Background
Total anomalous pulmonary venous return is a rare cyanotic congenital heart disease in which pulmonary veins connect to the systemic veins, right atrium or coronary sinus. Given its variability, it is essential to define the pathway and drainage site.
Case summary
We describe a 3-day-old patient with total anomalous pulmonary venous return, in which the four pulmonary veins drained into a common vertical vein that had a double connection at the cardiac (coronary sinus) and infracardiac (portal vein) levels. The echocardiographic diagnosis was confirmed by CT scan. At 1.5 months she underwent surgery by anastomosis of the venous collector with the left atrium, unroof of the coronary sinus and clousure of its comunication with right atrium. The vertical vein was ligated. There were no complications after one year of follow-up.
Discussion
This is an unusual anatomy that has not been described in the literature. In this article, we discuss the embryological origin. Furthermore, we highlight the importance of an accurate diagnosis of the venous pathway and its impact on surgical planning and patient prognosis.
Refractory neonatal supra-ventricular tachycardia may require the use of flecainide as anti-arrhythmic. Close control of plasma levels is mandatory due to pro-arrhythmic complications. However, inadvertent hemolysis during blood sampling may cause an increase in measured plasma flecainide concentrations. We conclude, therefore, that dosing of the drug should be always done with caution, and in case of suspected haemolysis plasma levels should be repeated with a new blood sample.
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