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Biomedical subjects

R Juneja

Publications and source records attributed to R Juneja.

At least 19 recordsLinked to original sources

Efficacy of nitroglycerin inhalation in reducing pulmonary arterial hypertension in children with congenital heart disease.

BACKGROUND: There has been a renewed interest in nitric oxide donor drugs, such as nitroglycerin, delivered by the inhalational route for treatment of pulmonary arterial hypertension (PAH). We investigated the acute effects of inhaled nitroglycerin on pulmonary and systemic haemodynamics in children with PAH associated with congenital heart disease. METHODS: Nineteen children with acyanotic congenital heart disease and a left to right shunt with severe PAH, undergoing routine diagnostic cardiac catheterization were included in this study. Systolic, diastolic and mean systemic as well as pulmonary artery pressures, right atrial pressure and pulmonary capillary wedge pressure (PCWP) were recorded and systemic vascular resistance index (SVRI) and pulmonary vascular resistance index (PVRI) were calculated at room air, following 100% oxygen as well as after nitroglycerin inhalation in all patients. RESULTS: Systolic, diastolic and mean pulmonary artery pressure and PVRI decreased significantly, whereas heart rate, systolic, diastolic and mean systemic arterial pressure, PCWP and SVRI did not change significantly following 100% oxygen or inhalation of nitroglycerin. CONCLUSION: Inhaled nitroglycerin significantly decreases systolic, diastolic and mean pulmonary artery pressure as well as PVRI without affecting systemic haemodynamics, and thus can be used as a therapeutic modality for acute reduction of PAH in children with congenital heart disease.

Administration, Inhalation↗

Comparison of echo and MRI in the imaging evaluation of intracardiac masses.

We compared the efficacy of echocardiography (ECHO) and magnetic resonance imaging (MRI) for evaluating intracardiac masses. Over an 8-yr period, 28 patients, 21 males, 7 females, 16 days-60 years of age (mean 25 years) with a suspected intracardiac mass on ECHO (transthoracic in all; transesophageal in 9) underwent an MRI examination. Five patients had a contrast-enhanced MRI. ECHO and MRI were compared with respect to their technical adequacy, ability to detect and suggest the likely etiology of the mass, and provide additional information (masses not seen with the other technique, inflow or outflow obstruction, and intramural component of an intracavitary mass). With MRI, the image morphology (including signal intensity changes on the various sequences) and extracardiac manifestations were also evaluated. The diagnosis was confirmed by histopathology in 18, surgical inspection in 4, by follow- up imaging on conservative management in 5, and by typical extracardiac manifestations of the disease in 1 patient. Fifteen (54%) patients had tumors (benign 12, malignant 3), 5 had a thrombus or hematoma, and 4 each had infective or vascular lesions. Thirty-four masses (13 in ventricle, 11 septal, 7 atrial, 2 on valve and 1 in pulmonary artery) were seen on MRI, 28 of which were detected by ECHO. Transthoracic ECHO (TTE) and MRI were technically optimal in 82% and 100% of cases, respectively. Nine patients needed an additional transesophageal ECHO (TEE). Overall, MRI showed a mass in all patients, whereas ECHO missed it in 2 cases. In cases with a mass on both modalities, MRI detected 4 additional masses not seen on ECHO. MRI suggested the etiology in 21 (75%) cases, while the same was possible with ECHO (TTE and TEE) in 8 (29%) cases. Intramural component, extension into the inflow or outflow, outflow tract obstruction, and associated pericardial or extracardiac masses were better depicted on MRI. We conclude that MRI is advantageous over a combination of TTE and TEE for the detection and complete morphological and functional evaluation (hemodynamic effects) of cardiac masses.

Adolescent↗

Detection of coronary artery anomalies in tetralogy of Fallot using a specific angiographic protocol.

Patients with tetralogy of Fallot were studied with a prespecified angiographic protocol aimed to delineate the types and incidence of coronary artery anomalies and to look for any correlation with other abnormalities detected by simultaneous detailed angiography. The incidence of coronary anomalies was found to be 7.0%; no association was found with these anomalies with any of the other abnormalities detected.

Abnormalities, Multiple↗

Mitochondrial myopathy presenting as ataxia with dilated cardiomyopathy.

