Prevalence of Cardiac Dysfunction Among Adult Patients With Congenital Heart Disease: A Single-Center Investigation

Document Type: Original Article

Authors

1 Rajaie Cardiovascular, Medical, and Research Center, Iran University of Medical Sciences, Tehran, IR Iran.

2 Health Science Research Center, Addiction Institute, Mazandaran University of Medical Sciences, Sari, Iran, IR Iran.

Abstract

Background: In spite of achievements in the field of pediatric cardiology and surgical techniques, which have increased the chance of children with congenital heart diseases to reach adulthood, the inherent problems with the disease create a large number of complications for them in later life, including cardiac dysfunction. It is important to know the prevalence of cardiac dysfunction and its influential factor among adults with congenital heart diseases (CHDs); hence, the present study aimed to answer this question.
Methods: We measured the prevalence of cardiac dysfunction based on echocardiographic guidelines among ACHDS referred to Rajaie Cardiovascular, Medical, and Research Center between December 2017 and June 2018. Data analysis was performed using the χ2 test and logistic regression through the SPSS software.
Results: Left and right ventricular dysfunction was 60.6% and 77.7%, respectively. Moreover, 58.7% of the patients were affected by both left and right ventricular dysfunction, while 20.9% had only one of the left or right ventricular dysfunction. Eighty-eight (20.4%) patients did not have dysfunction at the time of the study. The variables of moderately complex congenital heart disease, cyanosis, moderate pulmonary hypertension, the Eisenmenger syndrome, and the type of intervention were the predictors of left ventricular dysfunction.
Conclusions: The prevalence of cardiac dysfunction among our adult patients with CHDs was very high. Given that cardiac dysfunction starts at a young age in this group of patients in comparison with the general population, the quality of life of the former group is more seriously threatened. Our results identified factors that increased the likelihood of developing cardiac dysfunction. These factors should be considered when approaching patients with cardiac dysfunction.

