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Background: Adults with complex congenital heart disease (CHD) have reduced aerobic capacity and impaired muscle function. We therefore hypothesized that patients have a lower skeletal muscle mass and higher fat mass than controls.

Methods: Body composition was examined with full body Dual-Energy x-ray Absorptiometry (DXA) in 73 patients with complex CHD (mean age 35.8 ± 14.3, women n = 22) and 73 age and sex matched controls. Patients fulfilling criteria for low skeletal muscle mass in relation to their height and fat mass were defined as sarcopenic.

Results: Male patients (n = 51) were shorter (177.4 ± 6.6 cm vs. 180.9 ± 6.7 cm, p = 0.009) and weighed less (76.0 ± 10.8 kg vs. 82.0 ± 12.4 kg, p = 0.01) than controls. Also, patients had a lower appendicular lean mass-index (ALM-index) (7.57 ± 0.97 kg/m2 vs. 8.46 ± 0.90 kg/m2, p < 0.001). Patients’ relative tissue fat mass (27.9 ± 7.0% vs. 25.4 ± 8.6%, p = 0.1) did not differ. Forty-seven percent of the men (n = 24) were classified as sarcopenic.

Female patients (n = 22) were also shorter (163.5 ± 8.7 cm vs. 166.7 ± 5.9 cm, p = 0.05) but had a higher BMI (25.7 ± 4.2 vs. 23.0 ± 2.5, p = 0.02) than controls. Patients also had a lower ALM-index (6.30 ± 0.75 vs. 6.67 ± 0.55, p = 0.05), but their relative body fat mass (40.8 ± 7.6% vs. 32.0 ± 7.0%, p < 0.001) were higher. Fifty-nine percent of the women (n = 13) were classified as sarcopenic.

Conclusions: The body composition was altered toward lower skeletal muscle mass in patients with complex CHD. Approximately half of the patients were classified as sarcopenic. Contrary to men, the women had increased body fat and a higher BMI. Further research is required to assess the cause, possible adverse long-term effects and whether sarcopenia is preventable or treatable.

Aims: The majority of children with complex congenital heart disease (CHD) survive into adulthood due to advances in medical care. Adult patients with CHD have an increased incidence of diagnoses related to ageing such as heart failure, dementia, cancer and sarcopenia, despite a relatively low age. They also have a shorter life expectancy. It is unknown if their bone structures also show signs of premature ageing. We therefore investigated Bone Mineral Content (BMC) and bone mineral density (BMD) in an adult population with complex CHD.

Methods: The total body BMC and BMD was examined using dual energy X-ray absorptiometry (DXA) in 73 adults with complex CHD (mean age 35.8 ± 14.3, women n = 22) and 73 age and sex matched controls.

Results: The adults with complex CHD had lower total body BMC (2.6 ± 0.5 kg vs. 2.9 ± 0.5 kg, p < 0.001) and BMD (1.18 ± 0.12 g/cm² vs. 1.26 ± 0.11 g/cm², p < 0.001) compared to controls. BMD was lower for patients with single ventricle physiology and for the other complex diagnoses, and it persisted after correction for most common risk factors for osteoporosis.

Conclusion: Adults with complex CHD have reduced total body BMC and BMD compared to healthy controls. These results are a sign of frailty that conforms with other previously reported signs of premature ageing. The risk of osteoporosis is low in our relatively young population, but it is assumed to increase with ageing. We recommend that clinicians pay close attention to risk factors for osteoporosis, and are generous in administering DXA-measurements in order to prevent future fractures among adults with complex CHD.

Background: Adults with congenital heart disease (CHD) have a low muscle mass (measured with Dual energy x-ray absorptiometry). Our aims were to confirm these results using peripheral quantitative computed tomography (pQCT), and to study the relationship between muscle cross sectional area (CSA) and isometric muscle strength. 

Methods: In this cross sectional study, we performed pQCT and tested isometric muscle strength in the forearm and calf of 49 adults with complex CHD and 49 age and sex matched controls (for each group: mean age 36±15.5 years; n=23 females, 47%). 

Results: In the forearm, after adjusting for height, patients had lower muscle CSA and lower isometric strength compared to controls (women: 24.1±6.7 vs. 26.2±6.7 cm2, p=0.05 and 30.0±1.0 vs. 33.4±1.0 kg, p=0.03; men: 36.0±1.2 vs. 42.5±1.2 cm2, p=0.001 and 47.2±1.9 vs. 53.4±1.9 kg, p=0.03). In addition, both patients and controls had strong correlations between muscle CSA and isometric strength. In the calf, female patients did not have lower muscle CSA than controls after correcting for height (60.5±1.4 vs. 62.7±1.4cm2, p=0.3), whereas men did (69.9±1.7 vs. 80.3±1.7 cm2, p<0.001). Isometric strength in the calf was lower in female patients than controls (487±35 vs. 614±35 N, p=0.02) but not in males (588±44 vs. 697±44 N, p=0.08), and there was no correlation with muscle CSA. 

Conclusion: In conclusion, adults with complex CHD have low forearm muscle CSA as assessed with pQCT. Grip strength correlates well with forearm muscle CSA and may serve as an indirect measurement of muscle mass.

Objective 

To study bone mineral density (BMD) and skeletal strength in adults with complex congenital heart disease (CHD) in comparison to a matched control group.

Methods

Peripheral quantitative computed tomography (pQCT) was performed on standardized sites of the radius and tibia in 49 adults with complex CHD, and in 49 age and sex matched controls (n = 23 [47 %] female, mean age 36 ± 15.5 years.) Strength for lateral and anterior acting forces in the radius and tibia were presented, respectively, in terms of x- and y-Strength-Strain Index (SSI).

Results 

Patients had similar total BMD as controls in both the radius (807 ± 82 vs. 792 ± 75 mg/cm3, p = 0.3) and tibia (663 ± 86 vs.689 ± 67 mg/cm3, p = 0.1). In the radius, patients had a lower xSSI than controls (154 ± 46 vs. 175 ± 54 mm3, p = 0.04) but a similar ySSI (178 ± 58 vs. 195 ± 55 mm3, p = 0.1). In the tibia, patients had a lower xSSI (1492 ± 399 vs. 1780 ± 372 mm3, p < 0.001) as well as ySSI (1066 ± 304 vs. 1250 ± 281, p = 0.002). In a multiple linear regression model, only height was independently associated with decreased skeletal strength in patients tibia in terms of xSSI [F(2,46) = 40.002, p < 0.001, R2 = 0.635]. Being a patient, with single ventricle physiology or other diagnose of complex CHD, was independently associated with decreased tibia xSSI [F(2,95) = 7.085, p = 0.001, η2 = 0.13].

Conclusion 

Adults with complex CHD had reduced strength in the radius and tibia, despite a normal BMD as assessed with pQCT. Whether their reduced bone strength entails increased risk of future fractures is currently unknown.