Machine measurement set with published single-ventricle cohort values
Cohort values, not normal limits. Normal values of functional indexes for the RV or the univentricular heart “are not uniformly agreed on or readily available” (AHA 2019, p. e240). Values here are published single-ventricle cohort results (means ± SD unless a row says median, %, r or sensitivity). SVR trial (Pediatric Heart Network): infants with a single right ventricle (mostly HLHS) randomised at the Norwood to a modified Blalock–Taussig shunt (MBTS) or an RV-to-PA shunt (RVPAS, Sano), 15 North American centres, core-lab echo; values are given MBTS / RVPAS. PHN Fontan study: 546 children 6–18 y. FUEL: 400 Fontan adolescents, mean age 15.5 y. Rios 2017: 100 consecutive Fontan patients, 17 months to 31 years, one centre.
| Cohort | Values | Source |
|---|---|---|
| Short-Axis Fractional Area Change (FAC) · LV area d, s (PSAX, papillary muscles) · (EDA − ESA) / EDA How to measure
| ||
| Single LV | No single-LV short-axis value; median 0.4 (0.25–0.44) in ≤4 LV-dominant patients, view not stated; all morphologies: FAC vs elastance r = 0.69 The best echo correlate of invasive contractility among the indices tested. 15 single-ventricle patients, 3 months to 19 years (11 RV-dominant; 3 after stage 1, 5 after a superior cavopulmonary connection, 7 after Fontan), pressure–volume loops under anesthesia; n = 13 for this correlation. FAC also correlated with dP/dtmax (r = 0.6) and stroke work/EDV (r = 0.64); MPI, AV-valve dP/dt and isovolumic acceleration did not. View for FAC not stated. | Butts 2015, Tables 3–4 |
| Long-Axis Length and Ejection Fraction (EF) · LV major d, s (A4C) · V = 5/6 × CSA × length How to measure
| ||
| Infants, LV + RV combined | EF before the Glenn 57.4 ± 9.3% Infant Single Ventricle trial (PHN): 156 infants with paired studies, 71% RV-dominant, LV and RV analysed together; biplane modified Simpson; superior cavopulmonary connection at a median 5.3 months. At 14 months: 58.8 ± 9.9%. | Margossian 2017, Table 1 |
| Fontan | EF: single LV 60 ± 9%, single RV 56 ± 12% PHN Fontan cross-sectional study, 6–18 y: n = 219 (LV) and 156 (RV); mixed morphology 61 ± 11% (n = 39); biplane Simpson. CMR in a subset: 57 ± 9, 55 ± 11 and 59 ± 7%. | Anderson 2008, Table 3 |
| Fontan vs CMR | Echo volumes 66–76% of CMR; LV EF limits −23.3 to 26.5 124 PHN Fontan patients with both studies (12.2 ± 3.3 y). 95% limits of agreement for EF. Mean EF difference small: LV 1.6%, RV 0.2% (RV limits −24.2 to 24.6). Visual grading of function agreed poorly between observers (kappa 0.42 for LV, 0.12 for RV; Table 2). | Margossian 2009, Table 4 |
| dP/dt · LV dP/dt · 32 mmHg / t (1 to 3 m/s, AV-valve regurgitant jet) How to measure
| ||
| Fontan | Single LV 1464.76 ± 1029.75 mmHg/s; adolescents 965.5 ± 220.0 100 consecutive Fontan patients, 11.95 ± 6.8 y, one centre: 48 had a single LV and 52 a single RV (single RV 945.45 ± 352.82; P = .010). dP/dt was measurable in 55% of studies; the number measured in each group is not printed. Adolescents (Di Maria 2022): FUEL trial baseline (mean age 15.5 y), n = 18; severe ventricular dysfunction or AV-valve regurgitation excluded. RV or other 934.8 ± 176.8 (n = 29). | Rios 2017, Table 1; Di Maria 2022, Table 3 |
