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 Table of Contents  
Year : 2017  |  Volume : 7  |  Issue : 1  |  Page : 52-53

Delivery in a 33-year-old woman with fontan palliation

1 Department of Anesthesia and Intensive Care, Pisa University, Pisa, Italy
2 Department of Anesthesia and Intensive Care, FTGM “G. Pasquinucci” Heart Hospital, Massa, Italy
3 Department of Obstretric and Gynecology, FTGM “G. Pasquinucci” Heart Hospital, Massa, Italy

Date of Web Publication1-Jun-2017

Correspondence Address:
A Viappiani
Department of Anesthesia and Intensive Care, Pisa University, Pisa
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/joacc.JOACC_60_15

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Fontan procedure is a surgical treatment used for patients with various forms of congenital heart disease who cannot support biventricular circulation. It is very important in pregnant patients that the right atrium and the single ventricle can tolerate the cardiovascular adjustment. The following case report describes a parturient who underwent Fontan procedure for tricuspid atresia and pulmonic stenosis and delivered, by caesarean section at 36.5 weeks of gestation, a female infant who weighed 2400 g, without any cardiovascular complications.

Keywords: Biventricular circulation, congenital heart disease, delivery

How to cite this article:
Viappiani A, Sarto P D, Morosan A, Pelliccia E, Forfori F. Delivery in a 33-year-old woman with fontan palliation. J Obstet Anaesth Crit Care 2017;7:52-3

How to cite this URL:
Viappiani A, Sarto P D, Morosan A, Pelliccia E, Forfori F. Delivery in a 33-year-old woman with fontan palliation. J Obstet Anaesth Crit Care [serial online] 2017 [cited 2023 Apr 1];7:52-3. Available from: https://www.joacc.com/text.asp?2017/7/1/52/207395

  Introduction Top

Fontan procedure is a surgical treatment used for patients with various forms of congenital heart disease who cannot support biventricular circulation.[1] The single ventricle must supply cardiac output to both systemic and pulmonary circulations.[2] Approximately 85% of the children born with a congenital heart disease are now surviving into adulthood.[3] Despite severe heart disease, and subsequent palliation, mortality is lower than 1% with a high morbidity.[4] During pregnancy 25% of deaths caused by cardiac complications are due to congenital heart diseases.[5] In women with Fontan circulation, pregnancy is a challenging state because the right atrium and the single ventricle have to tolerate cardiovascular adjustments such as circulatory overload, cardiac output which is 30–40% above non-pregnant levels, and heart rate.[6] In children, the risk of congenital heart disease remains unknown.[7] Venous circulation problems can modify the uterine circulation and the placenta leading to a premature rupture of membranes and a preterm labor.

  Case Report Top

A 33-year-old Fontan patient at 6 weeks of gestation came to the outpatient clinic. The patient had a medical history of congenital tricuspid atresia and pulmonic stenosis. At the age of 1 year, she was subjected to aorta-pulmonary artery cooley's anastomosis; at 9 years of age she underwent a Glenn operation and at 17 years of age a Fontan palliation. Her echocardiography showed a stable cardiac function (66% ejection fraction, slight atrioventricular valve incompetence) [Figure 1] and 96% saturation seen by pulse oximetry in the ambient air. She took warfarin regularly which was replaced with 200 mg of acetylsalicylic acid 2 years ago. At the beginning of her pregnancy, ASA was replaced with subcutaneous (s.c.) nadroparin 2850 U.I. anti X activated factor twice daily with anti X activated factor monitoring. For values of anti Xa under therapeutic range confirmed with trromboelastogram, we decided to increase the dosage to 4750 U.I. anti X activated factor BID. From 32 weeks of gestational period s.c. UFH (5000 U.I. BID). At 36.5 weeks, under general anesthesia, we carried out the caesarean. In the operating room, a radial artery cannula for blood pressure (BP) monitoring and a central venous catheter in the right jugular vein were inserted. An epidural catheter was placed at the L1-L2 vertebral interspace. Oxygen was supplied via nasal cannula at 4 l/min. After a negative test dose with 3 ml of 2% lidocaine through the epidural catheter, we administered 1000 ml of lactated Ringer's solution with 25 mg of ephedrine. We started with epidural boluses (2 ml) of 2% lidocaine for a total of 14 ml with 75 mcg of fentanyl. At the beginning of the surgery, arterial BP and central venous pressure was 130/80 and 12 mmHg, respectively, and the heart rate was 80/min in a normal sinus rhythm. Hemodynamic parameters were stable during the caesarean. A female neonate weighing 2400 g was delivered, with an Apgar score of 9/10. Twenty units of oxytocin and 2000 mg of tranexamic acid were administered. The patient was not given a transfusion due to minimal blood loss (50 ml). After the surgical procedure was completed the patient was transferred to the intensive care unit (ICU) for continuous hemodynamic monitoring. During the ICU admission, she was hemodynamically stable without any complications and was discharged to the cardiac ward after 24 h. Six days later she was discharged to go home.
Figure 1: A: Inferior V. cava-pulmonary artery conduit. B: Superior V. cava. C: Aorta. D: Single ventricle. E:Right Pulmonary artery. F: Left Pulmonary artery

