|Year : 2017 | Volume
| Issue : 4 | Page : 121-124
The burden of truncus arteriosus in an Urban City in Africa: How are we fairing?
Barakat Adeola Animasahun, Aminat Titilayo Ogunlana, Henry Olusegun Gbelee
Department of Paediatrics and Child Health, Lagos State University College of Medicine, Ikeja; Department of Paediatrics, Lagos State University Teaching Hospital, Lagos, Nigeria
|Date of Web Publication||20-Dec-2017|
Barakat Adeola Animasahun
Department of Paediatrics and Child Health, Lagos State University College of Medicine, Ikeja, Lagos
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Background: The true incidence of truncus arteriosus in underdeveloped countries is difficult to determine. This is due largely to underreporting as a result of nonavailability of technologically advanced facilities to make definitive diagnosis prenatally. There is a lack of data on the profile and outcome of patients with persistent truncus arteriosus (PTA) in Nigeria. This study aims to document the demographic characteristics, mode of presentation, indications for echocardiography, associated anomalies, average age at diagnosis, and outcome of patients with truncus arteriosus in our center.
Methods: Prospective and cross-sectional involving consecutive patients diagnosed with PTA using echocardiography at the Paediatric Department of Lagos State University Teaching Hospital, Lagos, Nigeria as part of a large study between January 2008 and December 2015.
Results: Only 25 patients had PTA during the study period. The prevalence of PTA among children presenting at the study center during the study period was 7.9/100,000. It constituted 2.4% of the cases of congenital heart disease and 7.1% of cases of cyanotic congenital heart disease. The male:female ratio was 1:1.1. The ages of the patients at diagnosis ranged between 0.75 and 153 months with a mean age at diagnosis ± standard deviation of 18.4 months ± 37.7. Only about 40% of patients were diagnosed within the neonatal period. Cyanosis was the most frequent indication for evaluation.
Conclusion: PTA is as common in Nigeria as in the other parts of the world but diagnosed late. Cyanosis is the most common presenting feature.
Keywords: Arteriosus, burden, children, cyanosis, Nigeria, truncus
|How to cite this article:|
Animasahun BA, Ogunlana AT, Gbelee HO. The burden of truncus arteriosus in an Urban City in Africa: How are we fairing?. Heart Views 2017;18:121-4
| Introduction|| |
Persistent truncus arteriosus (PTA) is best defined as that condition, in which a single arterial trunk leaves the heart through a single semilunar valve and supplies the aorta, one or both pulmonary arteries, and the coronary arteries. The true incidence of truncus arteriosus in underdeveloped countries is difficult to determine , but reported an incident from the developed world appears to be larger than that reported in the developing world. This is due largely to underreporting as a result of nonavailability of technologically advanced facilities to make definitive diagnosis prenatally or in early neonatal life.
In developing countries, poor awareness, poverty, late diagnosis, and dearth of diagnostic facilities in resource-poor countries have been the bane of timely and appropriate diagnosis and early intervention. Only a few children benefit from diagnostic investigation  and much fewer benefit from surgical treatment.
Knowledge of the relative frequencies of different congenital heart lesions associated with truncus arteriosus is important to physicians and health planners for the purpose of training, management, and the planning of health-care systems.
Several comorbid structural heart lesions have been found to be associated with PTA with their attendant influence on age, mode of presentation, surgical outcome, and overall survival. Although the absence of comorbidities with truncus arteriosus is documented to be associated with a better immediate postsurgical survival rate, the presence of several associated cardiac anomalies, has no adverse effect on overall survival rates, provided definitive diagnosis is made early, and repair is undertaken within the neonatal period.,
While surgical repair in infancy has improved to elective primary repair in early neonatal life in the developed world,,, children with truncus arteriosus in resource-poor countries still present late, are underdiagnosed, and corrective surgical repair are unaffordable to their caregivers. With delayed intervention, the prognosis is uniformly poor as most infants die within the first few months of life while others may develop complications such as pulmonary vascular obstructive disease later in life.
