Anorectal malformations (ARM) and VACTERL association and severity of congenital heart diseases (CHD): Experience of 396 consecutive patients in a tertiary center
Objective: Congenital heart diseases (CHD) are the most frequently associated anomalies with anorectal malformations (ARM). Nevertheless, many specific aspects of CHD in ARM patients have yet to be studied. The aims of this study were to evaluate the prevalence and distribution of CHD in neonates-in...
Saved in:
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Elsevier
2024-07-01
|
| Series: | Pediatrics and Neonatology |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S1875957223002243 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1849768659994542080 |
|---|---|
| author | Patrizio Moras Angelo Zarfati Pietro Bagolan Andrea Conforti Alessandra Toscano Barbara Daniela Iacobelli |
| author_facet | Patrizio Moras Angelo Zarfati Pietro Bagolan Andrea Conforti Alessandra Toscano Barbara Daniela Iacobelli |
| author_sort | Patrizio Moras |
| collection | DOAJ |
| description | Objective: Congenital heart diseases (CHD) are the most frequently associated anomalies with anorectal malformations (ARM). Nevertheless, many specific aspects of CHD in ARM patients have yet to be studied. The aims of this study were to evaluate the prevalence and distribution of CHD in neonates-infants with ARM, and to explore whether the severity of ARM, and the presence of VACTERL association, had an impact on CHD rate, severity, and timing at first cardiac surgery. Study design: All consecutive newborn-infants with ARM managed in our tertiary center (January-1999; December-2021) were collected from a prospective database and retrospectively analyzed. Prevalence and distribution of CHD in ARM patients were assessed. Patients were divided into groups depending on ARM severity and presence of VACTERL association. Pairwise comparison for CHD prevalence, severity, and timing at first cardiac surgery was performed between groups. Results: Of 396 ARM patients identified, those with severe ARM showed a higher number of overall CHD compared to patients with non-severe ARM (36.7 % vs. 25.2 %, p = 0.032). VACTERL + patients had a significantly higher prevalence of CHD (73.4 % vs. 16.4 %; p <0.001) and major CHD (51.7 % vs. 26.9 %; p = 0.008) when compared with VACTERL-patients. Furthermore, VACTERL + patients underwent first cardiac surgery at a significantly younger age than VACTERL-patients (5.2 ± 15.2 months vs. 11.9 ± 6.3, p = 0.039). Conclusions: Patients with severe ARM had a higher number of CHD compared to patients with non-severe ARM. VATERL + patients had significantly more CHD and more severe CHD than VACTERL-patients. Early screening for CHD is strongly recommended in all newborns diagnosed with ARM before surgery. Level of evidence: III retrospective comparative study. |
| format | Article |
| id | doaj-art-6cc5c1ccbaf1421ebd23d1f64cf675c8 |
| institution | DOAJ |
| issn | 1875-9572 |
| language | English |
| publishDate | 2024-07-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Pediatrics and Neonatology |
| spelling | doaj-art-6cc5c1ccbaf1421ebd23d1f64cf675c82025-08-20T03:03:42ZengElsevierPediatrics and Neonatology1875-95722024-07-0165438138510.1016/j.pedneo.2023.08.011Anorectal malformations (ARM) and VACTERL association and severity of congenital heart diseases (CHD): Experience of 396 consecutive patients in a tertiary centerPatrizio Moras0Angelo Zarfati1Pietro Bagolan2Andrea Conforti3Alessandra Toscano4Barbara Daniela Iacobelli5Perinatal Cardiology Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, ItalyNeonatal Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy; Department of Systems Medicine, University of Rome “Tor Vergata”, Rome, Italy; Corresponding author. Piazza Sant’Onofrio, 4 – 00165, Rome, Italy.Neonatal Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy; Department of Systems Medicine, University of Rome “Tor Vergata”, Rome, ItalyNeonatal Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, ItalyPerinatal Cardiology Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, ItalyNeonatal Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, ItalyObjective: Congenital heart diseases (CHD) are the most frequently associated anomalies with anorectal malformations (ARM). Nevertheless, many specific aspects of CHD in ARM patients have