scholarly journals Neutrophil-to-Lymphocyte and Platelet-to-Lymphocyte Ratio in Univentricular Patients From Birth to Follow-Up After Fontan—Predicting Lymphatic Abnormalities

2021 ◽  
Vol 9 ◽  
Author(s):  
Julia Moosmann ◽  
Christian Schroeder ◽  
Robert Cesnjevar ◽  
Kathrin Rottermann ◽  
Annika Weigelt ◽  
...  
Keyword(s):  
Fontan Operation ◽  
Lymphatic Malformations ◽  
Protein Losing Enteropathy ◽  
Lymphocyte Ratio ◽  
Lymphatic Abnormalities ◽  
Grade Classification ◽  
Magnetic Resonance Imaging Mri ◽  
Fontan Surgery ◽  
Lymphatic Imaging

Background: Reliable laboratory parameters identifying complications after Fontan surgery including the lymphatic abnormalities and the development of protein-losing enteropathy (PLE) are rare. Neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocte ratio (PLR) are inflammatory markers and have been studied to predict outcome and prognosis in various diseases. The aim of this study was to investigate NLR and PLR from birth to follow-up after Fontan and evaluate their use as prognostic parameters for single ventricle patients regarding the development of lymphatic malformations during follow-up.Materials and Methods: Sixty-six univentricular patients who underwent Fontan surgery and had 6-month follow-up magnetic resonance imaging (MRI) with T2 weighted lymphatic imaging after total cavopulmonary connection (TCPC) surgery were included in the study. NLR and PLR were determined at specific time points, from neonatal age to follow-up after Fontan operation and correlated to data from the MRI 6 months after Fontan.Results: NLR and PLR increase significantly over time from the first surgery during infancy to the follow-up after Fontan (both p < 0.0001), with a significant increase after the Glenn surgery for both ratios (each p < 0.0001). Higher NLR (p = 0.002) and higher PLR (p = 0.004) correlated with higher-grade classification of lymphatic abnormalities in T2-weighted imaging 6 months after Fontan surgery and higher NLR correlated with higher transpulmonary gradient prior to Fontan surgery (p = 0.035) Both ratios showed a significant correlation to total protein at follow-up (NLR p = 0.0038; PLR<0.0001).Conclusion: Increased NLR and PLR correlate with higher degree lymphatic malformations after TCPC and therefore might contribute as valuable additional biomarker during follow-up after TCPC. NLR and PLR are simple, inexpensive and easily available parameters to complement diagnostics after TCPC.

Impact of the Fontan Operation on Organ Systems

2019 ◽  
Vol 19 (3) ◽  
pp. 205-214
Author(s):  
Miriam Michel ◽  
Manuela Zlamy ◽  
Andreas Entenmann ◽  
Karin Pichler ◽  
Sabine Scholl-Bürgi ◽  
...  
Keyword(s):  
Systematic Study ◽  
Fontan Operation ◽  
Gastrointestinal System ◽  
Adjunct Treatment ◽  
Organ Systems ◽  
Fontan Surgery ◽  
Fontan Patients

: In patients having undergone the Fontan operation, besides the well discussed changes in the cardiac, pulmonary and gastrointestinal system, alterations of further organ systems including the hematologic, immunologic, endocrinological and metabolic are reported. As a medical adjunct to Fontan surgery, the systematic study of the central role of the liver as a metabolizing and synthesizing organ should allow for a better understanding of the pathomechanism underlying the typical problems in Fontan patients, and in this context, the profiling of endocrinological and metabolic patterns might offer a tool for the optimization of Fontan follow-up, targeted monitoring and specific adjunct treatment.

