Postprandial cardiac autonomic function in Prader–Willi syndrome

Abstract

Summary ContextIndividuals with Prader-Willi syndrome (PWS) have a high cardiovascular risk, the mechanism of which is unclear. There may be dysfunction in the autonomic nervous system (ANS) in PWS. Objective To measure, as indicators of cardiac autonomic function, postprandial heart rate variability (HRV) and arterial stiffness in adults with PWS. Methods Ten adults with PWS were compared with 11 matched healthy obese subjects and 9 healthy lean subjects. Electrocardiographic traces and arterial stiffness were recorded over a period of 10 minutes at −60, 0, 30, 60, 120 and 240 minutes after consumption of a standardized 600--kCal breakfast. Frequency domain analysis was performed using fast Fourier transform to estimate power spectral density in the full spectrum and in low--frequency (LF 0·04-0·15 Hz) and high--frequency (HF 0·15-0·40 Hz) bands. Results ANCOVA revealed a reduced LF HRV meal response in adults with PWS compared with obese controls, with no differences in HF HRV, LF/HF ratio, heart rate, total power or arterial stiffness meal responses. Conclusions This study assessed cardiac autonomic function in adults with PWS compared with matched obese and lean subjects in response to a meal. Results suggest impaired postprandial ANS responsiveness in PWS, which could contribute to both the known increased cardiovascular risk and obesity.

Cardiac autonomic regulation in autism and Fragile X syndrome: A review.

“…Because Prader-Willi syndrome is the most common genetic form of obesity, it has been used as a model to explore autonomic contributions to weight and metabolic problems. For example, Purtell et al (2013) examined cardiac activity before and after eating a meal to determine whether impaired autonomic responsiveness contributes to excessive appetite in Prader-Willi syndrome. The heart rate and vagal estimates of adults with Prader-Willi syndrome ( n = 10) did not differ from those of obese and lean controls matched on age, gender, and BMI, although low frequency spectral power (an index of sympathetic activity) was reduced, possibly suggesting an impaired sympathetic meal response in Prader-Willi syndrome (Purtell et al, 2013).…”

“…For example, Purtell et al (2013) examined cardiac activity before and after eating a meal to determine whether impaired autonomic responsiveness contributes to excessive appetite in Prader-Willi syndrome. The heart rate and vagal estimates of adults with Prader-Willi syndrome ( n = 10) did not differ from those of obese and lean controls matched on age, gender, and BMI, although low frequency spectral power (an index of sympathetic activity) was reduced, possibly suggesting an impaired sympathetic meal response in Prader-Willi syndrome (Purtell et al, 2013). Others have examined cardiac responses to exercise as a potential contributor to obesity in the syndrome, with conflicting results.…”

Obesity and Prader-Willi Syndrome Affect Heart Rate Recovery from Dynamic Resistance Exercise in Youth

“…However, the delayed HRR in young people with PWS at both 30 and 60 s indicate that both parasympathetic and sympathetic function during recovery may be compromised. Therefore, HRR in young people with PWS may be affected by compromised parasympathetic and sympathetic function inherent to the syndrome, as earlier hypothesized by previous researchers [1,9,10,11]. …”

“…In addition to having distinct morphological features such as high fat mass, low lean mass and short stature, people with PWS present with hypotonia, poor muscle strength, low cardiovascular capacity and poor cardiovascular fitness [5,6,7,8]. Additionally, previous studies evaluating ANS dysregulation in PWS are equivocal and it remains unclear whether detriments are a result of parasympathetic [9,10] or sympathetic [1,11] inactivity.…”

Cardiac autonomic control during non‐REM and REM sleep stages in paediatric patients with Prader‐Willi syndrome

“…Along these lines, heart rate variability (HRV) has been used in different populations as a strong and independent predictor of cardiac mortality and diseases (Villareal, Liu, & Massumi, 2002), which could also be useful in PWS patients. HRV alterations were observed in PWS patients postprandially (Purtell et al, 2013), and recently a case study presented a patient with baroreflex dysfunction (Kaur, Srivastav, Jaryal, & Deepak, 2016 (Tobaldini et al, 2013). Cardiac autonomic imbalance during sleep is possibly implicated in adverse cardiovascular events that occur mainly at the end of the night period (Drager et al, 2009).…”