Seminars in Fetal & Neonatal Medicine (2005) 10, 504e514

www.elsevierhealth.com/journals/siny

Fetal brady- and tachyarrhythmias: New and accepted diagnostic and treatment methods
Edgar T. Jaeggi*, Masaki Nii
Fetal Cardiac Program, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada

KEYWORDS
Fetal; Arrhythmia; Diagnosis; Complete AV block; Atrial utter; Supraventricular tachycardia

Summary Sustained bradyarrhythmias are typically the result of symptomatic sinus bradycardia, atrial bigeminy or complete atrioventricular (AV) block. Fetal tachyarrhythmias relate to sinus tachycardia, atrial utter and supraventricular tachycardia as the main aetiology. Ultrasound is essential to understand the underlying arrhythmia mechanism, to study the impact on cardiac function, to exclude cardiac defects or tumours, and to survey the fetal heart rate and well-being, e.g. during anti-arrhythmic treatment. Based on retrospective studies, several more or less safe, effective and well-tolerated anti-arrhythmic agents are currently available for the treatment of atrial and supraventricular tachycardia. Isolated congenital complete AV block is mainly related to maternal anti-Ro/La autoantibodies. The rationale to treat a fetus at this irreversible stage of AV nodal damage is primarily to mitigate or prevent concomitant myocardial inammation and to augment cardiac output. A recently published study demonstrated a signicant improved outcome with transmaternal dexamethasone and b-stimulation. 2005 Elsevier Ltd. All rights reserved.

Introduction
Depending on gestational age and degree of fetal activity, the normal fetal cardiac rhythm is characterized by a regular heart rate ranging between 100 and 180 beats/min (bpm) with a normal 1:1 atrioventricular (AV) electromechanical relationship during each cardiac cycle. Accordingly, fetal cardiac arrhythmias may present as an irregular heart
* Corresponding author. Tel.: C1 416 813 7466; fax: C1 416 813 7547. E-mail address: edgar.jaeggi@sickkids.ca (E.T. Jaeggi).

rate, as an abnormally slow or fast heart rate, or as a combination of irregular rhythm and an abnormal heart rate. Such fetal rhythm disturbances, which are diagnosed in at least 2% of pregnancies during routine ultrasound scanning, are common reasons for referral to a fetal cardiologist.1 In more than 90% of cases, arrhythmias are brief and isolated events of little clinical relevance. Of more concern are the prolonged or incessant episodes of heart rates that are too fast (O180 bpm) or too slow (!100 bpm). This includes supraventricular tachycardia (SVT), atrial utter and complete AV block as the major fetal arrhythmia mechanisms.

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Fetal rhythm assessment

Echocardiography
As in extra-uterine life, the classication of fetal rhythm and conduction anomalies is based primarily on the chronology of electrophysiological events. However, assessment of the fetal cardiac rhythm is more challenging as conventional realtime electrocardiograms cannot be obtained. M-mode, pulse-wave and tissue Doppler echocardiography have been used as surrogates as they enable study of the chronology of atrial and ventricular electrical events indirectly by their respective mechanical consequences.2e4 Guided by two-dimensional echocardiography, the Mmode ultrasound beam is aligned simultaneously through the atrial and ventricular walls and the sequence of their systolic wall motions is studied (Fig. 2). A limitation of M-mode recordings is that the onset and maximum of the atrial and ventricular contraction is often not clearly dened, and therefore precise measurement of AV time intervals is not possible. Recording of blood ow velocities that represent atrial and ventricular events is more accurate in this regard as ow signals are more readily recognized. Simultaneous pulsewave Doppler interrogation of the superior vena cava and the ascending aorta, two vessels that are in close anatomical relation to each other,

Figure 1 Distribution of major fetal bradyarrhythmias (n Z 28) and tachyarrhythmias (n Z 38) seen at the Hospital for Sick Children during the last 5 years (2000e 2 0 0 4 ) . S V T, s u p r a v e n t r i c u l a r t a c h y c a r d i a ; AV, atrioventricular.

Major arrhythmias are not common. Even at the authors large tertiary care centre, only a handful of fetal cases are seen every year, as illustrated in Fig. 1. The outcome of affected fetuses is inuenced by numerous variables including arrhythmia characteristics, age at diagnosis, cardiac defects and choice of treatment. Thus, the primary objective of every newly detected fetal rhythm disorder must be: (1) to understand the underlying arrhythmia mechanism; (2) to clarify its impact on the fetal circulation; and (3) to conclude on the need for treatment.

