Risk Factors Associated With Mortality and Interventions in 472 Neonates With Interrupted Aortic Arch: A Congenital Heart Surgeons Society Study

McCrindle, B.W.; Tchervenkov, Christo I.; Konstantinov, Igor E.

doi:10.1016/j.accreview.2005.05.079

Cited by 53 publications

(131 citation statements)

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“…The association between TA and IAA, typical of del22q11.2, has been reported as a risk factor for increased mortality in a recent Congenital Heart Surgeons Society (CHSS) study on IAA [McCrindle et al, 2005]. In spite of that, there is no evidence in the literature of increased risk at surgery of patients with TA and del22q11.2, even at long-term follow-up [Anaclerio et al, 2004].…”

Section: Truncus Arteriosusmentioning

confidence: 97%

Cardiac defects and results of cardiac surgery in 22q11.2 deletion syndrome

Carotti

Digilio

Piacentini

et al. 2008

Dev Disabil Res Revs

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Specific types and subtypes of cardiac defects have been described in children with 22q11.2 deletion syndrome as well as in other genetic syndromes. The conotruncal heart defects occurring in patients with 22q11.2 deletion syndrome include tetralogy of Fallot, pulmonary atresia with ventricular septal defect, truncus arteriosus, interrupted aortic arch, isolated anomalies of the aortic arch, and ventricular septal defect. These conotruncal heart defects are frequently associated in this syndrome with additional cardiovascular anomalies of the aortic arch, pulmonary arteries, infundibular septum, and semilunar valves complicating cardiac anatomy and surgical treatment. In this review we describe the surgical anatomy, the operative treatment, and the prognostic results of the cardiac defects associated with 22q11.2 deletion syndrome. According to the current literature, in patients with tetralogy of Fallot with/without pulmonary atresia and truncus arteriosus, in spite of the complex cardiac anatomy, the presence of 22q11.2 deletion syndrome does not worsen the surgical prognosis. On the contrary in children with pulmonary atresia with ventricular septal defect and probably in those with interrupted aortic arch the association with 22q11.2 deletion syndrome is probably a risk factor for the operative treatment. The complex cardiovascular anatomy in association with depressed immunological status, pulmonary vascular reactivity, neonatal hypocalcemia, bronchomalacia and broncospasm, laryngeal web, and tendency to airway bleeding must be considered at the time of diagnosis and surgical procedure. Specific diagnostic, surgical, and perioperative protocols should be applied in order to provide appropriate treatment and to reduce surgical mortality and morbidity.

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Section: Truncus Arteriosusmentioning

confidence: 97%

Cardiac defects and results of cardiac surgery in 22q11.2 deletion syndrome

Carotti

Digilio

Piacentini

et al. 2008

Dev Disabil Res Revs

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“…Several publications have reported on the association of IAA and further abnormalities [4,5,18] . In our series, there were 2 cases in which a malformation syndrome was diagnosed prenatally.…”

Section: Discussionmentioning

confidence: 99%

“…In up to 98%, other cardiovascular anomalies are present, including ventricular septum defects (VSDs) in 90%, atrial septum defects, subaortic stenosis, bicuspid aortic valve, truncus arteriosus communis, aortopulmonary window, transposition of the great arteries, tricuspid atresia and double outlet right ventricle, all with varying incidences according to different studies [4][5][6][7][8][9] . In approximately half of the individuals with IAA, the disorder is associated with chromosome 22q11.2 deletion.…”

Section: Introductionmentioning

confidence: 99%

Fetal and Neonatal Diagnosis of Interrupted Aortic Arch: Associations and Outcomes

et al. 2011

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Objective: Interrupted aortic arch (IAA) is a rare but serious anomaly. Prenatal diagnosis is challenging and published data are limited. The aim of the study was to review the data of fetuses and neonates diagnosed with IAA during a 16-year period at Children’s Hospital Giessen. Methods: Retrospective ascertainment of 8 fetuses and 20 neonates with a confirmed diagnosis of IAA from 1994 to 2010 by reviewing the hospital database of the cardiovascular program of the prenatal and pediatric cardiology clinics at the University Hospital Giessen. Results: Eighteen cases with IAA type B and 10 cases with IAA type A were found. After 2005, prenatal diagnosis was achieved in 8 cases and postnatal imaging confirmed IAA in all 8 neonates. Twenty-nine percent of individuals had a chromosomal anomaly, with microdeletion 22q11.2 being the most common abnormality (n = 6, 21%). In 46% (13/28) other complex cardiac anomalies were present. Mortality after surgery was 18%. Long-term morbidity and mortality was due to neurological impairment in the presence of microdeletion 22q11.2 and the need of surgical or catheter re-intervention. Conclusion: Despite the difficulties and challenges in diagnosis, the prenatal detection rate of IAA is increasing. Associated complex cardiac and chromosomal abnormalities influence the outcome of patients with IAA and are important issues of parental counseling.

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“…The protective effect of the more recent era has been documented in other complex congenital lesions 23 coincident with improvements in patient triage, preoperative and postoperative care, diagnostic modalities, myocardial protection, and operative technique. In addition, our recent experience favored autografts and avoided the use of bioprostheses, which likely contributed to improved outcomes over time.…”

Section: Valve Longevitymentioning

confidence: 99%