Mechanism for temporal changes in exercise capacity after Fontan palliation: Role of Doppler echocardiography.

The objective was to better understand Doppler hemodynamics and exercise capacity in patients with Fontan palliation by delineating the hemodynamic mechanism for temporal changes in their peak oxygen consumption (V̇o2). We performed a retrospective review of adult Fontan patients with systemic left ventricle (LV) who underwent serial transthoracic echocardiograms (TTE) and cardiopulmonary exercise tests (CPET) at Mayo Clinic in 2000-2015. TTE and CPET data were used (1) to determine agreement between V̇o2 and Doppler-derived LV function indices (eg, stroke volume index [SVI] and cardiac index [CI]) and (2) to determine agreement between temporal changes in peak V̇o2 and LV function indices. Seventy-five patients (44 men; 59%) underwent 191 pairs of TTE and CPET. At baseline, mean age was 24±3 years, peak V̇o2 was 22.9±4.1 mL/kg/min (63±11 percent predicted), SVI was 43±15 mL/m2, and CI was 2.9±0.9 L/min/m2. Peak V̇o2 correlated with SVI (r=0.30, P<.001) and with CI (r=0.45, P<.001) in the 153 pairs of TTE and CPET in patients without cirrhosis. Temporal changes in percent predicted peak V̇o2 correlated with changes in SVI (r=0.48, P=.005) and CI (r=0.49, P=.004) among the 33 patients without interventions during the study. In the 19 patients with Fontan conversion, percent predicted peak V̇o2 and chronotropic index improved. Overall, there was a temporal decline in peak V̇o2 that correlated with decline in Doppler SVI. In the patients who had Fontan conversion operation, there was a temporal improvement in peak V̇o2 that correlated with improvement in chronotropic index.

Egbe A ，Khan AR ，Miranda WR ，Ammash NM ，Warnes CA ，Said SS ，Taggart NW ，Akintoye E ，Veldtman GR ，Connolly HM ... -  《-》

Role of Doppler echocardiography for cardiac output assessment in Fontan patients.

To determine (1) correlation between Doppler stroke volume index (SVI) and cardiac magnetic resonance imaging (CMRI) SVI and (2) association between Doppler SVI and Fontan-associated diseases (FAD) and Fontan failure. Review of Fontan patients who underwent same-day CMRI and transthoracic echocardiography (TTE), 2005 to 2015. We defined FAD as cardiac thrombus, protein-losing enteropathy, arrhythmia, and hospitalization for heart failure. Fontan failure was defined as Fontan conversion or revision, heart transplantation or listing, or death. Fifty-three patients with systemic left ventricle (LV) underwent 86 sets of TTE/CMRI. Mean (SD) age 31 (6) years. SVI (45 [16] vs 42 [13] mL/m2), CI (3.0 [1.1] vs 2.8 [0.8] L min-1 m-2), and ejection fraction (53 [4]% vs 51 [5]%) were similar for both modalities (P>.05 for all). Doppler SVI correlated with CMRI (r=0.68; P<.001). Sixteen patients had cirrhosis, and these patients had a higher CI (3.9 [0.9] vs 2.8 [1.0] L min-1 m-2; P<.01). Among the 37 patients without cirrhosis, Doppler SVI <39 mL/m2 was associated with FAD (odds ratio [OR], 2.11; 95% confidence limit, 1.26-3.14; P=.02); Fontan failure was more common in patients with CI was <2.5 L min-1 m-2 (3/9 [33%] vs 0/28 [0%], P=.01). Another 11 patients with systemic right ventricle (RV) underwent 17 sets of TTE/CMRI, mean (SD) age 17 (3) years, and CMRI SVI also correlated with Doppler SVI (r=0.75; P<.001). Doppler SVI correlated with CMRI SVI in patients with systemic LV and systemic RV. The association between output measures (SVI and CI) and FAD were seen only in single LV patients (single RV patients not assessed for this outcome due to small numbers). An association between low Doppler CI and Fontan failure was suggested in a small number of single LV patients.

Egbe A ，Khan AR ，Khan SF ，Anavekar NS ，Said SM ，Young PM ，Akintoye E ，Miranda WR ，Al-Otaibi MN ，Veldtman GR ，Connolly HM ... -  《-》

Cardiopulmonary exercise testing in the evaluation of liver disease in adults who have had the Fontan operation.

