Page 8 - Heart Failure Guidelines
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Pediatric Heart Failure Guidelines

                     A variant of unknown significance (VUS) that has a high possibility of causing
                       cardiomyopathy, if there is high parental concern, or if there is high concern amongst the
                       heart function team.
                     Prior to testing relatives (see table below) - preferably within ninety days of the
                       proband’s results (as the family testing is then free of charge).

               Genetic Testing
               Genetic testing can be done using either saliva or blood. Testing takes around three weeks for
               the result. See the specific diseases for the individual panels to be requested.

               Inpatient testing should be done only when it will influence clinical management. Examples
               include a likelihood of dying or requiring transplant or mechanical support.

               Transfusions of blood products may invalidate the result as the sample may be “contaminated”
               with donor DNA. However the testing is done on leukocytes and most blood products are filtered
               to minimize the number of leukocytes. An isolated red cell transfusion is therefore unlikely to
               cause a false result. If a transfusion is with large volumes of blood which includes
               cardiopulmonary bypass then if possible to test before the transfusion, otherwise wait as long as
               possible (up to two months) before testing is performed.

               The table below details recommendations for family screening from the AHA 2013 Heart Failure
               Guidelines: Circulation 2013;128:e240-e327. DOI: 10.1161/CIR.0b013e31829e8776

               Dose Escalation and Approach to Heart Rate and Blood Pressure Management

               Dosage of heart failure medications should be up titrated until the target dose is reached, side-
               effects or symptoms occur (see medications for details at end of guideline). At each outpatient
               visit with the heart failure team, cardiac medication should be assessed and up titrated as
               necessary. Due to side effects, often only one medication is up titrated at each visit. The
               attending physician may decide to up titrate more than one medication, or prioritize one over the
               other if clinically warranted (e.g. tachycardia, hypertension, myocardial fibrosis, edema).  The
               general approach will to be initiate heart failure treatment with ACE-I and once optimized add

               Updated 9/21/2018                                                                       page 8
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