scholarly journals Effect of Sildenafil on Pressure–Volume Loop Measures of Ventricular Function in Fontan Patients

2015 ◽  
Vol 37 (1) ◽  
pp. 184-191 ◽  
Author(s):  
Ryan J. Butts ◽  
Shahryar M. Chowdhury ◽  
George H. Baker ◽  
Varsha Bandisode ◽  
Andrew J. Savage ◽  
...  
Keyword(s):  
Ventricular Function ◽  
Fontan Patients

scholarly journals Myocardial Deformation in Fontan Patients Assessed by Cardiac Magnetic Resonance Feature Tracking: Correlation with Function, Clinical Course, and Biomarkers

2021 ◽  
Author(s):  
Alessia Callegari ◽  
Simona Marcora ◽  
Barbara Burkhardt ◽  
Michael Voutat ◽  
Christian Johannes Kellenberger ◽  
...  
Keyword(s):  
Ventricular Function ◽  
Feature Tracking ◽  
Heart Diseases ◽  
Fontan Operation ◽  
Myocardial Deformation ◽  
Time Interval ◽  
Ventricular Volumes ◽  
Atrioventricular Valve Regurgitation ◽  
Cine Mr ◽  
Fontan Patients

AbstractCardiac MR (CMR) is a standard modality for assessing ventricular function of single ventricles. CMR feature-tracking (CMR-FT) is a novel application enabling strain measurement on cine MR images and is used in patients with congenital heart diseases. We sought to assess the feasibility of CMR-FT in Fontan patients and analyze the correlation between CMR-FT strain values and conventional CMR volumetric parameters, clinical findings, and biomarkers. Global circumferential (GCS) and longitudinal (GLS) strain were retrospectively measured by CMR-FT on Steady-State Free Precession cine images. Data regarding post-operative course at Fontan operation, and medication, exercise capacity, invasive hemodynamics, and blood biomarkers at a time interval ± 6 months from CMR were collected. Forty-seven patients underwent CMR 11 ± 6 years after the Fontan operation; age at CMR was 15 ± 7 years. End-diastolic volume (EDV) of the SV was 93 ± 37 ml/m2, end-systolic volume (ESV) was 46 ± 23 ml/m2, and ejection fraction (EF) was 51 ± 11%. Twenty (42%) patients had a single right ventricle (SRV). In single left ventricle (SLV), GCS was higher (p < 0.001), but GLS was lower (p = 0.04) than in SRV. GCS correlated positively with EDV (p = 0.005), ESV (p < 0.001), and EF (p ≤ 0.0001). GLS correlated positively with EF (p = 0.002), but not with ventricular volumes. Impaired GCS correlated with decreased ventricular function (p = 0.03) and atrioventricular valve regurgitation (p = 0.04) at echocardiography, direct atriopulmonary connection (p = 0.02), post-operative complications (p = 0.05), and presence of a rudimentary ventricle (p = 0.01). A reduced GCS was associated with increased NT-pro-BNP (p = 0.05). Myocardial deformation can be measured by CMR-FT in Fontan patients. SLVs have higher GCS, but lower GLS than SRVs. GCS correlates with ventricular volumes and EF, whereas GLS correlates with EF only. Myocardial deformation shows a relationship with several clinical parameters and NT-pro-BNP.

Ventricular function and cardiac reserve in contemporary Fontan patients

2015 ◽  
Vol 196 ◽  
pp. 73-80 ◽  
Author(s):  
S.S.M. Bossers ◽  
L. Kapusta ◽  
I.M. Kuipers ◽  
G. van Iperen ◽  
A. Moelker ◽  
...  
Keyword(s):  
Ventricular Function ◽  
Cardiac Reserve ◽  
Fontan Patients

Improved Transplant Survival in Failed Fontan Patients With Preserved Ventricular Function in the Current Era

2015 ◽  
Vol 34 (4) ◽  
pp. S36-S37
Author(s):  
K. Simpson ◽  
J. Miller ◽  
T. Lancaster ◽  
M. Henn ◽  
D. Epstein ◽  
...  
Keyword(s):  
Ventricular Function ◽  
Transplant Survival ◽  
Fontan Patients

scholarly journals 3-Month Enalapril Treatment in Pediatric Fontan Patients With Moderate to Good Systolic Ventricular Function

2021 ◽  
Author(s):  
Lisette M. Harteveld ◽  
Nico A. Blom ◽  
Covadonga Terol Espinosa de Los Monteros ◽  
Irene M. Kuipers ◽  
Lukas A.J. Rammeloo ◽  
...  
Keyword(s):  
Ventricular Function ◽  
Fontan Patients ◽  
Enalapril Treatment

Abstract 1672: Glomerular Insufficiency, An Early Marker Of Renal Dysfunction In Patients With A Fontan Circulation.

