Insights After 40 Years of the Fontan Operation

Abstract

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.

How insights from cardiovascular developmental biology have impacted the care of infants and children with congenital heart disease

“…Although patients with transposition of the great arteries (TGA) and large ventricular septal defect (VSD) treated with atrial inversion and VSD closure did experience ongoing attrition unrelated to sinus node dysfunction, the evidence that patients with TGA alone suffered RV dysfunction has not been persuasive (reviewed in Chin et al, 2010). Moreover, although several analyses of Fontan survivors have identified having a morphological RV as a risk factor for poor long-term outcome, other reports disagree (reviewed in Chin et al, 2010). …”

“…As last year was the 40 th anniversary of Fontan’s original report (Fontan and Baudet, 1971) describing a way to redirect the systemic venous flow directly into the pulmonary arteries while confining the pulmonary venous flow to the systemic arterial side of the circulation, the surgical treatment of univentricular disorders has been the subject of several recent reviews (Kreutzer et al, 2010; Chin et al, 2010) and will not be covered here.…”

Pediatric Cardiac Surgery

“…Considerable uncertainty remains concerning factors that influence the durability of the functionally univentricular "Fontan circulation." 12 4. The idea that improved survival has been accompanied by a pattern of increasing interventions during a patient's lifetime.…”

Surveillance for hepatocellular carcinoma secondary to cardiogenic cirrhosis in patients with congenital heart disease

“…Surgical advancements such as the Blalock–Taussig shunt and the Fontan procedure have improved the outlook of patients diagnosed with CHD, such that patients can expect a 20-year survival rate approaching 90 % [1]. The improved survival of children with CHD is now affecting adult medicine.…”