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University Journal of Medicine and Medical Sciences ISSN 2455-2852 Volume 2 Issue 3 2016 Congenital Complete Heart Block A Case Report GANESAPANDIAN PITCHAIMALLAIAN Department of Paediatrics, MADRAS MEDICAL COLLEGE AND GOVERNMENT GENERAL HOSPITAL be completely asymptomatic in presence of these autoimmune antibodies or may have a diagnosis of a collagen vascular disease Abstract : (eg, systemic lupuserythematosus(SLE), Congenital complete heart block or third- Sjogren syndrome). Isolated CAVB can degree congenital atrioventricular block also occur due to myocarditis and rarely (CAVB) is seen in a fetus or in a neonate hereditary conditions, such as storage disyounger than 28 days. CAVB can occur orders (eg, Hurler syndrome, Hunter synin a structurally normal heart (isolated drome). Often, no etiology is found for an CAVB) or with congenital heart disease isolated CAVB. The prognosis in complete (complex CAVB with congenital heart de- CAVB is relatively good but may be influfects). Congenital complete heartblock enced by the patient's age at presentation. occurs in approximately 1 per 15,000- Patients presenting as fetuses or at birth 20,000 live births. Structural congenital have significantly higher morbidity and heart block is rare, but with a higher pro- mortality rates than do patients presenting portion of fetal loss. Patients with iso- later in childhood. Herewith we are prelated CAVB typically have a better over- senting a rare case of congenital complete all outcome than do infants with complex heart block reported in a tertiary care hosstructural cardiac defects and or ven- pital in Chennai. tricular dysfunction. Keyword :Congenital complete heart Case report: block, congenital atrioventricular block A 10 days old full term newborn female child weighing 3 kg was referred as having Introduction: low heart rate and admitted with respiratory Isolated CAVB occurs in the absence of distress since birth. The child was delivother congenital heart defects. It is seen ered by emergency caeserian section and in association with certain autoimmune cried immediately after birth. During the anantibodies in the mother that cross the tenatal ultrasonography, fetal bradycardia placenta and damage the atrioventricular was madeout for (AV) node of the fetus The mother can An Initiative of The Tamil Nadu Dr. M.G.R. Medical University University Journal of Medicine and Medical Sciences which mother was investigated. She was positive for anti SSA(Ro) and anti SS-B(La) antibodies but was asymptomatic. There was no history of consanguinity, maternal fever, rashes, joint pain or drug intake. The patient was first in order of birth and there was no history of abortion or still birth.On examination, the child was pink and normothermic.. There was marked respiratory distress. Respiratory rate was 68/min with intercostal and subcostal retractions..Heart rate was 42 per minute. The child was warm and all peripheral pulses were palpable. Cardiovascular system examination revealed the apex beat on left side in the fourth intercostal space. A pansystolic murmur of grade III intensity was audible all over precordium. Liver was 2 cm below left costal margin and the liverspan was 6.5cm. Bilateral crepitations were heard in the chest. No other obvious congenital anomaly was und. The oxygen saturation was 94%. Chest X-ray showed levocardia with cardiomegaly. On electrocardiogram, atrial rate was 150/min, and ventricular rate was 40/ min with complete A-V dissociation. 2D-Echo revealed dilated cardiomyopathy, gross LA/ LV dilatation, mild MR, pulmonary hypertension- physiological and global hypokinesia. The baby was negative for anti SS-A and anti SS-B antibodies. The child was diagnosed as a case of complete congenital heart block without congenital heart defect (isolated CAVB). The baby was managed with warmer care, oxygen inhalation, iv fluids and Inj. Isoprenaline(0.05micrograms/kg/ min) as stop gap procedure. Baby underwent pacemaker implantation. The baby is now on followup without any specific new complaints. Discussion: The incidence of congenital complete heart block has been estimated to be about 1 out of 15,000 to 22,000 live births(4,5). There is a tendency for female preponderance in CAVB(6). Approximately 25% to 33% of all congenital complete heart blocks are associated with congenital heart disease like ventricular septal defect, endocardial cushion defect, patent ductus arteriosus, mitral incompetence, persistent foramen ovale, transposition