Year : 2010 | Volume
: 13 | Issue : 2 | Page : 154--158
Anesthetic management for surgical repair of Ebstein's anomaly along with coexistent Wolff-Parkinson-White syndrome in a patient with severe mitral stenosis
Prabhat Kumar Sinha1, Bhupesh Kumar2, Praveen Kerala Varma3,
1 Consultant Anesthesiologist, Campbellton Regional Hospital, Campbellton, NB, Canada
2 Department of Anesthesia, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram-695011, Kerala, India
3 Department of Cardiac Surgery, Brigham and Women Hospital, Harvard University, Boston, USA
Prabhat Kumar Sinha
Consultant Anesthesiologist, Campbellton Regional Hospital, 189, Lily Lake Road, Campbellton, NB, E3N3H3
Ebstein«SQ»s anomaly (EA) is the most common cause of congenital tricuspid regurgitation. The associated anomalies commonly seen are atrial septal defect or patent foramen ovale and accessory conduction pathways. Its association with coexisting mitral stenosis (MS) has uncommonly been described. The hemodynamic consequences and anesthetic implications, of a combination of EA and rheumatic MS, have not so far been discussed in the literature. We report successful anesthetic management of a repair of EA and mitral valve replacement in a patient with coexisting Wolff-Parkinson-White (WPW) syndrome.
|How to cite this article:|
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;13:154-158
|How to cite this URL:|
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 [serial online] 2010 [cited 2022 Jul 2 ];13:154-158
Available from: https://www.annals.in/text.asp?2010/13/2/154/62934
EA is the most common cause of congenital tricuspid regurgitation (TR) . The associated anomalies commonly noted are, ASD and ACP.  Its association with MS has uncommonly been described. , Although anesthetic concerns in EA have been addressed previously but to the best of our knowledge, hemodynamic consequences and anesthetic implications of EA with coexisting MS have not been reported so far.  We report a case of EA along with Wolff-Parkinson-White (WPW) syndrome and coexisting MS who underwent Danielson's repair and MVR.
A 23-year-old female, weighing 48 kg, presented to the hospital with history of palpitation and breathlessness on exertion for several years. She had rheumatic fever in childhood.
Her physical examination revealed regular pulse rate 86/min, arterial blood pressure was 110/70 mm Hg with raised jugular venous pressure and prominent a-wave. There was no cyanosis. Auscultation revealed a mid-diastolic murmur with presystolic accentuation at apex and end-diastolic murmur of grade 2/6 at left lower sternal border. She was receiving oral triamterine 50 mg and benzthiazide 25 mg daily. Laboratory test results were unremarkable. Electrocardiogram (ECG) showed sinus rhythm with pre-excitation suggesting right free wall ACP [Figure 1]. Chest X-ray revealed enlarged right atrium (RA) and features of pulmonary venous hypertension. Preoperative transthoracic echocardiography (TTE) showed biatrial and right ventricular (RV) enlargement with interventricular septum shifted towards left, thickened noncalcific anterior, and posterior mitral leaflets, severe MS (mitral valve orifice area 0.9 cm 2 ) with peak and mean gradient of 22 and 12 mm Hg, respectively, at heart rate of 76/min, large ostium secundum ASD with bidirectional flow [Figure 2], moderate tricuspid stenosis with mild regurgitation, and normal Doppler derived pulmonary arterial pressure. The septal tricuspid leaflet was displaced apically by 21 mm with thickening of other leaflets and subvalvular chordal apparatus. Cardiac catheterization confirmed echocardiography findings.
Before surgery, patient was subjected to electrophysiological study, which revealed multiple ACP in right posterior free wall and parahisian area. Radiofrequency ablation (RFA) was not attempted due to lack of ideal signals and difficulty in locating multiple accessory pathways involving parahisian area.
She was premedicated with oral diazepam 5 mg night before and 1hour prior to surgery along with intramuscular injection of morphine sulphate 5 mg. In the operating room (OR) after establishing a peripheral venous line, left radial artery and right internal jugular vein were cannulated with 20G arterial cannula and 7.5 F central venous catheter, respectively, under local anesthesia. Defibrillator and antiarrythmic drugs (Amiodarone, Adenosine) were kept handy prior to induction of general anesthesia.
Anesthesia was induced with intravenous administration of fentanyl 200 μg, midazolam 2 mg, and propofol 30 mg. Administration of Vecuronium 8 mg intravenously facilitated tracheal intubation. Monitoring included ECG, pulse oximetry, end tidal carbon dioxide, nasopharyngeal and rectal temperature, urine output and arterial blood gas. Anesthesia was maintained with fentanyl, midazolam, isoflurane, and vecuronium as needed.
