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Group II - Cyanotic Heart Disease

  1. Cyanotic HD with  PBF
    1. Tetralogy of Fallot (TOF) 
  1. Four constituents of tetralogy -
    1. VSD              
    2. PS                
    3. Overriding or dextroposed aorta            
    4. RVH 
  2. COMMONEST CYANOTIC CHD – After infancy.
  3. Sounds                        
    S1 - Normal
    S2 - Single only A2  Heard 
  4. Murmur Shunt murmur - absent
  5. Flow murmur - Pulmonary ejection systolic*
  6. The severity of cyanosis is directly proportional to severity of pulmonic stenosis but the intensity of systolic murmur is inversely related to the severity of pulmonic stenosis 
  7. Clinical features:
    1. Cyanosis may be present from birth or make its appearance some year later
    2. Dyspnea on exertion and exercise intolerance
    3. Infant may develop anoxic spells.
    4. Patient assumes a sitting posture or squatting on as soon as they get dyspneic 
  8. On examination
    1. Cyanosis              
    2. Clubbing      
    3. Normal size heart (Due to concentric hypertrophy) Q
    4. Normal first hear sound        
    5. Single second heart sound
    6. Ejection systolic murmur 
  9. ECG (right axis deviation with Rt ventricular hypertrophy) Q 
  10. P-pulmonale:
    CXR - Normal sized heart with upturned apex suggestive of RVH. The absence of main pulmonary artery segment gives the shape described as 'cor-en-sabot' or boot shaped heart
       • Oligemic lung field 
  11. Course and complicationChronic hypoxia → polycythemiaQ
    1. Dyspnea and increasing exercise intolerance limit the patient considerably
    2. Anemia by decreasing the oxygen carrying capacity of blood reduces the exercise tolerance still further [Normal Hb/Hct or t RBC indices indicates iron deficiency anemia]
    3. Cerebral thrombosis →due to polycythemia and dehydration (<2yr)
    4. Brain abscess →occurs after 2 yr
    5. Hemiplegia →due to anoxic infarction in the CNS &may occur during an anoxic spell 
  12. Treatment
  1. Palliative treatment consists of anastomosing a systemic artery with pulmonary artery to increase the PBF and thus increase the amount of oxygenated blood reaching the systemic circulation.
    1. Blalock- Taussig PA anastomose with subclavian
    2. Pott's Shunt ** PA anastomosis with descending aorta
    3. Waterston's shunt   Right PA anastomosis with Ascending aorta
  2. Definitive operation consists of closing the ventricular septal defect and resecting the infundibular stenosis.
    Hypoxic spells, also called cyanotic spells or tet spells occurs in young infants with TOF
    1. It consists of
      1. hyperpnea (i.e. rapid and deep respiration)
      2. worsening of cyanosis
      3. disappearance of heart murmur
    2. HyperCyanotic (Tet spell)
      Any event such as crying, defecation or increased physical activity that decreases- systemic vascular resistance (SVR) or produces R-L shunt initiate spell by establishing a vicious cycle of hypoxic spells.  
  3. Rx

Table: Management of anoxic spells

  1. Knee chest position
  2. Humidified oxygen
  3. Morphine 0.1 to 0.2 mg/kg, SC
  4. Obtain venous pH; sodium bicarbonate 1-3 ml/kg (diluted)
  5. IV
  6. Propranolol 0.1 mg/kg/IV (during spell); 0.5-1 mg/kg/6 hourly orally (alternatives: metoprolol, esmolol)
  7. Vasopressors: methoxamine (Vasoxyl) IM or IV drip
  8. Correct anemia
  9. Consider surgery 

Points to remember:


