CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to...

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CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS VIKAS KOHLI MD FAAP FACC PEDIATRIC CARDIOLOGY & CARDIAC SURGERY UNIT INDRAPRASTHA APOLLO HOSPITAL

Transcript of CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to...

Page 1: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS

VIKAS KOHLI MD FAAP FACCPEDIATRIC CARDIOLOGY &

CARDIAC SURGERY UNITINDRAPRASTHA APOLLO HOSPITAL

Page 2: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

CLASSIFICATION OF CHD

• ACYANOTIC• Increased PBF

– ATRIAL: ASD– VENTR: VSD– ARTERIAL: PDA– COMBINED:

VSD+PDA

• Normal PBF– Pulm or Aortic

Stenosis

• CYANOTIC• Decreased Flow

– TOF– Pulm Atresia

• Increased Flow– TAPVD– TGA– Truncus– Tricuspid Atresia

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Cyanotic Vs Acyanotic ?

• Clinically: nail beds/lips/tongue blue• But if saturation between 85-93% the

human eye cannot detect cyanosis• So, the gold standard of detection of

cyanosis is PULSE OXIMETER• Infact the pulse-ox is called the 5TH

VITAL SIGN

Page 4: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

CLASSIFICATION OF CHD

• ACYANOTIC• Increased PBF

– ATRIAL: ASD– VENTR: VSD– ARTERIAL: PDA– COMBINED:

VSD+PDA

• Normal PBF– Pulm or Aortic

Stenosis

• CYANOTIC• Decreased Flow

– TOF– Pulm Atresia

• Increased Flow– TAPVD– TGA– Truncus– Tricuspid Atresia

PULSE OXIMETER

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NEXT QUESTION

INCREASED/DECREASED PBF

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CLASSIFICATION OF CHD

• ACYANOTIC• Increased PBF

– ATRIAL: ASD– VENTR: VSD– ARTERIAL: PDA– COMBINED:

VSD+PDA

• Normal PBF– Pulm or Aortic

Stenosis

• CYANOTIC• Decreased Flow

– TOF– Pulm Atresia

• Increased Flow– TAPVD– TGA– Truncus– Tricuspid Atresia

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HOW TO ASSESS PBF ?

• Symptoms of incr PBF– Inc RR, Retractions, Incr infections

– Sweating while feeding– SOB

– Failure to thrive– Harrisons sulcus

• An objective method of assessing the PBF ?

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OBJECTIVE ASSESMENT OF

PBF IS BY CHEST XRAY

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ASD SECUNDUM

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ASD

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AV CANAL

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AV CANAL

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AV CANAL

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VSD

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MODERATE VSD

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LARGE VSD LARGE SHUNT

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LARGE VSD

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PDA

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EISENMANGERS

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TOF

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TGA

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TGA

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TRUNCUS

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PULM ATRESIA VSD

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CLASSIFICATION OF CHD

• ACYANOTIC• Increased PBF

– ATRIAL: ASD– VENTR: VSD– ARTERIAL: PDA– COMBINED:

VSD+PDA

• Normal PBF– Pulm or Aortic

Stenosis

• CYANOTIC• Decreased Flow

– TOF– Pulm Atresia

• Increased Flow– TAPVD– TGA– Truncus– Tricuspid Atresia

Page 26: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

CLASSIFICATION OF CHD

• ACYANOTIC• Increased PBF

– ATRIAL: ASD– VENTR: VSD– ARTERIAL: PDA– COMBINED:

VSD+PDA

• Normal PBF– Pulm or Aortic

Stenosis

• CYANOTIC• Decreased Flow

– TOF– Pulm Atresia

• Increased Flow– TAPVD– TGA– Truncus– Tricuspid Atresia

Page 27: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

CLASSIFICATION OF CHD

• ACYANOTIC• Increased PBF

– ATRIAL: ASD– VENTR: VSD– ARTERIAL: PDA– COMBINED:

