Abstract
Background
Children with unrepaired congenital heart defects (CHDs) are at risk of developing complications related to the scarcity of interventions, delayed diagnosis, financial constraints, and difficulty reaching facilities in low-and middle-income countries.
Objective
Describe the frequency of complications by age and CHD type in children with un-intervened CHDs seen at Bahir Dar University Tibebe-Ghion Specialized Teaching Hospital.
Method
We conducted a retrospective cross-sectional study on children with un-intervened CHDs seen from September 01, 2021, to August 31, 2024. Data were collected from September 20 to 30, 2024, retrospectively. Categorical variables were analyzed in the form of proportions. Discrete variables were summarized as means (SD). Binary logistic regression was performed with 95 % confidence interval. P -value <0.05 was considered significant. Data were analyzed using SPSS version 27.
Result
Of the 310 children with un-intervened CHDs, 47 % were female. The mean (SD) age was 28 months (42). Wasting (46 %) is the most common complication in children with CHD, followed by congestive heart failure (41 %), and recurrent respiratory tract infection (30 %). Seventy percent of children with un-intervened CHDs have at least one complication (68 % of acyanotic and 77 % of cyanotic CHDs). After adjusting for covariates, one month increase in age of children with un-intervened CHD increases the proportion of complications by 2 % {AOR = 1.019, 95 % CI = (1.007, 1.031)}.
Conclusion
A higher proportion of children with CHD had at least one or more complications. A one month delay in intervention of children with un-intervened CHDs has a 2 % increase in the occurrence of complications.
Highlights
- •
Seventy percent of children with un-intervened congenital heart defects had at least one complication/consequence.
- •
Wasting (45.5%) is the most common complication in children with CHD followed by congestive heart failure (41%), recurrent respiratory tract infection (30%) and pulmonary hypertension (27%) respectively.
- •
A one month delay in intervention of children with un-intervened CHDs has a 2% increase in occurrence of complications.
1
Introduction
Congenital heart defects (CHDs) are the most common congenital malformation, occurring in just over 1 % of live births [ ]. Pediatric congenital heart disease is a life-cycle condition [ ] and impacts all aspects of QOL (Quality of Life) of patients and their families and is associated with high comorbidity and complications [ ]. In low-and-middle-income countries (LMICs), children with CHDs are at risk of developing complications related to the scarcity and/or non-existence of surgical and interventional services, delayed diagnosis, insufficient caregiver education, financial constraints, difficulty reaching treatment centers, sociocultural stigma, and sex-based discrimination of patients with CHD. In addition, the lack of hospital resources and workforce, the need for prolonged hospitalization, and strained physician–patient relationships contribute significantly to delayed intervention, hence complications [ , ].
The burden of congenital heart defects is immense. It is an enormous problem in LMICs and particularly in sub-Saharan Africa [ ]. The WHO estimate suggests that 90 % of these children have suboptimal or no access to cardiac care at all due to the fact that most of these children are concentrated in LMICs, particularly in sub-Saharan Africa [ , ]. Despite this reality, survival in children with CHD has increased substantially [ ]. Over 85–90 % of babies born with a CHD now live to at least age 18–20 years [ , ]. Though they live to this age, children with CHDs face a life-long risk of health problems such as issues with growth and eating, developmental delays, difficulty with exercise, heart rhythm problems, heart failure, sudden cardiac arrest, or stroke [ , ].
There are notable pre-operative barriers to access of CHDs intervention and surgery in LMICs. These include delayed diagnosis, insufficient caregiver education, financial constraints, difficulty reaching treatment centers, and sex-based discrimination of patients with CHD [ ]. Recognizing these barriers to accessing health care and designing evidence-based interventions to increase access to CHD surgical care in LMICs is crucial so as to decrease the complications that decrease quality of life and increase psychosocial strain on the child and the family due to the high out-of-the-pocket expenditure [ , ].
Many children with CHD live with complications related to their condition [ ]. A study done in Rajshahi Medical College & Hospital, Bangladesh showed that 92 % of children with CHDs present with different complications [ ]. Another study from Sweden illustrates that 28 % have experienced at least one cardiac complication [ ].
A number of cardiac centers have been developed over the last decade. However, most are in the high-income countries (HICs) [ ], and are not geographically well-distributed in the LMICs where the service is already scarce to non-existent [ , ]. Children with un-intervened CHDs who are facing scarcity of interventional services are at risk of developing complications and co-morbidities that lead to long-term impact on health [ ]. These children are assumed to be vulnerable to congestive heart failure, stroke, atrial fibrillation (AF), infective endocarditis, recurrent respiratory tract infection, developmental delay, hypercyanotic spells, pulmonary vascular obstructive disease including Eisenmenger’s physiology, and growth impairment [ ].
