In myotonic dystrophy type 2 (DM2/PROMM), cardiac muscle involvement is usuall y more benign than in DM1, but clinically severe cardiomyopathy has been reporte d in some patients. Using a novel method of magnetic reso...In myotonic dystrophy type 2 (DM2/PROMM), cardiac muscle involvement is usuall y more benign than in DM1, but clinically severe cardiomyopathy has been reporte d in some patients. Using a novel method of magnetic resonance spectroscopy (MRS ), we examined the left ventricular myocardium and the left gastrocnemius muscle in 11 unselected DM2/ PROMM patients without overt cardiac disease. Data on car diac morphology and function were obtained by gradient echo two dimensional cin e magnetic resonance imaging (MRI); no significant differences were found betwee n DM2 patients and healthy controls, but using a median split approach older pat ients showed mildly increased left ventricular (LV) volumes, i.e., 59%increase of end systolic volume index (ESVI) and 35%increase of enddiastolic volume ind ex (EDVI), and an increase of LV mass (26%). On cardiac MRS, DM2/PROMM patients showed a reduction of phosphocreatine (PCr) and adenosine triphosphate (ATP) by 25 and 20%compared to matched healthy controls. No significant differences wer e found between younger and older patients. In skeletal muscle of the DM2 patien ts, no significant decrease of PCr and ATP concentrations was found. However, in older patients, who commonly show overt hip flexor muscle weakness, we observed reduced values for PCr and ATP. Our MRS and MRI findings reveal evidence for su bclinical cardiomyopathy in DM2/PROMM patients without overt heart disease. Futu re prospective studies are needed to clarify the risk of developing overt cardia c disease in DM2 and to define prognostic factors.展开更多
The physiology of isolated partially anomalous pulmonary venous connection of a single pulmonary vein has yet to be fully characterized. This study assessed the magnitude of the left-to-right shunt and right ventricul...The physiology of isolated partially anomalous pulmonary venous connection of a single pulmonary vein has yet to be fully characterized. This study assessed the magnitude of the left-to-right shunt and right ventricular(RV) dilation from a single anomalous pulmonary vein using cardiac magnetic resonance imaging. Subjects with >1 anomalous pulmonary vein or associated lesions, including atrial septal defects, were excluded. In the 6 subjects identified, the median pulmonary-to-systemic flow ratio was 1.55(range 1.3 to 1.6). The mean RV end-diastolic volume indexed to body surface area in the subjects was significantly larger than in a normal reference cohort(108±16 vs 78±18 cm3/m2, p=0.0009) and greater than the upper limit of normal in all 6 subjects. Older age did not correlate with increased magnitude of shunting(r=0.3, p=0.5), but increased age did correlate with RV end-diastolic volume indexed to body surface area(r=0.96, p=0.01). Isolated partially anomalous pulmonary venous connection with only 1 vein connecting anomalously results in a modest left-to-right shunt and mild RV dilation.展开更多
Objective： Comparison of global end-diastolic volume index （GEDVI） obtained by femoral and jugular transpulmonary thermodilution （TPTD） indicator injections using the EV1000NolumnView device （Edwards Lifesci- e...Objective： Comparison of global end-diastolic volume index （GEDVI） obtained by femoral and jugular transpulmonary thermodilution （TPTD） indicator injections using the EV1000NolumnView device （Edwards Lifesci- ences, Irvine, USA）. Methods： In an 87-year-old woman with hypovolemic shock and equipped with both jugular and femoral vein access and monitored with the EV1000NolumeView device, we recorded 10 datasets, each comprising duplicate TPTD via femoral access and duplicate TPTD （20 ml cold saline） via jugular access. Results： Mean femoral GEDVI （（674.6±52.3） ml/m2） was significantly higher than jugular GEDVI （（552.3±69.7） ml/m2）, with P=-0.003. Bland-Airman analysis demonstrated a bias of （＋122±61） ml/m2, limits of agreement of -16 and ＋260 ml/m2, and a percentage error of 22%. Use of the correction-formula recently suggested for the PiCCO device significantly reduced bias and percentage error. Similarly, mean values of parameters derived from GEDVI such as pulmonary vascular permeability index （PVPI; 1.244±0.101 vs. 1.522±0.139; P〈0.001） and global ejection fraction （GEF; （24.7±1.6）% vs. （28.1±1.8）%; P〈0.001） were significantly different in the case of femoral compared to jugular indicator injection. Fur- thermore, the mean cardiac index derived from femoral indicator injection （（4.50±0.36） L/（min.m2）） was significantly higher （P=0.02） than that derived from jugular indicator injection （（4.12±0.44） L/（min.m2））, resulting in a bias of （＋0.38±0.37） L/（min.m2） and a percentage error of 19.4%. Conclusions： Femoral access for indicator injection results in markedly altered values provided by the EV1000NolumeView , particularly for GEDVI, PVPI, and GEF.展开更多
