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100    PART I   Cardiovascular System Disorders



                          CHAPTER                                    5
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                            Congenital Cardiac


                                                        Disease













            GENERAL CONSIDERATIONS                               some patients. Congenital malformations vary widely in type
                                                                 and severity. The patient’s prognosis and options for therapy
            Common congenital cardiac malformations, as well as some   depend on the definitive diagnosis and severity. Initial
            that occur more sporadically, are described in this chapter.   noninvasive testing usually includes thoracic radiographs,
            Most congenital heart defects produce an audible murmur   a complete echocardiogram (M-mode, two-dimensional
            (Fig. 5.1), although some serious malformations do not.   [2-D], and color and spectral Doppler), and sometimes an
            Murmurs caused by congenital disease range in intensity   electrocardiogram (ECG). A packed cell volume (PCV) is
            from very loud to very soft depending on the type and   indicated in cases of right-to-left shunting to document
            severity of the defect and on hemodynamic factors. In addi-  erythrocytosis. Cardiac catheterization with selective angio-
            tion to murmurs of congenital disease, clinically irrelevant   cardiography can be useful to define some structural abnor-
            “innocent” murmurs are relatively common in puppies and   malities and is used to guide transvascular interventional
            kittens. The physiologic cause of these murmurs is incom-  procedures. Surgical repair or palliation, balloon valvulo-
            pletely understood but may involve mismatch in growth   plasty, transcatheter shunt occlusion, or other interventional
            rate between the heart and great vessels, relative anemia, or   techniques may be helpful for some cases.
            high sympathetic tone compared to adult animals. Innocent   Patent ductus arteriosus (PDA), pulmonic stenosis (PS),
            murmurs are usually soft systolic ejection–type murmurs   and subaortic stenosis (SAS) have been identified in different
            heard best at the left heart base; their intensity may vary   surveys  as  the most  common  congenital  cardiovascular
            with  heart  rate  or body position. Innocent  murmurs  tend   anomalies in the dog. Persistent right aortic arch (a vascular
            to get softer and usually disappear by about 4 months of   ring anomaly), ventricular septal defect (VSD), malforma-
            age. Murmurs caused by congenital disease usually persist   tions (dysplasia) of the atrioventricular (AV) valves, atrial
            beyond 6 months of age and may get louder with time,   septal defect (ASD), and tetralogy of Fallot (T of F) occur
            although this  is  not  always the  case. Careful  examination   less frequently but are not rare. The most common con-
            and auscultation are important, not only in animals intended   genital malformation in cats is VSD; other lesions include
            for breeding but also in working dogs and pets. Puppies and   AV valve dysplasias, stenotic lesions (SAS and PS), ASD, T
            kittens with a soft left basilar systolic murmur and no other   of F, and PDA. An AV septal (endocardial cushion) defect
            clinical or radiographic signs can be auscultated repeatedly   occurs more commonly in cats and consists of all or some of
            as they grow to determine whether the murmur disappears.   the following: a high VSD, a low ASD, and malformations of
            Further diagnostic tests are indicated in puppies or kittens   one or both AV valves. Endocardial fibroelastosis, mainly in
            with  a  murmur  that  is  loud  (audible  on  both  sides  of  the   Burmese and  Siamese  cats,  also  has been  reported.  Con-
            chest), is heard loudest on the right hemithorax, or has a dia-  genital malformations are more prevalent in male than
            stolic component, or with any murmur that persists beyond   female cats. Congenital malformations in both species can
            4 to 6 months of age. Additional reasons to pursue diag-  occur as isolated defects, which is most often the case, or in
            nostic testing early include patients with clinical signs and   various combinations.
            those for which economic or breeding-potential decisions     The prevalence of congenital defects is higher in purebred
            are pending.                                         than in mixed-breed animals. In some studies, a polygenic
              Congenital heart defects most often involve either a valve   inheritance pattern has been suggested, although there is
            (or valve region) or an abnormal communication between   more recent focus on a single major gene effect influenced
            the systemic and pulmonary circulations. Abnormally   by other modifying genes. Recognized breed predispositions
            formed valves can be insufficient, stenotic, or both. Other   are listed in Table 5.1; animals of other breeds can be affected
            malformations can exist, and multiple anomalies occur in   with any of these defects as well.

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