Management of Pregnancy in 31-Year-Old Woman with Persistent Atrial Flutter After Cone Reconstruction for Ebstein’s Anomaly

There is a growing number of mothers suffering innate heart disease which could potentially risk their gestation period. Ebstein’s anomaly refers to a rare innate disease in the heart which accounts for less than 1% of most cases of innate heart disease, and only 5% of the patients surviving beyond 50 years of age. Cone reconstruction (CR) is an option for the repairment of tricuspid valve in patients with Ebstein anomaly. However, persistent arrhytmia, such as AFL, is possible to occur after CR. This case report aims to opt suitable pregnancy management of patients with persistent atrial flutter (AFL) after Cone reconstruction for Ebstein’s Anomaly. We report a 31 year-old woman who consulted for pregnancy planning due to history of Ebstein’s Anomaly, and had undergone successful cone reconstruction. No recorded history of AFL is reported. Physical examination finding was tricuspid regurgitation murmur without signs and symptoms of right ventricular failure. Electrocardiography showed counter-clockwise typical AFL. Echocardiography finding demonstrated post Cone reconstruction (CR), mild tricuspid regurgitation, and EF of 76%. Case Report Management of Pregnancy in 31-Year-Old Woman with Persistent Atrial Flutter After Cone Reconstruction for Ebstein’s Anomaly E. Susilowati, V. Tedjamulia, R. Myrtha 1 Internship Doctor Programe, Indonesia 2 Cardiovascular Department, Mardi Waluyo Hospital, Blitar, Indonesia 3 Cardiovascular Department, Faculty of Medicine, Universitas Sebelas Maret, Surakarta, Indonesia


Introduction
The statistical data of mothers suffering from innate heart disease during their gestation period is rising, because more than 90% grew with it when they reached adulthood. Most common problems during gestation and delivery are bleeding, arrhythmias, failure of the heart, and infrequently maternal death [6] . Ebstein's anomaly refers to a rare innate disease in the heart which accounts for less than 1% of most cases of innate heart disease, and only 5% of the patients surviving beyond 50 years of age [7,8] . It is characterized by the attachment of septal and posterior leaflet to the myocardium underlying it with the dislocation of the functional tricuspid annulus downward, resulting in the dilation of atrialized portion of right ventricle (RV) and true tricuspid annulus [9] . Several methods have been tried for the surgical repairment of the patients suffering the anomaly Ebstein. March 2020 | Vol 1 | Article 4 The managing preparations mainly focused on the repairment of the monocusp of the tricuspid valve with discerning plication of the atrialized right ventricle. Cone reconstruction was an option to repair tricuspid valve in Ebstein anomaly patients. A leaflet tissue of 360 degrees and made to surround the real annulus is the result of this strategy [10] . Actually, after conducting CR, the risk of arrhythmia for young patients with EA is very small [11] . However, despite the low risk arryhthmia, a persistent arrhytmia, such as AFL could still occur after CR. Nevertheless, both AFL and pregnancy could influence to thromboembolic problems [7] . Pregnancy, especially after first stability, raised the risk of thromboembolism up to 1.5 times [2] . It is presumed that AFL patients have a similar risk of thromboembolism to those with atrial fibrillation [3] . This case report focuses on elaborating suitable pregnancy management of persistent atrial flutter patients after Cone reconstruction for Ebstein's Anomaly.  with EF of 64% ( Figure 6).

