Dextrocardia with Situs Inversus Associated with Non-Compaction Cardiomyopathy

Gonçalves, Luiz Flávio Galvão; Souto, Fernanda Maria Silveira; Faro, Fernanda Nascimento; Mendonça, Rodrigo De Castro; Oliveira, Joselina Luzia Menezes; Sousa, Antônio Carlos Sobral

doi:10.5935/abc.20130158

Cited by 10 publications

(4 citation statements)

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“…From the clinical perspective, heterotaxy syndrome has been associated with increased postoperative mortality and morbidity in patients undergoing cardiac surgical procedures [20,24,29,33]. Single cases have documented co-occurrence of LVNC and heterotaxy syndrome, but no data exist on the prevalence of this association [34]. In this study, we sought to determine whether a nonrandom association of LVNC and heterotaxy exists by evaluating the prevalence of LVNC in patients with heterotaxy and CHD.…”

Section: Introductionmentioning

confidence: 99%

Noncompaction cardiomyopathy and heterotaxy syndrome

Martinez

Ware

Schamberger

et al. 2017

Progress in Pediatric Cardiology

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Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by compact and trabecular layers of the left ventricular myocardium. This cardiomyopathy may occur with congenital heart disease (CHD). Single cases document co-occurrence of LVNC and heterotaxy, but no data exist regarding the prevalence of this association. This study sought to determine whether a non-random association of LVNC and heterotaxy exists by evaluating the prevalence of LVNC in patients with heterotaxy. In a retrospective review of the Indiana Network for Patient Care, we identified 172 patients with heterotaxy (69 male, 103 female). Echocardiography and cardiac magnetic resonance imaging results were independently reviewed by two cardiologists to ensure reproducibility of LVNC. A total of 13/172 (7.5%) patients met imaging criteria for LVNC. The CHD identified in this subgroup included atrioventricular septal defects [11], dextrocardia [10], systemic and pulmonary venous return abnormalities [7], and transposition of the great arteries [5]. From this subgroup, 61% (n = 8) of the patients developed arrhythmias; and 61% (n = 8) required medical management for chronic heart failure. This study indicates that LVNC has increased prevalence among patients with heterotaxy when compared to the general population (0.014–1.3%) suggesting possible common genetic mechanisms. Interestingly, mice with a loss of function of Scrib or Vangl2 genes showed abnormal compaction of the ventricles, anomalies in cardiac looping, and septation defects in previous studies. Recognition of the association between LVNC and heterotaxy is important for various reasons. First, the increased risk of arrhythmias demonstrated in our population. Secondly, theoretical risk of thromboembolic events remains in any LVNC population. Finally, many patients with heterotaxy undergo cardiac surgery (corrective and palliative) and when this is associated with LVNC, patients should be presumed to incur a higher peri-operative morbidity based on previous studies. Further research will continue to determine long-term and to corroborate genetic pathways.

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Section: Introductionmentioning

confidence: 99%

Noncompaction cardiomyopathy and heterotaxy syndrome

Martinez

Ware

Schamberger

et al. 2017

Progress in Pediatric Cardiology

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“…With interest we read the article by Gonçalves et al 1 about a patient with dextrocardia and left-ventricular hypertrabeculation/noncompaction (LVHT) 1 . We have the following comments and concerns.…”

Section: Dear Editormentioning

confidence: 99%

Dextroversion and Noncompaction

Finsterer¹,

Stöllberger²

2014

Arquivos Brasileiros De Cardiologia

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“…A Dextrocardia é uma malformação embriológica rara caracterizada por uma anomalia posicional do coração, em que ele está localizado no hemitórax direito com sua base e ápice orientados para a direita e inferiormente (GONÇALVES et al, 2013). Pode ser associada a situs solitus ou situs inversus totalis.…”

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