Abstract 15665: Can Pulmonary Vasodilators Grow Pulmonary Vasculature in Cases With Bidirectional Glenn for Fontan Candidates?

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Kousuke Yonehara
Keyword(s):  
Fontan Procedure ◽  
Pde5 Inhibitor ◽  
Pulmonary Arteries ◽  
Operative Management ◽  
Pulmonary Vasculature ◽  
Endothelin Receptor ◽  
Cross Sectional ◽  
Pulmonary Vasodilators ◽  
Pulmonary Flow ◽  
Fontan Patients

Background: The condition of pulmonary vasculature is a key to determine the outcome of Fontan candidates. The previous reports described no significant growth of pulmonary arteries (PA) after having bi-directional Glenn shunt (BDG), which may influence peri- and post-operative management of Fontan procedure. Aim: We hypothesized the pulmonary vasodilators (PVDs) facilitate to grow small PA even after BDG in Fontan Candidates. Method: The twenty-one cases after BDG, whose PA index (Nakata index) were less than 140 mm 2 /m 2 , were enrolled. The enrolled cases were divided into two group: 15 with PVDs treatment ( PV+) and 6 without (PV-). PVDs used in this study were PDE5 inhibitor, Endothelin-receptor-blocker, oral prostacyclines , and their combination. In addition to measure the hemodynamic parameters ( mean PA pressure(mPAp), indexed pulmonary vascular resistance (Rp), Pulmonary flow (Qp) ), PA index and PA branch index ( a sum of cross-sectional area of first PA branches at both side indexed by body surface area) were measured and compared at the timing of after BDG and at after Fontan completion, respectively. Result: PA index and PA branch index after BDG of (PV+) and (PV-)were 104±33 vs 104±17 mm 2 /m 2 : 151±33 vs 194±45 mm 2 /m 2 . PA index in both groups after Fontan procedure was increased up to 153±42 vs 152±56 mm 2 /m 2 in same fashion, while PA branch index of (PV+) was significantly increased up to 199±60 than that of (PV-) 226±65 mm 2 /m 2 (% increase was 131% vs 116%). Rp and mPAp in both groups were not changed , while Qp in (PV+) was significantly more increased (140%) than that in (PV-)(107%). In conclusion, PVDs facilitates the growth of PA branches by increasing Qp, which brings a favor outcome of Fontan patients.

Effect of Caval Waveform on Energy Dissipation of Failing Fontan Patients

2009 ◽  
Author(s):  
Onur Dur ◽  
Ergin Kocyildirim ◽  
Curt G. Degroff ◽  
Peter Wearden ◽  
Victor Morell ◽  
...  
Keyword(s):  
Inferior Vena ◽  
Superior Vena ◽  
Fontan Procedure ◽  
Pulmonary Arteries ◽  
Surgical Reconstruction ◽  
Power Losses ◽  
Last Stage ◽  
Cavopulmonary Connection ◽  
Failing Fontan ◽  
Fontan Patients

Last stage of the palliative surgical reconstruction (i.e. Fontan procedure) for the infants with functional single-ventricle is total cavopulmonary connection (TCPC), where the superior vena cavae (SVC) and inferior vena cavae (IVC) are routed directly into the pulmonary arteries. Limited pumping energy available due to the absence of right-ventricle and altered venous characteristics require optimized hemodynamics inside the TCPC pathway, which can be achieved by minimizing the power losses.

Pulmonary artery wall thickness in children with Fontan physiology: an optical coherence tomography case control study

2019 ◽  
Vol 29 (4) ◽  
pp. 524-527
Author(s):  
Eimear McGovern ◽  
Christine Voss ◽  
Nathan W. Brunner ◽  
Stephanie Duncombe ◽  
Kevin C. Harris ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Pulmonary Artery ◽  
Wall Thickness ◽  
Structural Changes ◽  
Pulmonary Arteries ◽  
Causative Factor ◽  
Optical Coherence ◽  
Artery Wall ◽  
Cross Sectional ◽  
Fontan Patients

