If unrepaired, concomitant moderate-to-severe atrioventricular valve regurgitation significantly worsens the results of the Fontan procedure. Longer observation is needed to define the strategy in patients with mild atrioventricular valve regurgitation that remained unrepaired during Fontan operation.

Total cavopulmonary anastomosis is accompanied by lower mortality rate and a more favourable course of early postoperative period. However, the best long-term functional results are obtained after atriopulmonary anastomosis.

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The most common form of partial anomalous pulmonary venous connection is drainage of the right pulmonary veins to the superior vena cava. On the contrary, partial anomalous venous connection of right pulmonary veins to the left vertical vein is described in few reports. The goal of this publication is to present the case of diagnosis and surgical repair of this extremely rare pathology in a 19-year old female. Methods of preoperative examination included but were not limited to spiral computed tomography and angiocardiography. Intraoperative examination confirmed that the collector of right upper pulmonary veins was connected to the vertical vein that continued to the innominate vein and then to the superior vena cava draining into the right atrium. During surgical intervention, the vertical vein was transected in the area of connection to the innominate vein with the suturing of inlet area. The collector of the right upper pulmonary veins was then anastomosed with the left atrial appendage. Postoperative course was uneventful. In conclusion, isolated partial anomalous pulmonary venous connection of the right upper pulmonary veins to the left vertical vein is a very rare congenital defect. Existing modern techniques contribute to the precise and timely diagnosis of this anomaly.

Three cases of criss-cross heart with severe and complex congenital defects are reported. Precise preoperative diagnosis of the morphologic abnormalities made it possible to choose an optimal method of surgical treatment: all patients successfully underwent a Fontan-type surgical procedure.

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