Case Study: Genetic Dilated Cardiomyopathy
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Case Study: Genetic Dilated Cardiomyopathy

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  • According to the Korean Journal of Pediatrics, “use of ventricular assist devices has been shown to significantly improve the survival of adults and children with end-stage DCMP who are awaiting heart transplantation”

Case Study: Genetic Dilated Cardiomyopathy Presentation Transcript

  • 1. +Genetic Dilated Cardiomyopathy Melissa Ciampo, Dietetic Intern University of Maryland College Park Children’s National Medical Center Case Study April 5, 2013
  • 2. + Outline  Overview of Dilated Cardiomyopathy (DCMP)  Case Study Background  Patient Assessment  PES Statement  Plan and Goals  Follow-up
  • 3. + Dilated Cardiomyopathy  Myocardium becomes enlarged & thickened, preventing normal heart contractions  As the heart works harder, the heart muscle dilates (stretches & becomes thinner) leading to the inner chamber enlarging  Results in poor contractions insufficient blood delivery to the rest of the body  PotentialCauses: viral infections, autoimmune disease, toxin exposure, gene mutations
  • 4. + Dilated Cardiomyopathy  Compared to a normal heart, an enlarged & dilated left ventricle is less efficient pumping blood to the rest of the body http://stanfordhospital.org/cardiovascularhealth/arrhythmia/overview/causes/heart-conditions.html
  • 5. + Dilated Cardiomyopathy  Genetically inherited in ~30 – 48% of cases  Symptoms: labored breathing, poor appetite, slow weight gain, heart failure (severe cases)  Treatment Options:  No single proven surgical technique  Pacemakers  Ventricular assist devices: Improved the survival rate of adults & children w/end- stage DCMP who are awaiting heart transplantation  Prognosis: 9-year survival rate ~69.8%
  • 6. + Nutrition and DCMP Source: Miller TL, Neri D, Extein J, Somarriba G, Strickman-Stein N. "Nutrition in pediatric cardiomyopathy." Progress in Pediatric Cardiology 24 (2007): 59 - 71.
  • 7. + Case Study: Background  Name: CW; Ex-35 week preemie (twin)  Gender: Female  DOB: 5/4/2012  Birth Weight: 2.06 kg (10th%tile on preemie growth chart)  Twin brother with intrauterine growth retardation (IUGR), but otherwise healthy  Diagnosed with Genetic Dilated Cardiomyopathy in August 2012  Genetictesting revealed mutation of TNNI3 gene (involved in coding for cardiac muscle tissue)
  • 8. + Case Study: Background  Hospitalized at CNMC from July – Sept. 2012  From previous admission report patient with recurrent food aversions, poor intake, & difficulty gaining weight  On 9/23/12, sent home on Similac Sensitive (28 kcal/oz.) 96 ml q 3 hrs  Notesreport patient consuming ~75% upon discharge
  • 9. + Case Study: PTA  Takingsome solid foods, oatmeal and pureed baby foods  CW refusing feeds, in response- parents reported decreasing formula concentration from 28 kcal/oz to 22 kcal/oz  Parents changed formula to Similac Advance  Eats very well for babysitter, but not for parents  Motherfeels that eating has become a very negative and stressful event, therefore has developed food aversions
  • 10. + Case Study: PTA  Parents report increased WOB, new post- prandial emesis, and continued feeding difficulties  Worried about poor weight gain  Twin sister is ~2-3 pounds heavier
  • 11. + Case Study: Assessment 3/27  10.7 month old female (9.5 month CGA)  Admitted for new post-prandial emesis, increased WOB, and continued feeding difficulties  Diagnosis:Genetic Dilated Cardiomyopathy, Heart Failure, and Failure to Thrive (FTT) ANTHROPOMETRICS Weight 7.11 kg (Just below 10th%tile) Length 70 cm (Just below 50th%tile) Head Circumference 43 cm (10th-25th%tile)
  • 12. + Case Study: Assessment 3/27  On3/26, Similac Advance concentrated to 22 kcal/oz.  Goal rate (40ml/hr) reached & tolerating it well 1 emesis- ~60 ml undigested formula 1 BM  Weight is up 10 gm since admission on 3/22
  • 13. + Weight-for-Age Currently trending just below the 10th%tile At end of previous admission: 25th-50th%tile.
  • 14. + Length-for-Age Trending relatively well, at just below the 50th%tile.
  • 15. + Head Circumference for Age Current admission, down to the 10th-25th%tile. Trending up during prior admission, reaching 50th- 75th%tile
