Right and Left Isomerism: The Cardiac Surgeon's View

Marcelletti, Carlo; Donato, Roberto Di; Nijveld, A.; Squitieri, C.; Bulterijs, A.H.; Naeff, M.S.J.; Schuller, J.; Becker, Anton E.

doi:10.1016/s0003-4975(10)61592-5

Cited by 43 publications

(26 citation statements)

References 24 publications

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“…Methods for repairing or palliating complex heart defects associated with left isomerism developed gradually during the study period [3,[9][10][11]]. In the current study, the survival rate did not differ significantly between the single-ventricular repair (73%) and biventricular repair (75%) groups (p = 0.93).…”

Section: Discussionmentioning

confidence: 51%

Outcome of Left Atrial Isomerism at a Single Institution

et al. 2012

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Left atrial isomerism includes a complex spectrum of cardiac and extracardiac anomalies. The records of all patients with left isomerism born during the period of 1973-2010 and treated at the Children's Hospital, Helsinki were reviewed. The short- and long-term outcomes were studied. The review included 38 patients (50% females). The overall survival with left atrial isomerism was 63% during a median follow-up time of 16 years (range, 4-30 years). Extracardiac anomalies were noted in 14 (37%) of 38 cases. Cardiac defects included dextrocardia in 26%, partially or totally anomalous pulmonary venous return in 29%, common atrium in 50%, atrioventriculoseptal defect in 73%, single ventricle in 40%, ventriculoseptal defect without atrioventricular defect in 11%, transposition in 21%, double outlet of the right ventricle in 26%, pulmonary stenosis or atresia in 61%, and left ventricular outflow obstruction in 24% of the cases. Cardiac arrhythmias were presented in 71% and pacemaker treatment in 29% of the cases. Of the 38 patients, 33 had cardiac surgery. Simple palliative methods were used in 11 cases, single-ventricle palliation in 12 cases, and operation with a biventricular track in 10 cases. In the groups that had surgery, 3 of 11 patients, 3 of 12 patients, and 3 of 10 patients died, respectively. In this review, 14 deaths occurred, associated with extracardiac anomalies in five cases and with cardiac arrhythmia in four cases. Five postoperative deaths occurred. At this writing, all three patients who had heart transplantation are alive. Complicated heart defects associated with severe arrhythmias and extracardiac anomalies contribute to a high mortality rate with left isomerism. Cardiac transplantation was considered a good option for selected patients.

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Section: Discussionmentioning

confidence: 51%

Outcome of Left Atrial Isomerism at a Single Institution

et al. 2012

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“…Çalışmamızda aortopulmoner şant cerrahi mortalitesi %20.8 olarak saptandı, bu değer literatürde bu hastalara yapılan şantların sonuçları ile uyumlu idi. [22,23] Literatürde Kawashima ameliyatının cerrahi mortalitesi %0-33 aralığındaydı. [24,25] Bizim çalışmamızda Kawashima ameliyatından sonra erken dönemde bir olgu kaybedildi.…”