Figure 2 M-mode tracing using a four-chamber view to demonstrate atrial (A) and ventricular (V) wall motions. The M-mode is directed simultaneously through the left ventricle (LV) and right atrium (RA). There is atrial utter with an atrial rate of 414 beats/min (bpm), functional 2:1 atrioventricular block, and a regular ventricular rate of 207 bpm.

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Figure 3 Pulsed Doppler ow modalities. (A) Simultaneous ascending aorta (V) and superior vena cava (A). Doppler ow recording at 20 weeks of gestation showing sinus bradycardia with normal 1:1 atrioventricular conduction. (B) Simultaneous inow/outow Doppler of a 19-week fetus with long ventriculo-atrial tachycardia of 190 beats/min due to fetal thyrotoxicosis. The mother had a medical history of Graves disease for which she had been treated with propylthiouracil and radioactive iodine.

uses this concept (Fig. 3A). The beginning of retrograde ow in the superior vena cava indicates the beginning of atrial systole, whereas the onset of aortic forward ow marks the beginning of ventricular systole. Alternatively, simultaneous Doppler recording of mitral valvar inow and ascending aorta ow waves may be used; the beginning of A-wave inow marks the onset of active atrial contraction (Fig. 3B). Colour-coded tissue velocity imaging (TVI) allows ofine quantication of segmental wall motion in any area of the heart during the same cardiac cycle (Fig. 4). A typical TVI curve from the basis of the heart is composed of two diastolic wave curves that are produced by longitudinal tissue motion away from the apex during the early diastolic lling phase and during atrial contraction. During ventricular contraction, the ventricles move towards the apex; as a result, a third wave occurs in the opposite direction. Atrial (A) systolic velocities may also be recorded but are much smaller and point in the opposite direction when compared with Aa in ventricular sampling. TVI recordings have an excellent temporal resolution but need to be analysed ofine using special software. All ultrasound modalities are useful in assessing the integrity of electrical AV conduction. However, it is important to realize that normal conduction

time values differ among modalities and prolong with gestational age. Normal AV conduction times are 190 G 36 ms by M-mode, 120 G 11 ms by left ventricular inow/outow Doppler, and 111 G 17 ms by superior vena cava/aorta Doppler sampling.3 To assess fetal arrhythmias, M-mode and simultaneous Doppler of the superior vena cava and ascending aorta are particularly user friendly as they allow fast, reproducible and accurate online analysis in most cases.2,5,6

Electrocardiogram (ECG) and magnetocardiogram (MCG)

Transabdominal fetal ECG and MCG have recently become commercially available and they are now used by a few centres.7,8 Fetal ECG is based on signal averaging of electrocardiographic complexes and does not allow beat-to-beat analysis. As a consequence, ECG is not helpful in diagnosing fetal rhythm and conduction anomalies with irregular heart rates. Fetal MCG provides better signal quality than ECG because of more favourable transmission properties of magnetic signals. The use of the magnetic analogue of ECG requires a magnetically shielded room. Both MCG and ECG may provide

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Figure 4 Normal tissue Doppler imaging from the right atrioventricular (AV) groove and the right atrial wall. Sequential analysis of regional tissue motion at the AV groove results in two diastolic [early diastolic lling phase (Ea) and atrial contraction (Aa)] waves and one systolic (Sa) wave.

useful information on cardiac time intervals such as the QRS and QT duration.

Fetal bradycardia
Brief episodes of sinus bradycardia that last less than 1e2 min are frequent and benign ndings, particularly during the rst and second trimesters. No treatment is required. A prolonged or persistent decrease in heart rate !100 bpm may be observed during symptomatic sinus bradycardia, atrial bigeminy with blocked premature beats, and high-degree AV block. As the ventricular rate may be comparable among these rhythm anomalies, ultrasound imaging is used for their diagnostic differentiation. During sinus bradycardia, M-mode and Doppler echocardiography show a normal 1:1 AV activation sequence (Fig. 3A). Causes of prolonged episodes include fetal distress secondary to conditions leading to fetal hypoxia and acidosis, long QT syndrome and congenital sinus node dysfunction.9 Fetal adaptation to hypoxaemia is marked by changes in regional blood ow with an increased blood supply from the venous duct to the brain and myocardium (brain and heart sparing). Runs of ventricular tachycardia and 2:1 AV block are additional ndings that suggest fetal long QT syndrome. Careful examination and, in the absence of fetal MCG,

