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Ann Thorac Surg 2001;71:S73-S76
© 2001 The Society of Thoracic Surgeons

Session 1: acute heart failure

Mechanical circulatory support for patients with acute-fulminant myocarditis

Michael A. Acker, MDa

a Division of Cardiothoracic Surgery, Department of Surgery, University of Pennsylvania Medical Center, Philadelphia, Pennsylvania, USA

Address reprint requests to Dr Acker, University of Pennsylvania Medical Center, 3400 Spruce St, 6 Silverstein, Philadelphia, PA 19104
e-mail: michael.acker{at}uphs.upenn.edu

Presented at the Fifth International Conference on Circulatory Support Devices for Severe Cardiac Failure, New York, NY, Sept 15–17, 2000.

Abstract

This report provides a review of mechanical circulatory support for patients in cardiogenic shock secondary to acute/fulminant myocarditis. Experience and outcomes with extracorporeal membrane oxygenation, left ventricular assist device support (ABIOMED, Thoratec, Thermo Cardiosystems, Novacor), and biventricular ventricular assist device support (ABIOMED, Thoratec) are described. Patients in cardiogenic shock secondary to acute myocarditis in its fulminant presentation can recover, surprisingly with normal cardiac function. An aggressive approach to the use of mechanical support is strongly justified. Survival, either by bridge to transplant or recovery, should approach 70%. Transplantation can often be avoided.

Acute myocarditis is a disease of unclear pathophysiology and etiology, uncertain diagnosis, and variable presentation. The purpose of this review is to describe the natural history of acute myocarditis, the experience to date with mechanical support, and the optimal type of mechanical support indicated. Most cases remain subclinical with cardiac signs and symptoms overshadowed by systemic manifestations of fever, myalgias, muscle tenderness, and fatigue. Cardiac symptoms of congestive heart failure, arrhythmias, and chest pain generally follow a viral prodrome. Presentation with hemodynamic instability or frank cardiogenic shock is relatively rare. The clinical diagnosis is based on the development of unexplained cardiac symptoms of new onset after ischemic, valvular, endocrinopathies, collagen vascular, and autoimmune etiologies are ruled out. Although an endomyocardial biopsy often is not obtained, the definitive diagnosis depends on the presence of an inflammatory infiltrate (lymphocytic) associated with myonecrosis. The diagnosis is considered borderline if no myonecrosis is identified [1]. In the myocarditis treatment trial [2], the incidence of a positive biopsy in clinically suspected myocarditis was only 10%. Because no specific therapy is currently available, treatment remains primarily supportive. Standard immunosuppressive therapy has been ineffective in adults [2], although a recent report indicates that intravenous immunoglobulin may have a beneficial effect [3]. Conventional heart failure management (rest, afterload reduction, diuretics, etc) may need to be supplemented with inotropic support and will most often stabilize the patient and allow either recovery or slow progression to a dilated cardiomyopathy. More rarely, the patient’s course is more fulminant, leading to hemodynamic decompensation requiring mechanical ventricular support [4].

In the myocarditis treatment trial [2], 1-year survival after biopsy-proved acute myocarditis was 80% and 4-year survival was 44%. In contrast, transplant-free survival for giant cell myocarditis was only 11%, with a median survival of 5.5 months [5]. McCarthy and coworkers [6] recently reviewed the Johns Hopkins’ experience with myocarditis in 147 patients (giant cell myocarditis excluded). They divided their patients into 15 with fulminant myocarditis (severe hemodynamic compromise requiring high-dose vasopressor agents or ventricular assist device [VAD] support; rapid onset of symptoms; fever; positive biopsy results) and 132 with acute myocarditis (less ill; indistinct, prolonged onset of symptoms; less severe symptoms of heart failure; positive biopsy results). Of the 15 patients, 2 required VAD support. Surprisingly, they found a 1-year transplant-free survival of 93% and an 11-year transplant-free survival of 93% in patients with fulminant myocarditis, whereas patients with acute myocarditis had a 1-year transplant-free survival of 85% and an 11-year transplant-free survival of 45% [6] (Fig 1). McCarthy and coworkers concluded that if adequately supported, patients presenting with fulminant acute myocarditis (ie, sudden, severe congestive heart failure with circulatory collapse) will recover fully without long-term sequelae. In contrast to those with giant cell myocarditis, patients who require acute ventricular support for fulminant acute myocarditis can be expected to recover and transplantation can be avoided [6, 7].



