Scholarly article on topic 'Prenatal 2D Ultrasonic Diagnosis of Fetal Complete Atrioventricular Block and Bilateral Hydroceles with Occult Maternal SS-A'

Prenatal 2D Ultrasonic Diagnosis of Fetal Complete Atrioventricular Block and Bilateral Hydroceles with Occult Maternal SS-A Academic research paper on "Clinical medicine"

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{"anti-Ro antibody" / "bilateral hydrocele" / "fetal bradycardia" / "fetal complete atrioventricular block" / "maternal systemic lupus erythematosus" / SLE}

Abstract of research paper on Clinical medicine, author of scientific article — Chih Yao Yi, Chen Cheng Wu, Man Li Yang

Summary Objective We present a case of fetal complete atrioventricular block, diagnosed by the M mode of two-dimensional ultrasound, that was complicated with polyhydramnios and bilateral hydrocele Case Report The fetus was delivered at 38 weeks' gestation by caesarean section. Postpartum autoimmune survey disclosed positive anti-Ro antibodies (SS-A) for both the mother and the newborn. Severe neonatal bradycardia with complete atrioventricular block was identified. Conclusion Antenatal evaluation for a maternal history of autoimmune disease (especially systemic lupus erythematosus) and prevention of fetal bradycardia by reducing immune-mediated injuries on the cardiac conduction system are important.

Academic research paper on topic "Prenatal 2D Ultrasonic Diagnosis of Fetal Complete Atrioventricular Block and Bilateral Hydroceles with Occult Maternal SS-A"

-■ SHORT COMMUNICATION ■-

Prenatal 2D Ultrasonic Diagnosis of Fetal Complete Atrioventricular Block and Bilateral Hydroceles with Occult Maternal SS-A

Chih Yao Chen, Yi Cheng Wu*, Man Li Yang Department of Obstetrics and Gynecology, Taipei Veteran's General Hospital and National Yang Ming University, Taipei, Taiwan.

summary

Objective: We present a case of fetal complete atrioventricular block, diagnosed by the M mode of two-dimensional ultrasound, that was complicated with polyhydramnios and bilateral hydrocele. Case Report: The fetus was delivered at 38 weeks' gestation by caesarean section. Postpartum autoimmune survey disclosed positive anti-Ro antibodies (SS-A) for both the mother and the newborn. Severe neonatal bradycardia with complete atrioventricular block was identified.

Conclusion: Antenatal evaluation for a maternal history of autoimmune disease (especially systemic lupus erythematosus) and prevention of fetal bradycardia by reducing immune-mediated injuries on the cardiac conduction system are important. [TaiwaneseJ Obstet Gynecol 2006;45(1):56-59]

Key Words: anti-Ro antibody, bilateral hydrocele, fetal bradycardia, fetal complete atrioventricular block, maternal systemic lupus erythematosus, SLE

Introduction

Fetal bradycardia is a rare condition that is identified during prenatal ultrasound surveys, and misdiagnosis in outpatient departments is not uncommon. Fetal bradycardia at an early gestational age inevitably leads to fetal loss, and nonimmune fetal hydrops or anasarca is noticeable, while cardiac decompensation develops. Obscure clinical prenatal manifestations of fetal bradycardia can be difficult to differentiate from transient variations of fetal heart deceleration. Some cases of fetal bradycardia show complete atrioventricular block related to maternal autoimmune disease, especially systemic lupus erythematosus (SLE). Administration of an appropriate treatment is crucial in order to avoid irreversible consequences.

* Correspondence to: Dr. Yi Cheng Wu, Department of Obstetrics

and Gynecology, Veteran's General Hospital, 201, Section 2,

Shihpai Road, Beitou District, Taipei City 112, Taiwan.

E-mail: chenchihyao@yahoo.com.tw

Received: July 11, 2005

Revised: July 13, 2005

Accepted: July 14, 2005

Case Report

A 38-year-old female, gravida 3, para 2, received regular antenatal examination and had no complications until 32 weeks' gestation, at which time routine ultrasound revealed suspicious fetal bradycardia (117 bpm) and a bi-parietal diameter of 80 mm, which was consistent with gestational age. No intrauterine growth restriction or severe bradycardia was detected during subsequent examinations. Persistent fetal bradycardia (< 100 bpm) was identified after advanced sonographic examination at 37 weeks' gestation. Repeat ultrasound showed severe bradycardia (53 bpm), bilateral hydrocele, and polyhydramnios at 37 weeks' gestation (Figures 1-3). We performed caesarean section for the mother, and a male infant weighing 3,590 g, with Apgar scores of 8 and 9 at 1 and 5 minutes, was delivered. Cardiac evaluation revealed an atrial rate of 120 bpm and a ventricular rate of 62 bpm. Third-degree atrioventricular block was identified using 12-lead electrocardiography (Figure 4). The newborn was immediately sent to the neonatal intensive care unit for a transvenous pacemaker to be fitted. During admission, the heart rate was controlled at around 100-120 bpm,

Figure 1. M-mode echocardiogram recorded at 37 weeks' gestation. Complete atrioventricular block was detected (atrial rate, 109 bpm; ventricular rate, 53 bpm). A = atrial contraction; V = ventricular contraction.

Figure 2. Fetal scrotal hydrocele diagnosed by ultrasound at 37 weeks' gestation, left side picture.

