Approach to congenital heart disease

1
(No Transcript)
2
Approach to chd

• Dr. Raheel Ahmed Shaikh
• Paeds unit -1
• CMCH,larkana

3
Prevalence

• Acquired
• Kawasaki disease
• Rheumatic
• Tubercular
• Collagen

• Congenital
• Cyanotic 22
• Acyanotic 68
• VSD 25
• ASD 6
• PDA 6
• TOF 5
• PS 5
• AS 5

Ceylon Med J 2001 Sep 46 (3) 96-8 Indian J
Pediatr. 2001 Aug68 (8)757-7 Nelsons Textbook
of pediatrics 17 ed.
4
Etiology

• Multifactorial inheritance pattern mostly
• Chromosomal abnormality (5-10).
• -Trisomy 21 (50) gt A-V canal,VSD,ASD,
  others.
• -Trisomy 18 (80)gt VSD,ASD,others.
• -Trisomy 13 (40)gt VSD,ASD,PDA,others.
• -Turner syndrome (xo)gtBicuspid aortic valve
  and co-ao
• -others.
• Maternal infections gtRubellaPDA,PS
• Maternal diseasesgt PKU-VSD,ASD
• DMleft septal
  hypertrophy
• Drugsgtfetal hydntoin syndrome- VSD
• Valproate effect-co ao left
  heart hypoplasia
  
• Fetal alcohol syndromegt VSD,ASD,CO-AO.
• Advance maternal age.
• Majority of cases of the congenital heart
  diseases are unknown

5
History

• Maternal history of medication, drugs, alcohol
  abuse, excessive smoking.
• Prenatal history infection
• Family history of CHD, hereditary, chromosomal

6
Symptoms
Difficult Breathing Chest indrawing Fast
breathing
Difficult Feeding Sweating during feeding
Syncope Exercise intolerence Easy fatigability
Poor growth Poor weight gain
Frequent respiratory infections
Seizure Focal neurological lesion
7
Stridor
Cynosis Bluish spells
8
Physical Examination
Appearance Pale, Dusky, Polycythemic,
Syndromic Presence of Cyanosis,
Clubbing,Edema Tachypnea, Respiratory
distress Weight, Height for physical
development Skeletal abnormalities
Polydactyly, others Pulse Tachycardia,
Arrhythmia, Volume, Palpability BP All 4 limb
BP in complex CHD JVP - Elevated in Tricuspid
Atresia, Eisenmenger physiology -
Normal in TOF Abd - Sidedness of liver/spleen
palpation of Apical Impulse
to rule out Dextrocardia -
Hepatomegaly
9
Blood pressure

• Methods sphingnonaometer(different cuffs)
• -Palpation method
• -Doppler method
• Wide pulse pressure
• -Aortic insufficiency
• -A-V communication
• -PDA
• Low blood pressure(H.F, pericardial
  tamponade,cardiomyopathy).
• Difference in BP between upper and lower
  extremities Co-ao.

10
What is cyanosis?

• Cyanosis is a bluish discoloration of skin and
  mucus membrane that results when the absolute
  level of reduced hemoglobin in the capillary bed
  exceeds 3 g/dL.

11
Cyanosis is it a cardiac cause or lung cause

• Hyperoxia test
• Neonates with cyanotic congenital heart disease
  usually do not have significantly raised arterial
  Pao2 during administration of 100 oxygen.

12
Physical examination

• Inspection look for
• -Prominence of the precordium (cardiomegaly,Rt.he
  art enlargement )
• -Jugular veins engorgement older children
  
• -Any associated defects or findings (down
  syndrome, Digorge syndromeetc)
• Palpation
• -Pulses (rate, rythem,volume,peripherial
  pulses ,radio-femoral delay)
• -Cardiac impulses.
• -Thrill.
• -Hyper dynamic precordium/heave.
• -Hepato-splenomegaly

13

• Auscultation
• a-First heart sound (A-V valves closure)
• Best heard at the Lt. lower sternal
  border or apex
• b-Second heart sound (semilunar valve
  closure)
• Best heard on the 1st and 2nd I.C.S
  , normally there
  
  
  is normal splitting of the 2nd heart
  sound ,
• -Single Aortic atresia,Pulmonary
  Artesia
• -Fixed splitting ASD,PS,Rt.B.B.B
• c-Murmurs

Systolic
Diastolic
Continous
14
(No Transcript)
15
If we suspect C.H.D Investigation

• CBC---- polycythemia, anemia.etc
• CXR----heart size and shape
• ECG---HR,axis ,rythm
• LVH,RVH,BVH,BBB.
• Echocardiography
• MRI
• Cardiac catheterization

