Commentary: Superior cavopulmonary connection: A false sense of security?

Central MessageThe interstage attrition between superior cavopulmonary connection to Fontan remains significant for patients with hypoplastic left heart syndrome.See Article page 385. The syndrome. See When Marc de Leval introduced the total in 1988, it was, like all other previous versions of operation, meant be performed as a single-stage procedure. initial concept preceding an interval (SCPC) was reduce mortality and morbidity high-risk patients. However, staged approach became popular management syndrome (HLHS), is now standard practice most single-ventricle hearts, regardless anatomy.1Mazzera E. Corno A. Picardo S. Di Donato R. Marino B. Costa D. et al.Bidirectional shunts: clinical applications or definitive palliation.Ann Thorac Surg. 1989; 47: 415-420Abstract Full Text PDF PubMed Scopus (85) Google Scholar In addition early volume unloading single right ventricle minimizing acute change ventricular mass ratio seen Fontan, SCPC confers ever-growing source pulmonary blood flow that avoids flaws associated systemic-to-pulmonary artery shunts bilateral arterial bands. fact, so preferable stage-1 circulation HLHS palliation some advocate transition stage-2 operation before patient reaches age 3 months.2Viegas M. Diaz-Castrillon C.E. Castro-Medina Da Fonseca Silva L. Morell V.O. Bidirectional Glenn procedure less than months age: 14-year experience.Ann 2020; 110: 622-629Abstract (4) renders such well-tolerated assumption our are out danger from attrition. Therefore, long time, we have focused attention on mitigating stages 1 2, well long-term sequelae state. Getting cause celebration. As turns out, sense security regarding false. Building upon growing number smaller studies, including derived Single Ventricle Reconstruction Trial, exceptional group at Children's Hospital Philadelphia further raises awareness about unrelenting Fontan.3Lawrence K.M. Ittenbach R.F. Hunt M.L. Kaplinski Ravishankar C. Rychik J. al.Attrition syndrome.J Cardiovasc 2021; 162: 385-393Abstract authors tapped into their massive institutional experience over decades reveal (ie, death transplantation) 2 3, 851-patient cohort, has not changed all. confirming 8%-10% rate attrition, new insights uncovered this study include identifying use ventricle-to-pulmonary (RV-PA) shunt stage important antagonist. Other risk factors, needing atrioventricular valve repair longer post-SCPC course, were reaffirmed influence outcomes. But everything doom gloom. good news many hazards contribute poor outcomes, low gestational age, genetic anomalies, anomalous venous drainage, intact atrial septum, no pertinent. This survival effect suggests that, these achieving major milestone those nonmodifiable anatomic features insignificant. Nonetheless, descriptive based historical information incomplete details, there areas missed opportunities raise more questions. One reported doubling within later eras who received RV-PA 1, compared had modified Blalock-Taussig shunts. Because inter 2-to-3 remained same throughout 4 eras, suggesting they stayed patients, era outcomes would been better? It also unfortunate unable provide whether dysfunction growth (or both) mechanistic driver worse Similarly, need only surrogate regurgitation. Without complete echocardiographic after repair, wanting more. Finally, although tell us mode failure underwent transplantation primarily dysfunction, account 52 died. Were candidates because function hypertension? quite used call arms redouble efforts but rather admitting failure, I play devil's here. lack improvement due understanding diligence, may very nature circulation. Being transitional circulatory arrangement typically lasts years, promotes return without power source. Perhaps, just circulation, despite optimize its construction effects, fundamentally flawed physiology continuing unyielding quality life failure. We should continue invest monitoring, yet entertain possibility best industry dedication will always reaching Fontan. inception 1940s 1950s, anastomosis neither pertained nor applied pathologies. Rather, described various procedures connect SVC treat range defects where insufficient flow, tetralogy Fallot. Later, term bidirectional added denote modifications connected both arteries. commended adopting describe often connection. After all, forms (including connection) provides sides. Attrition syndromeThe Journal Thoracic Cardiovascular SurgeryVol. 162Issue 2PreviewWe investigated incidence predictors undergo children survived Full-Text