Effects of Pulmonary Vasodilator Therapy on Ventilatory Efficiency during Exercise in Adults with Eisenmenger Syndrome

2011 ◽  
Vol 6 (2) ◽  
pp. 139-146 ◽  
Author(s):  
Showshan Yang-Ting ◽  
Jamil Aboulhosn ◽  
Xing-Guo Sun ◽  
John S. Child ◽  
Kathy E. Sietsema
Keyword(s):  
Eisenmenger Syndrome ◽  
Vasodilator Therapy ◽  
Ventilatory Efficiency ◽  
Pulmonary Vasodilator

Pulmonary vasodilator therapy is associated with greater survival in Eisenmenger syndrome

Heart ◽  
2017 ◽  
Vol 104 (9) ◽  
pp. 732-737 ◽  
Author(s):  
Clare Arnott ◽  
Geoff Strange ◽  
Andrew Bullock ◽  
Adrienne C Kirby ◽  
Clare O’Donnell ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Adult Congenital Heart Disease ◽  
Single Agent ◽  
Eisenmenger Syndrome ◽  
Vasodilator Therapy ◽  
Risk Of Death ◽  
Complex Anatomy ◽  
Pulmonary Vasodilator

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.

scholarly journals Selective Pulmonary Vasodilator Therapy and Improved Survival in Eisenmenger Syndrome: The ANZ CHD-PAH Registry

2017 ◽  
Vol 26 ◽  
pp. S326
Author(s):  
C. Arnott ◽  
G. Strange ◽  
C. O’Donnell ◽  
A. Bullock ◽  
D. Radford ◽  
...  
Keyword(s):  
Eisenmenger Syndrome ◽  
Vasodilator Therapy ◽  
Pulmonary Vasodilator

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 Thiamine-Responsive Acute Pulmonary Hypertension of Early Infancy (TRAPHEI)—A Case Series and Clinical Review

Children ◽  
2020 ◽  
Vol 7 (11) ◽  
pp. 199
Author(s):  
Nalinikanta Panigrahy ◽  
Dinesh Kumar Chirla ◽  
Rakshay Shetty ◽  
Farhan A. R. Shaikh ◽  
Poddutoor Preetham Kumar ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Case Reports ◽  
Case Series ◽  
Early Infancy ◽  
Right Atrial ◽  
Voice Leading ◽  
Vasodilator Therapy ◽  
Polished Rice ◽  
Pulmonary Vasodilator ◽  
Acute Pulmonary Hypertension

Persistent pulmonary hypertension of the newborn (PPHN) is a syndrome of high pulmonary vascular resistance (PVR) commonly seen all over the world in the immediate newborn period. Several case reports from India have recently described severe pulmonary hypertension among infants in the postneonatal period. These cases typically present with respiratory distress in 1–6-month-old infants, breastfed by mothers on a polished rice-based diet. Predisposing factors include respiratory tract infection such as acute laryngotracheobronchitis with change in voice, leading to pulmonary hypertension, right atrial and ventricular dilation, pulmonary edema and hepatomegaly. Mortality is high without specific therapy. Respiratory support, pulmonary vasodilator therapy, inotropes, diuretics and thiamine infusion have improved the outcome of these infants. This review outlines four typical patients with thiamine-responsive acute pulmonary hypertension of early infancy (TRAPHEI) due to thiamine deficiency and discusses pathophysiology, clinical features, diagnostic criteria and therapeutic options.

scholarly journals Successful Treatment of Idiopathic Pulmonary Vasculopathy With Targeted Pulmonary Vasodilator Therapy

CHEST Journal ◽  
2016 ◽  
Vol 150 (4) ◽  
pp. 1141A
Author(s):  
Mariam Anis ◽  
Kathryn Wikenheiser-Brokamp ◽  
Jean Elwing
Keyword(s):  
Successful Treatment ◽  
Vasodilator Therapy ◽  
Pulmonary Vasodilator
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