congenital

							Dr Fasola O.A.

CONGENITAL HEART DISEASES
OUTLINE

 Introduction
 Classification

 Distribution

 Clinical features

 Diagnosis

 treatment
INTRODUCTION

 Present in 1% of neonates
 Accounts for 50% of deaths caused by all types
  of congenital defects
 A knowledge of cardiac embryology is
  necessary
CLASSIFICATION
CYANOTIC HEART DEFECTS

1)Those with reduced pulmonary blood flow
   Tetralogy of fallot
   Pulm atresia with ventricular septal defect

   Pulm atresia with no septal defect

   Tricuspid atresia

   Ebstein anomaly

   Pulmonary stenosis
CYANOTIC HEART DEFECTS
2)Those with increased pulm. blood flow
   Transposition  of the great arteries
   Total anomalous venuos drainage
   Cor triatriatum

3)Complex ‘mixing defects’
   Double outlet right ventricle
   Univentricular heart
   Truncus arteriosus
   Hypoplastic left heart syndrome
ACYANOTIC HEART DISEASE
   Obstructive defects
     Stenosis of left ventricular outflow tract
     Aortic stenosis
     Aortic arch anomalies
     Coarctation of aorta
   Shunt defects
     Patent ductus arteriosus
     Atrial septal defect
     Ventricular septal defect
     Atrio-ventricular septal defect (endo cushion defect)
     Aorto-pulmonary window
     Single atrium
DISTRIBUTION

Ventricular septal defect         30
Atrial septal defect              12
Patent ductus arteriosus          10
Pulmonic stenosis                 10
Coarctation of aorta              10
Tetralogy of fallot               10
Aortic stenosis                   8
Transposition of great arteries   8
others                            2
General Clinical Features
a) In Infants
* Dyspnoea which may be paroxysmal
* Stridor
* Cyanosis and anoxic attacks
* Dificulty with feeding
* Recurrent respiratory tract infections
Failure to gain weight
* Heart murmurs
* Tachycardia
b. In Children
* Dyspnoea
Cyanosis
* Growth retardation
* Decreased exercise tolerance
* Recurrent chest infections
* Squatting
* Heart murmurs and thrill
* Clubbing of fingers and toes
CYANOTIC HEART DISEASE
Overview
Factors which contribute to the development of cyanosis
include the following:
   1. right to left shunting with reduction of the volume of
the pulmonary blood flow (PBF) Tricuspid Atresia, and
Tetralogy of Fallot
   2. Intracardiac mixing of oxygenated and desaturated
blood in a chamber proximal to the aorta (e.g. single atrium,
single ventricle, double outlet right ventricle, truncus
arteriosus).
   3. Failure of delivery of pulmonary venous blood to the
systemic circulation (eg.Transposition of the Great Arteries,
total anomalous pulmonary venous correction)
   4. Severe low cardiac output states
 Patency of the ductus arteriosus is essential for
  the survival of severely ill cyanotic patients.
  Patients who are critically ill at birth may need
  surgical palliation,.
 Ductus patency is achieved by administration of
  Prostaglandin eg. PGE, 0.1 micro gm/kg.lmin.
 PGE is a potent vasodilator and volume infusion
  may become necessary.
 Many patients with severe cyanosis will
  additionally require intubation and mechanical
  ventilation
Palliative Procedures
Palliative procedures employed in the management of
cyanotic heart disease include the following:
A. Procedures to increase Pulmonary Blood Flow:
a)   Classical Blalock-Taussig (BT) shunt where the subclavian artery is divided
     and swung round and anastomosed end-side to the ipsilateal pulmonary
     artery.

