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Genetic Dilated Cardiomyopathy Melissa Ciampo, Dietetic Intern

University of Maryland College ParkChildren’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

Potential Causes: viral infections, autoimmune disease, toxin exposure, gene mutations

+ Dilated Cardiomyopathy

http://stanfordhospital.org/cardiovascularhealth/arrhythmia/overview/causes/heart-conditions.html

Compared to a normal heart, an enlarged & dilated left ventricle is less efficient pumping blood to the rest of the body

+ 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 techniquePacemakersVentricular 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

Genetic testing 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 Notes report patient consuming ~75% upon

discharge

+ Case Study: PTATaking some solid foods, oatmeal and

pureed baby foods

CW refusing feeds, in response- parents reported decreasing formula concentration from 28 kcal/oz to 22 kcal/ozParents changed formula to Similac Advance

Eats very well for babysitter, but not for parents

Mother feels that eating has become a very negative and stressful event, therefore has developed food aversions

+ Case Study: PTAParents report increased WOB, new post-

prandial emesis, and continued feeding difficulties

Worried about poor weight gainTwin sister is ~2-3 pounds heavier

+ Case Study: Assessment 3/2710.7 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 3/27On 3/26, 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

At end of previous admission: 25th-50th%tile.

Currently trending just below the 10th%tile

+ Length-for-Age

Trending relatively well, at just below the 50th%tile.

+ Head Circumference for Age

Trending up during prior admission, reaching 50th-75th%tile

Current admission, down to the 10th-25th%tile.

+ Weight for Length

Trending at ~5th%tile. Suggests she is growing well in length, but is not adequately gaining weight.

Medication Function Nutritional Implications

Chlorothiazide - Antihypertensive- Diuretic (K+ wasting)

- May deplete K+, Zinc, Q 10, Mg- Anorexia- Nausea/Vomiting- Electrolyte Abnormalities

Lasix - Loop-diuretic (K+ wasting)- used to treat fluid overload

- Depletes: Ca, Mg, Phos, K+, Vit B1, B6 & C- ↓ 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 Antagonist - used to treat GERD

-↑ gastric pH- In premature infants may cause bacterial overgrowth- may↑ incidence of NEC in infants

Spironolactone - Antihypertensive- Diuretic (K+ sparing): prevents Na reabsorption & K+ secretion- used to treat hypokalemia

- Nausea/Vomiting- Avoid vit. K supplements - ↑ excretion of Na, Cl, &Ca

Medications

+ 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 StatementInadequate 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 Needs100 – 130 kcal/kg

Catch-up Growth for Children with CHD

1.5 – 2.5 gm/kg proteinCatch-up Growth for Children with CHD

100 ml/kg fluidHolliday-Segar equation

+ RecommendationsIncrease 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.

+ RecommendationsIf feeds are tolerated running over 20 hrs,

consider condensing to bolus feeds q 3 hrsSimilac 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).

After transfer 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 Since admission CW has experienced an overall

weight gain of 500 gms (~45 gm/day)

+ ReferencesHong, 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.

 

+ Any Questions?