CONGRESS ABSTRACT

A 44-year-old woman presented to emergency department with dyspnea and anasarca. No past history of cardiologic diseases. Physical exam was notable for jugular turgor, hepatomegaly, rales and tachy-arrhythmic tones. Laboratory tests showed severe anemia (Hb 3.2 g/dl) and a severe mixed acidosis. ECG showed atrial fibrillation, 150/min. On echocardiogram left ventricle had normal size and systolic function while right chambers were dilated, hypokinetic (TAPSE 14 mm, RVFAC 30%), systo-diastolic D-shape, severe tricuspid regurgitation; high probability of pulmonary hypertension (VRT 3.2 m/s, and accessory signs). Angio-CT chest was performed, ruling out pulmonary embolism. She was admitted with a working diagnosis of right heart failure complicated by respiratory failure, intercurrent undated AF and severe pulmonary hypertension. On a routinal echo aorto-pulmonary shunt and ASD and coronary venous sinus dilatation were reavaled. A cardiac-CT was performed, for suspected congenital heart disease, and showed ASD sinus venosus (Fig.1), patency of Botallo ‘s duct (PDA), aortic coarctation (Fig.2) and persistence of left superior vena cava (PLSVC, type III) draining into coronary sinus (Fig. 3). The patient ‘s clinical condition deteriorated progressively until she died from cardiac arrest on the fifth day from the admission, preventing further investigations. Our patient was a so called GUCH with ASD and PDA resulting in group I (WHO) pulmonary hypertension due to a prevalent left – right shunt (Simonneau et al). The clinical presentation, on the other hand, could be due to the onset of atrial fibrillation leading to hemodynamic instability and thus reversal to right-to-left shunt (Eisenmerger ‘s Syndrome) with multiple organ involvment facilitated by anemia. In our case, it is unclear, as well as in the literature, whether PLSVC play a role in the development of pulmonary hypertension; further investigation (MRI, autopsy) would have allowed a better anatomo-functional characterisation excluding a possible unroofed coronaric sinus with left atrial drainage. In our opinion, it is important to recognize PLSVC not only for its implications during interventional procedures but as markers of associated congenital heart disease.