Journal of Obstetrics and Gynaecology, August 2014; 34: 492–494 © 2014 Informa UK, Ltd. ISSN 0144-3615 print/ISSN 1364-6893 online DOI: 10.3109/01443615.2014.914480
OBSTETRICS
Feto-maternal outcome in pregnancies complicated by isolated fetal congenital complete heart block K. K. Roy, M. Subbaiah, S. Kumar, J. B. Sharma & N. Singh
J Obstet Gynaecol Downloaded from informahealthcare.com by Nyu Medical Center on 06/19/15 For personal use only.
Department of Obstetrics and Gynecology, All India Institute of Medical Sciences (AIIMS), New Delhi, India
A retrospective analysis of eleven pregnancies complicated by isolated fetal congenital complete heart block (CCHB) in anti-SSA/Ro antibody positive women was carried out at a tertiary hospital in India to study the perinatal outcome. The mean gestational age at the time of detection of fetal CCHB was 24.5 ⴞ 3.1weeks. Six mothers were asymptomatic; two had Sjögren’s syndrome and three had systemic lupus erythematosus. Oral dexamethasone was given to all the patients after the diagnosis was made. There was one case of intrauterine death. Seven (63.6%) neonates needed a permanent pacemaker. There was no significant difference in the perinatal outcome in asymptomatic women with fetal CCHB and in women with connective tissue disorder and fetal CCHB. To conclude, fetal CCHB is associated with high morbidity but the presence of underlying connective disorder in the mother does not worsen the prognosis of the affected neonate. Keywords: Anti-SSA/Ro antibody, congenital heart block, connective tissue disorder
Introduction Congenital complete heart block (CCHB) is a rare disorder with an incidence of about one in 22,000 liveborn infants (Buyon and Clancy 2005). Isolated CCHB occurs in fetuses with structurally normal hearts around 16–24 weeks’ gestation. It has been associated with maternal connective tissue diseases like systemic lupus erythematosus (SLE) and Sjögren’s syndrome, and the transplacental passage of anti-SSA/Ro and anti-SSB/La antibodies from affected mother to fetus has been implicated in its pathogenesis (Tincani et al. 2006). Although most of the mothers with affected fetus have these antibodies, almost half of them do not have any manifestation of connective tissue disorders (Rivera et al. 2009). Many of them subsequently develop connective tissue disorders. It is not known whether perinatal outcome in these asymptomatic patients with fetal CCHB is better than those with connective tissue disorder and fetal CCHB. We carried out this retrospective study to study the perinatal outcome in this rare condition and to compare the outcome between the two groups.
Methods The medical records of all cases in which CCHB was diagnosed in utero in our hospital between July 2008 and July 2013 were reviewed. All pregnant women who were positive for anti-SSA/Ro
or anti-SSB/La antibodies and in whom CCHB was detected in utero by fetal echocardiography were included in the study. The exclusion criteria were structural cardiac anomalies, mothers who tested positive for IgM antitoxoplasma, herpes or rubella virus and cytomegalovirus in pregnancy. Details regarding maternal age, parity, past obstetric and medical history, primary rheumatological diagnosis, medications taken during the pregnancy and their dosages, were noted. Anti-SSA/Ro or anti-SSB/La antibody levels were tested using ELISA. Details of fetal echocardiograms and anomaly scan were reviewed to note the degree of heart block, presence of pericardial effusion, endomyocardial fibroelastosis or other evidence suggestive of hydrops fetalis. Antenatal complications, mode of delivery, complications at delivery and in the postpartum period and perinatal outcome were reviewed. These women were contacted by telephone to assess their present condition. The women were divided into two groups: asymptomatic women with fetal CCHB and those with connective tissue disorder and fetal CCHB. Maternal and fetal outcomes were compared between these two groups. The categorical variables between the two groups were compared using Fisher’s exact test and Wilcoxon rank-sum test was used for continuous variables. A p value of ⬍ 0.05 was considered statistically significant.
