scholarly journals Pulmonary Vascular Disease in the Single-Ventricle Patient: Is it Really Pulmonary Hypertension and if So, How and When Should We Treat it?

2019 ◽  
Vol 18 (1) ◽  
pp. 14-18
Author(s):  
Stephanie S. Handler ◽  
Jeffrey A. Feinstein
Keyword(s):  
Pulmonary Blood Flow ◽  
Single Ventricle ◽  
Fontan Circulation ◽  
Pulmonary Vascular Disease ◽  
Vasodilator Therapy ◽  
Pulmonary Vasodilators ◽  
Pulmonary Vasodilator ◽  
Late Morbidity ◽  
Fontan Patients

Despite significant improvements in the surgical and postoperative care for patients with single-ventricle physiology culminating in the Fontan circulation, significant late morbidity and mortality remains. In the setting of passive (ie, non-“pump” driven) pulmonary blood flow, pulmonary vascular resistance (PVR) plays a key role in determining cardiac output, and even slight elevations in PVR may result in significant morbidity. There is now great interest to treat Fontan patients with pulmonary vasodilators in an attempt to “optimize” PVR (and by extension, quality of life) and/or improve an elevated PVR. This review discusses the hemodynamic implications of the Fontan circulation, the evidence for use of pulmonary vasodilator therapy, and possible target physiologic mechanisms.

scholarly journals Fontan failure and the role of pulmonary vasodilator therapy

2020 ◽  
Vol 4 (S1) ◽  
Author(s):  
Paul Clift
Keyword(s):  
Fontan Circulation ◽  
Atrial Arrhythmias ◽  
Fontan Patient ◽  
Vasodilator Therapy ◽  
Protein Losing Enteropathy ◽  
Palliative Procedure ◽  
Pulmonary Vasodilators ◽  
Pulmonary Vasodilator ◽  
Late Morbidity

Abstract Background The Fontan circulation is a palliative procedure for patients born with a single ventricle physiology. The Fontan circulation is associated with significant late morbidity commonly including atrial arrhythmias. Case presentation We report a case of an adult Fontan patient who developed protein losing enteropathy (PLE) following persistent atrial arrhythmias. Treatment with a pulmonary vasodilator, sildenafil, resolved the PLE. Conclusions The importance of a low pulmonary vascular resistance to maintain wellbeing in the Fontan patient is discussed, as is the role of pulmonary vasodilators.

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 Pulmonary Vasodilator Therapy in Children with Single Ventricle Physiology: Effects on Saturation and Pulmonary Arterial Pressure

2020 ◽  
Vol 41 (8) ◽  
pp. 1651-1659
Author(s):  
Ida Jeremiasen ◽  
Karin Tran-Lundmark ◽  
Nikmah Idris ◽  
Phan-Kiet Tran ◽  
Shahin Moledina
Keyword(s):  
Arterial Pressure ◽  
Single Ventricle ◽  
Pulmonary Arterial Pressure ◽  
Vasodilator Therapy ◽  
Single Ventricle Physiology ◽  
Pulmonary Vasodilators ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Vasodilator ◽  
Pulmonary Arterial ◽  
Stage 1

AbstractIn children with single ventricle physiology, increased pulmonary vascular resistance may impede surgical progression or result in failing single ventricle physiology. The use of pulmonary vasodilators has been suggested as a potential therapy. However, knowledge on indication, dosage, and effect is limited. A retrospective case notes review of all (n = 36) children with single ventricle physiology, treated with pulmonary vasodilators by the UK Pulmonary Hypertension Service for Children 2004–2017. Therapy was initiated in Stage 1 (n = 12), Glenn (n = 8), or TCPC (n = 16). Treatment indications were high mean pulmonary arterial pressure, cyanosis, reduced exercise tolerance, protein-losing enteropathy, ascites, or plastic bronchitis. Average dose of sildenafil was 2.0 mg/kg/day and bosentan was 3.3 mg/kg/day. 56% had combination therapy. Therapy was associated with a reduction of the mean pulmonary arterial pressure from 19 to 14 mmHg (n = 17, p < 0.01). Initial therapy with one or two vasodilators was associated with an increase in the mean saturation from 80 to 85%, (n = 16, p < 0.01). Adding a second vasodilator did not give significant additional effect. 5 of 12 patients progressed from Stage 1 to Glenn, Kawashima, or TCPC, and 2 of 8 from Glenn to TCPC during a mean follow-up time of 4.7 years (0–12.8). Bosentan was discontinued in 57% and sildenafil in 14% of treated patients and saturations remained stable. Pulmonary vasodilator therapy was well tolerated and associated with improvements in saturation and mean pulmonary arterial pressure in children with single ventricle physiology. It appears safe to discontinue when no clear benefit is observed.