postnatal ECG are recommended in these children and their family members. Persistent atrial bigeminy and trigeminy with blocked premature beats may lower the average heart rate of the fetus to 70e100 bpm, which may be confused with second- or third-degree AV block. Blocked premature beats and AV block are characterized by higher atrial than ventricular rates. In AV block, however, the time interval between consecutive atrial impulses hardly varies while every second event occurs prematurely in atrial bigeminy (Fig. 5). This differentiation is essential as bradycardia secondary to blocked premature beats resolves spontaneously without treatment, while complete AV block (CAVB) is an irreversible and potentially life-threatening disorder. AV block refers to a disturbance of electrical conduction within the AV node, the bundle of His or the bundle branches. First-degree AV block is characterized by a prolonged AV conduction time but all impulses are conducted. Second-degree AV block refers to a failure to conduct some, but not all, atrial impulses to the ventricles. Type I (Wenckebach) second-degree AV block denotes progressive lengthening of the AV conduction time, until an isolated impulse is blocked. Type II (Mobitz II) block is characterized by occasional or repetitive sudden AV block without prior lengthening of the AV conduction time. In 2:1 AV block, only every second atrial beat is conducted to the

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Figure 5 M-mode echocardiography at 20 weeks showing signicant fetal bradycardia (VeV: 67 beats/min) related to atrial bigeminy. There is atrial beat-to-beat variation with the premature atrial beats (AeA interval: 370 ms) being blocked and the normal atrial impulses (450 ms) being conducted. A repeat echocardiogram showed normal cardiac rhythm 1 week later.

ventricles. In third-degree AV block or CAVB, the electrical AV communication is completely interrupted and the atria and ventricles beat independently (Fig. 6). Signicant bradycardia is only seen with high-degree second- or third-degree AV block. The fetal heart compensates to the evolving heart

block by increasing its stroke volume. If the heart fails to adapt, fetal hydrops develops with a high risk of fetal or neonatal death.10e13 CAVB accounts for nearly 40% of major fetal arrhythmias referred to the authors institution. In roughly half of the cases, CAVB is associated

Figure 6 Simultaneous Doppler of the aorta and superior vena cava showing complete atrioventricular block. The atrial (AeA) rate was 138 beats/min (bpm) and the ventricular (VeV) rate was 54 bpm, with complete atrioventricular dissociation.

Fetal brady- and tachyarrhythmias: New and accepted diagnostic and treatment methods with left isomerism and congenitally corrected transposition.13 The outcome of fetal CAVB with major congenital heart disease is particularly poor with less than 20% survival of the fetal and neonatal periods.13 In the absence of congenital heart disease, CAVB is mainly associated with the transplacental passage of maternal auto-antibodies to 48-kDa SSB/La, 52-kDa and/or 60-kDa SSA/ Ro ribonucleioproteins.13 These antibodies are typically found in Sjogrens syndrome and systemic lupus erythematosus, but may also be demonstrated in 1e2% of pregnant women with no clinical evidence of auto-immune disease. Anti-Ro/La antibodies progressively enter the fetal circulation in the second trimester and may elicit an immunemediated tissue injury resulting in progressive destruction of the AV node, myocardial inammation, endocardial bro-elastosis and dilated cardiomyopathy in the susceptible offspring.12e14 Fetal CAVB emerging between 20 and 24 weeks gestation affects 1e2% of the cohort of antibody-positive mothers, while the other cardiac manifestations are less common.15,16 The reported probability of having a second child with immune-mediated CAVB varies between 8% and 18%.12 So far, isolated CAVB has been associated with a signicant mortality that ranges between 18% and 43%.10e13 Risk factors of adverse outcome included fetal hydrops, endocardial bro-elastosis, premature delivery and fetal heart rate %55 bpm.10e13 Of the survivors, most required pacemaker implantation during the rst year of life.11 To improve the pregnancy outcome, numerous preventive and therapeutic approaches have been used with variable success.17e30 Theoretically, all mothers at risk of delivering a child with immune-mediated CAVB may be considered for preventive treatment. In fact, this approach has been chosen for a small number of pregnancies by maternal plasma exchange and administration of maternal immunoglobulin, steroid or azathioprine.19,25,26 This approach aims primarily at reducing maternal auto-antibody titres, but may result in a state of severe antibody depletion that renders both the mother and her offspring at an increased risk of infection. Considering the low fetal CAVB risk, it is difcult to advocate preventive treatment to mainly unaffected fetuses and mothers. Unfortunately, there are no reliable markers that predict which fetus of an anti-Ro/La-antibody-positive woman will develop immune-mediated cardiac complications. Moreover, fetal CAVB seems to develop rapidly within a few days.6 Therefore, it is no surprise that most fetuses are diagnosed with established CAVB. The rationale to