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  		Fig 1. Unadjusted transplantation-free survival according to clinicopathologic classification. Patients with fulminant myocarditis were significantly less likely to die or require transplantation during follow-up than were patients with acute myocarditis (p = 0.05 by the log rank test). (Reprinted with permission from McCarthy RE, Boehmer JP, Hruban RH, et al. Long-term outcome of fulminant myocarditis as compared with acute [nonfulminant] myocarditis. N Engl J Med 2000;342:690–5.)

 
Several recent reports [4, 8] state that there is little accumulative experience of using mechanical support for acute myocarditis. Actually a review of individual ventricular device companies’ voluntary registries, in addition to a review of the literature, reveal more than 135 patients with myocarditis supported by mechanical devices (Table 1). Myocarditis is a rare indication for mechanical support, accounting for less than 5% of devices implanted in most databases. Most patients with acute myocarditis have been supported either by extracorporeal devices (ABIOMED, Thoratec, Pleasanton, CA) or extracorporeal membrane oxygenation (ECMO) with peripheral cannulation. Intracorporeal left ventricular assist devices (LVADs) (ThermoCardiosystems [TCI], Woburn, MA; Novacor, Edwards Life Sciences, San Diego, CA) and a smattering of other devices have been used to a lesser extent. Extracorporeal membrane oxygenation and extracorporeal devices have also supported infants and children with cardiac failure secondary to acute myocarditis [9–13]. The following summary is not complete but reflects most of what is available in the published literature and voluntary registries.


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  		Table 1. Mechanical Support for Acute Myocarditis and Cardiac Failure

 
ABIOMED Inc (Danvers, MA) reports a total of 32 patients supported by their BVS 5000. There have been 24 (75%) biventricular assist devices (BVADs), 6 (19%) LVADs, and 2 (6%) right ventricular assist devices (RVADs). Eighteen (56%) patients were weaned to recovery and 12 (38%) were discharged. Six (19%) were transplanted and 5 (16%) of these patients were discharged. Overall survival was 53% (17 of 32 patients). The mean duration of support was 9.5 days ± 9 days [14] (Table 1).

Thoratec reports a total of 40 patients supported by Thoratec VADs. Twenty-eight patients were supported by BVADs (70%) and 12 (30%) by LVADs. Sixteen patients (40%) recovered and 14 (35%) patients were discharged. Eighteen patients (45%) were transplanted and 17 (45%) were discharged (Table 1). Overall survival was 78% (31 of 40 patients) [15] (Table 1).

A review of recent literature reveals 37 patients with acute myocarditis supported by peripheral ECMO. Most of these patients were from Japan. Twenty-seven patients (73%) were successfully weaned. Overall survival to discharge was 70% (26 of 37 patients) [16–20] (Table 1).

Overall success rates for intracorporeal LVADs have not been as good. The Novacor has been implanted in 20 patients with acute myocarditis. Only 2 patients were weaned to recovery (10%). Eight patients (40%) were transplanted, but only 4 patients left the hospital. Overall survival was only 30% [21]. TCI reports a total of 23 patients with acute myocarditis who have had LVADs inserted. Of these, 6 have ongoing support. Of the 17 patients remaining, 2 recovered (12%) and 6 were transplanted. Overall survival was 47% not including the patients still with LVADs in place with ongoing support [22] (Table 1).

The choice of mechanical support for an otherwise healthy patient who develops rapid onset of hemodynamic instability of nonischemic origin progressing to cardiogenic shock should be dictated by the likelihood of a diagnosis of fulminant acute myocarditis or giant cell myocarditis and current understanding of the disease’s natural history. The recent report from Johns Hopkins Hospital suggests strongly that such patients, if the diagnosis is not giant cell myocarditis [5], have an excellent prognosis for complete long-term recovery if they are supported aggressively [6, 7]. Success rates using ABIOMED, Thoratec, or ECMO should approach 50% to 70% [14–20] (Table 1). Just as important, transplantation should be avoided, if possible, for fulminant acute myocarditis. In most cases of fulminant acute myocarditis, ventricular recovery takes place within several weeks of onset. The average duration of ABIOMED support was about 10 days [14]. A recent patient with fulminant acute myocarditis from the University of Pennsylvania Health System was in continuous ventricular fibrillation for 10 days following a Thoratec BVAD insertion. The patient’s heart converted spontaneously to normal sinus rhythm after 10 days of biventricular support followed by a gradual return of normal ventricular function. The BVADs were removed 3 weeks after insertion. Patients supported by ECMO were usually weaned in less than 1 week, although some were supported successfully up to 3 weeks [16–20]. Because every attempt should be made to allow for ventricular recovery if a diagnosis of acute myocarditis has been confirmed by biopsy, either ABIOMED or Thoratec devices should be used because of their ease of removal [23–26].