PTA has been reported only as one of the congenital heart diseases in Nigeria. However, there is lack of data on the profile and outcome of patients with PTA as a specific congenital heart disease in Nigeria. This study aims to document the demographic characteristics, mode of presentation, indications for echocardiography, associated anomalies, average age at diagnosis, and outcome of patients with truncus arteriosus in our center. This will help provide data to justify the need for planning and provision of the state of the art care for these patients, it will also help create awareness of truncus arteriosus, and the need for early intervention in these patients in the region.
| Methods|| |
All children presenting to the Paediatric Cardiology Unit of the Lagos State University Teaching Hospital between May 2008 and May 2015, with echocardiographic diagnosis of truncus arteriosus were recruited into the study. Data on the age, sex, indication for referral to the Paediatric Cardiology Unit or indication for echocardiography, associated cardiac and noncardiac congenital anomalies, presenting complaints, oxygen saturation, echocardiographic findings, and outcome were documented on each patient.
Patients were appropriately managed according to individual presenting complaints and followed up prospectively. Patients whose parents could afford or got sponsored for surgical management benefitted from it while others continued to receive appropriate medical/conservative management. Patients' condition as at May 31, 2015, was reported for the purpose of this study. However, close follow-up and monitoring are ongoing as long as patient is alive.
Echocardiography was done as part of routine care for the children thereby excluding the need for clearance by Hospital Ethical Committee.
Analysis was done using Microsoft Excel statistical package supplemented with Statistical Package for Social Sciences version 17.0 (SPSS Inc, Chicago). Measures of statistical location such as mean and standard deviation (SD) were determined. Strength of association was tested using correlation and regression analysis. Probability values <0.05 were considered statistically significant.
| Results|| |
During the 7-year period under review, 25 patients with echocardiographic evidence of truncus arteriosus were recruited. A total of 315,319 children were seen at the study center during the study period. Patient with PTA constituted 25/315,319. The prevalence of PTA among children presenting at the study center during the study period was 7.9/100,000. It constituted 2.4% of the cases of congenital heart disease and 7.1% of cases of cyanotic congenital heart disease. There were 12 (48%) males giving a male:female ratio of 1:1.1. The ages of the patients at diagnosis ranged between 0.75 and 153 months with a mean age at diagnosis ± SD of 18.4 months ± 37.7. Only about 40% of patients were diagnosed within the neonatal period. Another 32% were diagnosed in infancy and the remaining beyond the infancy period as shown in [Table 1].
Cyanosis was the most common (48%) primary indication for echocardiography among the patients. Both in isolation and in combination with other symptoms. This was followed by congestive cardiac failure, which had supervened in four (16%) of the patients. Other indications for echocardiography (observed in one patient each) included congenital anomalies (necessitating full cardiac evaluation), persistent tachypnea, Down's syndrome, failure-to-thrive, and precardiac catheterization evaluation.
Transcutaneous oxygen saturation levels range between 70% and 97% with a mean and SD of 83.8 ± 10.9.
At echocardiographic diagnosis of truncus arteriosus, 15 (60%) patients were found to have concomitant congenital cardiac defects. The associated cardiac anomalies included ventricular septal defect (VSD), atrial septal defect (ASD), and atrioventricular septal defect in seven (28%), four (16%), and two (8.0%) patients, respectively, whereas patent ductus arteriosus (PDA) and pulmonary stenosis (PS) were observed in one (4.0%) patient each as shown in [Figure 1].
All the patients were referred for surgical intervention outside Nigeria because there is currently no facility for surgical intervention for these patients in Nigeria. In terms of the outcome of these patients, only five were able to attempt surgical intervention, others either died or were lost to follow-up. Two of the patients died onboard while traveling to the referral center for surgery. Only three of the patients had surgical intervention. Two during the neonatal period and one after 2 years of age. One of the patients who had surgery done within the neonatal period is still on follow-up although very ill and with features suggestive of pulmonary obstructive vascular disease. The second died a few month postsurgery. The patient who had surgery done after infancy is stable on follow-up.
| Discussion|| |
Truncus arteriosus represented 2.4% of all congenital cardiac anomalies in our center. This is similar to that obtainable in several previous studies. The male:female ratio of 1:1.2 is also comparable to obtained by Samánek et al. Interestingly, recent local studies ,, have failed to record any case of PTA in clinical profile studies. The reason for this is not immediately clear. It may be due to the fact that these studies were carried out over a shorter period and hence involved fewer cases of congenital heart disease.
