Radioimmunoassay for the measurement of plasma renin activity (PRA) was performed in 43 normal Koreans and 45 patients with essential hypertension. Plasma samples were drawn in supine position in the morning and after upright posture for 4 hours. Urinary sodium excretion rates were measured in the concurrent 24 hour urine samples, as an index of their sodium balance. The results were as follows: 1. There was an inverse correlation between 24hr sodium excretion and PRA. The normal values of PRA in supine position ranged from 1.0 to 7.0 ng/ml/hr. when 24 hour sodium excretion were between 50 to 150 mEq. PRA in elderly tended to be low. 2. When stimulated by 4 hour upright posture, PRA increased by 2.6 times from the baseline value. 3. Of the 45 patients with essential hypertension, PRA was low in 10 cases (22.2%), normal in 28 cases (62.2%), and high in 7 cases (15.6%). 4. In the normal and high renin groups, who tended to be younger in ages, mean diastolic blood pressure and BUN were higher than in low renin group. Though hypertensive retinopathy and left ventricular hypertrophy in ECG were more prevalent in the former, no significant differences were noted as in the case of serum cholesterol. 5. There were 8 cases of cardiovascular complications (7 with cerebral vascular accident, 1 with myocardial infarction); 3 in low renin group (30%), 2 in normal renin (7.1%) and 3 in high renin group (42.9%). This figure indicated higher rate of cardiovascular complications in high renin groups, and lower rate in normal renin group. But the incidence of the complication was not significantly low in low renin group.
Fabry disease (FD) is a rare X-linked lysosomal storage disorder caused by the deficiency of ${\alpha}$-galactosidase A. Patients with classical FD present acroparesthesia, hypohidrosis, cornea verticillata, disseminated angiokeratoma, and microalbuminuria in childhood, and develop life-threatening renal, cardiac, and cerebrovascular complications typically after the fourth decade of life. To date, more than 700 mutations responsible for FD have been identified in the human GLA gene. Herein, we report a novel GLA mutation, c.1117_1141del25 (p.Gly373Profs*10), identified in an 11-year-old Korean boy with FD presenting early cardiac and neurologic manifestation and in other affected family members. The boy had acroparesthesia, hypohidrosis, cornea verticillata, and left ventricular hypertrophy. His mother and sister also had acroparesthesia. Two males on the mother's side had similar pain and died of unknown causes. The plasma ${\alpha}$-galactosidase A activity (4.1 nmol/hr/mg protein) of the patient was markedly lower than the mean value of the controls. The plasma level of globotriaosylsphingosine was elevated in the patient and all the carriers. We concluded the novel GLA mutation c.1117_1141del25 is a pathogenic mutation for FD, probably related to the early cardiac manifestation of FD.
Purpose: The purpose of this study was to evaluate the prevalence, clinical characteristics, and long-term clinical effects of hypertension in Korean childhood-onset systemic lupus erythematous (SLE) patients. Methods: The medical records of SLE patients, diagnosed by 2019 SLE European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria, who visited Samsung Medical Center from January 2009 to May 2019 were reviewed. Disease activity and long-term damage were evaluated using the Modified Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and the Pediatric Systemic Lupus International Collaborating Clinics/ACR Damage Index (Ped-SDI), respectively. The sex-, age- and height-blood pressure standards recommended by the American Academy of Pediatrics 2017 guideline was used to define hypertension. Results: A total of 32 patients were enrolled in this study. The median follow-up duration was 7.3 years and females were predominant. The median ages at SLE and hypertension diagnoses were 14.2 and 14.3 years, respectively. The biopsy-proven lupus nephritis was detected in 90.6% and 37.5% were class IV. During the follow-up, 12 patients (37.5%) had hypertension. Among them, 2 patients had 3 episodes of posterior reversible encephalopathy syndrome and 5 patients had left ventricular hypertrophy (LVH). Univariate analysis showed baseline hypertension was significantly correlated with a lower estimated glomerular filtration rate, higher body mass index and SLEDAI at baseline. The development of hypertension during the follow-up was significantly correlated with obesity, LVH, and higher Ped-SDI. Conclusion: Our study revealed that hypertension in pediatric SLE is associated with obesity and renal function at SLE diagnosis and could affect long-term damage.
