Abnormalities in Skeletal Muscle Metabolism in Cyanotic Patients with Congenital Heart Disease: A 31P Nuclear Magnetic Resonance Spectroscopy Study

1993 ◽  
Vol 85 (1) ◽  
pp. 105-109 ◽  
Author(s):  
I. Adatia ◽  
G. J. Kemp ◽  
D. J. Taylor ◽  
G. K. Radda ◽  
B. Rajagopalan ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Haemoglobin Concentration ◽  
Muscle Metabolism ◽  
Atp Synthesis ◽  
Resonance Spectroscopy ◽  
Cytosolic Ph ◽  
Control Subjects ◽  
Resting Muscle

1. Exercise tolerance is impaired in congenital heart disease. To examine the possible contribution of abnormalities in skeletal muscle bioenergetics, we used 31P nuclear magnetic resonance spectroscopy to investigate muscle metabolism in 10 subjects with congenital heart disease with cyanosis (median age 17.3 years) and in eight healthy age-matched control subjects. Spectra were collected from the gastrocnemius muscle at rest and during exercise and recovery. 2. In resting muscle there were significant elevations in cytosolic pH and in the cytosolic concentration of inorganic phosphate in the patients, and a strong positive correlation between cytosolic pH and blood haemoglobin concentration in all subjects. 3. During plantar flexion exercise the patients showed increased phosphocreatine depletion and cytosolic acidification over a shorter duration of exercise. The rise in calculated cytosolic ADP concentration was similar in both groups. 4. After cessation of exercise, the recovery half-times of phosphocreatine, ADP and phosphate were two to three times longer in the patients, and the initial rate of phosphocreatine resynthesis (a measure of the rate of mitochondrial ATP synthesis) was half the control value, consistent with a reduction in the effective maximum rate of oxidative ATP synthesis (expressed per volume of muscle). Also, recovery was faster in the young control subjects than in our earlier studies of older healthy control subjects. 5. The high phosphate concentration in resting muscle and the abnormalities found in exercise and recovery are consistent with a decrease in oxidative ATP synthesis due to reduced oxygen delivery by the blood in chronic hypoxaemia. The correlation between cytosolic pH and haemoglobin concentration remains to be explained.

Altered autonomic cardiac regulation in individuals with Down syndrome

2005 ◽  
Vol 289 (6) ◽  
pp. H2387-H2391 ◽  
Author(s):  
Ferdinando Iellamo ◽  
Alberto Galante ◽  
Jacopo M. Legramante ◽  
Maria Enrichetta Lippi ◽  
Claudia Condoluci ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Heart Disease ◽  
Congenital Heart ◽  
Sinus Node ◽  
Control Subjects ◽  
Arterial Blood ◽  
Cardiac Regulation ◽  
Autonomic Cardiac Regulation ◽  
And Control

We tested the hypothesis that individuals with Down syndrome, but without congenital heart disease, exhibit altered autonomic cardiac regulation. Ten subjects with Down syndrome (DS) and ten gender-and age-matched healthy control subjects were studied at rest and during active orthostatism, which induces reciprocal changes in sympathetic and parasympathetic traffic to the heart. Autoregressive power spectral analysis was used to investigate R-R interval variability. Baroreflex modulation of sinus node was assessed by the spontaneous baroreflex sequences method. No significant differences between DS and control subjects were observed in arterial blood pressure at rest or in response to standing. Also, R-R interval did not differ at rest. R-R interval decreased significantly less during standing in DS vs. control subjects. Low-frequency (LFNU) and high-frequency (HFNU) (both expressed in normalized units) components of R-R interval variability did not differ between DS and control subjects at rest. During standing, significant increase in LFNU and decrease in HFNU were observed in control subjects but not in DS subjects. Baroreflex sensitivity (BRS) did not differ between DS and control subjects at rest and underwent significant decrease on going from supine to upright in both groups. However, BRS was greater in DS vs. control subjects during standing. These data indicate that subjects with DS exhibit reduced HR response to orthostatic stress associated with blunted sympathetic activation and vagal withdrawal and with a lesser reduction in BRS in response to active orthostatism. These findings suggest overall impairment in autonomic cardiac regulation in DS and may help to explain the chronotropic incompetence typically reported during exercise in subjects with DS without congenital heart disease.