Results A total of eleven cases of CCHB were diagnosed in our hospital during the study period. The baseline characteristics of the study group are summarised in Table I. Six mothers were asymptomatic; two had Sjögren’s syndrome and three SLE. Connective tissue disease was diagnosed before the index pregnancy in these five patients. Four (36.3%) were anti-SSB/La-positive. There was no history of CCHB in previous pregnancies in any of these patients. Table I. Baseline characteristics of patients (n ⫽ 11). Maternal characteristics Age of the patients (years) (mean ⫾ SD) BMI (kg/m2) (mean ⫾ SD) Obstetric history Primigravida Underlying connective tissue disorder Systemic lupus erythematosus Sjögren’s syndrome Asymptomatic patients
n
(%) 28.3 ⫾ 3.6 23.1 ⫾ 1.4
4
36.3
3 2 6
27.3 18.2 54.5
Correspondence: M. Subbaiah, Department of Obstetrics and Gynecology, Room No 3076, Third floor, Teaching block, All India Institute of Medical Sciences (AIIMS), Ansarinagar, New Delhi, India-110029. E mail: [email protected]
Isolated fetal congenital complete heart block 493 Table II. Perinatal outcome in pregnancies complicated by fetal congenital complete heart block. Perinatal outcome
n
Mean gestational age at detection (weeks) Mean fetal heart rate at diagnosis (bpm) Mean gestational age at delivery (weeks) Mean birth weight (kg) Mean heart rate at birth (bpm) Mean Apgar 5 min Need for permanent pacemaker Mean age at pacemaker insertion, days Intrauterine death Small for gestational age fetus
(%) 24.5 ⫾ 3.1 60.4 ⫾ 8.2 36.5 ⫾ 2.5 2.63 ⫾ 0.5 63.4 ⫾ 8.9 8.51 ⫾ 1.83
7
63.6 87.7 ⫾ 128.2 1 9 2 18.1
features of SLE. The mean follow-up period was 4.2 ⫾ 2.2 years. The other four women were still asymptomatic and are being followed-up. The perinatal outcome in asymptomatic women with fetal CCHB and in women with connective tissue disorder and fetal CCHB were compared. In the asymptomatic group, four (66.7%) neonates required a pacemaker compared with three (60%) in the connective tissue group (p ⫽ 1). There was one intrauterine death in the connective tissue group compared with none in the asymptomatic group (p ⫽ 0.45). The difference in mean fetal heart rate at birth, age at pacemaker insertion, gestational age at delivery and birth weight between the two groups was also found to be statistically insignificant.
bpm, beats/min.
J Obstet Gynaecol Downloaded from informahealthcare.com by Nyu Medical Center on 06/19/15 For personal use only.