AbstractIntroduction:Failure of the Fontan circulation is not a well-understood clinical phenomena.For some patients, a gradual increase in pulmonary vascular resistance (PVR) and structural changes in the pulmonary artery may be an important causative factor. To further investigate this issue, we employed optical coherence tomography (OCT) to evaluate structural changes within the pulmonary arteries of Fontan patients and compared to those with a normal pulmonary circulation.Materials and Methods:Pulmonary artery OCT was performed, without complications, in 12 Fontan and 11 control patients. Wall thickness and wall:vessel cross-sectional area (CSA) ratio were calculated after image acquisition, using digital planimetry.Results:There was no difference in wall thickness between both groups. Median wall thickness for Fontan patients was 0.12 mm (IQR, 0.10–0.14) and for controls was 0.11 mm (IQR, 0.10–0.12; p = 0.62). Wall:vessel CSA ratio for Fontan patients was 0.13 (IQR, 0.12–0.16) and for controls was 0.13 (IQR, 0.11–0.15) (p = 0.73). There was no association between wall thickness and ventricle morphology, age at catheterisation, age at Fontan, years since Fontan completion, pulmonary artery pressure, and PVR. The vessel media was more readily visualised in control patients.Discussion:OCT of the pulmonary arteries in Fontan patients is safe and feasible. Our OCT findings suggest that during childhood, pulmonary artery wall dimensions are normal in Fontan children with reassuring hemodynamics. Further evaluation of Fontan patients with abnormal hemodynamics and serial evaluation into adulthood are required to conclude on the utility of OCT for identifying early pulmonary artery structural changes.

scholarly journals Pulmonary artery size is associated with functional clinical status in the Fontan circulation

Heart ◽  
2019 ◽  
pp. heartjnl-2019-314972 ◽  
Author(s):  
Floris-Jan S Ridderbos ◽  
Bregje E Bonenkamp ◽  
Sophie L Meyer ◽  
Graziella Eshuis ◽  
Tjark Ebels ◽  
...  
Keyword(s):  
Blood Flow ◽  
Pulmonary Artery ◽  
Pulmonary Blood Flow ◽  
Independent Predictor ◽  
Clinical Status ◽  
Fontan Circulation ◽  
Pulmonary Vasculature ◽  
Cross Sectional ◽  
Fontan Patients

ObjectiveIn the Fontan circulation, non-pulsatile pulmonary blood flow is suggested to negatively affect pulmonary artery growth. The pulmonary vasculature is regarded a key determinant of outcome after Fontan completion. We hypothesised that in Fontan patients pulmonary artery size correlates with follow-up and functional clinical status.MethodsThis is a single-centre, cross-sectional cohort study. Thirty-nine paediatric and adult Fontan patients with a concomitant cardiac magnetic resonance (CMR) scan and a cardiopulmonary exercise test between 2012 and 2013 were included. CMR-derived left and right pulmonary artery cross-sectional areas were expressed as Nakata index. Functional status was defined as peak oxygen consumption (pVO2) indexed for weight, as percentage of predicted (pred) and as New York Heart Association Functional Class (NYHA-FC).ResultsAge at CMR was 18±7.2 years. Time since Fontan completion was 11.9±7.4 years. Nakata index was lower versus the reference values (238.6±78.5 vs 330±30 mm2/m2, p<0.001). Nakata index correlated negatively with age at CMR (r=−0.393, p=0.013) and time since Fontan completion (r=−0.341, p=0.034). pVO2 was 27.9±8.9 mL/min/kg and pVO2pred was 58.1%±14.1%. Nakata index correlated positively with pVO2 (r=0.468, p=0.003) and pVO2pred (r=0.353, p=0.028). Nakata index correlated negatively with NYHA-FC (r=−0.450, p=0.004). Nakata index was an independent predictor (β=0.359, p=0.007) for pVO2 (adjusted R2=0.442, with maximum heart rate and oxygen pulse at peak exercise).ConclusionsPulmonary artery size expressed as Nakata index is a novel independent predictor for functional clinical status. Nakata index negatively correlated with follow-up duration, suggesting that chronic abnormal non-pulsatile pulmonary blood flow plays a role in lagging pulmonary arterial growth in the Fontan circulation.

