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Congenitally corrected transposition with isolated atrial septal defect and apicocaval juxtaposition: a hitherto undescribed cardiac cause of central cyanosis.

ABSTRACT: In our practice, we have been struck by the presence of central hypoxemia in individuals with congenitally corrected transposition of the great arteries (CCTGA) with an atrial septal defect (ASD) as the only association, despite existence of normal pulmonary artery pressures. This right to left shunting at the atrial level occurs only in those patients where there is additional presence of apicocaval juxtaposition (ACJ). Patients with CCTGA but without ACJ manifest only left to right shunting at the level of the ASD. We present a case series of patients with CCTGA with intact ventricular septum and incomplete atrial septum with a possible basis for this phenomenon.

SUBMITTER: Awasthy N

PROVIDER: S-EPMC7525385 | biostudies-literature | 2019 Apr

REPOSITORIES: biostudies-literature

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Congenitally corrected transposition with isolated atrial septal defect and apicocaval juxtaposition: a hitherto undescribed cardiac cause of central cyanosis.

Awasthy Neeraj N Sharma Rajesh R

Indian journal of thoracic and cardiovascular surgery 20190117 2

In our practice, we have been struck by the presence of central hypoxemia in individuals with congenitally corrected transposition of the great arteries (CCTGA) with an atrial septal defect (ASD) as the only association, despite existence of normal pulmonary artery pressures. This right to left shunting at the atrial level occurs only in those patients where there is additional presence of apicocaval juxtaposition (ACJ). Patients with CCTGA but without ACJ manifest only left to right shunting at ...[more]

PMID: 33061015

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Project description:BackgroundAdults with unoperated congenitally corrected transposition of the great arteries are rare but form a distinct group among adults with congenital heart disease. Patients with congenitally corrected transposition of the great arteries often have one or more associated cardiac anomalies that dictate the need for, and timing of, surgical intervention in childhood. However, in a proportion of patients, the hemodynamics does not require surgical attention during childhood, and, in some patients, a correct diagnosis is not established until adulthood. Here we report an adult case of unoperated congenitally corrected transposition of the great arteries with a large ventricular septal defect and probable pulmonary arterial hypertension.Case presentationOur patient was a 46-year-old Korean man. Transthoracic echocardiography and cardiac catheterization demonstrated hemodynamically balanced ventricles with a non-regurgitant systemic atrioventricular valve, normal pulmonary arterial pressure, and a reasonable difference between the oxygen saturation values of the aorta and pulmonary trunk, even with the presence of a large ventricular septal defect. Further morphological assessments using cardiac computed tomography and three-dimensional modeling/printing of his heart revealed that the mitral valve was straddling over the posteriorly positioned ventricular septal defect, which could explain the functional and anatomical subvalvular pulmonary stenosis and a small amount of shunt flow through the large ventricular septal defect. We interpreted this combination of cardiac defects as able to sustain his stable cardiac function. Thus, we decided to maintain his unoperated status.ConclusionA detailed anatomical understanding based on transthoracic echocardiography, cardiac computed tomography, and three-dimensional printing can justify a decision to not operate in cases of congenitally corrected transposition of the great arteries with hemodynamically balanced pulmonary stenosis and a ventricular septal defect, as observed in the present case.

Project description:Background Delayed brain development, brain injury, and neurodevelopmental disabilities are commonly observed in infants operated for complex congenital heart defect. Our previous findings of poorer neurodevelopmental outcomes in individuals operated for simple congenital heart defects calls for further etiological clarification. Hence, we examined the microstructural tissue composition in cerebral cortex and subcortical structures in comparison to healthy controls and whether differences were associated with neurodevelopmental outcomes. Methods and Results Adults (n=62) who underwent surgical closure of an atrial septal defect (n=33) or a ventricular septal defect (n=29) in childhood and a group of healthy, matched controls (n=38) were enrolled. Brain diffusional kurtosis imaging and neuropsychological assessment were performed. Cortical and subcortical tissue microstructure were assessed using mean kurtosis tensor and mean diffusivity and compared between groups and tested for associations with neuropsychological outcomes. Alterations in microstructural tissue composition were found in the parietal, temporal, and occipital lobes in the congenital heart defects, with distinct mean kurtosis tensor cluster-specific changes in the right visual cortex (pericalcarine gyrus, P=0.002; occipital part of fusiform and lingual gyri, P=0.019). Altered microstructural tissue composition in the subcortical structures was uncovered in atrial septal defects but not in ventricular septal defects. Associations were found between altered cerebral microstructure and social recognition and executive function. Conclusions Children operated for simple congenital heart defects demonstrated altered microstructural tissue composition in the cerebral cortex and subcortical structures during adulthood when compared with healthy peers. Alterations in cerebral microstructural tissue composition were associated with poorer neuropsychological performance. Registration URL: https://www.clinicaltrials.gov; Unique identifier: NCT03871881.

Dataset Information

Congenitally corrected transposition with isolated atrial septal defect and apicocaval juxtaposition: a hitherto undescribed cardiac cause of central cyanosis.

Publications

Congenitally corrected transposition with isolated atrial septal defect and apicocaval juxtaposition: a hitherto undescribed cardiac cause of central cyanosis.

Similar Datasets

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