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Table of Contents   
CASE REPORT  
Year : 2017  |  Volume : 10  |  Issue : 3  |  Page : 284-287
Chronic antepartum maternal hyperoxygenation in a case of severe fetal Ebstein's anomaly with circular shunt physiology


1 Department of Pediatrics, Division of Pediatric Cardiology, Stanford University School of Medicine, Palo Alto, CA 94304, USA
2 Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Stanford University School of Medicine, Stanford, CA 94304, USA

Click here for correspondence address and email

Date of Web Publication21-Aug-2017
 

   Abstract 


Perinatal mortality remains high among fetuses diagnosed with Ebstein's anomaly of the tricuspid valve. The subgroup of patients with pulmonary valve regurgitation is at particularly high risk. In the setting of pulmonary valve regurgitation, early constriction of the ductus arteriosus may be a novel perinatal management strategy to reduce systemic steal resulting from circular shunt physiology. We report the use of chronic antepartum maternal oxygen therapy for constriction of the fetal ductus arteriosus and modulation of fetal pulmonary vascular resistance in a late presentation of Ebstein's anomaly with severe tricuspid valve regurgitation, reversal of flow in the ductus arteriosus, and continuous pulmonary valve regurgitation.

Keywords: Congenital heart disease, Ebstein's anomaly, fetal intervention, maternal hyperoxygenation

How to cite this article:
Arunamata A, Axelrod DM, Bianco K, Balasubramanian S, Quirin A, Tacy TA. Chronic antepartum maternal hyperoxygenation in a case of severe fetal Ebstein's anomaly with circular shunt physiology. Ann Pediatr Card 2017;10:284-7

How to cite this URL:
Arunamata A, Axelrod DM, Bianco K, Balasubramanian S, Quirin A, Tacy TA. Chronic antepartum maternal hyperoxygenation in a case of severe fetal Ebstein's anomaly with circular shunt physiology. Ann Pediatr Card [serial online] 2017 [cited 2018 Aug 17];10:284-7. Available from: http://www.annalspc.com/text.asp?2017/10/3/284/213361





   Introduction Top


Perinatal mortality remains high among fetuses diagnosed with Ebstein's anomaly, with reports of up to 45% in recent large multicenter series.[1] In the setting of pulmonary valve regurgitation, early constriction of the ductus arteriosus may be a novel perinatal management strategy to reduce systemic steal resulting from circular shunt physiology. We demonstrate the use of chronic antepartum maternal oxygen therapy in a case of severe Ebstein's anomaly for constriction of the fetal ductus arteriosus and modulation of fetal pulmonary vascular resistance before successful neonatal surgical repair.


   Clinical Summary Top


A 22-year-old mother with intrahepatic cholestasis was referred to our institution at 32 5/7 weeks gestation with a fetus with Ebstein's anomaly and severe tricuspid valve regurgitation, continuous pulmonary valve regurgitation, reversal of flow in the ductus arteriosus, mild ascites, and a small pericardial effusion [Figure 1]. Middle cerebral artery (MCA) Doppler revealed absent end-diastolic flow suggesting significant systemic steal [Figure 2]. With a cardiothoracic ratio of 0.75 and Celermajer index of 1.5, the fetus received a prognostic score of 8 based on a recent evaluation of fetuses with isolated tricuspid valve malformations; no patients in this study with a prognostic score of ≥5 survived past the first month of life.[2],[3]
Figure 1: Fetal echocardiogram before maternal oxygen therapy. (a) Color Doppler demonstrating severe tricuspid valve regurgitation (TR, annotated by the white arrow). RA: Right atrium, RV: Right ventricle, (b) Spectral Doppler noting continuous pulmonary regurgitation. (c) Color Doppler of reverse flow (left-to-right) in the patent ductus arteriosus (PDA). Ao: Aorta

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Figure 2: Abnormal middle cerebral artery Doppler profile with absent end-diastolic flow

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Given the high risk for fetal demise, maternal oxygen therapy was proposed to constrict the ductus arteriosus to reduce the aortic-to-pulmonary shunt and augment pulmonary blood flow by decreasing pulmonary vascular resistance. As alternative therapy for ductal constriction, maternal administration of nonsteroidal anti-inflammatory drugs (NSAIDs) was contraindicated due to the history of intrahepatic cholestasis. The mother subsequently consented to a trial of oxygen therapy.

