Physician Presentation Summaries from MF Family Support Meeting

During the 2015 Family Support Meeting several prominent physicians specializing or studying myocarditis gave presentations to the attendees to help them understand myocarditis and the advances in diagnostics and treatment over the years. The speakers include Dr. Leslie Cooper, Dr. Jack Price, Dr. Colan, Dr. McNamara and Dr. Bode. See some key points each speaker made in this blog post.

Dr. Cooper is Chief of Cardiology at Mayo Clinic in Jacksonville, Florida. He is the world’s leading expert in myocarditis and Co-Founder and Board Member of the Myocarditis Foundation.

  • The purpose of Dr. Cooper’s talk was to give the attendees hope on where we have been and where we are today as the Myocarditis Foundation
  • In 1837, “Myokarditis” was first termed in Germany as inflammatory heart muscle disease secondary to heart muscle cells being destroyed
  • Over the next 100 years very little was said about myocarditis, though it was associated with rheumatic fever and syphilis around World War I
  • The proportion of heart failure caused my myocarditis peaks at < 1 month of age, drops down but resurges again at puberty.
  • Sudden Death due to Myocarditis occurs in Approx. 2% in infants, Approx. 5% in childhood, Approx. 4-12% of Sudden Death in ages 35-40, and Up to 40% had no antecedent symptoms
  • If we identify myocarditis early, we can stop the advance to Chronic Heart Failure
  • Myocarditis is more frequent and more severe in men   2.5:1 male:female ratio

Dr. Jack Price is a pediatric cardiologist at Texas Children’s Hospital in Houston, TX and is a board member of the Myocarditis Foundation.

  • It is difficult to get the true incidence and prevalence of Myocarditis
  • In a study between 35 Children’s Hospitals in the US from January – December 2005, of the 427,615 children hospitalized, 216 went home with the diagnosis of Myocarditis
  • Many physicians miss the diagnosis. Of the 188 children with myocarditis that were seen at Texas Children’s Hospital last year, 48% of them were seen in a pediatrician’s office with Heart Failure and not diagnosed correctly with Myocarditis
  • 68% of them were male and the mean age was 6 and most of time caused by viruses
  • While there is no clear cut test to definitively diagnose myocarditis
  • ECMO is intended to rescue the circulation, but there are lots of complications associated with it and it should be used as a last “go to” effort, other mechanical assist devices can be used to support heart function usually as a bridge to transplant, such as with the Heartware Ventricular Assist System
  • Myocarditis is rare in kids and the prognosis is good if caught early but when it occurs it is a common cause of Heart Failure in children

Dr. Colan of Boston Children’s Hospital and Harvard Medical School spoke on diagnosis of infectious myocarditis and how to determine if myocarditis is the cause of a cardiomyopathy.

  • Therapy often depends on cause of myocarditis and prognosis is highly related to cause
  • Cardiomyopathy can be diagnosis with echocardiogram, abnormal ECG and elevated enzyme levels then cause of cardiomyopathy should be assessed for myocarditis
  • Biopsy can be used to look for inflammation and used to be considered the “Gold Standard” but is limited by high rate of missed diagnosis due to patchy distribution of inflammation. Biopsy also has many disadvantages such as risks due to invasive procedure, requires anesthesia, and variation in diagnostic criteria
  • Some alternatives for diagnostic techniques include cardiac magnetic resonance (CMR) but this technique also has limits such as not specific to inflammation, new method is transcriptome which analyzes all RNA for pattern, serum anti-heart antibodies, biomarkers
  • There is no real “Gold Standard” and more accurate method of spefic cause of myocarditis is needed to test targeted therapy

Dr. Dennis McNamara is Professor of Medicine at University of Pittsburgh Medical Center and Director of the Center for Heart Failure Research and spoke on myocardial inflammation and recovery and what we have learned over the years.

  • IMAC studies lead by McNamara looked at intervention in myocarditis, cardiomyopathy and peripartum cardiomyopathy such as left ventricular assist device
  • Females have significantly higher levels of fibrosis when assessing myocardium by histology
  • Blacks have significantly lower survival from heart failure hospitalization and lower myocardial recovery compared to whites
  • Peripartum cardiomyopathy is a major cause of maternal mortality and thought to be related to inflammatory and genetic etiologies
  • Recovery significantly lower in blacks compared to whites at all time points
  • Whether differences by race and gender reflect response to injury, severity of injury, or timing of presentation remains to be determined

Dr. Michael Bode from University of North Carolina at Chapel Hill is the 2015 Myocarditis Foundation Quinn M. Kirsch Memorial Grant Recipient. Dr. Bode presented about his research project on PAR-1 in CVB3-induced viral myocarditis.

  • His project investigates a protein involved in blood coagulation’s role in protection during myocarditis.
  • PAR-1 is a receptor that is expressed on many cells in the body including the heart.
  • Post-mortem studies suggest that myocarditis accounts for up to 20% of sudden cardiac death in young adults (<40 years)
  • PAR-1 is protective in CVB-3 induced myocarditis
  • PAR-1 on cardiomyocytes is protective in CVB3-induced myocarditis as it reduces cardiac viral loads, immune cell infiltrates and cardiac inflammation.
  • The protective mechanism of PAR-1 on cardiomyocytes is different from the one of PAR-1 on cardiac fibroblasts in that no enhancement of the innate immune response through CXCL10 is seen.
  • Stimulation of PAR-1 in CVB3-infected cardiomyocytes leads to decreased virus loads through an unknown mechanism.
  • Blood thinners that inhibit thrombin or PAR-1, such as dabigatran or vorapaxar, may lead to increased cardiac viral loads in patients with viral myocarditis.
  • More work is needed to exactly understand protective mechanisms in the heart and improve outcomes of patients with myocarditis in the future.

We had a very successful Family Support Meeting with presentations by families, support groups and as summarized above some of the top physicians studying myocarditis. We hope that each and every one of you will be able to attend next year’s Family Support Meeting to learn about the advances we will make over the next year.