TY - JOUR

T1 - Lifelong burden of small unrepaired atrial septal defect

T2 - Results from the Danish National Patient Registry

AU - Udholm, Sebastian

AU - Nyboe, Camilla

AU - Karunanithi, Zarmiga

AU - Christensen, Anne Illemann

AU - Redington, Andrew

AU - Nielsen-Kudsk, Jens Erik

AU - Hjortdal, Vibeke E

N1 - Copyright © 2019 Elsevier B.V. All rights reserved.

PY - 2019

Y1 - 2019

N2 - BACKGROUND: Adult patients with small, unrepaired atrial septal defects (ASD) have higher late mortality than the background population. In this nationwide study, we characterize the late natural history of adults with small, unrepaired ASD.METHODS: Using the Danish National Patient Registry, we included all Danish patients, diagnosed between 1953 and 2011 with an unrepaired ASD. Additionally, all patients, aged 18-65, were invited for clinical testing. Patients also completed a general health survey for comparison with the general population.RESULTS: We identified 723 patients with a small unrepaired ASD. Since the time of diagnosis, 182 patients had died, with an average lifespan of 63 years. The most common cause of death was heart failure. Furthermore, ASD patients had a higher burden of chronic disease than the general population (38.2% vs. 26.9%; p = 0.005), particularly lung disease (3.6% vs. 0.9%; p = 0.008). A total of 153 patients (mean age 32 y) underwent additional testing. On echocardiography an open defect was verified in 19.6% (n = 30) of the patients, of which half subsequently underwent intervention. Interestingly, 6-minute walking distance was markedly reduced (p < 0.0001 compared to normative values) no matter whether the defect was open or closed by echocardiography. Finally, 25.5% of the patients often felt stressed or nervous as compared with 16.3% of the general population (p = 0.004).CONCLUSIONS: Patients with small, unrepaired ASD in adult life have reduced lifespan, more chronic diseases, impaired submaximal exercise capacity, and higher levels of stress than the general population. The current guidelines for intervention and follow-up may need to be reconsidered.

AB - BACKGROUND: Adult patients with small, unrepaired atrial septal defects (ASD) have higher late mortality than the background population. In this nationwide study, we characterize the late natural history of adults with small, unrepaired ASD.METHODS: Using the Danish National Patient Registry, we included all Danish patients, diagnosed between 1953 and 2011 with an unrepaired ASD. Additionally, all patients, aged 18-65, were invited for clinical testing. Patients also completed a general health survey for comparison with the general population.RESULTS: We identified 723 patients with a small unrepaired ASD. Since the time of diagnosis, 182 patients had died, with an average lifespan of 63 years. The most common cause of death was heart failure. Furthermore, ASD patients had a higher burden of chronic disease than the general population (38.2% vs. 26.9%; p = 0.005), particularly lung disease (3.6% vs. 0.9%; p = 0.008). A total of 153 patients (mean age 32 y) underwent additional testing. On echocardiography an open defect was verified in 19.6% (n = 30) of the patients, of which half subsequently underwent intervention. Interestingly, 6-minute walking distance was markedly reduced (p < 0.0001 compared to normative values) no matter whether the defect was open or closed by echocardiography. Finally, 25.5% of the patients often felt stressed or nervous as compared with 16.3% of the general population (p = 0.004).CONCLUSIONS: Patients with small, unrepaired ASD in adult life have reduced lifespan, more chronic diseases, impaired submaximal exercise capacity, and higher levels of stress than the general population. The current guidelines for intervention and follow-up may need to be reconsidered.

KW - Adolescent

KW - Adult

KW - Aged

KW - Atrial Septum/diagnostic imaging

KW - Denmark/epidemiology

KW - Echocardiography

KW - Exercise Test

KW - Female

KW - Follow-Up Studies

KW - Heart Septal Defects, Atrial/diagnosis

KW - Heart Ventricles/diagnostic imaging

KW - Humans

KW - Male

KW - Middle Aged

KW - Morbidity/trends

KW - Prognosis

KW - Registries

KW - Retrospective Studies

KW - Survival Rate/trends

KW - Ventricular Function, Right/physiology

KW - Young Adult

U2 - 10.1016/j.ijcard.2019.02.024

DO - 10.1016/j.ijcard.2019.02.024

M3 - Journal article

C2 - 30826194

VL - 283

SP - 101

EP - 106

JO - International Journal of Cardiology

JF - International Journal of Cardiology

SN - 0167-5273

ER -