Mitochondrial disorders are multisystem diseases with very heterogeneous clinical manifestations. Common cardiac features include cardiomyopathy and conduction defects. We report a five-year-old boy who presented with signs of congestive cardiac failure and was diagnosed to have dilated cardiomyopathy. Six months later, he developed progressively worsening ataxia, hypotonia, other cerebellar signs, hearing loss, severe sensory peripheral neuropathy and lactic acidosis. Electronmicroscopy of skeletal muscle biopsy was consistent with mitochondrial myopathy.

Ataxia↗

Hypocalcemic heart failure masquerading as dilated cardiomyopathy.

Hypocalcemia is a rare, but reversible, cause of congestive heart failure. We report a 4-month-old boy diagnosed as dilated cardiomyopathy who had prolonged QoTc with low blood levels of calcium, normal phosphate, elevated alkaline phosphatase and findings suggestive of rickets. In view of non response to calcium and vitamin D3, a possible diagnosis of VDDR I (Vitamin D-dependent rickets) was made and he was treated with calcium and calcitriol. The serum calcium levels normalised within 10 days, along with resolution of the signs and symptoms of heart failure, near normal left ventricular function and normalisation of QoTc. Pediatricians should be aware of the association of hypocalcemia with cardiac dysfunction and should keep it as a possible reversible cause of heart failure in children.

Cardiomyopathy, Dilated↗

Are drugs and catheter ablation effective for treating ventricular arrhythmias in populations that cannot afford implantable cardioverter defibrillators?

Despite recent advances, ventricular arrhythmias continue to pose a therapeutic challenge, especially to the clinician in the developing world. Although the implantable cardioverter defibrillator (ICD) has improved survival in both primary and secondary prevention trials, it still remains a costly modality for the developing world. Even though certain subgroups stand to benefit unequivocally in survival from the ICD, there are others in whom this therapy may not offer a survival benefit over empiric antiarrhythmic drug therapy with amiodarone. The utility of optimized drug therapy (including either amiodarone or sotalol, b-blockers, and angiotensin-converting enzyme inhibitors) needs to be compared with the ICD in a randomized manner in these lower-risk patients with ventricular arrhythmias. The role of conventional catheter ablation techniques is mostly adjunctive to drugs and ICDs, although the newer mapping and ablation techniques may offer greater promise in the future.

Arrhythmias, Cardiac↗

Factors influencing late course of residual valvular and infundibular gradients following pulmonary valve balloon dilatation.

BACKGROUND: Though acute and follow-up benefits of pulmonary valve balloon dilatation (PVBD) for pulmonic valve stenosis are well known, the late course of residual gradients at individual valvular and infundibular levels is not well described. Furthermore, the factors influencing this late course have not been studied. MATERIALS AND METHODS: We assessed the transpulmonary gradients by echo-Doppler in 96 patients (61 male, mean age 10.7 years) at a mean follow-up interval of 58.8+/-32.1 months (minimum 2 years) following PVBD. The patients were divided into three groups based on the residual gradients at valvular and infundibular levels immediately following PVBD as assessed by pull-back of an end-hole catheter across the right ventricular outflow tract: Group A with minor pressure gradients at both valvular and infundibular levels of less than 30 mmHg (n=60, 62.5%), Group B with predominantly infundibular gradients of more than 30 mmHg with a valvular gradient of less than 30 mmHg (n=27, 28.1%), and Group C with a residual valvular gradient of greater than 30 mmHg irrespective of the infundibular gradient (n=9, 9.4%). Demographic characteristics, hemodynamic parameters and procedural variables were correlated with the change in gradient at follow-up (late fall) and with long-term results. RESULTS: The mean follow-up trans-pulmonary gradient was 20+/-14 mmHg which was significantly lower than that immediately post-PVBD (43+/-32 mmHg), P<0.001. The late fall (mean 24+/-29, range -55 to 110 mmHg) varied widely depending upon the acute result group: patients in Groups A and B showed significant late fall of 9+/-12 mmHg (P<0.05 for follow-up gradient compared to that following PVBD) and 58+/-31 mmHg (P<0.0001), respectively, while patients in Group C showed an insignificant late fall of 14+/-37 mmHg (P=0.21). In particular, each one of the patients in Group B showed decrease in trans-pulmonary gradients. On multivariate analysis, the extent of infundibular gradient emerged as the most important predictor of late fall (coefficient of determination 75%, P<0.0001). Patients who underwent PVBD at less than 2 years of age had a significantly greater late fall (41+/-33 mmHg) as compared to older patients (22+/-25 mmHg), P<0.05. A sub-optimal long-term result (transpulmonary gradient >25 mmHg, n=24) was significantly related to older age (P<0.001), dysplastic valve morphology (P=0.002), greater baseline trans-pulmonary gradients (P<0.001) and higher post-PVBD gradients (P=0.04). CONCLUSIONS: The long-term course of patients following PVBD depends upon the site and magnitude of the residual gradients. Even high residual infundibular gradients show marked reduction at follow-up, especially in infancy.

