Approach to patients with congenital heart disease

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Approach to patients with congenital heart disease

• Dr.Mazen Naghawi Pediatric Cardiologist
• Albasheer hospital
• Pediatric department

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Congenital heart disease

• congenital cardiac malformations resulting
  from defective embryonic development
  without obvious cause.
• Between 3-8 weeks gestation all the fetal
  heart structures are formed organogenesis

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Incidence

• 8-10/1000 live births in Jordan approximately
  2000/ year
• Incidence is more in -
• a-Premature
• b-abortions
• c-still births
• Incidence increased for siblings.
• 2-6,10-12,25

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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.

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Adverse maternal conditions (enviromential)

• -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.

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Syndrome complexes

• -VACTREL syndrome Vertebral,Anorectal,Ca
  rdiac(VSD,TOF and others),tracheal,Renal,Oesoph
  ageal and Limb abnormalities.
• -CHARGE syndrome
• Coloboma,Heart(VSD,TOF,A-V canal), Atresia
  choanal,Retardation,Gential,Ear abnormalties.
• -Kartagener syndrome Dextrocardia
• -TAR syndrome

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Majority of cases of the congenital heart
diseases are unknown cause
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Classification

• C.H.D with little or no cyanosis acyanotic
• a-With Lt. ventricular enlargement
• ex.- PDA8 ,CO-Ao 5
• - aortic stenosis 7
• b-With Rt. Ventricular enlargement
• ex.-ASD10 ,PS6,MS
• c-With both ventricular enlargement
• ex.- VSD 25

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Common acyanotic lesions

• Ventricular septal defects
• Atrial septal defects
• Atrio-ventricular septal defects
• Patent ductus arteriosus
• Truncus arteriosus
• Aortic stenosis
• Mitral stenosis/incompetence
• Coarctation of aorta
• Tricuspid regurgitation

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• C.H.D with cyanosis mainly Rt. Lt. shunt

• a-With decrease pulmonary vascularity
• ex.-TOF5 , Tricuspid
  Artesia1-3
• - PS with or without VSD5
• b-With increase pulmonary vascularity
• ex.-TGA 5 ,Truncus
  arteriosus2
• -TAPVR1

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Common Cyanotic Lesions

• Decreased flow
• 1. Tetralogy of Fallot
• 2. Tricuspid Atresia
• 3. Severe Pulmonic Stenosis
• 4. Ebsteins anamoly
• Increased Flow
• 5. Transposition of great vessles
• 6. VSD with pulmonary atresia

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Cont-Common Lesions producing cyanosis

• 7. Truncus Arteriosus
• 8. Hypoplastic left heart
• 9. Single ventricle
• 10. TAPVR with infradiaphragmatic obstruction

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Prevalence

• 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.
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Diagnosis

• Early diagnosis of C.H.D mean better results.
• 40 of C.H.D diagnosed at 1st w of life.
• 50-60 diagnosed at 1st two months .
• Others are usually later during routine medical
  examination.
• Diagnosis depend on good clinical history good
  medical examination and investigations.

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History

• Age of the patient.
• Ask for
• -Feeding difficulties
• -Vomiting
• -Lethargy
• -Increased perspiration
• -Rapid respiration
• -Hypoactivity
• -F.T.T
• All may be manifestation of congestive heart
  failure.

D.D
Sepsis Metabolic disorders Hypothermia
IVH Others
Neonate
Infant
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Older child

• -History of dysnea on exertion.
• -Shortness of breathing.
• -Orthopnea.
• -Lower limbs swelling
• -Palpitation.
• -Convulsion.
• -All may be manifest of congestive heart
  failure.

