Open Access

ARTICLE

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

by Liesbeth van Dessel¹, Béatrice Santens², Els Troost², Pieter De Meester^2,3, Leen Roggen², Filip Rega^3,4, Bart Meyns^3,4, Bjorn Cools⁵, Marc Gewillig⁵, Philip Moons^6,7,8, Lukas Meier⁹, Werner Budts^2,3, Alexander Van De Bruaene^2,3,*

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.

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