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).