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Anesth Analg 2003;96:1598-1602
© 2003 International Anesthesia Research Society

CARDIOVASCULAR ANESTHESIA

Right Ventricular Exclusion Surgery for Arrhythmogenic Right Ventricular Dysplasia with Cardiomyopathy

Pablo Motta, MD, Emad Mossad, MD, and Robert Savage, MD

Department of Cardiothoracic Anesthesia, Cleveland Clinic Foundation, Cleveland, Ohio

Address correspondence and reprint requests to Pablo Motta, MD, Department of Cardiothoracic Anesthesia, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195. Address e-mail to mottap{at}ccf.org


   Abstract
Top
 Abstract
Introduction
 Case Report
 Discussion
 References
 

IMPLICATIONS: The authors describe the management of a patient with arrhythmogenic right ventricular dysplasia treated with right ventricular exclusion surgery.


   Introduction
Top
 Abstract
 Introduction
Case Report
 Discussion
 References
 
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD) is an inherited condition characterized by myocardial tissue replacement with fat and fibrous tissue, leading to myocardial dysfunction and ventricular arrhythmia (1–8). The right ventricle and, more rarely, the left ventricle (LV) are involved. ARVD has been involved in unexpected cardiac death in the perioperative period (1–8). Surgery is the last resort when medical therapy fails (9–12). Recently, right ventricular (RV) exclusion surgery (RVE) (Fontan-type repair) has been used in the management of ARVD (13). We report a case of RVE surgery in the management of severe ARVD, focusing on the intraoperative echocardiographic findings and the perioperative anesthetic management.


   Case Report
Top
 Abstract
 Introduction
 Case Report
Discussion
 References
 
A 21-yr-old, 76-kg, 185-cm man with ARVD, refractory right heart failure, and ventricular tachycardia was scheduled for RVE. The patient had a 2-yr history of progressive right-sided heart failure (New York Association Class III), managed with low-sodium diet, furosemide, captopril, and anticoagulation. He also presented with sotalol-refractory ventricular tachycardia, requiring amiodarone, and implantable cardioverter-defibrillator (AICD) placement. Preoperative evaluation included electrocardiogram, chest radiograph, cardiovascular computed tomography, echocardiography, and cardiac catheterization. Findings included severe right atrial (RA) and RV dilation and thrombus on the atrial surface. The LV was normal in size and function. The mitral valve and tricuspid valve (TV) were normal with 2+ tricuspid regurgitation. Cardiac catheterization demonstrated a mean pulmonary artery (PA) pressure of 17 mm Hg and a pulmonary capillary wedge pressure of 12 mm Hg, with a pressure gradient of 5 mm Hg (which made the patient suitable for RVE surgery).

Under sedation with midazolam, local anesthesia with 1% lidocaine, and standard ASA monitors, a large-bore IV catheter, radial arterial catheter, and 7F triple-lumen central venous catheter were inserted. General anesthesia was induced with etomidate (0.25 mg/kg), fentanyl (7 µg/kg), and pancuronium (0.1 mg/kg). The anesthetic was maintained with a balanced technique, which included cumulative doses of fentanyl (40 µg/kg total dose), midazolam (0.2 mg/kg), pancuronium (0.2 mg/kg), and isoflurane (range, 0.7–1.5%) in an air/oxygen mixture. Intraoperative transesophageal echocardiogram (TEE) was obtained, confirming preoperative findings of enlarged RV and RA, a RA thrombus, 2+ tricuspid regurgitation, patent foramen ovale with left to right shunt, and a 1+ mitral regurgitation (MR) (Fig. 1).



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  		Figure 1. Preoperative transesophageal echocardiogram (TEE). A, Midesophageal right ventricular inflow-outflow view: right atrial (RA) and right ventricular (RV) dilation. B, Midesophageal bicaval view: RA thrombus and patent foramen ovale (PFO). LA = left atrium.

 
The patient was cannulated with a 24F arterial cannula in the ascending aorta, an 18F right angle venous cannula in the innominate vein, and an 18F percutaneous right venous femoral cannula. During normothermic cardiopulmonary bypass (CPB), the patient underwent extracardiac RVE surgery with a Fontan-type repair that included right ventriculectomy, TV closure, and atrial septal defect enlargement. A superior vena cava to right PA anastomosis and an inferior vena cava to right PA anastomosis with 24-mm nonreinforced Gore-Tex graft was used (Fig. 2). The entire RV was excised from the RV outflow tract, 1 cm below the pulmonic valve over to the septum, 1 cm away from the left anterior descending artery, and medially to the TV annulus, leaving 1 cm with the body of the right coronary artery. Finally, the entire RV was excised all the way down to the posterior septum.



