Single ventricle function and exercise tolerance in adult patients after Fontan operation

2014 ◽  
Vol 69 (2) ◽  
pp. 155-160 ◽  
Author(s):  
Lidia Tomkiewicz-Pajak ◽  
Piotr Podolec ◽  
Leszek Drabik ◽  
Jacek Pajak ◽  
Jacek Kolcz ◽  
...  
Keyword(s):  
Exercise Tolerance ◽  
Single Ventricle ◽  
Fontan Operation ◽  
Adult Patients ◽  
Ventricle Function

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.

Exercise tolerance and cardiorespiratory response to exercise after the fontan operation for tricuspid atresia or functional single ventricle

1986 ◽  
Vol 7 (5) ◽  
pp. 1087-1094 ◽  
Author(s):  
David J. Driscoll ◽  
Gordon K. Danielson ◽  
Francisco J. Puga ◽  
Hartzell V. Schaff ◽  
Charles T. Heise ◽  
...  
Keyword(s):  
Exercise Tolerance ◽  
Single Ventricle ◽  
Fontan Operation ◽  
Tricuspid Atresia ◽  
Cardiorespiratory Response ◽  
Response To Exercise

P3656Fontan operation performed in adult patients

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
T Santos Monteiro ◽  
C Cruz Lamas ◽  
M C Terra Cola ◽  
A J Oliveira Monteiro ◽  
M Machado Melo ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Fontan Operation ◽  
Cardiopulmonary Exercise Testing ◽  
Adult Patients ◽  
Venous Blood Flow ◽  
Inhospital Mortality ◽  
Vo2 Max ◽  
Venae Cavae ◽  
The Mean

Abstract Introduction Treatment of patients with univentricular physiology is based on a sequence of palliative surgeries which end with the Fontan operation, when all venous blood flow is diverted to the lungs, bypassing the heart. Most centers advise to complete this process around 4 years of age, and there are few data about the performance of the Fontan operation in adults. Purpose To describe the results of the Fontan surgery when performed in adult patients. Methods A retrospective review of patients submitted to the Fontan operation between 2014 and 2018, with data collection from charts, regarding their pre-operative state and follow up, including improvement in exercise capacity and hemoglobin levels. Results There were 12 patients submitted to the Fontan operation in the study period, with mean age 24±5 years, 8 female and 4 male. Two patients had no previous surgery, 2 only had bandage of the pulmonary artery, 7 had the Glenn surgery and 1 had the Damus and the Glenn surgery. Five patients had tricuspid atresia (TA) with valvular pulmonary stenosis (PS) or atresia, 1 patient had TA alone, 2 had TA with transposition of the great arteries, 1 patient had double inlet left ventricle (LV) with PS, 2 had double inlet LV with coarctation of the aorta, and 1 patient had hypoplastic right heart. One patient had suspected Noonan Syndrome. The patients who did not have Glenn surgery were submitted to connection of superior and inferior venae cavae with the pulmonary artery in the same procedure (4 patients). Seven patients had the fenestrated Fontan procedure. Six patients had a combined operation. Inhospital mortality was 0%. One patient died 4 months after the surgery due to bilateral subdural hematoma. The immediate post operative complications were tachyarrhythmia (2); important bleeding (2); pericardial effusion (4); pleural effusion (7); provisional pacemaker (1); junctional rhythmn (1); temporary hemodialysis (1); infection of the operative wound (1); fungal endocarditis (1); and mild stroke (1). The mean duration of hospitalization was 41.5±18.7 days. The length of hospital stay after surgery was 31.1±16.2 days. The exercise functional capacity improved in all patients. Before surgery there was 1 patient NYHA II that became NYHA I, 10 were NYHA III and became II or I, and 1 patient who was NYHA IV became II. The average oxygen saturation before surgery was 82% ± 8.2% and after was 91.7% ± 4.7%. The mean hemoglobin went from 17.8 g/dL to 13.9 g/dL. Eight patients performed cardiopulmonary exercise testing (CPX) before surgery, 1 patient was Weber B, 4 patients Weber C, 1 D and 1 E. Mean VO2 max was 11.7 ml/kg.min (± 3.69), and the mean slope was 71.8±35.0. Four patients performed CPX after surgery, mean VO2 max was 16.5±7.3, and mean slope was 39±16.6. Mean follow up was 20.3±17.7 months. Conclusions The Fontan operation is safe in adult patients and may still confer them significant benefits.