Sports participation, activity, and obesity in children who have undergone the Fontan procedure

2021 ◽  
pp. 1-5
Author(s):  
Marica E. Baleilevuka-Hart ◽  
Ayesha Khader ◽  
Cesar E Gonzalez De Alba ◽  
Kathryn W. Holmes ◽  
Jennifer H. Huang
Keyword(s):  
Fontan Procedure ◽  
Sports Participation ◽  
Patient Characteristics ◽  
Cross Sectional Study ◽  
Cardiac Complications ◽  
Activity Levels ◽  
Cross Sectional ◽  
Increased Risk ◽  
Cardiology Clinic ◽  
Fontan Patients

Abstract Background: Children with CHD are at risk for obesity and low levels of activity. These factors are associated with an increased risk of poor outcome. Participation in organised sports is an important avenue for children to maintain physical activity, though the relationship between sports participation and obesity has not been examined in the Fontan population. Methods: We performed a cross-sectional study of children aged 8–18 who had been evaluated between January 1, 2015 and October 1, 2019 at the Doernbecher Children’s Hospital outpatient paediatric cardiology clinic and had previously undergone a Fontan. Patients were excluded if they were unable to ambulate independently or if they had undergone a heart transplant. Patient characteristics were recorded from the electronic medical record. Parents were interviewed via a telephone survey and asked to describe their child’s activity levels and sports participation. Results: Our final cohort included 40 individuals, 74% were male. The overall prevalence of obesity (CDC BMI >95% for sex/age) in the cohort (23%) was significantly higher in non-athletes (33%) than athletes (0) (p = 0.02). There was no difference in cardiac complications or comorbidities between athletes and non-athletes. Athletes were more likely to meet daily activity recommendations (p = 0.05). Conclusion: Fontan patients who do not participate in sports are significantly more likely to be obese and less likely to be active than those who do. This is the first study to demonstrate the association between competitive sports participation and decreased likelihood of obesity in the Fontan population.

scholarly journals Case Report of a Gore-Tex TCPC conduit dissection causing severe stenosis

2021 ◽  
Author(s):  
Oliver Bates ◽  
Thomas Semple ◽  
Sylvia Krupickova ◽  
Carles Bautista-Rodriguez
Keyword(s):  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Cross Sectional ◽  
Perimembranous Ventricular Septal Defect ◽  
Complex Anatomy ◽  
Cross Sectional Imaging ◽  
Accessory Hepatic Vein ◽  
Work Up ◽  
Atrioventricular Discordance ◽  
Fontan Patients

Abstract Background The patient is a 15-year-old male with situs inversus, dextrocardia, bilateral superior caval veins, atrioventricular discordance with a single outlet, large perimembranous ventricular septal defect, aortic override, pulmonary atresia and right aortic arch. The complex anatomy with a VSD distant from the aorta (unsuitable for baffling to the aorta) meant he was unsuitable for biventricular repair and proceeded down a univentricular palliation pathway. Case Summary Post TCPC his clinical course was uneventful until the age of five when he developed fatigability with desaturation. An accessory hepatic vein was surgically banded with improved saturations and exercise tolerance. At the age of fifteen CMR was performed to investigate borderline saturations and as work up for transition to adult services. CMR and cardiac CT imaging demonstrated an eccentric thrombus causing stenosis of the extra cardiac conduit and a thrombus outside of the lumen contained by the thin outer membrane of the Gore-Tex conduit. Collateralisation suggested this was longstanding. Cardiac catheterisation demonstrated a 4x6mm stenosis at the junction of the conduit with the pulmonary arteries. The region was successfully balloon dilated and stented with a 34 mm-long Cheatham Platinum (CP) stent, with no complications. Discussion To date this is the first documented case of a dissecting thrombus of a Gore-Tex graft in the literature. This case emphasises the need for anticoagulation and serial cross-sectional imaging (CT or CMR) in Fontan patients with prosthetic grafts throughout a patients’ lifetime.