  • 16. + Weight for Length Trending at ~5th%tile. Suggests she is growing well in length, but is not adequately gaining weight.
  • 17. Medications Medication Function Nutritional ImplicationsChlorothiazide - Antihypertensive - May deplete K+, Zinc, Q 10, Mg - Diuretic (K+ wasting) - Anorexia - Nausea/Vomiting - Electrolyte AbnormalitiesLasix - Loop-diuretic (K+ wasting) - Depletes: Ca, Mg, Phos, K+, Vit B1, B6 & C - used to treat fluid overload - ↓ utilization of folate - Can cause: GI distress, dry mouth, weight gain, & swelling of extremitiesPrednisolone - Corticosteroid - Hyperglycemia - ↓ calcium absorption - weight gainZantac - Histamine H2 Receptor -↑ gastric pH Antagonist - In premature infants may cause bacterial - used to treat GERD overgrowth - may↑ incidence of NEC in infantsSpironolactone - Antihypertensive - Nausea/Vomiting - Diuretic (K+ sparing): prevents - Avoid vit. K supplements Na reabsorption & K+ secretion - ↑ excretion of Na, Cl, &Ca - used to treat hypokalemia
  • 18. + Pertinent Labs Lab 3/27/13 Significance Na 124 (L) - Commonly ↓ with CHF, due to diuretic use. - Levels fluctuate with fluid shifts. - Spironolactone (diuretic) ↑ urinary excretion. Cl 86 (L) - Commonly ↓ with CHF, due to diuretic use. - Levels fluctuate with fluid shifts. - Spironolactone (diuretic) ↑ urinary excretion. BUN 57 (H) - ↑ in dehydration - ↑ with heart failure, CHF, and renal insufficiency. Cr 0.7 (H) - ↑ in dehydration - ↑ with heart failure, CHF, and renal insufficiency.Glucose 110 (H) - Slightly elevated. - ↑ during stress, & may be from corticosteroid therapy. BNP >20,000 (H) - Important biomarker for poor heart function - ↑ with degree of heart failure.
  • 19. + PES Statement  Inadequate Oral Intake (NI-2.1) related to genetic dilated cardiomyopathy, heart failure, and food aversions as evidenced by parent report and poor weight gain (10th%tile for weight, 5th-10th%tile weight-for-length, and 87% IBW).
  • 20. + Estimated Nutritional Needs  100 – 130 kcal/kg  Catch-up Growth for Children with CHD  1.5 – 2.5 gm/kg protein  Catch-up Growth for Children with CHD  100 ml/kg fluid  Holliday-Segar equation
  • 21. + Recommendations  Increase concentration of Similac Advance to 24kcal/oz (goal) & continue @ 40ml/hr. x 24 hrs.  Will provide 135ml/kg fluid, 108kcal/kg, & 2.16gm/kg protein (meeting 100% estimated needs).  If feeds tolerated x 24 hrs, condense to run over 20 hrs.  Similac Advance 24kcal/oz @ 48ml/hr x 20 hrs via NGT. Can divide 4-hr break into 2 hrs off BID.
  • 22. + Recommendations  Iffeeds are tolerated running over 20 hrs, consider condensing to bolus feeds q 3 hrs  Similac Advance 24kcal/oz, 120 ml q 3 hrs.  Allow pt to PO trial 20 min before each bolus feed  NG gavage remaining volume.  Recommend initially running each bolus feed over 2 hrs, & condense by 15 min as tolerated to a goal of each bolus run over 30 – 60 min. By slowly increasing the rate and condensing to bolus feeds, it allows enteral nutrition to be more physiologic.
  • 23. + Recommendations  Obtain weights daily. Goal weight gain is 15 – 25 gms/day for catch-up growth.  Measure HC & length weekly.  Start Poly-vi-sol w/Iron. (Pt is a preemie and currently on standard infant formula).
  • 24. + Follow-up  On 3/28 CW was transferred from HKU to CICU for Milrinone drip (heart failure medication, she responded well to during previous admission).  Aftertransfer to CICU, pt was visited by sick relatives. Pt became ill and TF was stopped for the day.  Until she is hemodynamically stable, close monitoring of her enteral intake and tolerance will be key during assessment at the next follow-up.
  • 25. + Follow-up  Sinceadmission CW has experienced an overall weight gain of 500 gms (~45 gm/day)
  • 26. + References Hong, Y. "Cardiomyopathies in Children." The Korean Pediatric Society 56.2 (2013): 52 - 59. Ku L, Feiger J, Taylor M, Mestroni L. "Familial dilated cardiomyopathy. ." Circulation 108 (2003): 118 - 121. Miller TL, Neri D, Extein J, Somarriba G, Strickman-Stein N. "Nutrition in pediatric cardiomyopathy." Progress in Pediatric Cardiology 24 (2007): 59 - 71. Pronsky, Zaneta M. and Jeanne P. Crowe. Food Medication Interactions. 17th Edition. Birchrunville: Food Medication Interactions, 2012. Towbin JA, Lowe AM, Colan SD, et al. "Incidence, Causes, and Outcomes of Dilated Cardiomyopathy in Children ." JAMA 296.15 (2006): 1867 - 1876.
  • 27. + Any Questions?