Anomalous Pulmonary Venous Connection

The pulmonary veins, instead of conn¬ecting into the left atrium, connect into the right side of the circulation. If only some of the veins drain (connect) abnormally, the term ‘partial’ is used. If all the conn¬ections drain abnormally, the condition is described as ‘total’.

There are several sites into which the veins may drain. These are shown in the diagram and may be above the heart into the large veins (l or 2), within the heart either into the right atrium or coronary sinus (3 or 4); or below the heart into the liver or into the major lower vein (5).

If the connection is narrow (obstructed) then the child will present in the early days of life with blueness and breath¬lessness because blood has difficulty in returning from the lungs into the heart. If the connection is wide open (non-obstructed) then the problems are breath¬lessness, chest infections and poor weight gain and come to light during the first months of life rather than the first few days.

Open heart surgery to redirect the blood flow back into the left atrium is required when symptoms (complaints) occur. Although the operation is complex and initial recovery slow, particularly in those where the connection is obstructed, the long term outlook is often good. Occasionally, further obstruction occurs with growth which may be at the new connection into the heart or within the lung veins themselves. Long term follow¬-up is required but long term antibiotic prophylaxis is not necessary beyond a year after the operation.

 

Victoria & Tasmania