Characteristics and outcomes of acute COVID-19 infection in paediatric and young adult patients with underlying cardiac disease

2021 ◽  
pp. 1-7
Author(s):  
Joshua M. Fisher ◽  
Sarah Badran ◽  
John T. Li ◽  
Jodie K. Votava-Smith ◽  
Patrick M. Sullivan
Keyword(s):  
Risk Factors ◽  
Young Adult ◽  
Pulmonary Disease ◽  
Cardiac Disease ◽  
Single Ventricle ◽  
Cardiac Risk ◽  
Adult Patients ◽  
Chronic Pulmonary Disease ◽  
Cardiac Risk Factors ◽  
Increased Risk

Abstract Objective To describe outcomes of acute coronavirus disease 2019 in paediatric and young adult patients with underlying cardiac disease and evaluate the association between cardiac risk factors and hospitalisation. Study design We conducted a retrospective single-institution review of patients with known cardiac disease and positive severe acute respiratory syndrome coronavirus 2 RT-PCR from 1 March, 2020 to 30 November, 2020. Extracardiac comorbidities and cardiac risk factors were compared between those admitted for coronavirus disease 2019 illness and the rest of the cohort using univariate analysis. Results Forty-two patients with a mean age of 7.7 ± 6.7 years were identified. Six were 18 years of age or more with the oldest being 22 years of age. Seventy-six percent were Hispanic. The most common cardiac diagnoses were repaired cyanotic (n = 7, 16.6%) and palliated single ventricle (n = 7, 16.6%) congenital heart disease. Fourteen patients (33.3%) had underlying syndromes or chromosomal anomalies, nine (21%) had chronic pulmonary disease and eight (19%) were immunosuppressed. Nineteen patients (47.6%) reported no symptoms. Sixteen (38.1%) reported only mild symptoms. Six patients (14.3%) were admitted to the hospital for acute coronavirus disease 2019 illness. Noncardiac comorbidities were associated with an increased risk of hospitalisation (p = 0.02), particularly chronic pulmonary disease (p = 0.01) and baseline supplemental oxygen requirement (p = 0.007). None of the single ventricle patients who tested positive required admission. Conclusions Hospitalisations for coronavirus disease 2019 were rare among children and young adults with underlying cardiac disease. Extracardiac comorbidities like pulmonary disease were associated with increased risk of hospitalisation while cardiac risk factors were not.

Abstract 16822: Growth Curves Describing a Single Ventricle Population After Fontan Procedure

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Bethany Runkel ◽  
Vincent Staggs ◽  
Chelsea Hosey Cojocari ◽  
Jonathan B Wagner
Keyword(s):  
Single Ventricle ◽  
Multiple Stressors ◽  
Pediatric Population ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Growth Patterns ◽  
Growth Charts ◽  
Genetic Syndrome ◽  
Adiposity Rebound ◽  
Extracardiac Conduit

Introduction: Complex single ventricle congenital heart disease (CHD) patients are subject to multiple stressors early in life that affect somatic growth. Pre-Fontan growth patterns are well-described, but post-Fontan growth has not been extensively studied in the current era. We sought to describe post-Fontan growth patterns by creating sex-specific body mass index (BMI)-for-age curves. Hypothesis: There is no difference in the growth of post-Fontan pediatric patients when compared to published normative data. Methods: A single-center, preexisting database was retrospectively queried for all patients who underwent Fontan procedure between 2006 and 2018. Patients with a genetic syndrome, a primary endocrine disorder, or significant prematurity were excluded. BMI-for-age curves were created for both male and female patients using anthropometric data extracted from the electronic medical record. Curves were then visually compared with Centers for Disease Control and Prevention (CDC) growth charts. Results: Of 227 patients who underwent primary Fontan operation, 37 were excluded. Of the remaining 190 patients, 59% were male. All had an extracardiac conduit, half had right-ventricular dominant CHD, and 15% had a Fontan fenestration. Median age and weight at surgery were 3.9 years and 15 kilograms, respectively. BMI curves were similar in appearance to CDC BMI-for-age growth charts, with adiposity rebound at age 6. BMI increased more rapidly in teen boys compared with girls, and a total of five patients (2.6%) had BMI values greater than 25 kg/m 2 at 16 years of age. Conclusion: Post-Fontan patients at our institution demonstrate BMI-for-age patterns similar to those of the general population during childhood and early adolescence, with adiposity rebound occurring at a typical age. Though excess weight gain does not completely spare patients with single ventricle CHD, overweight status appears to be less common than in the general pediatric population.