Low-and-middle-income countries, including our country, are under a great challenge to accommodate pediatric cardiac care due to financial constraints, health-seeking behavior of the community, lack of awareness and delay in diagnosis, maldistribution of resources and limited to non-existent services [ , , ]. This all, in turn, will increase the vulnerability of children with CHDs to develop complications by delaying the timing of intervention.
Hence, our study has aimed to show the burdens of complications associated with un-intervened CHDs in children, which otherwise was expected to be not significant had it been intervened early. This finding is expected to inform concerned national/ international institutions to take steps for improving the health of vulnerable and marginalized children with un-intervened CHDs through their various programs and schemes.
2
Methods and materials
2.1
Study setting and participants
Bahir Dar University Tibebe-Ghion specialized teaching hospital is a referral center for pediatric cardiac services in Northwest Ethiopia with a capacity of 493 beds and an estimated 5 million catchment population. The hospital’s pediatric cardiac unit provides only pediatric diagnostic and medical management services. There is no intervention service (Trans-catheter and or surgical palliation/correction service in the hospital. The hospital is located in Bahir Dar city, the capital city of Amhara National regional state, the second populous region in Ethiopia. Three hundred ten children with un-intervened CHDs from birth to fifteen years of age were included in the study.
2.2
Study design
Institution-based retrospective cross-sectional study was conducted on children with un-intervened CHDs who were seen in Bahir Dar University Tibebe-Ghion Specialized teaching hospital pediatric cardiac unit from September 01, 2021 to August 31, 2024. Children with un-intervened CHDs who fulfilled the inclusion criteria were included in the study. Children with incomplete file documentation and CHD diagnosis without echocardiography confirmation were excluded. Data were collected from September 20, 2024 to September 30, 2024 on a retrospective basis from the archives of the hospital. Data were collected by trained nurses and pediatric and child health residents.
2.3
Sample size and selection
The sample size was determined using a formula to estimate a population parameter based on known prevalence estimates from previously published literature (P) = 28 % with CHD has at least one complication [ ]; 5 % type I error ( P < 0.05) (Z1 α/2 =), 1.96 and 5 % precision/absolute error (d).
Sample Size=Z1/22∗P1−P]d2=1.962∗0.28∗1−0.28/0.052=310.
All children seen as an outpatient or admitted to the ward with un-intervened CHDs in the specified period fulfilling the inclusion criteria were included.
2.3.1
Exclusion criteria
Children whose medical record number (MRN) was missing or not addressing appropriate medical record files.
Children with incomplete medical record file documentation.
2.4
Diagnosis and classification [ ]
Critical CHD: defined as lesions that require surgery or catheter intervention in the first 28 days of life [ ]. Incompatible with survival without intervention in newborn period/early infancy.
Transposition of great arteries, Obstructed TAPVC, Duct-dependent pulmonary or systemic circulations.
Major CHD: a heart defect which requires an intervention during the first year of age [ ] for optimal long-term outcome.
TOF, DORV, large VSD and PDA, complete atrioventricular canal, truncus arteriosus, aorto-pulmonary window, single ventricle physiology, unobstructed TAPVC, ALCAPA, severe outflow tract obstruction.
CHD that typically manifests at older age: diagnosis seldom made in early childhood; intervention required to prevent long-term sequelae in adulthood.
Moderate or large ASD, some patients with Ebstein’s anomaly, relatively less severe forms of aortic/pulmonary valve stenosis, ccTGA with intact septum.
Minor CHD: long-term, symptom-free survival expected without any specific intervention.
Small left-to-right shunts (ASD, VSD, PDA), Bi-commissural aortic valve.
2.5
Operational definition
Complication: an event or occurrence that is associated with a disease or a healthcare intervention, is a departure from the desired course of events, and may cause or be associated with suboptimal outcome [ , ].
Consequences: something that happened and was predictable, nevertheless, could not be avoided [ ].
Wasting : defined as weight for height < −2 standard deviation from the median of the World Health Organization (WHO) Child Growth Standards in under-five children or MUAC <125 mm in children between 6 and 59 months of age. BMI-for-age < -2SD for children >5 years [ ].
Stunting: defined as having a height-for-age z score (HAZ) < −2 standard deviation from the median of the World Health Organization (WHO) child growth standards [ ].
