Multifactorial Genesis of a Seeming Case of Pulmonary Hypertension

Herein, we report the case of a 44-year-old female with end-stage renal disease on hemodialysis. She was admitted to our hospital to evaluate if she was eligible for a kidney transplant. Transthoracic echocardiography showed a markedly dilated coronary sinus and an unexpected finding of increased right ventriculoatrial gradient. A saline contrast echocardiography to confirm the presence of persistent left superior vena cava (PLSVC) was not performed because of arteriovenous fistula (FAV) for hemodialysis on the left forearm. Therefore, computed tomography angiography was performed, and it showed the PLSVC. We also proceeded with a transesophageal echocardiography which showed an atrial septal defect (ASD) of the sinus venous type hemodynamically significant. In this case, we identified a rare association of PLSVC with a ASD; therefore, there is a right ventricular volume overload because of the ASD hemodynamically significant and high flow FAV leading to a condition of a seeming pulmonary hypertension.