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  		Figure 2. Diagrammatic illustration of the right ventricular exclusion surgery. SVC = superior vena cava; PA = pulmonary artery; IVC = inferior vena cava; RV = right ventricle; TV = tricuspid valve; LV = left ventricle.

 
The operative course was remarkable for 4 h of CPB and 8 min of aortic cross-clamp time. Separating from the CPB, the patient developed ventricular fibrillation requiring cardioversion, amiodarone, and ventricular pacing. The hemodynamic profile was compatible with vasodilatory shock, low systemic blood pressure (mean arterial blood pressure (MAP) <50 mm Hg), normal contractility assessed by TEE, and normal filling pressures (left atrial pressure, 17 mm Hg; central venous and mean pulmonary pressure, 19 mm Hg). The patient required norepinephrine (0.05–0.25 µg · kg-1 · min-1) and vasopressin (0.15–0.25 mU · kg-1 · h-1) to achieve adequate perfusion pressures. In addition, nitroglycerin and mild hyperventilation were used to maintain an adequate transpulmonary gradient. Postrepair TEE showed a nonrestrictive atrial septal defect, anastomosis of inferior vena cava/superior vena cava to PA without significant gradient, residual RV pouch, no pulmonary regurgitation or MR, and a good LV function (Fig. 3).



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  		Figure 3. Postoperative transesophageal echocardiogram (TEE): midesophageal four-chamber view showing excluded right ventricle, tricuspid valve (TV) patch, and large atrial septal defect. LA = left atrium; RA = right atrium; RV = right ventricle; MV = mitral valve.

 
After surgery, the patient was transferred to the intensive care unit under sedation. Mechanical ventilation was achieved with a pressure control mode to limit peak inspiratory pressure. Fentanyl was used as an infusion (0.75–2 µg · kg-1 · h-1) for sedation and pain management while on controlled ventilation and as needed for pain when breathing spontaneously. Vasopressors were weaned gradually on the fourth postoperative day, and the patient was tracheally extubated once he was hemodynamically stable.

Several complications occurred postoperatively, including atrial fibrillation managed with amiodarone and cardioversion, nonoliguric renal failure, and recurrent right pleural effusion. The patient was discharged home on the 28th postoperative day.


   Discussion
Top
 Abstract
 Introduction
 Case Report
 Discussion
References
 
ARVD was recently reviewed in the anesthesiology literature (3). Specific diagnostic criteria have been initially described by McKenna et al. (7). Diagnosis is made with two major criteria, one major plus two minor criteria, or four minor criteria (3–5,7) (Table 1). Several diagnostic tools have been used, including electrocardiography, transthoracic echocardiography (TTE), magnetic resonance imaging, endomyocardial biopsy, and RV angiography. Biopsy at the time of surgery is still considered the "gold standard" (4–6).


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  		Table 1. Diagnostic Criteria of ARVD
 
TTE has a high degree of concordance with angiographically detected diffuse RV enlargement and hypokinesia (14). The three most common sites of ARVD are the outflow tract, the apex, and the inflow portion of the RV (15). TEE is more accurate than TTE for the identification of patients with suggestive features that do not meet diagnostic criteria, with increased sensitivity (100% vs 70%) (16).

Long-term follow-up indicates that ARVD is a progressive heart disease, with ventricular arrhythmia and heart failure as the main causes of death. The effectiveness of medical therapy is limited, and sotalol is the most effective drug (17). Alternatives to failed therapy are radiofrequency catheter ablation, AICD, or surgical therapy (9–13,18,19). Surgical therapy has evolved from total disconnection of the RV free wall to RVE or heart transplantation (9–13). Total RV disconnection achieved by dissecting the RV free wall from its LV attachments, although effective, has been associated with progressive RV dilation and failure (9–12). RVE procedures are an alternative treatment for refractory cases (13). Because of the limited availability of heart transplant donors, RVE could be offered to all refractory patients with a normal and nonarrhythmogenic LV and interventricular septum. In addition to resolving the arrhythmia and RV failure, RVE surgery can improve LV function by decreasing paradoxical interventricular septal motion and improving ventricular filling by decreasing compression in the left atrium (LA) by the RA (13).

