Asian Cardiovasc Thorac Ann 2006;14:428-431
© 2006 Asia Publishing EXchange Ltd
Pacing in Preterm with Hydrops Fetalis due to Congenital Complete Heart Block
Anant Khositseth, MD,
Piya Samankatiwat, MD1,
Wichaya Withurawanit, MD1,
Pongsak Khowsathit, MD
Division of Pediatric Cardiology, Department of Pediatrics
1 Division of Cardiovascular-thoracic Surgery, Department of Surgery, Faculty of Medicine, Ramathibodi Hospital, Mahidol University Bangkok, Thailand
For reprint information contact: Anant Khositseth, MD Tel: 66 22 011 685 Fax: 66 22 011 850 Email: alaks{at}diamond.mahidol.ac.th, Division of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Bangkok 10400, Thailand.
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ABSTRACT
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Hydrops fetalis due to congenital complete heart block (CCHB) is a rare condition. The outcome of the preterm fetus with hydrops fetalis due to CCHB is poor, and is frequently associated with significant morbidity and mortality. The management of this condition is difficult. We report our experience in a hydropic preterm using staged pacing by applying left ventricular epicardial pacing with a temporary pacemaker and subsequently, left ventricular epicardial pacing with a permanent pacemaker.
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INTRODUCTION
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Hydrops fetalis due to congenital complete heart block (CCHB) is a rare condition associated with a preterm delivery and significant morbidity and mortality. The management of this condition is challenging. This report describes staged pacing with a temporary pacemaker followed by a permanent pacemaker in a 33-week hydrops.
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CASE REPORT
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A 25-year-old primigravida who previously had normal antenatal care with non-reactive VDRL, negative HBsAg and anti-HIV was referred to our center. At 33 weeks of gestation an obstetrical ultrasound was performed due to fetal size greater than date. Fetal ultrasound demonstrated hydrops fetalis: bilateral pleural effusion, ascites, and skin edema. Fetal echocardiography demonstrated a dilated right atrium and right ventricle as well as moderate tricuspid and mitral valve regurgitation. M-mode of the atrial and ventricular walls showed severe ventricular bradycardia, 50 beats·min–1 but a normal atrial rate of 146 beats·min–1 (Figure 1
). The left ventricular systolic function was preserved with an ejection fraction of 65%. Dexamethasone 12 mg was given intramusculary and delivery was performed by cesarean section 17 hours later.
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Figure 1. Fetal M-mode echocardiography demonstrates a slow ventricular rate of 50 beats·min–1 and a ventricular rate of 146 beats·min–1 (upper arrows show atrial contraction and lower arrows show ventricular contraction).
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A girl infant was born prematurely at 33 weeks of gestational age with a birth weight of 2850 g and an APGAR score of 1 and 6 at 1 and 5 minutes, respectively. She was intubated in the operating room and transferred to the neonatal intensive care unit. Physical examination revealed anasarca, pallor, and acyanosis. Her blood pressure was 70/33 mm Hg and she had a heart rate of 50 beats·min–1. A chest X-Ray showed cardiomegaly with bilateral pleural effusion (Figure 2A). A 12 lead electrocardiography showed high-grade AV block (Figure 3). Echocardiography demonstrated a small pericardial effusion, concentric left ventricular hypertrophy, normal left ventricular systolic function (ejection fraction of 68%), severe bradycardia (ventricular rate of 50 beats·min–1), and patent ductus arteriosus (PDA). There were no other associated heart defects. Basic laboratory studies revealed anemia (hemoglobin = 9.5 g·dL–1), thrombocytopenia (platelets = 111,000/cu. mm), and hypoalbuminemia (total protein/albumin = 34.6/20.8 mg·dL–1). There were no skin lesions consistent with lupus erythematosus. Further investigation revealed maternal positive antinuclear antibody (ANA) [coarse speckled 1:3200, positive anti-SSA (anti-Ro) and weakly positive anti-SSB (anti-La)].
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Figure 2. Chest X-Rays demonstrate massive bilateral pleural effusions (A), resolution of bilateral pleural effusions after temporary pacing (B), and the position of the epicardial wire and permanent pacemaker in the abdominal wall (C).
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In an emergency setting, the patient underwent an operation at 5 hours after birth. A left anterolateral thoracotomy incision was made at the sub-mammary skin crease. Two temporary pacing wires were placed on a selected area on the epicardium over the left ventricle (Figure 2B
). Initially, the external pacemaker was set at a rate of 150 beats·min–1 and a threshold of 1.5 mA, but this was increased to 3 mA due to non-capturing. Clinically, hydrops fetalis was resolving (body weight decreased to 2000 g, diminished pleural effusion, resolved anasarca). Follow-up echocardiography on day 8 of life demonstrated a closing PDA, resolved pericardial effusion, and decreased severity of tricuspid and mitral regurgitation to a mild degree, and a mild to moderate degree, respectively.