Long-term outcome after atrioventricular valve surgery following modified Fontan operation

2010 ◽  
Vol 21 (1) ◽  
pp. 83-88 ◽  
Author(s):  
Shaji C. Menon ◽  
Joseph A. Dearani ◽  
Frank Cetta
Keyword(s):  
Ventricular Function ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Valve Surgery ◽  
Atrioventricular Valve ◽  
Term Outcome ◽  
Protein Losing Enteropathy ◽  
Long Term Outcome

AbstractObjectiveThe objective of this study was to evaluate the early and late results of atrioventricular valve surgery after Fontan operation.BackgroundAtrioventricular valve regurgitation is a known perioperative risk factor for Fontan operation. There are limited data on the outcomes of late atrioventricular valve surgery following Fontan operation.MethodsPatients who underwent atrioventricular valve surgery following Fontan procedure were identified from the Mayo Clinic Fontan database. Medical records were reviewed for pre-operative, operative, and post-operative clinical and haemodynamic data. All patients not known to be deceased were sent health status questionnaires.ResultsA total of 61 patients (28 females) underwent atrioventricular valve surgery following Fontan procedure. The median age at atrioventricular valve surgery was 14 years. The median duration between Fontan and atrioventricular valve surgery was 4.7 years. Median follow-up was 9 years. There were a total of 32 (52%) deaths with 8 (13%) within 30 days of surgery. The 5-, 10-, and 15-year survival rates were 67%, 57%, and 45%, respectively. On follow-up, 44 of 61 (72%) had arrhythmias, 21 of 29 (72%) were symptomatic, and 12 of 61 (20%) developed protein-losing enteropathy. On multivariate analysis, reduced ventricular function and development of protein-losing enteropathy were associated with decreased survival.ConclusionAtrioventricular valve surgery after Fontan procedure is associated with substantial late morbidity and mortality. Atrioventricular valve surgery in this cohort of patients portends poor long-term outcome and is associated with a high incidence of protein-losing enteropathy. Reduced ventricular function and development of protein-losing enteropathy were associated with decreased survival.

(AVK) Anti Vitamin K Treatment In patients with Fontan Surgery

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4397-4397
Author(s):  
Maria Stefania Infante ◽  
Cristina Pascual Izquierdo ◽  
Fernando Carretero Lopez ◽  
Jose Manuel Sanchez Ramirez ◽  
Ana Maria Rodriguez Huerta ◽  
...  
Keyword(s):  
Pediatric Patients ◽  
Fontan Operation ◽  
Thrombotic Event ◽  
Bleeding Complications ◽  
Target Range ◽  
Group A ◽  
Group B ◽  
Fontan Surgery ◽  
Fontan Patients

Abstract Abstract 4397 OBJECTIVES Thrombotic events have been reported as a major cause of morbidity after the Fontan procedure. There is no consensus about the type and duration of postoperative anticoagulation prophylaxis, due to the high risk of bleeding complications, the difficulties in monitoring and the questionable therapeutic compliance in children. In spite of the lack of long term prospective studies in this situation, the ACCP has recommended in their guidelines OAT following Fontan or Glenn operation for at least 6 months. This has also been our practice in our institution during the period of study. AIM OF THE STUDY To analyze the efficacy and complications of OAT in our pediatric patients after undergoing the Fontan operation. METHODS Retrospective chart review of all the children treated with OAT in our institution between 1995 and 2009. All patients were treated initially with acenocumarol 0,2 mg/kg, except the Fontan patients, who received 0,1 mg/kg. Target INR was 2–3 for all patients. The Mann-Whitney test was used to compare the rate of complications, and the percentage of visits out of target INR between the Fontan patients and the rest of the cohort. RESULTS There were 61 children (26 female/35 males) aged between 1 month and 17 years, who received OAT with a range of follow up between 4 months and 14 years: 27 after Fontan operation (Group A), an 34 for other reasons (Group B: n=13 non prothetic valve cardiopathy, n=21 treatment of thromboembolic disease). The average follow-up was similar in both groups (median of 6.5 months in group A vs.7.5 months in group B). There were few complications: 1 mild epistaxis and 1 thrombotic event in group B, and none in group A. There were no differences in the proportion of controls in normal range between both groups; there was a moderate proportion of controls outside the target range of INR, with higher distribution below the range than above the range. The median dose used to achieve the target INR was 0.3 mg/kg/d in Group A and 0.4 mg/kg/d in Group B. CONCLUSIONS Oral Anticoagulant therapy is safe and effective in pediatric patients,with very low rates of thrombotic or hemorragic complications including those undergoing the Fontan surgery. There were not any differences between both groups in any of the analizyed parameters. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Outcomes following modified extracardiac Fontan procedure of direct total cavopulmonary connection with autologous vessels: a single-centre 10-year experience

