with hypoplastic left heart syndrome and intact atrial septum was delivered in the cardiac catheterization suite. Using radio frequency energy, a transseptal perforation of the thickened and intact atrial septum was immediately performed following transcatheter cannulation of the right atrium via the umbilical vein. Serial cutting balloon septostomies followed by static balloon septostomies resulted in effective left atrial decompression, atrial mixing, and optimal pulmonary and systemic perfusion. The child is now thriving after both stage I Norwood and bidirectional Glenn procedures.

Adult↗

Giant fetal magnetocardiogram P waves in congenital atrioventricular block: a marker of cardiovascular compensation?

BACKGROUND: Cardiogram signal amplitude is a key index of hypertrophy but has not been investigated extensively in utero. In this study, magnetocardiography was used to assess P and QRS amplitude in normal subjects and subjects with fetal arrhythmia. METHODS AND RESULTS: The study cohort consisted of 68 normal fetuses and 25 with various arrhythmias: 9 reentrant supraventricular tachycardia (SVT), 2 ventricular tachycardia (VT), 2 sinus tachycardia, 2 blocked atrial bigeminy, 2 congenital second-degree atrioventricular (AV) block, and 8 congenital complete AV block. Subjects with congenital AV block, all presenting with bradycardia, showed large QRS amplitude, exceedingly large P-wave amplitude, and long P-wave duration. The 2 subjects with VT, both with poor ventricular function, also exhibited large P waves. SVT was associated with only moderate signal amplitude elevation. CONCLUSIONS: The data imply that AV block in utero is accompanied by hypertrophy, which is more pronounced for the atria than the ventricles. We hypothesize that the hypertrophy results from a compensatory response associated with regulation of cardiac output and is likely to be observable in other arrhythmias and disease states. Magnetocardiography may be more sensitive than fetal echocardiography for detection of atrial hypertrophy in utero.

Bradycardia↗

Amiodarone therapy for drug-refractory fetal tachycardia.

BACKGROUND: Fetal tachycardia complicated by ventricular dysfunction and hydrops fetalis carries a significant risk of morbidity and mortality. Transplacental digoxin is effective therapy in a small percentage, but there is no consensus with regard to antiarrhythmic treatment if digoxin fails. This study evaluates the safety, efficacy, and outcome of amiodarone therapy for digoxin-refractory fetal tachycardia with heart failure. METHODS AND RESULTS: Fetuses with incessant tachycardia and either hydrops fetalis (n=24) or ventricular dysfunction (n=2) for whom digoxin monotherapy and secondary antiarrhythmic agents (n=13) were not effective were treated transplacentally with a loading dose of oral amiodarone for 2 to 7 days, followed by daily maintenance therapy for <1 to 15 weeks. Digoxin therapy was continued throughout gestation. Newborns were studied by transesophageal pacing or ECG monitoring to determine the mechanism of tachycardia. Three fetuses were delivered urgently in tachycardia during amiodarone loading, and 3 required additional antiarrhythmic agents for sustained cardioversion. Amiodarone or amiodarone combinations converted 14 of 15 (93%) with reentrant supraventricular tachycardia, 2 of 2 with ventricular or junctional ectopic tachycardia, and 3 of 9 (33%) with atrial flutter. Amiodarone-related adverse effects were transient in 5 infants and 8 mothers. Mean gestational age at delivery was 37 weeks, with 100% survival. CONCLUSIONS: Orally administered amiodarone is safe and effective treatment for drug-refractory fetal tachycardia, specifically reentrant supraventricular tachycardia, junctional ectopic, or ventricular tachycardia, even when accompanied by hydrops fetalis or ventricular dysfunction.

Amiodarone↗

Prospective echocardiographic evaluation of atrioventricular conduction in fetuses with maternal Sjögren's antibodies.

OBJECTIVE: Fetal complete atrioventricular block (AVB) occurs in 2-5% of Sjögren's antibodies (SSA/SSB)-positive pregnancies with substantial morbidity and mortality. We evaluated the incidence of 1 degrees and 2 degrees AVB by measuring Doppler-derived mechanical PR intervals. STUDY DESIGN: We compared mechanical PR intervals of fetuses of SSA/SSB positive mothers referred to a single cardiology center between 1997 and 2003 with control fetuses and fetuses with 1 degrees or 2 degrees AVB confirmed by magnetocardiography or postnatal electrocardiogram. RESULTS: One hundred thirty-nine fetal echocardiograms performed on 59 SSA/SSB-positive pregnant women at 24.3 +/- 5.0 weeks gestation were compared with 150 controls. Mechanical PR intervals of the study group (120.5 +/- 9.8 milliseconds) and controls (120.6 +/- 8.7 milliseconds) were the same but differed significantly from fetuses with 1 degrees and 2 degrees AVB (191.5 +/- 29.6 msec). CONCLUSION: The incidence of 1 degrees and degrees 2 fetal AVB in SSA/SSB-positive pregnancies is low and can be identified by abnormal mechanical PR interval.

Antibodies↗

Trends in prenatal diagnosis of critical cardiac defects in an integrated obstetric and pediatric cardiac imaging center.

OBJECTIVE: Prenatal diagnosis of critical (requiring neonatal intervention) cardiac defects (critical congenital heart diseases (CCHD)) improves survival, yet detection of such malformations is poor. Our institution changed its practice and integrated a pediatric cardiologist into the perinatal team. The purpose of this study was to evaluate how this change affected the rate of detected congenital heart disease (CHD) and the diagnostic accuracy. STUDY DESIGN: Obstetrical ultrasounds of mothers at high and normal risk for fetal CCHD at a single center between 1991 and 2001 were reviewed. Rate of detected CCHD, positive predictive values and false positives were compared before and after pediatric cardiology integration. RESULTS: Between the first and second time periods, the rate of detected CCHD increased from 6.8/1000 ultrasounds to 12.9/1000 ultrasounds (p=0.007), and positive predictive value increased from 75 to 96%. CONCLUSION: Collaboration with pediatric cardiology can significantly improve the rate of detected CCHD. These findings have significant implications for sonographer education and patient care.

False Positive Reactions↗