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MIMICKERS OF DILATED CARDIOMYOPATHY
VIKAS KOHLI MD FAAP FACC
DIRECTOR AND HOD CHILDRENS HEART INSTITUTE
BLK SUPERSPECIALITY HOSPITAL
HISTORY
• CHILD UNWELL
• MURMUR
• CHEST XRAY
• CARDIAC ENLARGEMENT
• ECHO ASKED FOR
• DIAGNOSIS DILATED CARDIOMYOPATHY
POSSIBILITIES-THE REAL ONE AND THE MIMICKERS
1. MYOCARDITIS & DILATED CARDIOMYOPATHY
2. ALCAPA
3. METABOLIC DISORDERS
4. COARCTATION OF AORTA
5. SEVERE AORTIC STENOSIS
6. KAWASAKI DISEASE
7. HYPERTENSIVE CARDIOMYOPATHY
8. TACHYCARDIA-SVT
THE REAL ONE: MYOCARDITIS
• VIRAL INEFCTION HISTORY IN 60-70% PTS
• 15% FULMINANT MYOCARDITIS
• FIRST SUSPICION ON XRAY
• ECHO CONFIRMS THE FUNCTION NOT THE DIAGNOSIS
• ECHO CAN HELP R/O OTHER ETIOLOGIES
• TROPONIN I IS DIAGNOSTIC + IN 90%
• COMPLETE RECOVERY TO NORMAL FUNCTION IN 80% OF THE PATIENTS W APPROPRIATE TREATMENT
DILATED CARDIOMYOPATHY VS MYOCARDITIS
• IT MAY BE DIFFICULT TO DIFFERENTIATE AT TIMES
• DILATED CARDIOMYOPATHY IS BURNT OUT MYOCARDITIS
• DEFINITIVE DIAGNOSIS IS MADE BY DEMONSTRATING FIBROSIS ON CARDIAC MRI (CMRI)
• WITH MEDICAL MANAGEMENT PTS CAN BE MANAGED IN COPENSATED HEART FAILURE FOR DECADES
• IF ON MEDICAL MANAGEMENT THERE IS WORSENING
• LVAD
• CARDIAC TRANSPLANT
OBSTRUCTUVE LESIONS OF LEFT HEART
• WHEN OBSTRUCTION IS NOT CRITICAL, LV WILL HYPERTROPHY
• WHEN IT FURTHER INCREASES THE LV MAY FAIL AND BECOME DILATED WITH LOW EJECTION FRACTION
• SUDDEN BLOCKAGE BECOMING APPARENT WOULD ALSO HAVE THE SAME EFFECT
• Eg PDA CLOSING IN CRITICAL COARCTATION: SUDDEN DETERIORATION OF LV FUNCTION
• THESE MAY PRESENT AS NEWBORNS
• THUMB RULE: ALL NEWBORNS IN SHOCK NEED TO HAVE AN ECHO DONE
• LESIONS:
• CRITICAL COARCTATION
• CRITICAL AORTIC VALVE STENOSIS
OBSTRUCTIVE LESIONS OF LV
• FOR CRITICAL COARCTATION IN A NEWBORN THE IMMEDIATE SOLUTION MAYBE IF BABY IS < 14 DAYS OLD IS TO START PROSTAGLANDIN
• DOSE 0.05MCG/KG/MIN
• ADDITIONALLY INOTROPES WILL HELP IN BOTH CRITICAL AORTIC STENOSIS & COARCTATION
• FOR COARCTATION DEFINITIVE TREATMENT IS SURGERY
• FOR AORTIC STENOSIS CRITICAL WITH LOW EJECTION FRACTION THE TREATMENT IS BALLOON DILATATION
CORONARY ABNORMALITY: ALCAPA
• RARE BUT TRUE: ANOMALOUS LEFT CORONARY ARTERY CAN PRESENT AT 6-8 WEEKS AS A FAILAING HEART
• THE BLOOD SUPPY TO LV IS FROM LEFT CORONARY
• TILL 4 -6 WEEKS THE NEONATAL PHYSIOLOGIC PAH ALLOWS FORWARD FLOW INTO THE CORONARY
• AFTER THAN, WHEN THE PAH HAS REGRESSED THE FLOW REVERSES IN THE CORONARY ARTERY
• THIS RESULTS IN DIASTOLIC STEALING OF BLOOD FROM THE CORONARY SUPPLY AREA IE LV MYOCARDIUM
• THIS RESULTS IN LV FAILURE WITH LOW EJECTION FRACTION
• THE ECHO WILL LOOK EXACTLY LIKE THAT OF ALCAPA UNLESS ONE DEMONSTRATES THE CORONARY FLOW
ALCAPA
• THE TREATMENT IS SURGICAL REIMPLANTATION OF THE CORONARY ARTERY TO THE AORTA
• RESULTS OF THE BABY SURVIVES THE SURGERY ARE CURATIVE FOR THE REST OF LIFE
OTHE RCORONARY ABNORMALITY: ACQUIRED HEART DISEASE: UBIQUITOUS RESENTATION
• PRESRENTS LIKE MEASLES
• FEVER
• RASH
• UNRELENTING FEVER
• SOLITARY LNE
• STRAWBERRY TONGUE
• PEELING OF SKIN
• Kawasaki disease IN A RARE PRESENTATION MAY RESULT IN DILATED CRDIOMYOPATHY
SKIN PEELING
METABOLIC CAUSES OF DILATED CARDIOMYOPATHY
• PRESENTATION MAY BE DRAMATIC
• PRECIPITATING EVENT IS A STRESS USUALLY A VIRAL
• MAKES IT VRY CONFUSING W MYOCARDITIS
• IMPORTANT TO RECOGNIZE THIS ENTITY BECAUSE MAY BE ABLE TO REVERT
• CLUES CAN BE IN HSTORY, INCLUDING FAMILY X, ECHO, LABS
• ANION GAP
• SEVERE INTRACTABLE UNCORRECTABLE ACIDOSIS
• STORE URINE SAMPLE AND BLOOD AND SERUM SAMPLES IN THESE CASES
WHICH METABOLIC DISORDERS
Algorithm for CM associated with hypoglycemia.
