@Article{CHD.2020.011512,
AUTHOR = {Liesbeth van Dessel, Béatrice Santens, Els Troost, Pieter De Meester, Leen Roggen, Filip Rega, Bart Meyns, Bjorn Cools, Marc Gewillig, Philip Moons, Lukas Meier, Werner Budts, Alexander Van De Bruaene},
TITLE = {Pulmonary Hemodynamics and Outcome in a Large Cohort of Patients with  Sinus Venosus Septal Defect},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {15},
YEAR = {2020},
NUMBER = {2},
PAGES = {69--78},
URL = {http://www.techscience.com/schd/v15n2/39427},
ISSN = {3071-1738},
ABSTRACT = {<b>Background:</b> Left-to-right shunt in sinus venosus septal defect (SVSD) 
may affect resistive (pulmonary vascular resistance–PVR) and elastic (pulmonary 
artery compliance-PAC) pulmonary artery properties. This study aimed at evaluating 
(1) impact of age, (2) pulmonary hemodynamics, and (3) outcome in a large cohort 
of SVSD patients. <b>Methods:</b> This study included 136 patients with SVSD (median 
age at diagnosis 14 (IQR 5–48) years, 47% male) of which 87 underwent 
catheterization. Pressures were measured and cardiac output was evaluated using the 
Fick principle at diagnosis. PVR, PAC and their product (RC time) were calculated. 
<b>Results:</b> Surgical repair was performed in 128 (94%) at a median age of 13 (IQR 5–
43) years. During a median follow-up time of 31 (IQR 17–55) years, 12 (9%) patients 
died, 13 (10%) developed heart failure, 4 (3%) Eisenmenger syndrome, 19 (14%) 
atrial arrhythmia, 6 (4%) sick sinus syndrome and 7 (5%) required pacemaker 
implantation. In those who underwent catheterization, median shunt ratio was 2.5 
(IQR 2.0–2.9). Thirty (34%) had mean PA pressure ≥25 mmHg. PVR indexed, PAC 
indexed, and RC time was 3.5 (IQR 2.4–7.5) WU.m², 1.8 (IQR 1.3–2.5) 
mL/mmHg.m² and 0.39 (0.26–0.53) sec with an inverse hyperbolic relationship 
between PVR and PAC. Mean PA pressure (P < 0.0001); wedge pressure (P = 0.001), 
PVR indexed (P = 0.002) and PAC indexed (P = 0.002) changed significantly with 
age at diagnosis, but shunt ratio did not. <b>Conclusion:</b> SVSD has good long-term 
outcome, albeit with late morbidities. Thirty-four percent has mean PA pressure ≥25
mmHg, but Eisenmenger syndrome is rare (3%). PVR and PAC are inversely related 
and change significantly with older age.},
DOI = {10.32604/CHD.2020.011512}
}