Fetal supraventricular tachycardia diagnosed and treated at twenty-four weeks of gestation and after birth: a case report
Romeo E, D’Alto M, Russo MG, Sarubbi B, Cardaropoli D, Paladini D, Pacileo G, Annunziata A, CalabrÃ² R
Chair of Cardiology, Second University of Naples, Naples, Italy
Supraventricular tachycardia is the most common clinically significant fetal tachycardia. The diagnosis is usually made at routine sonographic workup during the second-third trimester of pregnancy. Treatment goals are cardioversion to sinus rhythm and reversal of cardiac dysfunction. We describe a case of fetal supraventricular tachycardia diagnosed at 24 weeks of gestation. The first-line treatment was oral maternal digoxin and sotalol. This therapy was not sufficient for complete control of the tachycardia. Hence, second-line treatment with digoxin and flecainide was started and successfully achieved conversion to sinus rhythm. No adverse maternal side effects were noted during the 14 weeks of therapy. A normal male infant was delivered at elective cesarean section performed for obstetric indications at 38 weeks of gestation. A persistent junctional reciprocating tachycardia with a ventriculo-atrial/atrioventricular ratio > 1 was diagnosed following delivery at transesophageal electrophysiological study. At the age of 8 months the child is on therapy with sotalol (4 mg/kg/day) and flecainide (3 mg/kg/day) and is in good clinical conditions.
Italian Heart Journal 2004; 5 : 777-780
Prevalence and prognosis of atrial septal aneurysm in high risk fetuses without structural heart defects
Papa M, Fragasso G, Camesasca C, Di Turi RP, Spagnolo D, Valsecchi L, Calori G, Margonato A
Division of Cardiology, Scientific Institute/University San Raffaele, Milan, Italy
The aim of this study was to evaluate the prevalence and prognostic implications of the association between atrial septal aneurysm (ASA) and fetal arrhythmias in a population of high risk fetuses.
METHODS. One thousand three hundred and two fetal echocardiograms performed during high risk pregnancies from the 17th to the 41st week of gestation were retrospectively evaluated for the presence of ASA and/or arrhythmias. An ASA was defined as redundant tissue extending at least halfway across the left atrium. Patients with an ASA were distinguished in two subgroups according to whether there was (subgroup 1) or was not (subgroup 2) cyclical contact of the atrial septum with the left atrial wall or with the mitral valve. Arrhythmias were documented during mono/two-dimensional echocardiography and Doppler evaluation.
RESULTS. Out of 1223 patients considered for the study, 93 (7.6%) fetuses had an ASA; among these 93 fetuses, 33 (36%) had premature atrial beats (p < 0.001). Ten of these patients were included in subgroup 1, and 7 of them (70%) exhibited premature atrial beats (p = 0.016 vs subgroup 2). No arrhythmias other than premature atrial beats were observed in these patients. None of them received any therapy during observation. All of them developed a regular sinus rhythm within 3 months of life.
CONCLUSIONS. On the basis of these data, we can speculate that, if accurately searched for, ASA is often present (7.6%) and is likely to represent a mechanical stimulus for the generation of premature atrial beats. Indeed, our data show an important correlation between the degree of bulging and the presence of arrhythmias, supporting the hypothesis of a mechanical stimulus. However, the observed arrhythmias did not appear to be prone to degeneration. In conclusion, ASA observed during fetal life is often associated with premature atrial beats, which are apparently in direct relation with the degree of bulging of the atrial septum. On the other hand, an ASA almost invariably disappears at birth and is not associated with major arrhythmias.
Italian Heart Journal 2002; 3 : 318-321
Right Ventricular Pre-ejection Myocardial Velocity and Myocardial Acceleration in Normal Fetuses Assessed by Doppler Tissue Imaging
Kenji Harada, MD, Masaki Ogawa, MD, and Toshinobu Tanaka, MD, Akita, Japan
Myocardial acceleration during isovolumic contraction obtained from Doppler tissue imaging has been introduced as an index of right ventricular contractile function that is unaffected by the shape of the ventricle and loading conditions, but normal value of myocardial acceleration during isovolumic contraction and the effect of aging on the index are not known in normal fetuses.