Discussion The mean gestational age at the time of diagnosis was 24.5 ⫾ 3.1 weeks (Table II). CCHB was diagnosed in utero in all these eleven cases and none of these reverted to a lesser degree. Five (45.4%) cases were female. There was one case of intrauterine death. CCHB was detected in this pregnancy at 25 weeks’ gestation. The patient was diagnosed to have SLE, 2 years before. She was not on any medication for the disease. Ultrasound showed cardiomegaly and other features of cardiac failure (hydrops) in the fetus. Oral dexamethasone 4 mg daily was started at 25 weeks. However, intrauterine death was detected at 28 weeks. Pericardial effusion, endomyocardial fibroelastosis or other evidence suggestive of hydrops fetalis, was not seen in any other fetus. Seven (63.6%) neonates were given a pacemaker; all were alive at the end of the follow-up period (Table III). Intrauterine treatment with oral dexamethasone 4 mg daily was given to all these patients after the diagnosis was made. None of the patients received prophylactic steroids, beta-adrenergic agonists, plasmapheresis, intravenous immunoglobulin or hydroxychloroquine. The incidence of past miscarriage in these patients was 12% but none of them had history of recurrent miscarriages. None of the pregnancies were complicated by pre-eclampsia, gestational diabetes or hypothyroidism. None of the patients with connective tissue disorders had any flare up in pregnancy. Anti-inflammatory drugs and steroids were not needed in any of these patients during pregnancy for treatment of the underlying connective tissue disorder. Caesarean section was performed in three (27%) pregnant women, for obstetric indications. During follow-up, it was found that two of the six initially asymptomatic women had developed
CCHB is an uncommon disorder but is associated with high neonatal morbidity. Almost 65% of newborns may require a pacemaker and the mortality rate can be as high as 20% (Friedman et al. 2007). Brucato et al. (2009) studied the outcome of 34 newborns presenting with CCHB and born to anti-SSA/Ro positive mothers. There were two cases of sudden death in utero and three died immediately after birth due to heart failure. A total of 26 neonates (72.2%) required a pacemaker. In our study, there was one case of intrauterine death and 63.6% of the neonates required a pacemaker. Although only about 2% of mothers with anti-SSA/Ro and anti-SSB/La antibodies will have a child with CCHB, the risk of recurrence of CCHB in a subsequent pregnancy can be as high as 16% (Eronen et al. 2000). Various therapeutic agents, such as steroids, sympathomimetics, plasmapheresis, hydroxy chloroquine and intravenous immunoglobulin have been used for intrauterine treatment of congenital heart block (Miyoshi et al. 2012). However, the utility of these agents is still controversial, especially if the heart block is complete. In CCHB, the potential of these agents to diminish an inflammatory fetal response in the conduction system is plausible. Jaeggi et al. (2004) reported 90% survival rates in patients treated with transplacental steroids compared with 46% in the untreated group in pregnancies complicated by CCHB. In our study, all the patients received transplacental steroids and the survival rate was 91%. Maternal outcome in these pregnancies is generally good. Presence of anti-SSA/Ro and anti-SSB/La antibodies has not been found to be associated with adverse maternal events, such as pre-eclampsia and preterm labour (Brucato et al. 2002). However,
Table III. Clinical characteristics of infants with isolated congenital complete heart block. Underlying maternal disease SLE 1. 2. 3. Sjögren’s syndrome 4. 5. Asymptomatic 6. 7. 8. 9. 10. 11.
Gestational age at detection (weeks)
Fetal HR at diagnosis (bpm)
Gestational age at delivery (weeks)
HR at birth (bpm)
Age at permanent pacing
Current outcome
24 25 22
68 58 74
34 – 38
62 – 70
3 months No 3 months
Alive Died in utero Alive
21 24
63 54
39 37
66 58
No 2 days
Alive Alive
24 22 23 25 28 32
56 62 70 50 48 62
38 40 34 36 32 37
58 62 82 60 48 68
4 days 2 months No 1 year 3 days No
Alive Alive Alive Alive Alive Alive
HR, heart rate; bpm, beats/min; SLE, systemic lupus erythematosus.
J Obstet Gynaecol Downloaded from informahealthcare.com by Nyu Medical Center on 06/19/15 For personal use only.