文摘In myotonic dystrophy type 2 (DM2/PROMM), cardiac muscle involvement is usuall y more benign than in DM1, but clinically severe cardiomyopathy has been reporte d in some patients. Using a novel method of magnetic resonance spectroscopy (MRS ), we examined the left ventricular myocardium and the left gastrocnemius muscle in 11 unselected DM2/ PROMM patients without overt cardiac disease. Data on car diac morphology and function were obtained by gradient echo two dimensional cin e magnetic resonance imaging (MRI); no significant differences were found betwee n DM2 patients and healthy controls, but using a median split approach older pat ients showed mildly increased left ventricular (LV) volumes, i.e., 59%increase of end systolic volume index (ESVI) and 35%increase of enddiastolic volume ind ex (EDVI), and an increase of LV mass (26%). On cardiac MRS, DM2/PROMM patients showed a reduction of phosphocreatine (PCr) and adenosine triphosphate (ATP) by 25 and 20%compared to matched healthy controls. No significant differences wer e found between younger and older patients. In skeletal muscle of the DM2 patien ts, no significant decrease of PCr and ATP concentrations was found. However, in older patients, who commonly show overt hip flexor muscle weakness, we observed reduced values for PCr and ATP. Our MRS and MRI findings reveal evidence for su bclinical cardiomyopathy in DM2/PROMM patients without overt heart disease. Futu re prospective studies are needed to clarify the risk of developing overt cardia c disease in DM2 and to define prognostic factors.
文摘The physiology of isolated partially anomalous pulmonary venous connection of a single pulmonary vein has yet to be fully characterized. This study assessed the magnitude of the left-to-right shunt and right ventricular(RV) dilation from a single anomalous pulmonary vein using cardiac magnetic resonance imaging. Subjects with >1 anomalous pulmonary vein or associated lesions, including atrial septal defects, were excluded. In the 6 subjects identified, the median pulmonary-to-systemic flow ratio was 1.55(range 1.3 to 1.6). The mean RV end-diastolic volume indexed to body surface area in the subjects was significantly larger than in a normal reference cohort(108±16 vs 78±18 cm3/m2, p=0.0009) and greater than the upper limit of normal in all 6 subjects. Older age did not correlate with increased magnitude of shunting(r=0.3, p=0.5), but increased age did correlate with RV end-diastolic volume indexed to body surface area(r=0.96, p=0.01). Isolated partially anomalous pulmonary venous connection with only 1 vein connecting anomalously results in a modest left-to-right shunt and mild RV dilation.
文摘Objective： Comparison of global end-diastolic volume index （GEDVI） obtained by femoral and jugular transpulmonary thermodilution （TPTD） indicator injections using the EV1000NolumnView device （Edwards Lifesci- ences, Irvine, USA）. Methods： In an 87-year-old woman with hypovolemic shock and equipped with both jugular and femoral vein access and monitored with the EV1000NolumeView device, we recorded 10 datasets, each comprising duplicate TPTD via femoral access and duplicate TPTD （20 ml cold saline） via jugular access. Results： Mean femoral GEDVI （（674.6±52.3） ml/m2） was significantly higher than jugular GEDVI （（552.3±69.7） ml/m2）, with P=-0.003. Bland-Airman analysis demonstrated a bias of （＋122±61） ml/m2, limits of agreement of -16 and ＋260 ml/m2, and a percentage error of 22%. Use of the correction-formula recently suggested for the PiCCO device significantly reduced bias and percentage error. Similarly, mean values of parameters derived from GEDVI such as pulmonary vascular permeability index （PVPI; 1.244±0.101 vs. 1.522±0.139; P〈0.001） and global ejection fraction （GEF; （24.7±1.6）% vs. （28.1±1.8）%; P〈0.001） were significantly different in the case of femoral compared to jugular indicator injection. Fur- thermore, the mean cardiac index derived from femoral indicator injection （（4.50±0.36） L/（min.m2）） was significantly higher （P=0.02） than that derived from jugular indicator injection （（4.12±0.44） L/（min.m2））, resulting in a bias of （＋0.38±0.37） L/（min.m2） and a percentage error of 19.4%. Conclusions： Femoral access for indicator injection results in markedly altered values provided by the EV1000NolumeView , particularly for GEDVI, PVPI, and GEF.
