This article is more than 5 years old. Information may no longer be current.
Cardiac biomarkers, low BMI may predict poor outcomes in muscular dystrophy
Click Here to Manage Email Alerts
Patients with Duchenne muscular dystrophy, especially those who were underweight, had an increased risk for cardiomyopathy, according to a study published in the Journal of the American Heart Association.
Patients with Duchenne muscular dystrophy who died had lower BMI, elevated cardiac biomarkers and worse respiratory profiles compared with those who survived, researchers reported.
“Previously, Duchenne muscular dystrophy was really considered a pediatric disease; however, due to advances in medical knowledge and care over the past 2 decades, many of these patients are now living into their 20s and 30s, some even into their 40s, but then are dying of a cardiomyopathy,” Pradeep P. A. Mammen, MD, associate professor of internal medicine and medical director of the Neuromuscular Cardiomyopathy Clinic at UT Southwestern Medical Center in Dallas, told Cardiology Today.
Daniel Cheeran, MD, and Shaida Khan, MD, research fellows at University of Texas Southwestern Medical Center, and colleagues analyzed data from 43 men (median age when entering the clinic, 21 years; median age at analysis, 24 years) with Duchenne muscular dystrophy who visited the UT Southwestern Neuromuscular Cardiomyopathy Clinic from January 2006 to June 2016. A cardiopulmonary assessment was performed, and data such as demographics, ECGs, echocardiography, hospitalizations and pulmonary function were collected.
“The implications ar that we need to identify patients earlier,” Mammen said. “What’s unique in this population is that many doctors don’t see a lot of these patients on a regular basis. Providing a way to identify key risk factors would be an important contribution to both the patients and the physicians who treat them.”
Researchers also reviewed data from an age-matched subgroup with seven non-survivors and 14 survivors to account for potential age differences between the two groups.
Patients were followed up at the clinic for a median of 28 months.
All of the patients in the main group developed cardiomyopathy. The majority of patients were on angiotensin II receptor blockers or ACE inhibitors (86%) and 56% were on oral steroid therapy. Only 37% of the patients were on beta-blockers.
Compared with survivors, patients who did not survive had lower BMI (17.3 kg/m2 vs. 28.5 kg/m2; P < .01), maximum inspiratory pressures (13 cmH2O vs. 33 cmH2O; P = .03), alanine aminotransferase levels (26 units/L vs. 53 units/L; P < .01) and elevated N-terminal pro-brain natriuretic peptide (288 pg/mL vs. 35 pg/mL; P = .03).
“Our hope is that this study will raise awareness of [Duchenne muscular dystrophy]-associated cardiomyopathy, enable my colleagues and the entire medical community to start evidence-based medicine sooner than later including ACE inhibitors, beta-blockers, spironolactone, drugs that are known to improve cardiac function and delay progression of cardiomyopathy,” Mammen said. – by Darlene Dobkowski
For more information:
Pradeep P.A. Mammen, MD, can be reached at pradeep.mammen@utsouthwestern.edu.
Disclosures: Mammen reports he consults for Catabasis, HeartWave and PhaseBio and received a research grant from PhaseBio. The other authors report no relevant financial disclosures.
Click Here to Manage Email Alerts