scholarly journals 610 Fontan associated kidney and liver disease: can we predict organ involvement with echocardiographic assessment of systolic function and atrioventricular valve insufficiency?

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Marta Rotella ◽  
Roberta Biffanti ◽  
Jolanda Sabatino ◽  
Davide Meneghesso ◽  
Nicola Bertazza Partigiani ◽  
...  
Keyword(s):  
Renal Function ◽  
Cystatin C ◽  
Single Ventricle ◽  
Systolic Function ◽  
Fontan Operation ◽  
Lateral Tunnel ◽  
Pearson's R ◽  
Valve Insufficiency ◽  
Fontan Patients ◽  
Pearson’S R

Abstract Aims Fontan operation represents the surgical palliative option for congenital heart disease with single ventricle physiology. With the improvement of surgical and percutaneous technique, we are facing a growing population of patients with an unique pathophysiology and potential complications. Methods and results Patients that underwent Fontan palliation in our centre between 1993 and 2016 were included in this prospective study. We excluded patients with major congenital renal anomalies, those that underwent cardiac transplantation, and redo-Fontan patients. All the subjects underwent clinical evaluation, laboratory exams with complete renal and hepatic function, transient hepatic elastography, and complete cardiac evaluation. We used Schwartz equation for estimating glomerular filtration rate in patients younger than 18 years, and CDK-EPI equation for adult patients. We enrolled 35 patients, 46% female (N = 16), and 54% male (N = 19). Medium age was 17 years old, median age 15 years old (range: 10–31 years old). Medium time from Fontan completion was 160 months (range: 57–340 months). Regarding to cardiac anatomy, 10 patients had functional single left ventricle (FSLV, 28.5%) and 21 a functional single right ventricle (FSRV, 60%); 4 patients had undetermined single ventricle (11.5%). Total cavo-pulmonary connection (TCPC) with intracardiac lateral tunnel was performed in 7 patients (20%, N = 7), whereas 28 patients had TCPC with external conduit (80%). Data from echocardiographic evaluation showed a medium EF established with Simpson’s method of 60% in patients with FSLV; patients with a FSRV or undetermined single ventricle had a medium FAC of 41.1%, with 15.1% having a reduced FAC < 35%. No FSLV patients had an EF < 50%. When using creatinine-based formula, data about renal function in our population showed a stage 2 chronic kidney disease (eGFR: 60–89 ml/min 1.73 mq) in 11% of total population (N = 4), that became 26% when using cystatin C-based equation (N = 9), with one patient showing a moderate reduced loss of kidney function (eGFR: 40–59 ml/min 1.73 mq). Urinalysis showed 29% (N = 10) of patients having microalbuminuria (microalbumin/creatinine ratio between 30 and 300 mg/g). Statistical analysis demonstrated a negative correlation between systolic function (TAPSE for FSRV) and cystatin C blood levels (Pearson’s R −0.428, P = 0.053), and between systolic function (FAC and Simpson) and microalbuminuria (Pearson’s R −0.414 with P = 0.049 and Pearson’s R −0.754 with P = 0.019, respectively). Transient elastography reported 10 patients (29.4%) with abnormal hepatic stiffness for Fontan patients. That condition appeared to be more frequent in patients with higher grade of AV valve insufficiency (P < 0.05). Conclusions Our population showed an higher prevalence of FSRV Fontan patients, with an expected lower systolic function compared with FSLV. 2D evaluation of systolic function showed a linear inverse correlation with renal function, suggesting that Fontan patients need a closer renal monitoring. Hepatic stiffness, which is a warning sign of potential hepatic cirrhosis need to be monitored in all Fontan patients, especially those with a worse AV valve insufficiency.

scholarly journals 613 Fontan associated nephropathy: does diastolic function play a role?

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Marta Rotella ◽  
Davide Meneghesso ◽  
Jolanda Sabatino ◽  
Roberta Biffanti ◽  
Nicola Bertazza Partigiani ◽  
...  
Keyword(s):  
Renal Function ◽  
Diastolic Function ◽  
Glomerular Filtration ◽  
Tubular Damage ◽  
Systemic Complications ◽  
Pearson's R ◽  
Fontan Patients ◽  
D Wave ◽  
Pearson’S R ◽  
Deceleration Time

