Total anomalous pulmonary venous connection history and symptoms

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief:Cafer Zorkun, M.D., Ph.D. [2]; Keri Shafer, M.D. [3]; Priyamvada Singh, MBBS [4]; Assistant Editor-In-Chief: Kristin Feeney, B.S. [5]

The clinical features in total anomalous pulmonary venous connection depend on the type of anatomic variant present in the patient. This, in turn, determines the amount of mixing between the pulmonary and systemic circulation. In patients with obstructed TAPVC, pulmonary venous circulation drains into the systemic venous circulation. This causes increased returns to the right side of the heart and pulmonary hypertension that can manifest as cyanosis, dyspnea, pulmonary edema, respiratory failure, shock, and hypotension. In patients with unobstructed TAPVC clinical findings are quite similar to conditions with left-to-right shunting like dyspnea, difficulties in feeding, and failure to thrive.

The total anomalous pulmonary venous connection (TAPVC) can be divided into two types i.e. obstructed and non-obstructed.

In patients with obstructed TAPVC, pulmonary venous circulation drains into the systemic venous circulation. This causes increased returns to the right side of the heart and pulmonary hypertension that can manifest as:^[1][2]

• Cyanosis
• Dyspnea
• Pulmonary edema
• Respiratory failure
• Shock
• Hypotension (decrease in the blood to the left side of the heart)

In patients with unobstructed TAPVC clinical findings are quite similar to conditions with left-to-right shunting like:

• Dyspnea
• Difficulties in feeding
• Failure to thrive

Mostly they are acyanotic on presentation.

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