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?