Single-centre experience in stenting arterial shunts for adult CHD patients with single-ventricle physiology and pulmonary blood flow dependent on arterial shunts

2018 ◽  
Vol 28 (12) ◽  
pp. 1431-1435
Author(s):  
Mohamed Kasem ◽  
James Bentham ◽  
John Thomson
Keyword(s):  
Blood Flow ◽  
Pulmonary Blood Flow ◽  
Single Ventricle ◽  
Well Being ◽  
Adult Patients ◽  
P Value ◽  
Intermediate Step ◽  
Medium Term ◽  
Single Ventricle Physiology ◽  
Post Procedure

AbstractObjectivesThe objectives of this study were to describe the approach to stenting arterial shunts in adult congenital patients with single-ventricle physiology and to assess the medium-term clinical and haemodynamic outcomes following stent insertion.BackgroundAdult patients with single-ventricle physiology and pulmonary blood flow dependent on a surgically placed arterial shunt who did not progress to venous palliation are extremely challenging to manage. Progressive cyanosis secondary to narrowing of the shunt has a marked impact on exercise tolerance and results in intolerable well-being for these patients. Stenting arterial shunts in adult patients is one method that can help improve pulmonary blood flow. There is very limited information in the literature about this patient.MethodsThis is a retrospective study for arterial shunts stenting conducted between 2008 and 2016. The peripheral oxygen saturations, the NYHA status, the haemoglobin, and the degree of atrio-ventricular valve regurgitation as assessed on transthoracic echo were compared before and 6–12 months after procedures.ResultsThere was a short-term improvement in oxygen saturations; the pre-procedure mean was 75.8 (SD 2.55)% (range 70–85%) and post-procedure mean was 83 (SD 2.52)% (range 78–87%), with a p value of 0.04. Haemoglobin level decreased from a pre-procedure mean of 22.06–20.28 g/L 6 months post procedure (range 18.1–24.4 to 13–23.3 g/L), with a p value of 0.44. NYHA class decreased from a mean of 3.2–2.2 post procedure. Left atrial volume for four of the cases did not change (22.6–76.6 ml [mean 48.4 ml] to 29.6–72.9 ml [mean 52 ml], p value: 0.83).ConclusionsStenting stenotic arterial shunts is a useful method to gain a medium-term improvement on the oxygen saturation and clinical symptoms, and may act as a useful intermediate step for further management plans.

Effect of Accessory Pulmonary Blood Flow on Survival After the Bidirectional Glenn Procedure

Circulation ◽  
1999 ◽  
Vol 100 (suppl_2) ◽  
Author(s):  
Richard D. Mainwaring ◽  
John J. Lamberti ◽  
Karen Uzark ◽  
Robert L. Spicer ◽  
Mark W. Cocalis ◽  
...  
Keyword(s):  
Blood Flow ◽  
Mortality Rate ◽  
Pulmonary Blood Flow ◽  
Single Ventricle ◽  
Fontan Operation ◽  
Right Ventricular Outflow Tract ◽  
Bidirectional Glenn ◽  
Actuarial Analysis ◽  
Bidirectional Glenn Procedure ◽  
Glenn Procedure

Background —The bidirectional Glenn procedure (BDG) is used in the staged surgical management of patients with a functional single ventricle. Controversy exists regarding whether accessory pulmonary blood flow (APBF) should be left at the time of BDG to augment systemic saturation or be eliminated to reduce volume load of the ventricle. The present study was a retrospective review of patients undergoing BDG that was conducted to assess the influence of APBF on survival rates. Methods and Results —From 1986 through 1998, 149 patients have undergone BDG at our institution. Ninety-three patients had elimination of all sources of APBF, whereas 56 patients had either a shunt or a patent right ventricular outflow tract intentionally left in place to augment the pulmonary blood flow provided by the BDG. The operative mortality rate was 2.2% without APBF and 5.4% with APBF. The late mortality rate was 4.4% without APBF and 15.1% with APBF. Actuarial analysis demonstrates a divergence of the Kaplan-Meier curves in favor of patients in whom APBF was eliminated ( P <0.02). One hundred seven patients have subsequently undergone completion of their Fontan operation, so the actuarial analysis includes the operative risk of this second operation. Conclusions —The results suggest that the elimination of APBF at the time of BDG may confer a long-term advantage for patients with a functional single ventricle.

