This document summarizes a case study of a 10-month-old female infant diagnosed with genetic dilated cardiomyopathy. The infant was experiencing poor weight gain, recurrent food aversions, and increased work of breathing. An assessment found the infant's weight and weight-for-length were below the 10th percentile. The patient's medications could impact nutrition by causing electrolyte abnormalities and gastrointestinal side effects. The dietitian recommended increasing the concentration of the infant's formula to meet her estimated caloric and protein needs and support catch-up growth. Follow-up found the infant had gained weight but was unstable and enteral intake needed close monitoring.

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 Implications
Chlorothiazide - Antihypertensive - May deplete K+, Zinc, Q 10, Mg
- Diuretic (K+ wasting) - Anorexia
- Nausea/Vomiting
- Electrolyte Abnormalities
Lasix - 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 extremities
Prednisolone - Corticosteroid - Hyperglycemia
- ↓ calcium absorption
- weight gain
Zantac - Histamine H2 Receptor -↑ gastric pH
Antagonist - In premature infants may cause bacterial
- used to treat GERD overgrowth
- may↑ incidence of NEC in infants
Spironolactone - 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?