Cardiac intervention : defined as patients with CHD having undergone at least 1 cardiovascular surgery or cardiac interventional catheterization related to CHD [ ]. Children who had no these interventions prior to the evaluation time will be considered as children with “un-intervened CHD”.
2.6
Statistical analysis
Recorded data were cleared, coded, and entered into SPSS version 27 for analysis. Categorical variables, including sex, type of cardiac lesion, complications/consequences, place of residency, and anthropometric and clinical profiles, were analyzed in the form of proportions and percentages and presented in tables and figures. Discrete variables, including age, were analyzed and summarized as means (SD) and medians (IQR). In our study, binary logistic regression was conducted with 95 % confidence interval to predict the binary outcome of complications – 1 for presence and 0 for absence of complications/consequences based on the independent variables. P -value <0.05 was considered significant.
3
Result
We collected data from a total of 310 medical record files of children with echocardiography confirmed congenital heart defects (CHDs) with complete documentation who were seen from September 01, 2021, to August 31, 2024. Of all children with CHDs included in the study, 46.5 % were female, and the remaining 53.5 % were male. The mean (SD) age of children included in the study was 28.4 months (41.6), with the median (IQR) being 9 months (1.5–36). Acyanotic CHDs constitute 70 % of all children with CHD, leaving the other 30 % to be cyanotic. Isolated Ventricular septal defect is the most common acyanotic CHD, constituting for 18 % of all CHDs. Tetralogy of Fallot is the leading cyanotic CHD with 9 % contribution to all forms of CHD. Suspected genetic/ syndromic abnormality was detected in 27 % of all children with CHD. Critical and major CHDs comprise 71 % of all children with CHD enrolled in the study ( Table 1 ).
| Socio-demographic and clinical parameters | At least one complications | Total | |||||
|---|---|---|---|---|---|---|---|
| Yes (218) | % | No (92) | % | # (310) | % | ||
| Age in months | Mean (SD b ) | 35 (45) | 13 (27) | 28.4 (41.6) | |||
| Sex of children | Female | 106 | 49 | 38 | 41 | 144 | 46 |
| Male | 112 | 51 | 54 | 59 | 166 | 54 | |
| Place of residency | Urban | 128 | 59 | 67 | 73 | 195 | 63 |
| Rural | 90 | 41 | 25 | 27 | 115 | 37 | |
| Suspected syndrome/ genetic abnormality | No | 155 | 71 | 70 | 76 | 225 | 73 |
| Yes | 63 | 29 | 22 | 24 | 85 | 27 | |
| Cardiac lesion category | Acyanotic CHDs | 146 | 67 | 70 | 76 | 216 | 70 |
| Cyanotic CHDs | 72 | 33 | 22 | 24 | 94 | 30 | |
| Acyanotic CHDs | ASD c , Isolated | 17 | 8 | 12 | 13 | 29 | 9 |
| VSD d , Isolated | 37 | 17 | 19 | 21 | 56 | 18 | |
| PDA e , Isolated | 21 | 10 | 11 | 12 | 32 | 10 | |
| AVSD f , Isolated | 25 | 11 | 2 | 2 | 27 | 9 | |
| Two or more acyanotic CHDs | 36 | 17 | 18 | 20 | 54 | 17 | |
| Other acyanotic CHDs | 10 | 5 | 8 | 9 | 18 | 6 | |
| Cyanotic CHDs | TOF g | 25 | 11 | 3 | 3 | 28 | 9 |
| d-TGA h | 9 | 4 | 2 | 2 | 11 | 4 | |
| Tricuspid atresia | 7 | 3 | 6 | 7 | 13 | 4 | |
| Double Outlet Right Ventricle | 11 | 5 | 1 | 1 | 12 | 4 | |
| RVOTO i with shunt lesion | 10 | 5 | 6 | 7 | 16 | 5 | |
| Other cyanotic CHDs | 10 | 5 | 4 | 4 | 14 | 5 | |
| CHDs Category based on natural history | Critical CHDs | 10 | 5 | 3 | 3 | 13 | 4 |
| Major CHDs | 169 | 78 | 39 | 42 | 208 | 67 | |
| CHD manifesting at older age | 26 | 12 | 7 | 8 | 33 | 11 | |
| Minor CHDs | 13 | 6 | 43 | 47 | 56 | 18 | |
| Total | 218 | 70 | 92 | 30 | 310 | 100 | |
f Atrioventricular septal defect.
h Dextro transposition of the great arteries.
