Improving results of the modified Fontan operation in patients with heterotaxy syndrome

Stamm, Christof; Friehs, Ingeborg; Duebener, Lennart F.; Zurakowski, David; Mayer, John E.; Jonas, Richard A.; Nido, Pedro J. del

doi:10.1016/s0003-4975(02)04124-3

Cited by 113 publications

(81 citation statements)

References 26 publications

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“…AVVR grade was based on the ratio of the color Doppler jet to the area of the atrium [8]. PVO was diagnosed if the pulsed-wave pulmonary venous Doppler pattern showed nonphasic flow or velocities >2 m/s, and we confirmed that the pulmonary vein diameter was reduced intraoperatively [9,10].…”

Section: Methodsmentioning

confidence: 91%

Early- and Middle-Term Surgical Outcomes in Patients with Heterotaxy Syndrome

Chen

Ma²,

Cui³

et al. 2015

Cardiology

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Objectives: Heterotaxy syndrome is a recognized risk factor for surgical cardiac interventions. We evaluated the early- and middle-term results of a surgical intervention for patients with heterotaxy syndrome. Methods: A total of 42 patients with heterotaxy syndrome were enrolled (September 2008 to March 2015). Left and right atrial isomerism were identified in 26% (11 out of 42) and 74% of patients (31 out of 42), respectively. The median age of the patients at the time of surgery was 6.8 months (range: 5 days to 22.3 years). Biventricular repair was completed in 3 patients with left atrial isomerism. Seventeen out of 39 patients who were scheduled for single ventricular repair completed a modified Fontan procedure. Results: The hospital mortality rate was 4.7% (2 out of 42). Another 5 deaths occurred in the remaining survivors following hospital discharge with a follow-up duration of 45.8 ± 23.6 months (range: 13-111 months). The 1-year and 5-year survival rates were 88.1% (37/42) and 83.3% (35/42), respectively. Univariate analysis and multivariate analysis identified pulmonary venous obstruction and atrioventricular valve replacement as additional risk factors for mortality. Conclusions: Right ventricular bypass surgery remains the preferred palliative procedure for patients with heterotaxy syndrome. Based on the current results, the early- and middle-term outcomes are satisfactory.

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Section: Methodsmentioning

confidence: 91%

Early- and Middle-Term Surgical Outcomes in Patients with Heterotaxy Syndrome

Chen

Ma²,

Cui³

et al. 2015

Cardiology

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“…These findings have potential relevance to human heterotaxy and suggest that polymorphisms or rare variants in genes that result in a net decrease in Nodal signal transduction may predispose to cardiovascular malformations related to aberrant LR patterning. In addition, single ventricle cardiac malformations, severe defects in humans associated with high morbidity and mortality (Laussen, 2001;Stamm et al, 2002), may constitute a subset of heterotaxy spectrum malformations caused by abnormal looping.…”

Section: ϫ/Ymentioning

confidence: 99%

Heart defects in X-linked heterotaxy: Evidence for a genetic interaction of Zic3 with the nodal signaling pathway

2006

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The complex cardiac defects that occur in heterotaxy result from abnormal left-right patterning. Mutations in the zinc finger transcription factor ZIC3 cause X-linked heterotaxy, HTX1. We previously have generated a targeted deletion of the murine Zic3 locus and demonstrated that these knockout mice correctly model HTX1. Fifty percent of Zic3 null embryos have cardiac looping anomalies at embryonic day 10.5 to 14.5, with ventral looping and sinistral looping as the predominant phenotypes. The penetrance of these phenotypes is increased in mice that are also haploinsufficient for Nodal. Zic3 ϩ/Ϫ ; Nodal ϩ/Ϫ compound heterozygous mice are born in significantly reduced numbers (P ‫؍‬ 0.0001), indicating a genetic interaction between the loci. Furthermore, an upstream Nodal enhancer is responsive to Zic3 in both Xenopus and mouse. These studies provide evidence that Zic3 interacts genetically with Nodal in left-right patterning and subsequent cardiac development and delineate a critical Zic3-responsive enhancer required for mediating Nodal expression at the node.

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“…Although antenatal diagnosis often represents a more severe spectrum of cardiovascular disease, 14 fetal diagnosis may permit more options, including termination of pregnancy or more tailored neonatal treatment. [15][16][17][18][19][20] We sought to assess the impact of prenatal diagnosis on management and outcome in a large cohort of cases with a diagnosis of either LAI or RAI.…”

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confidence: 99%