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Hui Beng Koh ◽  
Brian Stedman ◽  
Mary Rogerson ◽  
Gruschen Veldtman
Keyword(s):  
Renal Dysfunction ◽  
Ventricular Function ◽  
Cystatin C ◽  
Left Kidney ◽  
Visual Assessment ◽  
Retinol Binding Protein ◽  
Normal Range ◽  
Serum Cystatin C ◽  
The Right ◽  
Fontan Patients

Introduction : Fontan circulations have high central venous pressures and low dynamic cardiac output, risk factors for depressed renal perfusion. Objectives : Hypothesizing subclinical renal dysfunction, we sought to characterize renal pathophysiology and cardio-renal interaction in this group. Method: 17 Fontan patients were tested for early morning urine cystatin C, alpha-1-microglobulin (A 1 M), retinol binding protein (RBP), protein:creatinine and albumin:creatinine ratio; serum cystatin C (S.Cys-C) and creatinine; echocardiography including Tissue Doppler (TDI); and renal Doppler. Results : 6 (mean age 22.8 ± 5.0years) of 17 patients (mean age 23.5 ± 4.2years) had subclinical renal dysfunction, mean S.Cys-C-derived glomerular filtration rate (GFR S.Cys-C ) 77.31 ± 7.82ml/min/1.73m 2 , vs. 114.21 ± 36.38ml/min/1.73m 2 in the remainder. Whilst GFR S.Cys-C correlated highly with 2-D visual assessment of systemic ventricular function (correlation=0.618; p=0.014) this was not the case for any TDI parameters. Tubular function indices (urine cystatin C, RBP and A 1 M) were normal. Glomerular function indices were abnormal for S.Cys-C, with mean 1.04 ± 0.06mg/L, range 0.97–1.11mg/L; (normal range:0.53–0.95mg/L). Gross morphological renal abnormalities included 3 abnormal bipolar sizes in both right (mean 9.13 ± 0.49cm) and left (mean 9.38 ± 0.66cm) kidneys (normal range>10cm), 6 and 5 abnormal renal cortical thicknesses in right (mean 11.61 ± 1.27mm) and left (mean 12.31 ± 0.92mm) kidneys respectively (normal range>13mm) and 1 mild scarring of the right kidney. Abnormal resistive indices were present in the right kidney of 3 patients (mean 0.79 ± 0.01, range 0.78–0.80), and in the left kidney of 2 patients (mean 0.83 ± 0.02, range 0.81–0.84). Patients with abnormal resistive indices (>0.77) had lower arterial saturations (mean 88.67 ± 2.08%, range 87–91% vs. mean 94.77 ± 2.46%, range 90–97% in those without) and higher haematocrit (mean 0.49 ± 0.01L/L, range 0.48–0.49L/L vs. mean 0.46 ± 0.03L/L, range 0.42–0.52L/L in those without). Conclusion : Fontan patients often have subclinical renal dysfunction mainly due to glomerular insufficiency. Ventricular function, cyanosis and polycythemia appear to be important drivers for renal dysfunction.

scholarly journals Improved survival after heart transplant for failed Fontan patients with preserved ventricular function

2016 ◽  
Vol 35 (7) ◽  
pp. 877-883 ◽  
Author(s):  
Jacob R. Miller ◽  
Kathleen E. Simpson ◽  
Deirdre J. Epstein ◽  
Timothy S. Lancaster ◽  
Matthew C. Henn ◽  
...  
Keyword(s):  
Ventricular Function ◽  
Heart Transplant ◽  
Fontan Patients

scholarly journals Dobutamine stress testing for the evaluation of atrial and diastolic ventricular function in Fontan patients

Open Heart ◽  
2021 ◽  
Vol 8 (1) ◽  
pp. e001487
Author(s):  
Jelle P G van der Ven ◽  
Sjoerd S M Bossers ◽  
Eva van den Bosch ◽  
Niels Dam ◽  
Irene M Kuipers ◽  
...  
Keyword(s):  
Ventricular Function ◽  
Exercise Capacity ◽  
Cardiopulmonary Exercise Testing ◽  
Dobutamine Stress ◽  
Cox Proportional Hazards ◽  
Function Parameter ◽  
Extracardiac Conduit ◽  
Lateral Tunnel ◽  
Fontan Patients

