Characterization and treatment of systemic venous to pulmonary venous collaterals seen after the Fontan operation

2003 ◽  
Vol 13 (5) ◽  
pp. 424-430 ◽  
Author(s):  
Hisashi Sugiyama ◽  
Shi-Joon Yoo ◽  
William Williams ◽  
Lee N. Benson
Keyword(s):  
Fontan Operation ◽  
Fontan Circulation ◽  
Brachiocephalic Vein ◽  
Interventional Treatment ◽  
Mean Pulmonary Arterial Pressure ◽  
Venous Collaterals ◽  
Pulmonary Arterial ◽  
Transcatheter Interventions ◽  
Cardiac Catherization

Objectives: To determine the anatomical characteristics of systemic venous collaterals formed after the Fontan operation, and the efficacy of a transcatheter strategy for management. Methods: We reviewed retrospectively the data from cardiac catherization of 50 persistently cyanotic patients after the Fontan operation. Results: A total of 54 transcatheter interventions were performed, at a mean age of 6.3 ± 3.5 years, a mean interval of 2.7 ± 2.9 years from completion of the Fontan circulation. Of 38 patients who had fenestration of the baffle at the time of surgery, 25 had patency of the fenestration, and 24 had the fenestration occluded with a device at the time of interventional treatment for associated venous collaterals. We identified a total of 68 systemic venous collateral channels, of which 36 (53%) were supracardiac, 12 (18%) cardiac, and 20 (29%) infracardiac in origin. The most common site of origin was the brachiocephalic vein (44%), followed by the left phrenic vein (25%). A longer time from surgery, at 3.3 ± 3.4 years, was associated with the identification of collaterals having a diameter larger than 4 mm (p < 0.01). The mean pulmonary arterial pressure was higher in those with larger compared to those with smaller collaterals (13.3 ± 2.8 versus 11.1 ± 2.0 mmHg, p < 0.01). Coils were used for occlusion of 61 vessels, and a Rashkind™ occluder for the remaining 7. After exclusion of the patients undergoing simultaneous closure of their fenestration, systemic saturation of oxygen increased from 89 ± 6% to 95 ± 3% (p < 0.01). Conclusion: Venous collateral channels are common in patients suffering progressive cyanosis in the setting of the Fontan circulation. The collaterals increase in size with time, and are associated with higher pulmonary arterial pressures. Transcatheter treatment is feasible, and results in resolution of cyanosis. Only continuing follow-up will show whether further collateralization occurs in time.

Diagnosis and clinical investigation of patients presenting with pulmonary hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2507-2511 ◽  
Author(s):  
Daniela Calderaro ◽  
Luis Felipe Prada ◽  
Rogério Souza
Keyword(s):  
Pulmonary Hypertension ◽  
Clinical Investigation ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Reference Centre ◽  
Mean Pulmonary Arterial Pressure ◽  
Invasive Test ◽  
Pulmonary Arterial

The diagnosis of pulmonary hypertension (PH) relies on the haemodynamic criterion of mean pulmonary arterial pressure greater than or equal to 25 mmHg, assessed by right heart catheterization. The scope of this chapter is to discuss the key elements of clinical assessment of PH patients and the decision process to indicate right heart catheterization. Investigation must get through all the possible causes of PH according to their probability and frequency in the population. Echocardiography is the most important non-invasive test as an indicator for further diagnostic evaluation. Patients who are eligible for right heart catheterization should always be referred to PH centres, where technical skills and standardized procedures will enable maximal reliability of haemodynamic measurement. In the reference centre, a multidisciplinary team will discuss clinical and haemodynamic data, to propose the best therapeutic and follow-up schedule.

scholarly journals Echocardiographic parameters related to pulmonary arterial hypertension in patients with chronic myeloid leukemia under dasatinib treatment

