Chronic kidney disease in public renal practices in Queensland, Australia, 2011-2018.

AIM: To describe adults with (non-dialysis) chronic kidney disease (CKD) in nine public renal practice sites in the Australian state of Queensland. METHODS: 7,060 persons were recruited to a CKD Registry in May 2011 and until start of kidney replacement therapy (KRT), death without KRT or June 2018, for a median period of 3.4 years. RESULTS: The cohort comprised 7,060 persons, 52% males, with a median age of 68 yr; 85% had CKD stages 3A to 5, 45.4% were diabetic, 24.6% had diabetic nephropathy, and 51.7% were obese. Younger persons mostly had glomerulonephritis or genetic renal disease, while older persons mostly had diabetic nephropathy, renovascular disease and multiple diagnoses. Proportions of specific renal diagnoses varied >2-fold across sites. Over the first year, eGFR fell in 24% but was stable or improved in 76%. Over follow up, 10% started KRT, at a median age of 62 yr, most with CKD stages 4 and 5 at consent, while 18.8% died without KRT, at a median age of 80 yr. Indigenous people were younger at consent and more often had diabetes and diabetic kidney disease and had higher incidence rates of KRT. CONCLUSION: The spectrum of characteristics in CKD patients in renal practices is much broader than represented by the minority who ultimately start KRT. Variation in CKD by causes, age, site and Indigenous status, the prevalence of obesity, relative stability of kidney function in many persons over the short term, and differences between those who KRT and die without KRT are all important to explore.

Unique X-linked familial FSGS with co-segregating heart block disorder is associated with a mutation in the NXF5 gene.

Focal segmental glomerulosclerosis (FSGS) is the consequence of a disease process that attacks the kidney's filtering system, causing serious scarring. More than half of FSGS patients develop chronic kidney failure within 10 years, ultimately requiring dialysis or renal transplantation. There are currently several genes known to cause the hereditary forms of FSGS (ACTN4, TRPC6, CD2AP, INF2, MYO1E and NPHS2). This study involves a large, unique, multigenerational Australian pedigree in which FSGS co-segregates with progressive heart block with apparent X-linked recessive inheritance. Through a classical combined approach of linkage and haplotype analysis, we identified a 21.19 cM interval implicated on the X chromosome. We then used a whole exome sequencing approach to identify two mutated genes, NXF5 and ALG13, which are located within this linkage interval. The two mutations NXF5-R113W and ALG13-T141L segregated perfectly with the disease phenotype in the pedigree and were not found in a large healthy control cohort. Analysis using bioinformatics tools predicted the R113W mutation in the NXF5 gene to be deleterious and cellular studies support a role in the stability and localization of the protein suggesting a causative role of this mutation in these co-morbid disorders. Further studies are now required to determine the functional consequence of these novel mutations to development of FSGS and heart block in this pedigree and to determine whether these mutations have implications for more common forms of these diseases in the general population.

Early referral to specialist nephrology services for preventing the progression to end-stage kidney disease.

