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f�f0�f6dR�k6]r�k�d�d�kJ�dpVL�6_�,�VVVff�^dVVV�f����^^^d��^^^�^^^JJJJJJ����ANESTHETIC APPROACH  OF TWO PARTURIENT WITH EISENMENGER SYNDROME 
(Cases report)

Yasemin G�NE^ 1
Bar1_ G�ZEL2
Hafize YALINIZ* 3
Murat G�ND�Z 1
  



1 Cukurova University Faculty of Medicine Department of Aneesthesiology,01330 Saricam-Adana / TURKEY
2 Cukurova University Faculty of Medicine Department of  Obstetric and Gynecology, 01330 Saricam-Adana / TURKEY
 3   Cukurova University Faculty of Medicine Department of  Cardiovascular Surgery, 01330 Saricam-Adana / TURKEY





*Corresponding Adress:    Asssoc. Prof. Hafize Yaliniz 
                                        �ukurova University Faculty of Medicine 
               Department of   Cardiovascular Surgery  
                01330 Balcal1 Adana/TURKEY
e-mail:                              HYPERLINK "mailto:hafize101@yahoo.com" hafize101@yahoo.com
Tel-fax:                           +90 532 7114036

ABSTRACT
We reported that two cases  a 34-year-old (27 weeks  gestation) and a 28 year-old (  35 weeks� gestation)  primigravida, pregnant woman with Eisenmenger�s syndrome whose baby  was delivered by Caesarean section under with ketamine-remifentanil anesthesia or combined epidural spinal anesthesia. The first patient died in  Intensive Care Unite on the second postoperative day due to a pulmonary embolism or abrupt hypotension. 
Keywords;  Eisenmenger�s Syndrome, Pregnancy, Cesarean Section, General Anesthesia, 









