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treat a fetus at this irreversible stage of AV nodal damage is primarily to mitigate or prevent concomitant myocardial inammation and to augment fetal heart rate. Unlike prednisone, the uorinated steroid compounds dexamethasone and betamethasone are only minimally metabolized by the placenta, and become useful when antiinammatory fetal treatment is desired. Maternal dexamethasone administration has been shown to improve fetal AV block, hydrops and myocardial dysfunction.17,18,20,27,29 Various b-stimulants have been used to increase the fetal heart rate and myocardial contractility with variable success.23,28,30 However, an improved overall outcome was shown recently after introduction of prenatal treatment guidelines (Fig. 7).30 According to this protocol, maternal dexamethasone (4 mg/ day) is initiated at diagnosis of immune-mediated CAVB and, when possible, maintained for the duration of the pregnancy. A b-sympathomimetic agent (oral salbutamol 3 ! 10 mg/day) is added if the average heart rate is below 55 bpm. The affected pregnancy is closely surveyed, with elective delivery being at around 37 weeks of gestation in uncomplicated cases and neonatal care being in the critical care unit. Since introduction of this approach in 1997, the 1-year survival rate with isolated CAVB has increased from 47% to 95% (Fig. 8). Antenatal steroid treatment has its risks and future studies will need to address potential long-term effects, e.g. on brain development. Oligohydramnios did occur in 20% of cases after chronic steroid treatment and resulted in premature deliveries in some cases. Thus, the amniotic uid volume needs to be carefully monitored throughout the pregnancy course, and a timely reduction in dexamethasone dose to 2 mg/day may be required. To stimulate the fetal heart rate, the authors prescribe salbutamol in high doses. This commonly results in unpleasant b-adrenergic maternal side-effects including palpitations and tremor, but more serious adverse effects have not been encountered. In the present state of knowledge and with the obvious improvement in outcome, there is no solid ground to deny the benet of transplacental steroid treatment to fetuses with immune-mediated CAVB.

Fetal tachycardia
Anomalies that lead to an intermittent or sustained increase in heart rate of R180 bpm account for 60% of the major fetal tachyarrhythmias seen at the authors centre. Echocardiography is again the diagnostic cornerstone in establishing the

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Figure 7 Treatment protocol for the management of isolated fetal complete atrioventricular block at the Hospital for Sick Children. DXMT, dexamethasone; HR, heart rate. Reproduced with permission from Jaeggi ET, Fouron JC, Silverman ED, Ryan G, Smallhorn J, Hornberger LK. Circulation 2004;110:1542e8.30

arrhythmia mechanisms and haemodynamic consequences, and in detecting occasionally contributing cardiac malformations and tumours. SVT, atrial utter and sinus tachycardia are the predominant fetal causes.31e33 Persistent tachyarrhythmias may be well tolerated or, at the other end of the spectrum, may lead to congestive heart failure, dilated cardiomyopathy, neurological pathology and even death. Fetal hydrops has been associated with a mortality risk of up to 35%, compared with 0e4% in cases without signicant heart failure.33 Ventricular tachycardia is rarely detected in utero. Intermittent runs of ventricular rates between 170 and 400 bpm exceed the atrial rate, and there is complete dissociation between atrial and ventricular events.9 If the AV node is able to conduct retrograde to the atrium, a 1:1 AV relationship is possible. In the latter situation, ventricular tachycardia becomes difcult to differentiate from re-entrant SVT. Long QT syndrome should be suspected when there is fetal bradycardia during sinus rhythm, intermittent or permanent complete heart block, or both. Sinus tachycardia is characterized by atrial rates of 180e200 bpm, 1:1 AV conduction, normal duration of the AV interval and some variability of the heart rate. It is caused by a variety of fetal and maternal conditions including fetal distress, anaemia, infections, maternal b-stimulation and fetal thyrotoxicosis secondary to thyroid-stimulating maternal

