Abstract

Abstract Background Altered lymphatic circulation could be an indicator of risk of complications after Fontan-Kreutzer surgery. Purpose To determine the degree of lymphatic circulation abnormality in adult patients who underwent the Fontan-Kreutzer procedure (FKP) and explore association between type of abnormality and complications during follow-up. Methods A retrospective observational study was conducted in patients (p) with grown-up congenital heart disease included on the GUTI-GUCH database. Lymphatic circulation of patients who had undergone FK procedure (FKP) was assessed using Cardiac Magnetic Resonance (CMRI). Lymphatic abnormalities were divided into four types according to the classification by Dori; type III and IV abnormalities were considered complex. Association between complex abnormalities and complications during follow-up was explored. Statistical analysis was performed using SPSS 22 software. Results One-hundred and thirty-eight (4.7%) of the 2920 p on the database had undergone FKP; the 47 patients (34%) who had had a CMRI for lymphatic assessment were included. Fifty-three percent (25p) were women; mean age was 26.5 years ± 7.8 years. Analysis of patient data showed the following prevalence of lymphatic abnormalities according to Dori's classification: Type I: 14p (29.8%); Type II: 17p (36.2%); Type III: 13p (27.7%); Type IV: 3p (6.4%); severe/complex Types III+IV: 16p (34%). Prevalence of the studied complications was as follows: presence of ascites: 6p (12.8%); aortopulmonary collaterals: 7p (14.9%); NYHA functional classification more than II: 19p (40.4%); ventricular dysfunction: 14p (29.8%); tube mismatch: 11p (23.4%); moderate or severe AV valve dysfunction: 11p (23.4%); left-type ventricle: 30p (63.8); congestive heart failure (CHF): 4p (8.5%); arrhythmias: 16p (34%) hospitalization: 7p (14.9%); use of furosemide: 11p (23.9%); use of spironolactone: 25p (53.2%); use of IECA 38p (80.9%); and use of beta blockers: 28p (59.6%). The presence of type III+IV abnormalities (Dori) observed in 16p (34%) was associated with ascites p=0.006 R: 13 (1.4–130). No significant correlation was observed between type of lymphatic abnormality and the remaining studied complications. Conclusions One third of patients had complex lymphatic circulation abnormalities. The presence of severe complications was significantly associated with the presence of ascites, a late sign of dysfunction of the Fontan-Kreutzer system. Lymphatic circulation abnormalities are very frequent in patients who have undergone a Fontan-Kreutzer procedure. Further studies are necessary to determine their prognostic value. Funding Acknowledgement Type of funding sources: None.

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