Long-term Outcomes of Adults With Single Ventricle Physiology Not Undergoing Fontan Repair: A Multicentre Experience

26. PMID: 34118376


Long-term Outcomes of Adults With Single Ventricle Physiology Not Undergoing Fontan Repair: A Multicentre Experience.


Buendía-Fuentes F, Gordon-Ramírez B, Dos Subirà L, Merás P, Gallego P, González A, Prieto-Arévalo R, Segura T, Rodríguez-Puras MJ, Montserrat S, Sarnago-Cebada F, Alonso-García A, Oliver JM, Rueda-Soriano J.Can J Cardiol. 2022 Jul;38(7):1111-1120. doi: 10.1016/j.cjca.2021.06.001. Epub 2021 Jun 9.PMID: 34118376


Take Home Points


Univentricular heart patients, not having undergone a Fontan palliation were classified into 3 types in this large 7 centre study from Spain; Eisenmenger physiology, those with surgical aortopulmonary shunts, and those with restricted pulmonary blood flow. Mortality occurred in 22.6% (n=33) over 7.3+/- 4.1 years of follow-up with sudden death being most common (36.4%) versus heart failure related (27.%). Five and 10 year survival was 86% and 74% respectively overall, patients with restrictive pulmonary blood flow doing better. Four baseline risk factors, after adjustment for age, ventricular morphology, group and NYHA class, predicted increased mortality:

  • Moderate or more atrioventricular regurgitation
  • Thrombocytopaenia (<150×103/mm3)
  • Bundle branch block (QRS>120ms)
  • Renal dysfunction (<60mls/min/1.73m2)

Patients with restricted pulmonary blood flow had lower mortality (p=0.019), heart failure admissions (p=0.017) and fewer arrhythmias (p=0.028).


Commentary by Dr Simon MacDonald (London, UK), section editor of ACHD Journal Watch:


Long-term survival and cardiovascular outcomes in adult patients with a single ventricle, who have not undergone a Fontan palliation, have been unclear and understudied. Single ventricle patients include those who are not suitable for biventricular correction with the ventricular mass then supporting both systemic and pulmonary circulations. Most now undergo a Fontan palliation but some are not suitable and some present late. In perhaps the largest study of these patients, Francisco Buendía-Fuentes and team performed a retrospective review of data from 7 centres in Spain of 146 patients over a 21 year period. They subdivided the cohort into the groups of Eisenmenger (26 or 17.8%), those with restricted pulmonary flow (86 or 58.9%) and those with aortopulmonary shunts (34 or 23.3%) with death as the main endpoint and secondary endpoints of transplantation, heart failure admissions and transplantation, endocarditis, atrial arrhythmias, stroke, and brain abscesses.


Data was obtained from centre databases, kept prospectively, with charts reviewed from May 1999 to March 2020. The 146 patients studied had a mean age at presentation 32.5±11.1 years. Double-inlet left ventricle was the most common anatomy (48.6%), followed by tricuspid atresia (19.2%), with left ventricular morphology being most common (74%).


They had a mean follow-up of 7.3±4.1 years with 33 deaths (22.6%). Five and 10 year survival was 86% and 74% respectively in the whole cohort. Survival was different between the three groups (p=0.019) as below:


Patients with restricted pulmonary flow had the best survival with rate 93% and 81% at 5 and 10 years.


Univariate analysis suggested lower saturations, NHYA class and ventricular function were important, but not in the multivariate analysis which suggested moderate atrioventricular valve regurgitation, platelet count <150×103/mm3, GFR <60mls/min/1.73m2 and QRS>120ms predicted increased mortality. Interestingly ventricular morphology was not predictive of death.


Over a third (34.2%) were admitted with heart failure symptoms, 11 (7.5%) received heart transplant (10 heart, 1 heart/lung), with mean 5 year survival post-transplant 78%.  Atrial flutter/ fibrillation was seen in 28 (19.2%) patients with 15 having electrophysiology study. Of the 28, 8 of these patients had a stroke, two underwent transplantation and 9 died. Those with a restricted pulmonary flow had less atrial arrhythmias than the other groups (p=0.028).


These patients all have long-term sequalae of cyanotic congenital heart disease, developing a multisystem disorder. The population studied was large but there were non-standardised protocols between the centres and many patients seemed lost to follow-up initially (48%) with 32% followed-up by non ACHD cardiologists. Mortality and event rates of patients before entry to the study are not known, with survivorship bias present. Also survival outcomes of patients not followed in ACHD centres was unclear. The effect of developing new risk factors over time and the impact of particular treatments (like heart failure medications, valve replacement) was not studied and would need a larger still study group. The extent of advance care planning was also unclear. This multicentre study was however successful in reporting outcomes in this small and unique patient group and potential factors that influence death and cardiac outcomes, with the accompanying editorial emphasising the need for these patients to be cared for in specialist ACHD centres.