Abstract 10756: Chronic Kidney Disease in Long-term Survivors After Fontan Palliation

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Sheena Sharma ◽  
Rebecca L Ruebner ◽  
Susan L Furth ◽  
Kathryn M Dodds ◽  
Jack Rychik ◽  
...  
Keyword(s):  
Chronic Kidney Disease ◽  
Kidney Disease ◽  
Kidney Function ◽  
Healthy Subjects ◽  
Single Ventricle ◽  
Fontan Operation ◽  
Long Term Survivors ◽  
Fontan Palliation ◽  
Over Time

Background: The Fontan operation is a palliative procedure for children with congenital single ventricle heart disease. With advances in prenatal diagnosis and surgical techniques, more children are surviving into adulthood with unique cardiovascular physiology. Little is currently known about long-term kidney function in these patients. Hypothesis: We hypothesize that long-term survivors after Fontan palliation will have a higher prevalence of chronic kidney disease (CKD) compared to healthy controls. Methods: We performed a retrospective cohort study of subjects evaluated through the Single Ventricle Survivorship Program (SVSP) at the Children’s Hospital of Philadelphia between July 1, 2010 and December 5, 2014 and healthy children similar in sex and age. The primary outcome was CKD, defined as eGFR <90 ml/min/1.73m2 calculated with age-appropriate estimating equations using creatinine and cystatin C. Secondary outcomes included proteinuria and hyperparathyroidism. Results: The Fontan cohort included 68 subjects with mean age of 13.9 years (SD 5.8) at SVSP visit who were 11.2 years (SD 5.7) from Fontan operation. The healthy cohort included 70 patients with mean age of 15.9 years (SD 3.9). Mean eGFR was 102.6 versus 101.9ml/min/1.73m2 (p=0.89) in pediatric Fontan versus healthy subjects using the complete CKiD equation, and 128.5 versus 129.7ml/min/1.73m2 (p=0.56) in adult Fontan versus healthy subjects using the CKD-EPI creatinine and cystatin formula. 10% of Fontan subjects had an eGFR<90 ml/min/1.73m2. Mean intact parathyroid hormone level was higher at 68.0pg/mL (SD 35.4) in the Fontan group compared to 26.0pg/mL (SD 13.6) in the healthy group. Proteinuria was found within 34% of the Fontan group compared to 4.6% within the control group. Conclusion: We found that 10% of subjects have eGFR <90ml/min/1.73m2 after Fontan palliation which would indicate CKD if this remained persistent over time. Although the majority of the cohort had normal kidney function by eGFR, we found a higher proportion with proteinuria and increased parathyroid hormone levels which may indicate early kidney disease. Future studies will focus on evaluating changes in kidney function over time in long-term survivors after Fontan palliation.

scholarly journals Factors Related to Pleural Effusions After Fontan Procedure in the Era of Fenestration

Circulation ◽  
2001 ◽  
Vol 104 (suppl_1) ◽  
Author(s):  
Raymond T. Fedderly ◽  
Beth N. Whitstone ◽  
Stephanie J. Frisbee ◽  
James S. Tweddell ◽  
S. Bert Litwin
Keyword(s):  
Hospital Stay ◽  
Chest Tube ◽  
Single Ventricle ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Univariate Analysis ◽  
Ordinary Least Squares ◽  
Pleural Effusions ◽  
Tube Output ◽  
Chest Tube Output

Background Significant pleural effusions after the Fontan operation prolong hospital stay, may increase the risk of infection, and may necessitate a pleurodesis procedure. Methods and Results From February 1991 to April 2000, 98 consecutive patients under the age of 18 years underwent the fenestrated Fontan procedure at Children’s Hospital of Wisconsin. Ninety-four patients who survived at least 30 days after surgery were retrospectively evaluated for the following factors: age, ventricular morphology (right single ventricle, left single ventricle [RV/LV]), fenestration open (FO) or closed (FC) at end of operation, intracardiac Fontan (IF) or extracardiac Fontan (EF), days with chest tube output per day >5, 10, and/or 20 mL · kg −1 · d −1 (CTO5, CTO10, and CTO20, respectively), need for pleurodesis, length of hospital stay (LOS), operation during winter respiratory viral season of November through March (ReVS+, ReVS−), and pre-Fontan mean pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR). In univariate analysis, the ReVS+ patients had prolonged LOS, greater chest tube output, and more pleurodesis ( P <0.05), and PAP was related to CTO5 and CTO10 but not to CTO20 or LOS. No significant differences were found in LOS, CTO5, CTO10, CTO20, and need for pleurodesis between patients in RV/LV, FO/FC, IF/EF, or PVR groups. Patients <4 years of age had more instances of CTO20 ( P <0.05). When we used ordinary least squares regression analysis with age, FO or FC, RV or LV, PAP, and ReVS+ or ReVS− to predict each of CTO5, CTO10, CTO20, and LOS, only ReVS+ or ReVS− and age were statistically significant in all models. Conclusions Use of the Fontan procedure during the respiratory viral season appeared to be related to significant, prolonged pleural effusions and longer hospitalizations.

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