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Fulminant Myocarditis

The Role of Perioperative Echocardiography

Shillcutt, Sasha K. MD, FASE; Thomas, Walker R. BA, RDCS; Sullivan, James N. MD; Duhachek-Stapelman, Amy MD

doi: 10.1213/ANE.0000000000000508
Cardiovascular Anesthesiology: Echo Rounds
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From the Department of Anesthesiology, University of Nebraska Medical Center, Omaha, Nebraska.

Accepted for publication September 8, 2014.

Funding: Department of Anesthesiology, University of Nebraska Medical Center.

The authors declare no conflicts of interest.

Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal’s website.

Written consent for publication of this report was obtained.

Reprints will not be available from the authors.

Address correspondence to Sasha K. Shillcutt, MD, FASE, Department of Anesthesiology, University of Nebraska Medical Center, 4400 Emile St., UT 1467, Omaha, NE 68198. Address e-mail to sshillcu@unmc.edu.

A previously healthy 22-year-old female with a 3-day history of fevers and chills presented to a community hospital in cardiogenic shock. Transthoracic echocardiogram revealed severely depressed left ventricular (LV) function and a LV ejection fraction (EF) of 5%. Over the next 2 hours, she quickly decompensated and went into ventricular fibrillation. She underwent cardiopulmonary resuscitation and was transferred to our institution on inotropic support with a systolic blood pressure of 60 mm Hg and a heart rate of 140 bpm.

The patient was placed on venoarterial extracorporeal membrane oxygenation (ECMO). Transesophageal echocardiography (TEE) examination confirmed biventricular systolic dysfunction with an LVEF of 5%. Her LV internal diameter in diastole (LVIDd) was within normal limits at 4.8 cm and her septal wall thickness was also normal, measuring 0.9 cm in the transgastric midpapillary short-axis (TG mid-SAX) view as seen in Figure 1A, Video 1 (Supplemental Digital Content 1, http://links.lww.com/AA/B25). Three days later, she underwent percutaneous Impella LV device placement (Abiomed, Danvers, MA) which was maintained until hospital day 9. On hospital day 16, repeat TEE examination revealed a decompressed LV with doubling of the circumferential wall thickness in the TG mid-SAX view seen in Figure 1B, Video 1 (Supplemental Digital Content 1, http://links.lww.com/AA/B25). On hospital day 18, she underwent ECMO decannulation and placement of percutaneous right ventricular assist device (RVAD). TEE examination again showed severe LV wall thickening (Fig. 1C, Video 1, Supplemental Digital Content 1, http://links.lww.com/AA/B25). On hospital day 25, she was weaned from circulatory support and the percutaneous RVAD was removed. TEE examination on day 26 showed an improvement in LV thickening (Fig. 1D, Video 1, Supplemental Digital Content 1, http://links.lww.com/AA/B25), normal right ventricular function, and an LVEF of 55%.

Figure 1

Figure 1

Blood titers revealed human herpesvirus 6 infection with the presumed diagnosis of viral fulminant myocarditis. She was discharged in stable condition after 38 days. Six months later, transthoracic echocardiogram examination revealed LVEF of 55% and normal wall thickness.

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DISCUSSION

Myocarditis is inflammation of the heart muscle leading to cellular necrosis. Prevalence of the disease is unknown because symptoms vary from dyspnea to cardiogenic shock. The most common type of acute myocarditis is viral, typically from parvovirus B19 and human herpesvirus 6.1 Myocardial injury is caused by direct viral damage and the immune-mediated response. Fulminant myocarditis can be mistaken for acute myocarditis; however, there are distinct differences between the 2 entities.

Clinically, fulminant myocarditis follows a more severe course than acute myocarditis, often progressing to biventricular failure, arrhythmias, and cardiac arrest.2 Symptoms occur after a short prodrome of nonspecific signs such as fever and malaise. Recovery from fulminant myocarditis is likely if the patient survives initial cardiogenic shock.1–3 Patients with acute myocarditis tend to have less significant hemodynamic collapse when compared with those with fulminant myocarditis; however, acute myocarditis is more likely to lead to dilated cardiomyopathy and result in heart transplantation.3

Magnetic resonance imaging, speckle tracking, and endocardial biopsy may be used to make the diagnosis of either type. In the urgent perioperative period, echocardiography remains important in both diagnosis and the ongoing evaluation of response to therapy and may be more predictive of outcome than histological markers.4,5 For perioperative physicians who may perform echocardiography for these patients multiple times over the course of their hospitalization, recognizing the echocardiographic differences between fulminant versus acute myocarditis is important and will be discussed.

