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Canadian Journal of Cardiology

The “Forgotten Ones”: The Natural and Unnatural History of Univentricular Physiology Without Fontan Palliation

  • Danielle Massarella
    Affiliations
    Toronto Adult Congenital Heart Disease Program, Division of Cardiology, Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, Ontario, Canada
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  • Erwin Oechslin
    Correspondence
    Corresponding author: Dr Erwin Oechslin, Professor of Medicine, University of Toronto, Peter Munk Cardiac Centre, University Health Network/Toronto General Hospital, 5 NUW—519, 585 University Ave, Toronto, Ontario M5G 2N2, Canada. Tel.: +1-416-340-3338; fax: +1-416-340-5014.
    Affiliations
    Toronto Adult Congenital Heart Disease Program, Division of Cardiology, Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, Ontario, Canada
    Search for articles by this author
Published:November 10, 2021DOI:https://doi.org/10.1016/j.cjca.2021.11.001
      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.
      • Hager A.
      • Kaemmerer H.
      • Eicken A.
      • Fratz S.
      • Hess J.
      Long-term survival of patients with univentricular heart not treated surgically.
      • Ammash N.M.
      • Warnes C.A.
      Survival into adulthood of patients with unoperated single ventricle.
      • Suraci N.
      • Kassem H.
      • Mihos C.
      • Baquero S.
      • Santana O.
      Uncorrected univentricular heart in an adult.
      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.
      • Ammash N.M.
      • Warnes C.A.
      Survival into adulthood of patients with unoperated single ventricle.
      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.
      • 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.
      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.
      • Poterucha J.T.
      • Anavekar N.S.
      • Egbe A.C.
      • et al.
      Survival and outcomes of patients with unoperated single ventricle.
      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.
      • Erikssen G.
      • Aboulhosn J.
      • Lin J.
      • et al.
      Survival in patients with univentricular hearts: the impact of right versus left ventricular morphology.
      2018 111 Not available Not specified Not available 79% Single dominant right ventricle (P < 0.0001)
      Vermaut et al.
      • Vermaut A.
      • de Meester P.
      • Troost E.
      • et al.
      Outcome of the Glenn procedure as definitive palliation in single ventricle patients.
      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.
      • 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.
      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
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