A fetal echocardiogram was performed before and after a 30 min trial of maternal hyperoxygenation at 6 L/min through nonrebreather facemask (FiO2 1.0). Maternal hyperoxygenation for 30 min demonstrated increased pulmonary blood flow with an increased ratio of pulmonary artery forward: reverse velocity time integral (VTI) and pulmonary venous VTI by fetal echocardiogram; ductal constriction and reduction of systemic steal were demonstrated by restoration of continuous end-diastolic MCA flow and an acute decrease in MCA pulsatility index [Figure 3].
Figure 3: Fetal echocardiogram before and after maternal oxygen therapy for 30 min. (a) Increase in pulmonary artery forward (above baseline): Reverse (below baseline) velocity time integral ratio from 3.9 to 5.2. (b) Increase in pulmonary venous velocity time integral from 0.05 m to 0.14 m. (c) Acute decrease in middle cerebral artery pulsatility index from 3.1 to 1.7 attributed to ductus arteriosus constriction

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The mother was subsequently admitted to the hospital for maternal oxygen therapy with 6 L/min through nonrebreather facemask for 4 h, 3 times daily with serial fetal echocardiograms until delivery. After chronic maternal hyperoxygenation through 36 weeks gestation, there was evidence of moderate ductal restriction (peak instantaneous gradient of 35 mmHg) [Figure 4] and normal MCA flow.
Figure 4: Spectral Doppler in the ductus arteriosus at 36 weeks gestation after 1 month of maternal hyperoxygenation demonstrating moderate ductus arteriosus restriction, with a peak instantaneous gradient of 35 mmHg

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Emergent cesarean section was performed at 36 weeks gestation for late fetal heart rate decelerations; Apgar scores at delivery were 3, 3, and 4 at 1, 5, and 10 min, respectively. Initial peripheral arterial blood gas revealed significant respiratory and metabolic acidosis with a pH of 6.9 and hypoxemia with a partial pressure of oxygen (PaO2) of 40 mmHg.

The male infant was (i) cannulated onto veno-venous extracorporeal membrane oxygenation (ECMO) to support oxygenation, (ii) started on inhaled nitric oxide to decrease pulmonary vascular resistance, and (iii) underwent surgical ductal ligation to eliminate systemic steal as discussed during prenatal delivery planning. At 2 weeks of age, the infant underwent primary repair of the tricuspid valve and reduction annuloplasty, with right atrial reduction plasty. On inspection of the tricuspid valve in the operating room, there was complete absence of the posterior leaflet and a poorly developed and displaced septal leaflet; sutures were used to coapt the anterior and septal leaflets to create a functional bileaflet tricuspid valve. The infant tolerated patent foramen ovale closure with successful ECMO decannulation in the operating room, with a right atrial pressure of 8 mmHg and left atrial pressure of 5 mmHg at the end of the case. The infant was discharged home at 2 months of age after an uneventful cardiac postoperative course. He is followed in outpatient cardiology clinic and continues to thrive with normal growth and development.


   Discussion Top


The subgroup of fetuses with Ebstein's anomaly and pulmonary valve regurgitation with circular shunt physiology is at particularly high risk for perinatal demise.[1] In this scenario, there is retrograde (left-to-right) flow across the ductus arteriosus, continuous pulmonary valve regurgitation, severe tricuspid valve regurgitation, and right-to-left flow across the atrial septal communication leading to systemic steal. The absence of end-diastolic flow in the MCA Doppler is a marker of significant systemic steal. The chronic volume load on the right side of the heart leads to marked dilation of the right atrium and ventricle contributing to cardiomegaly, pulmonary hypoplasia, and subsequent pulmonary vascular disease well-described in neonatal Ebstein's anomaly.[4],[5]

There remain limited options for perinatal management of fetuses with this pathophysiology; however, neonatal studies have suggested that early ductal ligation or spontaneous ductal constriction may result in termination of the circular shunt and hemodynamic improvement.[6] While NSAIDs may be considered for fetal ductus arteriosus closure in late gestation, use of NSAIDs in utero carries a risk of maternal postpartum hemorrhage, fetal organ impairment, and potentially persistent pulmonary hypertension, a complication that is detrimental to neonates with Ebstein's anomaly.[7],[8]

Maternal oxygen use was first described in fetuses with congenital anomalies leading to pulmonary hypoplasia such as renal agenesis, obstructive uropathy, and congenital diaphragmatic hernia; poor reactivity to oxygen administration demonstrated on Doppler assessment of pulmonary blood flow predicted neonatal demise from pulmonary hypoplasia.[9] Clinical maternal oxygen use in the setting of fetal congenital heart disease has been described as a useful diagnostic tool to determine pulmonary vasculature response in hypoplastic left heart syndrome with concerns for a restrictive or intact atrial septum.[10] More recently, chronic antepartum oxygen therapy has been used to promote aortic and mitral annular growth in patients with left heart hypoplasia.[11],[12] Antepartum maternal hyperoxygenation appears to be technically feasible, with no reports of neonatal retinopathy on ophthalmologic examinations.[11]

As in neonatal life with an increase in blood oxygen-content stimulating closure of the ductus arteriosus, our case demonstrates that chronic maternal hyperoxygenation at 12 h/day in late gestation may constrict the ductus arteriosus and has a beneficial role in the management of severe fetal Ebstein's anomaly. Maternal hyperoxygenation may be additionally beneficial in fetuses with Ebstein's anomaly by reducing pulmonary vascular resistance and promoting antegrade pulmonary blood flow, demonstrated on fetal echocardiogram by the increase in antegrade: retrograde flow across the branch pulmonary arteries and pulmonary venous VTI.

