Congenital Heart Disease

Anomalous Pulmonary Venous Drainage

Total / Partial Anomalous Pulmonary Venous Drainage occurs when the pulmonary veins incorrectly connect to the right atrium or other blood vessels.

About Total / Partial Anomalous Pulmonary Venous Drainage

Total /partial anomalous pulmonary venous drainage is a rare congenital heart defect often diagnosed at birth, caused when the pulmonary veins fail to form and connect the heart as expected in utero.

It is characterised by the pulmonary veins (PV) not being connected to the Left Atrium (LA) and instead of being connected to other large vessels that return a combination of mixed oxygenated blood to the RA instead of the LA.

Patients may have other heart defects such as an Atrial Septal Defect and other structural heart abnormalities. The definition of Total v Partial Anomalous Pulmonary Venous Drainage will depend on the number of pulmonary veins involved in the condition.

If all four are involved, the diagnosis of Total Anomalous Pulmonary Venous Drainage is made, if less than four, Partial Anomalous Pulmonary Venous Drainage is considered appropriate.

Types of Total Anomalous Pulmonary Venous Drainage:

  • Supracardiac- the PV come together abnormally above the superior vena cava
  • Cardiac- the PV meet behind the heart and connect to the RA
  • Infracardiac- the PV form abnormal connections below the heart

 

Source: Centers for Disease Control and Prevention1

 

Diagnosis:

Diagnosis can occur in utero after a foetal ultrasound or shortly after birth if the child demonstrates symptoms such as:

  • Cyanosis of the babies lips / limbs (“Blue Baby”)
  • Rapid respiratory rate
  • High heart rates
  • Failure to thrive (if out of hospital)
  • Oxygen saturations < 90%

 

Treatment:

Cardiac surgery is required to correct the anatomical defects and restore regular blood flow to the left atrium and also correct any other cardiac abnormalities that may have been identified2.

 

What is Partial Anomalous Pulmonary Venous Drainage?

Cardiothoracic Surgeon, Dr Yishay Orr, explains what partial anomalous pulmonary venous drainage is. Dr Orr outlining that this cardiac lesion typically presents in young to middle-aged adults which is most commonly detected from an isolated murmur. Watch the below video to learn more.

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