ECHOGENIC FETAL HEART WITHOUT HEART BLOCK AND MATERNAL ANTI- Ro/ La ANTIBODIES POSITIVITY A LESS KNOWN ASSOCIATION

Transcription

1 ECHOGENIC FETAL HEART WITHOUT HEART BLOCK AND MATERNAL ANTI- Ro/ La ANTIBODIES POSITIVITY A LESS KNOWN ASSOCIATION DR PUNDALIK BALIGA FELLOW IN FETAL MEDICINE MEDISCAN SYSTEMS, CHENNAI

2 CASE 1 30 year old Mrs S G2P1L1 at 22 weeks POG DCDA twins with one twin demise and?hydrops in the surviving twin General history: Married life 8 years, Non consanguineous marriage, Height cm, Weight- 71.1kg, Blood group- O Positive. Obstetric history: 1 st Pregnancy- In 2014, Missed miscarriage at 2 months. 2 nd Pregnancy Present pregnancy, IVF Conception (ET transfer), own embryo Aneuploidy screening: Not done. External scans: Serial scans were done till 16 weeks - normal ( Total 4) 20/04/2017- DCDA twin gestation- 20 weeks + 3 days. Fetus A absent cardiac activity Fetus B Pericardial effusion was noted. Past Medical history: Diagnosed hypertensive a week back not on any

3

4 Hyper echogenic spots effusion FHR- 142 bpm Hd Down Aorta Inflows LVOT

5

6 Dichorionic Diamniotic Twin at gestation corresponding to a gestational age of 21 Weeks Fetus - A Intrauterine fetal demise Fetus - B Placenta - Anterior Liquor - Normal Estimated fetal weight / gms. Doppler study : Normal flow Impression MODERATE PERICARDIAL EFFUSION SITUS SOLITUS LEVOCARDIA NORMAL ATRIO-VENTRICULAR, VENTRICULO-ARTERIAL CONNECTION LEFT AORTIC ARCH HYPERECHOGENIC AREA IN THE CRUX, SUB-AORTIC AREA AND AORTIC VALVE HYPERECHOIC ENDOCARDIUM AND VALVES. Suggested review after 3 days with TORCH and antibody reports.

7 Follow up

8 Patient was advised Rheumatologist consultation Started on Tab Dexamethasone 4 mg OD and Tab Hydroxychloroquine.

9 Patient followed up every 2 weekly and Dexamethasone dose was reduced to 2 mg OD and then on alternate day in view of IUGR in Fetus B. Baby was delivered by Emergency LSCS in view of abnormal dopplers at 33 weeks in tertiary care hospital. BW- 1.2 kg Observed in NICU for 3 days Discharged in good condition. Follow up At 60 days of life readmitted in PICU with? sepsis and seizures and succumbed. SLE status of baby not evaluated.

10 CASE 2 29 yrs, G3A2 at 20weeks POG General history: Married life - 3 years, Non consanguineous marriage, Blood group- A Positive. Obstetric history: 1 st Pregnancy : In weeks - Spontaneous Miscarriage 2 nd Pregnancy: In at 5th month terminated due to complete heart block Aneuploidy screening Negative Previous scans: Serial scans were normal. Last scan at 18 weeks with Fetal echo was normal study Past Medical history: K/C/O Sjogren's syndrome with SLE, was diagnosed after her first pregnancy and was on Hydroxychloroquine sulfate, Prednisolone and Aspirin.

11 Single gestation corresponding to a gestational age of 20 Weeks Impression ECHOGENIC AREA SEEN IN THE MITRAL VALVE & AORTIC ANNULUS FETAL HEART RATE AND RHYTHM NORMAL Suggested repeat scan after 2 weeks.