494
K. K. Roy et al.
there may be higher risk of miscarriage in these pregnancies (Brucato et al. 2011). There may be higher incidence of caesarean delivery due to difficulty in monitoring fetal wellbeing during labour by fetal heart rate. Fetal scalp blood sampling and fetal pulse oximetry have been used in some studies to help in intrapartum monitoring (Sherman and Featherstone 1997; Amano et al. 1997). In our study, we used continuous electronic monitoring to monitor the baseline fetal heart rate. Fetal scalp blood sampling was done if there was any persistent or recurrent deceleration in the fetal heart rate. Many of the asymptomatic mothers eventually develop features of connective tissue disorders. Rivera et al. (2009) in a study reported that 26 out of 51 initially asymptomatic mothers developed features of a connective tissue disease on follow-up. The median time to develop any symptom was 3.15 years. In our study, 33.3% of the six initially asymptomatic mothers developed features of SLE. Clinical examination of these patients instead of telephone follow-up, can perhaps increase the rate of detection of connective tissue disorder. Factors associated with poor prognosis in the neonate include: hydrops fetalis, low heart rate (⬍ 50–55 beats/min), male sex, endocardial fibroelastosis, valvular dysfunction, dilated cardiomyopathy, low birth weight and preterm delivery (Eronen et al. 2000). We do not know if maternal characteristics such as the presence of underlying connective tissue can affect the prognosis of the neonate with CCHB. There have been no studies comparing the perinatal outcome in asymptomatic women with fetal CCHB and in women with connective tissue disorder and fetal CCHB. In our study, we compared the perinatal outcome between these two groups but did not find any statistically significant difference. But our study is limited by its small sample size and retrospective design. A larger prospective study is needed to address this issue. To conclude, CCHB is associated with high morbidity and the use of transplacental steroids may improve the survival of affected neonates. Presence of underlying connective disorder in the mother does not worsen the prognosis of the affected neonate. However, our results need to be confirmed in larger prospective studies.
Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
References Amano K, Hirano S, Maeda M, Nishijima M. 1997. Should C-section be applied to fetal arrhythmia? European Journal of Obstetrics, Gynecology, and Reproductive Biology 72:S73–S79. Brucato A, Cimaz R, Caporali R, Ramoni V, Buyon J. 2011. Pregnancy outcomes in patients with autoimmune diseases and anti-Ro/SSA antibodies. Clinical Reviews in Allergy and Immunology 40:27–41. Brucato A, Doria A, Frassi M, Castellino G, Franceschini F, Faden D et al. 2002. Pregnancy outcome in 100 women with autoimmune diseases and anti-Ro/SSA antibodies: a prospective controlled study. Lupus 11: 716–721. Brucato A, Grava C, Bortolati M, Ikeda K, Milanesi O, Cimaz R et al. 2009. Congenital heart block not associated with anti-Ro/La antibodies comparison with anti-Ro/La-positive cases. Journal of Rheumatology 36:1744–1748. Buyon JP, Clancy RM. 2005. Neonatal lupus: basic research and clinical perspectives. Rheumatic Diseases Clinics of North America 31:299–313. Eronen M, Siren MK, Ekblad H, Tikanoja T, Julkunen H, Paavilainen T. 2000. Short- and long-term outcome of children with congenital complete heart block diagnosed in utero or as a newborn. Pediatrics 106:86–91. Friedman DM, Rupel A, Buyon JP. 2007. Epidemiology, etiology, detection, and treatment of autoantibody-associated congenital heart block in neonatal lupus. Current Rheumatology Reports 9:101–108. Jaeggi ET, Fouron JC, Silverman ED, Ryan G, Smallhorn J, Hornberger LK. 2004. Transplacental fetal treatment improves the outcome of prenatally diagnosed complete atrioventricular block without structural heart disease. Circulation 110:1542–1548. Miyoshi T, Maeno Y, Sago H, Inamura N, Yasukohchi S, Kawataki M et al. 2012. Evaluation of transplacental treatment for fetal congenital bradyarrhythmia: nationwide survey in Japan. Circulation Journal 76: 469–476. Rivera TL, Izmirly PM, Birnbaum BK, Byrne P, Brauth JB, Katholi M et al. 2009. Disease progression in mothers of children enrolled in the Research Registry for Neonatal Lupus. Annals of the Rheumatic Diseases 68: 828–835. Sherman SJ, Featherstone LS. 1997. Congenital complete heart block and successful vaginal delivery. Journal of Perinatology 17:489–491. Tincani A, Rebaioli CB, Taglietti M, Shoenfeld Y. 2006. Heart involvement in systemic lupus erythematosus, antiphospholipid syndrome and neonatal lupus. Rheumatology 45:8–13.