Adolescent↗

Comparison of continuous thoracic epidural and paravertebral blocks for postoperative analgesia after minimally invasive direct coronary artery bypass surgery.

OBJECTIVE: To compare continuous thoracic epidural analgesia (TEA) and paravertebral block (PVB) for postoperative analgesia in patients undergoing minimally invasive direct coronary artery bypass (MIDCAB) surgery for quality of analgesia, complications, compliance to chest physiotherapy, hemodynamics, and respiratory effects. DESIGN: Prospective, randomized study. SETTING: Specialty research hospital. PARTICIPANTS: Forty-one consenting patients undergoing MIDCAB surgery. INTERVENTIONS: Patients in the TEA group had an epidural catheter inserted at the T4-5 interspace, whereas patients in the PVB group had a catheter inserted in the paravertebral space on the left side at the T4-5 level. MEASUREMENTS AND MAIN RESULTS: Parameters evaluated included visual analog scale pain scores at rest and while coughing, supplemental analgesic requirement, complications, hemodynamics, and respiratory parameters. Measurements were made at 2-hour intervals for 12 hours beginning at 10 minutes after endotracheal extubation. There was no statistically significant difference in visual analog scale scores and requirement of supplemental analgesia between the 2 groups. Cardiac index at 4 hours and 6 hours was significantly higher in the TEA group. Patients in the PVB group had significantly lower respiratory rates at 8, 10, and 12 hours. All other parameters were comparable. In 1 patient, the epidural space could not be catheterized. One patient in the TEA group had transient hypotension, and 1 patient complained of backache at the site of the epidural catheter insertion. CONCLUSION: PVB is as effective as TEA for postoperative analgesia after MIDCAB surgery. PVB is technically easier than TEA and may be safer than TEA because no complications were seen in the PVB group.

Analgesia, Epidural↗

Islet cell antibodies and glutamic acid decarboxylase antibodies, but not the clinical phenotype, help to identify type 1(1/2) diabetes in patients presenting with type 2 diabetes.

This study was undertaken to determine which type 1 diabetes-associated autoantibodies and what clinical characteristics are most useful to identify patients with type 1(1/2) diabetes. We studied 125 patients, recently diagnosed clinically with type 2 diabetes for the presence of islet cell antibodies (ICA), insulin autoantibodies (IAA), antibodies to glutamic acid decarboxylase(GADAb), and IA-2a (IA-2Ab). Patients with a diagnosis of type 2 diabetes who met all of the following criteria at diagnosis were studied: age > or = 30 years, no history of ketonuria or ketoacidosis, and not requiring insulin treatment. Thirty-six patients (29%) were positive for at least 1 antibody. Thirty-two (26%) were ICA positive and 20 (16%) GADAb positive. Insulin autoantibodies and IA-2Ab occurred less frequently in 2 (1.6%) and 8 (6.4%) patients, respectively. There was no significant difference in the ages at diagnosis between the Ab(+) and Ab(-) patients, age in years (range) 47.2 (32 to 64) versus 51.2 (31 to 77), respectively, P =.06. Body mass index (BMI) was different in the 2 groups, with Ab(+) patients being less obese, BMI (range) 28.3 kg/m(2) (17.6 to 54.9) versus 32.0 kg/m(2) (19.2 to 68.8), respectively, P =.01. Clinical presentation of diabetes was more commonly symptomatic with polyuria and polydipsia in Ab(+) patients, while in Ab(-) patients, diagnosis was more often incidental, P =.002. However, more than 95% of patients overlapped in both age and BMI irrespective of antibody status. Similarly, 42% of Ab(+) patients had their diabetes diagnosed incidentally, while 29% of Ab(-) patients presented with polyuria and polydipsia. We therefore conclude that screening with antibodies, mainly ICA and GAD, but not age, BMI, or clinical presentation should be used to identify type 1(1/2) diabetes.