2021 ◽  
Vol 21 (4) ◽  
pp. 682-691
Author(s):  
Karla Poot Noh ◽  
Ernesto Hernández Jiménez ◽  
María del Rayo Juárez Santiesteban ◽  
Patricia Zaqoya Martínez ◽  
Alvaro J. Monliel Jarquin ◽  
...  
Keyword(s):  
Treatment Time ◽  
Pearson Correlation ◽  
Systolic Pressure ◽  
Pulmonary Artery Systolic Pressure ◽  
Cross Sectional ◽  
Mean Pulmonary Arterial Pressure ◽  
Ventricular Diastolic Dysfunction ◽  
Echocardiographic Parameters ◽  
Pulmonary Arterial

Introduction: The use of dasatinib in patients with CML has improved life expectancy and follow-up with transthoracic echocardiography (ECOTT) for early detection of PAH allows modifications to the treatment. Objective: To determine the echocardiographic parameters and echocardiographic probability for PAH in patients with CML treated with dasatinib. Methods: Correlation, cross-sectional, retrospective, single-center study; patients with CML treated with dasatinib were included. Spearman and Pearson correlation was used. Results: 16 patients were analyzed, mean age 53.5 years; 62.5% men, 37.5% women. The dasatinib dose was 50 mg / day in 18.7%, and 100 mg / day in 81.2%, mean pulmonary arterial pressure (mPAP) 26.3 mmHg, mean maximum tricuspid regurgitation velocity (VmxRT) 2.9 m / s, mean pulmonary artery systolic pressure (PSAP) 41 mmHg. 56.2% had right ventricular diastolic dysfunction (RVDD). 43% were categorized as low probability for PAH, 18.7% intermediate, and 37.5% as high. Relationship between PAPm and VmxRT with p = 0.012. Relationship between mPAP and RV diastolic function, with p = 0.002. Relationship between probability for PAH and mPAP, with p = 0.008. Conclusion: The echocardiographic parameters PAPm, VmxRT, PSAP, DDVD and echocardiographic probability for PAH are useful and necessary for the diagnosis of PAH. The determination of all these parameters should be carried out early and as a follow-up, since a considerable positive relationship was found for each one with the presence of PAH, which is not dependent on the treatment time or the dose of dasatinib.

scholarly journals Incidence of pulmonary hypertension and determining factors in patients with systemic sclerosis

2018 ◽  
Vol 51 (4) ◽  
pp. 1701197 ◽  
Author(s):  
J. Gerry Coghlan ◽  
Matthias Wolf ◽  
Oliver Distler ◽  
Christopher P. Denton ◽  
Martin Doelberg ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Vena Cava ◽  
Diffusion Capacity ◽  
Mean Pulmonary Arterial Pressure ◽  
Right Heart Catheterisation ◽  
Determining Factors ◽  
Pulmonary Arterial ◽  
Velocity Diffusion

The objective of this study was to evaluate the incidence of pulmonary hypertension (PH) and determining factors in patients with systemic sclerosis (SSc) and a diffusing capacity of the lung for carbon monoxide (DLCO) <60% predicted.In this bicentric, prospective cohort study, patients with SSc were clinically assessed at baseline and after 3 years, including right heart catheterisation (RHC). Analysis of determining factors for the development of PH was performed using univariate and multivariate analyses.96 patients with a mean pulmonary arterial pressure (mPAP) <25 mmHg at baseline were followed for 2.95±0.7 years (median 3 years). Of these, 71 had a second RHC; 18 of these 71 patients (25.3%) developed PH, and five (7%) developed SSc-associated pulmonary arterial hypertension. For patients with an mPAP of 21–24 mmHg at baseline, the likelihood of presenting with PH as opposed to normal pressures on follow-up was significantly higher (p=0.026). Pulmonary vascular resistance, tricuspid regurgitation velocity, diffusion capacity and the size of the inferior vena cava at baseline were independent predictors for the development of PH during follow-up.In a selected cohort of SSc patients with a DLCO <60%, pulmonary pressures appeared to rise progressively during follow-up. In this population, it was possible to identify manifest PH in almost 25% of patients using prospective RHC during follow-up. Therefore, regular clinical assessment including RHC might be useful in patients with SSc.