b) Modified Blalock-Taussig Shunt (MBTS
A graft usually 4 or 5mm Polytetrafluoro-ethylene(PTFE) e.g. GORETEX is connected
between the subclavian artery and the ipsilateral pulmonary artery.
This avoids division of the subclavian artery which in some patients can cause
atrophy of muscles of the hand

c) Central Shunt: A shunt graft of PTFE connects the ascending aorta and the main
pulmonary artery.
2. Cavo-pulmonary shunts which connect the superior vena cava to the pulmonary
arterial system thereby increasing pulmonary blood flow. An example is the Genna
shun t.
3. Pulmonary valvotomy
4. Right ventricle-pulmonary artery conduit.
B. Procedure to Decrease Pulmonary Blood Flow
Pulmonary artery banding: involves passing a
  narrow tape or band around the stem of
the pulmonary artery to narrow it's lumen to
  reduce blood

C. Procedures to improve intracardiac mixing
a) Rashkind balloon atrial septostorny, which is
   performed at the time of initial catheterization.
b) Blalock-Hanlon Septostomy : With this
   procedure an artificial septal defect is created.
TETRALOGY OF FALLOT

 Commonest cyanotic cardiac defect beyond
  neonatal period. 8-10% of all cardiac defects.
  30% of congenital hrt defects. M:F 1:1
 Four components: VSD, Pulm stenosis,
  overriding aorta, rvh
 The first 2 are the main defects, the others
  complication
Pathophysiology
Tetralogy of Fallot is characterized by underdevelopment of
the infundibulum of the right ventricle
The severity of obstruction to the outflow tract of the right
ventricle affects the degree of cyanosis.
If the right ventricular outflow tract obstruction is severe,
pulmonary blood flow is reduced and there is right to left
shunting across the VSD and cyanosis is severe.
 Increased oxygen demand during exertion may cause
intense cyanosis and hypoxia which may lead to syncope,
seizures and even death.
In some patients, however, the outflow tract obstruction is
less severe, the pulmonary blood flow is greater and shunting
is predominantly left to right producing the so called "pink
fallot".
Most patients with Tetralogy of Fallot are diagnosed in
childhood but later presentation occurs.
Clinical Features
1. Dyspnoea and fatigue are common and worsen with
exercise. Patients characteristically adopt a squatting
posture after exertion.
2. Cyanosis of variable degree is always present.
3. There is symmetrical clubbing of fingers and toes
present in childhood and adults but not in infants.
4. Physical growth is usually retarded.
5. The jugular pressure is usually normal. The pulse is
usually of low volume and the blood pressure normal or
low.
6. A mid-systolic (pulmonary ejection) murmur, maximal in
the 2nd and 3rd intercostal space, may be present.
In neonates, there is reduction in arterial oxygen
saturation and sometimes metabolic acidosis.
Polycythaemia is rare and patients may even be anaemic
INVESTIGATIONS

   Chest xray- boot shaped heart
       In neonates – small heart
       Oligaemic lung fields
       (plethora of one lung= anomalous origin of one pulm. Artery from asc.
        Aorta)
   ECG- right ventricular hypertrophy, absent Q waves, low voltage
    R waves
   Echo- shows the anatomy
   Cardiac catheterization- check for presence of other VSDs and
    Morphology of pulmonary trunk
TREATMENT

   Palliative
     MBTS

   Definitive
     Cardiopulm  Bypass & hypothermia
     Relieve Rt ventricular outflow tract obstruction by
      valvotomy or resection of infundibular mm., or
      incision + patch
     Close VSD
PROGNOSIS

   Overall mortality <3%

               Complications
 Conduction abnormalities
 Rt ventricular failure
TRANSPOSITION OF THE GREAT ARTERIES
   Accounts for 5-8% of all chd
   Most cmmn cyanotic chd in newborn period
   M:F 3:1
   Aorta from rt ventricle
   Pulm. Artery from lt ventricle
   2 parallel circuits, must have ASD, VSD or PDA
                       CLINICAL FEATURES
   Cyanosis at birth & progressive with age
   Anoxic spells (incrsing tachypnea, and cyanosis)
   Reduced physical activity, growth and development
   Recurrent respiratory tract infxn>> cardiac failure
   Systolic murmurs if vsd is present
   Finger clubbing and polycythaemia in older px
INVESTIGATIONS

