Overview of the general approach to diagnosis and treatment of fetal arrhythmias
- Jami C Levine, MD
Jami C Levine, MD
- Assistant Professor
- Harvard Medical School
- Mark E Alexander, MD
Mark E Alexander, MD
- Assistant Professor of Pediatrics
- Harvard Medical School
- Section Editors
- Deborah Levine, MD
Deborah Levine, MD
- Section Editor — Imaging
- Professor of Radiology
- Director of Ob/Gyn Ultrasound
- Department of Radiology
- Beth Israel Deaconess Medical Center
- Louise Wilkins-Haug, MD, PhD
Louise Wilkins-Haug, MD, PhD
- Section Editor — Prenatal Diagnosis and Genetics
- Professor of Obstetrics, Gynecology, and Reproductive Biology
- Harvard Medical School
- John K Triedman, MD
John K Triedman, MD
- Section Editor — Pediatric Cardiology
- Professor of Pediatrics
- Harvard Medical School
Arrhythmias result from abnormal automaticity, abnormal conduction, or both. Fetal arrhythmias complicate 1 to 2 percent of pregnancies and have the potential to compromise fetal health. They are categorized according to their rhythm (irregular, regular) and rate (tachycardia, bradycardia) [1-4]. The type and distribution of arrhythmias in one large series are shown in the table (table 1).
Fetal arrhythmias will be reviewed here. Diagnosis and management of arrhythmias in neonates and children are discussed separately. (See "Irregular heart rate (arrhythmias) in children" and "Identifying newborns with critical congenital heart disease", section on 'Physical examination'.)
The conduction system of the fetal heart is functionally mature by 16 weeks of gestation, and produces a regular rhythm and rate between 110 and 160 beats per minute (bpm) for the remainder of the pregnancy . Fetal arrhythmias are defined by deviations from these parameters. They are typically detected when auscultating the fetal heart, while monitoring the fetal heart rate (FHR) with external or internal devices, or during an antenatal ultrasound examination. (See "Nonstress test and contraction stress test", section on 'Physiologic basis of fetal heart rate changes'.)
At present, ultrasonography is the best modality for evaluation of fetal arrhythmias. It is important to differentiate fetal arrhythmias, particularly intrapartum, from nonreassuring FHR changes related to hypoxemia or other factors. This distinction is usually obvious because of the clinical circumstances. For example, repetitive bradycardia that begins and ends with (or following) uterine contractions is probably related to hypoxemia, while tachycardia associated with prolonged fetal activity or maternal fever probably reflects a normal physiological response rather than a conduction abnormality. When there are concerns, fetal well-being can be evaluated by assessing the FHR response to fetal stimulation or movement, biophysical profile score, or through umbilical blood gas analysis. (See "Nonstress test and contraction stress test" and "Intrapartum fetal heart rate assessment" and "The fetal biophysical profile".)
Two-dimensional ultrasound — Two-dimensional ultrasound is used to diagnose the specific arrhythmia, evaluate cardiac anatomy, evaluate cardiac function, and look for signs of hydrops fetalis. The cardiac anatomy should be carefully reviewed, as arrhythmias can be associated with congenital heart disease. This risk is about 10 percent in patients with tachycardia and about 50 percent in patients with bradycardia. (See "Fetal cardiac abnormalities: Screening, evaluation, and pregnancy management".)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Shenker L. Fetal cardiac arrhythmias. Obstet Gynecol Surv 1979; 34:561.
- McCurdy CM, Reed KL. Fetal Arrhythmias. In: Doppler Ultrasound in Obstetrics and Gynecology, Copel JA, Reed KL (Eds), Raven Press, New York 1995. p.253.
- Kleinman CS, Nehgme R, Copel JA. Fetal cardiac arrhythmias: diagnosis and therapy. In: Maternal-Fetal Medicine, 4th ed, Creasy RK, Resnik R (Eds), WB Saunders Co, Philadelphia 1999. p.301.
- Fouron JC. Fetal arrhythmias: the Saint-Justine hospital experience. Prenat Diagn 2004; 24:1068.
- Macones GA, Hankins GD, Spong CY, et al. The 2008 National Institute of Child Health and Human Development workshop report on electronic fetal monitoring: update on definitions, interpretation, and research guidelines. Obstet Gynecol 2008; 112:661.
- Reed KL, Sahn DJ, Marx GR, et al. Cardiac Doppler flows during fetal arrhythmias: physiologic consequences. Obstet Gynecol 1987; 70:1.
- Indik JH, Chen V, Reed KL. Association of umbilical venous with inferior vena cava blood flow velocities. Obstet Gynecol 1991; 77:551.
- Rein AJ, O'Donnell C, Geva T, et al. Use of tissue velocity imaging in the diagnosis of fetal cardiac arrhythmias. Circulation 2002; 106:1827.