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Physical examination

• Inspection look for
• -Cyanosis
• -Digital clubbing
• -Tachypnea
• -Prominence of the precordium
  (cardiomegaly,Rt.heart enlargement )
• -Jugular veins engorgement older children
  
• -Any associated defects or findings (down
  syndrome, Digorge syndromeetc)
• - Focal neurological lesion.
• Palpation
• -Pulses (rate, rythem,volume,peripherial
  pulses ,brachio-femoral delay)
• -Cardiac impulses.
• -Thrill.
• -Hyper dynamic precordium.
• -Hepato-splenomegaly
• -Sacral edema(neonate, and infancy) ,Lower
  limb edema in older children

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• 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
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Blood pressure

• Methods sphingnonaometer(different cuffs)
• -Flush method
• -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.

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If we suspect C.H.D Investigation

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

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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.

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Ventricular Defect

• Small VSD
• Asymptomatic
• A loud, harsh, or blowing holosystolic murmur.
• Large VSD
• dyspnea, feeding difficulties, poor growth,
  profuse perspiration, recurrent pulmonary
  infections, and cardiac failure in early infancy.
  

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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.
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Ventricular Septal defects

• 3050 of small defects close spontaneously, most
  frequently during the 1st 2 yr of life.
• Small muscular VSDs are more likely to close (up
  to 80) than membranous VSDs are (up to 35).
• infants with large defects have repeated episodes
  of respiratory infection and heart failure
  despite optimal medical management.
• Surgical repair prior to development of an
  irreversible increase in pulmonary vascular
  resistance (usually prior to the patient's second
  birthday).

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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.

Secundum
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Atrial Septal Defectsprimum

• Situated in the lower portion of the atrial
  septum and 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
• C/F similar to that of an ostium secundum ASD

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Atrial Septal Defect

• Enlargement of the right ventricle
• Enlargement of atrium
• Large pulmonary artery
• increased pulmonary vascularity is.

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Atrial Septal Defects

• Secundum ASDs are well tolerated during
  childhood.
• Antibiotic prophylaxis for isolated secundum ASDs
  is not recommended.
• Surgery or transcatheter device closure is
  advised for all symptomatic patients and also for
  asymptomatic patients with a QpQs ratio of at
  least 21.
• Ostium primum defects are approached surgically

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Patent Ductus Arteriosus

• Small defect no symptoms.
• Large defect
• Wide pulse pressure
• Enlarged heart
• Thrill in L second IS
• Continuous murmur
• X-ray prominent pulmonary artery with increased
  vascular markings.

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Primary Pulmonary Hypertension

• Prominent pulmonary artery.
• Prominent right ventricle
• Prominent vascularity in the hilar areas
• Decreased vascualr marking in the periphery.
• No treatment

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Cardiac disease with normal/decreased vasculature

• Tetralogy of Fallot
• Pulmonary atresia
• Tricuspid atresia
• Endocardial fibroelastosis
• Aberrant left coronary artery
• Cystic medial necrosis
• IODM septal hypertrophy

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Tetralogy of Fallot

• Ventricular septal defect
• Pulmonic stenosis
• Overriding aorta
• Right ventricular hypertrophy

Cyanotic
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Cardiac disease with increased vasculature

• Atrioventricular septal defects
• Congestive cardiac failure
• Transposition of great arteries with VSD
• Total anomalous pulmonary venous drainage
• Truncus arteriosus
• Single ventricle without pulmonary stenosis
• Hypoplastic left heart syndrome

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Congestive Cardiac Failure

• Enlarged heart
• Plethoric lung fields specially at bases

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Boot shaped heart in pt. with TOF
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Snow man or figure8 in pt. with TAPVR
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Cardiomegaly
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Cardiomegaly with pulmonary edema
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Ribs notching in pt. with Co-Ao
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Situs inverses
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AcrocyanosisBluish discoloration of the hands
and feet commonly seen in newborns
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Suffused face due to cold around neck and not
central cyanosis
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2D echo Transthoraic echocardiography
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3D Echocardiography
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Transoseophageal echocardiography
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Fetal echocardiography
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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.