 Chest x-ray- egg on side shape + plethoric lung
  fields
 Ecg- rt atrial and ventricular enlargement

 Echo- basis of diagnosis

 Catheterization: angiograms in both ventricles
TREATMENT
 Once diagnosis is made, keep ductus patent with
  PGE
 Rashkind balloon atrial septostomy
 DEFINITIVE:
     mustard or senning atrial switch ( with pericardium or
      GORETEX)
     JATENE: anatomic replacement of great vessels(arterial
      switch)
     Usu. Pulm. Artery banding is done prior to Jatene to
      develop left ventricle
TRICUSPID ATRESIA
   Accounts for 1-2% of all cardiac defects in infancy.
  M:F 1:1
 Tricuspid valve absent or sometimes severely stenosed.
 Dilation of rt atrium, Rt to LT shunt through a dilated
  foramen ovale
 30% have dTGA
CLINICAL FEATURES ( depends on VSD size, degree of pulm.
          Stenosis, and venticulo-arterial connection)
 Cyanosis at birth
 Dyspnea, fatigue,
 Anoxic spells (worsening cyanosis, tachypnea, lethargy and
  LOC)
SIGNS

 Retarded growth/ devlopment with cyanosis
 Clubbing

 Prominent ‘a’ waves in jugular vein

 Strong left ventricular impulse

 +/- VSD murmur
INVESTIGATION

 Chest x-ray: oligaemic lung fields, rounded left
  hrt border
 ElectroCG- Left venticular hypertrophy, Lt axis
  deviation, biatrial enlargement
 Echo- absent tricuspid

 Catheterization: ASD, reduced arterial o2
  saturation, rt ventricle can not be entered
MEDICAL TREATMENT

   Keep ductus patent
SURGICAL TREATMENT

   Palliative: balloon atrial septostomy
     pulm  artery banding in large VSDs without pulm
      stenosis
     Modifeid blalock- taussig shunt

     Bidirectional glenn shunt

   Definitive: fontan procedure (done bw 2-5 yrs)
    A  valved conduit from rt atrium to pulm artery.
      Close the ASD
TRUNCUS ARTERIOSUS
 Single arterial vessel from the hrt>>coronary,
  systemic and pulmonary arteries
 1%
 There is always a membranous VSD.
                 CLINICAL FEATURES
 Heart failure in first few weeks of life
 Cyanosis
 Single s2, systolic ejection click and murmur ove A
  & P areas
INVESTIGATION

 ECG- biventricular hypertrophy
 CXR-cardiomegaly, + incrsd pulm vascular
  markings
 Echo will show the tuncus

 Cathterization +angio: will defint truncus type
  and chek competence of truncal valve
TREATMENT

 Medical treatment of ccf
 Pulm artery banding.

 Rastelli total correction where open heart
  surgery is possible
 Prophylaxis against infective endocarditis
ACYANOTIC HEART DEFECTS

 Ventricular septal defect
 Atrial septal defect

 Patene ductus arteriosus

 Atrio-ventricular canal defect

 Pulmonary stenosis

 Aortic stenosis

 Coarctation of the aorta
VENTRICULAR SEPTAL DEFECT
   Isolated VSD is the most common congenital hrt defect,
    (20-40%). M:F 1:1
   80% of VSDs are in the membranous part,
   Rarely multiple defects= ‘swiss cheese’ defects
   A LT to Rt shunt
   Small, moderate or large
   Ventricular Septal Defects
       A - Subarterial infudibular defect
       B - Perimembranous defects
       C - Muscular defects
   May reverse: eisenmenger sx
CLINCAL FEATURES