Abstract Aims In Fontan patients, the pathophysiology of diastolic function and its relationship with systemic complications are still not well understood. Methods and results This is a prospective study including patients who underwent Fontan completion in our centre between 1993 and 2016. We excluded patients with major congenital renal anomalies, those who underwent cardiac transplantation and redo-Fontan patients. All the subjects underwent clinical evaluation, laboratory exams with complete renal and hepatic function, transient hepatic elastography, and complete cardiac evaluation. We used Schwartz equation for estimating glomerular filtration rate in patients younger than 18 years, and CDK-EPI equation for adult patients. We enrolled 35 patients, 46% female (N = 16), and 54% male (N = 19). Medium age was 17 years old (range: 10–31 years old). Medium time from Fontan completion was 160 months (range: 57–340 months). Ten patients had a functional single left ventricle (FSLV, 28.5%) and 21 a functional single right ventricle (FSRV, 60%); four patients had an undetermined single ventricle (11.5%). Data from renal function assessment showed a prevalence of stage 2 chronic kidney disease (eGFR: 60–89 ml/min 1.73 mq). Of those, 11% with creatinine-based equation and 26% (N = 9) when using cystatin C-based equation, with one patients showing a moderate reduced loss of kidney function (eGFR: 40–59 ml/min/1.73 mq). Most of the patients with reduced eGFR measured with cystatin C were FSRV (89%). None had laboratory markers of acute tubular damage, but four patients had signs of chronic tubular dysfunction with elevation of beta 2 microglobulin (13%). Echocardiographic evaluation of diastolic function showed two patients with baseline E/A < 1 (6%, tot N = 33) and 11/33 (33%) pts with abnormal E/E′ (>12). All of them were FSRV patients (100%). Interestingly, statistical correlation between diastolic parameters and renal function showed a significant association between tubular damage parameters, such as alfa1microglobulin and beta2microglobulin, and E/E′ (Pearson’s R 0.4 and 0.48, respectively, P < 0.05), both for FSLV and FSRV patients. Diastolic function appeared to be associated also with glomerular filtration: we found a statistically significant direct correlation between diastolic pulmonary wave deceleration time (dt D wave) and creatinine value (Pearson’s R 0.49, P < 0.05). Supporting the role of diastolic function in Fontan systemic complications is the linear correlation we found with hepatic tests: higher values of aspartate aminotransferase and of gamma-glutamyltransferase were associated with worse diastolic ventricular filling (longer dt D wave and E wave deceleration time, lower TDI early diastolic wave; Pearson’s R 0.45, 0.5, and −0.41, respectively, P < 0.05). Conclusions Fontan-related nephropathy is associated with worsening diastolic function, which was more represented in FSRV patients. Diastolic function is also associated with liver disease in Fontan patients. Those data suggest renal and liver function should be closely monitored in patients with impaired diastolic function.

scholarly journals Transplantation for Congenital Heart Disease: Focus on the Impact of Functionally Univentricular Versus Biventricular Circulation

2021 ◽  
Vol 12 (3) ◽  
pp. 352-359
Author(s):  
Kyle W. Riggs ◽  
John T. Broderick ◽  
Nina Price ◽  
Clifford Chin ◽  
Farhan Zafar ◽  
...  
Keyword(s):  
Risk Factors ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Fontan Operation ◽  
Blood Type ◽  
Abo Blood Type ◽  
The Impact ◽  
Fontan Patients

Background: Varying single center data exist regarding the posttransplant outcomes of patients with single ventricle circulation, particularly following the Fontan operation. We sought to better elucidate these results in patients with congenital heart disease (CHD) through combining two national databases. Methods: The United Network for Organ Sharing (UNOS) transplantation database was merged with the Pediatric Health Information System (PHIS), an administrative database with 71% of UNOS patients matched. Patients undergoing transplantation at a PHIS hospital from 2006 to 2017 were categorized as single ventricle or biventricular strategy based on their diagnoses and procedures in 90% of patients. When known, single ventricle patients were further analyzed by their palliative stage post-Glenn or post-Fontan (known in 31%). Results: A total of 1,517 CHD transplantations were identified, 67% with single ventricle strategy (1,016). Single ventricle, biventricular, and indeterminate patients had similar survival (log-rank P > .1). Risk factors for mortality in patients with CHD were extracorporeal membrane oxygenation (ECMO) support at transplant (hazard: 2.27), ABO blood type incompatibility (hazard: 1.61), African American recipient (hazard 1.42), and liver dysfunction (hazard 1.29). A total of 130 confirmed Fontan and 185 confirmed bidirectional Glenn patients underwent transplantation, each with survival equivalent to biventricular patients (log-rank P > .500). For Fontan patients, renal dysfunction (hazard: 5.40) and transplant <1 year after Fontan (hazard 2.82) were found to be associated with mortality. Conclusions: Single ventricle patients, as a group, experience similar outcomes as biventricular patients with CHD undergoing transplantation, and this extends to Fontan patients. Risk factors for mortality correlate with end-organ dysfunction as well as race and ABO blood type incompatibility in the CHD population.

