TY - EJOU
AU - Dessel, Liesbeth van
AU - Santens, Béatrice
AU - Troost, Els
AU - Meester, Pieter De
AU - Roggen, Leen
AU - Rega, Filip
AU - Meyns, Bart
AU - Cools, Bjorn
AU - Gewillig, Marc
AU - Moons, Philip
AU - Meier, Lukas
AU - Budts, Werner
AU - Bruaene, Alexander Van De
TI - Pulmonary Hemodynamics and Outcome in a Large Cohort of Patients with Sinus Venosus Septal Defect
T2 - Congenital Heart Disease
PY - 2020
VL - 15
IS - 2
SN - 1747-0803
AB - 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.
KW - Heart septal defects; atrial; pulmonary hypertension; heart failure; outcome
DO - 10.32604/CHD.2020.011512