- Zhao H, Chen M, Van Veen BD, et al. Simultaneous fetal magnetocardiography and ultrasound/Doppler imaging. IEEE Trans Biomed Eng 2007; 54:1167.
- Wakai RT, Strasburger JF, Li Z, et al. Magnetocardiographic rhythm patterns at initiation and termination of fetal supraventricular tachycardia. Circulation 2003; 107:307.
- Taylor MJ, Smith MJ, Thomas M, et al. Non-invasive fetal electrocardiography in singleton and multiple pregnancies. BJOG 2003; 110:668.
- Simpson JM. Fetal arrhythmias. Ultrasound Obstet Gynecol 2006; 27:599.
- Nii M, Hamilton RM, Fenwick L, et al. Assessment of fetal atrioventricular time intervals by tissue Doppler and pulse Doppler echocardiography: normal values and correlation with fetal electrocardiography. Heart 2006; 92:1831.
- Zhao H, Cuneo BF, Strasburger JF, et al. Electrophysiological characteristics of fetal atrioventricular block. J Am Coll Cardiol 2008; 51:77.
- Donofrio MT, Moon-Grady AJ, Hornberger LK, et al. Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association. Circulation 2014; 129:2183.
- Boldt T, Eronen M, Andersson S. Long-term outcome in fetuses with cardiac arrhythmias. Obstet Gynecol 2003; 102:1372.
- Copel JA, Liang RI, Demasio K, et al. The clinical significance of the irregular fetal heart rhythm. Am J Obstet Gynecol 2000; 182:813.
- Simpson LL. Fetal supraventricular tachycardias: diagnosis and management. Semin Perinatol 2000; 24:360.
- Jaeggi E, Tulzer G. Pharmacological and interventional fetal cardiovascular treatment. In: Paediatric Cardiology, 3rd ed, Anderson RH, Baker EJ, Redington A, et al (Eds), Elsevier, Philadelphia 2009. p.199.
- Allan LD. Fetal arrhythmias. In: Fetal and Neonatal Cardiology, Long WA (Ed), WB Saunders, 1989. p.180.
- Abernathy A, Alsina L, Greer J, Egerman R. Transient Fetal Tachycardia After Intravenous Diphenhydramine Administration. Obstet Gynecol 2017; 130:374.
- van Engelen AD, Weijtens O, Brenner JI, et al. Management outcome and follow-up of fetal tachycardia. J Am Coll Cardiol 1994; 24:1371.
- Strasburger JF. Prenatal diagnosis of fetal arrhythmias. Clin Perinatol 2005; 32:891.
- Cuneo BF, Strasburger JF. Management strategy for fetal tachycardia. Obstet Gynecol 2000; 96:575.
- Simpson JM, Milburn A, Yates RW, et al. Outcome of intermittent tachyarrhythmias in the fetus. Pediatr Cardiol 1997; 18:78.
- van den Heuvel F, Bink-Boelkens MT, du Marchie Sarvaas GJ, Berger RM. Drug management of fetal tachyarrhythmias: are we ready for a systematic and evidence-based approach? Pacing Clin Electrophysiol 2008; 31 Suppl 1:S54.
- Johnson JA, Williams P, Lu Z, et al. Fetuses of Mothers with Thyroid Disease May Be at Higher Risk of Developing Supraventricular Tachycardia. Am J Perinatol 2015; 32:1240.
- Strizek B, Berg C, Gottschalk I, et al. High-dose flecainide is the most effective treatment of fetal supraventricular tachycardia. Heart Rhythm 2016; 13:1283.
- Vigneswaran TV, Callaghan N, Andrews RE, et al. Correlation of maternal flecainide concentrations and therapeutic effect in fetal supraventricular tachycardia. Heart Rhythm 2014; 11:2047.
- Jaeggi ET, Carvalho JS, De Groot E, et al. Comparison of transplacental treatment of fetal supraventricular tachyarrhythmias with digoxin, flecainide, and sotalol: results of a nonrandomized multicenter study. Circulation 2011; 124:1747.
- Sridharan S, Sullivan I, Tomek V, et al. Flecainide versus digoxin for fetal supraventricular tachycardia: Comparison of two drug treatment protocols. Heart Rhythm 2016; 13:1913.
- Strasburger JF, Cuneo BF, Michon MM, et al. Amiodarone therapy for drug-refractory fetal tachycardia. Circulation 2004; 109:375.
- Krapp M, Kohl T, Simpson JM, et al. Review of diagnosis, treatment, and outcome of fetal atrial flutter compared with supraventricular tachycardia. Heart 2003; 89:913.