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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 , some need IVF
  as PDA and PS , OTHERS need IVF when ever there
  are pulmonary congestion or volume overload).
• 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.

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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

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3-Rx of cardiac arrhythmias

• Digoxin (be aware of toxicity nausia
  ,vomiting,AV block, K, ca).
• Quinidine( hemolytic anemia.SLE,perodic
  paralysis)
• Procainamide(agranulocytosis,ve coombs hemolytic
  anemia)
• Lidocaine (confusion, convulsion,respiratory
  failure,AV block).
• Propranolol(bradycardia,bronchspasm,hypotensionet
  c).
• Verapamil(bradycardia,PR interval
  prolongation,CHF).
• Adenosine(chest pain,flushing,bronchospasm,bradyca
  rdia)

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4-Rx of the cyanotic spells

• Try to calm the patient .
• Knee chest position,
• O2
• Propranolol IV.
• Morphine s.c
• NaHCO3 iv
• Increase IV fluid.

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5-Prophylaxis against infective endocarditis

• By oral or parental antibiotics.
• Given in case of dental procedures, urinary and
  GIT .
• Instrumental proctoscopy,cystescopy.
• Now a days lot of controversy about who should
  take the prophylactic antibiotics.

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New guidelines regarding antibiotics to prevent
infective endocarditis

• The American Heart Association recently updated
  its guidelines regarding which patients should
  take a precautionary antibiotic to prevent
  infective endocarditis (IE) before a trip to the
  dentist.
• The guidelines, published in Circulation Journal
  of the American Heart Association, are based on a
  growing body of scientific evidence that shows
  that, for most people, the risks of taking
  prophylaxis antibiotics for certain procedures
  outweigh the benefits. These guidelines represent
  a major change in philosophy.
• The new guidelines show taking preventive
  antibiotics is not necessary for most people and,
  in fact, might create more harm than good.
  Unnecessary use of antibiotics could cause
  allergic reactions and dangerous antibiotic
  resistance.
• Only the people at greatest risk of bad outcomes
  from infective endocarditis  an infection of the
  heart's inner lining or the heart valves  should
  receive short-term preventive antibiotics before
  common, routine dental and medical procedures.

http//www.americanheart.org/presenter.jhtml?ident
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• Patients at the greatest danger of bad outcomes
  from IE and for whom preventive antibiotics are
  worth the risks include those with
• artificial heart valves
• a history of having had IE
• certain specific, serious congenital (present
  from birth) heart conditions, including-
  unrepaired or incompletely repaired cyanotic
  congenital heart disease, including those with
  palliative shunts and conduits- a completely
  repaired congenital heart defect with prosthetic
  material or device, whether placed by surgery or
  by catheter interventions, during the first six
  months after the procedure-any repaired
  congenital heart defect with residual defect at
  the site or adjacent to the site of a prosthetic
  patch or prosthetic device
• a cardiac transplant which develops a problem in
  a heart valve

http//www.americanheart.org/presenter.jhtml?ident
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Dont forget prostglandine PGE2, AND
indomethacine for selective cases of CHD
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Preventive measures

• Avoid pregnancy above age of 38 year.
• Avoid smoking.
• Avoid alcohol.
• Avoid exposure to radiation.
• Avoid drugs intake before medical consultation.
• Vaccination of all childbearing women for
  rubella.

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Innocent murmur

• It is functional,normal,insignificent.
• Patient is asymptomatic.
• Murmur heard on routine cardiac examination
  without pathological causes.
• Main age between 3-7 years.
• Incidence up to 30 especially child with
  fever,infections,anxiety,and with cardiac
  output, the incidence is decreased with age.

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Characteristics of the innocent murmur

• Mild murmur usually grade one.
• Brief in duration.
• Changing with position.
• No radiation.
• It is systolic,musical,shortand ejectional.
• Best heard over Lt. lower sternal and midsternal
  border.

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• Thanks