Adult Congenital Heart Disease: Report from a Public Reference Hospital in Northeastern Brazil

Diogenes, Maria Suely Bezerra; Valente, Acrísio Sales; Rocha, Hermano Alexandre Lima

doi:10.21470/1678-9741-2023-0039

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Brazilian Journal of Cardiovascular Surgery

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Sumário

ORIGINAL ARTICLE • Braz. J. Cardiovasc. Surg. 38 (6) • 2023 • https://doi.org/10.21470/1678-9741-2023-0039 copy

Adult Congenital Heart Disease: Report from a Public Reference Hospital in Northeastern Brazil

Authorship SCIMAGO INSTITUTIONS RANKINGS

ABSTRACT

Introduction:

The increasing worldwide number of adults with congenital heart disease (CHD) demands greater attention from health professionals. The purpose of this report is to describe the clinical demographic profile, frequency, and invasive treatment status of adults with CHD in a public reference hospital in northeastern Brazil.

Methods:

This is a retrospective cross-sectional study including 704 patients attended between August 2016 and August 2020. Data were collected from virtual database.

Results:

Patients’ age varied from 17 to 81 years (mean 32±14; median 27 years); 294 (41.8%) patients were male, and 410 (58,2%) were female; 230 (32,7%) had diagnosis from age 18 and up. Cardiac complexity categories were “simple defects” (134 [19%] patients), “moderate complexity” (503 [71.5%]), and “great complexity” (67 [9.5%]). Atrial septal defect (ASD) was diagnosed in 216 (30.7%) patients, ventricular septal defect (VSD) in 101 (14.3%), tetralogy of Fallot in 93 (13.2%), and other CHD in 294 (41.8%). New York Heart Association (NYHA) functional classes were I (401 [57%]), II (203 [28.8%]), III (76 [10.8%]), and IV (24 [3.4%]). Complications were arrhythmias (173 [24%]) and severe pulmonary hypertension (69 [9.8%]). Invasive treatments were corrective surgery (364 (51.6%]), reoperation (28 [4.0%]), palliation (11 [1.6%]), interventional catheterization (12 [1.7%]), surgery plus interventional catheterization (5 [0.7%]), and preoperation (91 [12.9%]). Treatment was not required in 102 (14,5%) patients, and 91 (12.9%) were inoperable.

Conclusion:

The leading diagnosis was ASD. Frequency of unrepaired patients was high, mainly ASD, due to late diagnosis, which favored complications and denotes a matter of great concern.

Keywords:
Congenital Heart Defects; Atrial Septal Defect 5; Tetralogy of Fallot; Cardiac Surgical Procedures; Reoperation

Age range (years)	N (%)	95% CI
		Inferior CL	Superior CL
17 – 20	151 (21.4)	18.8%	24.9%
21 – 30	270 (38.4)	34.7%	41.9%
31 – 40	119 (16.9)	14.2%	19.7%
41 – 50	77 (10.9)	8.8%	13.4%
51 – 60	56 (8.0)	6.1%	10.1%
61 – 70	25 (3.6)	2.4%	5.2%
71 – 80	5 (0.7)	0.3%	1.6%
81 – 90	1 (0.1)	0.0%	0.7%
Total	704 (100)

Age (years)	Mean (SD)	Median
		IQI: 25% 50% 75%
	32 (±14)	21 27 38
	N (%)	95% CI: inferior CL/superior CL
Gender
Female	410 (58.2)	54.6%/61.8%
Male	294 (41.8)	38.2%/45.4%
Total	704 (100)
Origin
Fortaleza and metropolitan area	347 (49.3)	45.7%/53.1%
Countryside	347 (49.3)	45.7%/53.1%
Other states	10 (1.4)	0.7%/2.5%
Age at diagnosis
Under 18 years	474 (67.3)	63.8%/70.7%
18 years and up	230 (32.7)	29.3%/36.2%
Type of CHD
Acyanotic	564 (80.1)	77.0%/82.9%
Cyanotic	140 (19.9)	17.1%/23.0%
Category/complexity
I: simple defects	134 (19.0)	16.3%/22.1%
II: moderate complexity	503 (71.5)	68.8%/74.7%
III: great complexity	67 (9.5)	7.5%/11.9%