of great arteries and Ebstein's anomaly. The most common among these associated lesions is L-transposition of the great arteries. Our case did not have any heart defects. The association of collagen vascular disease in mothers of infants with congenital complete heart block is significant(7) . About 60% of mothers who deliver children with CAVB have anti SSA and anti SSB antibodies. Mothers with SLE who have had one child with congenital heart block are at risk of having subsequent offspring with heart block (6). Maternal Lupus may not become manifest for years even after the birth of an infant with CAVB. In our case also the mother was asymptomatic. Maternal lupus is known to influence fetal and neonatal outcomes and is associated with increased incidence of obstetric complications such as stillbirth, abortion, prematurity, intrauterine growth restriction (IUGR), and neonatal complications such as neonatal lupus erythematosus syndrome, which is characterized by transient lupus dermatitis, hepatic and haematological abnormalities like hemolytic anemia, leucopenia, thrombocytopenia and/or isolated CAVB,CCF, endocardial fibroelastosis (8). Skin rash, hepatitis and thrombocytopenia generally resolve without sequel. By contrast, the heart block is permanent and An Initiative of The Tamil Nadu Dr. M.G.R. Medical University University Journal of Medicine and Medical Sciences requires a pacemaker in about 66% of cases (8). The presence of anti SS-A or SS-B antibodies in mothers and neonates with complete heart block has been clearly documented(9). These antibodies are IgG against SSA and SSB ribonucleoproteins, can cross the placenta and appear in the fetus at around the 16th week of gestation and affect the conduction system, cause sclerosis of the AV node. All fetuses are not affected. Other factors that could be responsible for the development of congenital complete heart block are HLA type, timing of antibody transfer, and inutero environment. The presence of certain HLA types (HLA-DR3, B8, DRW52, and DQW2) in the mother and fetus increase the possibility of complete heart block. The clinical features of the neonate depend on the effect of heart rate on cardiac output. At birth, the infant may present with congestive heart failure, anasarca, hepatomegaly and metabolic acidosis requiring emergency pacing. Isolated CAVB is only rarely accompanied by CCF(6) which was observed in our case. It is becoming increasingly recognised that dilated cardiomyopathy(DCM) either fetal or postnatal is a rare but serious outcome of autoimmune congenital complete heart block. Our case had dilated cardiomyopathy. The cause of the cardiomyopathy is unclear but may be related to an autoimmune myocarditis occurring inutero rather than primarily caused by bradycardia.(6) The SSA/Ro and SSB/La antibodies have been suggested to be a major determinant (10,11) in the patients with CAVB with DCM. Evidence for a potential immunopathologic role of the SSA/Ro and SSB/La antibodies in CAVB is described in several studies (12,13). Immunouorescent studies have shown IgG and IgM deposition throughout the myocardium on postmortem examination(14,15).However, affected newborns often appear asymptomatic and may have accelerated ventricular rates approaching those of healthy newborns. An associated finding in isolated CAVB may be the presence of discoid skin lesions. Our case did not have any skin lesions. Children with structural heart defects may present with cyanosis, failure to thrive or recurrent pneumonias or may be completely asymptomatic in childhood (such as children with L-transposition of the great arteries and intact ventricular septum). Prolonged QTc is not a constant finding in congenital block but is common with associated malformations and symptomatic patients. In asymptomatic patients, prolonged QTc may herald the onset of symptoms(16). There is an approximately 95% twenty year survival of patients without anatomic heart defect(17). After birth, electrocardiography (ECG) is recommended to assess for CAVB and to assess the QT interval that can be prolonged. Neonatal assessment should include a measurement of antiRo and anti-La antibody levels. Echocardiography should be performed initially and in periodic follow-up care in affected fetuses, infants and children to assess ventricular function and size and to rule out congenital or acquired cardiac malformations or valve dysfunction(18). In utero management of fetuses with transuterine fetal cardiac pacing have been tried without success(19).Administration