Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2016 Jun;10(3):194-9.
doi: 10.1177/1753465816636810. Epub 2016 Mar 4.

Bodily isomerism is an independent risk factor for pulmonary hypertension in adults with congenital heart disease

Affiliations

Bodily isomerism is an independent risk factor for pulmonary hypertension in adults with congenital heart disease

Rohit S Loomba et al. Ther Adv Respir Dis. 2016 Jun.

Abstract

Introduction: Bodily isomerism, or heterotaxy is a unique entity on which there is mirror imagery in various organ systems, leading to a deviation from the normal lateral arrangements of the viscera. Adults with such isomerism and associated congenital malformations of the heart are now reaching adulthood and developing long-term complications. This study investigates the prevalence and characteristics of pulmonary hypertension in adults with isomerism.

Methods: The 2012 iteration of the Nationwide Inpatient Sample was utilized and patients were identified as having or not having bodily isomerism and having or not having pulmonary hypertension. Univariate analysis utilizing Chi-square tabulation was done to assess characteristics associated with pulmonary hypertension. Next, a multivariate analysis was done on all patients to identify predictors of pulmonary hypertension followed by a multivariate analysis of patients with only isomerism to identify predictors of pulmonary hypertension specific to this subset.

Results: A total of 6,907,109 admissions were included in the analysis. Of these, 861 had isomerism (0.01%). Of those with isomerism, 5.6% were found to have pulmonary hypertension. When all patients were included in the multivariate analysis, isomerism was found to be an independent risk factor for pulmonary hypertension with an odds ratio of approximately 1.79. When only patients with isomerism were included in the multivariate analysis, advanced age, obesity, and history of anomalous pulmonary venous connection were independent risk factors of pulmonary hypertension.

Conclusion: Pulmonary hypertension is more common in those with isomerism, with isomerism being an independent risk factor for pulmonary hypertension. The prevalence of pulmonary hypertension is 5.6% in the setting of isomerism. Independent risk factors for pulmonary hypertension in patients with isomerism include age, obesity, and history of anomalous pulmonary venous connection.

Keywords: atrioventricular septal defect; congenital heart disease; double outlet right ventricle; heterotaxy; isomerism; malformations; pulmonary hypertension.

PubMed Disclaimer

Conflict of interest statement

Conflict of interest statement: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

References

    1. Evans W., Acherman R., Restrepo H. (2015) Heterotaxy in Southern Nevada: prenatal detection and epidemiology. Pediatr Cardiol 36: 930–934. - PubMed
    1. Galie N., Corris P., Frost A., Girgis R., Granton J., Jing Z., et al. (2013) Updated treatment algorithm of pulmonary arterial hypertension. J Am Coll Cardiol 62: D60–D72. - PubMed
    1. Galie N., Humbert M., Vachiery J., Gibbs S., Lang I., Torbicki A., et al. (2015) 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the joint task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J 46: 903–975. - PubMed
    1. Gatzoulis M., Beghetti M., Landzberg M., Galie N. (2014) Pulmonary arterial hypertension associated with congenital heart disease: recent advances and future directions. Int J Cardiol 177: 340–347. - PubMed
    1. Hsu D. (2015) The fontan operation: the long-term outlook. Curr Opin Pediatr 27: 569–575. - PubMed

LinkOut - more resources