Pentology: TOF + ASD


Triology: ASD/PFO + RVH + PS

  1. Tricuspid atresia
    1. Congenital absence of the tricuspid valve is called tricuspid atresia. The right ventricle is hypo-plastic. Q Inflow portion is absent. The only exit For systemic venous blood coming to the right atrium is through patent foramen ovale or ASD- There is complex mixing of systemic venous and pulmonary blood. 
    2. Feature suggestive of tricuspid atresia:
      1. Left ventricle type apex impulse
      2. Prominent large a wave in JVP
      3. Enlarged liver with presystolic pulsation
      4. ECG shows left axis deviation * and left ventricular hypertrophy, mean QRS axis = -45"Q
      5. Lung fields are oligemic 
    3. Clinical features - Similar to TOF - Cyanotic from birth
    4. Treatment modified Fontan operation
  2. Ebstein Anomaly (Atrialization of RV) Q 
    1. Downward displacement of tricuspid valve into Rt ventricle. The RV is divided into 2 parts – the atrialized part which is thin walled, in continuation of RA and smaller ventricular part – with normal musculature.
    2. RA shunts blood through PFO 2 LA producing cyanosis,
    3. Both the systolic and diastolic murmur produced have a scratchy character 
    4. ECG shows
      1. P pulmonale (tall p wave) as well as P mitrale (broad p wave) &RBBB, R wave in V1 does not exceed more than 7 mm
      2. Wolf Parkinson While type of conduction abnormally may also be present
    5. CXR Normal to massive box shaped * cardiomegalyQ
      oligemic lung field 
  1. Cyanotic CHD with PBF
    1. Transposition of great vessels  
      1. Is defined as aorta arising from Rt ventricle and Pulmonary artery from the left ventricle.
      2. Anatomically TGA can be further subdivided into
        1. Complete TGA
        2. Physiologically corrected type 
      3. Complete TGA  (D-TGA) Q
        1. The RA empties into RV from which aorta arises. The LA drains into LV to which pulmonary artery is connected.
        2. Since the systemic and pulmonary circulation are separate. Survival depends on the presence of atrial, ventricular or aorto-pulmonary communications. 
      4. Physiologically corrected type (L-TGA) Q
        1. Associated with inversion of ventricleQ
        2. RA is connected to an inverted morphologically LV which is connected to PA where as the LA is connected to the inverted morphologically RV connected to aorta
        3. Thus route of blood flow is normal and hence it is called corrected TGA.
        4. Normally the aorta is posterior and to the Rt of the pulmonary artery. In d-TGA the aorta is anterior and to the Rt of the pulmonary artery, (d-indicates dextropositioned aorta)
      5. ECG: Rt axis deviation and Rt ventricular hypertrophy 
      6. LTGA (corrected Transposition]
        1. The aorta arises to the left of the pulmonary artery [L- for levo-transposition]
        2. They are hemodynamically Normal. 
      7. Patient with complete TGA with VSD have large PBF AND good mixing at the ventricular level and so cyanosis is mild.
      8. They develop congestive failure at 4 – 10 wks of life.
      9. CXR Cardiomegaly with plethoric lung fields. (“Egg on side" appearance) Q
      10. Treatment The arterial switch (Jatene) procedure Q is the surgical treatment of choice. The operation involves dividing the aorta and pulmonary artery just above the sinuses and re anastomosing them in their correct anatomic positions.
    2. TAPVC 
      1. All the pulmonary veins instead of joining the left atrium are connected anomalously to Rt atrium
      2. Pulmonary Veins Opens into Rt atrium which also receives systemic venous blood
      3. Supra cardiac (SVC) –
        1. Pulmonary veins join together to form a common pulmonary vein which may drain into the left innominate vein or the Rt SVCQ
        2. Wide and fixed S2
        3. X-ray figure of snowman appearance(Figure of 8) - appear after 2yrs.
      4. Infracardiac (IVC/portal vein)
        1. Has obstructions
        2. Ground glass Appearance of lungs  
    3. Other conditions with cyanosis and PBF
      1. Single Ventricle without obstruction to pulmonary
      2. Persistent truncus arteriosus
      3. They have poor prognosis. 

Cyanotic Congenital Heart Disease with Pulmonary Arterial Hypertension


Eisenmenger syndrome


This group of patients are also termed Eisenmenger syndrome, implying the presence of severe pulmonary arterial hypertension resulting in a right to left shunt at the atrial, ventricular or pulmonary arterial level. Eisenmenger complex consists of pulmonary arterial hypertension with a ventricular septal defect providing the right to left shunt.