A 15-year-old, neutered male, Shih-Tzu, was presented at the Chonnam National Veterinary Medical Teaching Hospital for evaluation of acute onset of persistent coughing, exercise intolerance, and abnormal heart sound. On thoracic auscultation, a split-second heart sound and a wheezing sound were detected on both sides of the chest walls. On physical examination, the dog's body condition score (BCS) was 7/9, and had stenotic nares. Thoracic radiographs revealed right-sided enlargement of the cardiac silhouette (vertebral heart score (VHS) 11.2; reference interval = 8.9-10.1), mild main pulmonary artery (MPA) bulging, mild interstitial infiltration, and hepatomegaly. The electrocardiogram showed right axis deviation, suggesting right ventricular hypertrophy. The echocardiographic study showed moderate pulmonary hypertension and moderate tricuspid regurgitation. There were no findings of a tracheobronchial disease, pulmonary thromboembolism, congenital shunt, left heart disease, or parasitic disease. Based on clinical signs and diagnostic findings, the dog was diagnosed with pulmonary hypertension secondary to brachycephalic syndrome. To rectify respiratory exacerbating factors, the dog was recommended weight control by restricting dietary intake and managing concurrent Cushing's syndrome. Treatments included sildenafil, pimobendan, furosemide, and ramipril. After five months of taking medications and weight control, the severity of pulmonary hypertension improved from moderate to mild. The clinical signs of the patient, including coughing and exercise intolerance, improved a lot. For 5 months of follow-up, the patient has not reported further recurrence of respiratory distress.
Gustavo Gavazzoni Blume;Luka David Lechinewski;Isabela Pedroza Vieira;Nadine Clausell;Giovana Paludo Bertinato;Paulo Andre Bispo Machado-Junior;Pedro Goulart Berro;Lidia Ana Zytynski Moura;Teresa Tsang
Journal of Cardiovascular Imaging
/
v.30
no.1
/
pp.25-34
/
2022
BACKGROUND: The purpose of this study was to assess the utility of a handheld device (HH) used during common daily practice and its agreement with the results of a standard echocardiography study (STD) performed by experienced sonographers and echocardiographer. METHODS: A prospective follow-up was conducted in an adult outpatient echocardiography clinic. Experienced sonographers performed the STD and an experienced echocardiographer performed the HH. STD included 2-dimensional images, Doppler and hemodynamics analysis. Hemodynamic assessment was not performed with the HH device because the HH does not include such technology. The images were interpreted by blinded echocardiographers, and the agreement between the reports was analyzed. RESULTS: A total of 108 patients were included; and the concordance for left ventricle (LV) ejection fraction (EF), wall motion score index, LV and right ventricle (RV) function, RV size, and mitral and aortic stenosis was excellent with κ values greater than 0.80. Wall motion abnormalities had good concordance (κ value 0.78). The agreement for LV hypertrophy, mitral and aortic regurgitation was moderate, and tricuspid and pulmonary regurgitation agreements were low (κ values of 0.26 and 0.25, respectively). CONCLUSIONS: In a daily practice scenario with experienced hands, HH demonstrated good correlation for most echocardiography indications, such as ventricular size and function assessment and stenosis valve lesion analyses.
Ahn, Hye Mi;Jung, Sun Ok;Kwon, Jung Hyun;Hong, Young Mi
Clinical and Experimental Pediatrics
/
v.53
no.1
/
pp.72-79
/
2010
Purpose: Left ventricular (LV) hypertrophy and impaired diastolic function may occur early in systemic hypertension. Diastolic dysfunction is associated with increased cardiovascular risk. Tissue Doppler imaging (TDI)-derived tissue velocity and strain rate are new parameters for assessing diastolic dysfunction. The aim of this study is to determine whether TDI and strain rate imaging (SRI) would improve the ability to recognize early impaired diastolic and systolic functions compared with conventional echocardiography in hypertensive adolescents. Methods: We included 38 hypertensive patients with systolic blood pressure above 140 mmHg or diastolic blood pressure above 90 mmHg. Ejection fraction and myocardial performance index (MPI) were estimated by conventional echocardiography. Peak systolic myocardial velocity, early diastolic myocardial velocity (Em), and peak late diastolic myocardial velocity (Am) were obtained by using TDI and SRI. Results: In the hypertensive group, interventricular septal thickness was significantly increased on M-mode echocardiography. Em/Am was significantly decreased at the mitral valve annulus. Among hypertensive subjects, the E strain rate at basal, mid, and apex was significantly decreased. Systolic strain was significantly decreased at the septum in the hypertensive group. Conclusion: Strain rate might be a useful new parameter for the quantification of both regional and global LV functions and could be used in long-term follow up in hypertensive patients. Early identification by SRI of subjects at risk for hypertensive and ventricular dysfunction may help to stratify risk and guide therapy. Further studies, including serial assessment of LV structure and function in a larger number of adolescents with hypertension, is necessary.
