Interrupted aortic arch (IAA) is a rare condition in infants that occurs approximately three times per million births. It is considered incompatible with life after the ductus arteriosus closes if it is not surgically corrected. A review found 37 reported cases not identified until the patient was over the age of 18, and analysis of these cases and of our own case is reported.
Children with functional single ventricle heart disease are commonly palliated down a staged clinical pathway toward a Fontan completion procedure (total cavopulmonary connection). The Fontan physiology is fraught with long term complications associated with lower body systemic venous hypertension, eventually resulting in significant morbidity and mortality. The bidirectional Glenn shunt or superior cavopulmonary connection (SCPC) is commonly the transitional stage in single ventricle surgical management and provides excellent palliation. Some studies have demonstrated lower morbidity and mortality with the SCPC when compared with the Fontan. Unfortunately the durability of the SCPC is significantly limited by the development of pulmonary arteriovenous malformations (PAVMs) which have been commonly attributed to the absence of hepatic venous blood flow and the lack of pulsatile flow to the affected lungs. Abnormal angiogenesis has been suggested as a final common pathway to PAVM development. Understanding these fundamental mechanisms through the investigation of angiogenic pathways associated with the pathogenesis of PAVMs would help to develop medical therapies that could prevent or reverse this complication following SCPC. Such therapies could improve the longevity of the SCPC, potentially eliminate or significantly postpone the Fontan completion with its associated complications, and improve long-term survival in children with single ventricle disease.
The potential for necrotizing enterocolitis (NEC) in neonates undergoing cardiac surgery has contributed largely to wide feeding practice variations and a hesitation to initiate enteral feeding during the preoperative period, specifically those patients with hypoplastic left heart syndrome.
A retrospective chart review of neonates undergoing cardiac surgery at a single institution between July 2011 and July 2013 was performed. The primary objective of this study was to determine if preoperative feeding was associated with necrotizing enterocolitis (NEC) in neonates requiring cardiac surgery. Univariable and multivariable analyses were performed to evaluate the relationship between preoperative feeding and NEC. Secondary outcomes including growth failure, total ventilator days and total length of stay were analyzed.
One hundred thirty consecutive neonates who required cardiac surgery were included in the analysis. Preoperative feeding occurred in 61% (n=79). The overall prevalence of NEC was 9% (12/130), including 3 neonates with surgical NEC. There was no difference in the prevalence of NEC between the preoperative feeding and nil per os (NPO) groups. NPO preoperatively was associated with longer ventilator dependent days (p=0.01) but was not associated with worsened growth failure or longer length of stay.
In this study cohort, preoperative feeding was not associated with NEC. Larger prospective studies evaluating the safety and benefits of preoperative feeding in cardiac neonates are warranted.
Background: Neurodevelopmental disability is the most significant complication for survivors of infant surgery for congenital heart disease. In this study we sought to determine if perioperative circulating brain injury biomarker levels are associated with neurodevelopmental outcomes at 12 months.From the
Smart materials have intrinsic properties that change in a controlled fashion in response to external stimuli. Currently, the only smart materials with a significant clinical impact in cardiovascular implant design are shape memory alloys, particularly Nitinol. Recent prodigious progress in material science has resulted in the development of sophisticated shape memory polymers. In this article, we have reviewed the literature and outline the characteristics, advantages, and disadvantages of shape memory alloys and shape memory polymers which are relevant to clinical cardiovascular applications, and describe the potential of these smart materials for applications in coronary stents and transcatheter valves.
Traditionally, karyotype and fluorescence in situ hybridization (FISH) were
used for cytogenetic testing of infants with congenital heart disease who underwent
cardiac surgery at our institution. Recently, chromosome microarray analysis (CMA) has
been performed in lieu of the traditional tests. A standardized approach to cytogenetic
testing does not exist in this population. The purpose of this study was to assess the
utility of CMA based on our current ordering practice.
We reviewed the records of all infants (< 1 year old) who underwent
cardiac surgery at our institution from January 2010 to June 2013. Data included results
of all cytogenetic testing performed. Diagnostic yield was calculated as the percentage
of significant abnormal results obtained by each test modality. Patients were grouped by
classification of congenital heart disease (CHD).
Two hundred and seventy-five (51%) of 535 infants who underwent cardiac
surgery had cytogenetic testing. Of those tested, 154 (56%) had multiple tests
performed and at least 18% were redundant or overlapping. The utilization of CMA
has increased each year since its implementation. The diagnostic yield for karyotype,
FISH and CMA was 10%, 12% and 14% respectively. CMA yield was
significantly higher in patients with septal defects (33%, p = 0.01) compared to
all other CHD classes. CMA detected abnormalities of unknown clinical significance in
13% of infants tested.
In our center, redundant cytogenetic testing is frequently performed in infants
undergoing cardiac surgery. The utilization of chromosome microarray analysis has
increased over time and abnormalities of unknown clinical significance are detected in
an important subset of patients. A screening algorithm that risk-stratifies based on
classification of CHD and clinical suspicion may provide a practical, data-driven
approach to genetic testing in this population and limit unnecessary resource
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