2019 ◽  
Author(s):  
Xiaobing Liu ◽  
Haiyun Yuan ◽  
Jimei Chen ◽  
Jianzheng Cen ◽  
Zhiqiang Nie ◽  
...  
Keyword(s):  
Fontan Operation ◽  
Growth Potential ◽  
Total Cavopulmonary Connection ◽  
Long Term Outcomes ◽  
Protein Losing Enteropathy ◽  
Significant Difference ◽  
Cavopulmonary Connection ◽  
Extracardiac Fontan

AbstractOBJECTIVESThe modified extracardiac Fontan of direct total cavopulmonary connection (dTCPC) with entirely autologous vessels is a better solution in selected patients with functionally univentricular hearts because it combines the advantages of a tubular connection and of full growth potential. We investigated the mid-term to long-term outcomes of the physical condition of patients who had the dTCPC and assessed the growth potential of the autologous dTCPC pathway.METHODSFrom July 2005 to June 2014, 31 patients, aged 1.8–14.0 years, underwent a modified extracardiac Fontan with dTCPC at our institution. Twenty-two patients underwent a 1-stage dTCPC and 9 patients underwent a 2-stage dTCPC. The preoperative and postoperative data were reviewed retrospectively.RESULTSThere were 17 (54.8%) boys and 14 (45.2%) girls, with a median age of 6.4 years (range 1.8–14.0 years) and a mean weight of 17.8 kg (range 9–41 kg). The mean follow-up period was 6.0 years (range 2.1–10.2 years). There was 1 early death and 2 late deaths. The event-free survival rate for the 31 patients was 88.9% at 10 years during the follow-up period. Late protein-losing enteropathy, thromboembolism, arrhythmia or heart failure were not observed. There was a significant difference between the preoperative and postoperative data for body mass index and the diameters of the autologous dTCPC pathway and pulmonary artery branches, confirming that the growth potential of the autologous dTCPC pathway was realized.CONCLUSIONSThe dTCPC procedure could be performed with satisfactory midterm to long-term outcomes in selected patients. It has the potential of retaining the advantage of the extracardiac Fontan operation together with the potential for growth and the avoidance of prosthetic materials.

Abstract 2972: Permanent Atrial Pacing Lead Implantation Route After Fontan Operation

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Kazuhiro Takahashi ◽  
Frank Cecchin ◽  
Elizabeth B Fortescue ◽  
Charles I Berul ◽  
Mark E Alexander ◽  
...  
Keyword(s):  
Thromboembolic Event ◽  
Sinus Node ◽  
Fontan Operation ◽  
Atrial Pacing ◽  
Retained Foreign Body ◽  
Pacing Lead ◽  
Fontan Surgery ◽  
Skin Erosion ◽  
Fontan Patients