Section: Discussionunclassified

A retrospective analysis of cases with left atrial isomerism

Erdem¹

2017

Turk Gogus Kalp Dama

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Bul gu lar:Ortalama takip süresi 108±49.5 (dağılım 12-173) ay idi. Yetmiş iki hastanın dördüne cerrahi gerekmez iken, iki hastaya kalıcı kalp pili takıldı. Cerrahi gereken 68 hastanın 17'si iki ventrikül tamiri adayı iken, geriye kalan 51 hasta tek ventrikül tamiri adayıydı. Yirmi dokuz hastaya Kawashima işlemi uygulandı. Bu hastaların yedisinde pulmoner antegrad akım açık bırakıldı. Dört hastada hepatik venler aynı seansta Fontan dolaşımına dahil edildi. Altı hastada takip sırasında ilerleyici siyanoz nedeniyle hepatik venler, ekstrakardiyak kondüit yardımıyla, pulmoner artere yönlendirildi. Cerrahi sonrasında üç hasta kaybedildi. Hepatik venlerin aynı seansta ya da ikinci bir seansta Fontan dolaşımına dahil edildiği hastalar veya antegrad pulmoner akımın açık bırakıldığı hastalarda mortalite veya ilerleyici siyanoz gözlenmedi.So nuç: İki ventrikül fizyolojisine sahip hastaların genel prognozu mükemmeldir. Ancak, Kawashima işlemi yapılan hastalarda doku oksijenizasyonu düşme eğilimindedir. Bu nedenle, Kawashima işlemi yapılan hastalarda, aynı seansta hepatik venlerin dolaşıma dahil edilmesi veya uygun olgularda antegrad akımın açık bırakılması güvenli bir yaklaşım olarak görünmektedir.Anah tarsöz cük ler:Kesintili inferior vena cava; isomerizm; Kawashima. ABSTRACT Background:In this study, we aimed to evaluate accompanying cardiac pathologies in patients with left atrial isomerism and report the follow-up results.

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“…An atrioventricular septal defect is normally present in virtually all, and generally there is either a single ventricle or double-outlet right ventricle with noncommitted ventricular septal defect in most patients with situs ambiguous. Biventricular repair is possible when the two balanced ventricles are present, and the anatomic relation between the ventricles and the AV valves is favorable (5,25,26). Isomerism remains a challenge because of the intracardiac or extracardiac baffling required to coalesce and redirect systemic and pulmonary venous return within the heart (27).…”

Section: Y K Hong Et Al 680mentioning

confidence: 99%

Efficacy of MRI in Complicated Congenital Heart Disease with Visceral Heterotaxy Syndrome

Hong

Park

Ryu

et al. 2000

Journal of Computer Assisted Tomography

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Purpose:The authors' goal was to assess the diagnostic accuracy and clinical effect of MRI compared with echocardiography and catheterization in the evaluation of cardiac defects with situs ambiguous.Method: Twenty-two patients with visceral heterotaxy syndrome were included. Results: Because situs determined by the relation between the pulmonary artery and bronchi showed most predominantly a tendency toward lateralization, this was regarded as the standard reference of situs determination. For the purpose of this study, patients were classified as having right isomerism (n ‫ס‬ 13) or left isomerism groups (n ‫ס‬ 9). MRI has several advantages compared with echocardiography or cardiac angiography for examining patients with situs ambiguous. (1) The bronchial, pulmonary arterial, and splenic situs can be readily determined, and discrepancies (n ‫ס‬ 2) can be assessed easily. (2) Venoatrial connections are adequately imaged. In particular, all types of total and partial anomalous pulmonary venous return are delineated, regardless of whether restrictions of pulmonary blood flow or pulmonary venous obstructions are involved (n ‫ס‬ 4). The courses of vertical veins were easily identified, and the prearterial position was revealed in only one of seven right isomerisms with total anomalous pulmonary venous return. The drain pattern of the hepatic vein can be visualized using three-dimensional spatial information and is useful for total cavopulmonary connection design. (3) Associated complicated cardiac anomalies, particularly the size or peripheral stenosis of the pulmonary arteries, may be evaluated, and this information is useful for palliative shunt operations.Conclusion: Because of its wide field of view and imaging, which is not restricted by associated anomalies, a thorough understanding of the cardiovascular anatomy of the situs ambiguous can be achieved using MRI, which is of considerable value in the surgical correction of this complicated anomaly. MRI can obviate or facilitate catheterization in these critically ill patients.

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Right and Left Isomerism: The Cardiac Surgeon's View

Cited by 43 publications

References 24 publications

Outcome of Left Atrial Isomerism at a Single Institution

Outcome of Left Atrial Isomerism at a Single Institution

A retrospective analysis of cases with left atrial isomerism

Efficacy of MRI in Complicated Congenital Heart Disease with Visceral Heterotaxy Syndrome

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