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.

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 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.

Treatment strategies for protein-losing enteropathy in Fontan-palliated patients

2020 ◽  
Vol 30 (5) ◽  
pp. 698-709
Author(s):  
Anastasia Schleiger ◽  
Stanislav Ovroutski ◽  
Björn Peters ◽  
Stephan Schubert ◽  
Joachim Photiadis ◽  
...  
Keyword(s):  
Cardiac Transplantation ◽  
Fontan Procedure ◽  
Disease Onset ◽  
Treatment Strategies ◽  
Individual Treatment ◽  
Interventional Treatment ◽  
Vasodilator Therapy ◽  
Protein Losing Enteropathy ◽  
Pulmonary Vasodilator

AbstractObjective:Protein-losing enteropathy is an infrequent but severe condition occurring after Fontan procedure. The multifactorial pathogenesis remains unclear and no single proposed treatment strategy has proven universally successful. Therefore, we sought to describe different treatment strategies and their effect on clinical outcome and mortality.Material and Methods:We performed a retrospective observational study. From the total cohort of 439 Fontan patients treated in our institution during the study period 1986–2019, 30 patients (6.8%) with protein-losing enteropathy were identified. Perioperative, clinical, echocardiographic, laboratory, and invasive haemodynamic findings and treatment details were analysed.Results:Median follow-up after disease onset was 13.1 years [interquartile range 10.6]. Twenty-five patients received surgical or interventional treatment for haemodynamic restrictions. Medical treatment, predominantly pulmonary vasodilator and/or systemic anti-inflammatory therapy with budesonide, was initiated in 28 patients. In 15 patients, a stable remission could be achieved by medical or surgical procedures (n = 3 each), by combined multimodal therapy (n = 8), or ultimately by cardiac transplantation (n = 1). Phrenic palsy, bradyarrhythmia, Fontan pathway stenosis, and absence of a fenestration were significantly associated with development of protein-losing enteropathy (p = 0.001–0.48). Ten patients (33.3%) died during follow-up; 5-year survival estimate was 96.1%. In unadjusted analysis, medical therapy with budesonide and pulmonary vasodilator therapy in combination was associated with improved survival.Conclusions:Protein-losing enteropathy is a serious condition limiting survival after the Fontan procedure. Comprehensive assessment and individual treatment strategies are mandatory to achieve best possible outcome. Nevertheless, relapse is frequent and long-term mortality substantial. Cardiac transplantation should be considered early as treatment option.

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.

scholarly journals State of the art of the Fontan strategy for treatment of univentricular heart disease

F1000Research ◽  
2018 ◽  
Vol 7 ◽  
pp. 935 ◽  
Author(s):  
Jelle P. G. van der Ven ◽  
Eva van den Bosch ◽  
Ad J.C.C. Bogers ◽  
Willem A. Helbing
Keyword(s):  
State Of The Art ◽  
Surgical Techniques ◽  
Volume Overload ◽  
Ventricular Volume ◽  
Fontan Circulation ◽  
Circulatory Support ◽  
Univentricular Heart ◽  
High Morbidity ◽  
Fontan Patient ◽  
Protein Losing Enteropathy

In patients with a functionally univentricular heart, the Fontan strategy achieves separation of the systemic and pulmonary circulation and reduction of ventricular volume overload. Contemporary modifications of surgical techniques have significantly improved survival. However, the resulting Fontan physiology is associated with high morbidity. In this review, we discuss the state of the art of the Fontan strategy by assessing survival and risk factors for mortality. Complications of the Fontan circulation, such as cardiac arrhythmia, thromboembolism, and protein-losing enteropathy, are discussed. Common surgical and catheter-based interventions following Fontan completion are outlined. We describe functional status measurements such as quality of life and developmental outcomes in the contemporary Fontan patient. The current role of drug therapy in the Fontan patient is explored. Furthermore, we assess the current use and outcomes of mechanical circulatory support in the Fontan circulation and novel surgical innovations. Despite large improvements in outcomes for contemporary Fontan patients, a large burden of disease exists in this patient population. Continued efforts to improve outcomes are warranted. Several remaining challenges in the Fontan field are outlined.

Late presentation of the Fontan circulation

2020 ◽  
pp. 29-40
Author(s):  
Hannah Bellsham-Revell ◽  
Aaron Bell ◽  
Catherine Head
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Medical Therapy ◽  
Congenital Heart ◽  
Late Presentation ◽  
Fontan Circulation ◽  
Complex Congenital Heart Disease ◽  
Fontan Patient ◽  
Protein Losing Enteropathy ◽  
Life Threatening

A 16-year-old boy presented to the congenital heart disease department with oedema and chronic cough productive of casts. He had previously undergone Fontan completion for complex congenital heart disease overseas. Examination and basic investigations showed relapsed protein-losing enteropathy and plastic bronchitis, two significant complications of the Fontan circulation. He was extensively investigated, and his medical therapy optimized. Although initially referred for heart transplant assessment, he has been taken off the transplant list after good response to medical therapy alone. This case shows the complex multidisciplinary management of the adolescent Fontan patient with life-threatening complications.

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
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