VSD+PDA

• Normal PBF– Pulm or Aortic

Stenosis

• CYANOTIC• Decreased Flow

– TOF– Pulm Atresia

• Increased Flow– TAPVD– TGA– Truncus– Tricuspid Atresia

Page 28: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

ATRIAL SEPTAL DEFECT: SECUNDUM

• CLOSE >2 YRS OR WHEN DIAGNOSED

• ELECTIVE

• DEVICE vs SURGERY

• BOTH HAVE EXCELLENT OUTCOME

• DECISION RE MODE OF CLOSURE BY INTERVENTIONALIST:SHLD ECHO HIMSELF

Page 29: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

ATRIAL SEPTAL DEFECT

• AFTER FIRST YR: ANNUAL CARDIAC EVALUATION

• PT F/U CLOSELY BY PEDIATRICAN

• NL Q OF LIFE; NL LIFE SPAN

• DEVICE: NO MRI LIMITATION

Page 30: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

TRANSCATHETER ASD CLOSURE

• RAPID RECOVERY• NO SCAR• SUCCESS > 98% OF SELECTED

CASES• SELECTION IS THE KEY• NO BLOOD TRANSFUSION• PTS WITH SURGERY HAD LOWER

IQ SCORES

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ADVANCES IN CATH TECHNIQUES:

USE OF INTRA-CARDIAC ECHO FOR DEVICE PLACEMENT

• IMPROVED TECHNIQUES• BETTER UNDERSTANDING OF ANATOMY

JACC NOVEMBER 2003

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VENTRICULAR SEPTAL DEFECT

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VSD WHEN TO OPERATE ?

• LARGE VSD’s: 3 MONTHS; By 6 MONTHS – IF WEIGHT GAIN NOT APPR– AND/OR IF PULM HTN DEVELOPING

• MODERATE VSD’S INDIVIDUALIZED

• SMALL VSDS –• AORTIC INSUFFICIENCY• INFECTIVE ENDOCARDITIS

Page 34: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

Interventional PM VSD CLOSURE

• AN EXPERIMENTAL METHOD

• LONGTERM EFFECTS NOT KNOWN

• HEART BLOCK: NEED OF PACEMAKER

• AORTIC INSUFFICIENCY NOT KNOW

• WOULD NOT RECOMMEND

Page 35: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

VENTRICULAR SEPTAL DEFECTS

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MUSCULAR VSD CLOSURE

• AN ESTAB METHOD

• PREFERRED METHOD

• LONGTERM EFFECTS: NONE

• FAR FROM CONDUCTION SYSYTEM

• AORTIC INSUFFICIENCY UNKNOW

Page 37: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit
Page 38: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

PATENT DUCTUS ARTERIOSUS

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PINK BABY W/RESP DISTRESS

• PREMATURE– THIS IS THE BABY WITH PDA-HAS

BEEN DOING WELL NOW W INC O2 REQUIREMNTS; OR WITH APNEA; NEC; POOR PERFUSION !

– DIAGNOSIS IS BY ECHO ONLY !

• START THERAPY AFTER ECHO ONLY *

Page 40: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

PREMATURE-PDA

• THERAPY

– INDOMETHACIN: IV OR ORAL

• ORAL OR RECTAL BRUFEN SYRUP

– ADVANTAGES Vs DISADVANTAGES

Page 41: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

PREMATURE-PDA

• THERAPY

– ONLY RECORDED DIFFERENCE IN THE PDA CLOSURE BETWEEN BRUFEN AND INDOMETHACIN IS THE RECURRENCE RATE

– BENEFIT: RENAL PERFUSION IS NOT DECREASED WITH BRUFEN

Page 42: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

BRUFEN/INDOMT-COCHRANE ANALYSIS-2003

• BRUFEN AND INDOMETHACIN: equally potent in closing PDA; trials to evaluate long-term neuro issues needed

• PROPHYLACTIC INDOMTH: no longtermbenefit in survival or outcome; only short term decrease in IVH

• PROLONGED Vs SHORT COURSE INDOMTH: dec rate of PDA re-opening; prolonged course-assoc w/dec IVH & renal imapirment

