CASE REPORTS

Mechanical Circulatory Support in Fulminant Myocarditis: Case Report with Brief Review and Use of Novel Anterograde Limb Perfusi

Keshav R. Nayak, MD and *Brian E. Jaski, MD
Keshav R. Nayak, MD and *Brian E. Jaski, MD
Fulminant myocarditis (FM) is an uncommon but potentially fatal condition characterized by widespread myocardial inflammation leading to severe decompensated heart failure.1 Rapidly progressive biventricular dysfunction or malignant arrhythmias can precipitate sudden circulatory collapse and death.2 In some cases, the severity of myocardial stunning and associated hemodynamic compromise render conventional therapies, including intra-aortic balloon counterpulsation (IABP), ineffective. Mechanical circulatory support (MCS) including percutaneous cardiopulmonary support (PCPS) or ventricular assist device (VAD) implantation can serve as a bridge to recovery. If patients survive the acute phase of the illness, long-term prognosis is excellent.3 If aggressive bridging therapy is not instituted due to delayed recognition, patients can succumb to intractable cardiogenic shock and complications of multisystem organ failure. We describe a case of FM secondary to Echovirus 30 that was treated with IABP and PCPS, leading to excellent recovery of ventricular function and a favorable outcome. Additionally, the case exemplifies the use of a novel anterograde limb perfusion device which prevents the inherent risk of leg ischemia associated with long-term PCPS. Case Report A previously healthy 26-year-old male presented with a 2-day history of pleuritic chest pain, dyspnea, fevers and nonproductive cough. He denied any recent illness contacts, or pertinent travel history. There was no nausea, vomiting, abdominal pain or diarrhea. On physical examination, he appeared diaphoretic and in mild distress. He was afebrile, his blood pressure was 90/60 mmHg and his pulse was 130 beats per minute and regular. Head and neck examination was normal without evidence of jugular venous distension. The precordium was tachycardic with no murmurs or rubs. Lung fields were clear and the abdomen was soft. Initial laboratory tests revealed markedly elevated myocardial and liver enzymes. An electrocardiogram (ECG) showed sinus tachycardia at the rate of 137 beats per minute with diffuse ST elevation. CXR showed cardiomegaly and enlarged hila. The echocardiogram showed severe global hypokinesis without chamber dilation. The left ventricular ejection fraction (LVEF) was markedly decreased at 12%. The patient’s condition worsened over the first 12 hours, requiring IABP insertion. A right heart catheterization revealed a cardiac output of 1.6 L/minute. With pharmacologic inotropic support, cardiac output increased to 3.2 L/minute, and the patient’s condition stabilized. However, on the second hospital day, he became febrile, hypotensive and decompensated into cardiogenic shock, renal failure and respiratory failure, requiring mechanical ventilation. The patient underwent right ventricular (RV) biopsy which was remarkable for widespread myocytolysis without the presence of viral inclusion bodies, eosinophils or giant cells. (Figure 1). Coronary angiography showed normal coronary arteries. Due to persistent hypotension, the patient was placed on PCPS. Recognizing the inherent risk of leg ischemia associated with long-term PCPS, a percutaneous anterograde limb perfusion device was placed4 (Figure 2). Details of this procedure have been reported previously.4 Briefly, the anterograde limb device was inserted by making a percutaneous stick just below the CPS arterial cannula insertion site. A 6 Fr sheath was placed and was connected to the stopcock of the CPS cannula via the sidearm of the anterograde sheath. Proper functioning of the limb rescue device was confirmed with serial Doppler flow recordings. The patient’s clinical course improved over the next 48 hours. No immunosuppressive therapy was administered. Intravenous pressor support was discontinued on the fifth hospital day, as slight improvement in LVEF to 17% was noted. Low-dose intravenous ß-blockade was initiated to facilitate a decrease in myocardial oxygen consumption. On the sixth day, the lateral and posterior walls showed improved contractility corresponding to an LVEF of 29%. PCPS was discontinued 6 days after initiation, as the LVEF increased to 40%. The CPS arterial cannula and the limb perfusion device were removed in the operating room. On the ninth day, the IABP was removed followed by weaning of mechanical ventilation. The patient was discharged home on the fourteenth day. Echocardiographic LVEF recorded at discharge by the modified Simpson’s method was 60% (Figure 3). Subsequent convalescent viral titres were consistent with an etiologic agent of Echovirus 30. Discussion Fulminant myocarditis is an uncommon but life-threatening form of acute myocarditis. The fulminant variant of myocarditis can be differentiated from acute myocarditis (nonfulminant) based on the clinicopathological criteria delineated by Lieberman et al.5 The patient we present fulfilled all of the proposed criteria, including severe hemodynamic collapse refractory to high-dose inotropic support and histopathologic evidence of active myocarditis per the Dallas Criteria.6 Furthermore, the patient’s distinct onset of a decompensated congestive heart failure syndrome after a nonspecific, flu-like illness typified the clinical presentation of FM. Although there exists a wide spectrum of clinical manifestations, most patients with FM present with fatigue, fever, dyspnea, palpitations and chest pain.1 The period from onset of symptoms to presentation generally varies from between 5 days to 2 weeks. Patients are commonly triaged as acute ST-elevation myocardial infarctions due to the triad of chest pain, ECG changes and elevation of myocardial markers. The absence of coronary artery disease in the catheterization laboratory