scholarly journals Serum Levels of Soluble ICAM-1 in Children with Pulmonary Artery Hypertension

2014 ◽  
Vol 41 (2) ◽  
pp. 159-164 ◽  
Author(s):  
Melahat Melek Oguz ◽  
Ayse Deniz Oguz ◽  
Cihat Sanli ◽  
Ayhan Cevik
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Artery ◽  
Congenital Heart ◽  
Pulmonary Artery Hypertension ◽  
Assay Method ◽  
Enzyme Linked Immunosorbent Assay ◽  
Control Subjects ◽  
Healthy Control ◽  
The Mean

This prospective cross-sectional study attempted to determine both the usefulness of the serum intercellular adhesion molecule-1 (ICAM-1) as a biomarker for pulmonary artery hypertension secondary to congenital heart disease and the nature of this marker's association with catheter angiographic findings. Our study included a total of 70 male and female children, comprising 30 patients with both pulmonary artery hypertension and congenital heart disease, 20 patients with congenital heart disease alone, and 20 healthy control subjects. Levels of ICAM-1 in plasma samples from all groups were measured by the enzyme-linked immunosorbent assay method. Cardiac catheterization was also performed in all patients. The mean serum ICAM-1 levels in pediatric patients who had congenital heart disease with and without pulmonary artery hypertension were 349.6 ± 72.9 ng/mL and 312.3 ± 69.5 ng/mL, respectively (P=0.002). In healthy control subjects, the mean serum ICAM-1 level was 231.4 ± 60.4 ng/mL. According to the results of this study, the ICAM-1 level of the pulmonary artery hypertension group was significantly higher than those of the congenital heart disease group and the healthy control group. Correlation analysis showed that ICAM-1 level was correlated with systolic and mean pulmonary artery pressures (r=0.62, P=0.001; r=0.57, P=0.001)—which are 2 important values used in diagnosis of pulmonary artery hypertension. Moreover, receiver operating characteristic analysis yielded consistent results for the prediction of pulmonary artery hypertension. Therefore, we conclude that ICAM-1 has potential use as a biomarker for the diagnosis and follow-up of pulmonary artery hypertension.

scholarly journals Magnetic resonance spectroscopy in congenital heart disease.

Heart ◽  
1996 ◽  
Vol 75 (6) ◽  
pp. 614-619 ◽  
Author(s):  
V. M. Miall-Allen ◽  
G. J. Kemp ◽  
B. Rajagopalan ◽  
D. J. Taylor ◽  
G. K. Radda ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Magnetic Resonance ◽  
Magnetic Resonance Spectroscopy ◽  
Congenital Heart ◽  
Resonance Spectroscopy

The Ventilatory Response to Hypoxia during Exercise in Cyanotic Congenital Heart Disease

1973 ◽  
Vol 45 (1) ◽  
pp. 99-105 ◽  
Author(s):  
M. R. H. Taylor
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Ventilatory Response ◽  
Arterial Oxygen Saturation ◽  
Haemoglobin Concentration ◽  
Cyanotic Congenital Heart Disease ◽  
Arterial Oxygen ◽  
Normal Adults ◽  
Ventilatory Response To Hypoxia

1. In contrast to the diminished ventilatory response to hypoxia which has been found at rest in cyanotic congenital heart disease, hyperventilation was noted on exercise in children who were cyanosed. 2. Sixteen children had low arterial oxygen saturations on exercise and thirteen of these hyperventilated by an amount similar to that reported in normal adults breathing hypoxic gas mixtures. 3. The three children who had little ventilatory response in relation to the increase of hypoxia during exercise all had a triad of long-standing cyanosis starting early in life, high haemoglobin concentration and low arterial oxygen saturation at rest in air.

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