Total anomalous pulmonary venous connection history and symptoms

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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]

Overview

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.

Symptoms

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]

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

Mostly they are acyanotic on presentation.

References

  1. Gathman GE, Nadas AS (July 1970). "Total anomalous pulmonary venous connection: clinical and physiologic observations of 75 pediatric patients". Circulation. 42 (1): 143–54. doi:10.1161/01.cir.42.1.143. PMID 5425587.
  2. Pollack, Charles V. (2019). doi:10.1007/978-3-319-63895-9. Missing or empty |title= (help)

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