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

Maria Suely Bezerra DiogenesI; Acrisio Sales ValenteI; Hermano Alexandre Lima RochaI

DOI: 10.21470/1678-9741-2023-0039


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.


AF = Atrial fibrillation

AR = Aortic regurgitation

ASD = Atrial septal defect

BH = Blalock-Hanlon

CAVC = Complete atrioventricular canal

ccTGA = Congenitally corrected transposition of the great arteries

CHD = Congenital heart disease

CI = Confidence interval

CL = Confidence limit

DORV = Double outlet right ventricle

ECG = Electrocardiogram

FC = Functional class

IL = Inferior limit

IQI = Interquartile interval

MBTS = Modified Blalock-Taussig shunt

NSVT = Non-sustained ventricular tachycardia

NYHA = New York Heart Association

OP = Ostium primum

OS = Ostium secundum

PA = Pulmonary atresia

PAB = Pulmonary artery banding

PAH = Pulmonary arterial hypertension

PAPVC = Partial anomalous pulmonary venous connection

PDA = Patent ductus arteriosus

PS = Pulmonary stenosis

SD = Standard deviation

SL = Superior limit

SV = Sinus venosus

TAPVC = Total anomalous pulmonary venous connection

TGA = Transposition of the great arteries

VSD = Ventricular septal defect


The growing worldwide adult population with congenital heart disease (CHD) is a consequence of the survival of children with heart defects successfully treated, especially the ones with complex defects, as the cyanotic group. This outcome is a result of the advance in new diagnostic methods and invasive treatment, particularly surgery[1,2,3,4,5,6,7]. Although more than 90% of children with CHD reach adulthood, the exact prevalence of the adult population is unknown[8,9]. This expansion of adult congenital cardiacs, already foreseen decades ago, requires better profile evaluation and action from health professionals once there can be clinical and hemodynamic deterioration of patients left with significant residual lesions of previous invasive treatment[10,11,12].

In Brazil, publication is scarce, so little is known about the profile and frequency of adults with CHD[13,14,15,16,17]. It is possible that many patients repaired in childhood are not receiving any specialized assistance and many others did not undergo invasive treatment. Consequently, they may be evolving with complications like arrhythmias, heart failure, and pulmonary hypertension. Some patients could even be in inoperable situation resulting in increased morbimortality and gradually transforming this issue into a public health problem.

Since the clinical demographic profile, invasive treatment status, and frequency of adults with CHD are unknown in northeastern Brazil, the aim of the present paper is to outline these features in a cohort attended in a public reference facility.


An observational cross-sectional study with retrospective cohort was performed comprising 704 Brazilians with CHD, age ranging from 17 to 81 years, attended at the outpatient clinic of a reference public hospital in the cardiopulmonary field, in the city of Fortaleza (Ceará, Northeast Brazil). Patients’ clinical demographic information was obtained from a virtual database registered in a Microsoft Excel Program document collected during medical consultations in the period between August 2016 and August 2020 by the authors of the present paper. Physical medical records were reviewed whenever necessary. Patients were referred from pediatric cardiology and adult cardiology outpatient clinics of the hospital where the research was undertaken. Inclusion criteria were: patients born in Brazil, aged 17 years and up with CHD. Although most of the patients were adults, 17-year-old teenagers were also included since this age is considered a borderline between teenage and adulthood. According to Brazilian law, adulthood begins at 18 years[18]. Exclusion criteria were: foreigners, teenagers under 17 years of age, patients with concomitant Marfan Syndrome, and those with both CHD and rheumatic heart disease.

All patients had definite diagnosis, complemented by electrocardiogram (ECG), chest X-ray in the posterior-anterior view, and bidimensional echocardiogram with color flow Doppler, and other exams according with their needs.

