Rare case of a mixed type total anomalous pulmonary venous return – benefits of a patient specific 3D model in the preoperative preparation

Abstract

Accurate imaging of the morphology in a child with a rare and complex congenital heart disease (CHD) is crucial for the surgical planning and, if possible – the minimization of operative risk. We present a case of a 3-month-old child with a rare variant of Total Anomalous Pulmonary Venous Return (TAPVR) – mixed type – which illustrates the benefi t of additional imaging modalities and techniques for the operative planning. The patient’s anomalous drainage of the pulmonary veins (PV) is as follows: left and right lower PVs drained in the coronary venous sinus, left upper PV drained via a vertical vein into the innominate vein and the right upper PV drained into the superior vena cava (SVC). Clinical fi ndings consist of heart failure (HF), pulmonary venous congestion and mild hypoxemia developing within the fi rst days after birth. Transthoracic echocardiography (TTE) demonstrates the features of TAPVR, but the modality is unable to differentiate all of the described anatomic details. Accurate morphological diagnosis is obtained via contrast Computed Tomography (CT). The. additional techniques of 3D modelling and 3D printing of a patient-specifi c model, based on the acquired CT, contributed to the planning of the surgical strategy. The patient underwent a radical operation at age of 3 months. The unique aspect of the case is the application of all possible operative techniques for correction of TAPVR – coronary sinus deroofi ng, anastomosis of a draining vessel into the left atrial appendage and Warden procedure which involves tunnelling of the right upper PVs from the SVC into the left atrium. Feedback from the surgical team shows that the additional imaging methods and the printed 3D model represent the pathology in detail that completely corresponds to the intra-operative fi ndings