Atrial Right-to-left Shunt without Pulmonary Hypertension in a Patient with Biventricular Non-compaction Cardiomyopathy Accompanied by Ventricular and Atrial Septal Defects

Sakan, Hirokazu; Okayama, Satoshi; Uemura, Shiro; Somekawa, Satoshi; Ishigami, Kousei; Takeda, Yukiji; Kawata, Hiroyuki; Horii, Manabu; Fujimoto, Shinichi; Saito, Yoshihiko

doi:10.2169/internalmedicine.50.5290

Cited by 6 publications

(3 citation statements)

References 12 publications

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“…Multiple causes of RV dysfunction have been associated with right-toleft shunting, including RV infarction, carcinoid syndrome, cardiomyopathy, and TR. [6][7][8][9] Our patient had a known PFO and developed pacemaker-related TR. She initially presented with mild symptoms due predominantly to volume overload from the TR, but her symptoms progressed and she presented with upright hypoxemia (platypnea-orthodeoxia).…”

Section: Discussionmentioning

confidence: 81%

Stepwise Progression of Right-to-Left Atrial Shunting through a Combination of Patent Foramen Ovale and Tricuspid Regurgitation

Kransdorf¹,

Kransdorf²,

Fortuin³

et al. 2016

Texas Heart Institute Journal

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Patent foramen ovale is a common clinical finding that generally becomes a concern in the presence of transient ischemic attack or stroke. Rarely, patent foramen ovale is associated with hypoxemia in the presence of substantial right-to-left atrial shunting. We present the case of an 86-year-old woman with a pacemaker, who was initially asymptomatic notwithstanding a patent foramen ovale. Over 1.5 years, her symptoms progressed in a stepwise fashion, in the setting of progressive pacemaker-associated tricuspid regurgitation. Ultimately, the patient's symptoms and her hypoxemia resolved after percutaneous closure of her patent foramen ovale with use of a 25-mm "Cribriform" occluder device. This case highlights the fact that clinically significant right-to-left shunting requires an anatomic lesion, such as patent foramen ovale, together with elevated right atrial pressure, which in this case was contributed by severe tricuspid regurgitation.

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Section: Discussionmentioning

confidence: 81%

Stepwise Progression of Right-to-Left Atrial Shunting through a Combination of Patent Foramen Ovale and Tricuspid Regurgitation

Kransdorf¹,

Kransdorf²,

Fortuin³

et al. 2016

Texas Heart Institute Journal

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“…LVHT associated with atrial septal defect with or without pulmonary valve stenosis has been reported by several authors [2-9,11]. In most of these patients atrial septal defect and LVHT were associated with several other cardiac and extracardiac anomalies, and genetic studies revealed various mutations as listed in Table 2.…”

Section: Discussionmentioning

confidence: 85%

Left ventricular hypertrabeculation/noncompaction with epilepsy, other heart defects, minor facial anomalies and new copy number variants

Nagel¹,

Gruber-Sedlmayr

Uhrig

et al. 2012

BMC Med Genet

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BackgroundLeft ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality of unknown etiology which has been described in children as well as in adults with and without chromosomal aberrations. LVHT has been reported in association with various cardiac and extracardiac abnormalities like epilepsy and facial dysmorphism.Case presentationA unique combination of LVHT, atrial septal defect, pulmonary valve stenosis, aortic stenosis, epilepsy and minor facial anomalies is presented in a 5.5 years old girl. Microarray-based genomic hybridization (array-CGH) detected six previously not described copy number variants (CNVs) inherited from a clinically unaffected father and minimally affected mother, thus, most likely, not clinically significant but rare benign variants.ConclusionsDespite this complex phenotype de novo microdeletions or microduplications were not detected by array CGH. Further investigations, such as whole exome sequencing, could reveal point mutations and small indels as the possible cause.

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“…Left ventricular noncompaction (LVNC) arises during development of the myocardium after week 18 of gestation, and is sometimes associated with other congenital cardiac abnormalities 1,2. It is characterized by deep endothelium-lined trabeculations, identifiable by colored Doppler imaging,3 that are continuous with the lumen of the left ventricle and give the ventricular wall a two-layered appearance (noncompacted and compacted).…”

Section: Introductionmentioning

confidence: 99%

Myocardial infarction in a patient with left ventricular noncompaction: a case report

Toufan¹,

Shahvalizadeh²,

Khalili³

2012

IJGM

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We describe a 73-year-old male patient with left ventricular noncompaction (LVNC) who was diagnosed with acute myocardial infarction (MI), three-vessel coronary artery disease, a fresh intraventricular thrombus, and mitral regurgitation. He was treated with full anticoagulant therapy, coronary artery bypass grafting, and mitral valve repair. This case adds to a small but growing literature showing association between LVNC and MI and/or coronary artery disease. We suggest that patients with LVNC could be considered at heightened risk for MI, and the two conditions might have a common genetic underpinning in some cases.

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Atrial Right-to-left Shunt without Pulmonary Hypertension in a Patient with Biventricular Non-compaction Cardiomyopathy Accompanied by Ventricular and Atrial Septal Defects

Cited by 6 publications

References 12 publications

Stepwise Progression of Right-to-Left Atrial Shunting through a Combination of Patent Foramen Ovale and Tricuspid Regurgitation

Stepwise Progression of Right-to-Left Atrial Shunting through a Combination of Patent Foramen Ovale and Tricuspid Regurgitation

Left ventricular hypertrabeculation/noncompaction with epilepsy, other heart defects, minor facial anomalies and new copy number variants

Myocardial infarction in a patient with left ventricular noncompaction: a case report

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