Selection criteria for an RVE surgery (Fontan type) have been previously described and reviewed (Table 2) (20). Those criteria are applicable to patients with ARVD. Reported survival rates after Fontan-type repairs are 88% at 1 year, 81%–86% at 5 years, 84% at 10 years, and 74% at 15 years (21,22). Most common causes of death are heart failure and arrhythmia. There are no large outcome studies on RVE surgery for ARVD; only recently, two cases were successfully treated with this approach (13).


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  		Table 2. Selection Criteria for a Fontan Procedure
 
Management of Fontan physiology for ARVD shares the same considerations as other forms of single ventricle but with a significant risk of arrhythmia. Cardiac arrest in ARVD under anesthesia is related to hypoxemia and respiratory and/or metabolic acidosis, which activates the adrenergic system, precipitating ventricular arrhythmia (6). Effective airway and ventilation management on the induction of anesthesia, as well as maintenance of normal homeostasis, is essential. The sudden change to a passive pulmonary blood flow system after RVE surgery, without allowing ventricular geometry normalization and/or improvement of diastolic function, makes it different and perhaps more challenging than a staged bidirectional Glenn shunt surgery (23).

The goal at separation from CPB is to maintain adequate systemic cardiac output (CO) without excessive central venous pressure. This requires good ventricular systolic and diastolic function, a competent atrioventricular valve, and normal pulmonary vascular resistance (PVR) (low transpulmonary gradient and nonobstructed cavopulmonary anastomosis). Another goal during separation from CPB is to keep a PA-LA gradient of approximately 7 to 10 mm Hg, which maintains pulmonary vascular bed perfusion (20). The most effective methods to control PVR involve high fraction of inspired oxygen and hyperventilation. Pharmacological tools to increase inotropy while decreasing PVR and systemic vascular resistance include the combination of milrinone with nitroglycerine or nitroprusside (23).

Intraoperative TEE can provide information about LV function, atrioventricular valve function, volume status, and patency of anastomosis, RV, and TV patch placement (20). TEE can be used to calculate CO by measuring the flow through a cardiac valve (most often aortic) and LV outflow tract (LVOT) or main PA. Volumetric flow calculations are based on the principle that the flow through a fixed orifice is equal to the product of the cross-sectional area of the orifice and flow velocity (24):




where SV = systolic volume, AVA = aortic valve area, TVI = time velocity integral, and HR = heart rate. In addition, evaluation of PA flow by TEE could be used as a predictor of surgical outcome after a Fontan procedure. Biphasic forward flows with peak velocities during systole and diastole were associated with better outcomes (25).

Vasodilatory shock after cardiac surgery is a well known complication, with an incidence of 8%. It is characterized by the triad of MAP <70 mm Hg, cardiac index >2.5 L · min-1 · m-2, and norepinephrine (or other vasoconstrictor) dependence. Independent predictors for vasodilatory shock are low preoperative ejection fraction and angiotensin-converting enzyme inhibitor use, both of which were present in our patient. This type of shock is associated with inappropriately small levels of arginine vasopressin plasma concentration and responds to small-dose vasopressin infusion, as it did in our case (26).

Postoperatively, the goal after RVE surgery is to achieve an early extubation. Spontaneous ventilation will increase systemic venous return and pulmonary blood flow and may improve outcome, but little evidence supports this approach (27–29). This strategy should be balanced with the risks of early extubation that include hypoxia, hypercarbia, and atelectasis, which can cause increased PVR and hemodynamic instability.

In summary, we have presented a patient with refractory ARVD who was managed with RVE surgery. Anesthetic considerations and postoperative management of patients with ARVD undergoing a Fontan type repair were reviewed. RVE should be considered as an alternative for patients with refractory ARVD before heart transplantation is considered.


   References
Top
 Abstract
 Introduction
 Case Report
 Discussion
 References
 

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Accepted for publication January 22, 2003.




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Lippincott, Williams & Wilkins Anesthesia & Analgesia® is published for the International Anesthesia Research Society® by Lippincott Williams & Wilkins with the assistance of Stanford University Libraries' HighWire Press®. Copyright 2006 by the International Anesthesia Research Society. Online ISSN: 1526-7598   Print ISSN: 0003-2999 HighWire Press