Abstract
Amaç: Başta sistemik lupus eritematozus (SLE) ve Sjögren sendromlu (SS) olmak üzere annelerinde bağ dokusu hastalığı olan yenidoğanlarda kardiyak iletim sistemi etkilenmekte ve kalp blokları görülebilmektedir. Bu durumdan annenin dolaşımındaki anti-SS-A (Ro), anti- SS-B (La) antikorları sorumludur. Bu çalışmada hastanemizde bağ dokusu hastalığı olan annelerden doğan bebeklerin klinik özellikleri ve uzun dönem takip sonuçlarının değerlendirilmesi amaçlanmıştır.
Gereç ve Yöntemler: Yenidoğan yoğun bakım ünitesinde Ocak 2001 ile Ocak 2016 arasında annelerinde SLE, SS veya bağ dokusu hastalığı tanısı olması nedeniyle yatırılarak izlenen hastalar geriye dönük olarak tarandı, demografik ve klinik özellikleri ile elektrokardiyografik bulguları kayıt edildi.
Bulgular: Çalışmaya toplam 48 anneden 49 bebek alındı. Annelerin doğum sırasında ortalama yaşı 30,8±5,0 yıl (28-41), hastaların ortalama gestasyonel haftası 35,8±2,5 hafta (28-41), ortalama doğum ağırlığı ise 2614±680 gr (730-3810 gr) idi. On yenidoğanda (%20,4) 3. derece atrioventriküler (AV) blok, bir bebekte ise 1. derece AV blok tespit edildi. Beş hastaya yenidoğan döneminde kalıcı kalp pili takıldı, bu hastalardan ikisi yenidoğan döneminde yaşamını yitirdi. AV blok ile takip edilen bir hastaya ise 6. ayında kalp pili takıldı. Kardiyak iletim sorunu olmayan bir bebek ise prematüriteye bağlı nedenler ile yaşamını yitirdi. Yaşayan 46 bebeğin ortalama takip süresi 4,6±3,1 (1,2-10,75) yıl, bunlardan AV tam bloklu olup kalp pili takılmadan izlenen hastaların takip süresi 5,6±2 yıl idi.
Sonuç: Yenidoğan döneminde görülen tam AV blok hızlı müdahale edilmesi gereken ciddi bir kardiyak problemdir. Bilinen bağ dokusu hastalığı olan anne adayları üçüncü basamak merkezlerde izlenmeli, fetüs yakın takip edilmelidir. Gereken yenidoğanlara kalp pili takılarak normal yaşamlarına dönmeleri mümkündür.
References
- Hernstadt H, Regan W, Bhatt H, Rosenthal E, Meau-Petit V. Cohort study of congenital complete heart block among preterm neonates: a single-center experience over a 15-year period. Eur J Pediatr 2022;181:1047-54.
- Wainwright B, Bhan R, Trad C, Cohen R, Saxena A, Buyon J, et al. Autoimmune-mediated congenital heart block. Best Pract Res Clin Obstet Gynaecol 2020 ;64:41-51.
- Pruetz JD, Miller JC, Loeb GE, Silka MJ, Bar-Cohen Y, Chmait RH. Prenatal diagnosis and management of congenital complete heart block. Birth Defects Res 2019;111:380-8
- Clowse MEB, Eudy AM, Kiernan E, Williams MR, Bermas B, Chakravarty E, et al. The prevention, screening and treatment of congenital heart block from neonatal lupus: a survey of provider practices. Rheumatology (Oxford) 2018;57:v9-v17.
- Brito-ZerónP,Pasoto SG, Robles-Marhuenda A, Mandl T, Vissink A, Armagan B, et al. Autoimmune congenital heart block and primary Sjögren’s syndrome: characterisation and outcomes of 49 cases. Clin Exp Rheumatol 2020;38:95-102.
- Saxena A, Izmirly PM, Mendez B, Buyon JP, Friedman DM. Prevention and treatment in utero of autoimmune associated congenital heart block. Cardiol Rev 2014; 22:263–7.
- Pilar Brito-Zerón, Peter M. Izmirly, Manuel Ramos-Casals, Jill P. Buyon, and Munther A. Khamashta. The clinical spectrum of autoimmune congenital heart block. Nat Rev Rheumatol 2015; 11: 301–12.
- Hornberger LK, Al Rajaa N. Spectrum of Cardiac Involvement in Neonatal Lupus. Scandinavian Journal of Immunology 2010;72: 189–97.