Wasting (45.5 %) is the most common complication in children with CHD followed by congestive heart failure (41 %), recurrent respiratory tract infection (30 %), and pulmonary hypertension (27 %), respectively. Wasting is more common in children with cyanotic CHDs than in children with acyanotic CHDs. Pulmonary hypertension (56 %) and developmental delay (67 %) are more common in children with atrioventricular septal defect. Double outlet right ventricle is the leading cause of congestive heart failure (75 %) and recurrent lower respiratory tract infection (58 %). Circulatory collapse is more common in children with d-TGA than any other CHD (27 %) ( Table 2 ).
| Complications/consequences of un-intervened CHD | Category of the congenital heart defects (CHDs) | Total CHDs | ||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Acyanotic CHDs | Cyanotic CHDs | |||||||||||||||
| Isolated | ≥2 acyanotic CHD | Other acyanotic CHD | Total acyanotic CHD | TOF | d-TGA | Tricuspid atresia | DORV a | RVOTO with shunt | Other cyanotic CHDs | Total cyanotic CHD | ||||||
| ASD | VSD | PDA | AVSD | |||||||||||||
| CHF b | Yes/# | 9 | 23 | 12 | 14 | 26 | 7 | 91 | 5 | 6 | 2 | 9 | 4 | 9 | 35 | 126 |
| % | 31 | 41 | 38 | 52 | 48 | 39 | 42 | 18 | 55 | 15 | 75 | 25 | 64 | 37 | 41 | |
| PHT c | Yes/# | 8 | 22 | 10 | 15 | 14 | 4 | 73 | 1 | 3 | 0 | 3 | 1 | 4 | 12 | 85 |
| % | 28 | 39 | 31 | 56 | 26 | 22 | 34 | 4 | 27 | 0 | 25 | 6 | 29 | 13 | 27 | |
| Circulatory collapse | Yes/# | 0 | 2 | 0 | 0 | 0 | 0 | 2 | 0 | 3 | 0 | 0 | 0 | 0 | 3 | 5 |
| % | 0.0 | 3.6 | 0.0 | 0.0 | 0.0 | 0.0 | 0.9 | 0.0 | 27 | 0.0 | 0.0 | 0.0 | 0.0 | 3.2 | 1.6 | |
| Hepatic Dysfunction | Yes/# | 0 | 0 | 0 | 0 | 1 | 0 | 1 | 0 | 0 | 0 | 1 | 0 | 0 | 1 | 2 |
| % | 0.0 | 0.0 | 0.0 | 0.0 | 1.9 | 0.0 | 0.5 | 0.0 | 0.0 | 0.0 | 8.3 | 0.0 | 0.0 | 1.1 | 0.6 | |
| Renal Dysfunction | Yes/# | 0 | 1 | 1 | 0 | 0 | 1 | 3 | 2 | 1 | 0 | 1 | 0 | 0 | 4 | 7 |
| % | 0.0 | 1.8 | 3.1 | 0.0 | 0.0 | 5.6 | 1.4 | 7.1 | 9.1 | 0.0 | 8.3 | 0.0 | 0.0 | 4.3 | 2.3 | |
| Recurrent RTI d | Yes/# | 8 | 22 | 6 | 12 | 15 | 4 | 67 | 2 | 3 | 2 | 7 | 4 | 8 | 26 | 93 |
| % | 28 | 39 | 19 | 44 | 28 | 22 | 31 | 7 | 27 | 15 | 58 | 25 | 57 | 28 | 30 | |
| Polycythemia | Yes/# | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 7 | 1 | 0 | 2 | 1 | 1 | 12 | 12 |
| % | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 25 | 9 | 0 | 17 | 6 | 7 | 13 | 4 | |
| DIC e | Yes/# | 0 | 0 | 1 | 0 | 0 | 0 | 1 | 0 | 0 | 0 | 1 | 0 | 1 | 2 | 3 |
| % | 0.0 | 0.0 | 3.1 | 0.0 | 0.0 | 0.0 | 0.5 | 0.0 | 0.0 | 0.0 | 8.3 | 0.0 | 7.1 | 2.1 | 1.0 | |