We studied 61 normal fetuses aged 20 to 39 weeks (29.8 + – 5.1 weeks). Fetuses were divided
into 4 age groups: 20 to 24 weeks (n = 11); 25 to 29 weeks (n = 20); 30 to 34 weeks (n = 20); and 35 to 39 weeks (n = 10). Using Doppler tissue imaging, peak pre-ejection myocardial velocity was measured at the base of right ventricular free wall from 4-chamber view. Myocardial acceleration was calculated by dividing pre-ejection velocity by the time interval from onset of the pre-ejection myocardial velocity to the time at peak velocity of this wave.
The mean preejection myocardial velocity was 5.0 + – 1.1 cm/s. There was a stepwise increase in the pre-ejection myocardial velocity from the fetuses of 20 to 24 weeks to the fetuses of 35 to 39 weeks. The mean myocardial acceleration was 160 + – 30 cm/s2. The mean myocardial acceleration did not differ between the fetuses aged 20 to 24 weeks (139 + – 13 cm/s2) and the fetuses aged 25 to 29 weeks (143 + – 21 cm/s2), but after 30 weeks increased with gestational age. For the total group
combining the 4 different gestational age groups, the pre-ejection myocardial velocity and myocardial acceleration correlated with gestational age (r = 0.85 and 0.75). This study demonstrated the gestational agerelated changes in pre-ejection myocardial velocity and myocardial acceleration. The load-independent index of contractility, myocardial acceleration, increased mainly after 30 weeksâ€™ gestation.
(J Am Soc Echocardiogr 2005;18:370-4.)
Fetal Doppler echocardiographic diagnosis and successful steroid therapy of Luciani-Wenckebach phenomenon and endocardial fibroelastosis related to maternal anti-Ro and anti-La antibodies
Marie-JosÃ©e Raboisson, MD a 1 – Jean-Claude Fouron, MD a – Sven-Erik Sonesson, MD b –
Margareta Nyman, MD c – Francine Proulx, RT a – Sylvie Gamache, RT a
Complete fetal heart block (HB) and endocardial fibroelastosis (EFE) are known to be associated with maternal anti-Ro and anti-La antibodies. Complete fetal HB is irreversible.
We sought to (1) assess the value of the superior vena cava/ascending aorta Doppler approach in the early detection of abnormal delay in the fetal atrioventricular (AV) time of conduction, before appearance of complete fetal HB; and (2) report the effect of prenatal steroid therapy on EFE, HB, or both.
The clinical history, echocardiographic, and Doppler investigations of 3 fetuses and children born to mothers positive for anti-Ro and anti-La antibodies are reported. Two fetuses presented with EFE either isolated (29 weeks) or associated with AV block (25 weeks). In this last case, the superior vena cava/ascending aorta approach allowed the identification of a Luciani-Wenckebach phenomenon. In a third fetus, 2:1 AV block was noted at 23 weeks of gestation. Dexamethasone (4 mg/day) was administered to all 3 patients. Complete regression of the EFE and conduction abnormalities was documented in all cases.
Early prenatal detection of abnormal delay in fetal AV time conduction is possible with the Doppler superior vena cava/ascending aorta approach. Steroid therapy can cure fetal EFE and AV conduction delays associated with maternal anti-Ro and anti-La antibodies.
Publishing and Reprint Information
a – Fetal Cardiology Unit, Department of Pediatrics, St-Justine Hospital, University of Montreal, Montreal, Quebec, Canada
b – Pediatric Cardiology Service, Astrid Lindgren Childrenâ€™s Hospital, Stockholm, Sweden
c- Department of Obstetrics, Danderyd Hospital, Stockholm, Sweden
1 – Dr Raboisson is currently affiliated with the Louis Pradel Cardiovascular and Pulmonary Hospital of Lyon, France.
Reprint requests: Jean-Claude Fouron, MD, Fetal Cardiology Unit, St-Justine Hospital, 3175, CÃ´te Ste-Catherine, Montreal, Quebec, H3T 1C5 Canada
Email address: firstname.lastname@example.org (Jean-Claude Fouron)
Copyright Â© 2005 by American Society of Echocardiography
[Echogenic intracardiac structures (golf ball phenomenon) as predictors of chromosome anomalies]
Bettelheim D, Ulm MR, Deutinger J, Bernaschek G.
Universitatsklinik fur Frauenheilkunde, Abteilung fur Pranatale Diagnostik und Therapie, Wien.
Small echogenic areas in the fetal heart are known as the golf ball phenomenon. These structures are considered by some to be a marker for chromosomal anomalies.