INTRODUCTION

Eisenmenger�s syndrome consists of pulmonary hypertension, a right-to-left  intracardiac shunt resulting from pulmonary hypertension superimposed on a previously left-to-right shunt, and arterial hypoxemia[1-7].In previous reports, a variety of techniques have been used to anaesthetize patients with Eisenmenger�s syndrome for Caesarean Section in parturient [1,9-11]. These cases confirms the maternal outcome of a pregnant  womans  with Eisenmenger�s Syndrome. 
Patient 1:
A 34-year-old  woman, primigravida, 27 weeks gestation who has been diagnosed as ventricular septal defect  with Eisenmenger syndrome. 
She had devoloped proggressive dyspnea and tachypnea after 24.5 gestational weeks.   Caesarean section was planned immediately.   The electrocardiogram showed sinus tachycardia and right ventricular hypertrophy. Echocardiography and doppler USG demonstrated  right atrium and ventricular  enlargement,  defect of  interventricular septum (10 mm), and pulmonary  hypertension      [ pulmonary arterial pressure (PAP): 128 mmHg]. 
The laboratory assessment revealed hemoglobin  17 g/dl, and proteinuria. Blood gas analysis revealed  pH 7.452,     P02  43.2  mmHg, PC02  22.6 mmHg.  Routine monitors were applied (heart rate,  systolic, diastolic and mean arterial pressure [MAP], Sp02, and urine output). Peripheral oxygen saturation (Sp02)  was 80% when she was breathing room air and after pre-oxygenation Sp02 elevated 87 %. 
General anesthesia was chosen in this case. Before anesthesia induction an arterial line and a central venous catheter were inserted. Anaesthesia.was induced with intravenous ketamine.  Tracheal intubation was facilitated with vecuronium and anesthesia was maintained by ketamine (1.5 mg/kg/h ) and remifentanil infusion (0.15 �g/kg/min). The respiratory rate was adjusted to provide normocarbia.  The targeted range of  MAP values was 75-90 mmHg.  
An unhealthy newborn was delivered, (birth weight 600 g) and  the APGAR scores were  two  and four  at 1 and 5 min, respectively.  Throughout the operation hemodynamic parameters remained stable. At the end of the operation, the trachea of patient was extubated and the patient was transferred to the Intensive Care Unite(ICU). 
 	Oxygen saturation of patient was 80-86% during oxygen therapy in ICU.  Nitroglycerin infusion was started for to reduce pulmonary vascular resistance  (PAP: 105 mmHg) .  Anticoagulant therapy was planned 1 days after caeserean sectio.  After 27 h of uneventful recovery,   during the second  postoperative day the patient developed signs and symptoms of shock, presumably due to a pulmonary embolysm. Therefore, the patient was intubated immediately.  Epinephrine infusion was started  to provide rising in systemic vascular resistance (SVR).  During the mechanical ventilation was appliying,  cyanosis was persistent, whereas cardiopulmonary arrest was occurred.  Vigorous cardiopulmonary resuscitation was performed but the patient died. Postmortem examination was declined by her family.
Patient 2:
A 28-year-old  woman, primigravida, 35 weeks gestation who has been diagnosed as ventricular septal defect  with Eisenmenger syndrome. 
	Elective caesarean section was planned..  On physical assessment the pulse rate was 96/min, respiratory rate 26/min and blood pressure 150/120 mmHg. There was marked cyanosis and clubbing of fingers.  The electrocardiogram showed sinus tachycardia and right ventricular hypertrophy. Echocardiography demonstrated  right atrium and ventricular  minimal dilatation,  defect of  muscular type ventricular septal defect , and pulmonary  hypertension  [ pulmonary arterial pressure (PAP): 95 mmHg]. Ejection fraction ( Teicholz method )  was measured 60%.  
	The laboratory assessment revealed hemoglobin  18.6 g/dl, hct 56.9%.  Blood gas analysis revealed  pH 7.402,P02  52  mmHg,PC02  29.1 mmHg. The coagulogram was normal (prothrombin time 12.9 s/control 11-15 s, partial thromboplastin time 23.4s/ control 25-34.6 s), INR 1.03/control 0.85-1.25.Routine monitors were applied (heart rate,  systolic, diastolic and mean arterial pressure [MAP], Sp02, and urine output). Peripheral oxygen saturation (Sp02)  was 78% when she was breathing room air and after pre-oxygenation Sp02 elevated 84 %. 
	In this patient, regional anesthesia  (Combined epidural spinal anesthesia) was chosen.  Subarachnoid fentanil (25 �g ) and morphine (100 �g) were applied, then  lidocaine (100mg) was used via epidural catheter.  Throughout the operation, hemodynamic parameters remained stable; the MAP 75-90 mmHg) , oxygen saturation (75-%� 85% ),   respectively. 
	A healthy  infants  were  delivered. In the first infant  was measured   birth weight 1400 g and an Apgar score of 5 and 7 at 1 and 5 min , respectively.. In the second infant  was measured   birth weight 1600 g and an Apgar score of 6 and 8 at 1 and 5 min. 
At the end of the operation, the patient was transferred to the ICU. Oxygen saturation of patient was 80-86% during oxygen therapy in ICU.  Anticoagulant therapy was started planned 1 days after caeserean sectio.  After 48 h of recovery, the patient was transfered   to the Obstetric Clinic.  
DISCUSSION
Adult patients with Eisenmenger syndrome are at increased risk with noncardiac surgery,but with current/modern techniques, the risk of death is less than previously thought. In the vast majority of cases,noncardiac surgery can be undertaken without substantial morbidity,and early extubation is achievable. Pregnancy is contraindicated in Eisenmenger�s syndrome. Maternal hypoxemia can also diminish fetal oxygenation and result in intrauterine growth restriction.  In addition, the maternal (30�50%) and  perinatal mortality rate is higher in Eisenmenger syndrome[4,7,8,11-14]. Maternal mortality in first, second, and third pregnancies was not significantly different.Regardless of the anesthetic technique employed, some life-threatening complications such as hypoxemia, cardiac dysrhythmias, and thromboembolic events occur in the postpartum period [7,9-11]. 
	In previous reports, a variety of techniques have been used to anaesthetize patients with Eisenmenger�ssyndrome for non-cardiac surgery[1,2,10].Anesthetic management focuses on maintaining adequate SVR, intravascular volume, and preventing increases in pulmonary vascular resistance[4,5,9,13,14].The use of intraarterial and central venous catheters may facilitate hemodynamic management. By contrast, the use of pulmonary artery catheters and the role of anticoagulation  remains controversial[5,12,14,16].Judicious use of anticoagulants should be made when indications outweigh the risk of bleeding. We used anticoagulation therapy in our  cases during the early postpartum period (24 h).
	We chose cesarean section for the route of delivery in these cases  due to obstetric indications. We used CVP catheter, pulse oximeter and EKG to monitor in our patients, but we did not use a pulmonary catheter. Regional anesthesia remains preferable to general anesthesia and these  anesthetic techniques should be used with extreme caution  Although epidural anesthesia has been successfully  employed for  minor surgeries such as tubal ligation and cesarean section, the resulting sympathetic blockade and decrease in both preload and afterload may be very hazardous, so general anesthesia is preferable for these patients[5,14,17,18]. In the second patient we observed combined epidural spinal anesthesia for caeserean section succesfully.  Subarachnoid opioids may prove optimal in some circumstances. We used only subarachnoid opioids, (fentanil; 25 �g  and morphine;100 �g) did not use subarachnoid local anesthetics.   Throughout the operation, hemodynamic parameters remained stable; the MAP 75-90 mmHg) , oxygen saturation (75-%� 85% ),   respectively. 
Should regional anesthesia be contraindicated, a controlled induction of general anesthesia is recommended as critical decreases in SVR can be minimized[5,8,9,18]. Kopka et al.[2] reported that sevoflurane and total intravenous anesthesia to provide general anesthesia two non-parturients  with Eisenmenger�s syndrome.  Phupong et al.[1]  reported that  a  pregnant woman with Eisenmenger�s Syndrome and preeclampsia. In our first case, Caesarean section was performed by general anesthesia but  the patient died on the second postoperative day due to  pulmonary embolism.  In our first case, anesthesia induction was induced  with ketamine.  Ketamine increases to SVR. Intravenously opioid administration may minimize decreases in SVR.  Also, anesthesia  was maintained with ketamine and remifentanil continuous infusion. Any transient hypertension or hypotension did not  occure during the drug infusion, but after 27 h of uneventful recovery,  the patient died on the second postoperative day due to  pulmonary embolism.   Our second patient was younger than the first patient and  gestational age was older.  Also, pulmonary arterial pressure  was lower  than the first  patient.      
	Our results showed  that either combined epidural spinal anesthesia  or  general anesthesia with ketamine-remifentanil infusion may offer good control of intraoperative hemodynamic stability  in the   parturients with Eisenmenger�s syndrome.   Also,  the probable fatal outcome of pregnancy should be explained to the patients and her family. The management of these patients especially complicated by pulmonary embolism requires the availability of neonatal and obstetric intensive care units.

