Does Down syndrome affect prognosis of surgically managed atrioventricular canal defects?

BACKGROUND: Early surgical management of complete atrioventricular (AV) canal defect is the optimal treatment option. Since the published evidence on outcomes is inconclusive, we retrospectively studied the outcomes of patients in our institution.OBJECTIVE: Study outcomes of complete AV canal repair.DESIGN: Retrospective, descriptive.SETTINGS: Single institute.PATIENTS AND METHODS: Medical records of patients under 2 years of age who underwent complete AV canal repair from January 2004 to December 2014 were retrospectively reviewed.MAIN OUTCOME MEASURES: Pre- and postoperative morbidity and mortality.SAMPLE SIZE: 140 patients.RESULT: The median (IQR) age at the time of surgery was 5.4 (3.9-8.2) months. Down syndrome was diagnosed in 98 (70%) of patients. AV valve regurgitation was found preoperatively in 129 (92%) and postoperatively in 135 (96%) patients. There was a significant association between preoperative pulmonary hypertension and the development of pulmonary hypertension in the postoperative period (P=.04). Thirty-three patients needed reoperation. Arrhythmia was found in 19 patients, 16 of whom required pacemaker insertion. Seven patients died (5%).CONCLUSION: The presence of preoperative and postoperative AV valve regurgitation was common in this cohort but did not significantly affect patient survival. Our findings suggest an acceptable outcome for repair of complete AV septal defect with few complications postoperatively.LIMITATION: Retrospective in single institute.CONFLICT OF INTEREST: None.

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“…Rizzoli concluded that Down syndrome was not an independent risk factor for operative mortality, nor reoperation. 11…”

Section: Discussionmentioning

confidence: 99%

Outcomes of surgical repair of complete atrioventricular canal defect in patients younger than 2 years of age

et al. 2019

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“…Along the same lines, Formigari et al [16] argued a higher prevalence of recurrent/residual subaortic stenosis in non-Down patients, and a lower prevalence of reoperation in patients with Down syndrome. Similarly, Rizzoli et al [17] described that Down syndrome was not an independent risk factor for operative mortality, and that they underwent fewer reoperations. Somewhat contrary to these findings, some have stated that Down syndrome is a potential risk factor for hospital and late death [13,18], as well as for reoperation [13].…”

Section: Down Syndromementioning

confidence: 92%

Outcomes and reoperations after total correction of complete atrio-ventricular septal defect

Dodge‐Khatami¹,

Herger²,

Rousson³

et al. 2008

European Journal of Cardio-Thoracic Surgery

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“…Endocardial cushion defects, patent ductus arteriosus, ventricular septal defect, and tetralogy of Fallot are common. Conduction defects can result from surgical repair of cardiac anomalies (25).…”

Section: Cardiac and Other Defectsmentioning

confidence: 99%

Frequency of anesthesia‐related complications in children with Down syndrome under general anesthesia for noncardiac procedures

Borland

Colligan

Brandom

2004

Pediatric Anesthesia

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Comprehensive reporting is needed to capture all significant adverse events. The incidences of bradycardia on induction, natural airway obstruction, and postintubation (or instrumentation) croup were significantly higher in the DS noncardiac group compared with the remaining population. Current anesthesia techniques and agents must be compared using quantitative QA data to ensure use of the safest options for each patient.

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Does Down syndrome affect prognosis of surgically managed atrioventricular canal defects?

Cited by 42 publications

References 13 publications

Outcomes of surgical repair of complete atrioventricular canal defect in patients younger than 2 years of age

Outcomes of surgical repair of complete atrioventricular canal defect in patients younger than 2 years of age

Outcomes and reoperations after total correction of complete atrio-ventricular septal defect

Frequency of anesthesia‐related complications in children with Down syndrome under general anesthesia for noncardiac procedures

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