OBSTETRICS
Feto-maternal outcome in pregnancies complicated by isolated fetal congenital complete heart block K. K. Roy, M. Subbaiah, S. Kumar, J. B. Sharma & N. Singh
J Obstet Gynaecol Downloaded from informahealthcare.com by Nyu Medical Center on 06/19/15 For personal use only.
Department of Obstetrics and Gynecology, All India Institute of Medical Sciences (AIIMS), New Delhi, India
A retrospective analysis of eleven pregnancies complicated by isolated fetal congenital complete heart block (CCHB) in anti-SSA/Ro antibody positive women was carried out at a tertiary hospital in India to study the perinatal outcome. The mean gestational age at the time of detection of fetal CCHB was 24.5 ⴞ 3.1weeks. Six mothers were asymptomatic; two had Sjögren’s syndrome and three had systemic lupus erythematosus. Oral dexamethasone was given to all the patients after the diagnosis was made. There was one case of intrauterine death. Seven (63.6%) neonates needed a permanent pacemaker. There was no significant difference in the perinatal outcome in asymptomatic women with fetal CCHB and in women with connective tissue disorder and fetal CCHB. To conclude, fetal CCHB is associated with high morbidity but the presence of underlying connective disorder in the mother does not worsen the prognosis of the affected neonate. Keywords: Anti-SSA/Ro antibody, congenital heart block, connective tissue disorder
Introduction Congenital complete heart block (CCHB) is a rare disorder with an incidence of about one in 22,000 liveborn infants (Buyon and Clancy 2005). Isolated CCHB occurs in fetuses with structurally normal hearts around 16–24 weeks’ gestation. It has been associated with maternal connective tissue diseases like systemic lupus erythematosus (SLE) and Sjögren’s syndrome, and the transplacental passage of anti-SSA/Ro and anti-SSB/La antibodies from affected mother to fetus has been implicated in its pathogenesis (Tincani et al. 2006). Although most of the mothers with affected fetus have these antibodies, almost half of them do not have any manifestation of connective tissue disorders (Rivera et al. 2009). Many of them subsequently develop connective tissue disorders. It is not known whether perinatal outcome in these asymptomatic patients with fetal CCHB is better than those with connective tissue disorder and fetal CCHB. We carried out this retrospective study to study the perinatal outcome in this rare condition and to compare the outcome between the two groups.
Methods The medical records of all cases in which CCHB was diagnosed in utero in our hospital between July 2008 and July 2013 were reviewed. All pregnant women who were positive for anti-SSA/Ro
or anti-SSB/La antibodies and in whom CCHB was detected in utero by fetal echocardiography were included in the study. The exclusion criteria were structural cardiac anomalies, mothers who tested positive for IgM antitoxoplasma, herpes or rubella virus and cytomegalovirus in pregnancy. Details regarding maternal age, parity, past obstetric and medical history, primary rheumatological diagnosis, medications taken during the pregnancy and their dosages, were noted. Anti-SSA/Ro or anti-SSB/La antibody levels were tested using ELISA. Details of fetal echocardiograms and anomaly scan were reviewed to note the degree of heart block, presence of pericardial effusion, endomyocardial fibroelastosis or other evidence suggestive of hydrops fetalis. Antenatal complications, mode of delivery, complications at delivery and in the postpartum period and perinatal outcome were reviewed. These women were contacted by telephone to assess their present condition. The women were divided into two groups: asymptomatic women with fetal CCHB and those with connective tissue disorder and fetal CCHB. Maternal and fetal outcomes were compared between these two groups. The categorical variables between the two groups were compared using Fisher’s exact test and Wilcoxon rank-sum test was used for continuous variables. A p value of ⬍ 0.05 was considered statistically significant.