BACKGROUND: Early referral of patients with chronic kidney disease (CKD) is believed to help with interventions to address risk factors to slow down the rate of progression of kidney failure to end-stage kidney disease (ESKD) and the need for dialysis, hospitalisation and mortality. OBJECTIVES: We sought to evaluate the benefits (reduced hospitalisation and mortality; increased quality of life) and harms (increased hospitalisations and mortality, decreased quality of life) of early versus late referral to specialist nephrology services in CKD patients who are progressing to ESKD and RRT. In this review, referral is defined as the time period between first nephrology evaluation and initiation of dialysis; early referral is more than one to six months, whereas late referral is less than one to six months prior to starting dialysis. All-cause mortality and hospitalisation and quality of life were measured by the visual analogue scale and SF-36. SF-36 and KDQoL are validated measurement instruments for kidney diseases. SEARCH METHODS: We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library, 2012; Issue 1) which contains the Cochrane Renal Group's Specialised Register; MEDLINE (1966 to February 2012), EMBASE (1980 to February 2012). Search terms were approved by the Trial Search Co-ordinator. SELECTION CRITERIA: Randomised controlled trials (RCTs), quasi-RCTs, prospective and retrospective longitudinal cohort studies were eligible for inclusion. DATA COLLECTION AND ANALYSIS: Two authors independently assessed study quality and extracted data. Events relating to adverse effects were collected from the studies. MAIN RESULTS: No RCTs or quasi-RCTs were identified. There were 40 longitudinal cohort studies providing data on 63,887 participants; 43,209 (68%) who were referred early and 20,678 (32%) referred late.Comparative mortality was higher in patients referred to specialist services late versus those referred early. Risk ratios (RR) for mortality reductions in patients referred early were evident at three months (RR 0.61, 95% CI 0.55 to 0.67; I² = 84%) and remained at five years (RR 0.66, 95% CI 0.60 to 0.71; I² = 87%). Initial hospitalisation was 9.12 days shorter with early referral (95% CI -10.92 to -7.32 days; I² = 82%) compared to late referral. Pooled analysis showed patients referred early were more likely than late referrals to initiate RRT with peritoneal dialysis (RR 1.74, 95% CI 1.64 to 1.84; I² = 92%).Patients referred early were less likely to receive temporary vascular access (RR 0.47, 95% CL 0.45 to 0.50; I² = 97%) than those referred late. Patients referred early were more likely to receive permanent vascular access (RR 3.22, 95% CI 2.92 to 3.55; I² = 97%). Systolic blood pressure (BP) was significantly lower in early versus late referrals (MD -3.09 mm Hg, 95% CI -5.23 to -0.95; I² = 85%); diastolic BP was significantly lower in early versus late referrals (MD -1.64 mm Hg, 95% CI -2.77 to -0.51; I² = 82%). EPO use was significantly higher in those referred early (RR 2.92, 95% CI 2.42 to 3.52; I² = 0%). eGFR was higher in early referrals (MD 0.42 mL/min/1.73 m², 95% CI 0.28 to 0.56; I² = 95%). Diabetes prevalence was similar in patients referred early and late (RR 1.05, 95% CI 0.96 to 1.15; I² = 87%) as was ischaemic heart disease (RR 1.05, 95% CI 0.97 to 1.13; I² = 74%), peripheral vascular disease (RR 0.99, 95% CI 0.84 to 1.17; I² = 90%), and congestive heart failure (RR 1.00, 95% CI 0.86 to 1.15; I² = 92%). Inability to walk was less prevalent in early referrals (RR 0.66, 95% CI 0.51 to 0.86). Prevalence of chronic obstructive pulmonary disease was similar in those referred early and late (RR 0.89, 95% CI 0.70 to 1.14; I² = 94%) as was cerebrovascular disease (RR 0.90, 95% CI 0.74 to 1.11; I² = 83%).The quality of the included studies was assessed as being low to moderate based on the Newcastle-Ottawa Scale. Slight differences in the definition of early versus late referral infer some risk of bias. Generally, heterogeneity in most of the analyses was high. AUTHORS' CONCLUSIONS: Our analysis showed reduced mortality and mortality and hospitalisation, better uptake of peritoneal dialysis and earlier placement of arteriovenous fistulae for patients with chronic kidney disease who were referred early to a nephrologist. Differences in mortality and hospitalisation data between the two groups were not explained by differences in prevalence of comorbid disease or serum phosphate. However, early referral was associated with better preparation and placement of dialysis access.

Cephalic arch stenosis in the arteriovenous fistula: A retrospective analysis of predisposing factors.

