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