 Small VSDs usu asymptomatic
 Mod or large: features of hrt failure,

 Precordial bulge

 Thrill and systolic murmur at lower sternal
  border
INVESTIGATIONS

 Ecg- normal in small VSD; LVH and /or Lt atrial
  hypertrophy
 Cxr: cardiomegaly, pulmonary plethora

 Echo- shows the anatomy and the shunt

 Catheterizzation: step-up in O2 saturation in rt
  ventricle.
 A Lft ventricle angiogram: no, position of VSDs
NATURAL HISTORY

 30-40% spontaneous closure usu at 5yrs
 Large defectd dvlop ccf by 6mths

 some have progressive increases in the
  pulmonary vascular resistance with eventual
  reversal of shunt
 bacterial endocarditis in VSD associated with
  aortic regurgitation
TREATMENT

 Prophylaxis against infective endocarditis
 Small VSD- no treatment

 Pulmonary artery banding

 Total corrective surgery, if open hrt syrgery is
  available
ATRIAL SEPTAL DEFECT
 Accounts for 5-10% of all congenital cardiac
  defects
 F>M
 There are three types of asd:
     Ostium secundum type (most common)
     Ostium primum type
     Sinus venosus
 In uncomplicated ASDs, there’s always a Lt to Rt
  shunt.
 There are various sizes of defect
CLINICAL FEATURES
 Most are asymptomatic
 Rarely go into heart failure
 With time pulmonary vascular resistance
  increases, then become symptomatic
 Well developed and nourished
 Soft systolic ejection murmur at pulm. area
 Splitting of s2
 Diastolic rumble
INVESTIGATIONS

 ECG- (px with pulm hypertension) right axis
  deviation.
 Xray – normal, with large defects- some
  cardiomegaly
 Echo- diagnostic
TREATMENT

 If hrt fails, manage medically.
 ASD closure, primary suturing.

 Patch, for large defects from pericardium, or
  GORE-TEX
 More complex for ostium primum.

 PROGNOSIS

 Mortality <3%
PATENT DUCTUS ARTERIOSUS
 The ductus’ fxn is to bypass blood fom the
  immature lungs in utero.
 After birth, closes and anatomically in 1st 2 wks of
  life
 Patent ductus results in a Lt to Rt shunt, since
  aorta pressure> pulm. Aa pressure, this increases
  pulm blood flow.
 High pulm bld flow causes congestion..pulm
  oedema
   PDA may be critical to survival in complex cong. Hrt defct (ta, tof,pa)
CLINCAL FEATURES

 Cardiac failure features
 Some lead normal lives

 Underdevelopment, failure to thrive

 Limited physical activity

 Recurrent resp tract infections

 Pistol shot pulse

 Thrill at pulm. Area

 Murmur- continuous , loud rumbling ’machinery’
DIAGNOSIS

 Often clincally
 2-d doppler echo

 C-Xray: may show normal hrt with increased
  pulm vascularity
 ECG- biventricular hypertrophy

 Catheterization- traverse shunt: increased O2
  saturation at pulm aa.
INDICATION FOR SURGERY

 If it is the cause of resp failure
 Failure of pharmacological treatment

 If px >3mths, to prevent pulm vascular disease,
  endocarditis
 PHARMACOLOGICAL RX
Indomethacin (PG inhibitor ) 0.2mg/kg every 24hr up to a total of
0.6mg/kg PO/IV
CONTRA INDICATIONS
REDUCED renal fxn
Active infection
NEC
Bleeding tendency
SURGERY
 Double ligation
 Suture ligation

 Cut and suture both ends

 May need bypass in adults

COMPLICATIONS
 Hemorrhage
 Injury to recurrent laryngeal nerve

 Chylothorax

 Mortality >1%
CONCLUSION

 These defects affect many innocent and
  helpless children: the leaders of tomorrow.
  These kids die silently because facilities for
  their surgical correction are not available.
 We should become moved to cause a change, I
  don’t mind if we all become paediatric
  cardiosurgeons and politicians.
 thanks