Pyykkönen Henri Juhani, Rahkonen Otto, Tikkanen Heikki, Khanji Karim, Piirilä Päivi, Pitkänen-Argillander Olli
Faculty of Health Sciences, School of Medicine, University of Eastern Finland, Kuopio, Finland.
Department of Pediatric Cardiology, New Children's Hospital, Helsinki, Finland.
CJC Pediatr Congenit Heart Dis. 2024 Aug 23;3(6):265-271. doi: 10.1016/j.cjcpc.2024.08.002. eCollection 2024 Dec.
Cardiac output in Fontan circulation depends on systemic venous pressure, pulmonary vascular resistance, and ventricular function. Because myocardial function is dependent on coronary perfusion, we studied whether retrograde flow to aortic root in the mitral/aortic atresia subgroup of hypoplastic left heart syndrome (HLHS) affects cardiopulmonary performance.
We studied 26 stable Fontan patients (14.4 ± 2.4 years) with right (RV, n = 17) and left (LV, n = 9) systemic ventricle morphology. All RV patients had HLHS and were subdivided according to postnatal flow to the hypoplastic ascending aorta being antegrade (HLHS-A) or retrograde (HLHS-R) due to valve atresia. Physical activity was assessed by questionnaire (LASERI, a questionnaire for Finnish children regarding physical activity), cardiopulmonary exercise test (1-minute ramp protocol), body composition (Biacorpus RX 4000), and muscle fitness (EUROFIT). These data were correlated with the postnatal aorta size and current branch pulmonary artery size index (McGoon index).
Patients with HLHS-R seldom self-reported engagement in vigorous physical activity and had significantly lower cardiopulmonary performance (peak oxygen uptake [VOpeak]) than patients with LV morphology ( = 0.037), but not compared with patients with HLHS-A. Branch pulmonary artery size did not correlate with VOpeak. Patients with HLHS-R had most severe lung restrictions (forced vital capacity -score -3 ± 0.9, = 0.0073; forced expiratory volume in 1 second -score -3.3 ± 1.1, = 0.001).
Young Fontan patients with LV had better cardiopulmonary performance than patients with HLHS. Patients with HLHS-R were the least active and had the lowest VOpeak and most restrictive lungs. It is important to recognize postnatally single ventricle patients at high risk for inactivity to promote an active and healthy lifestyle.
Fontan循环中的心输出量取决于体静脉压力、肺血管阻力和心室功能。由于心肌功能依赖于冠状动脉灌注，我们研究了左心发育不全综合征（HLHS）二尖瓣/主动脉闭锁亚组中主动脉根部的逆向血流是否会影响心肺功能。
我们研究了26例稳定的Fontan患者（14.4±2.4岁），其体心室形态分为右心室（RV，n = 17）和左心室（LV，n = 9）。所有RV患者均患有HLHS，并根据出生后流向发育不全升主动脉的血流因瓣膜闭锁而呈顺行（HLHS-A）或逆行（HLHS-R）进行细分。通过问卷（LASERI，一份针对芬兰儿童身体活动的问卷）、心肺运动试验（1分钟斜坡方案）、身体成分（Biacorpus RX 4000）和肌肉适应性（EUROFIT）评估身体活动。这些数据与出生后主动脉大小和当前分支肺动脉大小指数（McGoon指数）相关。
HLHS-R患者很少自我报告参与剧烈体育活动，其心肺功能（峰值摄氧量[VOpeak]）显著低于LV形态的患者（P = 0.037），但与HLHS-A患者相比无差异。分支肺动脉大小与VOpeak无相关性。HLHS-R患者的肺限制最为严重（用力肺活量评分-3±0.9，P = 0.0073；第1秒用力呼气量评分-3.3±1.1，P = 0.001）。
年轻的Fontan患者中，LV患者的心肺功能优于HLHS患者。HLHS-R患者活动最少，VOpeak最低，肺部限制最严重。识别出生后单心室患者中缺乏运动的高风险人群，以促进积极健康的生活方式非常重要。
