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Pulmonary Hemodynamics and Outcome in a Large Cohort of Patients with Sinus Venosus Septal Defect

Liesbeth van Dessel1, Béatrice Santens2, Els Troost2, Pieter De Meester2,3, Leen Roggen2, Filip Rega3,4, Bart Meyns3,4, Bjorn Cools5, Marc Gewillig5, Philip Moons6,7,8, Lukas Meier9, Werner Budts2,3, Alexander Van De Bruaene2,3,*

1 Faculty of Medicine, KU Leuven, Department of Internal Medicine, KU Leuven, Leuven, 3000, Belgium
2 Division of Structural and Congenital Cardiology, University Hospitals Leuven, Leuven, 3000, Belgium
3 Department of Cardiovascular Sciences, KU Leuven, Leuven, 3000, Belgium
4 Division of Cardiac Surgery, University Hospitals Leuven, Leuven, 3000, Belgium
5 Division of Pediatric Cardiology, University Hospitals Leuven, Leuven, 3000, Belgium
6 Department of Public Health and Primary Care, KU Leuven, Leuven, 3000, Belgium
7 Institute of Health and Care Science, University of Gothenburg, Göteborg, 41346, Sweden
8 Department of Paediatrics and Child Health, University of Cape Town, Cape Town, 7700, South Africa
9 Adult Congenital Heart Disease Program, University Heart Center, Zurich, Switzerland

* Corresponding Author: Alexander Van De Bruaene. Email: email

Congenital Heart Disease 2020, 15(2), 69-78.


Background: 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. Methods: 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. Results: 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. Conclusion: 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.


Cite This Article

Dessel, L. V., Santens, B., Troost, E., Meester, P. D., Roggen, L. et al. (2020). Pulmonary Hemodynamics and Outcome in a Large Cohort of Patients with Sinus Venosus Septal Defect. Congenital Heart Disease, 15(2), 69–78.

cc This work is licensed under a Creative Commons Attribution 4.0 International License , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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