| Myocardial Performance Index (MPI) · LV CTETRT, AoV ET · (a − b) / b How to measure
| ||
| Fontan | Single LV 0.45 ± 0.17; adolescents 0.42 ± 0.17 Same Fontan cohort as dP/dt above (48 single LV; MPI not obtainable in 6). Single RV 0.51 ± 0.18, not significantly different. Adolescents (Di Maria 2022): FUEL baseline, MPI by blood pool (Doppler), as given in the text; RV or other 0.49 ± 0.15. Table 3 rounds to 0.4 (0.2), n = 148, and 0.5 (0.2), n = 157. | Rios 2017, Table 1; Di Maria 2022 |
| Cohort | Values (MBTS / RVPAS) | Source |
|---|---|---|
| RV Fractional Area Change (FAC) · RV area d, s (A4C) · (EDA − ESA) / EDA, % How to measure
| ||
| Pre-Norwood | 35 ± 9 / 35 ± 8 SVR baseline exam at 2.1 ± 3.3 days, before any shunt (groups as later randomised); n = 245 and 258. | Frommelt 2012, Table 2 |
| Post-Norwood | 35 ± 8 / 38 ± 7 Post-Norwood exam at 22.5 ± 13.4 days (n = 224, 236). Before stage II (4.8 ± 1.8 months): 33 ± 7 / 34 ± 8 (n = 167, 208). | Frommelt 2012, Table 3 |
| 14 mo | 33 ± 7 / 32 ± 7 After stage II, 14.3 ± 1.2 months (n = 134, 170). Short-axis FAC (mid-ventricular parasternal short axis, speckle-tracking software): 33.6 ± 8.9 / 31.2 ± 9.3 (Hill 2015, Table 1). | Frommelt 2012, Table 3 |
| Pre-Fontan | 31 ± 6 / 31 ± 6 Within 6 months of the planned Fontan, at 33.6 ± 9.6 months (n = 102, 112). | Frommelt 2014, Table 3 |
| 6 y | 32.47 ± 5.70 / 33.26 ± 5.69 After Fontan, mean age 71.1 months (n = 90, 102). | Frommelt 2019, Table 1 |
| Adult biventricular | Normal >35% Adult biventricular RV grades: normal >35%; mild ≤35 to >29; moderate ≤29 to >22; severe ≤22. Not a single-ventricle limit: the SVR cohort means above were 31–38%. | ASE 2025, Table 1 |
| dP/dt · RV dP/dt · 32 mmHg / t (tricuspid jet), mmHg/s How to measureAs for the LV (above): 1 to 3 m/s on the CW tricuspid regurgitant jet (Rios 2017). The SVR papers read do not state their dP/dt method; it could be measured in only a minority of their studies. | ||
| Pre-Norwood | 1062 ± 430 / 1038 ± 249 n = 14 and 10. | Frommelt 2012, Table 2 |
| Post-Norwood | 1032 ± 330 / 983 ± 336 n = 46 and 41. Before stage II: 865 ± 301 / 871 ± 335 (n = 35, 47). | Frommelt 2012, Table 3 |
| 14 mo | 798 ± 318 / 854 ± 402 n = 24 and 28. | Frommelt 2012, Table 3 |
| Fontan | Single RV 945.45 ± 352.82; adolescents 934.8 ± 176.8 Rios 2017: 52 single-RV Fontan patients (39 HLHS), part of the cohort described above; number measured not printed. Di Maria 2022 (FUEL): RV or other morphology, n = 29. | Rios 2017, Table 1; Di Maria 2022, Table 3 |
| Myocardial Performance Index (MPI) · RV CTETRT, PV ET · (a − b) / b; Doppler, tissue Doppler (DTI) Which ejection time
| ||
| Post-Norwood | Doppler 0.38 ± 0.18 / 0.58 ± 0.25; DTI 0.65 ± 0.35 / 0.64 ± 0.25 Before stage II: Doppler 0.39 ± 0.18 / 0.49 ± 0.20; DTI 0.56 ± 0.21 / 0.56 ± 0.19. | Frommelt 2012, Table 3 |
| 14 mo | Doppler 0.44 ± 0.16 / 0.44 ± 0.18 14 months, DTI: 0.59 ± 0.21 / 0.60 ± 0.18. Pre-Fontan (Frommelt 2014, Table 3): Doppler 0.45 ± 0.17 / 0.45 ± 0.19; DTI 0.63 ± 0.27 / 0.59 ± 0.19. | Frommelt 2012, Table 3 |
| 6 y, Fontan | Doppler 0.49 ± 0.18 / 0.47 ± 0.19; other Fontan cohorts: single RV 0.51 ± 0.18, adolescents 0.49 ± 0.15 SVR at 6 y, DTI: 0.63 ± 0.20 / 0.65 ± 0.22. Rios 2017: single RV, Table 1. Di Maria 2022 (FUEL): RV or other morphology, blood-pool MPI from the text. | Frommelt 2019, Table 1; Rios 2017; Di Maria 2022 |