16
Congenital Heart Disease

17
(No Transcript)
18
Common Acyanotic CHD

• Left to right lesions
• Ventricular septal defects
• Atrial septal defects
• Atrio-ventricular septal defects
• Patent ductus arteriosus
• Aortopulmonary window defect
• Coronary artery fistula
• Obstructed lesion
• Pulmonary stenosis
• Aortic stenosis
• Coarctation of aorta
• Congenital Mitral stenosis
• Pulmonary venous HTN
• Regurgitate lesions
• Congenital Mitral incompetence
• Mitral valve prolapse
• Tricuspid regurgitation

19
Common Cyanotic Lesions

• Decreased Pulmonary blood flow
• Tetralogy of Fallot
• Tricuspid Atresia
• Severe Pulmonic Stenosis
• Pulmonary atresia
• Ebsteins anamoly
• Double Outlet Right ventricle
• Increased Pulmonary blood Flow
• Transposition of great vessles
• VSD with pulmonary atresia.
• Truncus Arteriosus
• Hypoplastic left heart
• Single ventricle
• TAPVR

20
Ventricular Defect

• Subtype
• Small (lt0.5cm2)
• Moderate (0.5-1 cm2)
• Large (gt1cm2)
• Perimembraneous
• Muscular
• Supracristal (superior to crista
  supraventricularis)

80
21
Ventricular Septal Defect

• Small VSD (lt0.5cm2)
• Asymptomatic
• A loud, harsh, or blowing holosystolic murmur at
  LSE
• Large VSD(gt1cm2)
• dyspnea, feeding difficulties, poor growth,
  profuse perspiration, recurrent pulmonary
  infections, and cardiac failure in early infancy.
  
• Apical thrust, systolic thrill at LSE
• Pansystolic murmur(less harsh more blowing) at
  LSE

22
Ventricular Septal Defect (VSD)
Small VSDs, the chest radiograph is usually
normal
Large VSD The presence of right ventricular
hypertrophy, olegeimic lung fields (pulmonary
hypertension or an associated pulmonic stenosis),
gross cardiomegaly with prominence of both
ventricles, the left atrium.
23
Ventricular Septal defects management

• 3050 of small defects close spontaneously, most
  frequently during the 1st 2 yr of life. Vast
  majority will close up to 4yaers.
• Small muscular VSDs are more likely to close (up
  to 80) than membranous VSDs are (up to 35).
• Medical management treat heart failure if
  present.
• Surgical repair prior to development of an
  irreversible increase in pulmonary vascular
  resistance (usually prior to the patient's second
  birthday), chronic volume overload and heart
  failure.

24
Indications of surgery

• Failure to controle CCF
• Failure to thrive
• Supracristal VSD
• Associated Pulmonary stenosis
• Development of AR
• 6-12 month child with rising P.HTN
• gt2years child PBF twice of Systemic BF

25
Atrial Septal Defects

• Subtypes
• Sinus venosus (high)
• Ostium secundum (mid portion) most common
• Ostium primum (low )

26
Atrial Septal Defects secundum

• Most common form of ASD (fossa ovalis)
• In large defects, a considerable shunt of
  oxygenated blood flows from the left to the right
  atrium.
• Mostly asymptomatic
• The 2nd heart sound is characteristically widely
  split and fixed.
• Soft systolic murmur at upper LSE

Secundum
27
Atrial Septal Defectsprimum

• Situated in the lower portion of the atrial
  septum
• overlies the mitral and tricuspid valves.
• In most instances, a cleft in the anterior
  leaflet of the mitral valve is also noted.
• Combination of a left-to-right shunt across the
  atrial defect and mitral insufficiency
• Apical pansystolic murmur(AVr)
• The 2nd heart sound is split and fixed

28
Atrial Septal Defect

• X-ray
• Enlargement of the right ventricle
• Enlargement of atrium
• Large pulmonary artery
• increased pulmonary vascularity
• ECG supeior axis in primum, RVH, partial RBBB

29
Atrial Septal Defects

• Secundum ASDs are well tolerated during
  childhood.
• Antibiotic prophylaxis for isolated secundum ASDs
  is not recommended.(except if MR present)
• Surgery or transcatheter device closure is
  advised for all symptomatic patients and also for
  asymptomatic patients with a shunt ratio of at
  least 21. or those with RVH
• Intervention after 1 year before school entery.
• Ostium primum defects are approached surgically

