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• W4226272951 abstract "Purpose Development of chronic kidney disease is well described in patients with complex congenital heart disease such as palliated single ventricle patients. It is also well known that presence of kidney disease contributes to morbidity and mortality in these patients. However, there is no study if the progression of kidney disease in these patients necessitates kidney transplantation and risk factors associated with need for kidney transplantation (KTX). Methods A retrospective cohort study of patients undergoing single ventricle palliation in the Pediatric Cardiac Care Consortium (PCCC), a large US-based registry. Outcomes and transplants were captured in the PCCC and by linkage with the Organ Procurement Transplant Network (OPTN) through 2019. Results Between 1982 -2019, 7751 patients with SV palliation were seen at US centers and 6385 patients had identifiers allowing appropriate linkage with OPTN database. Of these, 14 patients (0.22%) underwent KTX. Patients undergoing KTX were predominantly male (71%), white race (64.3%) and underwent KTX at a mean age of 21 (±9) years, mean time to KTX from last cardiac surgery 15.2 (±8.7) years. Cardiac diagnostic group was systemic right ventricle in 8/14 (57%). At follow-up, 11/14 (78%) were alive while 3/14 (22%) died at a mean age of 29 (±10.6) years. There were no differences in the demographic and diagnosis between those who received KTX versus those who did not. However, at follow up only 50.4% of patients without KTX were alive compared to 78.6% in the KTX group (p=0.03) while the mean age at death was 1 (0, 14) years in non-KTX group compared to 33.0 (17.0, 37.0) years in the KTX group (p= 0.011). Two patients needed kidney transplantation after heart transplantation. Conclusion First of its kind study shows that kidney transplantation is rarely performed in patients with single ventricle palliation although kidney disease is well documented in this population. This study findings potentially reflect selection bias and survival bias. We plan to elucidate the same further. Need for KTX appears to be higher in males with systemic right ventricle anatomy in the second or early third decade. Given that there is high mortality in the overall cohort, and given improved survival of patients undergoing combined heart kidney transplantation, further studies should focus on patient selection and early intervention. Development of chronic kidney disease is well described in patients with complex congenital heart disease such as palliated single ventricle patients. It is also well known that presence of kidney disease contributes to morbidity and mortality in these patients. However, there is no study if the progression of kidney disease in these patients necessitates kidney transplantation and risk factors associated with need for kidney transplantation (KTX). A retrospective cohort study of patients undergoing single ventricle palliation in the Pediatric Cardiac Care Consortium (PCCC), a large US-based registry. Outcomes and transplants were captured in the PCCC and by linkage with the Organ Procurement Transplant Network (OPTN) through 2019. Between 1982 -2019, 7751 patients with SV palliation were seen at US centers and 6385 patients had identifiers allowing appropriate linkage with OPTN database. Of these, 14 patients (0.22%) underwent KTX. Patients undergoing KTX were predominantly male (71%), white race (64.3%) and underwent KTX at a mean age of 21 (±9) years, mean time to KTX from last cardiac surgery 15.2 (±8.7) years. Cardiac diagnostic group was systemic right ventricle in 8/14 (57%). At follow-up, 11/14 (78%) were alive while 3/14 (22%) died at a mean age of 29 (±10.6) years. There were no differences in the demographic and diagnosis between those who received KTX versus those who did not. However, at follow up only 50.4% of patients without KTX were alive compared to 78.6% in the KTX group (p=0.03) while the mean age at death was 1 (0, 14) years in non-KTX group compared to 33.0 (17.0, 37.0) years in the KTX group (p= 0.011). Two patients needed kidney transplantation after heart transplantation. First of its kind study shows that kidney transplantation is rarely performed in patients with single ventricle palliation although kidney disease is well documented in this population. This study findings potentially reflect selection bias and survival bias. We plan to elucidate the same further. Need for KTX appears to be higher in males with systemic right ventricle anatomy in the second or early third decade. Given that there is high mortality in the overall cohort, and given improved survival of patients undergoing combined heart kidney transplantation, further studies should focus on patient selection and early intervention." @default.
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• W4226272951 date "2022-04-01" @default.
• W4226272951 modified "2023-09-26" @default.
• W4226272951 title "Kidney Transplantation in Single Ventricle Palliated Patients: A Pediatric Cardiac Care Consortium Study" @default.
• W4226272951 doi "https://doi.org/10.1016/j.healun.2022.01.1260" @default.
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