Schwartz M L et al. Circulation. 1996;94:2021-2038
Copyright © American Heart Association, Inc. All rights reserved.
Algorithm for CM associated with metabolic acidosis.
Schwartz M L et al. Circulation. 1996;94:2021-2038
Copyright © American Heart Association, Inc. All rights reserved.
Algorithm for CM associated with encephalopathy.
Schwartz M L et al. Circulation. 1996;94:2021-2038
Copyright © American Heart Association, Inc. All rights reserved.
Algorithm for CM associated with neuromuscular disease.
Schwartz M L et al. Circulation. 1996;94:2021-2038
Copyright © American Heart Association, Inc. All rights reserved.
TACHYCARDIA ASSOCIATED CMO
• Presentation and Mx similar to Fulminant 3 pts
• Troponin I was normal
• Presenting EF was 15-25 %
• Ventricular tachycardia: 1
• Supraventricular 2
• Renal Failure 2/3
• Ventilation 2/3
• MSOD 2/3
HYPERTROPHIC CARDIOMYOPATHY - MASQUERADING
• POMPE’S DISEASE IN A NEWBOR OR AN INFANT
• GLYCOGEN DEPOSITED IN THE MUSCLE
• RESULTS IN HYPERTROPHIC MUSCLE THICKENING AND FINALLY DECREASED FUNCTION
• REVERSIBLE IN EARLY STAGES THEN IRREVERSIBLE WITH ENZYME THERAPY
NON GENETIC CAUSES OF CARDIOMYOPATHY
• Cardiovascular conditions
• Atherosclerotic coronary artery disease
• Kawasaki disease
• Hypertension
• Dysrhythmia
• Congenital heart defect
• Chronic alteration of circulatory volume
• Cardiac transplantation
• Major cardiac surgery or invasive cardiothoracic procedure within 1 month
• Obesity
• Infection
• Toxin or drug
• Radiation
• Immunologic disease
• Connective tissue disease
• Endocrine disease, including disease in an infant of a diabetic mother
• Nutritional deficiency
• Granulomatous disease
• Malignancy
Single gene or gene pair defect (mendelian disorder)Autosomal dominant inheritanceNoonan syndrome (H)78,79Cardio-facio-cutaneous syndrome (H)80LEOPARD syndrome/lentiginosis/multiple lentigines (H)81,82Neurofibromatosis (H)83Beckwith-Wiedemann syndrome (H)84-86Telecanthus, multiple congenital anomalies (H)87Deaf-mutism (H)88Rubinstein-Taybi syndrome (H)89Autosomal recessive inheritanceHypogonadism, multiple congenital anomalies, mental retardation (D)90Microcephaly, mental retardation (D)91Palmoplantar keratosis (D)92Total lipodystrophy, insulin resistance, leprechaunism (H)93Costello syndrome (H)94
TACHYCARDIA ASSOCIATED CARDIOMYOPATHY
• SVT FOR MORE THAN 48 HOURS IN NEWBORNS AND INFANTS RESULTS IN CARDIOMYOPATY
• CAN LAST UPTO 1 WEEK
• MAY PRESENT WITH CHF AND EF OF 5-25%
• TREATABLE CAUSE OF DILATED CARDIOMYOPATHY
• PHARMACOLOGICAL TREATMENT
• OR RADIOFREQUENCY ABLATION
• SINCE CHILDREN WITH LOW EF ARE ALREADY TACHYCARDIC, THE SVT MAY BE MISSED IN THESE PATIENTS
NON COMPACTION
NON COMPACTION
RESTRICTIVE CARDIOMYOPTAHY
DILATED CARDIOMYOPATHY
HYPERTROPHIC CARDIOMYOPATHY: POMPES DISEASE
ECHO
LONGTERM OUTCOME
OUTCOME