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966   Tetralogy of Fallot


           •  The overriding aorta acts as a conduit that    TREATMENT           •  Aspirin or clopidogrel should be used if a
            further contributes to the right-to-left shunt-  Treatment Overview    surgical shunt is performed to reduce the
  VetBooks.ir  •  Severity of the RV obstruction often deter-  There are surgical and palliative medical man-  Behavior/Exercise
                                                                                   risk of thrombosis.
            ing of blood.
                                              agement  strategies.  Definitive  surgical  repair
            mines direction and volume of shunting.
           •  Flow direction and volume can additionally
            be altered by systemic blood pressure.  may need to be delayed if patient size is not   Vigorous or extended exercise restrictions should
                                              amenable. Palliative surgical options may be
                                                                                 be implemented.
           •  Exercise and systemic hypotension are likely   considered to allow patient to grow to a larger
            to increase the right-to-left shunting.  size for definitive repair. Alternatively, palliative   Drug Interactions
           •  Increased blood flow through bronchial artery   surgical and medical options may provide relief   Hydroxyurea may be difficult to dose with the
            collateral vessels increases venous admixture   of symptoms for a period of time.  commercially available product, especially in
            and further lowers the arterial PO 2 .                               patients with low body weights. Compounding
           •  Chronic hypoxemia caused by the right-to-  Acute General Treatment  is frequently needed, and caution is warranted
            left shunting can lead to erythrocytosis and   •  The  asymptomatic  patient  may  not  need   when chronically administering a chemothera-
            signs of hyperviscosity.            acute treatment.                 peutic drug. Periodic monitoring of the CBC
                                              •  Sedation with butorphanol 0.2-0.3 mg/kg   is warranted.
            DIAGNOSIS                           may be beneficial if the patient is anxious
                                                and dyspneic. Avoid sedatives and anxiolytics   Recommended Monitoring
           Diagnostic Overview                  that can decrease systemic blood pressure.  •  Monitor PCV or hematocrit; CBC if patient
           TOF should be suspected based on young   •  Supplemental  oxygen,  although  of  little   receiving hydroxyurea
           age  of  animal,  left  basilar  systolic  murmur,   benefit with right-to-left shunts, should be   •  If surgical shunt is created, serial echocardio-
           and cyanosis. CBC, electrocardiography, and   provided when clinical signs are apparent.   grams are warranted to monitor for patency.
           thoracic radiographs may help raise or lower   In addition to oxygen therapy, the quieter   •  Resting  heart  rate,  especially  when  using
           the  likelihood  of TOF,  but  the  most  useful   environment of oxygen cages may provide   beta-blockers; resting respiratory rate
           diagnostic test is a complete echocardiogram.  a  buffer  from  hospital  noise.  Monitor
                                                temperature in cage and avoid hyperthermia.   PROGNOSIS & OUTCOME
           Differential Diagnosis             •  If  necessary,  increase  systemic  vascular
           •  Pulmonic stenosis with VSD or ASD  resistance with an alpha-adrenergic agonist   Median survival time reported in one recent
           •  ASD,  VSD,  or  PDA  with  pulmonary   (phenylephrine)  to  reduce  right-to-left   publication  was  approximately  2  years  for
            hypertension (Eisenmenger’s physiology)  shunting.                   dogs and cats being treated medically. Pallia-
           •  Double-chamber  right  ventricle  with  a    •  If packed cell volume (PCV) or hematocrit   tive surgery can significantly improve clinical
            VSD                                 is  >  70%,  perform  a  phlebotomy  with  a   outcome. A low-grade murmur appears to have
           •  Double-outlet right ventricle     target of 10%-12% drop in PCV. Replace the   poorer prognosis than a higher-grade murmur
           •  Truncus and pseudotruncus arteriosus  removed volume with a balanced crystalloid   with TOF. Sudden cardiac death is common.
           •  Extracardiac causes of erythrocytosis (pul-  (100%-200%).
            monary, renal, and bone marrow)                                       PEARLS & CONSIDERATIONS
                                              Chronic Treatment
           Initial Database                   •  Definitive  surgical  repair  is  available  but   Comments
           •  CBC: erythrocytosis               requires cardiac bypass and open-heart   Erythrocytosis is commonly seen with TOF
           •  Thoracic radiography: thoracic radiographs   surgery. Veterinary facilities that can provide   secondary to hypoxemia, but hydration status
            commonly have minor abnormalities despite   cardiac bypass are limited at this time.  is commonly overlooked.
            the significant cardiovascular changes present.   •  Palliative surgical options to create a systemic
            RV, right atrial, and pulmonary artery (best   to pulmonary shunt (e.g., modified Blalock-  Prevention
            seen on the ventrodorsal or dorsoventral   Taussig shunt) can improve pulmonary blood   Breeding of affected dogs and their immediate
            views) enlargement may be seen. Thoracic   flow and increase systemic arterial oxygen   relatives is not recommended.
            radiographs are most helpful in ruling out   concentrations. This surgical option does not
            primary respiratory disease.        require bypass and can be performed by an   Technician Tips
           •  Electrocardiography (ECG): RV hypertrophy   experienced thoracic surgeon.  If routine phlebotomy is indicated, care should
            pattern is typically present.     •  Another palliative surgical option is balloon   be taken to preserve vessels during venipuncture.
           •  Systemic  blood  pressure:  clinical  assess-  valvuloplasty if the pulmonic stenosis is
            ment of systemic vascular resistance should   valvular.  Caution  is  warranted  because   Client Education
            be assessed before treatment with beta-   overdilation with the balloon catheter can   If animals acquire heartworm infection, it could
            blockers.                           lead to severe left-to-right shunting and acute   be a fatal combination with TOF. Heartworm
                                                pulmonary edema.                 preventives should be given regularly, especially
           Advanced or Confirmatory Testing   •  Control of erythrocytosis (PCV > 70%) with   in endemic areas.
           •  Echocardiography with color Doppler: should   periodic phlebotomy may be necessary.
            allow confirmation of all four components   •  If frequent phlebotomies are needed to control    SUGGESTED READING
            of TOF. Contrast echocardiography (bubble   PCV, consider hydroxyurea at 30-50 mg/kg   Chetboul  V,  et  al:  Epidemiological,  clinical,  and
            study) may be beneficial if there is equivo-  PO q 24-48h; titrate based on PCV.  echocardiographic features and survival times of
            cal right-to-left shunting or bidirectional   •  Propranolol, a nonselective beta-adrenergic   dogs  and  cats  with  tetralogy  of  Fallot:  31  cases
            shunting.                           blocker,  should  be  considered.  The  beta-  (2003-2014). J Am Vet Med Assoc 249:909-917,
           •  Cardiac  catheterization  and  angiography   2-blocking effects may reduce systemic vasodi-  2016.
            are typically not needed but may be con-  lation,  minimizing  right-to-left  shunting.     AUTHOR: Ryan Baumwart, DVM, DACVIM
            sidered if definitive surgical correction is    It may also reduce dynamic RV obstruction   EDITOR: Meg M. Sleeper, VMD, DACVIM
            planned.                            from RV hypertrophy. The dose range for
           •  CT/MRI are typically not needed but may   propranolol is 0.5-1.0 mg/kg q 8h starting
            be beneficial if definitive surgical correction   at the lower dosage and titrating upward
            is planned.                         based on symptoms and resting heart rate.

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