However, in some other local studies, truncus arteriosus was found to account for 21% of all congenital anomalies that formed underlying causes of pneumonia.
The average age at diagnosis in our study was 18.4 months. This age is very high. This very high average age at diagnosis further underscores late presentation which characterizes the health-seeking behavior in the developing countries like Nigeria. The average age at diagnosis in the current study was statistically significantly higher than that seen in a cohort study by Williams et al.
There was also a downward trend in the age at presentation as the cohort study progressed over the years. The latest average age at diagnosis among the last batch of patients in the cohort was 1 day despite the fact that the study was concluded more than a decade before our study commenced.
In our opinion, this further supports the belief that there are better awareness and health-seeking behavior in the developed countries which has unfortunately eluded us in resource-poor countries. Another plausible explanation may be because of the most common complaint at presentation. Cyanosis in our study compared with congestive cardiac failure in their study. Congestive cardiac failure which is likely to manifests tachypnea is more likely to affect the overall well-being of the baby and make the parent see the need to presentation at the hospital for help unlike in our environment. Cyanosis as the most common sign at presentation may also explain the reason for late presentation in our study because the parent may not recognize cyanosis nor identify it as a sign of illness in the baby.
The outcome of these patients is not surprising, considering the fact that most of the patients presented after the neonatal period when they could no longer benefit from the advocated elective primary repair in early neonatal life in the developed world.,, It is obvious from this study however that those that presented within the neonatal period could still not be helped in their immediate environment due to nonavailability of surgical center where the needed surgery could be carried out. Many of the parents could not afford the needed corrective surgery even when referred to the appropriate center. Some also died while traveling the long hours to go and earn the needed surgery; this further emphasizes the need to have the needed surgical intervention available in the immediate environment.
With delayed intervention, the prognosis is uniformly poor as most infants die within the first few months of life while others may develop complications such as pulmonary obstructive vascular disease later in life.
The clinical signs at presentation necessitating admission and cardiac evaluation were similar to that observed by Williams et al. though not in the same order of occurrence. Cyanosis was the most common clinical sign in our study as against congestive cardiac failure in the study by Williams et al. This difference is likely due to the larger number of patients observed as well as valve regurgitation observed in 69% of their study patients.
Associated cardiac anomalies are known to have a remarkable influence on the average age at diagnosis, clinical presentation, and prognosis in children with PTA. The true incidence of cardiac anomalies associated with truncus arteriosus is difficult to establish as no identifiable local study (and only a few studies outside the West African subregion) has considered truncus arteriosus in isolation. VSD was the most common (28%) congenital cardiac anomaly in our study followed in descending order of occurrence by ASD, atrioventricular septal defect (AVSD), PDA, and PS. This is in contrast to concomitant cardiac anomalies as seen by Williams et al. in a retrospective study that showed ASD (62%) as the most common associated cardiac anomaly with PTA. Others were right aortic arch (30%), PDA (18%), PS (16%), and AVCD (1%). Despite numerous noncardiac anomalies described in previous studies. Down syndrome was the only noncardiac congenital anomaly observed in our study patients compared with DiGeorge syndrome which was the most common in the study by Williams et al.
| Conclusion|| |
Truncus arteriosus is as common among Nigerian patients as in other parts of the world but is often diagnosed late, and it appears to be underreported. Cyanosis is the most common indication for echocardiography.
There is currently no facility for surgical intervention in these patients locally. Outcome is currently poor in these patients.
There is a need to increase awareness of truncus arteriosus and its presenting features to aid early diagnosis; there is also a need for collaboration with cardiac centers in the developed world for the establishment of cardiac centers locally to enable prompt intervention to improve outcome in these patients.
We greatly acknowledge the patients who participated in this study and their caregivers including other health-care professionals who participated in their care.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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