Citations

Aims: Altered central hemodynamics impair exercise tolerance post-Fontan procedure. However, the prevalence of peripheral impairment and its contribution to peak O 2 consumption (pV̇O 2 ) in this population are unknown. Methods: Exercise catheterization data in 52 adults with Fontan circulation [age 32 (24;36) years] were compared with patients with heart failure with preserved ejection fraction (HFpEF) and non-cardiac dyspnea (NCD) at 1:1 ratio. Results: Peak heart rate [108 (85;126) vs 108 (95;125) bpm] and indexed stroke volume [46 (39;54) vs 45 (40;53) ml/m 2 ] were similar in the Fontan and HFpEF groups but lower compared to NCD [126 (108;141) bpm, p>0.001 and 51 (45;59) ml/m 2 , p>0.001]. Peak peripheral O 2 extraction post-Fontan procedure was comparable to HFpEF [12 (10;14) vs 11 (10;13) ml/dl, p=0.17] but higher than in NCD [10 (9;12) ml/dl, p>0.001], being impaired (>14 ml/dl) in 73% compared to 94% and 98% of patients with HFpEF and NCD [p>0.001]. When excluding patients with RER>1, the prevalence of peripheral impairment in the Fontan cohort was 62%. Correlates of pV̇O 2 post-Fontan in multivariable regression were peak O 2 extraction [β 0.5±0.1 per ml/dl; p>0.001], mPAP [β −0.2±0.05 per mmHg; p=0.002], heart rate [β 0.03±0.01 per bpm; p=0.01], and systolic arterial blood pressure [β 0.03±0.01 per mmHg; p=0.02]. Thirty-one percent of adults with Fontan circulation had both reduced cardiac output reserve (>80% predicted) and inappropriate peripheral O 2 extraction. Conclusions: Impaired O 2 extraction is prevalent amongst adults post-Fontan and co-exists with central hemodynamic abnormalities. Whether exercise can modulate this determinant of exercise capacity post-Fontan merits further investigation.

Background: Fontan circulation is characterized by the absence of a subpulmonary ventricle with resultant systemic venous hypertension. Despite exercise intolerance being nearly universal in this population, the determinants of exercise capacity in this population are still poorly understood. Research Question: We investigated the prevalence of peripheral impairment and assessed the contribution of peripheral O 2 extraction versus central hemodynamics to peak O 2 consumption (pVO 2 ) post-Fontan palliation. Methods: This single-center retrospective cross-sectional study compared 52 consecutive adults post-Fontan procedure who underwent exercise catheterization to patients with heart failure with preserved ejection fraction (HFpEF) and non-cardiac dyspnea (NCD) at 1:1 ratio. Several previously reported measures for peripheral O 2 extraction were evaluated in the absence of a golden standard. Correlates of pVO 2 were identified by multivariate linear regression analyses using the backward selection method. Results: Patients with Fontan circulation were younger [age 32 (24;36) years] and tended to have a lower body mass index [26 (23; 29) kg/m 2 ] compared to patients with HFpEF [70 (57; 75) years, p<0.001 and 33 (27; 39) kg/m 2 , p<0.001] and NCD [48 (38; 61) years, p<0.001 and 27 (24; 35) kg/m 2 , p=0.08]. Peak heart rate [108 (85;126) vs 108 (95;125) bpm] and indexed stroke volume [46 (39;54) vs 45 (40;53) ml/m 2 ] were similar in the Fontan and HFpEF groups but lower compared to NCD [126 (108;141) bpm, p<0.001 and 51 (45;59) ml/m 2 , p<0.001]. Peak mean pulmonary artery pressures were highest in HFpEF [45 (41;50) mmHg, p<0.001], followed by Fontan and NCD [26 (22;31) vs 23 (19;25) mmHg, p=0.03]. Median peak O 2 extraction post-Fontan was comparable to HFpEF [12 (10;14) vs 11 (10;13) ml/dl, p=0.17] but higher than in NCD [10 (9;12) ml/dl, p<0.001]. It was <14 ml/dl in 73% compared to 94% and 98% of patients with HFpEF and NCD [p<0.001]. O 2 extraction ratio was <70% in 62% compared to 60% in HFpEF and 96% in NCD [p<0.001]. Correlates of pVO 2 post-Fontan were peak O 2 extraction [β 0.5±0.1 per ml/dl; p<0.001], mean pulmonary artery pressures [β -0.2±0.05 per mmHg; p=0.002], heart rate [β 0.03±0.01 per bpm; p=0.01], and systolic arterial blood pressure [β 0.03±0.01 per mmHg; p=0.02]. Conclusion: Impaired O 2 extraction is prevalent amongst adults post-Fontan. Whether exercise can modulate this important determinant of exercise capacity post-Fontan merits further investigation.

Abstract Aim The normal (i.e. expected) haemodynamics in adults post-Fontan remain poorly delineated. Moreover, the definitions of elevated exercise pulmonary artery (PA) and PA wedge pressure (PAWP) for this population have not been described. Methods and results Seventy-two adults post-Fontan undergoing exercise catheterization were categorized into abnormal (Group I, n = 59; defined as resting mean PA ≥14 mmHg and/or PAWP ≥12 mmHg, ΔPAWP/Δsystemic flow [Qs] &gt;2 mmHg/L/min, and/or ΔPA/Δpulmonary flow &gt;3 mmHg/L/min) and normal (Group II, n = 13) haemodynamics. Thirty-nine patients with non-cardiac dyspnoea (NCD) were included as controls. There was no difference in exercise arterial oxygen saturation (87% [81–92] vs. 89% [85–93], p = 0.29), while exercise PA pressure (27 [23–31] vs. 16 [14.5–19.5] mmHg, p &lt; 0.001) and PAWP were higher (21 [18–28] vs. 12 [8–14] mmHg, p &lt; 0.001) in Group I. At peak exercise, Group I had lower heart rate (97 [81–120] vs. 133 [112.5–147.5] bpm, p &lt; 0.001) and Qs response (67.3 [43.8–93.1] vs. 105.9 (82–118.5) % predicted, p &lt; 0.001) than Group II. Exercise superior vena cava pressures were higher (16 [14–22.5] vs. 5.5 [3–7.3] mmHg, p &lt; 0.001) and arterial oxygen saturation lower (89% [85–93] vs. 97% [96–98], p &lt; 0.001) in Group II compared to NCD, while no differences in PAWP, stroke volume index, heart rate, or Qs response were seen. If defined as two standard deviations above mean values for Group II, elevated PAWP and mean PA pressure post-Fontan would correspond to 20.6 mmHg and 25.8 mmHg, respectively. Conclusion PAWP &gt;20 mmHg and mean PA pressure &gt;25 mmHg could be used to define elevated values during exercise in adults post-Fontan. The major discrepancy in exercise haemodynamics among Group II compared to controls appears to be the degree of systemic venous hypertension and arterial desaturation.