scholarly journals Right ventricular reverse remodeling assessed by echocardiography as a new goal-oriented treatment strategy in PAH patients

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
C Fauvel ◽  
O Raitiere ◽  
N Si-Belkacem ◽  
C Viacroze ◽  
E Artaud-Macari ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Cox Regression ◽  
Conditional Inference ◽  
Reverse Remodeling ◽  
Free Survival ◽  
Cox Regression Analysis ◽  
Mean Pulmonary Arterial Pressure ◽  
Conditional Inference Trees ◽  
Pulmonary Arterial

Abstract Background While in heart failure with reduced ejection fraction, left ventricular reverse remodeling assessed by transthoracic echocardiography (TTE) is associated with better prognosis, right ventricular reverse remodeling (RVRR) was less investigated in pulmonary arterial hypertension (PAH) Purpose We aimed to investigate whether RVVR assessed by echocardiography could help to stratify PAH patient's prognosis. Methods Between 2002 and 2019, all consecutive PAH patients were included, treated and followed in a single PAH center in accordance with the current ESC/ERS guidelines. In addition to regular risk stratification parameters, we measured several echocardiographic RV systolic function and size parameters, including tricuspid annular plane systolic excursion (TAPSE, mm) or RV-end diastolic area (cm2) from apical-4 chamber view both at baseline, 1-year of follow-up as well as their change. Primary composite outcome was three-year transplant-free survival and death from all cause from the 1-year evaluation. Conditional inference trees were used to determine which TTE parameters and cutoffs values were associated with primary outcome from hierarchy of multiple covariates in multivariable Cox regression analysis. Kaplan-Meier curves were then drawn and compared with log-rank test. Results 126 incident PAH patients were included (63% female, mean age 59±18 yo), mainly due to connectivite-tissue disease and idiopathic PAH (26% and 22% respectively). At baseline, mean pulmonary arterial pressure was 42 (33, 52) mmHg. At 1-y follow-up under pulmonary vasodilation therapy, NYHA (p&lt;0.01), NTproBNP (p&lt;0.01), mean pulmonary arterial pressure (p&lt;0.01) and cardiac index (p&lt;0.01) were significantly improved compared to baseline. Conditional inference trees showed that 1-year TAPSE gain &gt;1 mm and 1-year RV end-diastolic area decreased &gt;2 cm2 were associated with 3-year transplant-free survival in multivariable Cox regression analysis (HR=0.23, 95% CI [0.08–0.61] p=0,0035, HR=0.34, 95% CI [0.12–0.94], p=0.038). Simple score from 0 (absence of RVRR), 1 (partial RVRR) and 2 (complete RVRR), describing the number of TTE parameters reach at 1-year was then investigated. Patients with complete RVRR depicted better transplant-free survival than partial or absence of RVRR, log-rank p&lt;0.001 (figure). Conclusion Complete reverse remodeling from right ventricular size and function could represent a new goal-oriented treatment strategy in PAH patients. FUNDunding Acknowledgement Type of funding sources: None. RVRR survival curves

Abstract 17119: Sufficient Pulmonary Vascular Bed Before Fontan Surgery Secures Favorable Fontan Hemodynamics

Circulation ◽  
2018 ◽  
Vol 138 (Suppl_1) ◽  
Author(s):  
Seiko Kuwata ◽  
Hirofumi Saiki ◽  
Manabu Takanashi ◽  
Kenji Sugamoto ◽  
Hideaki Senzaki
Keyword(s):  
Organ Dysfunction ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Fontan Circulation ◽  
Cardiovascular Remodeling ◽  
Vascular Bed ◽  
Pulmonary Arterial ◽  
Fontan Surgery ◽  
Fontan Patients ◽  
Pulmonary Vascular Bed