Prior to institution of cardiopulmonary bypass (CPB), the patient developed multiple episodes of supraventricular tachycardia (SVT), which responded to intravenous bolus administration of adenosine 6mg and fluid administration. Total body heparinization was achieved with 300 units/kg of heparin. The CPB technique included use of membrane oxygenator (Capiox SX 25, Terumo corporation, Tokyo, Japan), centrifugal pump (Biopump BPX-80; Medtronic, Inc, Minneapolis, MN) and moderate hypothermia (28-30°C). CPB flow rate were maintained between 2.2-2.4 1/min/m 2 with mean blood pressure 50-80 mmHg and a- stat blood gas management were used. Magnesium 4 gm and dexamethasone 4 mg was added to pump. Myocardial protection was achieved by using antegrade tepid blood cardioplegia. Total CPB time and aortic cross clamp time was 104 and 82 minutes, respectively. The surgery included MVR with Chitra prosthetic valve # 25 (TTK-Chitra, TTK Pharma, Chennai, India) through ASD approach and Danielson repair for correction of EA. Separation from CPB was accomplished using adrenaline 0.02- 0.05 μg/kg/min with heart in sinus rhythm. Post CPB, RV pressure as measured on table with needle placement in RV was 41/9 (19) mm Hg against systemic pressure of 86/46 (62) mm Hg. In the immediate postoperative period, the patient developed multiple episodes of SVT and hypotension. Amiodarone 200 mg was administered intravenously over 30 minutes, followed by 750 mg over 24 hours as an intravenous infusion. Subsequently, patient maintained stable hemodynamic without further episodes of arrhythmias. Patient was electively ventilated over night and weaned off from ventilator next day. The inotropes was gradually tapered off in next 24 hours. She was discharged from hospital on fifth postoperative day with prescription of oral amiodarone. Patient was asymptomatic at 3 months of follow-up, but with no change in pre-excitation on surface ECG.
EA is progressively debilitating congenital heart defect with only 5% of affected living into sixth decade.  The anomaly includes apical displacement of septal and (in many) posterior leaflets of tricuspid valve (TV) with varying degree of leaflet dysplasia. The large anterior leaflet is usually normally attached.  It may be adherent to the RV, causing RV outflow obstruction. The displacement of TV divides RV into proximal atrialized portion and distal small function RV. Atrialized RV is thin walled due to partial congenital absence of myocardium and is functionally integrated with RA. Distal small RV and left ventricle (LV) may also have function impairment due to decrease in myocytes and increase in fibrous content. The ACPs are present in 25% of patients, as downward displacement of septal leaflet of TV may cause discontinuity between central fibrous body and septal atrioventricular ring, thus creating a substrate for pre-excitation. 
The hemodynamic consequences in EA are determined by functional status of TV, size of ASD, impairment of RV and/or LV function, and presence of WPW syndrome. The hemodynamics may be further modified by presence of coexisting MS. In EA; tricuspid regurgitation is frequent and of greater severity than tricuspid stenosis. Our patient had predominant tricuspid stenosis, probably due to rheumatic involvement of TV. However, such pathology may occur rarely with isolated EA.  TR imposes volume overload of both RA and RV leading to dilatation of RA and functional impairment of small RV. The functional impairment of RV is also determined by ratio of the combined area of RA and atrialized RV relative to area of functional RV, as the atrialized RV either behaves passively during the cardiac cycle or, as an aneurysm that expands paradoxically during systole. , The presence of an ASD would be helpful in patients with EA, as it would vent the blood to the left side of heart. Thus maintaining the cardiac output but at the expense of increase in cyanosis. With coexisting MS; ASD may not help in maintaining cardiac output. Pulmonary artery pressure is often normal in EA. 
Coexisting critical MS can modify the natural history of EA by increasing left atrial (LA) pressure that may lead either to bidirectional shunt or, left to right shunt across ASD aggravating RA volume over load. Combination of Ebstein's with MS results in early onset of complications such as development of atrial fibrillation and heart failure. Elevated LA pressure is also transmitted to pulmonary venous system, which lead to early onset of pulmonary arterial hypertension (PAH). PAH causes worsening of TR and early RV failure. Depending on size of ASD (if small) pulmonary edema may develop. 
Tachyarrhythmic sudden death is a threat regardless of severity of EA.  SVT are most common arrhythmia. Although ventricular arrhythmias are uncommon, stimulation of arrhythmogenic atrialized RV may initiate polymorphic ventricular tachycardia or, fibrillation. Therefore, one should avoid stimulation of heart during insertion of central venous catheter. In combination of Ebstein's with MS, atrial flutter or fibrillation with accelerated conduction via ACP may induce fast ventricular rate leading to precipitous increase in pulmonary venous pressure.  Therefore, tachyarrhythmia should be treated aggressively and ventricular rate should be controlled between 70 to 90 per minute.
