Abnormal lung function in adults with congenital heart disease: prevalence, relation to cardiac anatomy, and association with survival

Alonso-Gonzalez, Rafael; Borgia, Francesco; Diller, Gerhard-Paul; Inuzuka, Ryo; Kempny, Aleksander; Martinez-Naharro, Ana; Tutarel, Oktay; Marino, Philip; Wustmann, Kerstin; Charalambides, Menelaos; Silva, Margarida; Swan, Lorna; Dimopoulos, Konstantinos; Gatzoulis, Michael A. (2013). Abnormal lung function in adults with congenital heart disease: prevalence, relation to cardiac anatomy, and association with survival. Circulation, 127(8), pp. 882-890. Lippincott Williams & Wilkins 10.1161/CIRCULATIONAHA.112.126755

[img] Text
Circ2013 full.pdf - Published Version
Restricted to registered users only
Available under License Publisher holds Copyright.

Download (717kB) | Request a copy

BACKGROUND: Restrictive lung defects are associated with higher mortality in patients with acquired chronic heart failure. We investigated the prevalence of abnormal lung function, its relation to severity of underlying cardiac defect, its surgical history, and its impact on outcome across the spectrum of adult congenital heart disease. METHODS AND RESULTS: A total of 1188 patients with adult congenital heart disease (age, 33.1+/-13.1 years) undergoing lung function testing between 2000 and 2009 were included. Patients were classified according to the severity of lung dysfunction based on predicted values of forced vital capacity. Lung function was normal in 53% of patients with adult congenital heart disease, mildly impaired in 17%, and moderately to severely impaired in the remainder (30%). Moderate to severe impairment of lung function related to complexity of underlying cardiac defect, enlarged cardiothoracic ratio, previous thoracotomy/ies, body mass index, scoliosis, and diaphragm palsy. Over a median follow-up period of 6.7 years, 106 patients died. Moderate to severe impairment of lung function was an independent predictor of survival in this cohort. Patients with reduced force vital capacity of at least moderate severity had a 1.6-fold increased risk of death compared with patients with normal lung function (P=0.04). CONCLUSIONS: A reduced forced vital capacity is prevalent in patients with adult congenital heart disease; its severity relates to the complexity of the underlying heart defect, surgical history, and scoliosis. Moderate to severe impairment of lung function is an independent predictor of mortality in contemporary patients with adult congenital heart disease.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Cardiovascular Disorders (DHGE) > Clinic of Cardiology

UniBE Contributor:

Wustmann, Kerstin Brigitte

Subjects:

600 Technology > 610 Medicine & health

ISSN:

0009-7322

Publisher:

Lippincott Williams & Wilkins

Funders:

[UNSPECIFIED] British Heart Foundation

Language:

English

Submitter:

Kerstin Brigitte Wustmann

Date Deposited:

27 Feb 2014 13:23

Last Modified:

11 Sep 2017 21:00

Publisher DOI:

10.1161/CIRCULATIONAHA.112.126755

BORIS DOI:

10.7892/boris.42039

URI:

https://boris.unibe.ch/id/eprint/42039

Actions (login required)

Edit item Edit item
Provide Feedback