often leads to the presumptive diagnosis of myocarditis. Pertinent physical findings include tachycardia out of proportion to fever and hypotension. Signs of congestive heart failure such as shortness of breath, pulmonary edema and increased jugular venous distension may be present unless obscured by dehydration. Laboratory analysis reveal marked elevations in CRP, creatine kinase and cardiac troponins. In fact, elevated CRP and CK levels have been identified as predictive variables for a fulminant course of myocarditis.7 Inflammatory markers such interleukin-10 can also predict a fulminant course that may require mechanical circulatory support.8 In FM, cytokine-mediated left ventricular dysfunction is often much greater than expected by a low degree of myocardial marker elevation, and is consistent with the potential for recovery. There are no characteristic ECG findings specific for FM. Nonspecific T-wave abnormalities with sinus tachycardia are common, and intraventricular conduction delays were found to be an independent variable in predicting a fulminant course.7 Malignant arrhythmias including ventricular tachycardia and complete AV block can precipitate cardiogenic shock. Although endomyocardial biopsy may be subject to sampling error, the diagnosis of myocarditis can be confirmed by RV biopsy. Biopsies in our patient revealed widespread myocytolysis characteristic of active myocarditis. Finally, echocardiography can show severe LV dysfunction without LV dilation. Compared to acute myocarditis, FM usually presents with a relative preservation of echocardiographic chamber size despite regional as well as global wall motion abnormalities.9 A hallmark of FM is the potential for complete resolution of cardiac dysfunction. McCarthy et al reported the long-term outcome of FM patients measured as survival free from death or transplantation at 12 years to be 93%.3 However, a higher survival rate may have reflected referral bias of potential survivors; only a minority of the FM patients studied required MCS. Other reports studying the prognosis of FM patients requiring MCS suggest higher mortality.9 The National Survey of Japan reported a mortality rate of 58% after an index hospitalization.10 Despite this alarming rate, long-term outcomes of the surviving patients that received MCS matched the findings of McCarthy et al. Therefore, aggressive therapy with MCS can significantly decrease the high mortality associated with FM. Early therapy with MCS can benefit patient survival and ultimately lead to excellent long-term recovery of cardiac function.11 The greatest morbidity in those receiving PCPS in the National Survey was limb ischemia due to cannula insertion.10 In our patient, the use of a novel device for anterograde perfusion allowed for the uninterrupted use of PCPS until resolution of cardiac dysfunction.4 Ischemic complications of CPS or other MCS, such as IABP, can be avoided by implementing this simple but effective means of maintaining limb tissue perfusion. The use of anterograde limb perfusion in patients with cardiogenic shock requiring MCS has been reported by Reedy et al, however, the device used required a surgical cutdown for initial placement.12 In our patient, the device used can be placed percutaneously in the cardiac catheterization laboratory by the interventional cardiologist. Removal of the CPS cannula and limb perfusion device needs to be performed via cutdown due to the potential for arterial thrombosis in the arterial segment between the arterial cannula site and limb perfusion sheath. Conclusion FM is a rare and life-threatening condition. Early recognition of a fulminant course can lead to the institution of a life-saving bridge to recovery therapy such as MCS. If patients survive the acute phase of the illness, prognosis for cardiac function recovery is excellent. Anterograde limb perfusion devices can be employed in conditions that require long-term PCPS or IABP to ensure adequate limb perfusion and prevention of limb ischemia. The device described here is easy to implement and can be placed percutaneously in the cardiac catheterization laboratory.
References
1. Feldman AM, McNamara D. Myocarditis. N Engl J Med 2000;343;1388–1398. 2. Leprince P, Combes A, Bonnet N, et al. Circulatory support of fulminant myocarditis: Consideration for implantation, weaning and explantation. Eur J of Cardiothor Surg 2003;24:399–403. 3. McCarthy RE 3rd, Boehmer JP, Hruban RH, et al. Long-term outcome of fulminant myocarditis as compared with acute (nonfulminant) myocarditis. N Engl J Med 2000;342:690–695. 4. Jaski BE, McClendon PS, Branch KR, et al. Anterograde perfusion in acute limb ischemia secondary to vascular occlusive cardiopulmonary support. Cathet Cardiovasc Diagn 1995;35:373–376. 5. Lieberman EB, Hutchins GM, Herskowitz A, et al. Clinicopathologic description of myocarditis. J Am Coll Cardiol 1991;18:1617–1626. 6. Aretz HT, Billingham ME, Edwards WD, et al. Myocarditis: A histopathologic definition and classification. Am J Cardiovasc Pathol 1987;1:3–14. 7. Kato S, Morimoto S, Hiramitsu S, et al. Risk factors for patients developing a fulminant course with acute myocarditis. Circ J 2004;68:734–739. 8. Nishii M, Inomata T, Takehana H, et al. Serum levels of IL-10 on admission as a prognostic predictor of human fulminant myocarditis. J Am Coll Cardiol 2004;44:1292–1297. 9. Maejima Y, Yasu T, Kubo N, et al. Long-term prognosis of fulminant myocarditis rescued by PCS device. Circ J 2004;68:829–833. 10. Aoyama N, Izumi T, Hiramori K, et al. National survey of fulminant myocarditis in Japan. Circ J 2002;66:133–144. 11. Grinda JM, Chevalier P, D’Attellis N, et al. Fulminant myocarditis in adults and children: Bi-ventricular assist device for recovery. Eur J Cardiothorac Surg 2004;26:1169–1173. 12. Reedy JE, Swartz MT, Raithel SC, et al. Mechanical cardiopulmonary support for refractory cardiogenic shock. Heart Lung 1990;19:514–525.