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  Discussion Top

Cardiac disease is becoming more common in women during maternity care. Fontan is a palliative procedure in patients with highly complex congenital heart disease that cannot support a biventricular circulation.[1] Women with an adequate Fontan palliation can become pregnant without clinically significant long-term sequelae, however, they may be important complications.[8] Patients with Fontan palliation surgery are at an increased risk of intracardiac thrombus formation.[9] Arrhythmias are also common complications during pregnancy in women with this congenital heart disease.[2] It is very important to assess the volume status and to maintain normovolemia. Fontan circulation is a complex volume responsive state and needs an adequate preload and normal low afterload to maintain the cardiac output.[10] Adequate pressure gradient between systemic veins and the left atrium and a low pulmonary vascular resistances are mandatory. Keeping intrathoracic pressure low and maintaining spontaneous breathing are the best solutions to facilitate venous return.[11] In these patients, even a little increase in intrathoracic pressure and subsequent reduction in preload are not very well tolerated.[12] Although there were reports about successful vaginal delivery, in this case the caesarean and the time of delivery was an obstetrical decision related to placenta vascular abnormalities. Using epidural anesthesia we were able to obtain a better periprocedural pain control, avoiding the deleterious hemodynamic effects of the positive pressure ventilation (reduction of cardiac filling and output, and global oxygen delivery).[12]

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  References Top

Seijii I, Hisashi M, Teiji A, Yuji H. Pregnancy and delivery in patients with Fontan circulation: A report of two cases. J Obstet Gynaecol Res 2013;39:378-82.  Back to cited text no. 1
Jooste EH, Haft WA, Ames WA, Sherman FS, Vallejo MC. Anesthetic care of parturients with single ventricle physiology. J Clin Anesth 2013;25:417-23.  Back to cited text no. 2
Uebing A, Steer PJ, Yentis SM, Gatzoulis MA. Pregnancy and congenital heart disease. BMJ 2006;332:401-6.  Back to cited text no. 3
Drenther W, Pieper PG, Roos-hesselink JW, van Lottum WA, Voors AA, Mulder BJ, et al. Outcome of pregnancy in women with congenital heart disease: A literature review. J Am Coll Cardiol 2007;49:2303-11.  Back to cited text no. 4
Malhotra S, Yentis SM. Report on Confidential Enquiries into Maternal Deaths: A secondary analysis of cardiac deaths. Int J Obstet Anesth 2005;14:S8.  Back to cited text no. 5
Nora JJ, Nora AH. Maternal transmission of congenital heart disease: New recurrence risk figure and the question of cytoplasmic inheritance and vulnerability to teratogens. Am J Cardiol 1987;59:459-63.  Back to cited text no. 6
Drenther W, Pieper PG, Ross-Hesselink JW, van Lottum WA, Voors AA, Mulder BJ, et al. Pregnancy and delivery in women after Fontan palliation. Heart 2006;92:1290-4.  Back to cited text no. 7
Walker HA, Gatzoulis MA. Prophylactic anticoagulation following the Fontan operation. Heart 2005;91:854-6.  Back to cited text no. 8
Eyvazzadeh JA, Chtilian HV. Specific considerations with cardiac disease: III. Congenital heart disease. In Hurford WE, editor. Clinical Anesthesia Procedures of the Massachusetts General Hospital. 7th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2007.p28.  Back to cited text no. 9
Boltz MG, Ramamoorthy C. Pediatric cardiac catheterization and electrophysiology: Anesthetic considerations. In: Jaffe RA, Samuels SI, Schmiesing CA, Golianu B, editors. Anesthesiologist's Manual of Surgical Procedures. 4th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2009.p 1493-4.  Back to cited text no. 10
Oliver CW, Lynch JJ. Congenital heart disease: Single ventricle. In: Flesher L, editor. Anesthesia and Uncommon Diseases. 5th ed. Philadelphia, PA: Sainders Elsevier; 2006.p215-9.  Back to cited text no. 11
Carp H, Hayaram A, Vadhera R, Nichols M, Morton M. Epidural anesthesia for cesarean delivery and vaginal birth after maternal Fontan repair: report of two cases. Anesth Analg 1994;78:1190-2.  Back to cited text no. 12


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