scholarly journals Prediction of Volume Responsiveness by Stroke Volume Variation vs. Central Venous Pressure in Children Undergoing Fontan Operation

2020 ◽  
Author(s):  
Yun'an Song ◽  
Huiyan Hou ◽  
Jie Bai ◽  
Hongbin Gu
Keyword(s):  
Stroke Volume ◽  
Stroke Volume Variation ◽  
Single Ventricle ◽  
Fontan Operation ◽  
Venous Pressure ◽  
Central Venous ◽  
Volume Responsiveness ◽  
Before And After ◽  
Fontan Patients ◽  
Volume Challenge

Abstract Background: Fontan operation is a palliative medical procedure performed on children with single-ventricle defects. As postoperative success of the procedure largely depends on the preload volume, it is necessary to ensure the effective volume of systemic circulation by maintaining an appropriate pressure gradient between the systemic vein and the left atrium. However, there is a lack of effective indexes to evaluate volume responsiveness in Fontan patients. Stroke volume variation (SVV) is a dynamic hemodynamic parameter based on cardiopulmonary interaction in mechanical ventilation. This study is to validate the sensitivity and specificity of SVV vs. central venous pressure (CVP) in assessing volume responsiveness of Fontan patients.Method: 64 children were included in this prospective study with single-ventricle who underwent modified Fontan operation between May 2018 and January 2020. Patients were given 10ml·kg− 1 albumin for volume challenge within 10 min after CPB. Before and after volume challenge, the invasive arterial pressure module was connected to Mostcare™ equipment to collect SBP, MBP, DBP, SVV, CI and SVRI dynamically in a time window of 30 s at a frequency of 1000 Hz. According to the range of CI change, patients with ΔCI ≥ 15% were classified into response (R) group and patients with ΔCI < 15% into non-response (NR) group. Using SVV and CVP as indicators, the ROC of the patients was established, and the AUC, diagnostic threshold, sensitivity and specificity were calculated.Results: The 64 pediatric patients included with a mean age of 4.85 ± 1.20 years, a mean height of 98.00 ± 16.74 cm and a mean weight of 15.65 ± 5.37 kg. The SVV value was 17.15 ± 3.97% and 13.45 ± 2.45% before and after fluid challenge treatment vs. 18.60 ± 1.83 mmHg and 20.20 ± 2.39 mmHg for CVP in responders. The AUC of SVV was 0.74 (95% confidence interval [CI] 0.54–0.94, P < 0.05) and the cutoff value was 16%, offering a sensitivity of 50% and a specificity of 91.7% vs.0.70 (95% CI 0.50–0.92, P > 0.05), 19.5 mmHg, 58% and 76% for CVP.Conclusion: SVV exhibited a good predictive value for volume responsiveness in pediatric Fontan patients. Appropriate volume therapy according to SVV could improve the cardiac function of such patients.Trial registration: This research was registered in Chinese Clinical Trail Registry on Jan 26, 2018. Registration number is ChiCTR1800014654.Registry URL is http://www.chictr.org.cn/showproj.aspx?proj=25019. This observational prospective study was approved by the Local Ethics Committee of Shanghai Children’s Medical Center affiliated to Shanghai Jiao Tong University (SCMCIRB-K2017035)

Abstract 12874: Thebesian Veins Are Common and Progress in Adult Patients Late After Fontan Operation

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Toshikatsu Tanaka ◽  
Yasunobu Miki ◽  
Yasuharu Ogawa ◽  
Sachiko Kido
Keyword(s):  
Myocardial Ischemia ◽  
Single Ventricle ◽  
Fontan Operation ◽  
Pulmonary Atresia ◽  
Volume Overload ◽  
Adult Patients ◽  
Exercise Stress ◽  
Coronary Veins ◽  
Thebesian Veins ◽  
Fontan Patients