The following variables were analysed: age; gender; origin (Fortaleza and metropolitan area, countryside, other states in the Northeast); diagnosis of CHD; age at diagnosis (under 18 years, 18 years and up); clinical presentation according to heart defect (acyanotic, cyanotic); heart failure clinical functional class (FC), classified into I, II, III, and IV, according to the New York Heart Association (NYHA); pulmonary arterial hypertension (PAH) defined according to European guidelines[19]; arrhythmias registered in standard ECG or Holter monitoring; and treatment status (surgical treatment [patients who underwent corrective or palliative surgery; patients were called reoperated if they had undergone one or more than one surgical intervention for correction of residual lesions later after corrective surgery]; interventional cardiac catheterization; combined surgical and interventional catheterization; unrepaired [this category included three types of patients — the ones awaiting surgical treatment, or preoperative status; patients with small defects who didn’t need any treatment; and patients in inoperable situation according to the criteria used for invasive treatment]).

Patients diagnosed at 18 years of age and up were considered having late diagnosis.

When two or more congenital heart defects were present, main diagnosis was considered the one with greater pathophysiological impact.

According to CHD complexity based on the anatomical classification of the American Heart Association/American College of Cardiology (or AHA/ACC)[6], patients were classified into three categories:

    • Category I – Simple Defects: isolated small defects — ostium secundum atrial septal defect (ASD), ventricular septal defect (VSD) —; mild isolated pulmonic stenosis; repaired conditions with no significant residual lesion, shunt, or chamber enlargement — ligated or occluded patent ductus arteriosus (PDA), repaired ASD, sinus venosus defect, and VSD.

    • Category II – Moderate Complexity Defects: unrepaired moderate or large heart defects, repaired conditions with significant residual lesions — moderate and large unrepaired secundum ASD and PDA, VSD with associated abnormality and/or moderate or greater shunt, sinus venosus defect, partial or complete atrioventricular septal defect, moderate or severe pulmonic stenosis or regurgitation, infundibular right ventricular outflow obstruction, peripheral pulmonary stenosis (PS), congenital aortic valve disease, subvalvar aortic stenosis (excluding hypertrophic cardiomyopathy), supravalvar aortic stenosis, coarctation of the aorta, congenital mitral valve disease, Ebstein anomaly, tricuspid valve dysplasia, partial or total anomalous venous connection, straddling atrioventricular valve, anomalous coronary artery arising from the pulmonary artery, anomalous aortic origin of a coronary artery from the opposite sinus, aorto-left ventricular fistula, sinus of Valsava fistula/aneurysm, repaired tetralogy of Fallot.

    • Category III – Great Complexity Defects (complex heart disease): all forms of repaired, palliated, or unrepaired cyanotic CHD; congenitally corrected transposition of the great arteries (ccTGA).

Statistical Analysis

Data analysis was performed using software IBM Corp Released 2015, IBM SPSS Statistics for Windows, version 23.0, Armonk, NY: IBM Corp. Descriptive analysis was used and expressed in absolute and percentual (%) numbers for categorical variables, with their respective inferior limit (IL) and superior limit (SL) of the 95% confidence interval (CI). For numerical variables, mean and standard deviation values were used for continuous variables with normal distribution. For continuous variables without normal distribution, median and interquartile interval was applied. Chi-square test of Pearson, a non-parametric test, was used for comparison between categorical variables. Results were considered significant when P-value < 0.05.

The project of the present paper received approval of the Ethical Committee via “Plataforma Brasil”: “Certificado de Apresentação de Apreciação Ética (CAAE)” number 48865121.4.0000.5039.


Clinical demographic profile of adult patients with CHD is displayed in Tables 1, 2, 3. Frequency of CHD is shown in Tables 4 and 5.

