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 FACCPEDIATRIC CARDIOLOGY &
CARDIAC SURGERY UNITINDRAPRASTHA APOLLO HOSPITAL
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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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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
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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
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
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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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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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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
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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
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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
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Interventional PM VSD CLOSURE
• AN EXPERIMENTAL METHOD
• LONGTERM EFFECTS NOT KNOWN
• HEART BLOCK: NEED OF PACEMAKER
• AORTIC INSUFFICIENCY NOT KNOW
• WOULD NOT RECOMMEND
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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
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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 *
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PREMATURE-PDA
• THERAPY
– INDOMETHACIN: IV OR ORAL
• ORAL OR RECTAL BRUFEN SYRUP
– ADVANTAGES Vs DISADVANTAGES
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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
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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
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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
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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
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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.
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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
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SURGERY-WHEN ?
• HYPERCYANOTIC SPELL-EVEN ONE IS ENOUGH TO INDICATE SURGERY
• IF AN INFANT < 6 MO…DO A BT SHUNT
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Treatment -TOF
• Surgical– Palliative to improve the pulmonary
blood flow- Systemic to Pulmonary shunt
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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
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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
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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
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CYANOTIC CHD
• FURTHER OPTIONS– SEPTOSTOMY: MIXING LESION
– STENTING THE DUCT: DUCT DEPENDENT– BT SHUNT
• MOST IMP OUTCOME FACTOR– ACCURATE ECHO
– EARLY TRANSPORT– AVOID INFECTIONS
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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
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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
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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