Results A total of eleven cases of CCHB were diagnosed in our hospital during the study period. The baseline characteristics of the study group are summarised in Table I. Six mothers were asymptomatic; two had Sjögren’s syndrome and three SLE. Connective tissue disease was diagnosed before the index pregnancy in these five patients. Four (36.3%) were anti-SSB/La-positive. There was no history of CCHB in previous pregnancies in any of these patients. Table I. Baseline characteristics of patients (n ⫽ 11). Maternal characteristics Age of the patients (years) (mean ⫾ SD) BMI (kg/m2) (mean ⫾ SD) Obstetric history Primigravida Underlying connective tissue disorder Systemic lupus erythematosus Sjögren’s syndrome Asymptomatic patients
n
(%) 28.3 ⫾ 3.6 23.1 ⫾ 1.4
4
36.3
3 2 6
27.3 18.2 54.5
Correspondence: M. Subbaiah, Department of Obstetrics and Gynecology, Room No 3076, Third floor, Teaching block, All India Institute of Medical Sciences (AIIMS), Ansarinagar, New Delhi, India-110029. E mail: [email protected]
Isolated fetal congenital complete heart block 493 Table II. Perinatal outcome in pregnancies complicated by fetal congenital complete heart block. Perinatal outcome
n
Mean gestational age at detection (weeks) Mean fetal heart rate at diagnosis (bpm) Mean gestational age at delivery (weeks) Mean birth weight (kg) Mean heart rate at birth (bpm) Mean Apgar 5 min Need for permanent pacemaker Mean age at pacemaker insertion, days Intrauterine death Small for gestational age fetus
(%) 24.5 ⫾ 3.1 60.4 ⫾ 8.2 36.5 ⫾ 2.5 2.63 ⫾ 0.5 63.4 ⫾ 8.9 8.51 ⫾ 1.83
7
63.6 87.7 ⫾ 128.2 1 9 2 18.1
features of SLE. The mean follow-up period was 4.2 ⫾ 2.2 years. The other four women were still asymptomatic and are being followed-up. The perinatal outcome in asymptomatic women with fetal CCHB and in women with connective tissue disorder and fetal CCHB were compared. In the asymptomatic group, four (66.7%) neonates required a pacemaker compared with three (60%) in the connective tissue group (p ⫽ 1). There was one intrauterine death in the connective tissue group compared with none in the asymptomatic group (p ⫽ 0.45). The difference in mean fetal heart rate at birth, age at pacemaker insertion, gestational age at delivery and birth weight between the two groups was also found to be statistically insignificant.
bpm, beats/min.
J Obstet Gynaecol Downloaded from informahealthcare.com by Nyu Medical Center on 06/19/15 For personal use only.
Discussion The mean gestational age at the time of diagnosis was 24.5 ⫾ 3.1 weeks (Table II). CCHB was diagnosed in utero in all these eleven cases and none of these reverted to a lesser degree. Five (45.4%) cases were female. There was one case of intrauterine death. CCHB was detected in this pregnancy at 25 weeks’ gestation. The patient was diagnosed to have SLE, 2 years before. She was not on any medication for the disease. Ultrasound showed cardiomegaly and other features of cardiac failure (hydrops) in the fetus. Oral dexamethasone 4 mg daily was started at 25 weeks. However, intrauterine death was detected at 28 weeks. Pericardial effusion, endomyocardial fibroelastosis or other evidence suggestive of hydrops fetalis, was not seen in any other fetus. Seven (63.6%) neonates were given a pacemaker; all were alive at the end of the follow-up period (Table III). Intrauterine treatment with oral dexamethasone 4 mg daily was