Table 1 - Number of adult patients with congenital heart disease according to age range.
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)

CI=confidence interval; CL=confidence limit

Table 1 - Number of adult patients with congenital heart disease according to age range.
Table 2 - Clinical demographic profile of adult patients with congenital heart disease.
Age (years) Mean (SD) Median
IQI: 25% 50% 75%
32 (±14) 21 27 38
N (%) 95% CI: inferior CL/superior CL
Female 410 (58.2) 54.6%/61.8%
Male 294 (41.8) 38.2%/45.4%
Total 704 (100)
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%
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%

CHD=congenital heart disease; CI=confidence interval; CL=confidence limit; IQI=interquartile interval; SD=standard deviation

Table 2 - Clinical demographic profile of adult patients with congenital heart disease.
Table 3 - Clinical profile of adult patients with congenital heart disease (N=704).
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%
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%

AF=atrial fibrillation; CI=confidence interval; CL=confidence limit; NSVT=non-sustained ventricular tachycardia

Table 3 - Clinical profile of adult patients with congenital heart disease (N=704).
Table 4 - Most frequent congenital heart disease in adult patients according to diagnosis.
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%
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%

ASD=atrial septal defect; CHD=congenital heart disease; CI=confidence interval; CL=confidence limit; OP=ostium primum; OS=ostium secundum; PDA=patent ductus arteriosus; PS=pulmonary stenosis; SV=sinus venosus; VSD=ventricular septal defect

Table 4 - Most frequent congenital heart disease in adult patients according to diagnosis.
Table 5 - Less frequent congenital defects in adult patients according to diagnosis.
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%

AR=aortic regurgitation; CAVC=complete atrioventricular canal; ccTGA=congenitally corrected transposition of the great arteries; CI=confidence interval; CL=confidence limit; DORV=double outlet right ventricle; PA=pulmonary atresia; PAPVC=partial anomalous pulmonary venous connection; TAPVC=total anomalous pulmonary venous connection; TGA=transposition of the great arteries; VSD=ventricular septal defect

Table 5 - Less frequent congenital defects in adult patients according to diagnosis.

Most acyanotic heart defects were isolated defects but it was observed association of two or three defects with the same pathophysiology of equal magnitude, like shunt lesions, or defects of different pathophysiology but with equal magnitude, like PS and a shunt lesion.

Among patients with ASD, 148 (68.5%) had late diagnosis and 68 (31.5%) were diagnosed under 18 years of age. Both groups were compared (P-value < 0.0001). The most frequent type of ASD was ostium secundum (P-value < 0.0001). Most of the patients were women (155 – 71.7%) and were predominantly in NYHA FC I (P-value < 0.0001). Fifty-six (25.9%) patients had supraventricular arrhythmia, and among these, 37 (17.1%) had either atrial fibrillation or atrial flutter.

Most patients with VSD were diagnosed under 18 years of age (87 patients – 86.1%). Fifty-three (52.5%) had small defects and did not need repair, 32 (31.7%) had been repaired in childhood, and only three (3%) patients were awaiting surgery. One patient operated in adulthood was in atrial flutter. All patients with cyanotic heart disease were diagnosed in infancy and childhood. Among patients with tetralogy of Fallot, all had been repaired, except five patients, and this occurred because of parents’ choice. There were 53 (57.0%) in NYHA FC I, 34 (36.5%) in FC II, four (4.3%) in FC III, and two (2.2%) in FC IV. Seventeen patients (18.3%) were awaiting reoperation for pulmonary valve replacement due to severe residual pulmonary regurgitation.

Among patients with ccTGA, only two didn’t have any associated defect. The oldest one was a 54-year-old woman who was evolving in NYHA FC II, with mild systemic morphological right ventricular dysfunction, and sustaining sinus rhythm. She was one of the four patients who had late diagnosis. Two patients were evolving with episodes of sustained supraventricular tachycardia: one of them was awaiting ASD repair and the other didn’t have any associated defect. Five patients had dysfunctional systemic morphological right ventricle. In the overall, six patients had undergone cardiac surgical procedures: four patients repaired associated defects, one patient had a double-switch procedure in another reference center in São Paulo (Brazil), and another had a palliative modified Blalock-Taussig shunt in childhood for severe PS. The latter patient was one of the two patients who had complex anatomy, and criss-cross heart was suspected. Among operated patients, two of them had a pacemaker implantation postoperatively for 2nd degree atrioventricular block Mobitz II.

