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 Table of Contents  
Year : 2019  |  Volume : 9  |  Issue : 2  |  Page : 105-108

Anesthetic management of a case of wolf-parkinson-white syndrome with rheumatic mitral stenosis presenting for cesarean section

1 Clinical Associate Professor of Anesthesiology, Cleveland Clinic of Lerner College of Medicine of Case Western Reserve University, Ohio, United States
2 Professor, Department of Anaesthesia and Intensive Care, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission20-Jan-2019
Date of Acceptance05-May-2019
Date of Web Publication06-Sep-2019

Correspondence Address:
Dr. Amit Jain
Anesthesiology Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi
United States
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/joacc.JOACC_6_19

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The occurrence of Wolff-Parkinson-White syndrome with rheumatic mitral stenosis is not only an uncommon entity, but also a complex scenario. Although anesthetic concerns in a parturient with Wolff-Parkinson-White syndrome have been addressed previously, to the best of our knowledge, hemodynamic consequences and anesthetic implications of Wolff-Parkinson-White syndrome with coexisting mitral stenosis have not been reported so far. We describe the successful use of a modified low-dose, low-concentration, sequential combined spinal-epidural technique for emergency cesarean section in a parturient with Wolff-Parkinson-White syndrome and severe mitral stenosis.

Keywords: Anesthetic management of WPW syndrome, Wolff-Parkinson-White syndrome, Wolf-Parkinson-White syndrome with rheumatic mitral stenosis

How to cite this article:
Jain A, Bala I. Anesthetic management of a case of wolf-parkinson-white syndrome with rheumatic mitral stenosis presenting for cesarean section. J Obstet Anaesth Crit Care 2019;9:105-8

How to cite this URL:
Jain A, Bala I. Anesthetic management of a case of wolf-parkinson-white syndrome with rheumatic mitral stenosis presenting for cesarean section. J Obstet Anaesth Crit Care [serial online] 2019 [cited 2020 Jul 11];9:105-8. Available from: http://www.joacc.com/text.asp?2019/9/2/105/266150

  Introduction Top

The coexistence of Wolff-Parkinson-White syndrome (WPW) with rheumatic mitral stenosis (MS) is extremely rare. This condition has profound hemodynamic significance and poses a lot of specific management issues, making the anesthetic management of these cases a challenge. Only a few case-reports discussing either the radiofrequency ablation (simultaneously or prior to surgery) or the surgical management of this complex scenario exist.[1],[2],[3],[4],[5],[6] To our knowledge, there is no description in the literature about the anesthetic management of a patient with WPW syndrome and severe rheumatic MS undergoing cesarean section (CS). This case report describes successful use of modified low-dose, low-concentration, sequential combined spinal-epidural (CSE) technique for emergency CS in a patient with WPW syndrome and severe MS. The patient provided her consent to publish details about this case.

  Case Report Top

A 25-year-old multigravid parturient who was admitted in the obstetric ward at 37 weeks gestation in view of leakage of clear fluid per vaginum, was referred to obstetric anesthesia services for possible labor analgesia. She had received no prior antenatal care. Her obstetric history included an unsupervised, uneventful pregnancy 4 years back and one spontaneous miscarriage at 12 weeks gestation an year back. Her medical history revealed that at 32 weeks gestation, she had presented to a peripheral hospital with complaints of palpitations, dyspnea on exertion, and easy fatigability when a 12-lead electrocardiogram (ECG) revealed supraventricular tachycardia (SVT). Carotid sinus massage reduced heart rate from over 200 beats min −1 to 180 beats min −1 and adenosine 6 mg given in divided doses restored normal sinus rhythm. Repeat ECG revealed sinus rhythm with PR interval of 0.09 sec, QRS duration of 0.14 sec, and prominent delta waves in all leads suggesting right posteroseptal accessory pathway [Figure 1]. Echocardiography showed severe MS with mitral valve area of 1.0 cm 2. Cardiologists made the diagnosis of WPW syndrome with severe MS of rheumatic origin. Considering risk-benefits of electrophysiological (EP) study and radiofrequency ablation (RFA) at 32 weeks of pregnancy, a decision for medical management was taken. Oral metoprolol 50 mg and furosemide 40 mg, once a day, was prescribed.
Figure 1: ECG showing short PR interval, with broad QRS complex and well-marked delta waves in most leads (marked as black arrows in most leads). The mean frontal plane QRS axis of -15 degree, negative polarity of QRS in V1, positive QRS deflections in leads V2 and V3 along with positive delta waves in lead I and lead AVL suggest WPW syndrome with right posteroseptal accessory pathway

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On presentation, at 37 weeks' gestation, she was New York Heart Association class 2. Physical examination revealed blood pressure of 100/70 mmHg with a regular pulse of 70 beats per min. On auscultation, grade 4/6 mid-diastolic murmur with loud S1 was heard in the mitral area. She had hemoglobin of 9.8 gm% with normal platelet count, serum electrolytes, and renal function test. Repeat ECG and echocardiography confirmed the prior diagnosis of WPW syndrome with severe MS.