yet to be studied. The aims of this study were to evaluate the prevalence and distribution of CHD in neonates-infants with ARM, and to explore whether the severity of ARM, and the presence of VACTERL association, had an impact on CHD rate, severity, and timing at first cardiac surgery. Study design: All consecutive newborn-infants with ARM managed in our tertiary center (January-1999; December-2021) were collected from a prospective database and retrospectively analyzed. Prevalence and distribution of CHD in ARM patients were assessed. Patients were divided into groups depending on ARM severity and presence of VACTERL association. Pairwise comparison for CHD prevalence, severity, and timing at first cardiac surgery was performed between groups. Results: Of 396 ARM patients identified, those with severe ARM showed a higher number of overall CHD compared to patients with non-severe ARM (36.7 % vs. 25.2 %, p = 0.032). VACTERL + patients had a significantly higher prevalence of CHD (73.4 % vs. 16.4 %; p <0.001) and major CHD (51.7 % vs. 26.9 %; p = 0.008) when compared with VACTERL-patients. Furthermore, VACTERL + patients underwent first cardiac surgery at a significantly younger age than VACTERL-patients (5.2 ± 15.2 months vs. 11.9 ± 6.3, p = 0.039). Conclusions: Patients with severe ARM had a higher number of CHD compared to patients with non-severe ARM. VATERL + patients had significantly more CHD and more severe CHD than VACTERL-patients. Early screening for CHD is strongly recommended in all newborns diagnosed with ARM before surgery. Level of evidence: III retrospective comparative study.http://www.sciencedirect.com/science/article/pii/S1875957223002243Anorectal malformationsARMVACTERLVACTERL associationCongenital heart diseaseCHD |
| spellingShingle | Patrizio Moras Angelo Zarfati Pietro Bagolan Andrea Conforti Alessandra Toscano Barbara Daniela Iacobelli Anorectal malformations (ARM) and VACTERL association and severity of congenital heart diseases (CHD): Experience of 396 consecutive patients in a tertiary center Pediatrics and Neonatology Anorectal malformations ARM VACTERL VACTERL association Congenital heart disease CHD |
| title | Anorectal malformations (ARM) and VACTERL association and severity of congenital heart diseases (CHD): Experience of 396 consecutive patients in a tertiary center |
| title_full | Anorectal malformations (ARM) and VACTERL association and severity of congenital heart diseases (CHD): Experience of 396 consecutive patients in a tertiary center |
| title_fullStr | Anorectal malformations (ARM) and VACTERL association and severity of congenital heart diseases (CHD): Experience of 396 consecutive patients in a tertiary center |
| title_full_unstemmed | Anorectal malformations (ARM) and VACTERL association and severity of congenital heart diseases (CHD): Experience of 396 consecutive patients in a tertiary center |
| title_short | Anorectal malformations (ARM) and VACTERL association and severity of congenital heart diseases (CHD): Experience of 396 consecutive patients in a tertiary center |
| title_sort | anorectal malformations arm and vacterl association and severity of congenital heart diseases chd experience of 396 consecutive patients in a tertiary center |
| topic | Anorectal malformations ARM VACTERL VACTERL association Congenital heart disease CHD |
| url | http://www.sciencedirect.com/science/article/pii/S1875957223002243 |
| work_keys_str_mv | AT patriziomoras anorectalmalformationsarmandvacterlassociationandseverityofcongenitalheartdiseaseschdexperienceof396consecutivepatientsinatertiarycenter AT angelozarfati anorectalmalformationsarmandvacterlassociationandseverityofcongenitalheartdiseaseschdexperienceof396consecutivepatientsinatertiarycenter AT pietrobagolan anorectalmalformationsarmandvacterlassociationandseverityofcongenitalheartdiseaseschdexperienceof396consecutivepatientsinatertiarycenter AT andreaconforti anorectalmalformationsarmandvacterlassociationandseverityofcongenitalheartdiseaseschdexperienceof396consecutivepatientsinatertiarycenter AT alessandratoscano anorectalmalformationsarmandvacterlassociationandseverityofcongenitalheartdiseaseschdexperienceof396consecutivepatientsinatertiarycenter AT barbaradanielaiacobelli anorectalmalformationsarmandvacterlassociationandseverityofcongenitalheartdiseaseschdexperienceof396consecutivepatientsinatertiarycenter |