Patients with the severe form of PAH were inoperable (Table 3). There were 55 patients with a previous left-to-right shunt lesion who progressed to Eisenmenger syndrome (inverted shunt): ASD (34), VSD (12), complete atrioventricular canal defect (seven), and PDA (two). The remaining patients with severe PAH had transposition of the great arteries (three), pulmonary atresia with VSD (two), double outlet right ventricle (one), total anomalous pulmonary venous connection (one), single atrium (one), and other cyanotic heart defects (six). Patients with ASD, single atrium, and PDA were all diagnosed beyond 18 years of age.

Considering invasive treatment status of the cohort (Table 6), 182 (25.8% – 95% CI IL=22.2%, SL=28.6%) unrepaired patients were either awaiting repair (preoperation) or were inoperable, and this was a consequence of late diagnosis, that is, those diagnosed at 18 years of age and up. Most of the patients classified as preoperative status had ASD (55 patients).

Table 6 - Treatment status and types of invasive intervention in adults with congenital heart disease.
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)

BH=Blalock-Hanlon; CI=confidence interval; CL=confidence limit; MBTS=modified Blalock-Taussig shunt; PAB=pulmonary artery banding

Table 6 - Treatment status and types of invasive intervention in adults with congenital heart disease.

Interventional catheterization was applied in patients with ASD, PS, and PDA. Combined surgery plus interventional catheterization was applied in patients with coarctation of the aorta with significant residual coarctation.

Twenty-two patients with late diagnosis were following the natural history of the heart defect and were inoperable as a consequence of advanced heart failure. Half of them were ASD patients and over 60 years of age. The oldest was an 81-year-old woman with sinus venosus ASD. She was in atrial fibrillation and heart failure NYHA FC IV.


Global view of adults with CHD invasively treated in infancy and childhood reveals a new reality in opposition to five decades ago: the progressive and rapidly rising number of adult patients with residual lesions from previous corrective surgical procedures, predominantly survivals with tetralogy of Fallot and complex heart defects. Many of this new generation of postoperative adult patients will evolve with future need for invasive reintervention[1,2,3,4,5,6,7,12]. In Brazil, in addition to the need to cope with the new reality, old challenges like unrepaired patients due to late diagnosis and related complications still represent great concern[14,15,16,17].

Clinical demographic profile of the cohort studied herein showed significantly higher frequency of young adults, mainly women, similar to the findings observed in the southeastern Brazilian city of Ribeirão Preto[16] and in the Asian country of Taiwan[4]. When analysing patients’ origin, there was no difference between Fortaleza, including metropolitan area, and other towns.

Most defects belonged to the moderate complexity category, differently from the findings by Amaral et al.[16], in which predominated defects belonging to the low complexity category. Acyanotic CHD was significantly more frequent than cyanotic heart disease. In the overall, although most patients with acyanotic defects were diagnosed under 18 years of age, late diagnosis was a significant finding, especially in patients with ASD.

The most frequent CHD was ASD and the ostium secundum type significantly outnumbered other types. Although the number of unrepaired patients was high, in most of them invasive treatment was still feasible. The second most frequent CHD was VSD, mostly represented by a small defect which didn’t need any treatment. The majority of patients had early diagnosis and nearly all the ones who needed surgery had been repaired in childhood, the opposite to that observed in patients with ASD. Literature highlights septal defects, specially ostium secundum ASD and VSD as the most frequent CHD in adults[2,3,4,14,15,16].

Tetralogy of Fallot was the third most frequent CHD and nearly all patients had been repaired. Literature considers it the most frequent operated cyanotic heart disease that survives childhood[2,4,12]. In the report by Ruiz et al.[20], tetralogy of Fallot made up the greater part of the cohort. In the present study, severe pulmonary valve regurgitation was the residual lesion which led to reintervention in a considerable number of patients. These findings are in accord with the literature and call attention for the high incidence of adult patients with residual pulmonary regurgitation and increased morbidity as they reach middle age[6,7,21].