| Group | Values | Source |
|---|---|---|
| Descending Aorta Vmax and Arch Gradient · Ao desc (pre-coarctation) Vmax, Ao desc Vmax · ΔP = 4v² How to measure
| ||
| After Norwood | Discharge gradient >20 mmHg: sensitivity 31%, specificity 92% for later intervention SVR trial: 46 of 355 (13%) had a discharge peak echo-Doppler arch gradient >20 mmHg (sensitivity and specificity for a later intervention); 18% of the trial had a catheter or surgical intervention for recoarctation by 12 months, 54% of interventions at the pre-stage II catheterisation. Hazard ratio 1.07 per 1 mmHg. | Hill 2013 |
| After Norwood | Other cut-offs: ≥20 mmHg (catheter ≥10 mmHg), ≥26 mmHg (reintervention), isthmus >2.5 m/s (score)
| Sekar 2009; Eagam 2017; Wellen 2014 abstracts |
| Pediatric | Balloon angioplasty of recoarctation, Class I (LOE C): catheter systolic gradient >20 mmHg, or <20 mmHg in a univentricular heart; suitable anatomy in both | AHA 2011 |
| Single RV | Distal arch Vmax before Fontan 1.67 ± 0.51 / 1.81 ± 0.58 m/s SVR, MBTS / RVPAS, CW distal arch to descending aorta (n = 91, 96). At 6 y: 1.78 ± 0.48 / 1.84 ± 0.52 (Frommelt 2019, Table 1). | Frommelt 2014, Table 3 |
| Shunt in place | Retrograde diastolic flow, median 43% vs 0% (MBTS vs RVPAS) SVR trial, percentage of retrograde diastolic flow in the thoracic descending neo-aorta, median [IQR]: MBTS 43% [33–54%] post-Norwood and 39% [31–50%] pre-stage II; RVPAS 0% [0–0%] at both. | Frommelt 2012 |
| Fontan Fenestration Mean Gradient · relates loosely to the transpulmonary gradient (TPG) What it reflectsA fenestration lies between the systemic venous return and the pulmonary venous atrium and creates a controlled right-to-left shunt (AHA 2019, p. e236); Doppler across it theoretically estimates the TPG (Sanders 2025, abstract). The adolescent study's text does not describe how its gradient was measured. No guideline gives an echo threshold. | ||
| Fontan | R² 0.38 vs catheter TPG 59 fenestrated Fontan patients, echo within 6 months of catheterisation. Catheter TPG = 0.55 × mean gradient + 1.92; the echo gradient accounted for about 38% of TPG variance. The authors advise caution and direct catheter measurement. | Sanders 2025 abstract |
| Fontan, adolescents | Single LV 4.7 ± 1.8; RV or other 5.5 ± 2.0 mmHg FUEL baseline; a fenestration was present in about 1 in 5; n = 36 in each group. | Di Maria 2022, Table 3 |
| Group | Values | Source |
|---|---|---|
| AV-Valve Regurgitation · no validated single-ventricle grading How studies graded it
| ||
| Pediatric | Grade qualitatively Quantitative assessment (effective regurgitant area, vena contracta, regurgitant volume and fraction) is limited in children by multiple jets, inconsistent definitions of severity and poor reproducibility. Use colour assessment of the jet(s), atrial or ventricular dilation, and systolic flow reversal in the systemic or pulmonary veins. 3D quantification has not yet been validated in children. | ASE 2024, pp. 143–144 |
| Fontan | ≥Moderate: risk factor for long-term mortality | AHA 2019, Table 2 |
| Atrial Septum · before stage 1 | ||