Discussion
Women with congenital heart disease require careful monitoring before, during, and after pregnancy. In pregnancy, hemodynamic and hormonal alteration could disturb the arrhythmias and the prothrombic state that contribute to thromboembolic problems [1] . Haemodynamic changes in the course of pregnancy include plasma volume raise, heart rate, and decrease of aortic pressure and systemic vascular resistance. Plasma volume increase in the peak of 32 weeks of gestation, it counts higher by 40 to 50 percent than the pre-gestation period. Rate of heart also reaches its peak at 32 weeks of pregnancy; almost higher by 20% than in the pre-gestation period [12] . Variability of heart rate was oppressed during pregnancy, this impaired autonomic nerve activity and the overload volume of the heart and operational wound could count for the presence of tachyarrhythmia during pregnancy [14] .  [6] . Because of this hypercoagulability, patients with arryhthmia require thourough care.
AFL increased the risk of thromboembolism and may make the heart function worse [1,13] .
Presumably, AFL patients have a similar risk of thromboembolism to those with atrial fibrillation [13] , while risk of ischemic stroke only significantly increased at CHA2DS2-VASc score of >4, [5,15] or in CHADS2 score of more than 2 [15] .
Thrombophylaxis is needed for high risk pregnant woman, but careful consideration on the medication choice should be made. Oral anticoagulant use during pregnancy could increase the risk of bleeding in the time of delivery, also fetal deaths which could be resulted from sudden miscarriage.
Heparin was the preferred option since it does not cross the placenta and relatively safe [1] . Vitamin K antagonists are still tolerable for most cases (without history of congenital heart disease) from second trimester untill 1month before expected delivery [13] .
Vitamin K antagonists, that are associated with warfarin embryopathy, cross the placenta in the first trimester state, the increasing risk of CNS abnormalities, fetal and neonatal bleeding near the delivery time, and losing gestation,. LMWH is recommended rather than fondaparinux in the time of gestation. There was no LMWH or UFH that was detected in the breast milk in discernible quantity or crosses the placenta [2] . Hypodermic LMWH in a maintained dose could be used in the course of the gestation's first trimester and the last month.
Dabigatran which was said to be one of the new antagonists of oral thrombin, should not be used because a high dose of fetotoxicity was detected.
High risk patients with atrial fibrillation seemed to be March 2020 | Vol 1 | Article 4 lest effective to both single or dual antiplatelet therapy (clopidogrel and acetylsalicylic acid) than warfarin [15] .
Beside the increasing risk of thromboembolic in pregnant woman and especially with overt AFL, AFL itself during pregnancy results in some serious undesirable events. Studies showed that there is a raise in maternal mortality and low birth weight during pregnancy in AFL patients [1,4] . The increase of maternal mortality found in 12% of pregnancies complicated with AFL. Thus, it is proven that the association between serious results in pregnant women suffering structural heart disease and atrial arrhythmias. [1] Lapses of suffered tachycardia, in particular the atrial flutter, are intolerable and could inflict fetal hypoperfusion in structural heart disease [15] .
Ebstein anomaly tends to be related to tricuspid ejection, a RV that functions abnormally and a dilated right atrium. Patients may also have multiple accessory pathways. When there are RV size and systolic function that are reasonable, pregnancy is tolerated, as it is related to the absence of significant cyanosis or arrhythmias [14] . As the majority antiarrhythmic medication could be regarded as harmful to the fetus, starting AFL medical treatment shows complicated choice in its practice [2] . Beta-blockers cross the placenta and are associated with intra-uterine growth retardation, neonatal respiratory depression, bradycardia, and hypoglycemia, particularly for the exposure in 12-24 weeks of gestation [13] .
Calcium channel blocker may also be administered to treat atrial arrhythmias, however, since oral Verapamil is an FDA class C, intrevenous Verapamil is more likely to be avoided due to the hypotensive effect. Diltiazem was associated with teratogenicity. Digoxin is an FDA class C medication, while Amiodarone is opposed because it influenced the function of fetal thyroid in the period of gestation [1] . The hemodynamic changes in gestation time influence the pharmacokinetics of the antiarrhythmic drugs, for example, a higher dose of digoxin is required for the adequate serum level during pregnancy, compared for those used out of pregnancy [4] . presence of abnormal heart structure. [15] In patients with persistent tachyarrhythmia, including AFL, the high possibility of recurrent arrhythmias in gestation period leads to the need of performing a pre-pregnancy ablation [6,15,16] . It is known that AFL mechanism involves a critical isthmus of slow conduction restricted by anatomic structures. The CTI is involved in the re-entry circuit in typical AFL, and the implemented RF energy to this isthmus has a chance to restore.
Radiofrequency ablation proved to have high success rate to fight AFL, which is known to have been one of the atrial tachyarrhythmias. This results is noted with some smaller studies presenting 91% higher success with 1 procedure [1,7] .  [3] .
It is proved that radiofrequency catheter ablation of right sided atrial tachyarrhythmia is associated with high acute rate and significant long term recurrences, and repeated radiofrequency catheter ablation of recurrent atrial tachyarrhythmia was effective in most of the patients. However, the arrhythmogenic substance in patients with history of previous surgery proved to be complicated, and aiming for these arrhythmias needs 3D mapping that is detailed to raise the success rate of the ablation. [17]

Conclusion
Considering the efficacy and weighing the benefits, radiofrequency ablation with detailed 3D mapping prior to pregnancy is the most beneficial and safest choice for this 31 year-old woman with persistent typical AFL with a history Cone reconstruction due to Ebstein Anomaly