Kim, Sung Eun;Yin, Ming Zhe;Kim, Hae Jin;Vorn, Rany;Yoo, Hae Young;Kim, Sung Joon
The Korean Journal of Physiology and Pharmacology
/
v.24
no.1
/
pp.111-119
/
2020
In vascular smooth muscle, K+ channels, such as voltage-gated K+ channels (Kv), inward-rectifier K+ channels (Kir), and big-conductance Ca2+-activated K+ channels (BKCa), establish a hyperpolarized membrane potential and counterbalance the depolarizing vasoactive stimuli. Additionally, Kir mediates endothelium-dependent hyperpolarization and the active hyperemia response in various vessels, including the coronary artery. Pulmonary arterial hypertension (PAH) induces right ventricular hypertrophy (RVH), thereby elevating the risk of ischemia and right heart failure. Here, using the whole-cell patch-clamp technique, we compared Kv and Kir current densities (IKv and IKir) in the left (LCSMCs), right (RCSMCs), and septal branches of coronary smooth muscle cells (SCSMCs) from control and monocrotaline (MCT)-induced PAH rats exhibiting RVH. In control rats, (1) IKv was larger in RCSMCs than that in SCSMCs and LCSMCs, (2) IKv inactivation occurred at more negative voltages in SCSMCs than those in RCSMCs and LCSMCs, (3) IKir was smaller in SCSMCs than that in RCSMCs and LCSMCs, and (4) IBKCa did not differ between branches. Moreover, in PAH rats, IKir and IKv decreased in SCSMCs, but not in RCSMCs or LCSMCs, and IBKCa did not change in any of the branches. These results demonstrated that SCSMC-specific decreases in IKv and IKir occur in an MCT-induced PAH model, thereby offering insights into the potential pathophysiological implications of coronary blood flow regulation in right heart disease. Furthermore, the relatively smaller IKir in SCSMCs suggested a less effective vasodilatory response in the septal region to the moderate increase in extracellular K+ concentration under increased activity of the myocardium.
A hospital-based, matched case-control study was carried out to evaluate the relation ship of various suspected risk factors including snoring and serum level of cholesterol to cerebrovascular disease in Korea. A total of 127 incident cases of cerebrovascular disease(74 cases of cerebral infarction and 53 cases of intracerebral hemorrhage) admitted to. the department of neurology in a university hospital from December, 1993 to March, 1995 were compared with 127 matched controls admitted to same hospital in same period. A multivariate analysis suggested that ECG abnormality(left ventricular hypertrophy and atrial fibrillation), family history of cerebrovascular disease, fundoscopic abnormality, previous history of transient ischemic attack and hypercholesterolemia were risk factors of cerebrovascular disease, ECG abnormality, fundoscopic abnormality, smoking and hypercholesterolemia were also suggested as risk factors of cerebral infarction.