Magnetic resonance imaging in patients after a modified Fontan operation

1992 ◽  
Vol 2 (2) ◽  
pp. 158-167 ◽  
Author(s):  
Heiko Stern ◽  
Richard Bauer ◽  
Gerrit Schrötera ◽  
Ursula Sauer ◽  
Peter Emmrich ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Right Atrium ◽  
Fontan Procedure ◽  
Pulmonary Veins ◽  
Pulmonary Arteries ◽  
Right Atrial ◽  
Resonance Imaging ◽  
Cross Sectional ◽  
The Right

SummaryMagnetic resonance imaging was performed in 26 patients who underwent a modified Fontan procedure. Their age ranged from 1.8 to 31 years with a mean of 12.2 years. A valveless anastomosis was performed between the right atrium and the rudimentary right ventricle in 12 patients and between the right atrium and the pulmonary arteries in 10 patients. A cavopulmonary connection was established in the remaining four patients. Spin echo and gradient echo scans of the heart were performed in orthogonal and angulated projections. The cross-sectional area of the atrioventricular, atriopulmonary or cavopulmonary anastomoses was measured using diameters in two orthogonal imaging planes. Recordings were examined for the presence of right atrial thrombosis, the site of drainage of the coronary sinus, compression of the pulmonary veins, as well as for the presence and extent of pericardial effusions. Cine recordings were used for the assessment of the pattern of flow within the right atrium. The cross-sectional area of the anastomoses could be determined in 24 of 26 patients. This was not statistically different between patients with different surgical procedures. Patients with a cavopulmonary connection, however, tended to have a smaller anastomosis (mean 1.4 cm2/m2BSA, S.D. 0.62) than patients with atrioventricular (mean 3.0 cm2/m2, S.D. 2.1) or atriopulmonary (2.4 cm2/m2, S.D. 1.1) connections. When compared to normal values for the size of the tricuspid valve, the size of the anastomosis was within the normal range in only four patients, it was larger in one and smaller in 19 patients. There were signs of right atrial thromboses in the scans in eight of 26 patients, as observed by two independent investigators. The site of drainage of the coronary sinus was imaged in 20 of 26 patients and was in accordance with the description of surgical procedure in eight. Compression of the right pulmonary veins by an enlarged right atrium was present in seven patients. This was severe in two children. Presence and extent of pericardial effusions could be adequately assessed in 11 of26 patients. Systolic regurgitation from the rudimentary right ventricle into the right atrium was shown in eight of 12 patients with an atrioventricular valveless anastomosis. Cine recordings revealed slow forward flow from the right atrium into the pulmonary arteries in seven of 22 patients, and there was a markedly altered pattern of intraatrial flow in two patients with anomalous systemic venous connections. Magnetic resonance imaging allows adequate examination of right atrial anatomy, determination of the size of the anastomosis with the pulmonary arteries, and semiquantitative assessment of pulmonary blood flow in the majority of patients after a modified Fontan procedure.

scholarly journals Use of a right ventricular continuous flow pump to validate the distensible model of the pulmonary vasculature

2019 ◽  
pp. 233-243
Author(s):  
F. Vanden Eynden ◽  
P. Segers ◽  
T. Bové ◽  
F. De Somer ◽  
B. El Oumeiri ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Surface Area ◽  
Continuous Flow ◽  
Pulmonary Arteries ◽  
Pulmonary Vasculature ◽  
Flow State ◽  
Mean Flow ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Flow

In the pulmonary circulation, resistive and compliant properties overlap in the same vessels. Resistance varies nonlinearly with pressure and flow; this relationship is driven by the elastic properties of the vessels. Linehanet al. correlated the mean pulmonary arterial pressure and mean flow with resistance using an original equation incorporating the distensibility of the pulmonary arteries. The goal of this study was to validate this equation in an in vivo porcine model. In vivo measurements were acquired in 6 pigs. The distensibility coefficient (DC) was measured by placing piezo-electric crystals around the pulmonary artery (PA). In addition to experiments under pulsatile conditions, a right ventricular (RV) bypass system was used to induce a continuous pulmonary flow state. The Linehanet al. equation was then used to predict the pressure from the flow under continuous flow conditions. The diameter-derived DC was 2.4%/mmHg (+/-0.4%), whereas the surface area-based DC was 4.1 %/mmHg (+/-0.1%). An increase in continuous flow was associated with a constant decrease in resistance, which correlated with the diameter-based DC (r=-0.8407, p=0.044) and the surface area-based DC (r=-0.8986, p=0.028). In contrast to the Linehanet al. equation, our results showed constant or even decreasing pressure as flow increased. Using a model of continuous pulmonary flow induced by an RV assist system, pulmonary pressure could not be predicted based on the flow using the Linehanet al. equation. Measurements of distensibility based on the diameter of the PA were inversely correlated with the resistance.