Introduction: While improvement of perioperative care markedly expanded candidacy of Fontan surgery, increasing number of Fontan patients is currently subjected to advanced heart failure therapies. Low pulmonary flow before Fontan procedure has been considered to be acceptable as it keeps pulmonary resistance low, however, compromised growth of pulmonary vascular bed after Fontan completion may deteriorate potential to preserve better Fontan circulation later. We tested our hypothesis that sufficient growth of pulmonary vascular bed before Fontan surgery chronically preserves favorable Fontan hemodynamics and prevents patients from cardiovascular remodeling. Methods and Results: Consecutive 33 patients who had undergone Fontan operation (median year after the surgery: 5.3years) were enrolled in this study. During cardiac catheterization, pulmonary arterial index (PAI) as the representative of vascular bed both before and after Fontan procedure was measured and hemodynamics as well as blood samples as a marker for end-organ dysfunction were analyzed. Before Fontan procedure, PAI ranged 74-426 and resistance of pulmonary artery (PAR) ranged 0.4-3.1. After median of 5.3 years of Fontan procedure, CVP exhibited modest decline with preserving cardiac index (CI). Interestingly, central venous pressure (CVP) with chronic Fontan circulation was negatively correlated with PAI of pre-Fontan procedure (P=0.02) whereas it was independent of PAR. Consistent with this, higher PAI before Fontan procedure was also significantly associated with higher CI (P<0.01) and lower levels of plasma brain natriuretic peptides (P=0.03). Importantly, patients with low PAI showed markedly elevated activation of renin-angiotensin-aldosterone (RAAS) activation and elevation of serum gamma-gltamyl transferase (P<0.01), a surrogate marker of liver congestion. Multivariate analysis revealed that high PAI was determinant of low CVP in the chronic Fontan patients, independent of body size, postoperative interval and pulmonary arterial resistance. Conclusion: Sufficient pulmonary vascular bed before Fontan procedure independently lowers CVP, may prevent cardiovascular remodeling by RAAS suppression as well as end organ dysfunction in chronic Fontan patients. Our result suggested importance of strategies to accelerate pulmonary vascular growth before Fontan procedure.

scholarly journals Pulmonary hypertension in systemic sclerosis: diagnosis by systematic screening and prognosis after three years follow-up

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Verônica Silva Vilela ◽  
Marcio Macri Dias ◽  
Ângelo Antunes Salgado ◽  
Bruno Rangel Antunes da Silva ◽  
Agnaldo José Lopes ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Right Heart Catheterization ◽  
Diagnostic Tools ◽  
Heart Catheterization ◽  
Systematic Screening ◽  
Mean Pulmonary Arterial Pressure ◽  
Common Group ◽  
Pulmonary Arterial

Abstract Background Systemic sclerosis (SSc) is a rare disease, and the presence of pulmonary hypertension can be a determining factor in prognosis. The aim of this study was to evaluate the diagnosis, profile, and prognosis of systemic sclerosis pulmonary hypertension (SSc-PH) diagnosed by systematic screening in a Brazilian population. Methods A cohort of SSc patients underwent systematic screening for SSc-PH. Patients were referred for right heart catheterization (RHC) according to transthoracic echocardiogram or a combination of diagnostic tools. The clinical, immunological, and hemodynamic features and prognosis after 3 years were evaluated. Results Twenty patients underwent RHC. SSc pulmonary arterial hypertension (SSc-PAH) was the most common group of SSc-PH. These patients had long disease duration, high urate levels and highly elevated mean pulmonary arterial pressure (mPAP) and peripheral vascular resistance (PVR) on hemodynamics. Patients with mPAP > 20– < 25 mmHg had hemodynamic features of intermediate disease. Patients with SSc-PH associated to interstitial lung disease (SSc-ILD-PH) had signs of vasculopathy on hemodynamics. In patients with no-SSc-PH, the survival at 1, 2, and 3 years was 96%, 92% and 92%, respectively and in patients with SSc-PH it was 86.7%, 60% and 53.3%, respectively. Conclusions Patients identified with SSc-PAH and SSc-ILD-PH in our screening had severe clinical and hemodynamic features. Mortality remains high in SSc-PH but was more related to Bo-PAH and SSc-ILD-PH, while in SSc-PAH, the prognosis was better. Trial registration: Current Controlled Trials ISRCTN 72968188, July 8th, 2021. Retrospectively registered.