Keywords


1. Fahed AC, Roberts AE, Mital S, Lakdawala
NK. Heart failure in congenital heart disease: a
confluence of acquired and congenital. Heart
failure clinics. 2014 Jan 1;10(1):219-27.
2. Webb CL, Jenkins KJ, Karpawich PP, Bolger
AF, Donner RM, Allen HD, Barst RJ,
Congenital Cardiac Defects Committee of the
American Heart Association Section on
Cardiovascular Disease in the Young.
Collaborative care for adults with congenital
heart disease. Circulation. 2002 May
14;105(19):2318-23.
3. Hoffman JI, Kaplan S. The incidence of
congenital heart disease. Journal of the
American college of cardiology. 2002 Jun
19;39(12):1890-900.
4. Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme
E, Pilote L. Congenital heart disease in the
general population: changing prevalence and
age distribution. Circulation. 2007 Jan
16;115(2):163-72.
5. van der Linde D, Konings EE, Slager MA,
Witsenburg M, Helbing WA, Takkenberg JJ,
Roos-Hesselink JW. Birth prevalence of
congenital heart disease worldwide: a systematic
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American College of Cardiology. 2011 Nov
15;58(21):2241-7.
6. Van Der Bom T, Zomer AC, Zwinderman AH,
Meijboom FJ, Bouma BJ, Mulder BJ. The
changing epidemiology of congenital heart
disease. Nature Reviews Cardiology. 2011
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7. Khairy P, Ionescu-Ittu R, Mackie AS,
Abrahamowicz M, Pilote L, Marelli AJ.
Changing mortality in congenital heart disease.
Journal of the American College of Cardiology.
2010 Sep 28;56(14):1149-57.
8. Wu MH, Lu CW, Chen HC, Kao FY, Huang
SK. Adult Congenital Heart Disease in a
Nationwide Population 2000–2014:
Epidemiological Trends, Arrhythmia, and
Standardized Mortality Ratio. Journal of the
American Heart Association. 2018 Feb 8;7(4):
e007907.
9. Stout KK, Broberg CS, Book WM, Cecchin F,
Chen JM, Dimopoulos K, Everitt MD, Gatzoulis
M, Harris L, Hsu DT, Kuvin JT. Chronic heart
failure in congenital heart disease: a scientific
statement from the American Heart Association.
Circulation. 2016 Feb 23;133(8):770-801.
10. Oechslin EN, Harrison DA, Connelly MS,
Webb GD, Siu SC. Mode of death in adults with
congenital heart disease. The American journal
of cardiology. 2000 Nov 15;86(10):1111-6.
11. Diller GP, Kempny A, Alonso-Gonzalez R,
Swan L, Uebing A, Li W, Babu-Narayan S,
Wort SJ, Dimopoulos K, Gatzoulis MA.
Survival prospects and circumstances of death in
contemporary adult congenital heart disease
patients under follow-up at a large tertiary
centre. Circulation. 2015 Dec 1;132(22):2118-
25.
12. Stout KK, Broberg CS, Book WM, Cecchin F,
Chen JM, Dimopoulos K, Everitt MD, Gatzoulis
M, Harris L, Hsu DT, Kuvin JT. Chronic heart
failure in congenital heart disease: a scientific
statement from the American Heart Association.
Circulation. 2016 Feb 23;133(8):770-801.
13. Norozi K, Wessel A, Alpers V, Arnhold JO,
Geyer S, Zoege M, Buchhorn R. Incidence and
risk distribution of heart failure in adolescents
and adults with congenital heart disease after
cardiac surgery. The American journal of
cardiology. 2006 Apr 15;97(8):1238-43.
14. Piran S, Veldtman G, Siu S, Webb GD, Liu PP.
Heart failure and ventricular dysfunction in
patients with single or systemic right ventricles.
Circulation. 2002 Mar 12;105(10):1189-94.
15. Stefanescu A, Yeh DD, Dudzinski DM. Heart
failure in adult congenital heart disease. Current
treatment options in cardiovascular medicine.
2014 Sep 1;16(9):337.
16. Rodriguez FH, Marelli AJ. The epidemiology of
heart failure in adults with congenital heart
disease. Heart failure clinics. 2014 Jan
1;10(1):1-7.
17. Bolger AP, Coats AJ, Gatzoulis MA. Congenital
heart disease: the original heart failure
syndrome. European Heart Journal. 2003 May
1;24(10):970-6.
18. Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme
E, Pilote L. Congenital heart disease in the
general population: changing prevalence and
age distribution. Circulation. 2007 Jan
16;115(2):163-72.
19. Budts W, Roos-Hesselink J, Rädle-Hurst T,
Eicken A, McDonagh TA, Lambrinou E,
Crespo-Leiro MG, Walker F, Frogoudaki AA.
Treatment of heart failure in adult congenital
heart disease: a position paper of the Working
Group of Grown-Up Congenital Heart Disease
and the Heart Failure Association of the
European Society of Cardiology. European heart
journal. 2016 Jan 18;37(18):1419-27.

