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Outcome of Fetuses with Heart Disease Diagnosed in Utero

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Outcome of Fetuses with Heart Disease Diagnosed in Utero Archives of Disease in Childhood 1997;77:F41–F46 F41 Arch Dis Child Fetal Neonatal Ed: first published as 10.1136/fn.77.1.F41 on 1 July 1997. Downloaded from Outcome of fetuses with heart disease diagnosed in utero Marianne Eronen Abstract Notable exceptions are the atrial septal defect, Objective—To review the outcomes of 193 because of the extreme thinness and the fetuses with cardiac abnormalities de- presence of the patent foramen ovale of the tected by echocardiography. atrial septum in the fetus, and the arterial duct, Methods—A total of 422 fetuses between 16 which is a normal finding. Some defects can and 41 gestational weeks, referred to pae- also evolve or progress during fetal life, making diatric cardiologists for detailed echocar- a definitive diagnosis early in gestation diography, were included in this study. diYcult.7 Prenatal diagnosis not only allows for Results—Structural heart defects were planned management of the heart disease pre- found in 55 (28%), isolated arrhythmia in natally and postnatally, but also allows the 105 (54%), and other non-structural ab- families to consider the option of termination normalities (dilated cardiomyopathy, of pregnancy for severe defects. hypertrophic cardiomyopathy, aneurysm Echocardiographic assessment of fetal ar- of the foramen ovale, isolated pericardial rhythmias is usually accomplished using eVusion or echogenic foci) in 33 (17%) of M-mode and Doppler techniques. Echocardio- 193 fetuses. Total mortality was 26%. The graphy not only establishes the kind of arrhyth- prognosis was poor in fetuses with struc- mia present but can also identify associated tural heart defects; 37 of 55 cases (67%) structural and functional heart disease as well. died in utero or postnatally. Chromosomal Fetal congestive heart failure is diagnosed abnormality was associated with struc- when tissue perfusion of the fetus is inad- tural heart defect in 38% of fetuses, of equate, characterised by cardiomegaly, atriov- whom 38% died. Among fetuses with entricular valve regurgitation, and abnormal isolated arrhythmia survival was 95%. venous velocities.8 The presence of fetal Poor outcome was associated with com- congestive heart failure is also characterised by plete heart block (n=14) in 2 (14%) fetuses fetal hydrops in association with either tachy- with hydrops and heart rate of less than 55 cardia or bradyarrhythmia, and results in a very per minute, and with supraventricular poor prognosis.9–13 tachycardia (n=21) in three (14%) ne- onates delivered prematurely at a mean gestational age of 33 weeks. Furthermore, Methods nine of 12 fetuses (75%) with structural The study group comprised 422 fetuses http://fn.bmj.com/ heart defects and arrhythmia died. between 16 and 41 weeks gestational age that Among fetuses with non-structural car- had been referred to a paediatric cardiologist diac abnormalities, survival was 73%. for detailed echocardiology between January Poor outcome was evident in fetuses with 1983 and December 1995 at the Children´s dilated cardiomyopathy in eight of 13 hospital, University of Helsinki. The indica- (62%) and with hypertrophic cardiomy- tions for referral were: a strong family history of opathy in one of eight (13%) of cases. congenital heart disease; fetal heart arrhyth- on September 24, 2021 by guest. Protected copyright. Conclusions—Factors associated with a mia; abnormal four-chamber view; extracar- poor prognosis were: structural heart diac malformation; fetal hydrops; and other defect associated with chromosomal ab- conditions in which the risk of cardiac normality or arrhythmia, congestive heart abnormality was increased—for example, ma- failure associated with supraventricular ternal diabetes, chromosomal abnormality, or tachycardia or complete heart block, espe- cardiac teratogen. cially if delivery occurs preterm; and fetal The fetal heart was examined using two hydrops with congestive heart failure and dimensional guided, pulsed, and continuous atrioventricular valve regurgitation. wave Doppler equipment (Advanced Technol- (Arch Dis Child 1997;77:F41–F46) ogy Laboratories 600 MK, Toshiba SSH 65 A Hospital for Children and Acuson 128 Color Doppler system) with a and Adolescents, Keywords: Echocardiography; outcome; arrhythmia; 3.5 MHz or 5 MHz transducer. Two dimen- Division of Pediatric structural heart defects sional imaging was obtained from five ap- Cardiology, University of Helsinki, proaches including four-chamber, five- Stenbackinkatu 11, chamber, long axis, short axis and arch views. 