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14-Kuczkowsk1 KM.(2004) Labor analgesia for the parturient with cardiac disease: what does an obstetrician nee08����������������					 	&	�����ƽ��񰠑�t��e�S�FS��h�S�5�CJaJmHsH"h�E�h�E�5�CJH*aJmHsHh�S�5�CJH*aJmHsHh�-%hIG5�CJaJmHsHh�E�5�CJaJmHsHh�E�5�CJH*aJmHsHh�-%h��5�CJaJmHsHh�I�5�CJaJmHsHhIG5�CJaJh��5�CJaJh�-%h�b�5�CJaJhUG�5�CJPJaJhIG5�CJPJaJh�-%h�b�5�CJPJaJ�����	 	&	(	*	,	�	k
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15- Pitts JA, Crosby WM, Basta LL.(1977) Eisenmenger�s syndrome in pregnancy: does heparin prophylaxis improve the maternal mortality rate? Am Heart J.  93 :321�326.
16- Jeyamalar R, Sivanesaratnam V, Kuppuvelumani P. (1992) Eisenmenger syndrome in pregnancy. Aust N Z J Obstet Gynaecol 32 :275�277.
17-Martin JT, Tautz TJ, Antognini  JF.  (2002) Safety of regional anesthesia in Eisenmenger's syndrome. Reg Anesth Pain Med, 27: 509-513.
18-Solanki SL, Vaishnav V,  Vijay AK.(2010) Non Cardiac Surgery in a Patient with Eisenmenger Syndrome- Anaesthesiologist's Challenge. J Anaesth Clin Pharmacol26(4): 539-540
19- Lumley J,Whitwam JG,Morgan M.(1977) General anesthesia in the presence of Eisenmenger's syndrome. Anesth Analg, 56:543-547. 










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