PMC6088432_01
(A) Contrast enhanced chest computed tomography showed abnormal connection of the right upper pulmonary vein (RUPV: shaded) and superior vena cava (SVC) (arrow). (B) The RUPV (shaded) preserved normal connection to the left atrium (LA). (C) Coronal. The RUPV (shaded) had an abnormal connection (arrow) to the SVC and returned to the LA. RUPV: right upper pulmonary vein, SVC: superior vena cava, LA: left atrium, RA: right atrium.
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RUPV connected not only to the LA but also to the SVC (Fig. 1).
Schema of pulmonary circulation. RA: right atrium, RV: right ventricle, PA: pulmonary artery, LA: left atrium, LV: left ventricle, Qp: pulmonary blood flow, Qeff: effective pulmonary blood flow, Qs: systemic blood flow.
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Right cardiac catheterization (RHC) data were as follows: pulmonary arterial pressure (PAP) systolic/diastolic (mean): 47/12 mmHg (26 mmHg); left atrium pressure (LAP): 2 mmHg; cardiac index (CI) measured by Fick methods: 5.68 L/min/m2; left-to-right shunt ratio: 70.5%; right-to-left shunt ratio: 11.5%; pulmonary blood flow (Qp): 10.45 L/min; systemic blood flow (Qs): 3.49 L/min; effective pulmonary blood flow (Qeff): 3.09 L/min; Qp/Qs 3.00; pulmonary vascular resistance (PVR): 122.3 dyn.sec.cm-5 (Fig. 2). RHC also revealed increased oxygen saturation in the SVC (Fig. 3).
Oxygen saturation of cardiac catheterization. The oxygen saturation elevated between the superior vena cava (SVC) and right atrium (RA). RUPV: right upper pulmonary vein, SVC: superior vena cava, LA: left atrium, RA: right atrium, RV: right ventricle, IVC: inferior vena cava, LV: left ventricle, LUPV: left upper pulmonary vein.
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Right cardiac catheterization (RHC) data were as follows: pulmonary arterial pressure (PAP) systolic/diastolic (mean): 47/12 mmHg (26 mmHg); left atrium pressure (LAP): 2 mmHg; cardiac index (CI) measured by Fick methods: 5.68 L/min/m2; left-to-right shunt ratio: 70.5%; right-to-left shunt ratio: 11.5%; pulmonary blood flow (Qp): 10.45 L/min; systemic blood flow (Qs): 3.49 L/min; effective pulmonary blood flow (Qeff): 3.09 L/min; Qp/Qs 3.00; pulmonary vascular resistance (PVR): 122.3 dyn.sec.cm-5 (Fig. 2). RHC also revealed increased oxygen saturation in the SVC (Fig. 3).
(A) The catheter tip (arrowhead) is in the left atrium (LA) near the left upper pulmonary vein (LUPV) from the SVC via anomalous pulmonary vein and normal RUPV to the LA (arrow). (B) Three-dimensional computed tomography specifies the abnormal connection (arrow, purple) between the RUPV and SVC. The right cardiac system is shown in blue, the left cardiac system is shown in transparent pink. SVC: superior vena cava, RUPV: right upper pulmonary vein, RA: right atrium, IVC: inferior vena cava, LA: left atrium, LV: left ventricle, LUPV: left upper pulmonary vein. (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)
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The pulmonary angiography confirmed duplicated connection of the RUPV to both the SVC and LA (Fig. 4).