- Jaeggi ET, Silverman ED, Laskin C, Kingdom J, Golding F, Weber R. Prolongation of the atrioventricular conduction in fetuses exposed to maternal anti-Ro/SSA and anti-La/SSB antibodies did not predict progressive heart block. A prospective observational study on the effects of maternal antibodies on 165 fetuses. J Am Coll Cardiol 2011;57:1487e92.
- Butt S, Kiran S, Qadir N ,Menghani D, Tanzeem H. Cardiac Conduction Defects in Systemic Lupus Erythematosus. Cureus 2020;12:e10882.
- Cimaz R, Spence DL, Hornberger L, Silverman ED. Incidence and spectrum of neonatal lupus erythematosus: a prospective study of infants born to mothers with anti-Ro autoantibodies. J Pediatr 2003;142:678-83.
- Erden A, Fanouriakis A, Kiliç L, Sari A, Armağan B, Bilgin E, Şener YZ, et al. Geoepidemiology and clinical characteristics of neonatal lupus erythematosus: a systematic literature review of individual patients’ data.Turk J MedSci 2020;50:281-90.
- Julkunen H, Eronen M. Long-term outcome of mothers of children with isolated heartblock in Finland. Arthritis Rheum 2001;44:647-52.
- Shah MJ, Silka MJ, Silva JNA, Balaji S, Beach CM, Benjamin MN et al. 2021 PACES expert consensus statement onthe indications and management of cardiovascular implantable electronic devices in pediatric patients. Cardiol Young 2021;31:1738-69.
- Clowse MEB, Eudy AM, Kiernan E, Williams MR, Bermas B, Chakravarty E, Sammaritano LR et al. The prevention, screening and treatment of congenital heart block from neonatal lupus: a survey of provider practices. Rheumatology (Oxford) 2018;57:v9-v17.
- Mota RB, Santos NR, Éden P, Rodrigues M, Brito I, Soares H. Lupus Pneumonitis: Case Report of a Rare Manifestation of Neonatal Lupus. J Investig Med High Impact Case Rep 2022;10: 23247096221077815.
- Erden A, Bilgin E, Armağan B, Sarı A, Ceylan S, Şener YZ, et al. Evaluation of hematological, neurological, hepatic and pulmonary involvements of neonatal lupus erythematosus: patient-based systematic literature review. Med J Ankara Tr Res Hosp 2020;53:120-4.
Abstract
Objective: Cardiac conduction system is affected and heart blocks can be seen in newborns whose mothers have connective tissue disease, especially with systemic lupus erythematosus (SLE) and Sjögren's syndrome (SS). Anti-SS-A (Ro), anti-SS-B (La) antibodies in the mother's circulation are responsible for this situation. In this study, it was aimed to evaluate the clinical features and long-term follow-up results of babies born to mothers with connective tissue disease in our hospital.
Materials and methods: Patients who were hospitalized in the neonatal intensive care unit between January 2001 and January 2016 due to the diagnosis of SLE, SS or connective tissue disease in their mothers were retrospectively screened, and their demographic and clinical characteristics and electrocardiographic findings were recorded.
Results: A total of 49 babies from 48 mothers were included in the study. Mean age of mothers at birth was 30.8±5.0 years (28-41), mean gestational week of patients was 35.8±2.5 weeks (28-41), mean birth weight was 2614±680 g (730-3810 g). Ten newborns (20.4%) had 3rd degree atrioventricular (AV) block, and 1 baby had 1st degree AV block. Permanent pacemaker was implanted in five patients in the neonatal period, two of these patients died in the neonatal period. A pacemaker was inserted in a patient who was followed up with AV block in the 6th month. One baby who had no cardiac conduction problem died due to reasons related to prematurity. The mean follow-up period of 46 living babies was 4.6±3.1 (1.2-10.75) years, and the follow-up period of the patients with complete AV block and without a pacemaker was 5.6±2 years.
Conclusion: Complete AV block in the neonatal period is a serious cardiac problem that requires rapid intervention. Expectant mothers with known connective tissue disease should be followed in tertiary care centers and the fetus should be followed closely. It is possible for newborns in need to return to their normal lives by inserting pacemakers.
References
- Hernstadt H, Regan W, Bhatt H, Rosenthal E, Meau-Petit V. Cohort study of congenital complete heart block among preterm neonates: a single-center experience over a 15-year period. Eur J Pediatr 2022;181:1047-54.