30
Patent Ductus Arteriosus
31
Patent Ductus Arteriosus

• Connects pulmonary artery and descending aorta.
• Normally closed shortly after birth.
• Common in VLBW with pulmonary diseases and in
  congenital rubella.
• FM 21
• Spontaneously close in premature
• PDA persisting in term beyond 1st few weeks will
  rarely close spontaneously

32
Patent Ductus Arteriosus

• Small defect
• no symptoms.
• Pulses are normal
• Large defect
• Breathlessness while feeding
• Slow growth
• Repeated Lower RTI
• Wide pulse pressure
• Enlarged heart
• Apical heaving
• Thrill in L second IS
• Continuous murmur (machinary)in 2nd LICS
• X-ray prominent pulmonary artery with increased
  vascular markings, Left heart enlarge

33
Patent Ductus Arteriosus

• Medical Management
• Medical closure in preterm tried with
  indomethacin (0.2mg/kg/dose iv for 3 doses 8-12hr
  apart) or brufen (ibuprofen).
• Start therapy after echo only.
• Surgical Management
• Ligation and division of ductus is treatment of
  choice
• In asymptomic patiants before 1year
• P.HTN is not contraindication.

34
Coarctation of the Aorta 6

• In boys more than in girls
• Almos always juxtaductal in position.
• Weak femoral pulses, Radio femoral delay,
  hypotention in lower parts of body.
• High BP in upper body part in the arm 20mmhg more
  than leg, headache, vertigo, epistaxis.
• Murmur at left interscapular area in back.
• Treatment
• Digoxin diuretic PGE1 infusion.
• Surgical repair at the age of 2yrs

35
pulmonary stenosis 10

• Narrowing in pulmonary valve
• RT side heart failure
• Ejection Systolic murmur at Left 2nd ICS rediate
  to back, S2 widely split
• ECG Echo RT side hypertrophy
• Treatment
• Balloon valvuloplasty
• Surgical repair

36
Aortic stenosis

• 7 of total cases of CHD
• Increased pressure in the LF side of the heart
  (LV hypertrophy)
• Easy fatigability, exertional chest pain, syncope
• Ejection systolic murmur at right _at_nd ICS rediate
  to neck, high HR.
• Treatment..
• Beta-blocker or ca channel blocker to decreased
  hypertrophy
• Balloon valvoplasty
• Surgical repair

37
Cyanotic Congenital Heart Disease
- Cardinal Clinical features -
38
ONSET OF CYANOSIS
39
(No Transcript)
40
CLINICAL DIFFERENCES BETWEEN CYANOTIC HD WITH ?
AND ? PBF
DECREASED PBF (eg. TOF) INCREASED PBF (eg. TGA, TAPVC)
Presentation at Any age Neonate / Infant
Appearance Comfortable Sick, Lethargic, Irritable
Cyanosis Mild - Severe Mild (except TGA with intact IVS)
Squatting /Cyanotic spells Common Uncommon
Feeding difficulty / ?Sweating Absent Present
Failure to thrive Absent Present
Weight Gain Normal Suboptimal
Recurrent LRI No Yes
Tachypnea Absent Present
Heart size Normal Cardiomegaly
CHF, Tachycardia, S3, S4 Absent Present
Thrill Maybe Present Absent
Murmur Systolic Soft, Diastolic
CXR Ischemic Lungs, No Cardiomegaly Plethoric Lungs, Cardiomegaly
41
Tetralogy of Fallot

• Most common cynotic
• Ventricular septal defect
• Pulmonic stenosis
• Overriding aorta
• Right ventricular hypertrophy

Cyanotic
42
Risk factors

• Environmental factors
• maternal diabetes threefold increased risk,
  retinoic acids, maternal phenylketonuria (PKU),
  and trimethadione
• Genetic cause heterogeneous

43
Clinical Presentation

• Clinical presentation is directly related to the
  degree of pulmonary stenosis.
• Severe stenosis results in immediate cyanosis
  following birth.
• Mild stenosis will not present until later.
• Growth is retarded insufficient oxygen and
  nutrients
• SOB on exertion ? rest
• Digital clubbing
• Paroxysmal hypercynotic attacks (tet spells)
• Left parasternal heave
• Systolic thrill (50) at left PSE 3 and 4 ICS
• S2 is often single
• Harsh ejection systolic murmur at left PSE 3rd
  ICS

44
Tet Spell

• Tet spells at 2-3yo, child becomes cyanotic,
  restless, gasping respiration,may experience
  syncope
• More frequently in morning upon awakening or
  after vigorous cry.
• Children assume squatting position
• During spell, dec in intensity or disappearance
  of systolic murmur

45
Exams and Tests

• CBC
• - ? hematocrit
• ECG
• -RVH, RAD
• CXR
• -boot shaped heart, right sided aortic arch
• Echocardiogram
• -VSD, PS, RVH

