A Reminder From the Past: A 49-Year-Old Patient With Transposition of the Great Arteries Palliated With Dr. Thomas Baffes' and Dr. William Mustard's Original Operations

Greutmann, Matthias; Tobler, Daniel; Grewal, Jasmine; Biaggi, Patric; Oechslin, Erwin; Crean, Andrew; Silversides, Candice K.

doi:10.1016/j.echo.2009.06.003

Cited by 2 publications

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“…Although patients with TGA and large ventricular septal defect (VSD) treated with atrial inversion and VSD closure did experience ongoing attrition unrelated to sinus node dysfunction, 22 the evidence that newborns with TGA without VSD suffered a similar fate has not been persuasive. 23 –25 In addition, it has long been recognized that, regardless of their associated anomalies and whether their septation defect is at atrial, ventricular, or great arterial level, Eisenmenger syndrome patients rarely develop RV failure despite decades of systemic-level pressure. 26…”

Section: Myth 1: “Having a Single Ventricle Especially If It Is A Morphological Right Ventricle (Rv) Is Incompatible With A Normal Life Smentioning

confidence: 99%

Insights After 40 Years of the Fontan Operation

Chin

Whitehead

Watrous

2010

World J Pediatr Congenit Heart Surg

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Fontan's visionary operation and its modifications over the ensuing decades have re-established nonturbulent flow and substantially reduced cyanosis for patients with severe hypoplasia of one ventricle. However, a long list of largely unexpected sequelae has emerged over the last 40 years. Although it is not difficult to understand how care providers could become discouraged, a number of myths have arisen, which we will attempt to dispel with real-world counterexamples as well as with lessons learned from other disciplines: evolutionary, developmental, and computational biology. We argue that distinctive biochemical abnormalities pointing to dysfunction in multiple organs, including the largest organ system in the body, the endothelium, occur long before grossly observable changes in cardiac imaging can be recognized. With a rational redesign of both our surveillance scheme and our wellness strategies, we hope that Fontan survivors and their families, as well as physicians, nurses, and therapists, will see why Fontan's principle remains just as vibrant today as it was in 1971.

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Section: Myth 1: “Having a Single Ventricle Especially If It Is A Morphological Right Ventricle (Rv) Is Incompatible With A Normal Life Smentioning

confidence: 99%