State-of-the-art pulmonary arterial imaging – Part 2

VASA ◽  
2018 ◽  
Vol 47 (5) ◽  
pp. 361-375 ◽  
Author(s):  
Harold Goerne ◽  
Abhishek Chaturvedi ◽  
Sasan Partovi ◽  
Prabhakar Rajiah
Keyword(s):  
Pulmonary Artery ◽  
State Of The Art ◽  
Therapy Monitoring ◽  
Pulmonary Arteries ◽  
Imaging Modalities ◽  
Cross Sectional ◽  
Multiple Imaging ◽  
Cross Sectional Imaging ◽  
Pulmonary Arterial ◽  
Arterial Imaging

Abstract. Although pulmonary embolism is the most common abnormality of the pulmonary artery, there is a broad spectrum of other congenital and acquired pulmonary arterial abnormalities. Multiple imaging modalities are now available to evaluate these abnormalities of the pulmonary arteries. CT and MRI are the most commonly used cross-sectional imaging modalities that provide comprehensive information on several aspects of these abnormalities, including morphology, function, risk-stratification and therapy-monitoring. In this article, we review the role of state-of-the-art pulmonary arterial imaging in the evaluation of non-thromboembolic disorders of pulmonary artery.

A STUDY ON THE CORRELATION BETWEEN MORPHOLOGIC ANORMALIES AND LEFT VENTRICULAR DIASTOLIC FUNCTION IN DILATED CARDIO-MYOPATHY

2011 ◽  
pp. 137-144
Author(s):  
Thi Ngoc Ha Hoang ◽  
Anh Vu Nguyen ◽  
Minh Loi Hoang ◽  
Cuu Long Nguyen ◽  
Thi Thuy Hang Nguyen
Keyword(s):  
Pulmonary Artery ◽  
Dilated Cardiomyopathy ◽  
Diastolic Dysfunction ◽  
Diastolic Function ◽  
Pulmonary Arteries ◽  
Left Ventricular ◽  
University Hospital ◽  
Cross Sectional ◽  
X Ray ◽  
Key Words Dilated Cardiomyopathy

Purposes: Describe the morphological and diastolic function of left ventricular changes in the patients with dilated cardiomyopathy (DCM) on US, X-ray findings, and Evaluate the correlation between morphology and diastolic function of left ventricular. Materials and method: Cross sectional study from Dec 2009 to Aug 2010, on 39 patients with dilated cardiomyopathy were evaluated at the University Hospital of Hue College of Medical and Pharmaceutical. Results: 1. X-ray and US findings characteristics of DCM is significantly increased in diameter of L, H and mG; LVM, LVMI, LVDd and LAD. 2. The pression of pulmonary artery has been significantly increased with redistribution pulmonary arteries in 61.5% cases and 23.1% have reversed pulmonary artery distribution. 3. DCM have diastolic dysfunction in 100% patients, including severe disorders to 61.5%; the restrictive dysfunction has ratio E/A>2 and E/Em average was 23.89± 17.23. 4.The correlation between the morphology and function in DCM: the diameter of H and L on the X-ray, LAD and ratio LA/AO on US correlated with the level of diastolic dysfunction (p< 0.05). All three radiographic parameters on the radio standard (H, L, the index Cardio/Thoracic) and LVDd on US have negative correlated with EF and FS with p <0.05. Key words: dilated cardiomyopathy, diastolic dysfunction, cardiac tissue Doppler, reversed pulmonary artery distribution

Localization and Distribution of Endothelin Receptor Subtypes in Pulmonary Vasculature of Normal and Hypoxia-Exposed Rats

1999 ◽  
Vol 20 (4) ◽  
pp. 620-630 ◽  
Author(s):  
Sanae Soma ◽  
Hideki Takahashi ◽  
Masashi Muramatsu ◽  
Masahiko Oka ◽  
Yoshinosuke Fukuchi
Keyword(s):  
Pulmonary Vasculature ◽  
Receptor Subtypes ◽  
Endothelin Receptor
Sign in / Sign up

Export Citation Format

Share Document