Eisenmenger syndrome with unrepaired patent ductus arteriosus.

A 19-year-old girl with a history of pulmonary arterial hypertension secondary to patent ductus arteriosus (PDA) presented in respiratory failure after blood transfusion for severe menorrhagia. The patient had been raised previously in a rural mountain community that did not engage in medical care. She had been experiencing cyanosis and syncope for 2 years before emancipating herself from her family. One year before this admission, she was seen by a physician for the first time and underwent an echocardiogram, which showed a PDA and right ventricular enlargement. Right heart catheterization demonstrated severe pulmonary arterial hypertension with a mean pulmonary artery pressure of 68 mm Hg and a pulmonary capillary wedge pressure of 8 mm Hg. Cardiac output was 4 L/min. The patient was started on sildenafil 20 mg 3 times a day and bosentan 125 mg twice a day, and her exercise tolerance improved, with resolution of her syncopal episodes. In the week before admission, the patient developed progressive menorrhagia and presented to an outside hospital where she required blood transfusions. After transfusion, the patient developed respiratory failure and was intubated, started on continuous dobutamine infusion for inotropic support, and transferred to a quaternary hospital for further evaluation. On transfer to the cardiovascular intensive care unit, the patient was responsive and noted to have differential cyano-sis, with decreased oxygen levels in the left arm compared with the right (Figure 1). Cardiac auscultation revealed a harsh systolic and diastolic murmur, which varied with respiration (Audio I in the online-only Data Supplement) and was best heard at the left second intercostal space with radiation through to the back. Simultaneous bilateral radial artery blood gas analysis confirmed lower arterial oxygen saturation in the left arm compared with the right arm (Table 1). ECG showed right-axis deviation and severe right ventricular hypertrophy (Figure 2). Systemic arterial line blood pressure was 100/65 mm Hg. Right heart catheterization showed a right atrial pressure of 13 mm Hg, pulmonary arterial pressure of 92/60 mm Hg, mean pulmonary artery pressure of 71 mm Hg, pulmonary capillary wedge pressure of 10 mm Hg, and cardiac output of 3.8 L/min. Transthoracic echocardiogram confirmed right-to-left flow through the PDA. On right heart catheteriza-tion there was no significant step up in oxygen saturation from the main pulmonary artery to the branch vessels. The patient was continued on sildenafil and bosentan at the outpatient doses. The patient was started on intravenous treprostinil. On …