Echocardiography is often first used to exclude other causes of heart failure and for chamber quantification.2,4,5 Initial echocardiographic presentation of fulminant myocarditis can be difficult to distinguish from ischemic or infiltrative cardiomyopathy, both of which typically present in an older population when compared with fulminant myocarditis. Early echocardiographic findings of fulminant myocarditis may mimic ischemic cardiomyopathy because there may be segmental wall motion abnormalities.3 Right ventricular dysfunction is not as common as LV dysfunction in fulminant myocarditis but is the strongest predictor of poor outcome and need for cardiac transplantation.2,4 Common echocardiographic findings of fulminant myocarditis include the following4,5:

  • Systolic and diastolic dysfunction (restrictive pattern)
  • Pericardial effusion
  • Intracardiac thrombus (seen in 25% of patients)
  • Significant mitral regurgitation (typically central)
  • Significant ventricular wall thickening with normal LVIDd
  • Progressive ventricular wall thickening often followed by resolution

Although other types of cardiomyopathy may have similar findings, fulminant myocarditis is distinguished by significant thickening of the ventricular wall by direct myocardial injury (apoptosis and necrosis).5 LV wall thickness may be normal on initial presentation with progressive thickening that worsens over time mimicking hypertrophic cardiomyopathy.2–4 As seen in Figure 1, Video 1 (Supplemental Digital Content 1, http://links.lww.com/AA/B25), serial TEE images of our patient with fulminant myocarditis depict the initial circumferential wall thickness of the LV nearly doubling during her hospitalization. The dramatic increase in LV wall thickness documented by serial TEE examination was followed by subsequent improvement with mechanical support over the course of her hospitalization (Fig. 1, Video 1, Supplemental Digital Content 1, http://links.lww.com/AA/B25).

Ventricular edema routinely involves the septal and the inferior/inferolateral walls and can also involve papillary muscles.3 Increased wall thickness can be mild (1.1–1.3 cm), moderate (1.4–1.6 cm), or severe (≥1.7 cm). To obtain accurate measurement of wall thickness, both the septal and inferolateral walls of the LV should be measured in the TG mid-SAX view (Fig. 1C).6 In our patient, both the septal and inferolateral walls had measured moderately (1.6 cm) to severely (1.8 cm) thickened, respectively, as seen in Figure 1C. Septal wall edema can also be seen in the midesophageal (ME) 4-chamber view, the inferolateral wall in the ME long-axis view, and the inferior wall in the ME 2-chamber view. Hypertrophic cardiomyopathy can be distinguished from fulminant myocarditis by LV wall thickness; in all forms of hypertrophic cardiomyopathy, the inferolateral wall is sparred. Fulminant myocarditis can also be differentiated from acute myocarditis by LV wall thickness, which is increased in fulminant myocarditis and normal in acute myocarditis.

Along with wall thickness, fulminant myocarditis may be differentiated from acute myocarditis by LVIDd.2,4,5 Patients with fulminant type often have normal LVIDd, whereas those with acute type have dilated LVIDd that may progress to dilated cardiomyopathy. LVIDd should be measured in diastole in the TG mid-SAX view.6Figure 2A, Video 2 (Supplemental Digital Content 2, http://links.lww.com/AA/B26) demonstrates fulminant myocarditis with wall thickening but a normal LVIDd, whereas Figure 2B demonstrates a patient with acute myocarditis showing a significantly dilated LVIDd and normal wall thickness. Although LVID is key, it is important to note that it is a single dimension and may not be accurate in an abnormally shaped ventricle (Table 1).

Figure 2

Figure 2

Table 1

Table 1

Patients with fulminant myocarditis may require mechanical support, such as intra-aortic balloon pump, ECMO, or a ventricular assist device (Table 1). Besides providing a diagnosis in the acute perioperative period, echocardiography aids in evaluation of the adequacy of circulatory support, cannula placement, ventricular filling, detection of intracardiac thrombus, and recovery of ventricular function.3 Serial echocardiography can determine readiness for weaning from mechanical devices. Return to normal LV wall thickness (≤1.0 cm) and improvement of contractile function signal the ability to wean from mechanical support.3 Perioperative echocardiographic findings of LV wall thickening in the presence of a normal LVIDd in a patient presenting in cardiogenic shock should alert the clinician to the possible diagnosis of fulminant myocarditis.