Three weeks later, she underwent permanent single-chamber epicardial pacing with lead placement on the left ventricular free wall. A permanent pacemaker box, mode VVIR, was implanted in the sub-muscular layer of the anterior abdominal wall via a separated transverse abdominal incision (Figure 2C). The box was initially programmed at a constant rate of 150 beats·min–1. There were no complications related to the procedure.
Echocardiography demonstrated normal left ventricular function with only mild mitral regurgitation. Follow-up demonstrated a functioning pacemaker with a low ventricular pacing threshold of 0.75 mV. Her basic rhythm remained in complete heart block.
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DISCUSSION
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Congenital complete heart block (CCHB) in the fetus can cause hydrops fetalis, especially when the ventricular rate is below 55 beats·min–1.1 Pre-natal diagnosis of CCHB when associated with hydrops fetalis and low ventricular heart rate has a very poor prognosis with fetal and neonatal mortality exceeding 80%.2 Deloof and colleagues3 used a staged approach in two hydropic fetuses due to CCHB, using bipolar transesophageal pacing or epicutaneo-esophageal pacing. After the regression of the hydrops, epicardial pacing was started subsequent to implantation of two or three temporary epicardial 3/0 pacemakers, and implantation of a permanent abdominal pacing system with a steroid epicardial tip once the threshold exceeded 20 mA or when the baby weighed more than 1500 g. There were no major complications and the cardiac outcome at 37 and 34 months of follow-up was excellent.
In this report, a 33-week hydropic fetus was delivered using elective cesarean section after administration of dexamethasone. Initially, clinical hydrops with a large amount of ascites, bilateral pleural effusion, anasarca, and a small amount of pericardial effusion might potentially interfere with permanent pacemaker placement, so staged pacing using temporary epicardial pacing wire and an external pacemaker was performed without complications, resulting in resolved hydrops. Other temporary pacing wire techniques such as endocavitary, transcutaneous, or transesophageal techniques in the preterm may be associated with significant morbidity. Weindling and colleagues4 reported a safe and efficient pacing system using temporary epicardial leads for the treatment of CCHB in preterm infants. We selected the left ventricular site for the epicardial pacing wire in this report following a report by Shiraishi and colleagues5 which demonstrated that left ventricular pacing might be superior for treating hydropic fetuses with CCHB.
Congenital complete heart block is highly associated with maternal ANA and anti-SSA/Ro antibody (100%) and anti-SSB/La antibody (75%).6 In our case, maternal ANA, anti-SSA and anti-SSB antibody were all positive. However, this patient did not have other features of neonatal lupus erythematosus.
In conclusion, staged pacing with temporary left ventricular epicardial pacing, followed by permanent left ventricular epicardial pacing and abdominal VVIR pacemaker placement resulted in resolution of hydrops fetalis due to high-grade AV block.
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REFERENCES
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- Anandakumar C, Biswas A, Chew SS, Chia D, Wong YC, Ratnam SS. Direct fetal therapy for hydrops secondary to congenital atrioventricular heart block. Obstet Gynecol 1996;87(5 Pt 2):835–7.[Medline]
- Groves AM, Allan LD, Rosenthal E. Outcome of isolated congenital complete heart block diagnosed in utero. Heart 1996;75:190–4.[Abstract/Free Full Text]
- Deloof E, Devlieger H, Van Hoestenberghe R, Van den berghe K, Daenen W, Gewillig M. Management with a staged approach of the premature hydropic fetus due to complete congenital heart block. Eur J Pediatr 1997;156:521–3.[Medline]
- Weindling SN, Saul JP, Triedman JK, Burke RP, Jonas RA, Gamble WJ, et al. Staged pacing therapy for congenital complete heart block in premature infants. Am J Cardiol 1994;74:412–3.[Medline]
- Shiraishi H, Kikuchi Y, Hoshina M, Ohki T, Ayustawati, Momoi MY. Hemodynamic effect of the ventricular pacing site in fetal lambs with complete atrioventricular block. Pacing Clin Electrophysiol 2002;25:1731–6.[Medline]
- Lin MC, Fu YC, Tseng JJ, Jan SL, Chi CS. Congenital complete heart block. Acta Paediatr Taiwan 2001;42:42–5.[Medline]
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ABSTRACT
INTRODUCTION
