A 62‐year‐old woman with no medical history presented with 1 month of recurrent palpitations and near‐syncope. Two weeks previously she had a normal EKG and exercise stress echocardiogram. The patient's symptoms continued to worsen, and on re‐presentation, she was found to have multiple episodes of wide‐complex tachycardia at 150 beats/minute. EKG (Figure) revealed epsilon waves (low‐amplitude signals between the end of the QRS complex and the onset of the T wave (see Figure arrows) and inverted T waves in the right precordial leads. A list of diagnostic criteria for arrhythmogenic right ventricular cardiomyopathy (ARVC) was reviewed, indicating a positive diagnosis can be made with just 2 major criteria. Our patient satisfied 3 major criteria for ARVC by meeting all 3 major EKG criteria (presence of an epsilon wave, inverted T waves in the right precordial leads, and ventricular tachycardia [including nonsustained]) with superior QRS axis and left bundle branch morphology. The diagnosis was further confirmed with cardiac MRI (CMR), which revealed right ventricular (RV) free‐wall microaneursyms. The following day the patient was taken for ICD placement. Six months later she has experienced no recurrence of symptoms.
ARVC may be manifested by recurrent ventricular arrhythmias, RV failure, and sudden cardiac death (SCD). Prevalence is estimated 1 in between 2000 and 5000 individuals. Three times more common in women, ARVC has an average age of onset of 29 and is inherited in both autosomal dominant and recessive patterns. SCD is the third‐most‐common presenting symptom (behind syncope and palpitations) and is the initial symptom in 23% of patients. Recently established diagnostic criteria allow for positive diagnosis with the presence of just 2 major criteria, many of which are noninvasive to determine. These include EKG findings (see case), family history (first‐degree relative or genetic mutation), and RV wall dyskinesia/aneurysm on echocardiogram. Traditional diagnostic studies — RV biopsy and CMR — are expensive and may delay critical therapy. In addition, efficacy of RV biopsy is limited by the patchy distribution of fibrosis, and CMR is limited by considerable interobserver variability. ICD implantation is first‐line therapy and should be used in patients with documented ventricular arrhythmia, extensive disease, or SCD family history. Radiofrequency ablation (reserved for patients with frequent ICD shocks) is limited by the patchy and progressive nature of the disease. Related family members should abstain from high‐intensity exercise until adequate genetic screening can be performed.
An increased awareness of noninvasive ARVC diagnostic criteria has considerable lifesaving potential.
Electrocardiogram (EKG) revealing presence of epsilon waves (arrows) and T‐wave inversions in the right precordial leads, both major criteria for diagnosis of arrhythmogenic right ventricular cardiomyopathy.
To cite this abstract:Peters M, Katz M, Miller C. Making a List, Checking It Twice, an Early Diagnosis of Arvc Is Awfully Nice. Abstract published at Hospital Medicine 2013, May 16-19, National Harbor, Md. Abstract 256. Journal of Hospital Medicine. 2013; 8 (suppl 2). https://www.shmabstracts.com/abstract/making-a-list-checking-it-twice-an-early-diagnosis-of-arvc-is-awfully-nice/. Accessed April 10, 2020.