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Cardiopulmonary exercise testing in Fontan patients: unmasking the secret of “Super-Fontans”
Session:
Comunicações Orais (Sessão 16) - Doença CV em Populações Especiais
Speaker:
Mariana Sousa Paiva
Congress:
CPC 2022
Topic:
K. Cardiovascular Disease In Special Populations
Theme:
30. Cardiovascular Disease in Special Populations
Subtheme:
30.3 Cardiovascular Disease in Special Populations: Pediatric Cardiology
Session Type:
Comunicações Orais
FP Number:
---
Authors:
Mariana Sousa Paiva; João Rato; Rita Reis Santos; Gonçalo l Cunha; Daniel a Gomes; Rita Lima; Rita Amador; Rita Bello; Susana Cordeiro; Sara Guerreiro; Sérgio Madeira; Luís Moreno; Anaí Durazzo; Miguel Mendes; Rui Anjos
Abstract
<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><strong><span style="font-family:"Times New Roman",serif">Background</span></strong><span style="font-family:"Times New Roman",serif">:</span></span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Times New Roman",serif">Exercise capacity is usually reduced in Fontan patients, however there is a subset of patients who have normal exercise capacity and better outcomes, the “Super-Fontans”. The aim of this study was to characterize a cohort of Fontan patients undergoing cardiopulmonary exercise testing (CPET) and identify predictors of better functional capacity. </span></span></span></p> <p> </p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><strong><span style="font-family:"Times New Roman",serif">Methods</span></strong><span style="font-family:"Times New Roman",serif">:</span></span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Times New Roman",serif">Single center retrospective analysis of consecutive Fontan patients aged <u>></u> 10 years old who underwent CPET, between March 2018 and May 2021.</span></span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Times New Roman",serif">Peak respiratory exchange ratio </span></span></span><u><span style="font-size:12.0pt"><span style="font-family:"Times New Roman",serif">></span></span></u><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Times New Roman",serif"> 1.05 defined maximal CPET. </span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Times New Roman",serif">Peak VO2 (pVO2) as a percentage of its predicted value was used as reference value to stratify patients in tertiles. </span></span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Times New Roman",serif">Patients in 3<sup>rd</sup> tertile, with a percentage of predicted pVO2 superior to 75%, were considered good-performers. </span></span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Times New Roman",serif">Blood tests and transthoracic echocardiogram (TTE) were performed on the same day. Additional data were collected from electronic charts. </span></span></span></p> <p style="text-align:justify"> </p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><strong><span style="font-family:"Times New Roman",serif">Results</span></strong><span style="font-family:"Times New Roman",serif">:</span></span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Times New Roman",serif"> In total, 49 patients were included (mean age 19±7 years old, 67% male) with intra or extracardiac conduit implanted in mean<em> </em>12±7 years prior to the CPET. </span></span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Times New Roman",serif">The most common primary diagnoses were tricuspid/pulmonary atresia (43%), followed by unbalanced complete AV septal defect (14%) and double inlet left ventricle (14%). 12 patients had a systemic right ventricle. All, except 5 patients, had preserved systolic ventricular function and 37% had moderate to severe AV regurgitation. </span></span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Times New Roman",serif">The majority had normal hemoglobin levels (median 15.6 g/dL), hepatic enzymes (median total bilirubin 0.8 mg/dL), renal function (median creatinine 0.8 mg/dL) and low NT-proBNP (median 122 pg/mL). </span></span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Times New Roman",serif">All patients had maximal CPET, median %VO2 at VT1 was 57% of peak and mean pVO2 was 66±14% of the predicted. Most patients (69%) showed exercise limitation due to cardiovascular cause, followed by O2 desaturation, present in 22% of CPETs. The age of Fontan completion was not associated with functional capacity (p=0.6).</span></span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Times New Roman",serif">The good-performer group comprised 13 patients (27%)</span><span style="font-family:"Times New Roman",serif">, all in sinus rhythm, of which 10 were physically active</span><span style="font-family:"Times New Roman",serif">. Compared with the remainder, this group had higher VO2 at VT1 (18.7 vs. 14.6, p=0.011) and VO2 at VT2 (25.9 vs. 22.1, p=0.019), both in mL/kg/min. Also, peak heart rate (% predicted) (90 vs. 80.5, p=0.028) was higher in this subgroup – <strong>fig.1</strong>. Conversely, differences on TTE parameters (GLS and AV valve regurgitation) and blood biomarkers were not statistically significant. On multivariable analysis, no single variable predicted better functional capacity.</span></span></span></p> <p style="text-align:justify"> </p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><strong><span style="font-family:"Times New Roman",serif">Conclusion</span></strong><span style="font-family:"Times New Roman",serif">:</span></span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:"Times New Roman",serif">In our Fontan cohort, most patients had reduced exercise capacity, largely due to cardiovascular dysfunction. However, “Super-Fontans” stood out as they had a higher anaerobic threshold illustrating their better physical condition. These findings highlight the role of regular physical activity in Fontan patients as a cornerstone for better functional capacity.</span><span style="font-family:Calibri,sans-serif"><span style="font-family:"Times New Roman",serif"> </span></span></span></p>
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