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Occult Cardiomyopathy in Doberman Pinschers Rebecca E. Gompf, DVM, MS, DACVIM (Cardiology) BASIC INFORMATION Description Occult cardiomyopathy (OC) is a slowly progressive heart muscle disease that results in abnormal heart rhythms (arrhythmias) in Doberman pinschers. Because it causes arrhythmias, often ventricular premature contractions (VPCs) and ventricular tachycardia (VT), it is also called arrhythmogenic cardiomyopathy. Dobermans with OC may have no clinical signs for long periods, which is why the disease is referred to as occult. Eventually the dog develops arrhythmias and, later, dilated cardiomyopathy. Causes OC runs in families of Dobermans, so a genetic factor is involved. It can be traced back to the original seven sires of Doberman lines in the United States, three of which died suddenly when they were middle-aged. The exact gene or genes causing OC have not yet been identified, but it may be an autosomal dominant characteristic. Clinical Signs OC can start as early as 9-12 months of age, but most dogs are between 2-4 years old. By 6 years of age, about 50% of Dobermans have OC. Most dogs with OC have no symptoms. Their arrhythmias may be detected on a routine physical examination or on a screening electrocardiogram (ECG) or Holter monitor study. Dogs with more frequent VPCs or VT may have fainting episodes (syncope). About 30% of affected dogs die suddenly, without prior symptoms. Older dogs may have exercise intolerance. Diagnostic Tests Since OC is very prevalent in Dobermans, annual screening of adult dogs often starts at 2-3 years of age, and may include a 24-hour Holter monitor and echocardiogram (heart ultrasound) study. The likelihood of finding VPCs, VT, or echocardiographic changes increases as the dog ages. Once arrhythmias have been found, the only way to diagnose OC is to rule out all other causes of ventricular arrhythmias. Routine laboratory tests, chest and abdominal x-rays, thyroid hormone tests, an echocardiogram, and possibly other tests may be recommended. TREATMENT AND FOLLOW-UP Treatment Options If VT, severe VPCs, or fainting episodes are occurring, it may be necessary to hospitalize your dog for treatment with intravenous drugs. The more severe the arrhythmia, the greater the chance of sudden death. Once your dog’s heart rhythm has been stabilized, it can be managed on an outpatient basis with the use of several different drugs, alone or in combination. Not every drug works in every dog, so sometimes a dog must be switched from one drug to another to control the ventricular arrhythmias. • Oral drugs that are used for controlling ventricular arrhythmias caused by OC include the combination of mexiletine and atenolol, mexiletine and carvedilol, sotalol, or mexiletine alone. • If these medications do not control the arrhythmia, then a combination of mexiletine and sotalol may be tried. • Amiodarone is used as a last resort and often requires referral to a veterinary specialist for its administration. Side effects of antiarrhythmic drugs include lethargy, anorexia, vomiting, and diarrhea. The last three effects are more common with mexiletine. Occasionally, antiarrhythmic drugs can make the arrhythmia worse, so that the dog must be switched to another medication. Amiodarone has multiple side effects, including liver damage, decreased white blood cell counts, and damage to the thyroid gland. Follow-up Care Affected Dobermans require frequent ECGs until their VT or VPCs are controlled. Ideally, a Holter monitor is applied to dogs with non–life-threatening arrhythmias to document the frequency of the arrhythmias prior to treatment. Another Holter recording is done after the drug has reached adequate blood levels, to judge how effective it is in controlling the abnormal beats. Affected dogs require antiarrhythmic therapy for the rest of their lives. Echocardiograms may be done every 6-12 months to monitor for evidence of dilated cardiomyopathy, which can follow OC. Prognosis Dobermans with infrequent VPCs that are feeling well usually develop changes on their echocardiograms within 1 year. Within 2 years, their VPCs and heart changes often get worse, and 30-50% of these dogs may die suddenly. Dobermans with severe VPCs or VT that are well controlled with medication can live up to 1 year or longer. No drug is 100% effective in preventing sudden death. The better the arrhythmia is controlled, the less likely it is that sudden death will occur. Holter monitors provide the best way to evaluate how well the medications are controlling the arrhythmias. If dilated cardiomyopathy develops, the dog’s life span is only weeks to months (average, 3-4 months). Because OC has a genetic basis and runs in families, it is wise not to breed dogs that have the problem. IF SPECIAL INSTRUCTIONS HAVE BEEN ADDED, THEY WILL APPEAR ON THE LAST PAGE OF THE PRINTOUT. Copyright © 2011 by Saunders, an imprint of Elsevier Inc. All rights reserved.