scholarly journals Use of Pulmonary Arterial Hypertension Therapies in Patients with a Fontan Circulation: Current Practice Across the United Kingdom

2021 ◽  
Author(s):  
Andrew Constantine ◽  
Konstantinos Dimopoulos ◽  
Petra Jenkins ◽  
Robert M. R. Tulloh ◽  
Robin Condliffe ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Congenital Heart ◽  
Fontan Circulation ◽  
Adult Patients ◽  
The United Kingdom ◽  
Pulmonary Arterial

Background The Fontan circulation is a successful operative strategy for abolishing cyanosis and chronic volume overload in patients with congenital heart disease with single ventricle physiology. “Fontan failure” is a major cause of poor quality of life and mortality in these patients. We assessed the number and clinical characteristics of adult patients with Fontan physiology receiving pulmonary arterial hypertension (PAH) therapies across specialist centers in the United Kingdom. Methods and Results We identified all adult patients with a Fontan‐type circulation under active follow‐up in 10 specialist congenital heart disease centers in England and Scotland between 2009 and 2019. Patients taking PAH therapies were matched to untreated patients. A survey of experts was also performed. Of 1538 patients with Fontan followed in specialist centers, only 76 (4.9%) received PAH therapies during follow‐up. The vast majority (90.8%) were treated with a phosphodiesterase‐5 inhibitor. In 33% of patients, PAH therapies were started after surgery or during hospital admission. In the matched cohort, treated patients were more likely to be significantly limited, have ascites, have a history of protein‐losing enteropathy, or receive loop diuretics ( P <0.0001 for all), also reflecting survey responses indicating that failing Fontan is an important treatment target. After a median of 12 months (11–15 months), functional class was more likely to improve in the treated group ( P =0.01), with no other changes in clinical parameters or safety issues. Conclusions PAH therapies are used in adult patients with Fontan circulation followed in specialist centers, targeting individuals with advanced disease or complications. Follow‐up suggests stabilization of the clinical status after 12 months of therapy.

Emergency department utilisation and critical readmission in patients with Fontan circulation

2020 ◽  
Vol 30 (12) ◽  
pp. 1902-1909
Author(s):  
Katherine Cashen ◽  
Tara L. Petersen ◽  
Cailyn Rood ◽  
Daniel Cater ◽  
Sheila F. Waslawski ◽  
...  
Keyword(s):  
Emergency Department ◽  
Fontan Operation ◽  
Fontan Circulation ◽  
Emergency Department Care ◽  
Multi Centre Study ◽  
Centre Study ◽  
Racial Background ◽  
American Black ◽  
Emergency Department Utilisation

AbstractBackground:We aimed to conduct a multi-centre study characterising emergency department utilisation and critical readmissions experienced by children with Fontan circulation.Methods:We conducted a retrospective review of children who underwent the Fontan operation at three institutions (i.e., centres A, B, and C) between 2009 and 2014, with follow-up through December 2015. Multi-variable analyses were performed to determine factors associated for emergency department utilisation within 1 year of surgery, emergency department utilisation at any time following surgery, or critical readmission (defined as admission to ICU, operating room, or cardiac catheterisation).Results:We reviewed 297 patients, of which 147 patients (49%) had 607 emergency department encounters. Forty-six patients (15%) required 71 critical readmissions. Multi-variable analyses revealed centre C (p = 0.02) and post-operative hospitalisation ≥ 14 days (p = 0.03) to be significantly associated with emergency department utilisation within 1 year, whereas centre B (p < 0.001), post-operative hospitalisation ≥ 14 days (p = 0.002), and African-American/Black race (p = 0.04) were significantly associated with critical readmission.Conclusions:In this multi-centre study, nearly half of patients with Fontan circulation received emergency department care, often presenting with high disease acuity requiring readmission. Emergency department utilisation and need for critical readmission were independently influenced by the centre at which surgery was performed, prolonged post-operative hospitalisation, and racial background. These data could help guide quality improvement efforts aimed at reducing morbidity in this unique patient population.

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