Liver disease (LD) is a long-term complication in patients with a single ventricle who have had the Fontan operation. A decline in cardiopulmonary exercise testing (CPET) variables is associated with increased risk of hospitalization, but its association with LD is unknown. To determine the association between CPET variables and LD in adults who have had the Fontan operation. We retrospectively reviewed the medical records from two tertiary institutions. We identified 114 adults (≥18 years; mean 30.9±7.4 years) who had undergone the Fontan operation: 56% were women; 63% had total cavopulmonary connection; 66% had New York Heart Association (NYHA) class I status; 42% had arrhythmias; 22% had systemic right ventricle; and 35% had ventricular dysfunction. Of 81 patients with liver-imaging data, 41% had LD (i.e. imaging evidence of cirrhosis, with or without portal hypertension, splenomegaly or varices). There were no differences in clinical or echocardiographic variables between those with and without LD. Among the 58 patients with CPET data, mean peak oxygen consumption (VO2) was 18.6±5.7mL/kg/min, per-cent-predicted peak VO2 was 53.9±15.5%, peak oxygen pulse was 9.3±2.9mL/beat and per-cent-predicted peak oxygen pulse was 82.6±21.5%. Of the 44 patients with liver and CPET data, each standard deviation decrease in per-cent-predicted peak VO2 (16%) and per-cent-predicted peak oxygen pulse (22%) was associated with a 2.3-fold increase in the odds of LD, after adjusting for NYHA, institution and Fontan type (P=0.04). Similarly, each standard deviation decrease in per-cent-predicted peak VO2 and oxygen pulse was associated with an estimated 5.9-year and 4.9-year earlier onset of LD, respectively (P>0.05). Decline in per-cent-predicted peak VO2 and oxygen pulse was associated with increased odds of LD in adults who had undergone the Fontan operation. Our study supports more rapid hepatic evaluation among patients with abnormal or worsening CPET variables.

Agarwal A ，Cunnington C ，Sabanayagam A ，Zier L ，McCulloch CE ，Harris IS ，Foster E ，Atkinson D ，Bryan A ，Jenkins P ，Dua J ，Parker MJ ，Karunaratne D ，Moore JA ，Meadows J ，Clarke B ，Hoschtitzky JA ，Mahadevan VS ... -  《-》

Impact of age at Fontan completion on postoperative hemodynamics and long-term aerobic exercise capacity in patients with dominant left ventricle.

Shiraishi S ，Yagihara T ，Kagisaki K ，Hagino I ，Ohuchi H ，Kobayashi J ，Kitamura S ... -  《-》

When is the right time for Fontan conversion? The role of cardiopulmonary exercise test.

To determine if Fontan conversion (FC) resulted in improvement in exercise capacity (EC), and to determine the role of cardiopulmonary exercise test (CPET) in risk stratification of patients undergoing FC. A retrospective review of patients who underwent CPET prior to FC at Mayo Clinic from 1994 to 2014. The patients who also underwent post-operative CPET were selected for the analysis of improvement in EC defined as 10% increase in baseline peak oxygen consumption (VO2). 75 patients CPET prior to FC; mean age 24±6years; 44 males (59%); and 51 (68%) were in NYHA III/IV prior to FC. Pre-operative peak VO2 was 15.5±3.4ml/kg/min. A comparison of pre- and post-FC CPET data was performed using 42 patients (56%) that underwent CPET after FC. Improvement in EC occurred in 18 of 42 patients (43%). Baseline peak VO2 >14ml/kg/min was associated with improved EC (hazard ratio [HR] 1.85; P=.02). Improvement in New York Heart Association (NYHA) class occurred in 12 (67%) patients with improved EC vs 2 (8%) without improved EC. Improvement in NYHA class was more likely to occur in patients with improved EC compared to those without improvement EC (odds ratio 4.11, P=.01). There were 10 (13%) perioperative deaths, and baseline peak VO2 ≤14ml/kg/min was predictive of perioperative mortality (HR 3.74; P<.001). Baseline peak VO2 was predictive of perioperative survival, and improvement in EC. Performance on CPET in failing Fontan patients might be a useful clinical parameter in determining appropriate timing of FC.

Egbe AC ，Connolly HM ，Dearani JA ，Bonnichsen CR ，Niaz T ，Allison TG ，Johnson JN ，Poterucha JT ，Said SM ，Ammash NM ... -  《-》