Hearts with isomerism of the right atrial appendages – one of the worst forms of disease in 2005

Freedom, Robert M.; Jaeggi, Edgar; Lim, Jessie Mei; Anderson, Robert H.

doi:10.1017/s1047951105001708

Cited by 47 publications

(24 citation statements)

References 144 publications

(207 reference statements)

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“…2,11,15 Even excluding patients who did not require cardiac surgery, there was a higher proportion with a biventricular circulation in PSP compared with ASP group (44% vs 8%). In the PSP group, the survival with a biventricular circulation was significantly higher than previously reported.…”

Section: Discussionmentioning

confidence: 97%

“…At the more severe end of the spectrum are patients with ASP, who commonly have univentricular-type heart defects with complete AV canal, pulmonary stenosis or atresia, and TAPVC. 2 In the middle are a series of defects, alone or in combination, which range from ventricular septal defects to AV canal and conotruncal anomalies with varying degrees of severity. 21 Importantly, atrial bradycardia or AV block are common in PSP patients and likely contribute to fetal death and overall morbidity.…”

Section: Discussionmentioning

confidence: 99%

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Perinatal and Infant Outcomes of Prenatal Diagnosis of Heterotaxy Syndrome (Asplenia and Polysplenia)

Escobar‐Diaz

Friedman

Salem

et al. 2014

The American Journal of Cardiology

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Patients with heterotaxy syndrome (HS) have a range of anomalies and outcomes. There are limited data on perinatal outcomes after prenatal diagnosis. To determine the factors influencing perinatal and infant outcomes, we analyzed prenatal and postnatal variables in fetuses with HS from 1995 to 2011. Of 154 fetuses with HS, 61 (40%) had asplenia syndrome (ASP) and 93 (60%) had polysplenia syndrome (PSP). In the ASP group, 22 (36%) patients were elected for termination of pregnancy, 4 (10%) had fetal death, and 35 of 39 (90%) continued pregnancies were live born. In the PSP group, 12 (13%) patients were elected for termination of pregnancy, 5 (6%) had fetal death (4 with bradyarrhythmia), and 76 of 81 (94%) continued pregnancies were live born. Bradyarrhythmia was the only predictor of fetal death. In the live-born ASP group, 43% (15 of 35) died, 7 because of pulmonary vein stenosis, 4 postoperatively, and 4 because of noncardiac causes. In the live-born PSP group, 13% (10 of 76) died, 5 postoperatively, 2 from bradyarrhythmia, 1 from a cardiac event, and 2 from noncardiac causes. Pulmonary vein stenosis and noncardiac anomalies were independent risk factors for postnatal death. Only 8% of ASP patients achieved biventricular circulation, compared with 65% of PSP patients. In the live-born cohort, the 5-year survival rate was 53% for ASP and 86% for PSP. In conclusion, most PSP patients are currently alive with biventricular circulation in contrast with few ASP patients. Bradyarrhythmia was the only predictor of fetal death. Pulmonary vein stenosis and noncardiac anomalies were predictors of postnatal death.

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Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

Perinatal and Infant Outcomes of Prenatal Diagnosis of Heterotaxy Syndrome (Asplenia and Polysplenia)

Escobar‐Diaz

Friedman

Salem

et al. 2014

The American Journal of Cardiology

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“…Patients with RAI show a higher mortality rate because of more complex cardiac malformations and asplenia, which increases the risks of dying from sepsis [24,25]. We reviewed five literatures reporting significant numbers of prenatally diagnosed both atrial isomerism cases and their postnatal outcomes (Table 3).…”

Section: Discussionmentioning

confidence: 99%

Prenatal diagnosis of atrial isomerism in the Korean population

et al. 2014

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ObjectiveTo report our experiences in the prenatal diagnosis of atrial isomerism and postnatal outcomes.MethodsA total of 80 fetuses prenatally diagnosed with atrial isomerism were retrospectively analyzed between 1999 and 2011 at a single institution.ResultsOf 43 fetuses with prenatally diagnosed right atrial isomerism (RAI), 40 cases were analyzed. The diagnostic accuracy was 93%. The main intracardiac anomalies in RAI were atrioventricular septal defect (AVSD), abnormal pulmonary venous connection, bilateral superior vena cava (BSVC), and pulmonary atresia. Among 28 live births, three infants were lost to follow up, and the overall survival rate was 60%. Of 37 fetuses with prenatally diagnosed left atrial isomerism (LAI), 35 were evaluated. The diagnostic accuracy was 97%. The main intracardiac anomalies in LAI were ventricular septal defect, BSVC, AVSD, double outlet right ventricle, and bradyarrhythmia. Among seven patients with bradyarrhythmia, only one showed a complete atrioventricular block. All fetuses had an interrupted inferior vena cava with azygous continuation. The overall survival rate was 90%.ConclusionOur study confirms the previous findings of fetal atrial isomerism. We also demonstrates a much lower prevalence of AVSD and complete heart block in LAI and a better survival rate in RAI. Although the postnatal outcomes for RAI were worse than those for LAI, successful postnatal surgery with active management improved the survival rate.

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“…68, 69 Factors that deteriorate the prognosis of heterotaxy patients have been described as complications with pulmonary venous obstruction, pulmonary arterial distortion, regurgitation of the atrioventricular valve, elevated pulmonary vascular resistance, and impaired ventricular function. 70 …”

Section: Clinical Manifestation Of the Heterotaxy Syndromementioning

confidence: 99%

Human Heterotaxy Syndrome

Shiraishi

Ichikawa

2012

Circ J

118

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Hearts with isomerism of the right atrial appendages – one of the worst forms of disease in 2005

Abstract: The encyclopaedic nature of this review is a testament to his multiple contributions, which did so much to clarify our understanding of isomerism.

Cited by 47 publications

References 144 publications

Perinatal and Infant Outcomes of Prenatal Diagnosis of Heterotaxy Syndrome (Asplenia and Polysplenia)

Perinatal and Infant Outcomes of Prenatal Diagnosis of Heterotaxy Syndrome (Asplenia and Polysplenia)

Prenatal diagnosis of atrial isomerism in the Korean population

Human Heterotaxy Syndrome

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