However, in view of thick meconium-stained liquor, patient was re-evaluated by the anesthesia team for emergency CS. High-risk consent in view of WPW syndrome with severe MS and prior history of SVT was taken. Intravenous ranitidine 50 mg and metoclopromide 10 mg were administered before shifting the patient to the operating room and a low-concentration low-dose CSE was planned.

Cardiotonic medications and equipments for resuscitation including defibrillator were kept ready. After establishing ECG and pulse oximeter monitoring, a 16-gauge cannula was inserted under local anesthesia for intravenous access. A 20-gauge arterial line for continuous invasive arterial pressure monitoring was secured in left radial artery under local anesthesia. Her baseline blood pressure was 96/55 mmHg with heart rate of 80 beats per min. Defibrillator pads were placed on the patient's chest and connected to an external defibrillator as a stand-by to treat any significant arrhythmia that may occur. CSE was inserted in L3-4 space using needle-through-needle technique (18 G Tuohy's/27 G Whitacare needle). After an initial intrathecal dose of hyperbaric bupivacaine 5 mg (1.0 mL of 0.5%) and fentanyl 25 μgm diluted in normal saline to 2 ml solution, the patient was placed supine with left lateral tilt. Oxygen was administered through face mask and continuous fetal heart rate monitoring was established. After 8 min, T4 level of sensory block with the modified bromage score of 3 was attained and surgery started. Coloading was achieved with 150 ml of Ringer's lactate. The patient remained hemodynamically stable and received only two boluses of phenylephrine 50 μg to maintain mean arterial pressure above 70 mmHg. A male infant weighing 2.075 kg with APGAR score of 8 at 1 minute and 9 at 5 minutes was delivered. Umbilical cord blood pH and PaO2 were 7.29 and 20 mmHg, respectively. Intravenous infusion of oxytocin at the rate of 3.0 IU/h was started. As the level of sensory block regressed to T6 dermatome after 35 min, 6 ml of 1% lignocaine with 3 mg preservative-free morphine was given over a period of 5 min. Estimated blood loss was 300 ml and 500 ml of crystalloid was infused intraoperatively. Surgery lasted for 50 minutes. Postoperatively, the patient was monitored in the high dependency unit for 24 hours. A repeat dose of epidural morphine 3 mg was given after 24 hours for analgesia. After 7 days of uneventful hospital stay, the patient was discharged with the advice to continue her cardiac medications.

  Discussion Top

There is scarcity of literature regarding the anesthetic management of WPW syndrome with rheumatic MS. Recently, Sinha et al.[7] described the anesthetic implications of WPW syndrome with Ebstein anomaly and congenital MS in a patient scheduled for cardiac surgery. To the best of our knowledge, the anesthetic implications of WPW syndrome with rheumatic MS have not been described for noncardiac surgery.

WPW syndrome is often asymptomatic although in a small percentage of cases, two types of arrhythmias—atrial fibrillation (AF) which can progress to ventricular fibrillation and circus movement type of re-entrant tachycardia causing paroxysmal SVT—may result in significant morbidity and mortality.[8],[9] Concomitant cardiac lesions like MS may further predispose to AF.[10] Alper et al.[3] reported multiorgan failure in their patient of WPW syndrome and rheumatic MS with AF. The authors suggested that, in this patient, the accessory pathways with fast conduction and short refractory periods resulted in markedly shortened diastole and high ventricular conduction rates which in the presence of MS reduced ventricular filling to a point that pump failure ensued. This explanation was supported by the fact that liver function tests and serum creatinine returned to normal values five days after the radiofrequency ablation. Thus, in a patient with WPW syndrome and severe MS, hemodynamics are readily impaired by the appearance of SVT or AF, management of which is often difficult.