auto-antibodies (Fig. 3B). The importance of sinus tachycardia is recognizing and treating the underlying cause without delay. Atrial utter is the result of an intra-atrial electrical macro-re-entrant circuit around xed or functional anatomical conduction barriers. Atrial utter is mainly observed later in gestation as the propagation of a sustained re-entrant circuit depends on a critical atrial size.30 Atrial rates range between 300 and 550 bpm which is sufciently fast so that 1:1 AV conduction is not occurring. Typically only every second or third atrial beat is conducted, which results in better tolerated ventricular rates between 150 and 250 bpm (Fig. 2). Fetal atrial utter may also be associated with re-entrant SVT.31,33,34 Management of atrial utter aims to reverse atrial utter to sinus rhythm or otherwise to impair the AV conduction, thus reducing the ventricular response rate to a near-normal rate, allowing better ventricular lling and improved cardiac output. In terms of restoration to sinus rhythm, maternal administration of sotalol appears to be more effective than digoxin.31,35 Once sinus rhythm has been established after birth, recurrence of atrial utter is uncommon in the absence of structural heart disease and no anti-arrhythmic long-term prophylaxis is usually required.31 The term supraventricular tachycardia encompasses tachycardias with three different mechanisms including: (1) AV re-entrant tachycardia

Fetal brady- and tachyarrhythmias: New and accepted diagnostic and treatment methods

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Figure 8 Impact of transplacental fetal treatment with (Steroid) and without (No Steroid) dexamethasone on outcome of isolated CAVB. The survival was best with a protocol-guided approach that was introduced in 1997 (Tx Protocol). Reproduced with permission from Jaeggi ET, Fouron JC, Silverman ED, Ryan G, Smallhorn J, Hornberger LK. Circulation 2004;110:1542e8.30

(AVRT) related to retrograde fast accessory pathway conduction; (2) permanent junctional reciprocating tachycardia (PJRT) related to retrograde slow accessory pathway conduction; and (3) atrial ectopic tachycardia (AET) due to enhanced focal automaticity. SVT mechanisms may be quite accurately differentiated based on their arrhythmia pattern including the AV and ventriculo-atrial (VA) relationship. AV re-entry is by far the most common mechanism of fetal SVT.2,36 During typical orthodromic AVRT, there is normal AV conduction through the AV node, while an accessory pathway conducts retrograde from the ventricles to the atria. As the retrograde atrial activation occurs shortly after the ventricular activation, this yields a short VA time interval on Doppler or M-mode echocardiography (Fig. 9). Other features of short VA SVT include

intermittent or sustained episodes of regular fast heart rates between 180 and 300 bpm and 1:1 AV conduction. The onset and termination of AVRT is sudden. After birth, antegrade accessory pathway conduction (Wolff-Parkinson-White syndrome) is found in 10% of cases with fetal SVT.32,33 Long VA SVT is characterized by atrial contraction that closely precedes the ventricular systole. Apart from sinus tachycardia, this pattern is typically caused by AET or PJRT. AET and PJRT are far less common but more resistant to treatment compared with short VA SVT.2 In PJRT, the retrograde (VA) conduction occurs through a slow conducting accessory pathway. This results in sustained long VA tachycardia of about 220 bpm with a 1:1 AV relationship. In AET, an atrial focus exceeds the normal pacemaker activity of the sinus node. AET is mainly sustained due to multiple runs of fast heart rates between 200 and 250 bpm. The tachycardia may have a gradual onset and offset, and variable second-degree AV block may occur. In theory, three management options are available for fetal SVT and atrial utter: (1) no treatment; (2) anti-arrhythmic drug therapy; and (3) delivery. Abstention of treatment with close pregnancy monitoring is a valid option if the fetus presents with intermittent brief runs of tachycardia in the absence of haemodynamic impairment. More sustained tachyarrhythmia may signicantly affect the fetal cardiovascular function due to impaired ventricular lling, reduced cardiac contractility and venous congestion. In this situation, rapid and permanent conversion to sinus rhythm to prevent or resolve congestive heart failure is a primary task. Nevertheless, the choice of treatment is contentious as there are no controlled data that document the superiority of any antiarrhythmic drug treatment for fetal and paediatric tachyarrhythmia. There is, however, considerable non-randomized experience in the transmaternal treatment of fetal SVT with a number of antiarrhythmic agents, including digoxin,2,5,33 procainamide (class Ia),37 ecainide (class Ic),33,38,39 sotalol and amiodarone (class III).2,5,35,40e42 Direct fetal administration of adenosine, digoxin and/or amiodarone, e.g. into the umbilical vein, has been used for the acute treatment of incessant, poorly tolerated re-entrant SVT.43e45 Digoxin is still considered by many as drug of choice in treating fetal SVT. However, the likelihood of controlling arrhythmias within a reasonable time frame is rather low,46,47 and digoxin is almost certainly ineffective in long VA SVT.2 Moreover, in the presence of fetal hydrops, maternal-fetal digoxin transfer is poor and effective fetal drug