- Parilla BV, Strasburger JF, Socol ML. Fetal supraventricular tachycardia complicated by hydrops fetalis: a role for direct fetal intramuscular therapy. Am J Perinatol 1996; 13:483.
- Frohn-Mulder IM, Stewart PA, Witsenburg M, et al. The efficacy of flecainide versus digoxin in the management of fetal supraventricular tachycardia. Prenat Diagn 1995; 15:1297.
- Krapp M, Baschat AA, Gembruch U, et al. Flecainide in the intrauterine treatment of fetal supraventricular tachycardia. Ultrasound Obstet Gynecol 2002; 19:158.
- Jouannic JM, Delahaye S, Fermont L, et al. Fetal supraventricular tachycardia: a role for amiodarone as second-line therapy? Prenat Diagn 2003; 23:152.
- Ebenroth ES, Cordes TM, Darragh RK. Second-line treatment of fetal supraventricular tachycardia using flecainide acetate. Pediatr Cardiol 2001; 22:483.
- Oudijk MA, Michon MM, Kleinman CS, et al. Sotalol in the treatment of fetal dysrhythmias. Circulation 2000; 101:2721.
- Oudijk MA, Ruskamp JM, Ververs FF, et al. Treatment of fetal tachycardia with sotalol: transplacental pharmacokinetics and pharmacodynamics. J Am Coll Cardiol 2003; 42:765.
- Oudijk MA, Ruskamp JM, Ambachtsheer BE, et al. Drug treatment of fetal tachycardias. Paediatr Drugs 2002; 4:49.
- Etheridge SP, Craig JE, Compton SJ. Amiodarone is safe and highly effective therapy for supraventricular tachycardia in infants. Am Heart J 2001; 141:105.
- van der Heijden LB, Oudijk MA, Manten GT, et al. Sotalol as first-line treatment for fetal tachycardia and neonatal follow-up. Ultrasound Obstet Gynecol 2013; 42:285.
- Shah A, Moon-Grady A, Bhogal N, et al. Effectiveness of sotalol as first-line therapy for fetal supraventricular tachyarrhythmias. Am J Cardiol 2012; 109:1614.
- Cuneo BF, Benson DW. Use of maternal flecainide concentration in management of fetal supraventricular tachycardia: a step in the right direction. Heart Rhythm 2014; 11:2054.
- Grosso S, Berardi R, Cioni M, Morgese G. Transient neonatal hypothyroidism after gestational exposure to amiodarone: a follow-up of two cases. J Endocrinol Invest 1998; 21:699.
- Matsumura LK, Born D, Kunii IS, et al. Outcome of thyroid function in newborns from mothers treated with amiodarone. Thyroid 1992; 2:279.
- Lomenick JP, Jackson WA, Backeljauw PF. Amiodarone-induced neonatal hypothyroidism: a unique form of transient early-onset hypothyroidism. J Perinatol 2004; 24:397.
- Bartalena L, Bogazzi F, Braverman LE, Martino E. Effects of amiodarone administration during pregnancy on neonatal thyroid function and subsequent neurodevelopment. J Endocrinol Invest 2001; 24:116.
- Naheed ZJ, Strasburger JF, Deal BJ, et al. Fetal tachycardia: mechanisms and predictors of hydrops fetalis. J Am Coll Cardiol 1996; 27:1736.
- Moodley S, Sanatani S, Potts JE, Sandor GG. Postnatal outcome in patients with fetal tachycardia. Pediatr Cardiol 2013; 34:81.
- Texter KM, Kertesz NJ, Friedman RA, Fenrich AL Jr. Atrial flutter in infants. J Am Coll Cardiol 2006; 48:1040.
- Lisowski LA, Verheijen PM, Benatar AA, et al. Atrial flutter in the perinatal age group: diagnosis, management and outcome. J Am Coll Cardiol 2000; 35:771.
- Simpson JM, Maxwell D, Rosenthal E, Gill H. Fetal ventricular tachycardia secondary to long QT syndrome treated with maternal intravenous magnesium: case report and review of the literature. Ultrasound Obstet Gynecol 2009; 34:475.
- Eliasson H, Wahren-Herlenius M, Sonesson SE. Mechanisms in fetal bradyarrhythmia: 65 cases in a single center analyzed by Doppler flow echocardiographic techniques. Ultrasound Obstet Gynecol 2011; 37:172.
- Mitchell JL, Cuneo BF, Etheridge SP, et al. Fetal heart rate predictors of long QT syndrome. Circulation 2012; 126:2688.
- Lopes LM, Tavares GM, Damiano AP, et al. Perinatal outcome of fetal atrioventricular block: one-hundred-sixteen cases from a single institution. Circulation 2008; 118:1268.