of steroids, immunoglobulins andplasmapheresis in the mother are useful in first and second degree heartblocks. The use of intravenous dopamine and isoproterenol in babies has shown some success. Pacemaker therapy is needed in symptomatic babies. Temporary pacing can be done by transcutaneous, transesophageal and transvenous routes. However permanent pacemaker is eventually needed for most infants. It is of two types namely An Initiative of The Tamil Nadu Dr. M.G.R. Medical University University Journal of Medicine and Medical Sciences epicardial and endocardial pacing. The indications for pacing are HR < 50 per minute, atrial rate>140 per minute, atrio ventricular block pauses lasting > 3 seconds, features of CCF, low cardiac output, ventricular dysfunction, post surgical CAVB.. References: Claus R, Hickstein H, Kulz T, et al. Identification and management of fetuses at risk for or affected by congenital heart block associated with autoantibodies to SSA (Ro), SSB (La), or an HsEg5-like autoantigen. Rheumatol Int. Aug 2006;26 (10):886-95. 1. 2. Costedoat-Chalumeau N, Amoura Z, Villain E, et al. Anti-SSA/Ro antibodies and the heart: more than complete congenital heart block? A review of electrocardiographic and myocardial abnormalities and of treatment options. Arthritis Res Ther. 2005;7(2):69-73. 3. Costedoat-Chalumeau N, GeorginLavialle S, Amoura Z, et al. Anti-SSA/Ro and anti-SSB/La antibody-mediated congenital heart block.Lupus. 2005;14(9):660 -4. 4. Michaelson M, Engle MA. Congenital complete heartblock: an international study of the natural history. In: BreastAN, Engle MA, eds. Cardiovascular Clinics. Philadelphia, Pa:FA Davis Co; 1972:89101. 5. Camm AJ, Bexton RS. Congenital heart block. EurHeart J. 1984;5:1 15-117. 6. Text book of Clinical recognition of congenital heart disease Joseph K.Perloff 5th edition 7. McCue CM, Mantakas ME, Tinglestad JB, Ruddy S.Congenital heart block in newborns of mothers with connective tissue disease. Circulation. 1977;56:82-90. 8. Nathalie Costedoat-Chalumeau et al. Anti-SSA/Ro antibodies and the heart: more than completecongenital heart block? A review of electrocardiographic and myocardial abnormalities and of treatment options 9 Reed BR, Lee LA, Harmon C. Autoantibodies to SSA(Ro) in infants with congenital heart block. J Pediatr.1983;1 03:889-891 10. Michaelsson M, Riesenfeld T, Jonzon A. Natural history of congenitalcomplete atrioventricular block. Pacing Clin Electrophysiol 1997;20:2098 –101 11.Taylor-Albert E, Reichlin M, Toews WH, Overholt ED, Lee LA. Delayed dilated cardiomyopathy as a manifestation of neonatal lupus:case reports, autoantibody analysis, and management. Pediatrics 1997;99:733–5. 12 Buyon JP, Hiebert R, Copel JA, et al. Autoimmune-associated congenital heart block: demographics, mortality, morbidity and recurrence rates obtained from a national neonatal lupus registry. J Am Coll Cardiol 1998;31:1658 – 66. 13 Waltuck J, Buyon JP. Autoantibody-associated congenital heart block:outcome in mothers and children. Ann Intern Med 1994;120:544 –51. An Initiative of The Tamil Nadu Dr. M.G.R. Medical University University Journal of Medicine and Medical Sciences 14. Litsey SE, Noonan JA, O’Connor WN, Cotrill CM, Mitchell B. Maternal connective tissue disease and congenital heart block. N Engl J Med 1985;312:98 –100. 15.Lee LA, Coulter S, Erner S, et al. Cardiac immunoglobulin deposition in congenital heart block associated with maternal anti-Ro autoantibodies. Am J Med 1987;83:793– 6 16. Esscher E, Michaelsson M. Congenital complete AV block. In: Diagnosis and Treatment of Cardiac Arrythmias. Eds.Bayes A, Cosin J. Barcelona, Doyma,1978, pp 618-624. 17. Roberts N, Gelband H. Arrythmias in heart disease in infants, children and adolescents. In: Heart Disease in Infants, Children and Adolescents. Eds.Moss AJ, Ad- picture 1: chest Xray showing cardiams FH, EmmanouilldesGC. Baltimore, omegaly Williams and Wilkins,1977, pp 687-696. picture 2: ECG showing features of complete heart block 18 Jaeggi ET, Hornberger LK, Smallhorn JF, Fouron JC. Prenatal diagnosis of complete atrioventricular block associated with structural heart disease: combined experience of two tertiary care centers and review of the literature. Ultrasound Obstet Gynecol. Jul 2005;26(1):16-21. 19 Strasburger J, Carpenter R, Smith RT. Fetal transthoracic pacing of advanced hydrops fetalis secondary to complete atrioventricular block. Pace. 1986;9:295297. An Initiative of The Tamil Nadu Dr. M.G.R. Medical University University Journal of Medicine and Medical Sciences