Adult↗

Fate of mild aortic valve disease after mitral valve intervention.

OBJECTIVE: This study was performed to assess the long-term outcome of untreated mild aortic valve disease present at the time of initial mitral valve intervention. METHODS: A total of 284 patients with rheumatic heart disease aged 7 to 62 years (mean, 23.5 +/- 12.2 years) who underwent mitral valve intervention and had mild aortic valve disease initially were followed up for 2 to 18 years (mean, 10.8 +/- 3.7 years). At initial intervention, 232 patients had pure mild aortic regurgitation, and 52 patients had mild aortic stenosis with or without aortic regurgitation. RESULTS: Among patients with mild aortic regurgitation initially, 11 (5%) patients progressed to moderate (n = 6) or severe (n = 5) regurgitation over an interval of 9 to 17 years (mean, 12.1 +/- 2.8 years), and 1 patient had moderate aortic stenosis and severe aortic regurgitation after 10 years. Freedom from development of moderate-severe aortic valve disease in patients who initially had mild aortic regurgitation was 100%, 97.0% +/- 1.7%, and 87.4% +/- 4.6% at 5, 10, and 15 years, respectively. Seventeen (35%) patients with initial mild aortic stenosis (with or without regurgitation) had moderate or severe stenosis (with or without moderate-severe regurgitation) after an interval of 4.9 +/- 3.8 years. Freedom from development of moderate-severe aortic valve disease in patients who initially had mild aortic stenosis was 75.6% +/- 6.2%, 61.5% +/- 8.5%, and 46.1% +/- 11.2% at 5, 10, and 15 years, respectively. Ten patients required aortic valve replacement for aortic valve dysfunction. CONCLUSIONS: Mild aortic regurgitation present at the time of mitral valve intervention progresses very slowly and less frequently requires reintervention. However, mild aortic stenosis diagnosed initially progresses more often and more rapidly and thus needs closer follow-up.

Adolescent↗

Characterization of antibody response to human cytomegalovirus in Indian renal transplant patients.

BACKGROUND & OBJECTIVES: Cytomegalovirus (CMV) disease in seroendemic transplant populations is due to reactivation of the virus, or reinfection. In this context, the antibody response is likely to influence presentation, clinical severity and outcome of the disease, and may provide a diagnostic and prognostic marker. This study was carried out in Indian renal transplant patients and healthy adults to characterize the antibody response to cytomegalovirus. METHODS: Thirty three transplant recipients with CMV illness (symptomatology with IgM and/or nPCR positive status), 20 recipients who were asymptomatic in the 6 months of follow up after transplantation and 62 healthy controls were investigated for markers of CMV infection. These individuals were tested for IgG avidity and neutralizing antibody by ELISA techniques. RESULTS: All 53 transplant recipients were found to have an IgG avidity index of > 50 per cent. Antibody to a CMV envelope glycoprotein gB/AD-1 (putative neutralizing antibody) was expressed as S/N ratio and was > or = 5 in asymptomatic (65%) and symptomatic (27%) immunosuppressed renal transplant recipients. However, none of the 53 CMV IgG positive healthy controls were positive for neutralizing antibodies S/N ratio > or = 5 (S/N ratio = sample mean OD/mean OD of 3 negative controls in each run). We observed the simultaneous presence of CMV PCR signal in leukocytes and neutralizing antibody (S/N ratio > or = 5) in the plasma in 22 (41.5%) of the 53 renal transplant recipients. INTERPRETATION & CONCLUSIONS: In this study among the immunosuppressed transplant patients we observed an association between symptomatic disease and the relative absence of neutralizing antibodies. The neutralizing antibodies are less frequently demonstrable among controls; while appearance in a higher proportion of asymptomatic recipients especially in association with high IgG avidity (> 90%) is suggestive of its role in control of CMV disease despite reactivation as evidenced by DNAemia while on immunosuppressive therapy.

Adult↗

Percutaneous transluminal angioplasty of the aorta in children with nonspecific aortoarteritis: acute and follow-up results with special emphasis on left ventricular function.