Untreated L to R shunt lesion → Hyperkinetic pulmonary artery hypertension Obstructive pulmonary artery hypertension → Eisenmenger syndrome (R to L shunt) 


Factors influencing Left to Right shunt

  1. Size of the communication
    Large defect- large shunt
  2. Vascular resistances
    Lower relative (to SVR) or absolute PVR - higher magnitude of shunt Higher relative to (SVR) or absolute PVR – lower magnitude of shunt
  3. Decreased right ventricular stiffness (increased compliance)-large shunt
  4. Increased left ventricular stiffness (decreased compliance) – large shunt 

Untreated left to right shunt lesion

  1. Lead to pulmonary vascular injury due to elevated pulmonary blood flow and shear stress. Vascular remodeling ensues, leading to increased pulmonary vascular resistance (PVR) and pulmonary vascular disease (PVD)
  2. Patients whose PVR may exceed systemic vascular resistance, resulting in reversed shunting (Eisenmenger physiology), with consequent chronic hypoxaemia
  3. Early intervention and protection of the pulmonary circulation is therefore a major determinant of prognosis for patients with PAH associated with CHD 

Eisenmenger physiology :


Patients whose PVR may exceed systemic vascular resistance, resulting in reversed shunting with consequent chronic hypoxaemia

Eisenmenger complex :


Consists of pulmonary arterial hypertension with VSD providing the R to L shunt


Eisenmenger syndrome :


Patient have severe pulmonary arterial hypertension resulting in R to L shunt at atrial, ventricular or pulmonary arterial

Factor responsible for rapid development ES :

  1. Increased pulmonary blood flow            
  2. Increased pulmonary artery pressure
  3. Hypoxia                              
  4. Hypercarbia                        
  5. High altitude 

Clinical features :

  1. Cyanosis,            
  2. Fatigue,                
  3. Dyspnea,            
  4. Repeated chest infection 

On Examination :

  1. Cyanosis, Clubbing, Parasternal impulse
  2. The features indicative of pulmonary arterial hypertension consist of parasternal impulse and palpable P2' (pulmonary component of 52 is accen­tuated and louder than the aortic component).
  3. A pulmonary ejection click, which unlike patients of valvar pulmonic stenosis, is well heard during inspiration and expiration.
  4. A functional pulmonary regurgitation murmur can be present along the left sternal border.



Pulmonary plethora in hilar area &Oligemia in peripheral fields [Q]



RAD & P-pulmonale [Q]


EKG shows right axis deviation and right ventricular hypertrophy; P pulmonale may be present. The chest X-ray is characteristic with prominent pulmonary arterial segment and large right and left main pulmonary arteries, but oligemic lung fields. Thus, the hilar area shows pulmonary plethora while the peripheral fields suggest oligemia.



  1. There is no role for surgical treatment (Inoperable) Q.
  2. Medical manage­ment consists in treating chest infections, correcting anemia and managing CCF.
  3. Intermittent use of oxygen may help relieve syncope, severe headaches and angina-like chest pain.
  4. Ideally, pulmonary vascular obstructive disease should be prevented from developing. This means careful evaluation and follow up of patients of ventricular septal defect and PDA in the first two years of life, when pulmonary arterial hypertension is generally reversible.
  5. Patients with cyanosis and increased pulmonary blood flow develop Eisenmenger physiology very early and need to be operated by two to three months of age. Patients who have features of pulmonary arterial hypertension should be operated early to prevent the development of irreversible pulmonary arterial hypertension.
  6. Medications have recently become available for the management of pulmonary hypertension. These include agents that reduce pulmonary hypertension.
    1. Phos­phodiestrase-5 inhibitors (sildenafil, tadalafil) selectively increase the local availability of cyclic GMP in the pulmonary vascular smooth muscles, promoting pulmonary vasodilation.
    2. Bosentan, sitaxsentan and ambrisentan are endothelin receptor blockers, which inhibit the pulmonary vasoconstrictor effects of endo­thelin, promote vasodilation and enable vascular remodel­ing.
    3. Prostacyclin analogs promote pulmonary vaso­dilation. The results of preliminary studies with these agents in Eisenmenger syndrome are promising. 
  1. Obstructive Lesion
    1. Coarctation of Aorta:
      COA occurs in 8% to 10% of all cases of CHD
      More common in males than in females (2:1) Q
      Among patients with Turner's syndrome, 30% have COA Q



