Decreased Exercise Performance with Age in Children with Hypoplastic Left Heart Syndrome

Jenkins, Pamela C; Chinnock, Richard; Jenkins, Kathy J.; Mahle, William T.; Mulla, Neda F.; Sharkey, Angela M.; Flanagan, Michael F.

doi:10.1016/j.jpeds.2007.09.050

Cited by 36 publications

(40 citation statements)

References 15 publications

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“…Children with Fontan circulation have been reported to have reduced levels of physical activity (22,23) and an exercise capacity that is approximately 60-70% of that in healthy children (23)(24)(25)(26). Physical activity and exercise in the general paediatric population are accepted to be important preventive measures against acquired cardiovascular disease (27,28).…”

Section: Long-term Outcomementioning

confidence: 99%

“…Patients with Fontan circulation might experience an affected quality of life (37). Studies have reported a negatively affected quality of life (37)(38)(39)(40)(41)(42)(43) and reduced exercise performance even in patients without severe complications during surgical procedures and medical follow-ups (23,25,26,30). Also, it has been shown that patients with Fontan circulation have a lower quality of life compared with patients with less severe heart defects (38).…”

Section: Long-term Outcomementioning

confidence: 99%

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Impaired lung function in children and adolescents with Fontan circulation may improve after endurance training

et al. 2018

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Fontan patients have a restrictive lung pattern, reduced pulmonary diffusing capacity, and reduced maximal oxygen uptake compared with healthy controls. Endurance training may improve vital capacity in Fontan patients. The normal increase in pulmonary diffusing capacity with age and growth was reduced in Fontan patients, which is concerning. Apart from general health effects, exercise may improve lung function in young Fontan patients and should be encouraged.

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Section: Long-term Outcomementioning

confidence: 99%

Section: Long-term Outcomementioning

confidence: 99%

Impaired lung function in children and adolescents with Fontan circulation may improve after endurance training

et al. 2018

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“…Despite improved short- and medium-term outcomes, long-term morbidity and mortality continue to pose a challenge [10, 20, 24]. Systolic and diastolic ventricular performance are crucial determinants of cardiac output and are therefore intimately related to late morbidity and mortality [4, 6, 12].…”

Section: Introductionmentioning

confidence: 99%

Impact of Sildenafil on Echocardiographic Indices of Myocardial Performance After the Fontan Operation

et al. 2012

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The objective of this study was describe the impact of sildenafil on echocardiographic measures of myocardial performance in children and young adults with a functional single-ventricle physiology late after Fontan surgery. A double-blind, placebo-controlled, crossover trial was conducted in children and young adults after the Fontan operation at a single pediatric center. Subjects were randomized to receive placebo or sildenafil (20 mg tid) for 6 weeks. After a 6-week washout period, subjects were crossed for an additional 6 weeks. Each subject underwent an echocardiogram at the start and finish of each phase. A total of 27 subjects completed study testing at a mean age of 14.9 years and a mean time from Fontan surgery of 11.3 years. After sildenafil, subjects demonstrated improvement in their myocardial performance index (MPI; −0.051; 95% CI −0.095, −0.0077; p 0.02) and in the product of the velocity time integral (VTI) of the dominant outflow tract and the heart rate (HR; 110 cm × bpm; 95% CI 7.5, 220; p = 0.04). Measures of diastolic performance, including inflow velocities, myocardial velocities, and the ratio of blood pool velocity to myocardial velocity during passive inflow, did not change. In this cohort, there were significant improvements in both the MPI and the product of the VTI × HR after 6 weeks of treatment with sildenafil. These findings suggest that sildenafil may be a useful therapy to improve or maintain ventricular performance in select patients after the Fontan operation.

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“…[6] Exercise capacity and levels of daily physical activity have been shown to deteriorate through adolescence in Fontan subjects. [15–17] There is also felt to be an accelerating risk of cardiac complications,[18–20] as well as Fontan “failure” into adulthood, contributing to mortality and necessitating cardiac transplantation. Given the lack of rigorous, longitudinal assessment of functional health status, the rate of decline and associated factors are poorly understood.…”

Section: Discussionmentioning

confidence: 99%

The Relationship of Patient Medical and Laboratory Characteristics to Changes in Functional Health Status in Children and Adolescents After the Fontan Procedure

et al. 2013

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Despite hypothesized concerns about deterioration beginning in adolescence, longitudinal data and associated factors regarding standardized assessment of physical functioning are not available for Fontan patients. Parents who participated in the Fontan Cross-Sectional Study completed the Child Health Questionnaire at two time points for 245 subjects ages 6 to 18 years. Associations between change in Physical Functioning Summary Score and baseline patient, medical and laboratory characteristics (mean age 9.5±1.7 years) and follow-up patient and medical characteristics (mean age 16.2±1.6 years) were determined by regression analyses. Over a mean of 6.7±0.4 years, a small (not clinically important) but statistically significant decline in score from 46.2±11.7 to 44.5±12.1 (p<0.03) was noted. Subjects with higher baseline scores had a greater decline in score (r=−0.48; p<0.001). A multivariable model of patient and medical characteristics (R2=0.11) showed that a greater decline in score was significantly associated with interim development of asthma (n=13; parameter estimate [PE] −6.6; p<0.05) or other chronic respiratory, lung or breathing problems (n=13; PE −12.5; p<0.001), and the presence of protein-losing enteropathy at any time (n=12; PE −9.4; p=0.006). Change in score was not significantly associated with baseline laboratory measures of exercise capacity and ventricular characteristics and function. Therefore, although physical functioning may be stable during adolescence for many Fontan patients, deterioration occurs in some in association with respiratory conditions and protein-losing enteropathy. Further longitudinal study is necessary to better understand the relationship between clinical morbidities and functional health status as these patients transition into adulthood.

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Decreased Exercise Performance with Age in Children with Hypoplastic Left Heart Syndrome

Cited by 36 publications

References 15 publications

Impaired lung function in children and adolescents with Fontan circulation may improve after endurance training

Impaired lung function in children and adolescents with Fontan circulation may improve after endurance training

Impact of Sildenafil on Echocardiographic Indices of Myocardial Performance After the Fontan Operation

The Relationship of Patient Medical and Laboratory Characteristics to Changes in Functional Health Status in Children and Adolescents After the Fontan Procedure

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