Cyanotic heart defect

Cyanotic heart defect is a group-type of congenital heart defects (CHDs) that occurs due to deoxygenated blood bypassing the lungs and entering the systemic circulation or a mixture of oxygenated and unoxygenated blood entering the systemic circulation. It is caused by structural defects of the heart (ie:right-to-left , bidirectional shunting, malposition of the great arteries), or any condition which increases pulmonary vascular resistance. The result being the development of collateral circulation.

Signs & Symptoms

• Clubbing
• The patient assuming a crouching position
• Cyanosis - facial discolouration (particularly the lips) & digit discolouration (fingers & toes).
• Crying
• Crabbiness/irritablitity
• Tachycardia
• Tachypnea
• A history of inadequate feeding.
• Unusually large toe & fingernails.
• Delayed development (both biological & psychological).

Categories/Classifications

• Tetralogy of Fallot (ToF)
• Total anomalous pulmonary venous connection
• Hypoplastic left heart syndrome (HLHS)
• Transposition of the great arteries (d-TGA)
• Truncus arteriosus (Persistent)
• Tricuspid atresia
• Interrupted aortic arch
• Pulmonary atresia (PA)
• Pulmonary stenosis (critical)
• Eisenmenger syndrome(Reversal of Shunt due to Pulmonary Hypertension) .
• Patent ductus arteriosus may cause cyanosis in late stage^[1]

Pharmacological Treatment & Management

• Morphine during Tet spells to decrease associated infundibular spasm.
• Prophylactic: Propranolol/Inderall
• Prostaglandin E (to keep the ductus arteriosus patent)
• Prophylactic antibiotic to prevent endocarditis
• Surgery

Non Cyanotic Heart Defects

Non cyanotic heart defects are more common because of higher survival. In these the shunt is initially from left (oxygenated) to right ( non oxygenated). These are

• Atrial septal defect
• Ventricular septal defect
• Coarctation of aorta (may cause cyanosis in some cases)
• Double outlet left/right ventricle

See also: Acyanotic heart defect

References