Background:
Cardiac complications are now the leading cause of death in patients with Duchenne muscular dystrophy (DMD). While cardiac care may be variable in these patients, recent guidelines recommend early involvement of a cardiologist as part of the patients’ multidisciplinary care team. July 2020 marks the addition of a pediatric cardiologist specialized in management of patients with DMD at the Children’s Hospital of Richmond (ChoR) at Virginia Commonwealth University.
Aim:
This project highlights the importance of involving a cardiologist who is trained in implementing guideline-directed cardiac monitoring and medical therapy early in the care of DMD patients.
Methods:
We reviewed the electronic medical records of 36 patients diagnosed with DMD seen at CHoR. We analyzed the age of initial cardiology visit, cardiac imaging, and initiation of pharmacological interventions before and after July 2020.
Results:
In patients who turned 10 years old before July 2020 (n=25), the mean age of initial cardiology visit, echocardiogram, and cardiac-MRI were 13.1, 14.4, and 17.3, respectively. The mean age for starting an angiotensin-converting-enzyme inhibitor (ACE-i)/angiotensin receptor blocker (ARB) and aldosterone antagonist were 15.9 and 17.1, respectively. In patients who turn 10 years old after July 2020 (n=11), the mean age of initial cardiology visit, echocardiogram, and cardiac-MRI were 3.9, 4.6, and 10, respectively. The mean age for starting an ACE-i/ARB and aldosterone antagonist were 9.8 and 10, respectively
Conclusion:
Since July 2020, patients with DMD seen at CHoR have been evaluated by a pediatric cardiologist at a younger age, allowing for earlier guideline-directed cardiac monitoring and initiation of cardioprotective medications. This intervention allows for delay in progression of heart scarring and dysfunction, which reduces cardiac-induced morbidity and mortality.