Page 43: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

PDA BEYOND NEONATAL PERIOD

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PDA-WHEN TO CLOSE

• ANY PDA THAT CAN BE AUSCULTATED NEEDS TO BE CLOSED

• ANY SYMPTOMAYTIC PDA NEEDS TO BE CLOSED

• SILENT PDA’S MAY/MAYNOT BE CLOSED IN POST NEONATAL PERIOD

Page 45: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

PDA-WHEN TO CLOSE

• POST 5 KGS ANY PDA CAN BE CLOSED

• VERY LARGE PDAS: BABY DOES NOT GAIN WEIGHT-SO NO POINT WAITING

• SMALL PDAS MX MEDICXALLY TILL ABOUT 5-6 KG WEIGHT

Page 46: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

TRANSCATHETER CLOSURE PDA

• SUCCESS RATE >99 % FOR COMPLETE CLOSURE

• NO SCAR• FAST RECOVERY• NO BLOOD TRANSFUSION• COILS OR DEVICES• SIZE NO LIMITATION• WEIGHT: SMALLEST 2.2 Kg in IPAH-NO COA• >100 cases by Speaker• J Interv Cardiol. 2001 Apr;14(2):247-54.

Page 47: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

TETRALOGY OF FALLOTS

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Embryology

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HYPERCYANOTIC SPELL

• MANAGEMENT:• DONOT MAKE THE CHILD CRY• SEDATE MORPHINE/PHENERGAN• HEPLOCK: IV FLUIDS BOLUS• BICARB• IV METOPROLOL 0.05 MG/KG VERY

SLOW IV OVER ½ HR

Page 51: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

SURGERY-WHEN ?

• HYPERCYANOTIC SPELL-EVEN ONE IS ENOUGH TO INDICATE SURGERY

• IF AN INFANT < 6 MO…DO A BT SHUNT

Page 52: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

Treatment -TOF

• Surgical– Palliative to improve the pulmonary

blood flow- Systemic to Pulmonary shunt

Page 53: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

NO SPELLS:SURGERY-WHEN ?

• IF SATS APPROACHING 75% PLAN FOR SURGERY

• SURGERY DECIDED ON BASIS OF– AGE/WT AND PULM A SIZE – IF ANATOMY FAVOURABLE: WT> 7.5 KG REPAIR

• IF WT> 8 KG & ANATOMY FAVOURABLE –COMPLETE REPAIR OR ELSE BT SHUNT

Page 54: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

TOF- Interventional Cardiology

• Balloon Dilatation of pulmonary valve in cyanotic children with indication of shunt– In a randomised study evaluating for

postponement of palliative surgery and growth of pulmonary arteries.

• Coil closure of collaterals

Page 55: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

CYANOTIC CHD OTHERS

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BLUE BABY..CARDIAC

• DUCTAL DEPENDING LESIONS:RT HEART– PULM ATRESIA– TOF SEVERE– PULMONARY STENOSIS, SEVERE

• MIXING LESIONS– TRANSPOSITION

• START PROSTIN IN ANY OF THESE

Page 57: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit
Page 58: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit
Page 59: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

CYANOTIC CHD

• FURTHER OPTIONS– SEPTOSTOMY: MIXING LESION

– STENTING THE DUCT: DUCT DEPENDENT– BT SHUNT

• MOST IMP OUTCOME FACTOR– ACCURATE ECHO

– EARLY TRANSPORT– AVOID INFECTIONS

Page 60: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit
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Page 67: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

TGA:WHEN & WHAT SURGERY

• ALL TGA’s DIAGNOSED IN NEWBORN PERIOD: ARTERIAL SWITCH OPERATION

• IDEALLY IN SECOND WEEK

• CAN BE PERFORMED UPTO 4 WEEKS

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ARTERIAL SWITCH

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OTHER LESIONS

• TAPVD: OPERATE AT DIAGNOSIS; DON’T DELAY

• TRUNCUS; OPERATE AT DIAGNOSIS: NO ADVANTAGE IN DELAYING

Page 70: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

COARCTATION

• TO BE TREATED WHEN DETECTED

• BALLOON AS PRIMARY MANAGEMENT GOOD OPTION EVEN IN THE NEWBORN:

• RECURRENCE 30%; IF NO ASSOC PROBLEMS

• POST-6 MO BALLOON IS PREFERRED

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COARCTATION

• IF DETECTED IN ADOLESCENT AGE STENTING IS MODALITY OF CHOICE

• BALLOONING ALONE MAY ALSO WORK: RECURRENCE

• SURGERY IS ALSO AN OPTION

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DIAGNOSING CARDIAC LESION IN THE CRITICALLY

ILL NEWBORN

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INDICATORS OF CARDIAC PROBLEM

PRIMARY

• Desaturation

• Shock

• Resp Distress

SECONDARY

• Murmur

• Cardiac Enlargement

• Peripheral Pulse Abn

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DESATURATION

• Causes of Desaturation:

– INTRAPULMONARY SHUNTING• COLLAPSE• PNEUMONIA• PPHN

– INTRACARDIAC SHUNTING• Cyanotic CHD

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DIFFERENTIAL DIAGNOSIS

• Cyanosis all over– Intracardiac Mixing: Single Ventricle– Intrapulmonary mixing: PPHN

• Differential Cyanosis– Upper Limb Blue: TGA+PDA– Lower Limb Blue: Duct Shunting R to L

with normally related GA’s

• Combination: PPHN– Intrapulmonary + PDA + PFO shunting

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THINK CARDIAC WHEN THINKING PULMONARY

ESPECIALLY IF ANY OF THE SECONDARY FACTORS PRESENT

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IN SHOCKY PATIENTS : ALWAYS R/O CARDIAC

ESPECIALLY IF SECONDARY SIGNS PRESENT

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WITH RESP DISTRESS IN NEONATES: HAVE A LOW THRESHOLD TO SUSPECT

CARDIAC LESIONS

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INDICATORS OF CARDIAC PROBLEM

PRIMARY

• DESATURATION

• SHOCK

• RESP DISTRESS

SECONDARY

• Murmur

• Cardiac Enlargement

• Peripheral Pulse Abn

Page 84: CONGENITAL HEART DISEASE: APPROACH TO DIAGNOSIS to chd.pdf · congenital heart disease: approach to diagnosis vikas kohli md faap facc pediatric cardiology & cardiac surgery unit

HOW TO REACH A DIAGNOSIS ?

• SUSPECT: KNOWLEDGE BASE REQUIRED

• DIAGNOSTIC MODALITY : ECHOCARDIOGRAM: Ready availability of reliable Bedside Pediatric/Neonatal Echo remains the single most important hurdle in the diagnosis of neonatal CHD

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SUSPECTING CARDIAC LESION BEFORE THEY GET

CRITICALLY ILL

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DIAGNOSING CHD IN THE NEWBORN

• Effectiveness of pulse oximetry screening for conge nital heart

disease in asymptomatic newborns. Pediatrics. 2003 Mar;111(3):451-5.

• The use of pulse oximetry to detect congenital hear t disease. J Pediatr. 2003 Mar;142(3):268-72

• Oxygen saturation as a screening test for critical congenital heart

disease: a preliminary study. Pediatr Cardiol. 2002 Jul-Aug;23(4):403-9.

• Early clinical screening of neonates for congenital heart defects: the cases we miss. Cardiol Young. 1999 Mar;9(2):169-74 : 25% diagnosed after neonatal discharge

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CONCLUSION

• PRE DISCHARGE 4 EXTREMITY BP CHECK IS STANDARD OF PRACTISE TO R/O COA

• CY CHD CAN BEST BE EVALUATED PRIOR TO DISCHARGE FROM HOSPITAL BY CHECKING SATS IN THE LEG

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TAKE HOME MESSAGE

• EARLY REFERRAL

• INVOLVE PEDIATRIC CARDIOLOGIST IMMEDIATELY:DON’T WAIT

• RIGHT DIAGNOSIS WITH A GOOD ECHO• KEEP PROSTIN AVAILABLE

• DON’T HESITATE TO CALL IF IN DOUBT

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PEDS CARDIO TRAINING MODULE

• 4 HOUR SESSION TO RE-TRAIN PEDS PRACTITIONERS IN:

• AUSCULTATION

• READING ECG• CARDIAC XRAYS• UNDERSTANDING ECHO REPORT

• WHEN TO OPERATE• NEONATAL CARDIO

• FIRST SESSION MARCH 4TH: APOLLO: CONTACT 989136223