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254  Section 3  Cardiovascular Disease

            blood pressure and tissue perfusion activates a number   The overt phase follows, characterized by the onset of
  VetBooks.ir  of   neuroendocrine and cytokine systems intended to   clinical signs including syncope, SD, or those typical of
                                                              CHF (ACVIM stage C). In the Doberman, 25–30% expe-
            maintain stroke volume and blood pressure, primarily
            through sodium and water retention and vasoconstric-
                                                              of CHF or syncope is again breed and etiology depend-
            tion. Stretch receptors and remodeling pathways are   rience SD before CHF. Survival time following the onset
            triggered, leading to lengthening of individual myocytes   ent, with Dobermans and Great Danes experiencing the
            (eccentric hypertrophy) and chamber dilation, mala-  shortest survival times among breeds (see Prognosis).
            daptive extracellular matrix remodeling, and cell death.   Risk factors for the development of DCM apart from
            Consequences of these alterations in cardiac structure   breed may include taurine deficiency (in cocker spaniels,
            and function  eventually include congestive heart failure   Labrador and golden retrievers, or nontraditional DCM
            (CHF) and arrhythmias (see Chapter 18).           breeds), sustained tachyarrhythmias, myocarditis, or
                                                              chemotherapy (anthracyclines).

            Epidemiology
                                                              Signalment
            The prevalence of DCM among canine referral popula-
            tions has been reported to be 0.3–1% (equivalent to 1–3   Dilated cardiomyopathy occurs predominantly in mid-
            cases per 300 patients examined). Underreporting of sud-  dle‐aged  to  older,  medium‐  to  large‐breed  dogs.  The
            den death (SD) cases might result in underestimation of   most commonly reported breeds in North America and
            any figure, thus the true prevalence in the general dog   Europe include the Doberman pinscher, IW, Great Dane,
            population is unknown. Any prevalence data will be   Newfoundland, boxer, Labrador and golden retrievers,
            heavily influenced by the distribution of breeds and ages   cocker spaniel, Scottish deerhound, Afghan hound, St
            (prevalence increases with age) of the population under   Bernard, German shepherd, Old English sheepdog,
            study. The prevalence is high in certain breeds based on   Airedale terrier, standard poodle, Rottweiler, and   mastiff.
            retrospective studies and prospective screening studies.   Small‐breed dogs are uncommonly affected but sporadic
            One European study of Irish wolfhounds (IW) found a   cases can be found.
            prevalence of 24%. In the Great Dane, prevalence has   Age at diagnosis is typically between 4 and 8 years,
            ranged from 12% to 36% in different geographic areas.   depending on breed. Much younger and much older
            Prevalence in Dobermans has ranged from 45% to 60% in   dogs may certainly be affected, regardless of breed.
            various studies. Some studies report overrepresentation   Specific juvenile forms of DCM are described in the
            of males, while in Dobermans there are gender differ-  Portuguese water dog, Doberman, and toy Manchester
            ences in how the disease manifests and at what age, with   terrier,  resulting  in  rapidly  progressive  CHF or  SD  at
            females having a higher incidence of ventricular arrhyth-  weeks to months of age. Sporadic cases in very young
            mias as the sole abnormality and males manifesting echo-  dogs of other breeds may occur, and should prompt
            cardiographic abnormalities and CHF at an   earlier age.    consideration of viral myocarditis or primary tachyar-
              The mode of inheritance is known for specific breed   rhythmia as an underlying cause.
            groups. In the Doberman and the Newfoundland, DCM   Many studies report a gender bias in males. While this
            is inherited as an autosomal dominant trait with incom-  is not corroborated in all studies of individual breeds, it
            plete penetrance. Both X‐linked and autosomal dominant   is the case for the majority of studies involving multiple
            patterns of inheritance have been suggested in the Great   breeds. Male Dobermans and IWs may be affected at an
            Dane, whereas inheritance is autosomal recessive with   earlier age than females.
            sex‐specific alleles in the IW. The juvenile form described
            in the Portuguese water dog is autosomal recessive.  History and Clinical Signs
              The natural history  of the  disease is  such  that  dogs
            experience  a  preclinical  or  occult  phase during  which   History may be unremarkable and preclinical cases iden-
            echocardiographic or electrocardiographic evidence of   tified by an incidental abnormality on physical exam
            the disease is present (see Diagnosis) but clinical signs   (such as a heart murmur, gallop, or arrhythmia) or by
            are absent (ACVIM stage B). The length of this phase is   prospective screening in high‐risk breeds. Some of these
            variable and likely extends over months to years, depend-  dogs in the preclinical stage may have surprisingly pro-
            ing on the breed. In the Doberman, previous retrospec-  found cardiac dysfunction in the absence of clinical signs.
            tive work suggested a median preclinical phase of just   More often, dogs are presented because of clinical signs
            over a year whereas new prospective clinical trial data   of a fairly acute nature. Respiratory signs are the most
            suggest a median of two years with the use of pimobendan.   common, including coughing and/or increased respira-
            The preclinical phase can be quite prolonged in the IW,   tory rate or effort due to pulmonary edema from left‐
            averaging 48 months in one trial.                 sided CHF. With severe pulmonary edema, expectoration
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