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 cyanosis, 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 catheterization 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 day 4, the patient was successfully extubated, stabilized, and transferred to the cardiology floor service for further optimization and titration of prostacyclins. During this time period, the patient was referred for cardiac computed tomography and cardiac magnetic resonance imaging to assess size and shunt fraction, as well as direction of flow across the PDA. Cardiac computed tomography showed marked enlargement of the pulmonary artery with a large PDA. This was seen in both axial and reconstructed images (Figure 3 and Movie I in the online-only Data Supplement). Cardiac magnetic resonance imaging using a gradient echo imaging sequence and gadolinium contrast (MultiHance, Bracco Diagnostics, Monroe Township, NJ) demonstrated right-to-left shunting of blood through the PDA. First-pass perfusion imaging (Figure 4 and Movie II in the online-only Data Supplement) demonstrated initial flow of contrasted blood into the right heart followed by the pulmonary artery and then the descending aorta. The aortic root and ascending aorta fill only after flow of blood into the left heart and across the aortic valve. The shunt fraction from right to left through the PDA was calculated at 1.43 (Table 2). Over the course of the 2-week hospitalization, intravenous treprostinil was uptitrated to target doses, and the patient was discharged from the hospital with outpatient follow-up.
This is a unique case because it is unusual for PDA1 to remain untreated and to result in pulmonary arterial hypertension.2 The development of Eisenmenger syndrome from PDA is uncommon, and in this case the right-to-left shunting is well visualized through the use of cardiac magnetic resonance imaging.3 The patient is currently tolerating triple pulmonary vasodilator therapy and is being evaluated for lung transplantation.
The online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.114.013810/-/DC1.
- © 2015 American Heart Association, Inc.