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Pulmonary Hypertension Secondary to Congenital Systemic-to-Pulmonary (Left-to-Right) Shunts

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Textbook of Pulmonary Vascular Disease

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Abstract

Pulmonary arterial hypertension associated with congenital left-to-right shunts remains a matter of concern not only in underserved areas, but also in developed countries, in both un-operated on and operated on patients. The risk of developing advanced pulmonary vascular disease generally but not invariably depends on the size and location of the defect. Patients with restrictive ventricular septal defects (VSDs) are unlikely to acquire pulmonary arterial hypertension (the prevalence is only 3%). On the other hand, the likelihood of severe pulmonary hypertension and development of Eisenmenger syndrome is considerable in patients with nonrestrictive defects (greater than 1.5 cm in diameter); in this case, 50% will be affected. This is in contrast with subjects with ASDs, for whom the risk of acquiring pulmonary hypertension is not higher than 10% with a late onset (90% during the adulthood). However, pulmonary artery pressure and vascular resistance tend to be more frequently elevated in patients with sinus venosus defects than in those with secundum defects. Despite the general concept that pulmonary hypertension is not a matter of concern if patients undergo repair of the cardiac shunts during the first year of life, certain anomalies are known to be associated with early elevation of pulmonary vascular resistance. There is general agreement that this is the case for truncus arteriosus, atrioventricular septal defects, and transposition of the great arteries with VSD. For unknown reasons, some patients with simple defects such as VSD or patent ductus arteriosus have no history of pulmonary congestion or failure to thrive during the first months of life, suggesting early-onset pulmonary arterial hypertension. These patients cannot be safely assigned for repair of the defects on the basis of noninvasive evaluation only. Rather, complete evaluation including direct measurement of pulmonary vascular resistance is necessary to make a decision about the therapeutic strategies. This chapter explores how the appropriate recognition of a state of increased pulmonary blood flow or a state of increased pulmonary vascular resistance allows for decisions to be made between the therapeutic options that are applicable to patients with left-to-right shunts associated with pulmonary hypertension (correction of the defect, pulmonary artery banding, medical treatment, or combinations). Although there is not much evidence to support such decisions, expertise that has been accumulated in tertiary reference centers with high standards of medical assistance allows for proper selection of the therapeutic strategies. The issue of adults with ASD is particularly focused on. The pathogenic mechanisms of pulmonary vascular disease in left-to-right shunts and the correlations of pathological features with the outcomes are also addressed.

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Authors and Affiliations

  1. Department of Pediatric Cardiology and Adult Congenital Heart Disease, Heart Institute (InCor), University of São Paulo School of Medicine, São Paulo-SP, 05403-000, Brazil

    Antonio A. Lopes

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Editors and Affiliations

  1. Departments of Medicine, COMRB Rm. 3131 (MC 719), University of Illinois at Chicago, 909 South Wolcott Avenue, Chicago, 60612, Illinois, USA

    Jason X. -J. Yuan

  2. 310 Admin.Office Building (MC 672), University of Illinois at Chicago, 1737 W. Polk Street, Suite 310, Chicago, 60612, Illinois, USA

    Joe G.N. Garcia

  3. Department of Medicine, University of California, San Diego, 9500 Gilman Drive, La Jolla, 92093-0623, California, USA

    John B. West

  4. Dept. Pulmonary & Critical Care Medicine, Massachusetts General Hospital, 55 Fruit Street, Boston, 02114, Massachusetts, USA

    Charles A. Hales

  5. Department of Medicine, University of Chicago Medical Center, 5841 S. Maryland Ave., Chicago, 60637, Illinois, USA

    Stuart Rich

  6. Department of Medicine, University of Chicago School of Medicine, 5841 S. Maryland Ave., Chicago, 60637, Illinois, USA

    Stephen L. Archer

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Lopes, A.A. (2011). Pulmonary Hypertension Secondary to Congenital Systemic-to-Pulmonary (Left-to-Right) Shunts. In: Yuan, JJ., Garcia, J., West, J., Hales, C., Rich, S., Archer, S. (eds) Textbook of Pulmonary Vascular Disease. Springer, Boston, MA. https://doi.org/10.1007/978-0-387-87429-6_78

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