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Clinician’s Key Teaching Points

By Martin M. Stechert, MD, Roman M. Sniecinski, MD, and Nikolaos J. Skubas, MD

  • Fulminant myocarditis is an inflammatory process of the myocardium that is characterized by sudden onset of severe heart failure a week or less after a viral prodrome. Diffuse lymphocytic infiltration with myocardial necrosis results in extensive myocardial edema, creating a typical pseudo-hypertrophic appearance of the myocardial walls. Fulminant myocarditis is a distinct entity from acute, nonfulminant myocarditis that is more indolent and causes less severe ventricular dysfunction, but typically results in the longer lasting sequelae of a dilated cardiomyopathy.
  • Echocardiography is ideally suited for making serial measurements of the left ventricle (LV) that can help differentiate fulminant from nonfulminant myocarditis. In fulminant myocarditis, LV internal diameter is mostly preserved, but with a marked increase of wall thickness due to edema. This “pseudo-concentric hypertrophy” is not found in nonfulminant myocarditis, which causes an increase in LV internal diameter and thinning of the myocardium (i.e., eccentric hypertrophy).
  • In this case, serial echocardiographic examinations were able to document disease progression and resolution by repeated measurements of the LV wall thickness and ventricular dimensions. From the TG mid-SAX view, thickness of the LV septal and inferolateral walls was determined in the standardized fashion at end-diastole. Wall thickness went from a normal 0.9 cm at the onset of the disease, to double that (1.8 cm) at the peak of the disease, and was regressing to 1.4 cm as the patient recovered.
  • Because overall prognosis of fulminant myocarditis is dramatically different when compared with nonfulminant forms, early echocardiographic diagnosis is key to institute appropriate, often most aggressive, supportive treatment.
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DISCLOSURES

Name: Sasha K. Shillcutt, MD, FASE.

Contribution: This author helped design the study and prepare the manuscript.

Attestation: Sasha K. Shillcutt approved the final manuscript, and is the archival author.

Name: Walker R. Thomas, BA, RDCS.

Contribution: This author helped prepare the manuscript.

Attestation: Walker R. Thomas approved the final manuscript.

Name: James N. Sullivan, MD.

Contribution: This author helped prepare the manuscript.

Attestation: James N. Sullivan approved the final manuscript.

Name: Amy Duhachek-Stapelman, MD.

Contribution: This author helped design the study and prepare the manuscript.

Attestation: Amy Duhachek-Stapelman approved the final manuscript.

This manuscript was handled by: Martin J. London, MD.

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REFERENCES

1. Kindermann I, Barth C, Mahfoud F, Ukena C, Lenski M, Yilmaz A, Klingel K, Kandolf R, Sechtem U, Cooper LT, Böhm M. Update on myocarditis. J Am Coll Cardiol. 2012;59:779–92
2. Felker GM, Boehmer JP, Hruban RH, Hutchins GM, Kasper EK, Baughman KL, Hare JM. Echocardiographic findings in fulminant and acute myocarditis. J Am Coll Cardiol. 2000;36:227–32
3. Catena E, Paino R, Milazzo F, Colombo T, Marianeschi S, Lanfranconi M, Aresta F, Bruschi G, Russo C, Vitali E. Mechanical circulatory support for patients with fulminant myocarditis: the role of echocardiography to address diagnosis, choice of device, management, and recovery. J Cardiothorac Vasc Anesth. 2009;23:87–94
4. Skouri HN, Dec GW, Friedrich MG, Cooper LT. Noninvasive imaging in myocarditis. J Am Coll Cardiol. 2006;48:2085–93
5. Blauwet LA, Cooper LT. Myocarditis. Prog Cardiovasc Dis. 2010;52:274–88
6. Lang RM, Bierig M, Devereux RB, Flachskampf FA, Foster E, Pellikka PA, Picard MH, Roman MJ, Seward J, Shanewise JS, Solomon SD, Spencer KT, Sutton MS, Stewart WJChamber Quantification Writing Group; American Society of Echocardiography’s Guidelines and Standards Committee; European Association of Echocardiography. . Recommendations for chamber quantification: a report from the American Society of Echocardiography’s Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of Echocardiography, a branch of the European Society of Cardiology. J Am Soc Echocardiogr. 2005;18:1440–63

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