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Apparent normal arrangement pattern of three-vessel view in a fetus with transposition of great arteries and L-malposed aorta.

ABSTRACT: Transposition of great arteries (TGA) is more commonly associated with D-malposition of great arteries where anterior aorta produces characteristic "I" sign in the three-vessel view (3VV) in fetal heart imaging. We describe two cases with TGA and L-malposition of aorta where 3VV imaging showed an apparently normal arrangement of vessels while outflow tract imaging proved vital in diagnosing transposition anatomy. Apparently, normal 3VV in the presence of disproportionate vessel caliber and inability to produce normal outflow images should raise the suspicion. Attempts should be made to produce views to show great arteries originating from respective ventricles to rule out ventriculoarterial discordance and to complete segmental analysis.

SUBMITTER: Verma S

PROVIDER: S-EPMC5431040 | biostudies-literature | 2017 May-Aug

REPOSITORIES: biostudies-literature

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Apparent normal arrangement pattern of three-vessel view in a fetus with transposition of great arteries and L-malposed aorta.

Verma Sudeep S Chidambaratanu Shanthi S Vijaylakshmi Raja R Srinivasan Latha L Suresh Indrani I

Annals of pediatric cardiology 20170501 2

Transposition of great arteries (TGA) is more commonly associated with D-malposition of great arteries where anterior aorta produces characteristic "I" sign in the three-vessel view (3VV) in fetal heart imaging. We describe two cases with TGA and L-malposition of aorta where 3VV imaging showed an apparently normal arrangement of vessels while outflow tract imaging proved vital in diagnosing transposition anatomy. Apparently, normal 3VV in the presence of disproportionate vessel caliber and inabi ...[more]

PMID: 28566836

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Project description:BackgroundOur prior work on congenital heart disease (CHD) with heterotaxy, a birth defect involving randomized left-right patterning, has shown an association of a high prevalence of airway ciliary dysfunction (CD; 18/43 or 42%) with increased respiratory symptoms. Furthermore, heterotaxy patients with ciliary dysfunction were shown to have more postsurgical pulmonary morbidities. These findings are likely a reflection of the common role of motile cilia in both airway clearance and left-right patterning. As CHD comprising transposition of the great arteries (TGA) is commonly thought to involve disturbance of left-right patterning, especially L-TGA with left-right ventricular inversion, we hypothesize CHD patients with transposition of great arteries (TGA) may have high prevalence of airway CD with increased respiratory symptoms.Methods and resultsWe recruited 75 CHD patients with isolated TGA, 28% L and 72% D-TGA. Patients were assessed using two tests typically used for evaluating airway ciliary dysfunction in patients with primary ciliary dyskinesia (PCD), a recessive sinopulmonary disease caused by respiratory ciliary dysfunction. This entailed the measurement of nasal nitric oxide (nNO), which is typically low with PCD. We also obtained nasal scrapes and conducted videomicroscopy to assess respiratory ciliary motion (CM). We observed low nNO in 29% of the patients, and abnormal CM in 57%, with 22% showing both low nNO and abnormal CM. No difference was observed for the prevalence of either low nNO or abnormal ciliary motion between patients with D vs. L-TGA. Respiratory symptoms were increased with abnormal CM, but not low nNO. Sequencing analysis showed no compound heterozygous or homozygous mutations in 39 genes known to cause PCD, nor in CFTR, gene causing cystic fibrosis. As both are recessive disorders, these results indicate TGA patients with ciliary dysfunction do not have PCD or cystic fibrosis (which can cause low nNO or abnormal ciliary motion).ConclusionsTGA patients have high prevalence of abnormal CM and low nNO, but ciliary dysfunction was not correlated with TGA type. Differing from PCD, respiratory symptoms were increased with abnormal CM, but not low nNO. Together with the negative findings from exome sequencing analysis, this would suggest TGA patients with ciliary dysfunction do not have PCD but nevertheless may suffer from milder airway clearance deficiency. Further studies are needed to investigate whether such ciliary dysfunction is associated with increased postsurgical complications as previously observed in CHD patients with heterotaxy.

Dataset Information

Apparent normal arrangement pattern of three-vessel view in a fetus with transposition of great arteries and L-malposed aorta.

Publications

Apparent normal arrangement pattern of three-vessel view in a fetus with transposition of great arteries and L-malposed aorta.

Similar Datasets

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