Atrio-Ventricular Septal Defect in Pregnant Women , How to Deal with It : A Case Study

Pregnancy is not always well tolerated in women with congenital heart disease (CHD) such as atrio-ventricular septal defect (AVSD), predominantly due to heart failure deterioration and increasing pulmonary hypertension (PH). Managements of those patients are challenging, especially during third trimester and after delivery care. Decision about time of termination, mode of delivery and anesthetic management are also debatable. In this article we report two similar cases of pregnant women with AVSD and severe PH. The first patient was 27 years old, 28-29 weeks pregnant came with shortness of breath. She had history of miscarriage once. Based on her transthoracal echocardiography, she was diagnosed with AVSD partial type (primum ASD) with severe PH and then treated with intravenous furosemide, oral beraprost and oral sildenafil. The second patient was 27 years old 30-31 weeks pregnant with shortness of breath and appeared cyanotic. She delivered her first child spontaneously without any symptoms. Based on her transthoracal echocardiography she was diagnosed with AVSD transitional type (large primum ASD with small inlet VSD) and Eisenmenger syndrome. She was treated with intravenous furosemide and oral beraprost. Those two patients underwent planned C-section under general anesthesia, both babies were survived but the patient did not survived several days after the procedure due to PH crisis. Until now, management PH associated with CHD in pregnant women is complex. Fluid management and pulmonary artery hypertension (PAH)targeted therapies are important. Mode of delivery on this cases is also remain debated. Some studies stated planned C-section might be a better choice and combination epidural and lowdose spinal anesthesia might be better than general anesthesia. At the end, when a woman with CHD and PH chooses to continue pregnancy, multidisciplinary team approach is crucial to achieve good outcomes.

with suspected mitral stenosis from the previous hospital.She felt shortness of breath since the last 2 weeks and had to sit while sleeping at night.Patient had history of heart disease since she was 10 years old but rarely went to the doctor to get medication.She had history of spontaneous abortion once, at that time the second pregnancy was 28-29 weeks gestation.On physical examination, blood pressure was 105/73 mmHg, heart rate was 90 beat/ min regular, respiratory rate was 28 time/ min, and oxygen saturation was 96% breathing room air.She looked pale with no cyanosis.On cardiac auscultation there were systolic murmur in intercostal space II left parasternal line grade III / VI, systolic murmur in intercostal space IV left parasternal line and holosystolic murmur in the apex grade IV / VI radiates to axilla.On the lungs we found bibasilar fine rales.There was pitting edema on her feet.The abnormal laboratory result was only mild anemia (haemoglobin 11 g/dL) with mild hipoalbuminemia (albumin 3.2 g/dL).From the chest X-ray, there was cardiomegaly with prominent pulmonary cone and increased pulmonary vascularity (Figure 1).From the transthoracal echocardiography (TTE): there was mitral cleft with severe mitral regurgitation (RV 83 ml), severe tricuspid regurgitation (maxPG 84 mmHg), and moderate pulmonic regurgitation.
There was intra atrial septal defect 3.6 cm left to right shunt without anterior rim (Figure 2).We assessed the patient with primum atrial septal

INTRODUCTION
Atrio-Ventricular Septal Defect (AVSD) occurs due to fusion failure of the embryonic endocardial chusion in primitive AV channels.
The characteristic of AVSD is deficient atrioventricular (AV) septation and a variety of AV valve anomalies. 1The prevalence is only 4-5% of all congenital heart defects.Most AVSD patients can reach adulthood without marked clinical symptoms, and sometimes get pregnant and is able to deliver the baby spontaneously without any symptoms 2 .Pregnancy in women with AVSD is not always well tolerated to these patients, as there may be worsening of heart failure and an increasing of regurgitation of the AV valve.In addition, AVSD patients who become pregnant usually have pulmonary hypertension