Background: Atrial pacing is commonly used for sinus node dysfunction (SND) after Fontan surgery. The preferred route of lead implant has been debated. We compare procedural and clinical outcomes of transvenous (TV) and epicardial (Epi) atrial lead implants in this population. Methods: All Fontan patients having an atrial lead implant without other associated surgery between 1992 and 2007 were studied. Demographics, pacing lead performance data and procedural outcome were retrospectively analyzed. Results: 78 patients (22 TV and 56 Epi) had 90 leads implanted (25 TV/ 65 Epi). Mean follow-up was 3.3±4.1 yrs (TV) and 4.4±3.6 yrs (Epi). TV leads were implanted in older patients (mean age: 23.9 vs. 13.8 yrs, p<0.001), at longer interval after Fontan surgery (mean: 15.1 vs. 5.7 yrs, p<0.001). Indication for TV was more likely to be SND, while Epi pacing was indicated for AV block. Pacing modes were exclusively AAI/AAI-T in TV and mostly DDD in Epi leads. Acute complication occurred in 2/25 (8%) of TV (pneumothorax n=1, skin erosion n=1) and 12/65 (19%, p=0.23) of Epi implants (effusions n=5, heart failure n=1, retained foreign body n=1, pneumothorax n=1, hematoma n=1, disconnection n=1, sepsis n=1, blood loss n=1). Median hospital stay was shorter in TV (2.0 vs 4.5 days, p=0.03). All TV patients and 43 Epi (77%) were anticoagulated. At follow-up, pocket infection occurred in one Epi patient. No clinical thromboembolic event was observed in either group. 3 TV leads failed in 2 patients (9%), while 12 Epi leads failed in 10 patients (18%); however, mean duration of freedom from lead failure was not significantly different (TV 9.9 vs. Epi 8.0 yrs, P=ns). The mean energy threshold was lower at implant for TV leads (0.9 vs 2.2 μJ, P=0.049), but similar for both leads on follow-up (1.2 vs 2.6 μJ, P=0.35). Atrial sensing was unchanged over time for TV (2.2 to 2.0 mV, P=ns), but significantly decreased in Epi (3.4 to 2.4 mV, p=0.006). Conclusions: Transvenous atrial pacing leads may be placed in Fontan patients with lower procedural morbidity than epicardial leads, and equivalent expectation of lead performance and longevity. Although the thromboembolic event rate appears low in anticoagulated patients with both lead types, the present study design cannot fully address this important issue.

scholarly journals Long-term therapeutic effect of Fontan conversion with an extracardiac conduit

2019 ◽  
Vol 57 (5) ◽  
pp. 951-957 ◽  
Author(s):  
Takaya Hoashi ◽  
Masatoshi Shimada ◽  
Kenta Imai ◽  
Motoki Komori ◽  
Kenichi Kurosaki ◽  
...  
Keyword(s):  
Survival Rate ◽  
Therapeutic Effect ◽  
Survival Curve ◽  
Preoperative Evaluation ◽  
Fontan Operation ◽  
Actuarial Survival ◽  
Protein Losing Enteropathy ◽  
Extracardiac Conduit

Abstract OBJECTIVES The aim of this study was to identify the long-term therapeutic effect of total cavopulmonary connection (TCPC) conversion with an extracardiac conduit. METHODS Between 1991 and 2014, 36 patients underwent TCPC conversion with an extracardiac conduit. Half of these patients were diagnosed with tricuspid atresia or its variant. The left ventricle was dominant in 26 patients (72.2%). Median age at conversion and interval from initial Fontan operation to conversion were 24.1 years (interquartile range 18.9–29.2) and 17.8 years (15.4–20.9), respectively. Surgical cryoablation was concomitantly performed in 32 patients (88.9%). Cardiac catheter examination was performed preoperatively (36 patients, 100%) and at 1 year (31 patients, 86%), 5 years (25 patients, 69%) and 10 years (13 patients, 36%) after TCPC conversion. Symptom-limited treadmill exercise with expired gas analysis was performed preoperatively (32 patients, 88.9%) and at 1 year (27 patients, 75.0%), 5 years (20 patients, 55.6%) and 10 years (12 patients, 33.3%) after conversion. RESULTS All patients received follow-up; the mean follow-up period was 8.2 ± 4.8 years. Actuarial survival rate, protein-losing enteropathy-free survival rate and rate of survival with sinus rhythm maintenance at 10 years were 79.2%, 67.8% and 48.5%, respectively. The survival curve declined steeply when the duration of Fontan circulation exceeded 25 years. New cases of protein-losing enteropathy developed postoperatively in 2 patients. Permanent pacemakers were implanted in 12 patients (33%), but atrial tachyarrhythmia was not sustained in any of the remaining patients. Pulmonary arterial pressure (11.0 ± 3.1 to 9.5 ± 3.6 mmHg, P = 0.003), pulmonary vascular resistance (2.1 ± 0.7 to 1.3 ± 0.5 WU/m2, P &lt; 0.0001) and cardiac index (2.0 ± 0.3 to 2.9 ± 0.6 l/min/m2, P &lt; 0.0001) significantly improved from preoperative evaluation to 1 year after the conversion, and these improvements were maintained during the entire follow-up period. Peak oxygen uptake remained unchanged from the preoperative evaluation (49.7 ± 11.5% predicted) to 1 year (52.5 ± 12.0%), 5 years (56.2 ± 9.6%) and 10 years (51.2 ± 9.4%) after conversion (P = 0.19). CONCLUSIONS Owing to its anti-arrhythmic effect and Fontan pathway recruitment effect, TCPC conversion with an extracardiac conduit prevented the natural decline of exercise tolerance that is seen in classic Fontan patients.