This recommendation is in sharp contrast to patients with idiopathic, dilated cardiomyopathies that require ventricular support. Although recovery has been demonstrated [27–30], it is relatively rare, and long-term freedom from deterioration leading to death or retransplantation is not assured [27, 31]. Even patients recovered after ventricular support with a presumed diagnosis of fulminant myocarditis have been reported to deteriorate quickly after VAD explantation [8]. One patient was supported for 50 days, more than is typical for fulminant myocarditis, and had extensive fibrosis on later examination of the heart after transplantation. Houel and colleagues point out the difficulty in predicting success after explantation [8]. Given the current knowledge of acute myocarditis [2, 6, 7], patients who present with a more indolent course that requires ventricular support later after presentation would be expected to have an increased chance of deterioration after initial recovery.

In general, intracorporeal VADs designed primarily for long-term bridge or destination therapy should be avoided if recovery within several weeks is expected. Such devices are more expensive, more difficult to explant, and limited to left ventricular support only. The relatively rare patient who had either a TCI or Novacor explanted after insertion for myocarditis probably partly reflects the relative difficulty in removing these devices without transplantation and the surgeon’s bias that ventricular recovery will not occur or be long lasting. Conversely, if a diagnosis of giant cell myocarditis is made before or after VAD insertion, transplantation will most likely be needed and ventricular support suited for long-term bridge to transplantation should be chosen (Thoratec or TCI).

Though results of ECMO have been excellent in selected patients, prolonged support is associated with significant morbidity, that is, peripheral vascular complications, hemolysis, and a bedridden state. In addition, ECMO does not decompress the left ventricle (LV) and is associated with increased LV wall stress [32]. Extracorporeal membrane oxygenation should be used for patients who present with circulatory collapse, cardiac arrest, or severe pulmonary failure with inability to oxygenate. In these circumstances, ECMO will allow stabilization of the patient with initial neurologic, renal, and pulmonary recovery. If the heart shows signs of recovery over a few days, a wean to recovery is possible. If there is no improvement in cardiac function, the patient should be bridged, in general, to a Thoratec BVAD/VAD (Fig 2).



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  		Fig 2. Mechanical circulating support decision tree for acute myocarditis. (BVAD = biventricular assist device; ECMO = extracorporeal membrane oxygenation; IABP = intraaortic balloon pump; LVAD = left ventricular assist device; RV = right ventricle; TCI = Thermo Cardiosystems; VAD = ventricular assist device; VT = ventricular tachycardia.)

 
Both the ABIOMED and Thoratec devices have been used successfully to support either the left ventricle alone or both ventricles to recovery. Both devices can be placed easily and their versatility (BVAD, LVAD, and RVAD) is suited to the biventricular involvement of fulminant acute myocarditis or giant cell myocarditis. More than 70% of patients supported by either Thoratec or ABIOMED devices require biventricular support. In the past, left atrial cannulation was recommended when ventricular recovery was expected. Left atrial cannulation, however, is associated with inadequate LV decompression and an increased risk of left ventricular thrombosis and stroke [33]. For this reason, we have switched to left ventricular apical cannulation, if technically feasible, for both the Thoratec and ABIOMED LVADs. Repair of the left ventricular apical cannulation site can be done at removal without significant compromise of LV function. The ABIOMED does not allow for easy ambulation and is not suited for long-term bridge to recovery or transplant. The Thoratec, because it is paracorporeal, does allow full ambulation and can be used as a long-term bridge to recovery or transplant, if needed. Because of the ability to ambulate, patients can be fully rehabilitated with the Thoratec device, which is not possible with ABIOMED support. If by 2 to 3 weeks the heart has not recovered sufficiently to allow weaning of the ABIOMED, it can be switched to either a Thoratec device or an intracorporeal VAD, if indicated.

In conclusion, a reasonable experience with mechanical support for acute myocarditis has been accumulated. Overall success rates of 50% to 70% with mechanical ventricular support are attainable either by cardiac recovery or transplantation. Full cardiac recovery is possible and even likely despite fulminant and catastrophic presentation. Paradoxically, patients who present as less ill have the worst long-term prognosis.

References

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