Dilated Cardiomyopathy, specifically in Children

1. DILATED CARDIOMYOPATHY
AAKRITI DHIMAN
ROLL NO. 1

2. SCHEME OF PRESENTATION
• INTRODUCTION
• CLASSIFICATION
• ETIOLOGY
• PATHOGENESIS
• CLINICAL FEATURES
• INVESTIGATIONS
• TREATMENT

3. INTRODUCTION
• CARDIOMYOPATHIES- HETEROGENOUS HEART MUSCLE DISEASES,
A/W STRUCTURAL REMODELLING & ABNORMAL CARDIAC FUNCTION.
• MOST COMMON- DILATED TYPE- LV DILATION, DECREASED LV
SYSTOLIC FUNCTION
• SIGNIFICANT MORBIDITY AND MORTALITY, COMMON INDICATION
FOR TRANSPLANT.

4. CLASSIFICATION

6. ETIOLOGY & EPIDEMIOLOGY
• MOST COMMON- IDIOPATHIC
• DIVERSE ETIOLOGY- UNLIKE ADULT PTS, ISCHEMIC ETIOLOGY IS RARE IN
CHILDREN- BUT STILL INCLUDE ANOMALOUS ORIGIN OF LEFT CORONARY
ARTERY FROM PULMONARY ARTERY, PREMATURE CORONARY
ATHEROSCLEROSIS (HOMOZYGOUS TYPE 2 HYPERCHOLESTROLEMIA),
CORONARY INFLAMMATORY DISEASES- KAWASAKI.
• 50% GENETIC- AD/AR/XL
• A/W CONDUCTION DEFECTS & SNHL
• INCIDENCE- <18Y- 0.57/100K PER YEAR.
• RISK FACTORS- MALE, AFRICAN AMERICAN, INFANTS ,<1Y

7. PATHOGENESIS
• B/O UNDERLYING ETIOLOGY- INCLDUES SYSTOLIC DYSFUNCTION &
MYOCYTE INJURY
• AD,XL- GENETIC ABNORMALITY- SARCOMERE, CYTOSKELETON,
PROTEINS BRIDGING CONTRACTILE APPARATUS TO CYTOSKELETON
(PRIMARY/ GENETIC)- DIRECT MYOCARDIAL INJURY
• FOLLOWING VIRAL MYOCARDITIS (SECONDARY)- VIRAL INDUCED
INFLAMMATION
• IN BOTH- FINAL COMMON PATHWAY-SIMILAR DAMAGE, VENTRICLE
ENLARGEMENT, POOR FUNCTION

8. • 20-50%- AD
• 5-10%- DUCHENN, BECKER MUSCULAR DYSTROPHY- XL
• DYSTROPHINOPATHIES- ABNORMAL SARCOMERE- CYTOSKELETON
CONNECTION- IMPAIRED FORCE GENERATION, MYOCYTE DAMAGE,
SCARRING, CHAMBER ENLARGEMENT, ALTERED FUNCTION
• MITOCHONDRIAL MYOPATHIES- AR, MITOCHONDRIAL PATTERN- ALSO
HAVE EXTRACARDIAC C/F
• IEM- FATTY ACID OXIDATION- SYSTEMIC C/F- HYPOKETOTIC
HYPOGLYCEMIA, ACIDOSIS, LIVER DYSFUNCTION, PERIPHERAL NEURO &
MYOPATHY, SUDDEN DEATH, ARRYTHMIA
• ANTHRACYCLINE TOXICITY- IF >550MG/M2 CUMULATIVE DOSE

10. CLINICAL FEATURES
• MORE PREVALENT IN <1Y
• HEART FAILURE SYMPTOMS, PRONE TO EMBOLIC PHENOMENA
• PALPITATIONS, SYNCOPE, SUDDEN DEATH
• IRRITATION, LETHARGY, FTT, NAUSEA, VOMITING, ABOMINAL PAIN
• RS- TACHYPNEA, WHEEZING, COUGH, DYSPNEA ON EXERTION
• PALLOR, ALTERED SENSORIUM, HYPOTENSION, SHOCK
• TACHYCARDIA, NARROW PULSE PRESSURE, LIVER ENLARGED, RALES,
WHEEZING
• PRECORDIAL CARDIAC PULSE INCREASED- HEART ENLARGED ON
PERCUSSION AND PALPATION

11. • AUSCULTATION- TACHYCARDIA, S3 GALLOP RHYTHM, MITRAL/TC
INSUFFICIENCY MURMUR
• IF HYPOGLYCEMIA, ACIDOSIS, HYPOTONIA, LIVER DYSFN- S/O IEM
• NEURO/SKELETAL EFECTS- MTN/ MUSCULAR DYSTROPHIES

12. INVESTIGATIONS
• ECG- ATRIAL/ VENTRICULAR HYPERTROPHY, NON SPECIFIC T WAVE
ABNORMALITY, ATRIAL/ VENTRICULAR ARRYTHMIA
• ECHO-DIAGNOSTIC- CHARACTERISTIC- LV ENLARGEMENT WITHOUT
HYPERTROPHY , DECREASED VENTRICULAR CONTRACTILITY, GLOBULAR/
REMODELED LV CONTOUR, RV ENLARGEMENT, DEPRESSED FUNCTION
• CXR- CARDIOMEGALY, PULMONARY VASCULAR PROMINENCE, PLEURAL
EFFUSION
• ECHO DOPPLER- PULMONARY HTN, MR
• CBC, RFT, LFT, CK, CARDIAC TROP I, BNP, LACTATE, PLASMA AA, URINE
ORGANIC ACIDS, ACYLCARNITINE PROFILE
• GENETIC, ENZYMATIC TESTS

15. • CARDIAC CATHETERIZATION
• ENDOMYOCARIAL BIOPSY- MONONUCLEAR CELL INFILTRATES,
MYOCARDIAL DAMAGE, STORAGE ABNORMALITIES, INFECTION
• SCREENING- 1ST DEGREE FAMILY MEMBERS- ECHO, ECG

16. TREATMENT
• DIURETICS
• ACE INHIBITORS, ARB
• DIGITALIS
• BETA BLOCKERS- CARVEDILOL (PERIPHERAL VASODILATOR ALSO) –
0.1MG/KG/DAY, INCREASE TO 0.5MG/KG/DAY, METOPROLOL
• IV INOTROPES, DIURETICS, MECHANICAL VENTILATION, MECHANICAL
CIRCULATION SUPPORT- LVAD, ECMO, CARDIAC TRANSPLANTATION-MOST
COMMON CAUSE OF CARDIAC TRANSPLANTATION IN PEDIATRICS
• ANTIARRYTHMICS
• WEEKLY DOBUTAMINE/ LEVOSIMENAN INFUSION

17. REFERENCES
• NELSON TEXTBOOK OF PEDIATRICS- 20TH E
• OP GHAI TEXTBOOK OF PEDIATRICS- 9TH E

18. THANK YOU