Long-Term Renal Involvement in Association with Fontan Circulation
© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature..
Multiorgan dysfunction is a concern of Fontan patients. To clarify the pathophysiology of Fontan nephropathy, we characterize renal disease in the long-term observational study. Medical records of 128 consecutive Fontan patients [median age: 22 (range 15-37) years old] treated between 2009 and 2018 were reviewed to investigate the incidence of nephropathy and its association with other clinical variables. Thirty-seven patients (29%) showed proteinuria (n = 34) or < 90 mL/min/1.73 m2 of estimated glomerular filtration rate (eGFR) (n = 7), including 4 overlapping cases. Ninety-six patients (75%) had liver dysfunction (Forns index > 4.21). Patients with proteinuria received the Fontan procedure at an older age [78 (26-194) vs. 56 (8-292) months old, p = 0.02] and had a higher cardiac index [3.11 (1.49-6.35) vs. 2.71 (1.40-4.95) L/min/m2, p = 0.02], central venous pressure [12 (7-19) vs. 9 (5-19) mmHg, p < 0.001], and proportion with > 4.21 of Forns index (88% vs. 70%, p = 0.04) than those without proteinuria. The mean renal perfusion pressure was lower in patients with a reduced eGFR than those without it [55 (44-65) vs. 65 (45-102) mmHg, p = 0.03], but no other variables differed significantly. A multivariable analysis revealed that proteinuria was associated with an increased cardiac index (unit odds ratio 2.02, 95% confidence interval 1.12-3.65, p = 0.02). Seven patients with severe proteinuria had a lower oxygen saturation than those with no or mild proteinuria (p = 0.01, 0.03). Proteinuria or a decreased eGFR differentially occurred in approximately 30% of Fontan patients. Suboptimal Fontan circulation may contribute to the development of proteinuria and reduced eGFR.
| Medienart: |
E-Artikel |
|---|
| Erscheinungsjahr: |
2024 |
|---|---|
| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:45 |
|---|---|
| Enthalten in: |
Pediatric cardiology - 45(2024), 2 vom: 15. Jan., Seite 340-350 |
| Sprache: |
Englisch |
|---|
| Beteiligte Personen: |
Muraoka, Mamoru [VerfasserIn] |
|---|
| Links: |
|---|
| Themen: |
Fontan |
|---|
| Anmerkungen: |
Date Completed 29.01.2024 Date Revised 29.01.2024 published: Print-Electronic Citation Status MEDLINE |
|---|
| doi: |
10.1007/s00246-023-03334-3 |
|---|
| funding: |
|
|---|---|
| Förderinstitution / Projekttitel: |
|
| PPN (Katalog-ID): |
NLM364581085 |
|---|
| LEADER | 01000caa a22002652 4500 | ||
|---|---|---|---|
| 001 | NLM364581085 | ||
| 003 | DE-627 | ||
| 005 | 20240129231947.0 | ||
| 007 | cr uuu---uuuuu | ||
| 008 | 231226s2024 xx |||||o 00| ||eng c | ||
| 024 | 7 | |a 10.1007/s00246-023-03334-3 |2 doi | |
| 028 | 5 | 2 | |a pubmed24n1274.xml |
| 035 | |a (DE-627)NLM364581085 | ||
| 035 | |a (NLM)37966520 | ||
| 040 | |a DE-627 |b ger |c DE-627 |e rakwb | ||
| 041 | |a eng | ||
| 100 | 1 | |a Muraoka, Mamoru |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Long-Term Renal Involvement in Association with Fontan Circulation |
| 264 | 1 | |c 2024 | |
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a ƒaComputermedien |b c |2 rdamedia | ||
| 338 | |a ƒa Online-Ressource |b cr |2 rdacarrier | ||
| 500 | |a Date Completed 29.01.2024 | ||
