This case describes an 18-year-old female with a rare congenital heart defect – interrupted aortic arch type Celoria–Patton B combined with a ventricular septal defect (3.33 cm) and patent ductus arteriosus (0.60 cm). The patient presented with exertional dyspnea and severe pulmonary hypertension with mean pulmonary artery pressure of 82 mmHg. Advanced cardiac imaging with hemodynamic analysis revealed bidirectional shunting through the septal defect and elevated wall shear stress in the pulmonary artery (2.18 Pa) compared to the aorta (1.22 Pa). The pulmonary artery was mildly dilated. Inhaled nitric oxide reduced the pulmonary artery pressure from 82 to 64 mmHg. This case demonstrates that advanced imaging and hemodynamic analysis can characterize biomechanical changes in this rare congenital heart disease.