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Congenital cardiovascular defects in children with intestinal malrotation
M Kouwenberg1, R S V M Severijnen, L Kapusta
1Department of Paediatrics, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands. m.kouwenberg@cukz.umcn.nl
Insights
Congenital cardiovascular defects (CCVD) are common in intestinal malrotation (IM) patients, increasing post-operative risks. Early detection and specialized care are crucial for managing these complex cases.
Area of Science:
- Pediatric Surgery
- Congenital Defects
- Cardiology
Background:
- Intestinal malrotation (IM) and congenital cardiovascular defects (CCVD) are frequent co-occurring congenital anomalies.
- Understanding the interplay between IM and CCVD is vital for patient outcomes.
Purpose of the Study:
- To determine the prevalence and types of CCVD in IM patients.
- To analyze the association between CCVD and post-operative morbidity and mortality following IM surgery.
Main Methods:
- Retrospective review of IM patient records (1980-2005).
- Analysis of CCVD types, associated anomalies, and post-operative outcomes.
- Statistical analysis to identify risk factors for morbidity and mortality.
Main Results:
- 27.1% of IM patients had major or minor CCVD.
- Major CCVD was associated with higher rates of syndromes and other anomalies.
- Pre-existing CCVD signs significantly increased post-operative morbidity (OR 4.0).
- Mortality risk was elevated by intestinal ischemia, post-operative complications, and major CCVD.
Conclusions:
- Congenital cardiovascular defects are prevalent in IM patients and linked to significant post-operative morbidity and mortality.
- IM surgery in patients with CCVD necessitates specialized pediatric cardiac care.
- Further research is needed on the benefits of laparoscopic approaches in this population.
Abstract:
Intestinal malrotation (IM) and cardiovascular defects (CCVD) are both common congenital defects. We investigated the prevalence and types of CCVD in a 25-year IM population, and its association with post-IM-operative morbidity and mortality. Data on the type of CCVD, other congenital defects, syndromes, associations, post-IM-operative morbidity and mortality were retrospectively reviewed from the records of IM patients born between 1980 and 2005. Data were analyzed on (significant) differences between CCVD subgroups, and risk factors for both morbidity and mortality were calculated. Seventy-seven of 284 IM patients (27.1%) were diagnosed with a major or minor CCVD (37 and 40 patients, respectively). Syndromes and associations were more frequently diagnosed in patients with major than with a minor CCVD (67.6 vs. 40%, respectively). Post-IM-operative complications, although frequently observed (61%), did not differ between patients with major and minor CCVD. Physical CCVD signs before IM surgery increased post-IM-operative morbidity significantly (OR 4.0, 95% CI 1.4-11.0). Fifteen patients died (19.5%), seven due to cardiovascular cause. Mortality risk was increased by intestinal ischemia and post-IM-operative complications and by major CCVD after correction for age at weight at the time of IM operation. Congenital cardiovascular defects in children with intestinal malrotation are common, with high morbidity and mortality rates after IM operation. Elective IM surgery in young patients with CCVD should be performed in a centre with adequate paediatric cardiac care. Benefits of laparoscopic intervention need further study.
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