Frequency of congenital cardiac anomalies in patients with anorectal malformations.

Ali, Safdar; Uzair, Muhammad; Rehman, Fayaz Ur; Imran, Mohammad; Khan, Erum Behroz; Khan, Muhammad Asim

doi:10.29309/tpmj/2020.27.12.4782

Cited by 2 publications

(1 citation statement)

References 12 publications

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“…In addition to Down syndrome and VACTERL, ARM is also known to be associated with congenital heart disease (CHD). However, the reported prevalence of CHD among ARM patients is highly variable, ranging from as low as 9% to as high as 50% (4)(5)(6)(7)(8)(9)(10)(11)(12). Furthermore, there is a con icting report on the most common cardiac lesion in ARM patients, with ventricular septal defect (8, 13), atrial septal defect (4,12), and tetralogy of Fallot (6) described as frequent lesions.…”

Section: Introductionmentioning

confidence: 99%

Survival and factors associated with mortality among infants with anorectal malformation: a population-based study from a middle-income country

Mat Bah,

Zahari,

Kasim

et al. 2023

Eur J Pediatr

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Limited data on the survival of anorectal malformation (ARM) patients from lower-and-middle income countries is available. This retrospective population-based study from the State of Johor, Malaysia, determines the incidence, mortality rate, and survival of ARM patients and factors associated with mortality. Kaplan-Meier survival analysis was used to estimate the survival of ARM patients at 1-, 5-and 10-years. In addition, multivariate cox-regression analysis was used to analyze mortality-related factors. There were 175 ARM patients among 803850 live births, giving an overall ARM incidence of 2.2 (95% con dence interval [CI], 1.9 to 2.5) per 10,000 live births. The male-to-female ratio was 1.5: 1. There was 122 (69%) non-isolated ARM, of which 41 were Down syndrome and 34 had VACTERL. Seventy-three (41.7%) had CHD, with 38 severe and 35 non-severe CHD. Overall, 33 (18.9%) patients died, with a median age of death of 5.7 months (Interquartile range 25 days to 11.2 months). The overall estimated 1-, 5-and 10-year survival rate for ARM patients was 82.3% (95% CI, 76.0% -88.6%), 77.3% (95% CI, 70.4% -84.2%), and 77.3% (95% CI, 70.4%-84.2%), respectively. Univariate analysis shows that non-isolated ARM, VACTREL association, and severe CHD were associated with mortality. However, only severe CHD is the independent factor associated with mortality, with a hazard ratio of 4.03 (95% CI: 1.93-8.42). Conclusion: CHD is common among ARM patients, and one in ve ARM patients had a severe cardiac defect, signi cantly affecting their survival. What Is KnownVACTREL association and congenital heart disease is common in patient with anorectal malformation Low birth weight and prematurity are associated with a lower rate of survival What is new: Congenital heart disease is common in ARM patients in a middle-income country Severe congenital heart disease plays a signi cant role in the survival of patients with an anorectal malformation in lower-and middle-income countries.

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Section: Introductionmentioning

confidence: 99%

Survival and factors associated with mortality among infants with anorectal malformation: a population-based study from a middle-income country

Mat Bah,

Zahari,

Kasim

et al. 2023

Eur J Pediatr

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Survival and Factors Associated with Mortality Among Infants with Anorectal Malformation: A Population- Based Study from A Middle-Income Country

Bah

Zahari

KASIM³

et al. 2023

Preprint

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Limited data on the survival of anorectal malformation (ARM) patients from lower-and-middle income countries is available. This retrospective population-based study from the State of Johor, Malaysia, determines the incidence, mortality rate, and survival of ARM patients and factors associated with mortality. Kaplan-Meier survival analysis was used to estimate the survival of ARM patients at 1-, 5- and 10- years. In addition, multivariate cox-regression analysis was used to analyze mortality-related factors. There were 175 ARM patients among 803850 live births, giving an overall ARM incidence of 2.2 (95% confidence interval [CI], 1.9 to 2.5) per 10,000 live births. The male-to-female ratio was 1.5: 1. There was 122 (69%) non-isolated ARM, of which 41 were Down syndrome and 34 had VACTERL. Seventy-three (41.7%) had CHD, with 38 severe and 35 non-severe CHD. Overall, 33 (18.9%) patients died, with a median age of death of 5.7 months (Interquartile range 25 days to 11.2 months). The overall estimated 1-, 5- and 10-year survival rate for ARM patients was 82.3% (95% CI, 76.0% – 88.6%), 77.3% (95% CI, 70.4% - 84.2%), and 77.3% (95% CI, 70.4%- 84.2%), respectively. Univariate analysis shows that non-isolated ARM, VACTREL association, and severe CHD were associated with mortality. However, only severe CHD is the independent factor associated with mortality, with a hazard ratio of 4.03 (95% CI: 1.93-8.42). Conclusion: CHD is common among ARM patients, and one in five ARM patients had a severe cardiac defect, significantly affecting their survival.

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Frequency of congenital cardiac anomalies in patients with anorectal malformations.

Cited by 2 publications

References 12 publications

Survival and factors associated with mortality among infants with anorectal malformation: a population-based study from a middle-income country

Survival and factors associated with mortality among infants with anorectal malformation: a population-based study from a middle-income country

Survival and Factors Associated with Mortality Among Infants with Anorectal Malformation: A Population- Based Study from A Middle-Income Country

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