AIM : To prospectively study the relationship of echogenic intracardiac structures and chromosomal aberrations.
METHODS : Over a 15 month period (6/96-9/97) 4500 unselected fetuses between 16-31 weeks were screened for malformations; in each case echogenic intracardiac structures were sought.
RESULTS: In 77 cases (1.17%) single or multiple echogenic punctate intracardiac structures could be diagnosed. In 60 fetuses (78%) chromosome analysis was performed. Two (3.3%) had chromosomal abnormalities–trisomy 21 and 45.XO/46.XX.
The golf ball phenomenon appears to be a normal variation in the development of the papillary muscle.
This sign is usually easily to identify and if present, should lead to a more detailed screening for anomalies.
In the case of other sonographic abnormalities, with advanced maternal age or with a positive triple test, a chromosomal analysis should be performed.
Arrhythmogenic Right Ventricular Dysplasia :
Echocardiographic Findings in Patients
Meeting Task Force Criteria for Arrhythmogenic Right Ventricular Dysplasia
New Insights From the Multidisciplinary Study of Right Ventricular Dysplasia
Danita M. Yoerger, MD,* Frank Marcus, MD,â€ Duane Sherrill, PHD,â€ Hugh Calkins, MD,â€¡
Jeffery A. Towbin, MD,Â§ Wojciech Zareba, MD, PHD, Michael H. Picard, MD,*
Multidisciplinary Study of Right Ventricular Dysplasia Investigators
Boston, Massachusetts; Tucson, Arizona; Baltimore, Maryland; Houston, Texas; and Rochester, New York
The purpose of this study was to quantify the echocardiographic abnormalities in probands
who were newly diagnosed with arrhythmogenic right ventricular dysplasia (ARVD).
BACKGROUND The diagnosis of ARVD remains challenging. The Multidisciplinary Study of Right
Ventricular Dysplasia was initiated to characterize the cardiac structural, clinical, and genetic
aspects of ARVD.
Detailed echocardiograms were performed in 29 probands and compared with echoes from 29
normal control patients matched for age, gender, body size, and year of echo. Right atrial
(RA) and right ventricular (RV) chamber dimensions, RV regional function, and the presence
of morphologic abnormalities (hyper-reflective moderator band, trabecular derangement, and
sacculations) were assessed. The RV systolic function was calculated as RV fractional area
The RV dimensions were significantly increased, and RV FAC was significantly decreased in
probands versus control patients (27.2 16 mm vs. 41.0 7.1 mm, p 0.0003).
The right ventricular outflow tract (RVOT) was the most commonly enlarged dimension in
ARVD probands (37.9 6.6 mm) versus control patients (26.2 4.9 mm, p 0.00001).
A RVOT long-axis diastolic dimension 30 mm occurred in 89% of probands and 14% of
controls. The RV morphologic abnormalities were present in many probands (trabecular
derangement in 54%, hyper-reflective moderator band in 34% and sacculations in 17%) but
not in controls.
Probands with ARVD have significant RA and RV enlargement and decreased RV function,
which can be easily assessed on standard echocardiographic imaging. These parameters
should be measured when ARVD is suspected and compared with normal values.
Journal of the American College of Cardiology Vol. 45, No. 6, 2005
by the American College of Cardiology Foundation ISSN 0735-1097/05/$30.00
Published by Elsevier Inc. doi:10.1016/j.jacc.2004.10.070
Criteria for Diagnosis of Right Ventricular Dysplasia (1)
I. Global and/or regional dysfunction and structural alterations*
- Severe dilatation and reduction of right ventricular ejection fraction
with no (or only mild) left ventricular impairment
- Localized right ventricular aneurysms (akinetic or dyskinetic areas
with diastolic bulging)
- Severe segmental dilatation of the right ventricle
- Mild global right ventricular dilatation and/or ejection fraction
reduction with normal left ventricle
- Mild segmental dilatation of the right ventricle
Regional right ventricular hypokinesia
II. Tissue characterization of wall
- Fibrofatty replacement of myocardium on endomyocardial biopsy
III. Repolarization abnormalities
- Inverted T waves in right precordial leads (V2 and V3) in people
age 12 yrs, in absence of right bundle branch block
IV. Depolarization/conduction abnormalities
- Epsilon waves or localized prolongation (110 ms) of the QRS
complex in right precordial leads (V1â€“V3)
- Late potentials (signal-averaged ECG)
- Left bundle branch block type ventricular tachycardia (sustained
and nonsustained) by ECG, Holter, or exercise testing
Frequent ventricular extrasystoles (1,000/24 h) (Holter)
VI. Family history
- Familial disease confirmed at necropsy or surgery
- Family history of premature sudden death (35 yrs) due to
suspected right ventricular dysplasia
- Familial history (clinical diagnosis based on present criteria)
Arrhythmogenic right ventricular dysplasia diagnosis: two major criteria or one major
and two minor criteria, or four minor criteria. *Detected by echocardiography,
angiography, magnetic resonance imaging, or radionuclide scintigraphy.
