Long-Term Prostacyclin for Pulmonary Hypertension With Associated Congenital Heart Defects

Rosenzweig, Erika B.; Kerstein, Diane; Barst, Robyn J.

doi:10.1161/01.cir.99.14.1858

Cited by 454 publications

(244 citation statements)

References 15 publications

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“…The uptake of this form of therapy, however, was slow in CHD patients due to the short half life of the substances, demanding continuous intravenous infusion. Improvement of functional capacity, oxygen saturation and pulmonary hemodynamics have been reported in this cohort [48,49]. Intravenous prostacyclin administration, however, is technically challenging and prolonged intravenous therapy is associated with frequent complications such as sepsis and line dislocation [48].…”

Section: Prostacyclinsmentioning

confidence: 55%

The challenge of managing pulmonary arterial hypertension in adults with congenital heart disease

Radke

Diller

Baumgartner

2013

Expert Review of Cardiovascular Therapy

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Section: Prostacyclinsmentioning

confidence: 55%

The challenge of managing pulmonary arterial hypertension in adults with congenital heart disease

Radke

Diller

Baumgartner

2013

Expert Review of Cardiovascular Therapy

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“…Long-term intravenous prostacyclin improved hemodynamic and functional class in 20 patients with PAH associated with a range of CHD, although none of the patients had an acute hemodynamic response. 62 Continuous intravenous epoprostenol significantly improved functional class, arterial saturation and 6-minute walk distance, and decreased PVR in eight patients with ES after three months of therapy. 63 However, in an earlier series, treatment of patients with epoprostenol resulted in adverse events including increase in systemic vascular resistance, increased PVR and low arterial oxygen in eight out of 10 patients.…”

Section: Prostacyclin and Prostacyclin Analoguesmentioning

confidence: 82%

Pulmonary Hypertension in Congenital Shunts

Beghetti¹,

Tissot

2010

Revista Española de Cardiología (English Edition)

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“…For example, 1 year of prostacyclin therapy improved the hemodynamics and quality of life of 20 children with PAH-CHD (mean age 15 years) in an uncontrolled study. 4) Phosphodiesterase type-5 (PDE-5) is the enzyme responsible for the hydrolysis of cyclic guanosine monophosphate (cGMP), which is enriched in lungs where it enhances vasodilation of pulmonary arteries. PDE-5 inhibitors, such as sildena-S18 Int Heart J 2015…”

Section: )mentioning

confidence: 99%

Pulmonary Arterial Hypertension Associated With Tetralogy of Fallot

Yasuhara

Yamagishi

2015

Int. Heart J.

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SummaryPulmonary arterial hypertension (PAH) is a common postoperative complication in patients with congenital heart disease (CHD). Although the recent clinical classification of pulmonary hypertension divided PAH associated with CHD (PAH-CHD) into several subclasses, the anatomical and hemodynamic features of postoperative PAH-CHD vary enormously. Therefore, it is still difficult to obtain clinical evidence supporting the indication of pulmonary vasodilators for PAH-CHD. We often encounter patients with PAH occurring after surgical treatment of tetralogy of Fallot (TOF), especially patients with major aortopulmonary collateral arteries (MAPCAs). PAH might be caused by pulmonary agenesis, hypoplasia and/or thrombosis, inadequate closure of the ventricular septal defect, relief of the pulmonic stenosis, or an excessively large prior systemic-to-pulmonary shunt. Moreover, patients with TOF and MAPCAs who are diagnosed as inoperable because of the presence of PAH show similar hemodynamic and clinical features to patients with Eisenmenger syndrome. The MAPCAs in these patients usually show hypoplastic and abnormal arborization. Based on our experience, we believe that PAH-targeted therapies are effective in some patients with PAH occurring after surgical treatment of TOF and MAPCAs, especially as an adjunct to percutaneous pulmonary angioplasty. To help classify patients with PAH associated with TOF, especially with MAPCAs, we propose several new subclassifications: "PAH due to hypoplastic pulmonary arterial beds", "PAH due to abnormal pulmonary arborization", or "segmental PAH associated with CHD." A multicenter registry of patients using a unified protocol is essential to explore the indications and efficacy of pulmonary vasodilators for postoperative PAH-CHD. (Int Heart J 2015; 56: S17-S21) Key words: Major aortopulmonary collateral artery, Pulmonary vasodilator, Percutaneous pulmonary angioplasty, Pulmonary arterial bed P ulmonary arterial hypertension (PAH) is a frequent complication in patients with congenital heart disease (CHD) and has significant effects on their prognosis. In patients with systemic-to-pulmonary shunts, prolonged increases in blood flow and pressure in the pulmonary arteries promotes vascular remodeling and dysfunction, leading to increased pulmonary vascular resistance. Further progression of this condition ultimately results in shunt reversal and the development of Eisenmenger syndrome, one of the most advanced types of PAH associated with CHD (PAH-CHD), as discussed in another chapter of this issue.In recent years, the prevalence of PAH-CHD has decreased in developed countries and the number of patients surviving into adulthood has increased. The timing of corrective surgery of CHD is critical to avoid pulmonary vascular remodeling and PAH.1) Because the majority of patients with a systemic-to-pulmonary shunt undergo heart surgery at an appropriate time, the prevalence of Eisenmenger syndrome has decreased substantially in developed countries. However, 4-15% of patients with CHD still d...

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Long-Term Prostacyclin for Pulmonary Hypertension With Associated Congenital Heart Defects

Cited by 454 publications

References 15 publications

The challenge of managing pulmonary arterial hypertension in adults with congenital heart disease

The challenge of managing pulmonary arterial hypertension in adults with congenital heart disease

Pulmonary Hypertension in Congenital Shunts

Pulmonary Arterial Hypertension Associated With Tetralogy of Fallot

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