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ARTICLE
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:
Congenital Heart Disease 2020, 15(2), 69-78. https://doi.org/10.32604/CHD.2020.011512
Received 13 May 2020; Accepted 11 June 2020; Issue published 23 June 2020
Abstract
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.
Keywords
Cite This Article
APA Style
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. https://doi.org/10.32604/CHD.2020.011512
Vancouver Style
Dessel LV, Santens B, Troost E, Meester PD, Roggen L, Rega F, et al. Pulmonary hemodynamics and outcome in a large cohort of patients with sinus venosus septal defect. Congeni Heart Dis. 2020;15(2):69-78 https://doi.org/10.32604/CHD.2020.011512
IEEE Style
L.V. Dessel et al., "Pulmonary Hemodynamics and Outcome in a Large Cohort of Patients with Sinus Venosus Septal Defect," Congeni. Heart Dis., vol. 15, no. 2, pp. 69-78. 2020. https://doi.org/10.32604/CHD.2020.011512