Preoperative electrophysiological evaluation is often warranted to locate ACP and possible RFA to avoid recurrent arrhythmia and instability in the perioperative period. RFA prior to surgery is therefore recommended.  However, in our case ACP could not be ablated because of poor signal.
Surgical division of ACP may be considered as an option for selected patients.  This was not done in our patient because of parahisian location of ACP and fact that surgical repair of EA itself, may reduce the incidence of arrhythmias postoperatively.  We preferred to do Danielson repair of TV and MVR. Danielson repair consists of reconstruction of enlarged anterior leaflet into a competent monocusp valve, transverse plication of atrialized RV, excision of redundant atrial free wall and closer of the associated ASD. 
A defibrillator should be readily available prior to induction of anesthesia to terminate possible arrhythmias associated with hemodynamic instability. Intravenous procainamide or, ibutilide, may be useful to restore sinus rhythm in clinically stable pre-excited tachycardia. Intravenous adenosine may be used for narrow QRS complex tachycardia. Use of amiodarone is class IIa recommendation for long-term treatment of ACP mediated arrhythmia.  Magnesium has been used for treatment of paroxysmal atrioventricular tachycardia in WPW syndrome. It causes prolongation of PR interval without any significant effect on ante and retrograde refractory period of ACP. However in up to 40% of cases an anterograde block in the ACP may be observed after intravenous Mg 2+ injection.  Therefore, we used Mg 2+ prophylactically in CPB. Specifically AV nodal blocking agents such as Digoxin and nondihydropyridine Ca channel blockers are contraindicated, as it may encourage preferential conduction over the accessory pathways.
Transesophageal echocardiography (TEE) is an important monitoring modality in these patients. It provides real time information of RV and LV function, degree of TR or stenosis and atrial shunt. Before terminating CPB, it helps in proper deairing of heart. In immediate post bypass period it allows assessment of repaired tricuspid leaflet function, adequacy of closer of ASD, paravalvular leak and adequacy of function of prosthetic mitral valve. TEE also helps in preload optimization and assessment of postoperative RV and LV function thus guiding use of inotropes. However, we did not use TEE in this case because of its unavailability in OR.
Patients may benefit with light premedication to avoid anxiety induced tachycardia; over sedation at the same time should be avoided to prevent increase in PVR. Anesthetic agent used should not cause significant chronotropic effect or, decrease in refractory period of ACP in WPW syndrome. We used isoflurane for maintenance of anesthesia because it increases the refractory period of accessory and atrioventricular pathways thus may reduce incidence of tachyarrythmia in WPW syndrome. 
In EA, enlarged RA together with TR causes pooling of blood in the RA which may delay onset of effect of intravenously administered drugs. Furthermore, this delay can lead to administration of much higher dose of medication.  The pooled blood also act as depot, releasing administered medication subsequently, that may have profound hemodynamic effects,  requiring care in dosing and patience during induction of anesthesia. However same may not be seen in cases without massive enlargement of RA and associated large ASD, which may shunt intravenous anesthetic agent to systemic circulation and enhance the onset of action of the intravenous anesthetic agents. If hypotension occurs following induction of anesthesia the initial treatment should consist of intravascular volume and phenylephrine rather than a chronotropic agent. Care must also be taken to prevent the intravenous injection of air or debris, which could produce a paradoxical embolus as patent foramen ovale or ASD is almost invariably present with EA.
In patient of EA with coexisting MS, increase in afterload is poorly tolerated due to limited preload reserve together with depressed LV systolic function. So any increase in afterload should be avoided.
The anesthetic plan must also focus on maintenance of RV function and avoidance of increase in PVR. Reversible causes of increased PVR, such as acidemia, hypoxemia, and hypercarbia must be avoided. Agents that lower PVR, such as nitrates, and nitric oxide may be beneficial in patients with severe pulmonary hypertension. Because of small and impaired RV function in EA inotropes may be required both before and after CPB. When choosing an inotrope, agents without significant alpha-agonistic effect, such as phosphodiesterase inhibitors (milrinone) are probably the first choice because of their beneficial effects on PVR. Using a pure beta-agonistic inotropic agent, such as dobutamine, though will have beneficial effect on PVR, but the potential for induction of cardiac arrhythmia; caution should be exercised in its use. We used mild dose of adrenaline to have almost pure beta-agonist action without causing significant increase in heart rate, for weaning from CPB. 
Patients with EA and WPW syndrome should undergo preoperative RFA of ACP as first line therapy. When not possible, utmost care is required to prevent and/ or control arrhythmia perioperatively. With coexistent MS, which occurred in our patient, besides maintenance of sinus rhythm and control of ventricular rate, prevention of increase in PVR and support to preserve RV function are also required.
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