Nonspecific aortoarteritis (NSAA) is a chronic disease of unknown etiology and may result in stenosing and obstructive lesions of the aorta. Transluminal balloon angioplasty was carried out in 17 children, 4-12 years of age (mean 8.9 +/- 2.7 years) with symptomatic stenosis of thoracic or abdominal aorta due to NSAA. Hypertension was present in 15 children, and 11 had significant left ventricular dysfunction. All children had a clinically inactive disease. The stenosis was in descending thoracic aorta in 10, thoracoabdominal in 3 and only abdominal in 4. Symptomatic improvement with relief of stenosis was seen in 16 of 17 children, although in 3, the results were suboptimal. The peak systolic gradient fell from 70.9 +/- 19.3 mmHg to 32.0 +/- 19.2 mmHg immediately after dilation (P < 0.0001). Angiographically measured luminal diameter stenosis also fell from 82.5 +/- 7.7% to 19.4 +/- 11.8% (P < 0.0001). Dissection flap was seen in 13 cases. It was large in cases with long, diffuse and eccentric stenosis of the thoracic or thoracoabdominal aorta. On follow-up (available for 14 of 16 cases for a period of 2-87 months mean 20.7 +/- 21.5 months, median 15.5 months) two patients developed restenosis. Left ventricular function improved in all 8 children that had sustained benefit of angioplasty. Blood pressure normalized in 9 of 13 children and the drug requirements fell in 4 others. Further remodeling of the lesion with decrease in gradients and luminal stenosis was observed in 11 children that have been restudied. Balloon angioplasty of aorta in children with NSAA is a feasible and safe procedure. Small intimal flap is seen in the majority, extensive dissection is more likely in the long diffuse lesions in descending thoracic aorta. Hypertension and left ventricular dysfunction improve after relief of obstruction.

Angioplasty, Balloon↗

Congenital severe aortic stenosis with congestive heart failure in late childhood and adolescence: effect on left ventricular function after balloon valvuloplasty.

Left ventricular (LV) dysfunction with congestive heart failure (CHF) resulting from severe congenital aortic stenosis (AS) is a well-described condition in infancy, but it is rarely found in older children and adolescents. Aortic valve surgery in such cases may be associated with higher rates of morbidity and mortality. Aortic valve balloon dilatation (AVBD) is a viable alternative, but its effect on LV function has not been evaluated. We describe follow-up results of AVBD in 10 cases of severe congenital AS in older children and adolescents with CHF and LV dysfunction. The ages of these patients ranged from 5 to 18 yr (mean +/- SD: 10.8 +/- 4 yr), and nine were males. The follow-up period after AVBD ranged from 3 mo to 7 yr (mean +/- SD: 2.93 +/- 2.1 yr). Success was achieved in all cases, with no immediate complications. After valvuloplasty, the peak-to-peak systolic gradient declined from 74.7 +/- 30.8 to 33.9 +/- 18.2 mm Hg (P < 0.0001). The cardiac index increased slightly but significantly, from 1.9 +/- 0.27 to 2.2 +/- 0.5 L/min/m(2) (P < 0.015). Hemodynamic improvement was also confirmed by a significant decrease in mean pulmonary artery and pulmonary artery wedge pressures from 41.9 +/- 9 to 32.6 +/- 6.6 and from 25.5 +/- 2.9 to 19.3 +/- 3.4 mm Hg, respectively. The echocardiographically derived left ventricular ejection fraction (LVEF) improved from 21.6 +/- 5. 37% to 31 +/- 6.5% within 24 hr after AVBD, and it further improved in all cases on follow-up. Mean LVEF at last follow-up was 59.4 +/- 11.4%. The Doppler instantaneous peak systolic gradient (IPSG) increased from 37.3 +/- 18.8 to 64.8 +/- 30.7 mm Hg at late follow-up. Significant aortic regurgitation (AR) developed in 20% of patients. The Doppler IPSG across the aortic valve was > 60 mm Hg in five cases on follow-up. Two of these patients underwent another AVBD successfully 4 and 16 mo later, respectively. Aortic valve replacement was done in two patients, one for severe restenosis with mild AR 12 mo after AVBD and another for severe re-restenosis with moderate AR 21 mo after a second AVBD. Severe congenital AS can be associated with LV dysfunction and CHF in late childhood and adolescence. AVBD results in good palliation with improvement in LV function on follow-up.

Adolescent↗