ObjectiveTo assess the atrial and ventricular diastolic function response to dobutamine stress in Fontan patients, and to relate these measurements to exercise capacity and events during the follow-up.MethodsWe performed a secondary analysis of a cross-sectional multicentre study of Fontan patients with intra-atrial lateral tunnel (ILT) or extracardiac conduit (ECC) modification. Subjects underwent cardiac MRI during rest and low-dose dobutamine stress, and cardiopulmonary exercise testing. Atrial and diastolic ventricular function parameters were derived from volume-time curves.Medical records were abstracted for a composite end-point of death, listing for transplant, arrhythmia and reintervention. Spearman’s r correlation tests and Cox proportional hazards models were used to assess the relation between the dobutamine response for atrial and diastolic ventricular function and outcomes, including exercise capacity.ResultsWe included 57 patients (26 ECC; 31 ILT) aged 12.8 (IQR (10.3–15.5)) years. During dobutamine stress atrial cyclic volume change increased (3.0 (0.4–5.9) mL/m2, p<0.001), as did early (1.9 (−1.6 to 3.6) mL/m2, p=0.001) and late emptying volume (2.2 (0.2–4.4) mL/m2, p<0.001).Ventricular early filling decreased (−1.6 (−5.7 to 0.7) mL/m2,p=0.046) and ventricular late filling increased (1.0 (−0.4 to 3.4) mL/m2,p<0.001) while stroke volume remained similar.Only for patients with the ECC modification, atrial early emptying volume increase correlated with peak oxygen uptake (ρ=0.66,p=0.002). No other parameter related to exercise capacity.During a median 7.1-year follow-up, 22 patients reached the composite endpoint. No parameter predicted events during the follow-up.ConclusionsDobutamine stress augmented atrial reservoir and pump function for Fontan patients. Atrial early emptying reserve related to exercise capacity in ECC patients. No other atrial or diastolic ventricular function parameter related to outcomes.

Long term follow-up after pregnancy in Fontan patients

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
E Gribaudo ◽  
A Constantine ◽  
A Pires ◽  
I Ahmed ◽  
R Patel ◽  
...  
Keyword(s):  
Ventricular Function ◽  
Nyha Class ◽  
Fontan Procedure ◽  
Fontan Circulation ◽  
Atrial Arrhythmia ◽  
Class I ◽  
Protein Losing Enteropathy ◽  
Fontan Patients

Abstract Background Patients with single ventricle physiology who have benefited from the Fontan procedure are reaching childbearing age. In this population, complications are common around pregnancy, but the long term effect of pregnancy is unclear. Purpose To evaluate functional and structural changes several years after pregnancy in patients with Fontan circulation. Methods Women with Fontan circulation who had a pregnancy beyond the 20 weeks of gestation followed at our centre between 2005–2019 were included. The following data were collected before pregnancy and at the last follow up (f-up): cardiac anatomy, type of Fontan procedure, resting O2 saturation (O2sat), NYHA class, systemic atrioventricular valve (SAVV) regurgitation, ventricular function, history of arrhythmias, heart failure (HF), thromboembolism, Fontan-related liver disease (FLD), protein-losing enteropathy (PLE), plastic bronchitis and cardiopulmonary test data. Results 12 patients had 18 pregnancies, giving birth to 17 live newborns (1 newborn death at 24 weeks of gestation). 7 (58.3%)patients had a total cavo-pulmonary connection, 3 (25%)an atrio-pulmonary connection and 2 (16.7%)a Bjork type Fontan. Tricuspid atresia was the most common anatomy (6, 50%). Before pregnancy, O2sat was 95.4±1.9%and all patients were in NYHA class I or II. All patients (12, 100%)had a normal or mildly impaired ventricular function and 3 (25%)had a moderate or severe SAAV regurgitation. 6 (50%)patients had 2 pregnancies. Mean age at first pregnancy was 29.9±4.4 years. Cardiac events occurred in 7 (38%)pregnancies, most commonly atrial arrhythmia (4, 57%), 2 (16.6%)patients developed new cyanosis and 1 had a thromboembolic event. No maternal death occurred and 1 patient had severe haemorrhage. After a mean f-up of 84±48 months, there was no significant worsening in O2sat (94.3±3.1 at f-up, p=1) and all patients remained in NYHA class I or II (12, 100%, p=1). At f-up all patients (12, 100%)continued to have a normal or mildly impaired ventricular function (p=1) and there was no significant worsening in the SAAV regurgitation (3 patients with moderate or severe regurgitation, p=1). 3 (25%)patients developed FLD (p=0.3), 3 (25%) a new episode of atrial arrhythmia (p=0.3). There were no significantly differences in peak VO2 (22.5±3.8 pre-pregnancy, 22.8±5.5 at f-up, p=0.4) and VE/VCO2 slope (34.9±11.2 pre-pregnancy, 36.3±11.8 at f-up, p=0.2)before pregnancy and during f-up. Conclusions In this small cohort of Fontan patients who successfully completed at least 1 pregnancy, there was no significant decline in functional or structural cardiac parameters long term after delivery. Funding Acknowledgement Type of funding source: None

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