Author + information
- Adam Obaidi,
- Whitney Pack and
- Pankaj Madan
Two defining features of Eisenmenger syndrome are “severely increased” and “irreversible” pulmonary vascular resistance as a result of uncorrected large left to right shunt. It is important to distinguish it from other causes of pulmonary hypertension as treatment may differ.
48 year old female with progressive dyspnea was referred to adult congenital cardiology clinic with a diagnosis of large ASD with Eisenmenger syndrome. Physical examination showed uncharacteristically absent clubbing or cyanosis despite a loud P2 and RV heave.
A TTE demonstrated an inter-atrial communication with poorly defined margins, severe tricuspid regurgitation, severe right-sided enlargement and RVSP 90 mmHg. TEE demonstrated a large distended PFO rather than a true ASD. Pulmonary etiology of elevated RVSP was suspected. A chest CT showed high-grade stenosis of both pulmonary arteries, with increased vessel wall thickness and circumferential abnormal soft tissue consistent with associated vasculitis. Hence, the elevated RVSP was likely a result of vasculitis, treatment of which is steroids and/or immunomodulator therapy rather than pulmonary vasodilator therapy.
Careful assessment coupled with multimodality imaging is helpful to distinguish between different etiologies of pulmonary hypertension and can affect the choice of therapy.
Poster Hall, Hall F
Saturday, March 16, 2019, 10:00 a.m.-10:45 a.m.
Session Title: FIT Clinical Decision Making: Congenital Heart Disease 1
Abstract Category: Congenital Heart Disease
Presentation Number: 1118-233
- 2019 American College of Cardiology Foundation