given to all these patients after the diagnosis was made. None of the patients received prophylactic steroids, beta-adrenergic agonists, plasmapheresis, intravenous immunoglobulin or hydroxychloroquine. The incidence of past miscarriage in these patients was 12% but none of them had history of recurrent miscarriages. None of the pregnancies were complicated by pre-eclampsia, gestational diabetes or hypothyroidism. None of the patients with connective tissue disorders had any flare up in pregnancy. Anti-inflammatory drugs and steroids were not needed in any of these patients during pregnancy for treatment of the underlying connective tissue disorder. Caesarean section was performed in three (27%) pregnant women, for obstetric indications. During follow-up, it was found that two of the six initially asymptomatic women had developed
CCHB is an uncommon disorder but is associated with high neonatal morbidity. Almost 65% of newborns may require a pacemaker and the mortality rate can be as high as 20% (Friedman et al. 2007). Brucato et al. (2009) studied the outcome of 34 newborns presenting with CCHB and born to anti-SSA/Ro positive mothers. There were two cases of sudden death in utero and three died immediately after birth due to heart failure. A total of 26 neonates (72.2%) required a pacemaker. In our study, there was one case of intrauterine death and 63.6% of the neonates required a pacemaker. Although only about 2% of mothers with anti-SSA/Ro and anti-SSB/La antibodies will have a child with CCHB, the risk of recurrence of CCHB in a subsequent pregnancy can be as high as 16% (Eronen et al. 2000). Various therapeutic agents, such as steroids, sympathomimetics, plasmapheresis, hydroxy chloroquine and intravenous immunoglobulin have been used for intrauterine treatment of congenital heart block (Miyoshi et al. 2012). However, the utility of these agents is still controversial, especially if the heart block is complete. In CCHB, the potential of these agents to diminish an inflammatory fetal response in the conduction system is plausible. Jaeggi et al. (2004) reported 90% survival rates in patients treated with transplacental steroids compared with 46% in the untreated group in pregnancies complicated by CCHB. In our study, all the patients received transplacental steroids and the survival rate was 91%. Maternal outcome in these pregnancies is generally good. Presence of anti-SSA/Ro and anti-SSB/La antibodies has not been found to be associated with adverse maternal events, such as pre-eclampsia and preterm labour (Brucato et al. 2002). However,
Table III. Clinical characteristics of infants with isolated congenital complete heart block. Underlying maternal disease SLE 1. 2. 3. Sjögren’s syndrome 4. 5. Asymptomatic 6. 7. 8. 9. 10. 11.
Gestational age at detection (weeks)
Fetal HR at diagnosis (bpm)
Gestational age at delivery (weeks)
HR at birth (bpm)
Age at permanent pacing
Current outcome
24 25 22
68 58 74
34 – 38
62 – 70
3 months No 3 months
Alive Died in utero Alive
21 24
63 54
39 37
66 58
No 2 days
Alive Alive
24 22 23 25 28 32
56 62 70 50 48 62
38 40 34 36 32 37
58 62 82 60 48 68
4 days 2 months No 1 year 3 days No
Alive Alive Alive Alive Alive Alive
HR, heart rate; bpm, beats/min; SLE, systemic lupus erythematosus.
J Obstet Gynaecol Downloaded from informahealthcare.com by Nyu Medical Center on 06/19/15 For personal use only.