. Fahed AC, Roberts AE, Mital S, Lakdawala
NK. Heart failure in congenital heart disease: a
confluence of acquired and congenital. Heart
failure clinics. 2014 Jan 1;10(1):219-27.
2. Webb CL, Jenkins KJ, Karpawich PP, Bolger
AF, Donner RM, Allen HD, Barst RJ,
Congenital Cardiac Defects Committee of the
American Heart Association Section on
Cardiovascular Disease in the Young.
Collaborative care for adults with congenital
heart disease. Circulation. 2002 May
14;105(19):2318-23.
3. Hoffman JI, Kaplan S. The incidence of
congenital heart disease. Journal of the
American college of cardiology. 2002 Jun
19;39(12):1890-900.
4. Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme
E, Pilote L. Congenital heart disease in the
general population: changing prevalence and
age distribution. Circulation. 2007 Jan
16;115(2):163-72.
5. van der Linde D, Konings EE, Slager MA,
Witsenburg M, Helbing WA, Takkenberg JJ,
Roos-Hesselink JW. Birth prevalence of
congenital heart disease worldwide: a systematic
review and meta-analysis. Journal of the
American College of Cardiology. 2011 Nov
15;58(21):2241-7.
6. Van Der Bom T, Zomer AC, Zwinderman AH,
Meijboom FJ, Bouma BJ, Mulder BJ. The
changing epidemiology of congenital heart
disease. Nature Reviews Cardiology. 2011
Jan;8(1):50.
7. Khairy P, Ionescu-Ittu R, Mackie AS,
Abrahamowicz M, Pilote L, Marelli AJ.
Changing mortality in congenital heart disease.
Journal of the American College of Cardiology.
2010 Sep 28;56(14):1149-57.
8. Wu MH, Lu CW, Chen HC, Kao FY, Huang
SK. Adult Congenital Heart Disease in a
Nationwide Population 2000–2014:
Epidemiological Trends, Arrhythmia, and
Standardized Mortality Ratio. Journal of the
American Heart Association. 2018 Feb 8;7(4):
e007907.
9. Stout KK, Broberg CS, Book WM, Cecchin F,
Chen JM, Dimopoulos K, Everitt MD, Gatzoulis
M, Harris L, Hsu DT, Kuvin JT. Chronic heart
failure in congenital heart disease: a scientific
statement from the American Heart Association.
Circulation. 2016 Feb 23;133(8):770-801.
10. Oechslin EN, Harrison DA, Connelly MS,
Webb GD, Siu SC. Mode of death in adults with
congenital heart disease. The American journal
of cardiology. 2000 Nov 15;86(10):1111-6.
11. Diller GP, Kempny A, Alonso-Gonzalez R,
Swan L, Uebing A, Li W, Babu-Narayan S,
Wort SJ, Dimopoulos K, Gatzoulis MA.
Survival prospects and circumstances of death in
contemporary adult congenital heart disease
patients under follow-up at a large tertiary
centre. Circulation. 2015 Dec 1;132(22):2118-
25.
12. Stout KK, Broberg CS, Book WM, Cecchin F,
Chen JM, Dimopoulos K, Everitt MD, Gatzoulis
M, Harris L, Hsu DT, Kuvin JT. Chronic heart
failure in congenital heart disease: a scientific
statement from the American Heart Association.
Circulation. 2016 Feb 23;133(8):770-801.
13. Norozi K, Wessel A, Alpers V, Arnhold JO,
Geyer S, Zoege M, Buchhorn R. Incidence and
risk distribution of heart failure in adolescents
and adults with congenital heart disease after
cardiac surgery. The American journal of
cardiology. 2006 Apr 15;97(8):1238-43.
14. Piran S, Veldtman G, Siu S, Webb GD, Liu PP.
Heart failure and ventricular dysfunction in
patients with single or systemic right ventricles.
Circulation. 2002 Mar 12;105(10):1189-94.
Iranian Heart Journal; 2019; 20 (3)
Prevalence of Cardiac Dysfunction Among Adult Congenital Heart Disease Patients: A Single-Ccenter Iinvestigation Khajali et al
19
15. Stefanescu A, Yeh DD, Dudzinski DM. Heart
failure in adult congenital heart disease. Current
treatment options in cardiovascular medicine.
2014 Sep 1;16(9):337.
16. Rodriguez FH, Marelli AJ. The epidemiology of
heart failure in adults with congenital heart
disease. Heart failure clinics. 2014 Jan
1;10(1):1-7.
17. Bolger AP, Coats AJ, Gatzoulis MA. Congenital
heart disease: the original heart failure
syndrome. European Heart Journal. 2003 May
1;24(10):970-6.
18. Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme
E, Pilote L. Congenital heart disease in the
general population: changing prevalence and
age distribution. Circulation. 2007 Jan
16;115(2):163-72.
19. Budts W, Roos-Hesselink J, Rädle-Hurst T,
Eicken A, McDonagh TA, Lambrinou E,
Crespo-Leiro MG, Walker F, Frogoudaki AA.
Treatment of heart failure in adult congenital
heart disease: a position paper of the Working
Group of Grown-Up Congenital Heart Disease
and the Heart Failure Association of the
European Society of Cardiology. European heart
journal. 2016 Jan 18;37(18):1419-27.