Published online Dec 6, 2019. doi: 10.12998/wjcc.v7.i23.4098
Peer-review started: June 26, 2019
First decision: November 12, 2019
Revised: November 17, 2019
Accepted: November 20, 2019
Article in press: November 20, 2019
Published online: December 6, 2019
Processing time: 169 Days and 23.3 Hours
Duchenne muscular dystrophy (DMD), which is caused by a mutation/deletion in the dystrophin gene on the X-chromosome, is the most common type of neuromuscular disorder in pediatrics. Skeletal muscle weakness progressively develops in DMD patients and usually leads to respiratory failure in the early adolescent years. Cardiac muscle is frequently affected in DMD patients, which leads to a high burden of cardiomyopathy and heart failure. In the era of improved respiratory care, cardiac deaths are becoming the major cause of mortality in DMD patients.
We report the case of a 15-year-old boy who presented to the hospital due to recurrent orthopnea for 6 mo and palpitations for 4 mo. He was diagnosed with progressive muscular dystrophy at the age of 3 years and was confined to a wheelchair at 12 years. He was prescribed diuretics and digoxin at the outpatient clinic; however, his symptoms did not resolve. Sacubitril/valsartan was added 1 mo prior to presentation, but he experienced recurrent episodes of palpitations. The electrocardiogram showed atrial tachycardia with a heart rate of 201 bpm, and he was then hospitalized. Hypotension was found following the administration of sacubitril/valsartan tablets; he could not tolerate even a small dose, always developing tachyarrhythmia. His symptoms were relieved after discontinuing sacubitril/valsartan, and his heart rate was controlled by a small dose of metoprolol tartrate and digoxin. Atrial tachycardia spontaneously converted in this patient, and his symptoms attenuated in the following 6 mo, without palpitation episodes.
Blood pressure should be closely monitored in DMD patients with advanced heart failure when taking sacubitril/valsartan.
Core tip: A 15-year-old boy with Duchenne muscular dystrophy presented to the hospital due to recurrent orthopnea for 6 mo. He was diagnosed with heart failure and was prescribed oral diuretics and digoxin at the outpatient clinic, but his symptoms did not resolve. Sacubitril/valsartan was added to his therapeutic regimen 1 mo before presentation, but resulted in recurrent hypotension and palpitation episodes until discontinuation of this medication. Although sacubitril/valsartan has been shown to be beneficial for heart failure with a reduced ejection fraction, hypotension is a common side effect of this medication, and blood pressure should be closely monitored in Duchenne muscular dystrophy patients with advanced heart failure.