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- Daene, M, et al.
(författare)
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Outcome of Down patients with repaired versus unrepaired atrioventricular septal defect
- 2023
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Ingår i: International Journal of Cardiology Congenital Heart Disease. - : Elsevier BV. - 2666-6685. ; 12
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Tidskriftsartikel (refereegranskat)abstract
- Background and aims Patients with Down Syndrome (DS) are frequently born with an atrioventricular septal defect (AVSD). Surgical repair of the defect aims to minimize mortality and morbidity. However, a surgical intervention, specifically in DS patients, is not without risk and a subgroup of patients underwent only conservative non-surgical treatment. Outcome data of these different approaches are scarce. The aim of this retrospective study was to compare the long-term outcome of DS patients with and without surgery for AVSD. Methods DS patients registered with AVSD in the hospital's database from January 1980 till December 2020 were selected. Patient characteristics, peri-operative if appropriate, and follow-up data were obtained from the medical files. Results In total, 72 unrepaired (36 male, 50%) and 134 repaired patients (61 male, 46%) were included. After a maximum of 60 years of follow-up, the all-cause mortality was 45.8% and 17.1%, respectively. Thirty-six percent and 13%, respectively, were labeled as non-cardiovascular death. Mean survival time for unrepaired AVSD was 40.7 years (95% CI 36.1–45.2) and for repaired AVSD 38.5 years (95% CI 35.3–41.6) (Log rank p = 0.465). However, the survival rate 35 years after birth was 62.1% for unrepaired patients versus 81.7% for repaired patients. Mortality rates were the highest the first months after surgical repair. Conclusions The mean survival rate of Down patients, born with an AVSD, did not differ between repair or not. However, long-term survival rate was higher in patients who underwent surgical repair. Mortality was highest the first months after surgery.
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| 2. |
- Hubrechts, J., et al.
(författare)
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Case Report: Disseminated Systemic Embolism of Lipiodol After Lymphography for Plastic Bronchitis After Fontan Repair
- 2020
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Ingår i: Frontiers in Pediatrics. - : Frontiers Media SA. - 2296-2360. ; 8
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Tidskriftsartikel (refereegranskat)abstract
- Lipiodol-based lymphangiography is not only a diagnostic tool for visualization of lymphatic disorders such as plastic bronchitis (PB), but also aims a therapeutic effect by embolizing lymph leakages. We performed such percutaneous lymphatic embolization for PB in a Fontan patient with proven absence of right-to-left shunt, and demonstrated important lymphatic abnormalities in the mediastinum. Shortly after the procedure, the patient developed severe convulsive seizures, revealing multiple cerebral embolisms of Lipiodol. Radiological images were impressive, yet the clinical neurological outcome was favorable. Lipiodol-based lymphography in Fontan patients with plastic bronchitis should be avoided as this subgroup is more likely to have developed lympho-pulmonary venous connections which allow systemic emboli.
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| 3. |
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| 4. |
- van Dessel, L., et al.
(författare)
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Pulmonary Hemodynamics and Outcome in a Large Cohort of Patients with Sinus Venosus Septal Defect
- 2020
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Ingår i: Congenital Heart Disease. - : Computers, Materials and Continua (Tech Science Press). - 1747-079X. ; 15:2, s. 69-78
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Tidskriftsartikel (refereegranskat)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.m(2) , 1.8 (IQR 1.3-2.5) mL/mmHg.m(2) 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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