Introduction: Thebesian veins (ThVs) are coronary veins that drain directly into the atrial or ventricular chamber. Angiographically detectable ThVs are a rare finding sometimes associated with myocardial ischemia due to coronary steal and volume overload of ventricles in adults. However, there are limited data concerning ThVs in the setting of congenital heart disease. The purpose of this study is to demonstrate presence, character, and changes with growth of ThVs in adult patients late after Fontan operation. Methods: This study is single-center retrospective review of adults with Fontan circulation patients who had undergone cardiac catheterization for routine Fontan surveillance between 2009-2019. We assessed the presence of angiographically detectable ThVs from ascending aortography. After identification of the characteristic cases, we examined the ventricular morphology, the portion of ThVs drainage, and compared with previous findings of angiograms. We also reviewed clinical manifestation and findings of exercise stress ECG of the patients. Pulmonary atresia with intact ventricular septum was excluded because abnormal coronary arterial communications have been well documented in this condition. Results: Among 75 Fontan patients who had undergone catheterization, significant ThVs were observed in a total of 12 cases (16%). There were 6 right-dominant single ventricle, 4 left-dominant single ventricle, and 2 unbalanced two ventricles. In all cases, including left-dominant single ventricle, all ThVs drained into right ventricle cavity, never into left ventricle. The patients who underwent serial catheterizations demonstrated that ThVs dilation and tortuosity progressed over time. Among 6 patients who had underwent exercise stress ECG, only 1 patient showed ST segment depression, however, none had clinical history of chest pain. Conclusions: ThVs are common and progress in adult patients late after Fontan operation. Although the clinical significance of these findings remains uncertain, particular attention should be given to myocardial ischemia and volume overload for the future of adult Fontan patients who have prominent ThVs.

Are children with protein-losing enteropathy after the Fontan operation at increased risk of cytomegalovirus enteropathy? A report of two cases

2020 ◽  
Vol 30 (3) ◽  
pp. 431-432
Author(s):  
Christoph Bauer ◽  
Gerald Tulzer
Keyword(s):  
Risk Factors ◽  
Polymerase Chain Reaction ◽  
Single Ventricle ◽  
Fontan Operation ◽  
Disease Development ◽  
Chain Reaction ◽  
Protein Losing Enteropathy ◽  
Increased Risk ◽  
Polymerase Chain ◽  
Fontan Patients

AbstractIntroduction:Aetiology of protein-losing enteropathy in single-ventricle type CHD is multi-factorial.Report:We describe two Fontan patients with protein-losing enteropathy who presented with cytomegalovirus-associated colitis.Discussion:Fontan patients display risk factors for cytomegalovirus-induced gastroenteropathy that may affect lymph angiogenesis, disease development, and progression.Conclusion:Cytomegalovirus enteropathy may be common among Fontan patients who suffer from protein-losing enteropathy. Polymerase chain reaction is important for detection.

Abstract 12942: Association of Exercise Performance With Hemodynamic and Imaging Parameters in Fontan Patients

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Alyson R Pierick ◽  
Michael Kelleman ◽  
Megan Stark ◽  
Danish Vaiyani ◽  
Robert Whitehill ◽  
...  
Keyword(s):  
Exercise Capacity ◽  
Stress Test ◽  
Fontan Operation ◽  
Retrospective Chart Review ◽  
Exercise Stress Test ◽  
Exercise Stress ◽  
Surveillance Program ◽  
Primary Analysis ◽  
Lateral Tunnel ◽  
Fontan Patients

Introduction: The Fontan operation results in hemodynamic, anatomic and functional alterations in single ventricle (SV) heart disease. Studies have shown that SV patients have reduced exercise capacity. We sought to determine hemodynamic and anatomic associations with exercise capacity in our Fontan cohort. Methods: We performed a retrospective chart review of all patients born 2002-2012 who underwent Fontan palliation and followed in our Fontan Surveillance Program. Surveillance cardiac magnetic resonance (cMR), cardiac catheterization (cath), and exercise stress test (EST) data were collected. Primary analysis was association of cMR and cath data on EST outcomes including percent-predicted maximum oxygen consumption (ppVO 2 max) and oxygen pulse (ppO 2 Pulse). Results: A total of 222 patients were included, 45.5% with a systemic left ventricle (LV). Hypoplastic left heart syndrome was the underlying cardiac diagnosis in 1/3 of patients. The vast majority of the Fontan procedures performed were fenestrated, 44% extracardiac conduits and 42% with a lateral tunnel graft. A total of 185 cMRs, 274 caths, and 136 ESTs were performed. The median respiratory exchange ratio and ppVO 2 max were 1.11 L/sec and 73% respectively, with a preserved ppO 2 Pulse of 93.5%. Results of correlational analysis are shown in the table. Over serial ESTs, patients with a single RV had a more gradual, but not significant decline in ppVO 2 max than patients with a single LV, r s -0.321 and -0.544 respectively (p = 0.28). Conclusions: While hemodynamic parameters do not correlate with EST functional endpoints, anatomic factors such as the Nakata index are associated with EST performance. Surveillance of Fontan patients should include regular cMR to assess for subtle changes in ejection fraction, pulmonary artery diameter and differential pulmonary blood flow. Multicenter longitudinal studies with protocolized timing are needed to further elucidate correlates of good exercise capacity.

