Factors Associated With Normal Exercise Capacity in Patients With Single Ventricle
Factors Associated With Normal Exercise Capacity in Patients With Single Ventricle
The congenital heart disease (CHD) is the firts cause of congenital birth defects. Medical
 and surgical advances completely changed the epidemiology of CHD. Among these CHD, the
 univentricular heart, or single ventricle (SV), is a disease whose medical-surgical progress
 has completely changed the epidemiology.

 The SV at birth receives systemic and pulmonary venous returns in the single functional
 cavity and provides systemic and pulmonary flow. The SV undergoes a severe volumetric
 overload and considerably increases its work, which is not viable for the long time.

 In 1968, Professor Francis Fontan created the palliative surgery of the univentricular heart.
 It consists in establishing a derivation of the systemic venous blood from the two vena cava
 veins directly to the pulmonary arteries, without a sub-pulmonary ventricle. So, the VU no
 longer undergoes volumetric overload, and eliminates cyanosis.

 In this "Fontan circulation", there is no pump to push the blood in the pulmonary arteries,
 only the remaining systemic post-capillary energy (reflection of the central venous pressure)
 allows to drive the blood through the lungs with a risk of stasis upstream (right overload)
 and a limited pre-charge downstream, which can lead to severe consequences: limited exercise
 capacity, systolic and diastolic dysfunction of the SV, arrhythmia, cyanosis, cirrhosis and
 hepatic carcinoma, exudative enteropathy , plastic bronchitis, venous thromboses, deaths.

 There is a great heterogeneity within this family of SV with extremely varied evolutionary
 profiles. The cardiorespiratory exercise test, which has become a "gold standard", makes it
 possible to assess the severity of CHD. In our study on the aerobic fitness of children with
 CHD, the investigators found that 44% of children with SV had normal aerobic fitness (> 80%
 of the theoretical VO2max)1. Some good prognostic criteria are already described: left SV,
 good VU function, total cavopulmonary shunt in young age, etc. But the investigators know
 that these elements are not enough to explain why some SV, which the investigators will call
 the "great-SV", have a better aerobic fitness.

 The main objective of our study is to describe the population of "great SV" (VO2max ≥ 80%)
 within the general population of SV. The investigators thus wish to determine the prognostic
 criteria favorable to a good aerobic fitness in univenticular heart disease.
This study is cross-sectionnal, multicentric national (M3C network), descriptive,
 observational.

 Children ≥ 6 years old and adults with a single ventricle according to the international
 classification ACC-CHD and who had a cardiopulmonary exercise test in the year during usual
 follow-up will be included. Patients refuse the use of medical data will be excluded.

 The main objective of our study is to describe the rate of "great SV" (VO2max ≥ 80%) within
 the general population of SV. The secondary objectives are to compare the two groups: group
 "great SV" with VO2max ≥ 80% versus group SV with VO2max < 80%.

 The following criteria will be collected

 Anatomical : left SV, right SV, undetermined SV, total cavopulmoary shunt, partial
 cavopulmonary shunt

 Clinic: NYHA status, saturation, medical treatment, number of surgery procedure, number of
 catheterization procedure, presence of pacemaker or implantable defibrillator, presence of
 arrythmia.

 Morbidity: number oh hospitalization during the year, presence of arrhythmia, cirrhosis and
 hepatic carcinoma, exudative enteropathy , plastic bronchitis, venous thromboses, stroke.

 Echocardiography: ejection fraction of SV, 2D Strain of SV, atrioventricular valve
 regurgitation, aortic stenosis, aspect of the flow in the cavopulmonary shunt.

 Cardiac MRI: ejection fraction of SV

 Cardiac catheterization: mean pulmonary arterial pressure, pulmonary arterial wedge pressure,
 presence of fistulae.

 Cardiopulmonary exercise test : VO2max, anaerobic threesold, VE/VCO2 slope, oxygen uptake
 efficiciency slope, maximal heart rate, oxygene pulse and saturation at exercise.

 Pulmonary functional test : forced expiratory volume in 1 seconde (FEV1), the forced vital
 capacity (FVC), the FEV1/FVC ratio (FEV1/FVC%), DLCO and residual volume.
Inclusion criteria:

 - Children ≥ 6 years old and adults with a single ventricle according to the international
 classification ACC-CHD and who had a cardiopulmonary exercise test in the year during usual
 follow-up.

 Exclusion criteria:

 - Patients refuse the use of medical data will be excluded.