Figure 3. Apparent polyhydramnios with AVI of 41.75 cm (37 weeks' gestation).

determined by the newborn's state of well-being (Figure 5). Intravenous immunoglobulin (IVIG) was administered for 4 days. However, fever was noted and blood culture showed methicillin-resistant Staphylococcus aureus on day 13, after which vancomycin was administered. The baby's parents refused fitting of a permanent pacemaker after completion of the course of antibiotics. At the last follow-up, the heart rate was 73 bpm. No developmental retardation or cyanotic change was noticed during subsequent examinations.

Discussion

Persistent fetal bradycardia is a rare condition during pregnancy, with an incidence as low as 1 in 20,000 to 25,000 live births [1-4]. However, the actual incidence must be higher when allowing for intra-uterine fetal death caused by cardiac decompensation (hydrops). It may be possible to predict abnormal fetal cardiac rhythm on detection of an irregular or slow heart rate at an early gestational age.

Most obstetricians, however, pay little attention to fetal bradycardia during early gestation. The median gestational age at referral for fetal complete heart block (CHB) is 26 weeks, but this ranges from 17 to 38 weeks' gestation [5]. If fetal bradycardia is noted, it is important to differentiate this from structural heart diseases. A previous report has revealed that more than 50% of CHB is seen in association with severe congenital heart disease

The first case of CHB in a fetus was reported in 1945

[6]. A previous report had suggested that transplacental passage of some factor present in maternal serum was responsible for this [7]. A positive association between fetal CHB and maternal collagen vascular disease has also been reported [8]. SS-A (anti-Ro antibody) and SS-B (anti-La antibody) play key roles in the association between fetal CHB and specific maternal antibodies that circulate in numerous maternal collagen vascular diseases [9-12]. In the case reported here, neither a specific maternal medical condition nor abnormal fetal development was noticed during pregnancy.

In the literature, hydrocele is commonly associated with hydrops, meconium peritonitis, maternal puerperal sepsis, and inflammation of Meckel's diverticulum [13-15]. Hydrocele is commonly identified prenatally on ultrasound during the third trimester [16]. In one study, 123 fetuses were imaged between 27 and 41 weeks' gestation. Scrotal hydrocele was identified in 19 fetuses [16], of which only one required surgery at 16 months of age. Fourteen babies were normal at postnatal follow-up, but four babies were not followed-up.

C.Y. Chen, et al

Figure 4. First electrocardiogram after birth: complete atrioventricular block in all leads, especially in long lead II. Red arrow = P wave; black arrow = QRS wave; red broken-line arrow = ventricular premature contraction.

The infant that required surgery also had hydrocephalus.

In our case, no hydrocephalus or signs of hydrops were found. Bilateral hydrocele may have resulted from mild decompensation of cardiac function, which possibly caused hydrops fetalis. Furthermore, the reported polyhydramnios could have resulted from the diuretic effect of body fluid overload. Although no hydrops was identified, it was difficult to decide how

to deliver this baby with severe bradycardia (ventricular rate, 53 bpm). We performed cesarean section to prevent further deterioration of the fetal cardiovascular system and fetal distress.

Prenatal medical therapies for fetal bradycardia are divided into two categories. One strategy is to minimize the immunologic injury to the fetal heart and the other is to increase the fetal heart rate. We did not

Figure 5. Relatively regular cardiac rhythm after placement of a transient transvenous pacemaker (~ 120 bpm).

use aggressive treatment because there was no apparent fetal distress. However, prenatal fetal therapy with maternal steroids, intravenous y-globulin, ^-mimetic agents, and plasmapheresis could have been considered. The immunosuppressive effects of steroids and intravenous y-globulin could have prevented further transplacental passage of immunoglobulin occurring after 22 weeks of gestation, which correlates with progressive immune-mediated injury to the fetal conduction system [17].

In this case, we administered IVIG for 4 days to the newborn. Unfortunately, it is difficult to assess whether the IVIG was effective or not after CHB had been diagnosed.

Investigation of the history of the mother revealed suspected symptoms and signs of rheumatoid arthritis, including morning stiffness and arthralgia. Rheumatoid factor and antinuclear antibody were positively identified about 5 years previously, and she was suspected of having rheumatoid arthritis. However, she did not continue follow-up because no further exaggerating symptoms and signs were noticed. No definite autoimmune disease was diagnosed at that time. During the admission, an autoimmune study disclosed elevated erythrocyte sedimentation rate, positive rheumatoid factor, antinuclear antibody, and anti-Ro antibody in the mother, as well as positive antinuclear antibody and anti-Ro antibody in the newborn.

Fetal bradycardia could be the first manifestation of maternal collagen vascular disease, which would not necessarily be SLE. Early administration of steroid, y-globulin, or maternal plasmapheresis could prevent further damage to the fetal cardiac conduction system, if a high level of maternal anti-Ro antibody or anti-La antibody is discovered. In our case, instead of postpartum status, we should have checked the profiles of autoimmune disease at 32 weeks' gestation when bradycardia was suspected.

In conclusion, SLE as well as other autoimmune diseases with SS-A could result in fetal CHB. Hence, antenatal evaluation for a maternal history of autoimmune disease (especially SLE) and prevention of fetal bradycardia by reducing immune-mediated injuries on the cardiac conduction system are important.

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