Pregnancy is associated with an increased risk and exacerbation of symptoms of paroxysmal SVT, endangering the lives of both mother and fetus.[10] Drugs used during labor or CS (tocolytics and oxytocics) may also precipitate SVT.[10] As the commonly used antiarrhythmic drugs cross the placenta, their use during pregnancy is best reserved for those with hemodynamic changes, severe symptoms or sustained arrhythmias.[10],[11] Digoxin and verapamil are, however, contraindicated in patients of WPW syndrome with preexcited AF because they shorten the refractory period of the accessory pathway and may paradoxically increase the ventricular rate predisposing to ventricular fibrillation.[12] However, beta-blockers are generally well tolerated in pregnancy.[10] They are the agents of choice in WPW, where AV nodal blocking drugs may lead to acceleration of conduction through the accessory pathway.

Adenosine is rapidly becoming the first choice of therapy for termination of SVT. It transiently depresses sinus node activity, slows atrioventricular conduction and effectively terminates SVT.[10] Safety and efficacy of adenosine during pregnancy has been proved by various case reports and studies.[11] As the concentration of the enzyme responsible for adenosine degradation, adenosine deaminase, declines during pregnancy, doses of 6–12 mg are considered adequate.[10] Our patient was treated with 6 mg adenosine, at 32 weeks of gestation when she developed sudden onset SVT. Once the normal sinus rhythm was achieved, she was prescribed tab metoprolol which was continued in the perioperative period. Although no serious complications have been reported with use of adenosine during pregnancy, one report of transient fetal bradycardia [13] has been published. So monitoring of fetal heart rate should be done during its use in pregnancy.

Synchronized electrical cardioversion may become necessary for hemodynamically unstable paroxysmal SVT resistant to pharmacological therapy.[10] DC cardioversion has been used at all stages in pregnancy without significant complication. The amount of current reaching the fetus is thought to be negligible.[14]

RFA of the accessory pathway is a definitive treatment in WPW syndrome. Owing to radiation exposure and other uncertain risks for the mother and fetus, catheter ablation has rarely been performed and is often delayed until the postpartum period.[15] Recently, zero-fluoroscopy RFA has become an option during pregnancy.[15] However, our patient was first diagnosed with WPW syndrome with severe MS in the third trimester of pregnancy; as a result, EP study and RFA under anesthesia was not without untoward risks to mother and fetus. Furthermore, the expertise and the setup (electroanatomical system) necessary for zero-fluoroscopy RFA were not available at our institute at that time.

In a symptomatic patient of WPW syndrome, chances of life-threatening arrhythmias are highly increased especially under anesthesia as compared to asymptomatic ones.[13] Unmasking of WPW syndrome under anesthesia has been mentioned as anesthetic drugs tend to change the physiology of the atrioventricular conduction.[16],[17] While volatile anesthetic agents may precipitate conduction via. preexisting anomalous pathway leading to manifestation of WPW pattern,[18] drugs like atropine, glycopyrrolate, and ketamine precipitate tachycardia resulting in paroxysmal SVT or atrial fibrillation.[15] The chances of arrhythmias are further increased during laryngoscopy, pain or lighter plane of anesthesia. Regional anesthesia avoids all these complications; however, hemodynamic instability following central neuraaxial block may be worrisome.[10]

Coexisting severe MS in our patient with WPW syndrome further increased the risk of hemodynamic instability. Although there is no evidence to support any particular technique, low-dose spinal anesthesia given as part of a CSE technique has been used effectively for CS in patients with severe MS. Hamlyn et al.[16] used intrathecal hyperbaric bupivacaine 5 mg with fentanyl 20 μg, followed by 5.0 ml epidural normal saline to obtain T4 sensory level. We managed to obtain T4 level of sensory loss by increasing the volume of spinal drug to 2 ml by adding saline. A low concentration of bupivacaine (0.25%) with fentanyl 25 μg might have benefited our patient by maintaining stable hemodynamics. Six ml lignocaine 1% was given epidurally to supplement spinal anesthesia when two segment regression of sensory block occurred. Epidural morphine was added for postoperative analgesia which is desirable in a patient with cardiovascular compromise.

Preloading during regional anesthesia, helps prevent reduction in atrial filling and reduces sympathomimetic requirements, thereby, decreasing the arrhythmogenecity of heart.[10] However, in the presence of MS coloading has an advantage over preloading, as it avoids the risk of sudden increase in left atrial pressure and pulmonary edema. Avoidance of aortocaval compression by using a left tilt is also important to maintain atrial filling in pregnant females.[10] We did not insert central venous catheter to avoid delay in starting the emergency CS.

