scholarly journals The effects of oral pulmonary vasodilator therapy on poor candidates for fenestration closure in patients with a fontan circulation

Author(s):  
Tamir Dagan ◽  
Elchanan Bruckheimer ◽  
Gabriel Amir ◽  
Georgy Frenkel ◽  
Michael Levinson ◽  
...  
2020 ◽  
Vol 4 (S1) ◽  
Author(s):  
Paul Clift

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.


2019 ◽  
Vol 18 (1) ◽  
pp. 14-18
Author(s):  
Stephanie S. Handler ◽  
Jeffrey A. Feinstein

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.


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

2011 ◽  
Vol 6 (2) ◽  
pp. 139-146 ◽  
Author(s):  
Showshan Yang-Ting ◽  
Jamil Aboulhosn ◽  
Xing-Guo Sun ◽  
John S. Child ◽  
Kathy E. Sietsema

Heart ◽  
2017 ◽  
Vol 104 (9) ◽  
pp. 732-737 ◽  
Author(s):  
Clare Arnott ◽  
Geoff Strange ◽  
Andrew Bullock ◽  
Adrienne C Kirby ◽  
Clare O’Donnell ◽  
...  

ObjectiveEisenmenger syndrome (ES) is a severe form of pulmonary hypertension in adults with congenital heart disease (CHD) and has a poor prognosis. We aimed to understand factors associated with survival in ES and particularly to assess the potential benefits of advanced pulmonary vasodilator therapy (AT).MethodsFrom January 2004, when AT became generally available for patients with ES, we followed 253 ES adults from 12 adult congenital heart disease centres across Australia and New Zealand. Demographic, medical and outcome data were collected and analysed prospectively and retrospectively.ResultsThe patients with ES were predominantly female (60%), aged 31 (SD 12) years. At diagnosis of ES, 64% were WHO functional class ≥3. The most common underlying lesion was ventricular septal defect (33%) with 21% having ‘complex’ anatomy. Over a median follow-up time of 9.1 years, the majority (72%) had been prescribed at least one AT (49% single agent), mostly bosentan (66%, 168 patients). The mean time on AT was 6 (SD 3.6) years. Those on AT were more functionally impaired at presentation (69% WHO ≥3 vs 51%, p=0.007) and more likely to have been prescribed anticoagulation (47% vs 27%, p=0.003). The risk of death/transplant was 4.8 %/year in AT exposed versus 8.4% in those never exposed. On multivariable analysis, exposure to AT was independently associated with greater survival (survival HR 2.27, 95% CI 1.49 to 3.45; p<0.001). WHO ≥3 at presentation was associated with a worse prognosis (mortality HR 1.82, 95% CI 1.19 to 2.78; p=0.006).ConclusionTreatment with AT was independently associated with greater survival in patients with ES, even though they were comparatively sicker prior to treatment.


2020 ◽  
Vol 41 (8) ◽  
pp. 1651-1659
Author(s):  
Ida Jeremiasen ◽  
Karin Tran-Lundmark ◽  
Nikmah Idris ◽  
Phan-Kiet Tran ◽  
Shahin Moledina

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.


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