494
K. K. Roy et al.
there may be higher risk of miscarriage in these pregnancies (Brucato et al. 2011). There may be higher incidence of caesarean delivery due to difficulty in monitoring fetal wellbeing during labour by fetal heart rate. Fetal scalp blood sampling and fetal pulse oximetry have been used in some studies to help in intrapartum monitoring (Sherman and Featherstone 1997; Amano et al. 1997). In our study, we used continuous electronic monitoring to monitor the baseline fetal heart rate. Fetal scalp blood sampling was done if there was any persistent or recurrent deceleration in the fetal heart rate. Many of the asymptomatic mothers eventually develop features of connective tissue disorders. Rivera et al. (2009) in a study reported that 26 out of 51 initially asymptomatic mothers developed features of a connective tissue disease on follow-up. The median time to develop any symptom was 3.15 years. In our study, 33.3% of the six initially asymptomatic mothers developed features of SLE. Clinical examination of these patients instead of telephone follow-up, can perhaps increase the rate of detection of connective tissue disorder. Factors associated with poor prognosis in the neonate include: hydrops fetalis, low heart rate (⬍ 50–55 beats/min), male sex, endocardial fibroelastosis, valvular dysfunction, dilated cardiomyopathy, low birth weight and preterm delivery (Eronen et al. 2000). We do not know if maternal characteristics such as the presence of underlying connective tissue can affect the prognosis of the neonate with CCHB. There have been no studies comparing the perinatal outcome in asymptomatic women with fetal CCHB and in women with connective tissue disorder and fetal CCHB. In our study, we compared the perinatal outcome between these two groups but did not find any statistically significant difference. But our study is limited by its small sample size and retrospective design. A larger prospective study is needed to address this issue. To conclude, CCHB is associated with high morbidity and the use of transplacental steroids may improve the survival of affected neonates. Presence of underlying connective disorder in the mother does not worsen the prognosis of the affected neonate. However, our results need to be confirmed in larger prospective studies.
Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
References Amano K, Hirano S, Maeda M, Nishijima M. 1997. Should C-section be applied to fetal arrhythmia? European Journal of Obstetrics, Gynecology, and Reproductive Biology 72:S73–S79. Brucato A, Cimaz R, Caporali R, Ramoni V, Buyon J. 2011. Pregnancy outcomes in patients with autoimmune diseases and anti-Ro/SSA antibodies. Clinical Reviews in Allergy and Immunology 40:27–41. Brucato A, Doria A, Frassi M, Castellino G, Franceschini F, Faden D et al. 2002. Pregnancy outcome in 100 women with autoimmune diseases and anti-Ro/SSA antibodies: a prospective controlled study. Lupus 11: 716–721. Brucato A, Grava C, Bortolati M, Ikeda K, Milanesi O, Cimaz R et al. 2009. Congenital heart block not associated with anti-Ro/La antibodies comparison with anti-Ro/La-positive cases. Journal of Rheumatology 36:1744–1748. Buyon JP, Clancy RM. 2005. Neonatal lupus: basic research and clinical perspectives. Rheumatic Diseases Clinics of North America 31:299–313. Eronen M, Siren MK, Ekblad H, Tikanoja T, Julkunen H, Paavilainen T. 2000. Short- and long-term outcome of children with congenital complete heart block diagnosed in utero or as a newborn. Pediatrics 106:86–91. Friedman DM, Rupel A, Buyon JP. 2007. Epidemiology, etiology, detection, and treatment of autoantibody-associated congenital heart block in neonatal lupus. Current Rheumatology Reports 9:101–108. Jaeggi ET, Fouron JC, Silverman ED, Ryan G, Smallhorn J, Hornberger LK. 2004. Transplacental fetal treatment improves the outcome of prenatally diagnosed complete atrioventricular block without structural heart disease. Circulation 110:1542–1548. Miyoshi T, Maeno Y, Sago H, Inamura N, Yasukohchi S, Kawataki M et al. 2012. Evaluation of transplacental treatment for fetal congenital bradyarrhythmia: nationwide survey in Japan. Circulation Journal 76: 469–476. Rivera TL, Izmirly PM, Birnbaum BK, Byrne P, Brauth JB, Katholi M et al. 2009. Disease progression in mothers of children enrolled in the Research Registry for Neonatal Lupus. Annals of the Rheumatic Diseases 68: 828–835. Sherman SJ, Featherstone LS. 1997. Congenital complete heart block and successful vaginal delivery. Journal of Perinatology 17:489–491. Tincani A, Rebaioli CB, Taglietti M, Shoenfeld Y. 2006. Heart involvement in systemic lupus erythematosus, antiphospholipid syndrome and neonatal lupus. Rheumatology 45:8–13.