Vasopressors like ephedrine used to treat hypotension after subarachnoid block, initiate tachycardia.[10] But phenylephrine treats hypotension without causing increase in heart rate. It may increase vagal tone by indirectly stimulating baroreceptor reflexes and thus reduces the risk of SVT in patients with WPW syndrome. Even phenylephrine has been used to terminate SVT occurring following induction of anesthesia in a patient with WPW syndrome during.[17] The present patient required two boluses of 50 μg phenylephrine to maintain mean arterial pressure above 70 mmHg. As oxytocin may induce tachyarrhythmia in response to marked decrease in systemic vascular resistance and may adversely increase pulmonary vascular resistance in patient with MS,[16] we administered oxytocin as slow intravenous infusion rather than by bolus.

To conclude, in a patient with WPW syndrome and severe MS, a combination of low-dose, low-concentration spinal anesthesia with the flexibility of epidural supplementation provided hemodynamic stability during CS with good fetal outcome. Avoidance of general anesthetic drugs, fluid coloading and treating hypotension with phenylephrine were appropriate for preventing dangerous arrhythmias in our patient.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

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Namboodiri N, Rajeev E, Dora SK, Tharakan JA. Ebstein's anomaly, Wolff-Parkinson-White syndrome and rheumatic mitral stenosis: Role for combined electrophysiological and surgical management. Singapore Med J 2007;48:e133-5.  Back to cited text no. 2
Alper AT, Hasdemir H, Akyol A. Wolff-Parkinson-White Syndrome and rheumatic mitral stenosis: An uncommon coincidence that can cause severe hemodynamic disturbance. Indian Pacing Electrophysiol J 2008;8:304-7.  Back to cited text no. 3
Ikeshita M, Yamate N, Tanaka S, Asano T, Harada A, Yamauchi S, et al. A case report of simultaneous surgery for Wolff- Parkinson-White syndrome combined with hemolytic anemia and mitral stenosis. Jpn Circ J 1996;60:171-6.  Back to cited text no. 4
Lee CM, Lee YT, Liau CS Chen MF, Chen CC, Chu SH, et al. A case of Wolff-Parkinson-White syndrome with bilateral atrio-ventricular accessory pathways and mitral stenosis successfully treated by surgery. Taiwan Yi Xue Hui Za Zhi 1987;86:1012-7.  Back to cited text no. 5
Lesh MD, Rankin JS. Coexisting Wolff-Parkinson-White syndrome and cardiac valve disease: A staged treatment approach with catheter ablation before operation. J Thorac Cardiovasc Surg 1993;106:1234-6.  Back to cited text no. 6
Sinha PK, Kumar B, Varma PK. Anesthetic management for surgical repair of Ebstein's anomaly along with coexistent Wolff-Parkinson-White syndrome in a patient with severe mitral stenosis. Ann Card Anaesth 2010;13:154-8.  Back to cited text no. 7
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Gillette PC, Garson A, Kugler JD. Wolff-Parkinson-White syndrome in children: Electrophysiologic and pharmacologic characteristics. Circulation 1979;60:1487-95.  Back to cited text no. 8
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Robins K, Lyons G. Supraventricular tachycardia in pregnancy. Br J Anaesth 2004;92:140-3.  Back to cited text no. 10
Cox JL, Gardner MJ. Treatment of cardiac arrhythmias during pregnancy. Prog Cardiovasc Dis 1993;36;137-78.  Back to cited text no. 11
Sahu S, Karna ST, Karna A, Lata I, Kapoor D. Anaesthetic management of Wolff-Parkinson-White syndrome for hysterectomy. Indian J Anaesth 2011;55:378-80.  Back to cited text no. 12
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Wellens HJJ, Smeets JL, Gorgeis AP. WPW syndrome. In: Mandel WJJ, ed. Cardiac arrhythmias, III rd ed. Philadelphia: J. B. Lippincott Company Inc; 1995.p. 389-413.  Back to cited text no. 13
Klepper I. Cardioversion in late pregnancy. Anaesthesia 1981;36:611-6.  Back to cited text no. 14
Chen G, Sun G, Xu R, Chen X, Yang L, Bai Y, et al. Zero-fluoroscopy catheter ablation of severe drug-resistant arrhythmia guided by Ensite NavX system during pregnancy: Two case reports and literature review. Medicine (Baltimore) 2016;95:e4487.  Back to cited text no. 15
Low-dose sequential combined spinal-epidural: An anesthetic technique for cesarean section in patients with significant cardiac disease. Int J Obstet Anesth 2005;14:355-61.  Back to cited text no. 16
Jacobson L, Turnquist K, Masleys S. WPW syndrome: Termination of paroxysmal supraventricular tachycardia with phenylephrine. Anaesthesia 1985;40:657-60.  Back to cited text no. 17
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