The "Forgotten Ones": The Natural and Unnatural History of Univentricular Physiology Without Fontan Palliation.

Abstract

Univentricular heart (UVH) refers to a subset of congenital cardiac defects characterised by 1 hypoplastic ventricle ill-equipped to handle 50% of combined ventricular output and thus rendering biventricular repair unfeasible. Univentricular physiology (UVP) includes those with a Fontan-type palliation and those without a Fontan-type palliation. Those without a Fontan palliation remain cyanotic due to an intracardiac shunt and can live into adulthood without cardiac intervention. 1 Hager A. Kaemmerer H. Eicken A. Fratz S. Hess J. Long-term survival of patients with univentricular heart not treated surgically. J Thorac Cardiovasc Surg. 2002; 123: 1214-1217 Google Scholar , 2 Ammash N.M. Warnes C.A. Survival into adulthood of patients with unoperated single ventricle. Am J Cardiol. 1996; 77: 542-544 Google Scholar , 3 Suraci N. Kassem H. Mihos C. Baquero S. Santana O. Uncorrected univentricular heart in an adult. J Invasive Cardiol. 2020; 32: E44 Google Scholar A plethora of publications about risk factors for the long-term outcome in patients with a Fontan circulation contrasts with the paucity of data on adults with a univentricular circulation without a Fontan palliation (Table 1). Table 1Mortality and risk factors in adults with univentricular hearts without Fontan palliation Authors Year n Age Type of palliation Follow-up duration Mortality Predictors of death Ammash et al. 2 Ammash N.M. Warnes C.A. Survival into adulthood of patients with unoperated single ventricle. Am J Cardiol. 1996; 77: 542-544 Google Scholar 1996 13 Mean 42 (range 23-66) years Eisenmenger syndrome (n = 4) and restricted pulmonary blood flow (n = 9) Majority of patients (n = 7) seen only once; 6 patients (46%) followed 1-24 (mean 14) years 8% Best survival: DILV with transposed great arteries and moderate degree of PS Gatzoulis et al. 9 Gatzoulis M.A. Munk M.D. Williams W.G. Webb G.D. Definitive palliation with cavopulmonary or aortopulmonary shunts for adults with single ventricle physiology. Heart. 2000; 83: 51-57 Google Scholar 2000 50 At last palliation: median 11 years (range 1 day to 53 years) Group 1: cavopulmonary (n = 15); group 2: aortopulmonary shunt (n = 35) Mean 13.0 ± 6.2 years after the first visit in the ACHD clinic (at age 16-18 years) 10.6% at 10 and 48.1% at 20 years after the first visit in the ACHD clinic Predictors of death (multivariate analysis): NYHA 3 or 4 and previous episode of sustained VT; type of shunt, degree of cyanosis, and ventricular morphology were not predictive of arrhythmia or death (multivariate analysis) Poterucha et al. 18 Poterucha J.T. Anavekar N.S. Egbe A.C. et al. Survival and outcomes of patients with unoperated single ventricle. Heart. 2016; 102: 216-222 Google Scholar 2016 24 Most recent evaluation: median 52 (range 35-61) years Pulmonary hypertension / Eisenmenger syndrome (n = 11) and restricted pulmonary blood flow (n = 13) Median 25 (range 16-37) years Overall mortality: 54%; Eisenmenger syndrome: 55%; restricted pulmonary blood flow: 54% No difference between both groups (P = 0.8) Erikssen et al. 19 Erikssen G. Aboulhosn J. Lin J. et al. Survival in patients with univentricular hearts: the impact of right versus left ventricular morphology. Open Heart. 2018; 5e000902 Google Scholar 2018 111 Not available Not specified Not available 79% Single dominant right ventricle (P < 0.0001) Vermaut et al. 20 Vermaut A. de Meester P. Troost E. et al. Outcome of the Glenn procedure as definitive palliation in single ventricle patients. Int J Cardiol. 2020; 303: 30-35 Google Scholar 2020 25 At Glenn: median 6.3 (IQR 1.2-29.7) years Glenn anastomosis Median 11 (IQR 3-18) years after Glenn procedure 20% N/A Buendía-Fuentes et al. 4 Buendía-Fuentes F, Gordon-Ramírez B, Dos Subirà L, et al. Long-term outcomes of adults with single ventricle physiology not undergoing fontan repair: a multicentre experience. Can J Cardiol XXXX;XX:XXX-XX. Google Scholar 2022 146 At study entry: mean 32.5 ± 11.1 years Group 1: Eisenmenger; group 2: restricted pulmonary blood flow; group 3: aortopulmonary shunt Mean 7.3 ± 4.1 years Overall mortality: 22.6%; Kaplan-Meier survival analysis: 86% after 5 and 74% after 10 years Risk factors for mortality (multivariable analysis): at least moderate AV valve regurgitation; thrombocytopenia (< 150,000/m3); renal insufficiency (eGFR < 60 mL/min/1.73 m2); QRS > 120 ms AV, atrioventricular; DILV, double-inlet left ventricle; eGFR, estimated glomerular filtration rate; IQR, interquartile range; NYHA, New York Heart Association functional class; PS, pulmonary stenosis; VT, ventricular tachycardia Open table in a new tab AV, atrioventricular; DILV, double-inlet left ventricle; eGFR, estimated glomerular filtration rate; IQR, interquartile range; NYHA, New York Heart Association functional class; PS, pulmonary stenosis; VT, ventricular tachycardia Long-term Outcomes of Adults With Single Ventricle Physiology Not Undergoing Fontan Repair: A Multicentre ExperienceCanadian Journal of CardiologyPreviewTo describe long-term survival and cardiovascular events in adult patients with single ventricle physiology (SVP) without Fontan palliation, focusing on predictors of mortality and comparing groups according to their cardiovascular physiology. 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