	N (%)	95% CI: inferior CL/superior CL
Functional class
I	401 (57.0)	53.3%/60.6%
II	203 (28.8)	25.6%/32.3%
III	76 (10.8)	8.7%/13.2%
IV	24 (3.4)	2.3%/4.9%
Pulmonary hypertension
Absent	589 (83.8)	80.9%/86.4%
Present: mild/moderate	45 (6.4)	4.8%/8.4%
Present: severe	69 (9.8)	7.8%/12.2%
Arrhythmias
Absent	531 (76.0)	72.7%/79%
Present	173 (24.0)
Types of arrhythmia
AF or flutter	55 (7.8)	6.0%/10.0%
Other supraventricular	59 (8.4)	6.5%/10.6%
NSVT	5.0 (0.7)	0.3%/1.5%
Other ventricular	12 (1.7)	0.9%/2.9%
Supraventricular + ventricular	14 (2.0)	1.1%/3.2%
Bradycardia	28 (4.0)	2.7%/5.6%

CHD		N=577/704
	Subtype	N (%)	95% CI: inferior CL/superior CL

ASD	ASD OS	178 (25.2)	22.2%/28.6%
	ASD SV	23 (3.3)	2.1%/4.8%
	ASD OP	15 (2.1)	1.3%/3.4%
VSD		101 (14.3)	11.4%/17.9%
Tetralogy of Fallot		93 (13.2)	10.9%/15.9%
Shunt with PS		35 (5.0)	3.5%/6.8%
PS
	Valvar	23 (3.3)	2.1%/4.8
	Subvalvar	05 (0.7)	0.3%/1.5%
	Supravalvar	03 (0.4)	0.1%/1.1%
Aortic stenosis
	Subvalvar	16 (2.3)	1.4%/3.6%
	Valvar	10 (1.4)	0.7%/2.5%
	Supravalvar	03 (0.4)	0.1%/1.1%
Aortic coarctation		25 (3.6)	2.4%/5.1%
PDA		24 (3.4)	2.3%/4.9%
Ebstein anomaly		23 (3.2)	2.1%/4.8%

Congenital heart disease	N=127/704
	N (%)	95% CI: inferior CL/superior CL
Association of 2 or more shunts	13 (1.8)	1%/3%
ccTGA	11 (1.5)	0.9%/2.6%
CAVC defect	09 (1.3)	0.6%/2.3%
TGA	09 (1.3)	0.6%/2.3%
PA with VSD	09 (1.3)	0.6%/2.3%
DORV	08 (1.1)	0.5%/2.1%
Single ventricle	07 (1.0)	0.4%/1.9%
Bicuspid aortic valve with AR	07 (1.0)	0.4%/1.9%
Tricuspid atresia	06 (0.9)	0.4%/1.7%
Tricuspid valve dysplasia	06 (0.9)	0.4%/1.7%
Coronary anomaly	06 (0.9)	0.4%/1.7%
Coronary and aortic fistulae	06 (0.9)	0.4%/1.7%
PAPVC	03 (0.4)	0.1%/1.1%
Single atrium	02 (0.3)	0.1%/0.9%
TAPVC	02 (0.3)	0.1%/0.9%
Heterotaxy syndrome	01 (0.1)	0.0%/0.7%
Other defects	22 (3.1)	2.0%/4.6%

Treatment (status/types)	N (%)	95% CI: inferior CL/superior CL
Preoperative	91 (12.9)	10.6%/15.6%
Not required	102 (14.5)	12.0%/17.2%
Inoperable	91 (12.9)	10.6%/15.6%
Interventional catheterization	12 (1.7)	1.0%/3.0%
Surgery + interventional catheterization	05 (0.7)	0.3%/1.5%
Corrective surgery	352 (50.0)	46.3%/ 53.7%
Other surgeries			Type of CHD
Fontan	8 (1.1)	0.5%/2.1%	TA, SV
Senning	3 (0.4)	0.1%/1.1%	TGA
Jatene	1 (0.1)	0.0%/0.7%	TGA
Palliative surgery
MBTS	4 (0.6)	0.2%/1.3%	PA + VSD, PS, PA
BH	3 (0.4)	0.1%/1.1%	TGA
PA B	2 (0.3)	0.1%/0.9%	VSD, DORV
Bidirectional Glenn	2 (0.3)	0.1%/0.9%	SV
Reoperation	28 (4.0)	2.7%/5.6%	ToF
Total	704 (100)

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[1] No conflict of interest.