(1) – McKenna WJ, Thiene G, Nava A, et al.
Diagnosis of arrhythmogenic
right ventricular dysplasia/cardiomyopathy.
Task Force of the Working
Group Myocardial and Pericardial Disease of the European
Society of Cardiology and of the Scientific Council on Cardiomyopathies
of the International Society and Federation of Cardiology. Br
Heart J 1994;71:215â€“ 8.
Unguarded tricuspid valvar orifice in the fetus
Kenny K. Wong; Duncan I. Farquharson; Walter J. Duncan
Cardiology in the Young, October 2004, vol. 14, no. 5, pp. 557-559(3)
Publisher: Greenwich Medical Media
The unguarded tricuspid valve is uncommon. We describe herein a fetus with a grossly dilated right ventricle and atrium, with severe tricuspid and pulmonary valvar regurgitation. The right ventricle was akinetic, and no tricuspid tissue or valvar apparatus was identified. Colour Doppler showed a highly unusual retrograde flow of blood through the right heart. The pregnancy was terminated, and necropsy examination confirmed the gross dilation of the right heart chambers, with severely dysplastic valvar tissue at the right atrioventricular junction effectively giving an unguarded orifice. There was no valvar displacement, and the left heart was normal. The fetus had a normal karyotype, albeit with absent kidneys.
Ultrasound Obstet Gynecol 2004; 24: 629â€“632
Published online in Wiley InterScience (www.interscience.wiley.com). DOI: 10.1002/uog.1753
Prenatal diagnosis of Shoneâ€™s syndrome: parental counseling and clinical outcome
N. ZUCKER, A. LEVITAS and E. ZALZSTEIN
Pediatric Cardiology Unit, Department of Pediatrics, Soroka University Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel
KEYWORDS: fetal echocardiography; prenatal diagnosis; Shoneâ€™s syndrome
Objective To describe a series of fetuses diagnosed as
having Shoneâ€™s syndrome, which includes four cardiac
defects and for which there is a wide variety of clinical
presentations, surgical treatments and outcomes, and to
discuss the counseling strategy.
Methods We reviewed retrospectively the records of four
babies who were suspected prenatally to have Shoneâ€™s
Results The mean age at diagnosis was 27.5 (range,
22â€“35) weeks. A small left ventricle, mitral and aortic
annulus and ascending aorta were detected in three cases.
In three fetuses there was an abnormal mitral valve
apparatus and in two fetuses a sub-aortic membrane was
detected. Coarctation of the aorta was an impending
diagnosis in three babies. Following counseling, all
parents decided to continue the pregnancy to term.
Echocardiographic evaluation was performed soon after
birth. The mean mitral valve annulus diameter was 8.2
(range, 7â€“10) mm, and that of the aortic valve annulus
was 6 (range, 5â€“7) mm. The aortic valve was bicuspid in
all babies with an additional sub-aortic membrane in two
babies. Doppler ultrasound examination revealed highvelocity
flow through the mitral valve in three babies; two
of them had a parachute mitral valve. Coarctation repair
was performed in two babies during the first week of
life. One patient underwent mitral valvuloplasty followed
by later mitral valve replacement. All patients were alive
after a mean of 7.8 (range, 3.3â€“10.5) yearsâ€™ follow-up.
Conclusion When counseling families regarding fetal
Shoneâ€™s syndrome, it is important to emphasize the wide
variety of clinical presentations and possible outcomes.
Differentiation between hypoplastic left ventricle and
Shoneâ€™s complex is crucial and may influence the
physicianâ€™s attitude, the presentation of the case to the
family and the familyâ€™s final decision. Copyright ï›™ 2004
ISUOG. Published by John Wiley & Sons, Ltd.