12 Serially. followed up with no additional findings. Patient delivered a live term female baby. Neonatal Lupus evaluation Anti La and Anti Ro Positive (+++) Anti nuclear antibody- Positive (++) Postnatal echo report Delayed transitional circulation Large duct with bidirectional shunt Left Right Patent foramen ovale RV hypertrophy Pulmonary artery dilated Normal aortic arch Trivial TR Good contractility Follow up echo was said to be normal and the child is asymptomatic Next pregnancy she presented to us at 18 weeks POG as II opinion for Congenital heart block. We confirmed the same and she underwent termination of pregnancy.

13 Case 1 Negative Nil Anti Ro (+) Anti La (+) IgG CMV (+) IgG Rubella (+) Case 2 Positive 2 CAVB Anti Ro (+) Anti La (+) Case 3 Negative Nil Anti Ro (+) Anti La (+) IgG CMV (+) IgG Rubella (+) Case 4 Negative 1 CAVB ( Antibody positive) Anti Ro (++) Anti La (+) IgG CMV (+) IgG HSV 1 & 2 IgG Rubella (+) IgM Rubellaequivocal EFE (ENDOCARDIAL FIBROELASTOSIS) PERICARDIAL EFFUSION EFE EFE EFE SUPRAVENTRICULAR TACHYCARDIA PERICARDIAL EFFUSION VENTRICULAR DYSFUNCTION DIED POSTNATALLY SLE STATUS - NK NEONATAL LUPUS ALIVE & Asymptomatic ONGOING PREGNANCY ONGOING PREGNANCY

14 Infections -implications on fetal heart Infection Cardiac effects Toxoplasmosis Rubella Cytomegalovirus Myocarditis Pulmonary stenosis Patent ductus arteriosus Ventricular septal defect Dilated cardiomyopathy, Focal EFE Coxsackie virus Parvovirus Varicella zoster - Dilated cardiomyopathy Myocarditis Cardiac failure

15 Etiology and literature review Cardiac injury can present as myocardial dysfunction cardiomyopathy endocardial fibroelastosis (EFE) conduction abnormalities Factors such as maternal Vitamin D deficiency hypothyroidism immunological abnormalities in the fetus: Dysfunctions of toll-like receptors (TLRs) - all cause increased physiological apoptosis Leading to immune complex deposition, inflammation and fibrosis in the fetal heart

16 and CHB MATERNAL Autoimmune disease Trans placental passage of autoantibodies Fetal heart SSA RO and SSB LA proteins (fetal conduction system) Myocardium, papillary muscle, valvular tissue Effects Degrees of heart block Endocardial fibroelastosis

17 Congenital atrioventricular block (CAVB) in autoimmune disorders Incidence: 1% to 2% of live births. Recurrence risk - 14% to 18% Delayed dilated cardiomyopathy 11% cases Mortality rate: >70%, when associated with cardiac malformations 19% in immune-mediated cases.

18 Microchimerism maternally derived cells cross the placenta during pregnancy and remain in fetal tissues without being destroyed¹. Maternal anti Ro and anti La antibodies create inflammation and fibrosis, or target calcium channels on the surface of cardiomyocytes fetal heart rhythm disorders. Jaeggi et al. found that cardiac complications of NLE (Neonatal lupus erythematosus) were associated with moderate or high titers of maternal anti-ro antibodies². ¹ Kumar S. Neonatal lupus: An update. Indian J Rheumatol 2016;11: ²Jaeggi E et al. J Am Coll Cardiol 2010;55:

19

20

21 KEY MESSAGE Endocardial or Myocardial echogenicity could be the initial fetal finding in pregnant women with autoimmune disease. When there is a congenital heart block we always suspect Autoimmune disease Diagnosed autoimmune disease look for signs of cardiac inflammation ( endo-myocardial echogenecity) along with rhythm abnormalities Thorough history is of paramount importance Follow up - Stringent In- utero Fetal monitoring - Optimal delivery planning and management - Complete postnatal clinical evaluation of the baby for manifestations of lupus. - Postnatal echo should be done in all babies with maternal antibody positivity. - Maternal evaluation with Rheumatologist- further management and guidance.

22 Thank you