Hostname: page-component-8448b6f56d-m8qmq Total loading time: 0 Render date: 2024-04-16T08:21:24.323Z Has data issue: false hasContentIssue false

Idiopathic pulmonary arterial hypertension with coexisting CHD

Published online by Cambridge University Press:  10 January 2018

Thomas G. Day*
Affiliation:
Department of Cardiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK Department of Adult Congenital Cardiology, Bart’s Heart Centre, Bart’s Health NHS Trust, London, UK
Marina Hughes
Affiliation:
Department of Cardiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK
Bejal Pandya
Affiliation:
Department of Adult Congenital Cardiology, Bart’s Heart Centre, Bart’s Health NHS Trust, London, UK
*
Author for correspondence: Thomas G. Day, Department of Cardiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom. Tel: 07944326254; E-mail: tomgeorgeday@gmail.com

Abstract

Sinus venosus atrial septal defect can result in an increase in pulmonary blood flow and vascular resistance, leading to pulmonary hypertension. Rarely, the degree of pulmonary hypertension is out of proportion to the degree of intra-cardiac shunting. This case outlines the differences between pulmonary hypertension secondary to CHD and idiopathic pulmonary hypertension, and illustrates the investigation and management strategy used in a patient with features of both.

Type
Brief Report
Copyright
© Cambridge University Press 2018 

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.)

Footnotes

Current address: Department of Cardiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom.

References

1. Jost, CHA, Connolly, HM, Danielson, GK, et al. Sinus venosus atrial septal defect: long-term postoperative outcome for 115 patients. Circulation. 2005; 112: 19531958.CrossRefGoogle Scholar
2. Van Praagh, S, Carrera, ME, Sanders, SP, Mayer, JE, Van Praagh, R. Sinus venosus defects: unroofing of the right pulmonary veins – anatomic and echocardiographic findings and surgical treatment. Am Heart J 1994; 128: 365379.CrossRefGoogle Scholar
3. Pascoe, RD, Oh, JK, Warnes, CA, Danielson, GK, Tajik, AJ, Seward, JB. Diagnosis of sinus venosus atrial septal defect with transesophageal echocardiography. Circulation. 1996; 94: 10491055.CrossRefGoogle ScholarPubMed
4. Vogel, M, Berger, F, Kramer, A, Alexi-Meshkishvili, V, Lange, PE. Incidence of secondary pulmonary hypertension in adults with atrial septal or sinus venosus defects. Heart. 1999; 82: 3033.CrossRefGoogle ScholarPubMed
5. Dimopoulos, K, Wort, SJ, Gatzoulis, MA. Pulmonary hypertension related to congenital heart disease: a call for action. Eur Heart J 2014; 35: 691700.CrossRefGoogle ScholarPubMed
6. Diller, GP, Gatzoulis, MA. Pulmonary vascular disease in adults with congenital heart disease. Circulation. 2007; 115: 10391050.CrossRefGoogle ScholarPubMed
7. D’Alto, M, Diller, G-P. Pulmonary hypertension in adults with congenital heart disease and Eisenmenger syndrome: current advanced management strategies. Heart. 2014; 100: 17 https://doi.org/10.1136/heartjnl-2014-305574.Google ScholarPubMed
8. Cherian, G, Uthaman, CB, Durairaj, M, et al. Pulmonary hypertension in isolated secundum atrial septal defect: high frequency in young patients. Am Heart J 1983; 105: 952957.CrossRefGoogle ScholarPubMed
9. Wood, P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J 1958; 2: 755762.CrossRefGoogle ScholarPubMed