| Pediatric | HLHS, intact or restrictive septum: septostomy Class IIa (LOE B); no gradient threshold Class IIa (LOE B): to decompress a hypertensive pulmonary venous chamber in HLHS with an intact or restrictive atrial communication. Class IIb (LOE B): septostomy may be considered to decompress a hypertensive pulmonary or systemic venous chamber, e.g. tricuspid atresia with a restrictive atrial communication, if needed before surgery. | AHA 2011 |
| Single RV | Before surgery, mean septal gradient, median 4.87 / 4.55 mmHg SVR baseline exam, MBTS and RVPAS arms before the Norwood: mean atrial septal defect pressure gradient, median (IQR) 4.87 (2.41, 7.50), n = 163, and 4.55 (1.97, 6.98), n = 168. A cohort value, not a definition of restriction. | Frommelt 2012, Table 2 |
| Glenn and Fontan Flow | ||
| After Glenn | No Doppler criteria found. Branch PA flow reversal: lower transplant-free survival Colour Doppler flow reversal in a branch PA after a superior cavopulmonary connection or Fontan, 21 vs 539 patients, one centre; such patients were also less likely to reach the Fontan in early childhood. | Spearman 2020 abstract |
| Surveillance After Fontan | ||
| All ages | Echo yearly; MRI every 3 y (child <12 y), 2–3 y (adolescent 12–18 y, adult >18 y) Proposed by consensus of the writing group (surveillance toolkit, Tables 13–16; age groups from the text, p. e261). 2D echo may track serial change in function between CMRs (p. e240). | AHA 2019, Table 13 |
| Adult | TTE every 12 months (ACHD physiological stage B, C), 6–12 (stage D) Physiological stages are defined in the guideline's Section 2.2. TTE is not sufficient to delineate the Fontan pathway to rule out thrombus; with newly progressive or severe hypoxemia or hypotension, advanced imaging (CMR, CT or transesophageal echo) to rule out thrombus or emboli (Class 1). | ACHD 2025, Table 42 |
| Group | Values | Source |
|---|---|---|
| Neo-Aortic Valve and Root · after Norwood (MBTS / RVPAS) | ||
| Pre-Fontan | Annulus area z 5.59 ± 2.54 / 5.12 ± 3.12; ≥moderate AR 2.5% / 4.6% Ascending aorta z 3.43 ± 2.32 / 3.10 ± 2.41. Annulus z-scores use normal native aortic valve data. | Frommelt 2014, Table 3 |
| 6 y | Annulus area z 6.39 ± 3.16 / 5.44 ± 2.79; ≥moderate AR 6.9% / 6.5% Ascending neo-aorta z 3.14 ± 2.43 / 2.96 ± 2.69; no severe regurgitation. | Frommelt 2019, Table 1 |
| HLHS, Fontan | Root z >2 in 98%; AR >mild in 3 of 53 53 HLHS patients after Fontan, born before 1995; median follow-up 9.2 years (5.1–21); neo-aortic regurgitation (AR) in 61%, progressing in 49%. | Cohen 2003 abstract |
| Global Longitudinal Strain (GLS) · %, one apical view, same vendor for serial studies How to measureRV and single-ventricular GLS may be useful in targeted populations; usually measured in a single apical view, reproducible on one platform; high frame rates (ASE 2024, pp. 137, 145). Values differ by software: compare like with like. | ||
| 14 mo, single RV | −12.4 ± 3.3 / −12.4 ± 3.9 SVR survivors at 14.3 ± 1.2 months, MBTS / RVPAS; one off-line speckle-tracking software (TomTec), images at 30 frames/s; apical segments excluded; measurable in 214 of 275. Circumferential −13.4 ± 4.3 / −12.2 ± 4.2 (Table 2, n = 182). | Hill 2015, Table 3 |
| Fontan | Single RV −14.25 ± 4.39; single LV −16.94 ± 4.64 Longitudinal strain from an apical view at the crux, Siemens SC2000 at 70–120 frames/s; 52 single RV, 48 single LV (P = .004). One centre. | Rios 2017, Table 1 |