Case report
At the age of 11 months, the patient was diagnosed as TOF and received a TOF repair with a transannular patch for right ventricular outflow tract reconstruction and discharged without major complications. Routine cardiac echocardiogram was reviewed once a year, however, moderate PR and RV dilatation developed 10 years after the operation which was earlier than expected. Cardiac magnetic resonance image (MRI) studies and cardiac computed tomography (CT), which were decided for further evaluation, showed a rare type of PAPVR (Fig. 1). The right upper-middle pulmonary veins (RUMPVs) were connected to the right superior vena cava (SVC) and the left upper pulmonary vein had a connection with the left SVC which drained to the left atrium (Fig. 2A, 2B). There was a connecting vein between the bilateral SVC (Fig 1). The left lower pulmonary vein (LLPV) was draining to the left hepatic vein (LHV) (Fig. 2C), and LHV had communications between the middle hepatic vein (MHV) as a venous plexus (Fig. 2D). Kinking at the origin of the left pulmonary artery (LPA) was also revealed. Deciding to repair the anomalous pulmonary drainage when the pulmonary valve replacement (PVR) is necessary, cardiac MRI studies were reviewed every three years. The last MRI finding, which was performed 1 year before admission, showed increasing RV end-diastolic volume index (RV EDVI, 174ml/m2) and RV end-systolic volume index (RV ESVI, 71ml/m2) with 38 percent of PR fraction. The pulmonary to systemic flow ratio (Qp/Qs) was 2.86 in cardiac catheterization studies, and planned an elective operation.
The patient received an elective open heart surgery through a redo median sternotomy. Cardiopulmonary bypass (CPB) was supported by cannulating the ascending aorta, right SVC, and the right femoral vein. After cross clamping the aorta, an incision was made from the lateral wall of right SVC to right atrium, and followed by a partial atrial septectomy. RUMPVs were rerouted by baffling to the atrial septal defect using Gore-tex vein graft (GTVG, Gore-Tex vascular graft, W.L. Gore assoc. Inc, Elkton, MD). The diaphragmatic surface of the pericardium was opened to expose the LLPV and the LHV. Internal closure between the LLPV and the LHV was performed using GTVG to separate the hepatic flow with the LLPV. Rerouting the LLPV was performed by baffling from the opening of the LHV to the baffle for the RUMPVs, within the lumen of the inferior vena cava (IVC) (Fig. 3). The incision of right SVC and right atrium was closed with using an additional GTVG. Aortic cross clamp was released and root vent was started. PVR was performed using 25mm St. Jude valve (St. Jude Medical, Minneapolis, MN) with LPA angioplasty. After an uneventful weaning from CPB support, the left SVC was ligated using a surgical clip to prevent the systemic venous blood mixing with the LUPV drainage. Extubating after 12hr of ICU care, the patient moved to the general ward and discharged after 8 days without significant complication. Postoperative CT showed decreased RV EDVI (58 ml/m2), RV ESVI (21 ml/m2) and demonstrated unobstructed venous return from the lungs (Fig. 4).