Terms used in the past such as preductal and postductal as well as infantile or adult-type COA are misleading


COA is almost always in a juxtaductal position (i.e., neither preductal nor postductal)


98% below the origin of Lt subclavian artery at the origin of ductus arteriosus (Juxta-ductal)


85% of patients with COA have a bicuspid aortic valve Q


Associated with PDA, VSD, & ASD


When left sided obstructive lesion : MS, AS & CoA occur together known as SHONE COMPLEX Q



  1. Bimodal presentation
    1. Usually presents as signs of CHF in neonates and during infancy.
    2. If signs of CHF not present during infancy, rarely presents prior to adolescence.  
  2. Clinical Manifestation :
    - Weakness & pain in leg after exercise
    - Intermittent claudication
    - Dyspnea on running
    - Disparity of Pulse & BP in arms & legs
    • Weak or absent pulse in LL
    • Bounding pulse in UL
    • Radio femoral delay

    -Suzman sign: Visible pulsation below angle of scapula.

  • Hypertension on routine examination
  • Sounds & murmur:
    -Loud S1 & S2, S3 (With LV failure), S4 (With severe hypertension)
    -ESM in interscapular area
    -Continuous murmur over the chest wall
  • Severity determined by :
  1. X-ray: ‘3’ signs
    Rib notching: usually appear after 10 year of age, involve 3-8 ribs, lower border, due to collateral artery pulsation. 
  2. Barium swallow: ‘E’ sign / reverse ‘3’ sign. 


  1. Hypotension: Most common Q       
  2. CHF
  3. Left ventricular hypertrophy         
  4. Extra cardiac complication: Rupture of Berry aneurysm Q 





In symptomatic neonates, PGE1 infusion should be started to reopen the ductus arteriosus and establish flow to the descending aorta and the kidneys during the first weeks of life Q


Intensive anticongestive measures with short-acting inotropic agents (e.g., dopamine, dobutamine), diuretics, and oxygen should be started


Balloon angioplasty can be a useful procedure for sick infants Q


Balloon angioplasty is associated with a higher rate of recoarctation than surgical repair, and the rate of complications (including femoral artery injury) is high during infancy




Resection of coarctation and aortoplasty.

  1. Aortic stenosis 
    1. Concentric hypertrophy of the left ventricle
    2. History of angina on effort and syncope
    3. Presence of S3 &S4 

Assessment of Severity


In every patient it is necessary to assess the severity of aortic stenosis. Absence of an indication of severity means incomplete diagnosis. The clinical assessment of severity depends on the following

  1. Symptomatic patients have severe aortic stenosis. If the patient is asymptomatic, it does not exclude severe aortic stenosis.
  2. Narrower the pulse pressure, the more severe the aortic stenosis.
  3. Presence of a systolic thrill at the second right interspace suggests at least moderately severe aortic stenosis. If the thrill is felt only in the suprasternal notch and not at the second right interspace, it favors mild or critical aortic stenosis in failure.
  4. The later the peak of the ejection systolic murmur, the more severe the narrowing.
  5. The delay in the A2 is reasonably well correlated with severity. With mild aortic stenosis the S2 is normally split, with moderate aortic stenosis it is closely split, with severe or critical aortic stenosis it is Single or paradoxically split.
  6. Presence of S4 is indirect evidence for severe aortic stenosis.
  7. Presence of S3 indicates severe aortic stenosis and congestive cardiac failure.
  8. ST and T changes on the EKG favor severe aortic stenosis.
  9. Cardiac enlargement indicates severe aortic stenosis with left ventricular failure.
  10. Doppler echo can quantitate the gradient across the aortic valve very accurately.
    Left ventricular myocardial disease may alter the findings to favor a more severe lesion.

Supravalvular aortic stenosis (William's syndrome) is associated with characteristic "elfin" facies, mental retardation, dental abnormalities, strabismus and peripheral arterial stenosis. It is closely related to Idiopathic hypercalcemia and may be due to hypervitaminosis D.

  • Bad disease since it is one of the few lesions which can cause sudden death 

Pulmonic stenosis:

  1. Palpitation, easy fatigability
  2. On physical examination patients have a rounded face and hypertelorism
  3. Noonan syndrome is associated with P.S

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