Desmoid tumour of the distal forearm involving the distal radioulnar joint

2020 ◽  
Vol 13 (11) ◽  
pp. e237097
Author(s):  
Apoorv Sehgal ◽  
Pratyush Shahi ◽  
Avijeet Prasad ◽  
Manoj Bhagirathi Mallikarjunaswamy
Keyword(s):  
Distal Radioulnar Joint ◽  
Treatment Modalities ◽  
Desmoid Tumour ◽  
X Rays ◽  
Distal Forearm ◽  
Left Wrist ◽  
Radioulnar Joint ◽  
Magnetic Resonance Imaging Mri ◽  
Epidemiology Investigation

A 32-year-old woman presented with progressive pain and swelling of the left wrist for 6 months. Physical examination revealed a firm, tender, oval swelling over the left wrist. X-rays showed a pressure effect on the distal radius and ulna. Magnetic Resonance Imaging (MRI) revealed a well-defined, asymmetrical, dumbbell-shaped soft-tissue lesion involving the interosseous region of the distal forearm and extending until the distal radioulnar joint (DRUJ). Core needle biopsy confirmed the diagnosis of desmoid tumour. Marginal excision of the tumour was done. At the 2-year follow-up, the patient was doing well and had painless and improved left wrist motion. Desmoid tumour involving the DRUJ has not been previously reported. We, through this case, report new observation and discuss the epidemiology, investigation of choice, treatment modalities, and the need for a regular follow-up for appendicular desmoid tumours.

scholarly journals Brain atrophy and clinical characteristics predicting SDMT performance in multiple sclerosis: A 10-year follow-up study

2021 ◽  
Vol 7 (1) ◽  
pp. 205521732199239
Author(s):  
Cecilie Jacobsen ◽  
Robert Zivadinov ◽  
Kjell-Morten Myhr ◽  
Turi O Dalaker ◽  
Ingvild Dalen ◽  
...  
Keyword(s):  
Clinical Data ◽  
Grey Matter Volume ◽  
Lesion Volume ◽  
Information Processing Speed ◽  
Disability Status ◽  
Mri Scans ◽  
Patients At Risk ◽  
Magnetic Resonance Imaging Mri ◽  
Specific Volumes

Objectives To identify Magnetic Resonance Imaging (MRI), clinical and demographic biomarkers predictive of worsening information processing speed (IPS) as measured by Symbol Digit Modalities Test (SDMT). Methods Demographic, clinical data and 1.5 T MRI scans were collected in 76 patients at time of inclusion, and after 5 and 10 years. Global and tissue-specific volumes were calculated at each time point. For the primary outcome of analysis, SDMT was used. Results Worsening SDMT at 5-year follow-up was predicted by baseline age, Expanded Disability Status Scale (EDSS), SDMT, whole brain volume (WBV) and T2 lesion volume (LV), explaining 30.2% of the variance of SDMT. At 10-year follow-up, age, EDSS, grey matter volume (GMV) and T1 LV explained 39.4% of the variance of SDMT change. Conclusion This longitudinal study shows that baseline MRI-markers, demographic and clinical data can help predict worsening IPS. Identification of patients at risk of IPS decline is of importance as follow-up, treatment and rehabilitation can be optimized.

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