Signs and symptoms
right ventricular heave
Loud S1
fixed split S2
S3 gallop
systolic ejection murmur at left upper sternal border
cardiomegaly
right axis deviation on ECG
Snowman sign or figure of 8 configuration on chest radiograph
right ventricular hypertrophy
cyanosis, tachypnea, dyspnea since the overloaded pulmonary circuit can cause pulmonary edema
Cottage-loaf sign, that is, chest X-ray appearance similar to a cottage loaf, also known as the snow man sign or figure of 8 sign. Treatment
In TAPVC without obstruction, surgical redirection can be performed within the first month of life. The operation is performed under general anesthesia. The four pulmonary veins are reconnected to the left atrium, and any associated heart defects such as atrial septal defect, ventricular septal defect, patent foramen ovale, and/or patent ductus arteriosus are surgically closed. With obstruction, surgery should be undertaken emergently. PGE1 should be given because a patent ductus arteriosus allows oxygenated blood to go from the circulation of the right heart to the systemic circulation. Partial anomalous pulmonary venous connection
A Partial anomalous pulmonary venous connection (or Partial anomalous pulmonary venous drainage or Partial anomalous pulmonary venous return) is a congenital defect where the left atrium is the point of return for the blood from some (but not all) of the pulmonary veins.It is less severe than total anomalous pulmonary venous connection which is a life-threatening anomaly requiring emergent surgical correction, usually diagnosed in the first few days of life. Partial anomalous venous connection may be diagnosed at any time from birth to old age.
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