Skip to main content Accessibility help
×
Home
Hostname: page-component-768dbb666b-jrcft Total loading time: 0.408 Render date: 2023-02-06T10:50:47.205Z Has data issue: true Feature Flags: { "useRatesEcommerce": false } hasContentIssue true

Pulmonary vasodilator therapy in tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals: case series and review of literature

Published online by Cambridge University Press:  08 August 2017

Sotiria C. Apostolopoulou*
Affiliation:
Department of Pediatric Cardiology, Onassis Cardiac Surgery Center, Athens, Greece
George Vagenakis
Affiliation:
Department of Pediatric Cardiology, Onassis Cardiac Surgery Center, Athens, Greece
Spyridon Rammos
Affiliation:
Department of Pediatric Cardiology, Onassis Cardiac Surgery Center, Athens, Greece
*
Correspondence to: S. C. Apostolopoulou, MD, PhD, Department of Pediatric Cardiology, Onassis Cardiac Surgery Center, 356 Syngrou Ave, Athens, GR 176 74, Greece. Tel: +30-210-949-3865; Fax: +30-210-949-3853; E-mail: riapos@hol.gr

Abstract

We present the use of pulmonary vasodilators in three adult patients with unrepaired tetralogy of Fallot, pulmonary atresia, aortopulmonary collaterals, and segmental pulmonary arterial hypertension. Patients improved by 1–2 NYHA classes with modest exercise-tolerance increase, and remained stable without side effects during 2.5, 10, and 14 years. Literature review revealed five studies with pulmonary vasodilators in heterogeneous, mostly repaired patient populations.

Type
Brief Report
Copyright
© Cambridge University Press 2017 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Malhotra, SP, Hanley, FL. Surgical management of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a protocol-based approach. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2009; 145151.CrossRefGoogle ScholarPubMed
2. Gatzoulis, MA, Beghetti, M, Galie, N, et al. Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: results of the BREATHE-5 open-label extension study. Int J Cardiol 2008; 127: 2732.CrossRefGoogle ScholarPubMed
3. Grant, EK, Berger, JT. Use of pulmonary hypertension medications in patients with tetralogy of Fallot with pulmonary atresia and multiple aortopulmonary collaterals. Pediatr Cardiol 2016; 37: 304312.CrossRefGoogle ScholarPubMed
4. Lim, ZS, Vettukattill, JJ, Salmon, AP, Veldtman, GR. Sildenafil therapy in complex pulmonary atresia with pulmonary arterial hypertension. Int J Cardiol 2008; 129: 339343.CrossRefGoogle ScholarPubMed
5. Schuuring, MJ, Bouma, BJ, Cordina, R, et al. Treatment of segmental pulmonary artery hypertension in adults with congenital heart disease. Int J Cardiol 2013; 164: 106110.CrossRefGoogle ScholarPubMed
6. Yamamura, K, Nagata, H, Ikeda, K, Ihara, K, Hara, T. Efficacy of bosentan therapy for segmental pulmonary artery hypertension due to major aortopulmonary collateral arteries in children. Int J Cardiol 2012; 161: e1e3.CrossRefGoogle ScholarPubMed
7. Yasuhara, J, Yamagishi, H. Pulmonary arterial hypertension associated with tetralogy of Fallot. Int Heart J 2015; 56 (Suppl): S17S21.CrossRefGoogle ScholarPubMed
8. Shimazaki, Y, Maehara, T, Blackstone, EH, Kirklin, JW, Bargeron, LM Jr.. The structure of the pulmonary circulation in tetralogy of Fallot with pulmonary atresia. A quantitative cineangiographic study. J Thorac Cardiovasc Surg 1988; 95: 10481058.Google ScholarPubMed
9. Bull, K, Somerville, J, Ty, E, Spiegelhalter, D. Presentation and attrition in complex pulmonary atresia. J Am Coll Cardiol 1995; 25: 491499.CrossRefGoogle ScholarPubMed
10. Grady, RM, Gandhi, S, Sweet, SC, Mao, J, Huddleston, CB. Dismal lung transplant outcomes in children with tetralogy of Fallot with pulmonary atresia compared to Eisenmenger syndrome or pulmonary vein stenosis. J Heart Lung Transplant 2009; 28: 12211225.CrossRefGoogle ScholarPubMed
11. Schermuly, RT, Pullamsetti, SS, Kwapiszewska, G, et al. Phosphodiesterase 1 upregulation in pulmonary arterial hypertension: target for reverse-remodeling therapy. Circulation 2007; 115: 23312339.CrossRefGoogle ScholarPubMed
12. Savarese, G, Paolillo, S, Costanzo, P, et al. Do changes of 6-minute walk distance predict clinical events in patients with pulmonary arterial hypertension? A meta-analysis of 22 randomized trials. J Am Coll Cardiol 2012; 60: 11921201.CrossRefGoogle ScholarPubMed
4
Cited by

Save article to Kindle

To save this article to your Kindle, first ensure coreplatform@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Pulmonary vasodilator therapy in tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals: case series and review of literature
Available formats
×

Save article to Dropbox

To save this article to your Dropbox account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you used this feature, you will be asked to authorise Cambridge Core to connect with your Dropbox account. Find out more about saving content to Dropbox.

Pulmonary vasodilator therapy in tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals: case series and review of literature
Available formats
×

Save article to Google Drive

To save this article to your Google Drive account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you used this feature, you will be asked to authorise Cambridge Core to connect with your Google Drive account. Find out more about saving content to Google Drive.

Pulmonary vasodilator therapy in tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals: case series and review of literature
Available formats
×
×

Reply to: Submit a response

Please enter your response.

Your details

Please enter a valid email address.

Conflicting interests

Do you have any conflicting interests? *