46
Tetralogy of Fallot
Apex is lifted, concavity in pumonary segment,
oligemic lung field. Boot shaped Heart
47
Treatment

• Severe TOF with worsening cynosis in early
  neonatal period require prostaglandins E
  infusion and surgery (modified Blalock-taussing
  shunt)
• Corrective surgery carried out from 3 months to
  2year depending upon expertise availablity

Blalock-Taussig shunt
48
POTTS SHUNT WATERSTON
SHUNT
49
Treatment of the cyanotic spells

• Try to calm the patient .
• Knee chest position,
• O2
• Propranolol(0.1-0.2mg/kg slow IV).
• Morphine s.c
• NaHCO3 iv
• Increase IV fluid.
• Prevented by oral Propranolol (1mg/kg every 4hr)

50
(No Transcript)
51
Transposition Of great arteries

• Most serious cynotic lesion,
• Seen in neoborn period (5)
• More common in infants of diabetic mothers and in
  males.
• Survive when ASD, PDA, or VSD

52
Clinical Features

• TGA with intact ventricular septum
• Cynosis and tachypnea within 1st hours of life.
• Single S2, no murmur
• PGE1 (o.o5-o.2ug/kg/min infusion)is immediately
  started to maintain patency of DA.
• CCF is less common
• TGA with VSD
• Mild cynosis recognised 1st month of life.
• Murmur is pansystolic
• Many Neonates are large 4kg at birth then growth
  retardation occurs.
• They need anti-CCF measures

53
Transposition Of great arteries

• Slight cardiomegaly, narrow base
• Egg shaped Heart

54
Management

• Corrective surgery by age of 2 weeks
• Procedures
• Rashkind or ballon atrial septoplasty
• Mastured procedure
• Total repair Arterial swich technique.

55
Tricuspid Atresia
56
Clinical Features

• Progressive Cynosis
• Poor feeding
• Tachypnea over the first 2 weeks
• Holosystolic murmur due to VSD
• Single S2
• Left axis Deviation and left VH on ECG
  characteristics.
• Normal heart size

57
(No Transcript)
58
(No Transcript)
59
(No Transcript)
60
Clinical Findings
61
Treatment

• Medical management
• Anti-CCF measure
• Surgical Repair
• VSD closure and placement of conduit between
  right ventricle and Pulmonary artries.

62
Total Pulmonary venous return

• 2
• Pulmonary vein return to the right atrium or the
  superior vena cava instead of the left atrium
• Atrial level communication is required.
• Without obstruction Hyperactive RV impulse with
  wide split S2
• Systolic ejection murmur at Left USB
• Mid diastolic murmur at left LSB
• With obstruction signs of right sided heart
  failure, no murmur, no change in S2
• Treatment
• Give PGE, cath, and surgical treatment

63
Hypoplastic left Heart Syndrome

• Most common cause of death from cardiac side in
  1st month
• Failure of development of MV,Av,or arch.
• Infants may appear healthy at birth, but signs
  of HLHS soon become apparent after the ductus
  arteriosus closes.
• Cyanosis minimal, weak pulses, Cold extremities,
  greyish colour(low cardiac output)
• S2 single and loud no heart murmur
• Right ventricle Hypertrophy
• Treatment
• Give PGE,and surgical treatment

64
Treatment of C.H.D

• This is depend on the type of the C.H.D.
• No treatment (observationreassurance)
• Medical treatment(antifailure,antiarythmaic..etc).
  
• Surgical treatment (palliative or curative).
• Cardiac transplant or lung heart transplant.

65
1-General measures

• Special positions. (semisiting ,knee chest
  position (
• O2 (most patients need O2 and other need little
  O2).
• IVF(again depend on type of CHD ).
• Salt restriction.
• Exercise restriction.
• Rx of anemia.
• Rx of polycythemia. PCVgt65
• Avoidances of dehydration mainly polycythemic
  patients.
• Avoidances of high altitude.
• Avoidance of contraceptive thrombosishypertensio
  n.
• Correction of acidosis.
• Correction of electrolyte disturbances .
• Careful monitoring during surgery.

66
2-Rx of congestive heart failure

• Digoxin Digitalization 0.04mg/kg
• Maintenance 0.01mg/kg
• Loop diuretics frusemide 1-2 mg/kg/day.
• Potassium sparing diuretics spironlactone
• After load reducing agents
• eg. Captopril 0.5-6mg /kg/24 hours.
• Positive intropic agents .dopamine and
  dobutamine

67
(No Transcript)
68
(No Transcript)