DISCUSSION
These two patients were in stable condition before pregnancy, moreover the second  Ultimately, the necessary increase in cardiac output cannot be achieved resulting in right heart failure. 3,7Then these two pregnant patients were treated with bed rest, oxygenation, intravenous diuretic for volume overload and pulmonary vasodilators or PAH targeted therapy.The choice for PAH targeted therapy for these patients was beraprost (prostacyclin analog), its effect is vasodilatation on pulmonary vasculature.Even though the safety of beraprost during pregnancy was unknown or has not been established (and should not be used in pregnant woman), we decided to give the patient beraprost because of its availability, and also economic issue of it.
We also gave the first patient sildenafil (PDE5 inhibitor).Sildenafil causes vasodilation of the pulmonary vascular bed and in the systemic circulation.It also has a positive inotropic effect on the hypertrophic right ventricle with category B medication (safe for pregnancy).Using sildenafil to treat PH in pregnancy has been described in case reports and appears to be safe, but experience is still limited.We did not have endothelin receptor antagonist (ERA) such as bosentan or illoprost at that time.Moreover Bosentan was are pregnancy category X drugs and contraindicated in pregnancy. 3ter the careful management, those two patient underwent PH evaluation using TTE.
The first patient was able to reach moderate PH (from severe PH), but the second patient was not able to achieve moderate PH (she was already in Eisenmenger state).After having discussion with Obstetrician and Anesthesiologist then the decision for mode of termination was elective SC under general anesthesia.Recommendation for mode of delivery depends on the individual's condition and is still debatable.Vaginal delivery increases cardiac output and blood pressure especially during contractions (34% of CO will increase after cervical dilatation).This is a problem in mothers with pulmonary hypertension because unable to increase the cardiac output.
Elective SC is preferred over emergency.The type of anesthesia also become another problems.
General anesthesia drug administration has unfavorable effects such as cardiodepressive (by volatile agents) and increased pulmonary vascular resistance (PVR) during intubation and positive pressure ventilation.Some studies recommend combination epidural anesthesia and low-dose spinal anesthesia to avoid vasodilatation. 3,7,8 this case, few days after the SC procedure both of the patient's condition were deteriorated.
The blood pressure fell below 80/50 mmHg and were not respond to vassopressor and inotropic.

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Figure 1.Chest X-ray showed enlargement of the heart with CTR 70%.Prominent pulmonary cone (white arrow) with increasing pulmonary vascularity.CTR : Cardio Thoracic Ratio.

Figure 1 .
Figure 1.Chest X-ray showed enlargement of the heart with CTR 70%.Prominent pulmonary conus (white arrow) with increasing pulmonary vascularity.CTR : Cardio Thoracic Ratio.
found cleft mitral with severe mitral regurgitation (RV 50 ml), severe tricuspid regurgitation (maxPG 135.6 mmHg) with moderate pulmonal regurgitation.Right ventricle systolic function was decreased (TAPSE 1.5 cm).There was large intra ASD 6.2 cm bidirectional shunt right to left shunt dominantly and also small inlet VSD (0.3 cm).The estimation pulmonary arterial systolic pressure (PASP) was 145 mmHg.(Figure 4) We assessed the patient with AVSD transitional type (large primum ASD with small inlet VSD) and Eisenmenger Syndrome.We gave her oxygenation with simple mask, intravenous furosemide b.i.d and oral beraprost 20 mcg t.i.d.The patient was then performed an evaluation of PH 2 days after therapy, the PASP was decreased but still in severe PH.Then the patient underwent SC procedure under GA, the baby born weighing 1000 g, Apgar score 3-5.However, during intensive care management, the condition was deteriorating.The oxygen saturation was decreased and blood pressure fell to 70/40 mmHg which not respond with norepinephrin and finally the patient passed away on the second day after SC due to pulmonary hypertension crisis.DISCUSSION These two patients were in stable condition before pregnancy, moreover the second patient was able to deliver her baby spontaneously without serious complications.While not in pregnancy these two patients should had been in condition similar to those of secundum type ASD, in which the right atrium (RA) and right ventricle (RV) are dilated with increased pulmonary blood flow.The cleft mitral valve is usually insignificant from a hemodynamic point of view because blood regurgitated into the left atrium (LA) is immediately shunted to the RA, thereby decompressing the LA.The symptoms are shortness of breath, and easily fatigue. 5,6But during pregnancy, the hemodynamic is different.norepinephrinand finally the patient passed away on the second day after SC due to pulmonary hypertension crisis.

3 CONCLUSION
This condition was probably due to PH crisis which lead to low cardiac output and cardiogenic shock.Finally these two patients passed away during intensive care management post delivery care.This condition appropriate with most studies.They show maternal deaths in patients with pulmonary hypertension occur in the third trimester and the first months after delivery.Most of it is due to PH crisis, pulmonary thrombosis and refractory right heart failure.Risk factors for the occurrence of maternal death including late hospitalization, severity of pulmonary hypertension, and general anesthesia.We report two difficult cases of AVSD and severe PH in pregnant women.Both of patients were treated carefully with oxygenation, fluid restriction and pulmonary vasodilators, but few days after the SC procedure they experienced pulmonary hypertension crisis and did not survived.Due to a high maternal mortality risk, women with severe pulmonary hypertension and Eisenmenger syndrome had to be given a good counseling before pregnancy.If pregnancy occurs then termination should be considered, however when termination is declined, pregnancy and delivery should be managed by multidisciplinary services to achieving successful outcomes in these difficult cases.