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Neonatal Ebstein’s Anomaly: Surgical Decision Making

Awori M, Mutwiri RK, Mutunga WM, Jowi SO, Peninan KN, Mang’usu OE School of Medicine University of Nairobi

Correspondence to: Dr. Mark Awori, P. O Box 14677-00800, Nairobi, Kenya.

Email: mnawori@yahoo.com

Summary

Ebstein’s Anomaly (EA) is a rare form of congenital heart disease. Surgical decision-making in neonates is controversial. In developing countries, neonates with Ebstein’s anomaly requiring surgical intervention rarely present to health institutions capable of providing intervention. We present a 2.2 kg term female neonate who presented with Ebstein’s anomaly. Data was obtained retrospectively from clinical case notes. We describe our surgical decision-making process and outcome in relation to prevailing thought regarding the management of this condition and we make a recommendation on what we consider to be the best surgical option for these patients.

 

Keywords: Ebstein's anomaly, Neonatal, Treatment

Ann Afr Surg. 2018; 15(1):40-43 DOI:http://dx.doi.org/10.4314/aas.v15i1.9

 

© 2018 Author. This work is licensed under the Creative Commons Attribution 4.0 International License

Introduction

Ebstein’s anomaly is a rare form of congenital heart disease with and estimated prevalence of 1:20,000 live births (1). It has 3 characteristic features: (i) a downward displacement of the septal and posterior leaflets into the right ventricle; (ii) an atrialized region of the right ventricle between the tricuspid annulus and the attachment of septal and posterior leaflets; and (iii) a malformation of the right ventricle (RV) where it is small, thin-walled and often has abnormal systolic function. There may be right ventricular outflow tract obstruction (RVOTO). The key surgical decisions to be made are: (i) whether a two ventricle repair is feasible and (ii) where it is not, whether a Starne’s procedure performed on cardiopulmonary bypass is required to facilitate successful single ventricle repair. We present our surgical experience in 2.2 kg neonate presenting with Ebstein’s anomaly.

 

Case Report

A 2.2 kg term female neonate was referred to the Kenyatta National Hospital (the main teaching and referral hospital in Kenya) on the 8th day of life with a diagnosis of ‘complex congenital heart disease’; this had been confirmed by echocardiography on the 2nd day of life. She was born via emergency caesarean-section (CS) for fetal distress. At CS there was meconium stained liquor; the Apgar score was ‘4’at 1 minute, ‘6’ at 5 minutes and ‘8’ at 10 minutes. On admission (to our institution) she was ‘sick-looking’; in respiratory distress (respiratory rate of 42 breaths/minute) and was afebrile. She had both peripheral and central cyanosis; her heart rate was 154 beats each minute and she had a pan systolic murmur. The rest of her physical examination was unremarkable. The patient was tolerating full nasogastric feeds of expressed breast milk. A Prostin (Prostaglandin E₂) intravenous infusion was running at 0.05mcg/kg/minute and intravenous flucloxacillin and amikacin had been started at the referring institution for presumed neonatal sepsis (we changed the flucloxacillin to ceftazidime). A chest radiograph showed gross cardiomegaly (cardiothoracic ratio 0.8) and a repeat transthoracic echocardiogram confirmed Ebstein’s anomaly (EA). The septal leaflet of the tricuspid valve was moderately displaced inferiorly and the anterior leaflet was long and sail like. The right ventricular volume was approximately ¼ that of the left ventricle and there was severe tricuspid regurgitation associated with a 6mm (diameter) secundum atrial septal defect shunting right-to-left. The interventricular septum was intact. The pulmonary valve was atretic, the branch pulmonary arteries were confluent; the right pulmonary artery was 3mm (diameter) and the left was 4.5mm (diameter). There was a moderate sized patent ductus arteriosus that was tapering at the pulmonary end. Arterial blood gas analysis (on admission) revealed: a pH of 7.083 (7.35-7.45), a pCO2 of 10.21(4.2-6.00kpa), a pO2of 4.44 (10.00-13.33kpa) and an HCO3 of 22.4mmol/l (22-26mmol/l). We suspected that this was a venous sample; unfortunately another sample was not taken for a repeat analysis. Additional pre-operative blood works were performed on the 10th day of life and the renal function test and the full hemogram results were essentially normal for an 8 day old neonate.

After adequate resuscitation, and following a Cardiac team meeting, a right modified Blalock-Taussig shunt (3.5mm diameter ePTFE; figure 1) was created via a right thoracotomy on the 10th day of life. An intravenous infusion of heparin was started on return to the neonatal intensive care unit on the day of surgery. An echocardiogram performed on the 1st postoperative day (POD 1) showed a functioning shunt and liver function tests revealed the following (significant derangement):

  • Albumin= 32g/l (35-49g/l)

  • Total bilirubin= 42.5µmol/l (2.0-21.0µmol/l) o Indirect bilirubin= 20.5µmol/l

  •  Direct bilirubin= 22.0µmol/l (0.0-5.1µmol/l) o Alanine transaminase= 37U/l (0-34U/l)

  • Aspartate transaminase=115U/l (0-31U/l)

  • Gamma Glutamyl transferase= 138U/l (0-32U/l) o Alkaline phosphatase=209U/l (40-125U/L)