Pulmonary vasodilator therapy in the failing Fontan circulation: rationale and efficacy

2015 ◽  
Vol 25 (8) ◽  
pp. 1489-1492 ◽  
Author(s):  
Brian S. Snarr ◽  
Stephen M. Paridon ◽  
Jack Rychik ◽  
David J. Goldberg
Keyword(s):  
Single Ventricle ◽  
Fontan Operation ◽  
Vasodilator Therapy ◽  
Pulmonary Vasodilators ◽  
Pulmonary Vasodilator ◽  
Fontan Physiology ◽  
Fontan Surgery ◽  
Failing Fontan ◽  
Fontan Patients

AbstractThe Fontan operation is the final step of palliation for patients with a functionally single ventricle. Since its introduction in the 1970s, the Fontan surgery has become part of a successful surgical strategy that has improved single ventricle mortality. In recent years, we have become more aware of the limitations and long-term consequences of the Fontan physiology. Pulmonary vascular resistance plays an important role in total cavopulmonary circulation, and has been identified as a potential therapeutic target to mitigate Fontan sequelae. In this review, we will discuss the results of different pulmonary vasodilator trials and the use of pulmonary vasodilators as a treatment strategy for Fontan patients.

scholarly journals Prediction of Fluid Responsiveness by Stroke Volume Variation in Children Undergoing Fontan Operation

2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Yun’an Song ◽  
Huiyan Hou ◽  
Jie Bai ◽  
Hongbin Gu
Keyword(s):  
Stroke Volume ◽  
Fluid Responsiveness ◽  
Sensitivity And Specificity ◽  
Stroke Volume Variation ◽  
Single Ventricle ◽  
Fontan Operation ◽  
Fluid Challenge ◽  
Cutoff Value ◽  
Before And After ◽  
Fontan Patients

Background. Fontan operation is a palliative medical procedure performed on children with single-ventricle defects. As postoperative success of the procedure largely depends on the preload volume, it is necessary to maintain an appropriate pressure gradient between the systemic vein and the left atrium to ensure the effective volume of systemic circulation. However, there is a lack of effective indexes to evaluate fluid responsiveness in Fontan patients. Stroke volume variation (SVV) is a dynamic hemodynamic parameter based on cardiopulmonary interaction in mechanical ventilation. This study is aimed at validating the sensitivity and specificity of SVV and central venous pressure (CVP) in assessing the fluid responsiveness of Fontan patients. Method. Sixty-four children with single ventricle who underwent modified Fontan operation between May 2018 and January 2020 were included in this study. Patients were administered 10 ml·kg-1 albumin for fluid challenge within 10 min after cardiopulmonary bypass. Before and after fluid challenge, the invasive arterial pressure module was connected to MostCare™ equipment to collect the cardiac index (CI) and SVV dynamically in a time window of 30 s at a frequency of 1000 Hz. According to the range of CI change, patients with Δ CI ≥ 15 % were classified into the responder (R) group and those with Δ CI < 15 % into the nonresponder (NR) group. Using SVV and CVP as indicators, the receiver operating characteristic (ROC) curve of the patients was established, and the area under curve (AUC), diagnostic threshold, sensitivity, and specificity were calculated. Results. The SVV values were 16.28% (25th and 75th percentiles 14.17%-19.24%) and 13.68% (25th and 75th percentiles 12.90%-15.89%) before and after fluid challenge treatment in responders, respectively, and the values were 18.60 ± 1.83  mmHg before and 20.20 ± 2.39  mmHg for CVP after treatment. The AUC of SVV was 0.74 (95% confidence interval (CI) 0.54-0.94, P < 0.05 ), and the cutoff value was 16%, offering a sensitivity of 50% and a specificity of 91.7%. Meanwhile, the AUC of CVP was 0.70 (95% CI 0.50-0.92, P > 0.05 ), and the cutoff value was 19.5 mmHg, offering a sensitivity of 58% and a specificity of 76%. Conclusion. SVV exhibited a good predictive value for fluid responsiveness in pediatric Fontan patients. Appropriate fluid therapy according to SVV could improve the cardiac function of such patients. Trial registration. This study was registered in Chinese Clinical Trail Registry on Jan 26, 2018. Registration number is ChiCTR1800014654. Registry URL is http://www.chictr.org.cn/showproj.aspx?proj=25019. This observational prospective study was approved by the Local Ethics Committee of Shanghai Children’s Medical Center affiliated to Shanghai Jiao Tong University (SCMCIRB-K2017035).

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