Congenital heart defects of great complexity were the least frequent, and most of them had been totally corrected in childhood. Only a few patients were living with a palliative surgery. ccTGA slightly outnumbered other complex defects followed by other conotruncal anomalies. Almost half of the patients with ccTGA were evolving with systemic right ventricular dysfunction. Recently, a review article published by Amaral et al.[22] emphasized the need for early diagnosis of this entity because of the implications concerning the systemic right ventricle for patients generally evolve with no symptoms in childhood and could reach elderly age, unrecognized.

Most patients analysed in the present paper were in NYHA FC I followed by FC II for heart failure. However, not a negligible number of patients were in FC III and FC IV. It is possible that the ones with advanced heart failure were unrepaired patients who evolved with complications and were not suitable for repair anymore, as the ones with Eisenmenger syndrome and those who were following the natural history of the disease. There were also the ones who had residual lesions from previous repairs. According to the literature, residual lesions and Eisenmenger syndrome are among the main causes of heart failure in adults with CHD[5,6,7,23,24]. Arrhythmias were the most frequent complications, particularly supraventricular arrhythmias like atrial flutter and fibrillation, and outnumbered the reports by Wu et al.[4] and Amaral et al.[16]. According to the literature, supraventricular arrhythmias represent the main complications of adults with CHD and are the result of significant residual lesions, surgical scars, and depend on the degree of heart complexity and age at repair[4,6,7,23].

Severe PAH with Eisenmenger syndrome was a frequent complication in patients with unrepaired high pulmonary blood flow defects, particularly ASD, and its frequency was higher than the reports in the literature[6-7,25]. Differently, in the study by Amaral et al.[16], Eisenmenger syndrome was more frequently found in patients with unrepaired VSD. It is a serious illness and there are multiple factors involved in its etiology as the location of the shunt defect, genetic predisposition, and age[6,7,19,25]. So, it is possible that late diagnosis may have influenced the development of severe PAH and Eisenmenger syndrome in a meaningful parcel of the adult population with unrepaired high pulmonary blood flow defects studied herein, leading to obscure prognosis for these patients.

Briefly, the present study revealed a problem that requires substantial consideration, represented by high frequency of patients with unrepaired defects, predominantly ASD, as a consequence of late diagnosis. It is important to highlight that untreated children are future adults to be treated, as long as they are still feasible of treatment. Early diagnosis with invasive treatment is fundamental to avoid future complications and increase in morbimortality.


The limitations of the present research were its retrospective cross-sectional design and the lack of comparison between repaired and unrepaired patients in the clinical profile.


The profile of adults with CHD in the present study revealed predominantly young adults, mainly women, coming from Fortaleza and the countryside in equal proportion. Acyanotic heart defects were the most frequent ones. Most patients had CHD of moderate complexity while heart disease of great complexity made up the minority of the cohort. Ostium secundum ASD was the most frequent defect followed by VSD and tetralogy of Fallot. Although more than half of the cohort had been repaired, unrepaired patients due to late diagnosis were a frequent finding that calls attention for a serious problem. Late diagnosis collaborated with the development of complications like arrhythmias, heart failure, and severe PAH, indicating the need for pursuing CHD diagnosis and repair in childhood.


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Authors’Roles & Responsibilities

MSBD= Substantial contributions to the conception and design of the work; and the acquisition, analysis, and interpretation of data for the work; drafting the work and revising it critically for important intellectual content; agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved; final approval of the version to be published

ASV= Substantial contributions to the analysis and interpretation of data for the work; revising the work critically for important intellectual content; agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved; final approval of the version to be published

HALR= Substantial contributions to the analysis and interpretation of data for the work; revising the work critically for important intellectual content; agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved; final approval of the version to be published

Article receive on Monday, January 30, 2023

Article accepted on Monday, April 10, 2023

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