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
+Genetic Dilated Cardiomyopathy Melissa Ciampo, Dietetic Intern University of Maryland College Park Children’s National Medical Center Case Study April 5, 2013
+ Outline Overview of Dilated Cardiomyopathy (DCMP) Case Study Background Patient Assessment PES Statement Plan and Goals Follow-up
+ 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
+ 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
+ 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%
+ 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.
+ 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)
+ 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
+ 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
+ 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
+ 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)
+ 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
+ Weight-for-Age Currently trending just below the 10th%tile At end of previous admission: 25th-50th%tile.
+ Length-for-Age Trending relatively well, at just below the 50th%tile.
+ Head Circumference for Age Current admission, down to the 10th-25th%tile. Trending up during prior admission, reaching 50th- 75th%tile
+ Weight for Length Trending at ~5th%tile. Suggests she is growing well in length, but is not adequately gaining weight.
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
+ 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.
+ 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).
+ 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
+ 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.
+ 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.
+ 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).
+ 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.
+ Follow-up Sinceadmission CW has experienced an overall weight gain of 500 gms (~45 gm/day)
+ 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.