| Stroke | Yes/# | 0 | 0 | 1 | 0 | 2 | 0 | 3 | 2 | 0 | 0 | 2 | 0 | 1 | 5 | 8 |
| % | 0.0 | 0.0 | 3.1 | 0.0 | 3.7 | 0.0 | 1.4 | 7.1 | 0.0 | 0.0 | 17 | 0.0 | 7.1 | 5.3 | 2.6 | |
| Systemic embolization | Yes/# | 0 | 0 | 1 | 0 | 2 | 0 | 3 | 2 | 0 | 0 | 2 | 0 | 1 | 5 | 8 |
| % | 0.0 | 0.0 | 3.1 | 0.0 | 3.7 | 0.0 | 1.4 | 7.1 | 0.0 | 0.0 | 17 | 0.0 | 7.1 | 5.3 | 2.6 | |
| Infective endocarditis | Yes/# | 0 | 2 | 4 | 0 | 4 | 0 | 10 | 4 | 1 | 0 | 2 | 1 | 1 | 9 | 19 |
| % | 0.0 | 3.6 | 13 | 0.0 | 7.4 | 0.0 | 4.6 | 14 | 9.1 | 0.0 | 17 | 6.3 | 7.1 | 9.6 | 6.1 | |
| PTE f | Ye# | 0 | 0 | 1 | 0 | 0 | 0 | 1 | 0 | 0 | 0 | 1 | 0 | 1 | 2 | 3 |
| % | 0.0 | 0.0 | 3.1 | 0.0 | 0.0 | 0.0 | 0.5 | 0.0 | 0.0 | 0.0 | 8.3 | 0.0 | 7.1 | 2.1 | 1.0 | |
| Brain abscess | Yes/# | 0 | 0 | 1 | 1 | 0 | 0 | 2 | 1 | 0 | 0 | 1 | 0 | 1 | 3 | 5 |
| % | 0.0 | 0.0 | 3.1 | 3.7 | 0.0 | 0.0 | 0.9 | 3.6 | 0.0 | 0.0 | 8.3 | 0.0 | 7.1 | 3.2 | 1.6 | |
| Reduced LV g function | Yes/# | 1 | 3 | 1 | 0 | 3 | 2 | 10 | 2 | 0 | 0 | 2 | 1 | 2 | 7 | 17 |
| % | 3.4 | 5.4 | 3.1 | 0.0 | 5.6 | 11 | 4.6 | 7.1 | 0.0 | 0.0 | 17 | 6.3 | 14.3 | 7.4 | 5.5 | |
| Reduced RV h function | Yes/# | 2 | 4 | 2 | 0 | 1 | 3 | 12 | 4 | 0 | 0 | 2 | 0 | 2 | 8 | 20 |
| % | 6.9 | 7.1 | 6.3 | 0.0 | 1.9 | 17 | 5.6 | 14 | 0.0 | 0.0 | 17 | 0.0 | 14.3 | 8.5 | 6.5 | |
| Hypercyanotic attack | Yes/# | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 4 | 0 | 1 | 1 | 0 | 0 | 6 | 6 |
| % | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 14 | 0 | 8 | 8 | 0 | 0 | 6 | 2 | |
| Arrhythmia | Yes/# | 0 | 1 | 0 | 0 | 2 | 1 | 4 | 2 | 0 | 0 | 0 | 0 | 1 | 3 | 7 |
| % | 0.0 | 1.8 | 0.0 | 0.0 | 3.7 | 5.6 | 1.9 | 7.1 | 0.0 | 0.0 | 0.0 | 0.0 | 7 | 3.2 | 2.3 | |
| Developmental Delay | Yes/# | 7 | 13 | 6 | 18 | 14 | 2 | 60 | 6 | 3 | 3 | 3 | 2 | 3 | 20 | 80 |
| % | 24 | 23 | 19 | 67 | 26 | 11 | 28 | 21 | 27 | 23 | 25 | 13 | 21 | 21 | 25.8 | |
| Wasting | Yes/# | 9 | 28 | 15 | 13 | 24 | 6 | 95 | 14 | 5 | 6 | 8 | 4 | 9 | 46 | 141 |
| % | 31 | 50 | 47 | 48 | 44 | 33 | 44 | 50 | 46 | 46 | 67 | 25 | 64 | 49 | 45.5 | |
| Stunting | Yes/# | 7 | 19 | 10 | 10 | 16 | 5 | 67 | 10 | 4 | 3 | 3 | 6 | 5 | 31 | 98 |
| % | 24 | 34 | 31 | 37 | 30 | 28 | 31 | 36 | 36 | 23 | 25 | 38 | 36 | 33 | 32 | |
| Anemia | Yes/# | 4 | 5 | 3 | 4 | 3 | 1 | 20 | 1 | 0 | 0 | 2 | 1 | 0 | 4 | 24 |
| % | 14 | 9 | 9 | 15 | 6 | 6 | 9 | 4 | 0.0 | 0.0 | 17 | 6 | 0.0 | 4 | 7.7 | |
| Total | 29 | 56 | 32 | 27 | 54 | 18 | 216 | 28 | 11 | 13 | 12 | 16 | 14 | 94 | 310 | |
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