00290 Helsinki, Echocardiography is the only imaging modality The studies were judged to be of adequate Finland M Eronen available for diagnosis of structural heart diagnostic quality if the following were defined: defect, arrhythmias, and abnormal cardiac cardiac rhythm; cardiac situs; ventricular and Correspondence to: haemodynamics in the fetus.1–5 In trained atrial chambers; valves; and the aortic and pul- Dr Marianne Eronen. hands, fetal echocardiography can now detect monary arterial connections to the heart and Accepted 30 January 1997 most congenital heart lesions at 16–20 weeks.56 aortic and ductal arches. Colour and pulsed or F42 Eronen Arch Dis Child Fetal Neonatal Ed: first published as 10.1136/fn.77.1.F41 on 1 July 1997. Downloaded from Table 1 Indications for fetal echocardiography and results No (%) of fetuses with abnormal condition (n=193) No (%) of Structural congenital Non-structural cardiac Indication fetuses (n=422) heart disease abnormality Family history 145 (34) 3 (2) 5 (3) Previous child 126 Maternal 15 Other 4 Arrhythmia 144 (34) 7 (5) 106 (74) Abnormal four-chamber view 40 (9) 28 (70) 2 (5) Fetal hydrops 17 (4) 1 (6) 9 (53) Maternal diabetes 17 (4) 0 (0) 5 (29) Extracardiac malformations 12 (3) 4 (33) 2 (17) Chromosomal abnormality 10 (2) 6 (60) 0 (0) Small for gestational age 8 (2) 3 (38) 0 (0) Pregnancy induced hypertension 7 (2) 0 (0) 0 (0) Polyhydramnios 7 (2) 1 (14) 3 (43) Miscellaneous 15 (4) 2 (13) 6 (40) Twin-twin transfusion 8 0 4 Oligohydramnios 1 1 0 Thoracopagus 1 1 0 Acardiac fetus 1 0 1 Maternal hyperthyroidism 1 0 0 Exposure to ketoprofen 1 0 0 Exposure to lithium 1 0 0 Maternal HIV, antiviral medication 1 0 1 Total 422 55 (13) 138 (33) continuous wave Doppler echocardiography Results were carried out when a clinically significant Among the 422 fetuses examined by a paediat- structural defect was suspected by imaging. ric cardiologist, 193 (46%) had cardiac abnor- The type of arrhythmia was analysed using an malities. Of the 40 mothers referred because of M-mode echocardiogram at the atrial and ven- the obstetrician suspecting a structural heart tricular levels. The non-structural cardiac defect, 28 (70%) were confirmed as abnormal. abnormalities were identified as dilatated The reasons for the referral and the findings are cardiomyopathy, hypertrophic cardiomyopa- shown in table 1. thy, aneurysm of the foramen ovale, and The commonest indications for referral were isolated pericardial eVusion or echogenic foci. a family history of complete heart disease All studies were performed using the standard (34%) and arrhythmias (34%). Three fetuses transabdominal approach. The abnormal stud- (2%) scanned for family history were found to ies were recorded on videotape for subsequent have structural heart defects. All cases were playback and analysis. siblings. Of the 34 families with a history of left Personal data were recorded for each heart hypoplasia (LHH), one (3%) fetus had mother. After counselling, she returned to the the same defect. Five mothers also had fetuses referring hospital for further care unless it was with arrhythmia (three with premature atrial http://fn.bmj.com/ felt that the fetus would benefit from ongoing contractions and two with sinus bradycardia). combined cardiac/obstetric management, in Among 55 fetuses with structural heart which case, management was continued at the defect, poor outcome was seen in 37 cases Children’s Hospital. The progress and out- (67%). The type of heart defect, presence or come of each pregnancy (including those absence of chromosomal abnormality, treat- where no abnormality was detected) were ment and outcome of the aVected fetuses are followed up, and where appropriate, post mor- described in table 2. Total fetal mortality was on September 24, 2021 by guest. Protected copyright. tem examinations were performed. In most of 15%; four fetuses were aborted and four these cases the heart was examined at the Chil- fetuses died in utero. Twenty nine of the 55 dren’s Hospital. Follow up lasted 12 months. (53%) died neonatally (table 2). A total of 12 Table 2 Structural fetal heart disease, treatment, and outcome Operated No (%) of Chromosomal Fetal death Neonatal Diagnosis patients abnormality (aborted) death < 1 month of age 1-3 months of age > 3 months of age Ventricualr septal defect 12 (22) 11 3 (1) 6 0 1 2 Left heart hypoplasia 11 (20) 2 1 (1) 10 0 0 0 Atrioventriculoseptal defect 8 (15) 5 1 (1) 0 1 1 5 Univentricular heart 7 (13) 1 2 (1) 3 2 0 0 Double outlet right venticle 3 (5) 1 0 2 2 0 0 Truncus arteriosus 2 (4) 1 1 1 0 0 0 Tetralogy of Fallot 2 (4) 0 0 0 0 0 2 Intracardiac tumour 2 (4) 0 0 1 1 0 0 Pulmonary stenosis 2 (4) 0 0 1 1 0 0 Ectopia cordis 1 (2) 0 0 1 1 0 0 Single heart, conjoined twins 1 (2) 0 0 1 0 0 0 Ebstein’s anomaly 1 (2) 0 0 1 0 0 0 Endocardial fibroelastosis 1 (2) 0 0 1 0 0 0 Mitral valve dysplasia 1 (2) 0 0 1 0 0 0 Atrial septal defect 1 (2) 0 0 0 0 0 1 Total 55 21 8 (4) 29 8 2 10 F43 Outcome of fetuses with heart disease diagnosed in utero Arch Dis Child Fetal Neonatal
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