- Wainwright B, Bhan R, Trad C, Cohen R, Saxena A, Buyon J, et al. Autoimmune-mediated congenital heart block. Best Pract Res Clin Obstet Gynaecol 2020 ;64:41-51.
- Pruetz JD, Miller JC, Loeb GE, Silka MJ, Bar-Cohen Y, Chmait RH. Prenatal diagnosis and management of congenital complete heart block. Birth Defects Res 2019;111:380-8
- Clowse MEB, Eudy AM, Kiernan E, Williams MR, Bermas B, Chakravarty E, et al. The prevention, screening and treatment of congenital heart block from neonatal lupus: a survey of provider practices. Rheumatology (Oxford) 2018;57:v9-v17.
- Brito-ZerónP,Pasoto SG, Robles-Marhuenda A, Mandl T, Vissink A, Armagan B, et al. Autoimmune congenital heart block and primary Sjögren’s syndrome: characterisation and outcomes of 49 cases. Clin Exp Rheumatol 2020;38:95-102.
- Saxena A, Izmirly PM, Mendez B, Buyon JP, Friedman DM. Prevention and treatment in utero of autoimmune associated congenital heart block. Cardiol Rev 2014; 22:263–7.
- Pilar Brito-Zerón, Peter M. Izmirly, Manuel Ramos-Casals, Jill P. Buyon, and Munther A. Khamashta. The clinical spectrum of autoimmune congenital heart block. Nat Rev Rheumatol 2015; 11: 301–12.
- Hornberger LK, Al Rajaa N. Spectrum of Cardiac Involvement in Neonatal Lupus. Scandinavian Journal of Immunology 2010;72: 189–97.
- Jaeggi ET, Silverman ED, Laskin C, Kingdom J, Golding F, Weber R. Prolongation of the atrioventricular conduction in fetuses exposed to maternal anti-Ro/SSA and anti-La/SSB antibodies did not predict progressive heart block. A prospective observational study on the effects of maternal antibodies on 165 fetuses. J Am Coll Cardiol 2011;57:1487e92.
- Butt S, Kiran S, Qadir N ,Menghani D, Tanzeem H. Cardiac Conduction Defects in Systemic Lupus Erythematosus. Cureus 2020;12:e10882.
- Cimaz R, Spence DL, Hornberger L, Silverman ED. Incidence and spectrum of neonatal lupus erythematosus: a prospective study of infants born to mothers with anti-Ro autoantibodies. J Pediatr 2003;142:678-83.
- Erden A, Fanouriakis A, Kiliç L, Sari A, Armağan B, Bilgin E, Şener YZ, et al. Geoepidemiology and clinical characteristics of neonatal lupus erythematosus: a systematic literature review of individual patients’ data.Turk J MedSci 2020;50:281-90.
- Julkunen H, Eronen M. Long-term outcome of mothers of children with isolated heartblock in Finland. Arthritis Rheum 2001;44:647-52.
- Shah MJ, Silka MJ, Silva JNA, Balaji S, Beach CM, Benjamin MN et al. 2021 PACES expert consensus statement onthe indications and management of cardiovascular implantable electronic devices in pediatric patients. Cardiol Young 2021;31:1738-69.
- Clowse MEB, Eudy AM, Kiernan E, Williams MR, Bermas B, Chakravarty E, Sammaritano LR et al. The prevention, screening and treatment of congenital heart block from neonatal lupus: a survey of provider practices. Rheumatology (Oxford) 2018;57:v9-v17.
- Mota RB, Santos NR, Éden P, Rodrigues M, Brito I, Soares H. Lupus Pneumonitis: Case Report of a Rare Manifestation of Neonatal Lupus. J Investig Med High Impact Case Rep 2022;10: 23247096221077815.
- Erden A, Bilgin E, Armağan B, Sarı A, Ceylan S, Şener YZ, et al. Evaluation of hematological, neurological, hepatic and pulmonary involvements of neonatal lupus erythematosus: patient-based systematic literature review. Med J Ankara Tr Res Hosp 2020;53:120-4.
Details
| Primary Language | English |
|---|---|
| Subjects | Internal Diseases |
| Journal Section | ORIGINAL ARTICLES |
| Authors | |
| Early Pub Date | May 3, 2023 |
| Publication Date | September 25, 2023 |
| Submission Date | December 29, 2022 |
| Published in Issue | Year 2023 Volume: 17 Issue: 5 |
The publication language of Turkish Journal of Pediatric Disease is English.
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