| 500 | |a Date Revised 29.01.2024 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature. | ||
| 520 | |a Multiorgan dysfunction is a concern of Fontan patients. To clarify the pathophysiology of Fontan nephropathy, we characterize renal disease in the long-term observational study. Medical records of 128 consecutive Fontan patients [median age: 22 (range 15-37) years old] treated between 2009 and 2018 were reviewed to investigate the incidence of nephropathy and its association with other clinical variables. Thirty-seven patients (29%) showed proteinuria (n = 34) or < 90 mL/min/1.73 m2 of estimated glomerular filtration rate (eGFR) (n = 7), including 4 overlapping cases. Ninety-six patients (75%) had liver dysfunction (Forns index > 4.21). Patients with proteinuria received the Fontan procedure at an older age [78 (26-194) vs. 56 (8-292) months old, p = 0.02] and had a higher cardiac index [3.11 (1.49-6.35) vs. 2.71 (1.40-4.95) L/min/m2, p = 0.02], central venous pressure [12 (7-19) vs. 9 (5-19) mmHg, p < 0.001], and proportion with > 4.21 of Forns index (88% vs. 70%, p = 0.04) than those without proteinuria. The mean renal perfusion pressure was lower in patients with a reduced eGFR than those without it [55 (44-65) vs. 65 (45-102) mmHg, p = 0.03], but no other variables differed significantly. A multivariable analysis revealed that proteinuria was associated with an increased cardiac index (unit odds ratio 2.02, 95% confidence interval 1.12-3.65, p = 0.02). Seven patients with severe proteinuria had a lower oxygen saturation than those with no or mild proteinuria (p = 0.01, 0.03). Proteinuria or a decreased eGFR differentially occurred in approximately 30% of Fontan patients. Suboptimal Fontan circulation may contribute to the development of proteinuria and reduced eGFR | ||
| 650 | 4 | |a Observational Study | |
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Fontan | |
| 650 | 4 | |a Glomerular filtration rate | |
| 650 | 4 | |a Hypoxemia | |
| 650 | 4 | |a Liver dysfunction | |
| 650 | 4 | |a Proteinuria | |
| 700 | 1 | |a Nagata, Hazumu |e verfasserin |4 aut | |
| 700 | 1 | |a Yamamura, Kenichiro |e verfasserin |4 aut | |
| 700 | 1 | |a Sakamoto, Ichiro |e verfasserin |4 aut | |
| 700 | 1 | |a Ishikita, Ayako |e verfasserin |4 aut | |
| 700 | 1 | |a Nishizaki, Akiko |e verfasserin |4 aut | |
| 700 | 1 | |a Eguchi, Yoshimi |e verfasserin |4 aut | |
| 700 | 1 | |a Fukuoka, Shoji |e verfasserin |4 aut | |
| 700 | 1 | |a Uike, Kiyoshi |e verfasserin |4 aut | |
| 700 | 1 | |a Nagatomo, Yusaku |e verfasserin |4 aut | |
| 700 | 1 | |a Hirata, Yuichiro |e verfasserin |4 aut | |
| 700 | 1 | |a Nishiyama, Kei |e verfasserin |4 aut | |
| 700 | 1 | |a Tsutsui, Hiroyuki |e verfasserin |4 aut | |
| 700 | 1 | |a Ohga, Shouichi |e verfasserin |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |t Pediatric cardiology |d 1993 |g 45(2024), 2 vom: 15. Jan., Seite 340-350 |w (DE-627)NLM012996831 |x 1432-1971 |7 nnns |
| 773 | 1 | 8 | |g volume:45 |g year:2024 |g number:2 |g day:15 |g month:01 |g pages:340-350 |
| 856 | 4 | 0 | |u http://dx.doi.org/10.1007/s00246-023-03334-3 |3 Volltext |
| 912 | |a GBV_USEFLAG_A | ||
| 912 | |a GBV_NLM | ||
| 951 | |a AR | ||
| 952 | |d 45 |j 2024 |e 2 |b 15 |c 01 |h 340-350 | ||