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Figure 9 Tissue velocity imaging of a short ventriculo-atrial tachyarrhythmia with a heart rate of 230 beats/min. Right atrial systole (A) occurs at the very end of ventricular systole (S). Atrioventricular re-entry as supraventricular tachycardia mechanism was conrmed by postnatal transoesophageal electrophysiological study.

levels may not even be obtained at near-toxic maternal levels.47 The Hospital for Sick Children has used the following approach for fetal SVT for some years now (unpublished data). If a fetus presents with signicant SVT after 35 weeks of gestation, expedited delivery followed by postnatal conversion is an option. At an earlier gestational age, the risks associated with premature delivery probably outweigh the potential hazards of fetal-maternal pharmacological treatment. Oral sotatol (80e 160 mg bid) is used as the drug of choice if there is sustained or incessant short VA SVT, long VA SVT or atrial utter. Sotalol is usually well tolerated and has little or no negative inotropic effect on the fetal heart.48 Anti-arrhythmic treatment other than with digoxin is always initiated in hospital after the maternal documentation of a normal 12-lead ECG and normal serum electrolytes. The fetal and maternal cardiac rhythm is intermittently monitored during the rst 2 days of treatment, and no serious maternal adverse effects have occurred. If the arrhythmia is not controlled within a few days on the maximal sotalol doses, digoxin is usually added (oral doses of 1 mg/day for 2 days, followed by maintenance doses). Using this approach, permanent suppression of SVT is achievable in most (80%) cases. Oral ecainide (100 mg tid) is reserved for cases that are unresponsive to sotalol and digoxin. In situations of life-threatening

arrhythmia-related fetal compromise, direct fetal administration of adenosine and amiodarone is used as a last resort. Transient neonatal hypothyroidism has been reported after fetal amiodarone exposure.42,49 Occasional reports of fetal deaths have raised concerns regarding the safety of antiarrhythmic drugs such as ecainide and sotalol, although the demise could never be attributed with certainty to the drug therapy.35,38 Although data on the natural history of signicant SVT are largely missing, the benet of anti-arrhythmic treatment in reducing arrhythmia-related complications and death probably outweighs the low pro-arrhythmia risk. Once the arrhythmia is under control, the successful anti-arrhythmic treatment is maintained until delivery with weekly controls of the fetal cardiac rhythm. At the authors centre, newborns are treated (usually with a b-blocker) for at least 6e 12 months, if the SVT: (1) spontaneously recurs early after birth; or (2) is easily inducible by transoesophageal electrophysiological study.

Summary
Fetal echocardiography is essential in understanding arrhythmia mechanisms, to clarify the impact on the fetal circulation and to establish the need for treatment. A large amount of experience on anti-arrhythmic therapy has been accumulated

Fetal brady- and tachyarrhythmias: New and accepted diagnostic and treatment methods from retrospective analysis of paediatric and, less importantly, fetal data. Well-tolerated anti-arrhythmic drugs are available for the successful treatment of most fetal tachyarrhythmias. Unfortunately, direct comparison of efcacy and safety between different studies is not feasible because variable criteria for therapy success are used, and inclusion criteria are vague in terms of underlying arrhythmia mechanism. Preventive treatment of pregnancies at risk of isolated CAVB appears to be unjustied based on a 1e2% fetal risk in women with anti-Ro/La antibodies. The outcome of isolated fetal CAVB may be improved by transplacental treatment with dexamethasone and b-stimulation at heart rates !55 bpm. This and alternative treatment approaches require further study.

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