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Kannan BR Sivasankaran S Tharakan JA Titus T Ajith Kumar VK Francis B Krishnamoorthy KM Harikrishnan S Padmakumar R Nair K 《Indian heart journal》2003,55(2):161-166
BACKGROUND: There is a paucity of data regarding the long-term outcome of patients operated for ventricular septal defect with severe pulmonary arterial hypertension and elevated pulmonary vascular resistance. METHODS AND RESULTS: We evaluated the long-term follow-up results of a selected cohort of patients with nonrestrictive ventricular septal defect and elevated pulmonary vascular resistance (>6 Wood units). Thirty-eight patients, median age 7.5 years (range 6 months-27 years), with nonrestrictive ventricular septal defect with severe pulmonary hypertension were operated between 1985 and 1996 at our institute. Preoperative pulmonary vascular resistance, ratio of pulmonary blood flow to systemic blood flow, and ratio of pulmonary vascular resistance to systemic vascular resistance were 7.63+/-1.8 Wood units, 1.9+/-0.48, and 0.41+/-0.12, respectively. The majority (68.4%) had perimembranous ventricular septal defect. Thirty patients (79%) had a good outcome and were asymptomatic at a mean follow-up of 8.7 years, with significant reduction in pulmonary artery pressures. Eight patients (21%) had a poor outcome, which included 5 immediate postoperative deaths, 1 late death and 2 surviving patients with persistent severe pulmonary arterial hypertension. There was no significant difference regarding hemodynamic parameters at baseline between those who had a good outcome and those who did not. Eleven patients with a preoperative pulmonary blood flow to systemic blood flow ratio of <2:1. who had a good outcome following surgery, underwent repeat catheterization at follow-up. There was a significant reduction in their mean pulmonary vascular resistance (8.03+/-1.4 v. 4.16+/-1.6 Wood units, p=0.001) and pulmonary vascular resistance to systemic vascular resistance ratio (0.41+/-0.12 v. 0.19+/-0.06, p=0.05). CONCLUSIONS: The late results of surgery on this selected group of patients with nonrestrictive ventricular septal defect with high pulmonary vascular resistance are encouraging. Operative correction of the ventricular septal defect should be actively considered in all children presenting with nonrestrictive ventricular septal defect with a significant left-to-right shunt, despite moderately elevated pulmonary vascular resistance. Even among older patients with ventricular septal defect and moderately elevated pulmonary vascular resistance, there is a specific group that does well after operation. 相似文献
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Piyush Gupta Mehul Choksi Ashish Goel Uday Zachariah Kattiparambil Gangadharan Sajith Jeyamani Ramachandran George Chandy George Kurian Grace Rebekah Chundamannil Eapen Eapen 《Indian journal of gastroenterology》2018,37(1):31-38
Background
Experience with zinc in treating symptomatic hepatic Wilson’s disease (WD) is limited.Aim
To study the efficacy of Penicillamine followed by zinc in treating symptomatic hepatic Wilson’s disease.Methods
We retrospectively analyzed case records of 31 symptomatic hepatic WD patients for whom disease severity scores (Child’s, model for end-stage liver disease (MELD), Nazer’s, and New Wilson Index (NWI) score) and 24-h urinary copper were compared at 3-time points—baseline at presentation, at transition from penicillamine to zinc and at end of follow up.Results
Thirty-one patients (median age 11 [5–24] years) with symptomatic hepatic WD were studied; ten had associated neuropsychiatric manifestations of WD. Penicillamine was changed to zinc sulfate either due to financial constraints (28 patients) or due to adverse effects of penicillamine (3 patients). At presentation (baseline), six patients belonged to Child’s class A, five to Child’s B, and 17 to Child’s C. Duration of initial penicillamine chelation therapy was 134 (2–320) weeks, and of subsequent zinc therapy was 363 (35–728) weeks. There was a significant improvement in liver function tests and disease severity scores (Child’s, MELD, Nazer’s, and NWI score) at the transition from penicillamine to zinc compared to baseline. This improvement was maintained until the end of study period with 90% survival at 10 (2–20) years. Fifteen of the 17 Child’s C cirrhotic patients showed significant improvement in disease severity scores from baseline until end of follow up.Conclusions
Penicillamine followed by zinc may be a safe and effective treatment in resource-constrained setting for symptomatic hepatic WD patients in all grades of baseline disease severity. Some patients with decompensated cirrhosis due to WD may be managed with medical treatment, avoiding liver transplantation.6.
Namboodiri N Harikrishnan SP Ajitkumar V Tharakan JA 《The Journal of invasive cardiology》2008,20(1):E33-E35
A 43-year-old male with mirror-image dextrocardia and severe rheumatic mitral stenosis was subjected to successful percutaneous transvenous mitral commissurotomy (PTMC). The standard Inoue technique was modified by transseptal catheterization via the left femoral vein, image inversion, delineation of the interatrial septal anatomy via levophase pulmonary angiography, septal contrast staining and pigtail catheter insertion in the noncoronary aortic sinus, interatrial septal puncture with the transseptal needle rotated to a 7 o'clock position and left ventricular entry with a reverse loop technique. There were no procedural complications. Intracardiac pressures and mitral valvular planimetry suggested a successful procedural outcome. This case illustrates that PTMC can be accomplished safely in patients with this unusual cardiac anatomy with a few modifications in the standard technique. 相似文献
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