We propose that a prenatal management paradigm of chronic oxygen therapy for ductal constriction combined with pulmonary vascular resistance reduction, and a brief postnatal period of vascular remodeling and oxygenation support may enable early successful repair in patients with severe Ebstein's anomaly.[13]

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Freud LR, Escobar-Diaz MC, Kalish BT, Komarlu R, Puchalski MD, Jaeggi ET, et al. Outcomes and predictors of perinatal mortality in fetuses with Ebstein anomaly or tricuspid valve dysplasia in the current era: A multicenter study. Circulation 2015;132:481-9.  Back to cited text no. 1
    
2.
Celermajer DS, Cullen S, Sullivan ID, Spiegelhalter DJ, Wyse RK, Deanfield JE. Outcome in neonates with Ebstein's anomaly. J Am Coll Cardiol 1992;19:1041-6.  Back to cited text no. 2
    
3.
Andrews RE, Tibby SM, Sharland GK, Simpson JM. Prediction of outcome of tricuspid valve malformations diagnosed during fetal life. Am J Cardiol 2008;101:1046-50.  Back to cited text no. 3
    
4.
Celermajer DS, Bull C, Till JA, Cullen S, Vassillikos VP, Sullivan ID, et al. Ebstein's anomaly: Presentation and outcome from fetus to adult. J Am Coll Cardiol 1994;23:170-6.  Back to cited text no. 4
    
5.
Lang D, Oberhoffer R, Cook A, Sharland G, Allan L, Fagg N, et al. Pathologic spectrum of malformations of the tricuspid valve in prenatal and neonatal life. J Am Coll Cardiol 1991;17:1161-7.  Back to cited text no. 5
    
6.
Wald RM, Adatia I, Van Arsdell GS, Hornberger LK. Relation of limiting ductal patency to survival in neonatal Ebstein's anomaly. Am J Cardiol 2005;96:851-6.  Back to cited text no. 6
    
7.
Koren G, Florescu A, Costei AM, Boskovic R, Moretti ME. Nonsteroidal antiinflammatory drugs during third trimester and the risk of premature closure of the ductus arteriosus: A meta-analysis. Ann Pharmacother 2006;40:824-9.  Back to cited text no. 7
    
8.
Alano MA, Ngougmna E, Ostrea EM Jr., Konduri GG. Analysis of nonsteroidal anti-inflammatory drugs in meconium and its relation to persistent pulmonary hypertension of the newborn. Pediatrics 2001;107:519-23.  Back to cited text no. 8
    
9.
Broth RE, Wood DC, Rasanen J, Sabogal JC, Komwilaisak R, Weiner S, et al. Prenatal prediction of lethal pulmonary hypoplasia: The hyperoxygenation test for pulmonary artery reactivity. Am J Obstet Gynecol 2002;187:940-5.  Back to cited text no. 9
    
10.
Szwast A, Tian Z, McCann M, Donaghue D, Rychik J. Vasoreactive response to maternal hyperoxygenation in the fetus with hypoplastic left heart syndrome. Circ Cardiovasc Imaging 2010;3:172-8.  Back to cited text no. 10
    
11.
Lara DA, Morris SA, Maskatia SA, Challman M, Nguyen M, Feagin DK, et al. Pilot study of chronic maternal hyperoxygenation and effect on aortic and mitral valve annular dimensions in fetuses with left heart hypoplasia. Ultrasound Obstet Gynecol 2016;48:365-72.  Back to cited text no. 11
    
12.
Kohl T. Chronic intermittent materno-fetal hyperoxygenation in late gestation may improve on hypoplastic cardiovascular structures associated with cardiac malformations in human fetuses. Pediatr Cardiol 2010;31:250-63.  Back to cited text no. 12
    
13.
Bauser-Heaton H, Nguyen C, Tacy T, Axelrod D. A novel approach to the management of critically ill neonatal Ebstein's anomaly: Veno-venous extracorporeal membrane oxygenation to promote right ventricular recovery. Ann Pediatr Cardiol 2015;8:67-70.  Back to cited text no. 13
    

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Correspondence Address:
Alisa Arunamata
Department of Pediatrics, Division of Pediatric Cardiology, Stanford University School of Medicine, 750 Welch Road, Suite #325, Palo Alto, CA 94304
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.APC_20_17

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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