96 patients underwent cardiac valve replacement for valvular heart diseases consecutively between February 1986 to February 1990 in the Department of Thoracic and Cardiovascular Surgery of Yeungnam University Hospital. The follow up period was between 6 months and 4.5 years postoperatively[mean 23.4$\pm$13.1 months]. 75 cases got mitral valve replacement, 6 cases, aortic valve replacement, 15 cases, double valve replacement. 30[31.2%] patients were male and 66[68.8%] were female and the age ranged from 14 to 66 years old. Early hospital death within 30 days postoperation were 5 patients[5.2%], consisting of by low cardiac output in 2, infective endocarditis in 1, multiple organ failure with sepsis in 1 patient. There was no late postoperative death. Most common early postoperative complication was wound disruption [8.7%] and then low cardiac output, pneumothorax, pleural effusion in order. Most common late postoperative complications were minor bleeding episodes[8.7%] related to anticoagulant therapy which were consisted of frequent epistaxis in 3, gum bleeding in 2, hemorrhagic gastritis in 1, hypermenorrhea in 1, hematoma in right arm in 1 patient. Valve-related complications included valve thrombosis [1.6%/ patient-year], valve failure due to pannus formation[1.1% /patient-year], prosthetic valve endocarditis[1, 1%o/patient-year] and minor anticoagulant hemorrhage[4.4% /patient-year]. 5 cases of reoperations were performed in 4 patients due to valve failure and all of them were in the mitral positions[2.7% /patient-year]. Cardiothoracic ratios in the chest X-ray decreased at the 6th month and 1st year postoperation in all patients. But in New York Heart Association[NYHA] functional class IV, no change in cardiothoracic ratio was found between 6 months and 1 year postoperation. In the echocardiogram, the size of the cardiac chambers decreased, but ejection fraction increased postoperatively in each functional class. In the electrocardiogram, decreases were found in the incidence of atrial fibrillation, left atrial enlargement, left ventricular hypertrophy with right bundle branch block increasing postoperatively in each functional class. The actuarial survival rate was 98.4% for all patients, 98.7% for mitral valve replacement, 83.8% for aortic valve replacement, and 80% for double valve replacement at the end of a 4.5 year follow up period. Meanwhile the actuarial freedom rate was 91.5% for prosthetic valve endocarditis, 91.6% for thromboembolism, 89.0% for prosthetic valve failure and 83.7% for minor anticoagulant hemorrhage. Preoperative NYHA class III and IV were 75% of all patients, but 95% of all patients were up graded to NYHA class I and II postoperatively.
In candidates for aortic valve replacement [AVR]it is our primary intention to implant the largest possible vale prosthesis of at least 23 mm in diameter in patients with severe valvular aortic stenosis. However, in many patients there is an additional subvalvular asymmetric septal hypertrophy which in some cases may cause an postextrasystolic increase of the LV-aortic gradient. Another component of the aortic stenosis syndrome is a narrow valvular ring, or a combination of both. After complete removal of the diseased valve and decalcification the narrow aortic ring [< 23 mm] can be widened firstly by transaortic subvalvular septal myectomy- [TSM] thus unfolding the left ventricular outflow tract[LVOT]and secondly by extending the oblique aortic incision into the aortic valve ring or further down into the anterior leaflet of the mitral valve. The sub-and supra-valvular defect will be closed by patch enlargement of the aortic root [PEAR] using autologous pericardium. These techniques allow a considerable enlargement of the valvular ring of about 4 to 10 mm in circumference. In a retrospective study using a computerized program, 847 patients with AVR [1980-1984]were reviewed to evaluate the intraoperative hemodynamic results mainly concerning relief of the transvalvular gradient. In 626 patients AVR was performed, 151 patients had double valve replacement [AVR+MVR], and 70 patients had AVR plus additional surgical procedures. Concentrating on the AVR-group [n=626] there were 103 patients with TSM, 24 patients with PEAR and 20 patients with TSM+PEAR which demonstrated that in a total, of 147 patients of this groups [23.5%] an additional procedure was necessary. The Statistical evaluation of the intraoperative pressure measurements before and after AVR in relation to the size of the implanted prostheses indicated the lowest preoperative mean gradient in patients with AVR alone, the highest in patients who afforded TSM plus PEAR. However, after AVR the mean gradients in all three groups were very low [mean 5 to 10 mmHg]. These data indicate that in patients with a narrow aortic ring and additional considerable ASH, TSM and PEAR are suitable techniques to enlarge the aortic root to enable the implantation of an adequate aortic valve prosthesis. Long-term controls have shown that autologous pericardium is a qualified graft material for the ascending aorta.
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