A case report of isolated levocardia without intracardiac anomalies associated with sick sinus syndrome.

Fukuzawa, Jun; Haneda, Takashi; Ishii, Yoshinao; Kawashima, Eiji; Ogawa, Yasuhiro; Matsuhashi, Hironobu; Kawamura, Y.; Imamoto, Tetsuro; Tobise, Katsuyuki; Onodera, Sokichi

doi:10.1253/jcj.57.245

Cited by 9 publications

(8 citation statements)

References 9 publications

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“…The association of non-compaction with bronchial/abdominal situs abnormalities has not yet been reported in the literature, although some patients with situs inversus also have hypertrophic cardiomyopathy (Agirbasli et al 2000) or subaortic hypertrophic stenosis (Befeler 1975;Cochran and Wanamaker 1975;Wells and Befeler 1975). The association of situs abnormalities with azygous continuation of the inferior vena cava, and sick sinus syndrome without left atrial isomerism is typical in this family, and has only been reported in a few Japanese patients (Fukuzawa et al 1993;Kakura et al 1998;Noguchi et al 1997). However, cardiomyopathy or noncompaction of the ventricular myocard as present in our family, was not reported in these patients (although one patient had cardiomegaly) (Noguchi et al 1997).…”

Section: Discussionmentioning

confidence: 79%

A new syndrome with noncompaction cardiomyopathy, bradycardia, pulmonary stenosis, atrial septal defect and heterotaxy with suggestive linkage to chromosome 6p

et al. 2007

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We report a three-generation family with nine patients affected by a combination of cardiac abnormalities and left isomerism which, to our knowledge, has not been described before. The cardiac anomalies include non-compaction of the ventricular myocardium, bradycardia, pulmonary valve stenosis, and secundum atrial septal defect. The laterality sequence anomalies include left bronchial isomerism, azygous continuation of the inferior vena cava, polysplenia and intestinal malrotation, all compatible with left isomerism. This new syndrome is inherited in an autosomal dominant pattern. A genome-wide linkage analysis suggested linkage to chromosome 6p24.3-21.2 with a maximum LOD score of 2.7 at marker D6S276. The linkage interval is located between markers D6S470 (telomeric side) and D6S1610 (centromeric side), and overlaps with the linkage interval in another family with heterotaxy reported previously. Taken together, the genomic region could be reduced to 9.4 cM (12 Mb) containing several functional candidate genes for this complex heterotaxy phenotype.

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Section: Discussionmentioning

confidence: 79%

A new syndrome with noncompaction cardiomyopathy, bradycardia, pulmonary stenosis, atrial septal defect and heterotaxy with suggestive linkage to chromosome 6p

et al. 2007

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“…Preoperatively, the girl had episodes of bradycardia with a coronary sinus rhythm, interpreted as sinus node dysfunction (49 beats per minute; confirmed by Holter monitoring [Fukuzawa et al, 1993]). A permanent pacer was needed postoperatively due to sinus node arrest.…”

Section: Heart Defectsmentioning

confidence: 99%

Anomalous inferior and superior venae cavae with oculoauriculovertebral defect: Review of Goldenhar complex and malformations of left-right asymmetry

et al. 1998

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We observed a girl with an interrupted, left inferior vena cava with hemiazygous continuation, bilateral superior venae cavae, heart defects, and sacral agenesis. She had macrostomia and bilateral ear tags and pits, as in oculoauriculovertebral defect. Maternal diabetes was present. The combination, which we call OAV-heterotaxia complex, supports the view that some cases of oculoauriculovertebral defect may be part of a midline field defect of blastogenesis.

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“…Absence of the IVC is frequently associated with other congenital anomalies, including polysplenia, asplenia, situs inversus, and isolated levocardia. Ninety percent of previously reported cases of congenital absence of the IVC consisted of absence of the hepatic (supra-renal) segment of IVC with azygos continuation [8,10,11]. However, the patient from the present case demonstrated absence of the infra-renal segment of IVC, and venous drainage from the lower extremities was achieved via the ascending lumbar vein to the intact hepatic and renal segment of the IVC.…”

Section: Discussionmentioning

confidence: 53%

Multiple and Recurrent Systemic Thrombotic Events Associated with Congenital Anomaly of Inferior Vena Cava

Takehara

Hasebe

Enomoto³

et al. 2005

J Thromb Thrombolysis

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We describe a case of a 67-year-old woman with a history of cerebral infarction and pulmonary embolism that presented with chest pain. Subsequent evaluation resulted in a diagnosis of acute myocardial infarction and occult DVT, and imaging revealed a rare congenital absence of the infra-renal portion of the inferior vena cava, with lower extremity venous drainage diverted via an ascending lumbar vein. Associations between congenital absence of the inferior vena cava and thrombosis are discussed.3

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A case report of isolated levocardia without intracardiac anomalies associated with sick sinus syndrome.

Cited by 9 publications

References 9 publications

A new syndrome with noncompaction cardiomyopathy, bradycardia, pulmonary stenosis, atrial septal defect and heterotaxy with suggestive linkage to chromosome 6p

A new syndrome with noncompaction cardiomyopathy, bradycardia, pulmonary stenosis, atrial septal defect and heterotaxy with suggestive linkage to chromosome 6p

Anomalous inferior and superior venae cavae with oculoauriculovertebral defect: Review of Goldenhar complex and malformations of left-right asymmetry

Multiple and Recurrent Systemic Thrombotic Events Associated with Congenital Anomaly of Inferior Vena Cava

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