Korean Circ J.  2016 Jan;46(1):111-114. 10.4070/kcj.2016.46.1.111.

Pregnancy and Delivery in Functional Single Ventricle Patient; Successful Long-Term Outcome after Right Ventricle Exclusion and Fontan Operation

• Kim SY
• Cho S
• Choi E
• Kim WH

• KMID: 2223778
• DOI: http://doi.org/10.4070/kcj.2016.46.1.111

Abstract

Twenty-six-year-old Ebstein's anomaly patient, who had failed both biventricular and one-and-a-half repair, underwent right ventricle exclusion and Fontan operation. She completed pregnancy and delivery owing to the excellent long-term clinical course. Although the caesarean section was performed due to symptoms of heart failure on the gestational age of 32+6 weeks, preterm delivery was agreeable with neonatology support. In Korea, there has not yet been a case of pregnancy and delivery of functional single ventricle patient because most patients have been discouraged from getting pregnant. However, functional single ventricle patient can endure pregnancy and delivery, if valve function and ventricular contractility, status of Fontan pathway and absence of arrhythmia predict favorable outcome as presented in this case. Although the patient maintained her pregnancy without anticoagulation owing to laminar flow in the Fontan pathway and absence of thromboembolic event, anticoagulation should be considered, weighing the benefits and risks during the pregnancy.

Keyword

Fontan procedure; Ebstein anomaly; Pregnancy; Delivery, obstetric; Heart failure

MeSH Terms

Arrhythmias, Cardiac
Cesarean Section
Delivery, Obstetric
Ebstein Anomaly
Female
Fontan Procedure*
Gestational Age
Heart Failure
Heart Ventricles*
Humans
Korea
Neonatology
Pregnancy*
Risk Assessment

Figure

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  Fig. 1 Cardiomegaly has been improved on serial chest X-ray. (A) Before operation (B) Post-operative day 19 (C) 3 years after operation.

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  Fig. 2 Cardiac magnetic resonance imaging. (A) Before operation, right ventricle (RV) is enlarged, compressing left ventricle (LV). Interventricular septum (arrowhead) is deviated toward LV. (B) 1 month after operation, RV size is decreased. Interventricular septum (arrowhead) is still deviated toward LV. (C) 3 years after operation, RV is filled with thrombus (arrow) without inflow. LV function is improved with decreased paradoxical septal movement (arrowhead).

Reference

1. Canobbio MM, Cetta F, Silversides C, Warnes C, Aboulhosn J, Colman J. Pregnancy after Fontan operation: early and late outcomes. J Am Coll Cardiol. 2013; 61:E427.

2. Inoue S, Masuyama H, Akagi T, Hiramatsu Y. Pregnancy and delivery in patients with Fontan circulation: a report of two cases. J Obstet Gynaecol Res. 2013; 39:378–382.

3. Sano S, Ishino K, Kawada M, et al. Total right ventricular exclusion procedure: an operation for isolated congestive right ventricular failure. J Thorac Cardiovasc Surg. 2002; 123:640–647.

4. Cho S, Kim WH, Kwak JG, Park CS. Right ventricular exclusion procedure for right ventricular failure of Ebstein's anomaly. J Thorac Cardiovasc Surg. 2009; 138:1246–1248.

5. Chugh R. Management of pregnancy in women with repaired CHD or after the Fontan procedure. Curr Treat Options Cardiovasc Med. 2013; 15:646–662.

6. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association task force on practice guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation. 2008; 118:e714–e833.

7. Baumgartner H, Bonhoeffer P, De Groot NM, et al. ESC guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J. 2010; 31:2915–2957.

8. Monagle P, Cochrane A, Roberts R, et al. A multicenter, randomized trial comparing heparin/warfarin and acetylsalicylic acid as primary thromboprophylaxis for 2 years after the Fontan procedure in children. J Am Coll Cardiol. 2011; 58:645–651.