A Curious Case of Total Anomalous Pulmonary Venous Connection

First reported in the Philosophical Transactions of Royal Society of London in 1798, total anomalous pulmonary venous connection (TAPVC), also referred to as total anomalous pulmonary venous return (TAPVR), is a cyanotic congenital defect in which all four pulmonary veins fail to make their normal connection to the left atrium. This results in drainage of all pulmonary venous return into the systemic venous circulation. The incidence of total anomalous pulmonary venous connection (TAPVC) ranges from 0.6 to 1.2 per 10, 000 live births [1, 2]. Among patients born with congenital heart disease (CHD), the incidence of TAPVC ranges between 0.7 and 1.5 percent. It is the fifth most common cause of cyanotic CHD [1].75-90 % of symptomatic infants dont complete 1 year of life and most die in 3-6 months. Those who survive beyond 1 year of life almost always have a non-restrictive ASD. Exceptionally patient may reach even the 3rd-4th-5th decade of life with relatively little disability and a clinical picture that resembles Large ASD with mild cyanosis. Here, we review such an exceptional case of a patient diagnosed to have TAPVC at the age of 17 years.