scholarly journals Treatment of exercise pulmonary hypertension improves pulmonary vascular distensibility

2018 ◽  
Vol 8 (3) ◽  
pp. 204589401878738 ◽  
Author(s):  
William D. Wallace ◽  
Mehdi Nouraie ◽  
Stephen Y. Chan ◽  
Michael G. Risbano
Keyword(s):  
Pulmonary Hypertension ◽  
Vascular Disease ◽  
Arterial Compliance ◽  
Bicycle Ergometer ◽  
Peak Exercise ◽  
Pulmonary Vascular Disease ◽  
Heart Catheterization ◽  
Vasodilator Therapy ◽  
Pulmonary Vasodilators ◽  
Pulmonary Vasodilator

Exercise pulmonary hypertension (ePH) is an underappreciated form of exertional limitation. Despite normal resting pulmonary artery pressures, patients with ePH demonstrate early pulmonary vascular changes with reduced pulmonary arterial compliance (PAC) and vascular distensibility (α). Recent data suggest that targeted vasodilator therapy may improve hemodynamics in ePH, but it is not well-known whether such medications alter pulmonary vascular distensibility. Thus, we sought to evaluate if vasodilator therapy improved α a marker of early pulmonary vascular disease in ePH. Ten patients performed supine exercise right heart catheterization (exRHC) with bicycle ergometer to peak exercise. Patients diagnosed with ePH were treated with pulmonary vasodilators. A repeat symptom-limited exercise RHC was performed at least six months after therapy. Patients with ePH had evidence of early pulmonary vascular disease, as baseline PAC and α were reduced. After pulmonary vasodilator therapy, a number of peak exercise hemodynamics statistically improved, including a decrease of total pulmonary resistance and pulmonary vascular resistance, while cardiac output increased. Importantly, vasodilator therapy partially reversed the pathogenic decreases of α at the time of repeat exRHC. Pulmonary vascular distensibility, α, a marker of early pulmonary vascular disease, improves in ePH after therapy with pulmonary vasodilators.

The Fontan outcomes network: first steps towards building a lifespan registry for individuals with Fontan circulation in the United States

2020 ◽  
Vol 30 (8) ◽  
pp. 1070-1075 ◽  
Author(s):  
Tarek Alsaied ◽  
Kiona Y. Allen ◽  
Jeffrey B. Anderson ◽  
Julia S. Anixt ◽  
David W. Brown ◽  
...  
Keyword(s):  
Congenital Heart ◽  
Single Ventricle ◽  
Emotional Health ◽  
The United States ◽  
Fontan Circulation ◽  
Systematic Design ◽  
Neurodevelopmental Outcomes ◽  
New Learning ◽  
Outcomes For Children

AbstractThe Fontan Outcomes Network was created to improve outcomes for children and adults with single ventricle CHD living with Fontan circulation. The network mission is to optimise longevity and quality of life by improving physical health, neurodevelopmental outcomes, resilience, and emotional health for these individuals and their families. This manuscript describes the systematic design of this new learning health network, including the initial steps in development of a national, lifespan registry, and pilot testing of data collection forms at 10 congenital heart centres.

scholarly journals Pulmonary vasodilator therapy as treatment for patients with a Fontan circulation: the Emperor’s new clothes?

2018 ◽  
Vol 8 (4) ◽  
pp. 204589401881114 ◽  
Author(s):  
Floris-Jan S. Ridderbos ◽  
Quint A.J. Hagdorn ◽  
Rolf M.F. Berger
Keyword(s):  
Fontan Circulation ◽  
Vasodilator Therapy ◽  
Pulmonary Vasodilator

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.

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