- Jaeggi ET, Hornberger LK, Smallhorn JF, Fouron JC. Prenatal diagnosis of complete atrioventricular block associated with structural heart disease: combined experience of two tertiary care centers and review of the literature. Ultrasound Obstet Gynecol 2005; 26:16.
- Maeno Y, Himeno W, Saito A, et al. Clinical course of fetal congenital atrioventricular block in the Japanese population: a multicentre experience. Heart 2005; 91:1075.
- Jaeggi ET, Hamilton RM, Silverman ED, et al. Outcome of children with fetal, neonatal or childhood diagnosis of isolated congenital atrioventricular block. A single institution's experience of 30 years. J Am Coll Cardiol 2002; 39:130.
- Izmirly PM, Saxena A, Kim MY, et al. Maternal and fetal factors associated with mortality and morbidity in a multi-racial/ethnic registry of anti-SSA/Ro-associated cardiac neonatal lupus. Circulation 2011; 124:1927.
- Kuleva M, Le Bidois J, Decaudin A, et al. Clinical course and outcome of antenatally detected atrioventricular block: experience of a single tertiary centre and review of the literature. Prenat Diagn 2015; 35:354.
- Escobar-Diaz MC, Tworetzky W, Friedman K, et al. Perinatal outcome in fetuses with heterotaxy syndrome and atrioventricular block or bradycardia. Pediatr Cardiol 2014; 35:906.
- Nield LE, Silverman ED, Taylor GP, et al. Maternal anti-Ro and anti-La antibody-associated endocardial fibroelastosis. Circulation 2002; 105:843.
- Groves AM, Allan LD, Rosenthal E. Therapeutic trial of sympathomimetics in three cases of complete heart block in the fetus. Circulation 1995; 92:3394.
- Jaeggi ET, Fouron JC, Silverman ED, et al. Transplacental fetal treatment improves the outcome of prenatally diagnosed complete atrioventricular block without structural heart disease. Circulation 2004; 110:1542.
- Buyon JP, Hiebert R, Copel J, et al. Autoimmune-associated congenital heart block: demographics, mortality, morbidity and recurrence rates obtained from a national neonatal lupus registry. J Am Coll Cardiol 1998; 31:1658.
- Phoon CK, Kim MY, Buyon JP, Friedman DM. Finding the "PR-fect" solution: what is the best tool to measure fetal cardiac PR intervals for the detection and possible treatment of early conduction disease? Congenit Heart Dis 2012; 7:349.
- Mivelaz Y, Raboisson MJ, Abadir S, et al. Ultrasonographic diagnosis of delayed atrioventricular conduction during fetal life: a reliability study. Am J Obstet Gynecol 2010; 203:174.e1.
- Jaeggi ET, Silverman ED, Laskin C, et al. Prolongation of the atrioventricular conduction in fetuses exposed to maternal anti-Ro/SSA and anti-La/SSB antibodies did not predict progressive heart block. A prospective observational study on the effects of maternal antibodies on 165 fetuses. J Am Coll Cardiol 2011; 57:1487.
- Horigome H, Nagashima M, Sumitomo N, et al. Clinical characteristics and genetic background of congenital long-QT syndrome diagnosed in fetal, neonatal, and infantile life: a nationwide questionnaire survey in Japan. Circ Arrhythm Electrophysiol 2010; 3:10.
- Jaeggi ET, Friedberg MK. Diagnosis and management of fetal bradyarrhythmias. Pacing Clin Electrophysiol 2008; 31 Suppl 1:S50.
- Crotti L, Tester DJ, White WM, et al. Long QT syndrome-associated mutations in intrauterine fetal death. JAMA 2013; 309:1473.
- Two-dimensional ultrasound
- Pulsed wave Doppler
- Tissue Doppler imaging
- External fetal heart rate monitoring
- Fetal magnetocardiography
- Fetal electrocardiography
- IRREGULAR RHYTHMS
- Premature atrial contractions
- - Management
- Infrequent isolated ectopy (<3 to 5 beats per minute)
- Complicated ectopy
- Premature junctional and ventricular contractions
- Sinus tachycardia
- Supraventricular tachycardia
- - Approach to management
- - Initial maternal and fetal monitoring
- - Drug treatment
- Flecainide, sotalol
- - Dosing
- - Maternal and fetal heart rate monitoring
- Follow-up after rate control
- - Clinical vignettes
- Atrial flutter and fibrillation
- - Atrial flutter
- - Atrial fibrillation
- - Management of atrial flutter and fibrillation
- Ventricular tachycardia and fibrillation
- Complete heart block
- - Management
- - Prevention of AV block in fetuses of women with anti-lupus autoantibodies
- Sinus bradycardia
- Nonconducted bigeminy
- 2:1 AV block
- Long QT syndrome
- PRENATAL FOLLOW-UP
- SUMMARY AND RECOMMENDATIONS