Sildenafil therapy in complex pulmonary atresia with pulmonary arterial hypertension

Lim, Zek S.; Vettukattill, Joseph; Salmon, Anthony P.; Veldtman, Gruschen

doi:10.1016/j.ijcard.2007.08.016

Cited by 23 publications

(20 citation statements)

References 16 publications

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“…Hopefully, the continued refinement of both surgical and catheter interventions, plus the use of newer pulmonary specific vasodilators, will further forestall the consideration for lung transplantation in these problematic patients. 4,5 The underlying pathology in EIS patients is typically a large, unrestrictive ventricular septal defect that exposes the pulmonary vasculature to excessive blood flow and pressures, eventually leading to irreversible pulmonary hypertension. 6,7 Although no pediatric data are available for comparison, our overall survival of EIS patients undergoing lung transplantation is similar to that for adults, with our longest survivor being 13 years post-transplant.…”

Section: Discussionmentioning

confidence: 99%

Dismal Lung Transplant Outcomes in Children With Tetralogy of Fallot With Pulmonary Atresia Compared to Eisenmenger Syndrome or Pulmonary Vein Stenosis

Grady

Gandhi

Sweet

et al. 2009

The Journal of Heart and Lung Transplantation

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Section: Discussionmentioning

confidence: 99%

Dismal Lung Transplant Outcomes in Children With Tetralogy of Fallot With Pulmonary Atresia Compared to Eisenmenger Syndrome or Pulmonary Vein Stenosis

Grady

Gandhi

Sweet

et al. 2009

The Journal of Heart and Lung Transplantation

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“…A multicenter study by Schuuring et al on 7 patients with segmental PH caused by pulmonary atresia reported a significant improvement in functional class and exercise tolerance (6‐minute walk test distance improved by 62 m) with the endothelin‐receptor antagonist bosentan 3. Another observational study by Lim et al on 5 adult patients with complex pulmonary atresia or severe pulmonary stenosis and MAPCAs treated with the phosphodiesterase‐type 5 inhibitor sildenafil reported this therapy to be well tolerated in 4 of 5 patients, with a good clinical response to treatment in those patients 38. Yamamura et al presented 2 children with segmental PH after pulmonary atresia treated with bosentan, with an improvement in symptoms, hemodynamics, and brain natriuretic peptide concentration 39.…”

Section: Clinical Management Of Segmental Ph: Surgical Interventionamentioning

confidence: 98%

“…While some evidence suggests this approach may be promising, there have been cases where therapies were not tolerated 38, 39, 42. An increase in pulmonary blood flow may, theoretically, overload the left ventricle and this should be taken into account when considering PAH therapies in patients with established left ventricular dysfunction and/or aortic stenosis or regurgitation.…”

Section: Clinical Management Of Segmental Ph: Surgical Interventionamentioning

confidence: 99%

Definition and Management of Segmental Pulmonary Hypertension

Dimopoulos

Diller

Opotowsky

et al. 2018

JAHA

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“…22) Another paper showed that sildenafil was well tolerated and improved symptoms and SpO 2 in the majority of patients with PAH associated with TOF and MAPCAs when it was used alone or as an adjunct to percutaneous pulmonary angiopla sty. 23) We sent a questionnaire to pediatric cardiologists who were treating patients with PAH-TOF at 10 Japanese institutions to obtain their impressions of the efficacy of oral pulmonary vasodilators in these patients (unpublished data). The 10 cardiologists reported on 40 patients (age range 1-36 years) who were treated with oral pulmonary vasodilators, including 27 patients had TOF and MAPCAs and 13 cases with TOF without MAPCAs.…”

Section: Discussionmentioning

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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Sildenafil therapy in complex pulmonary atresia with pulmonary arterial hypertension

Cited by 23 publications

References 16 publications

Dismal Lung Transplant Outcomes in Children With Tetralogy of Fallot With Pulmonary Atresia Compared to Eisenmenger Syndrome or Pulmonary Vein Stenosis

Dismal Lung Transplant Outcomes in Children With Tetralogy of Fallot With Pulmonary Atresia Compared to Eisenmenger Syndrome or Pulmonary Vein Stenosis

Definition and Management of Segmental Pulmonary Hypertension

Pulmonary Arterial Hypertension Associated With Tetralogy of Fallot

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