BACKGROUND: Cephalic Arch Stenosis (CAS) is a frequently observed complication in brachiocephalic and radiocephalic arteriovenous fistulae (AVF) associated with high morbidity and healthcare expenditure. The predisposing factors and preventative strategies for CAS remain unclear. Our aim was to examine predisposing factors for CAS development in the AVF. METHODS: A retrospective case-control study was performed at Gold University Coast Hospital on patients with AVFs created from 2009 to 2018 with ⩾18 months follow-up. CAS was defined as a >50% narrowing on angiographic assessment with clinically significant symptoms (dialysis dysfunction, arm swelling, prolonged bleeding after access). RESULTS: About 187 patients with AVF were included in the analysis (36 brachiocephalic, 151 radiocephalic). CAS developed in 22 of 36 (61%) of brachiocephalic AVF and 9 of 151 (6%) of radiocephalic AVFs. Brachiocephalic AVF were ⩾12 times more likely to develop CAS than radiocephalic AVF (Hazard Ratio (HR) 12.7, 95% CI [5.6-28.3], p < 0.001). Each 1 mL/min increase in flow rate through the AVF, correlated with a 0.07% increase in the probability of development of CAS (HR 1.0007, 95% CI [1.0001-1.0012], p = 0.011). Brachiocephalic AVFs with CAS were associated with a higher number of interventional procedures per access-year compared with their non-CAS counterparts (Median [Interquartile range]: 1.76 [0.74, 3.97] vs 0.41 [0.27, 0.67], p = 0.003). CONCLUSION: Brachiocephalic AVF with higher access flow rates are more likely to develop CAS and earlier than radiocephalic AVF, and in a dose dependent fashion. AVF flow rate is a major factor in CAS development within brachiocephalic AVF and has potential utility in surveillance thresholds for prophylactic blood flow reduction procedures. AVFs with CAS are associated with a greater number of interventional procedures per access-year, heralding higher patient morbidity and healthcare expenditure. Further prospective studies will help define an AVF access flow rate threshold in the implementation of prophylactic strategies for CAS.

Hospitalizations Among Adults With CKD in Public Renal Specialty Practices: A Retrospective Study From Queensland, Australia.

Rationale & Objective: Little is known about hospital admissions in nondialysis patients with chronic kidney disease (CKD) before death or starting kidney replacement therapy (KRT). Study Design: Retrospective observational cohort study. Setting & Participants: Hospitalizations among 7,201 patients with CKD from 10 public renal clinics in Queensland (QLD), enrolled in the CKD.QLD registry starting in May 2011, were followed for 25,496.34 person-years until they started receiving KRT or died, or until June 30, 2018. Predictors: Demographic and clinical characteristics of patients with CKD. Outcomes: Hospital admissions. Analytical Approach: We evaluated the association of demographic and clinical features with hospitalizations, length of hospital stay, and cost. Results: Approximately 81.5% of the patients were admitted at least once, with 42,283 admissions, costing Australian dollars (AUD) 231 million. The average number of admissions per person-year was 1.7, and the cost was AUD 9,060, 10 times and 2 times their Australian averages, respectively. Single (1-day) admissions constituted 59.2% of all the hospital episodes, led by neoplasms (largely chemotherapy), anemia, CKD-related conditions and eye conditions (largely cataract extractions), but only 14.8% of the total costs. Approximately 41% of admissions were >1-day admissions, constituting 85.2% of the total costs, with cardiovascular conditions, respiratory conditions, CKD-related conditions, and injuries, fractures, or poisoning being the dominant causes. Readmission within 30 days of discharge constituted >42% of the admissions and 46.8% costs. Admissions not directly related to CKD constituted 90% of the admissions and costs. More than 40% of the admissions and costs were through the emergency department. Approximately 19% of the hospitalized patients and 27% of the admissions did not have kidney disease mentioned as either principal or associate causes. Limitations: Variable follow-up times because of different dates of consent. Conclusions: The hospital burden of patients with CKD is mainly driven by complex multiday admissions and readmissions involving comorbid conditions, which may not be directly related to their CKD. Strategies to prevent these complex admissions and readmissions should minimize hospital costs and outcomes. Plain-Language Summary: We analyzed primary causes, types, and costs of hospitalizations among 7,201 patients with chronic kidney disease (CKD) from renal speciality clinics across Queensland, Australia, over an average follow-up of 3.54 years. The average annual cost per person was $9,060, and was the highest in those with more advanced CKD, higher age, and with diabetes. More than 85% of costs were driven by more complex hospitalizations with longer length of stay. Cardiovascular disease was the single largest contributor for hospitalizations, length of hospital stay, and total costs. Readmission within 30 days of discharge, particularly for the same disorder, and multiday admissions should be the main targets for mitigation of hospital costs in this population.

Comparative study on safety, efficacy, and midterm results of balloon mitral valvotomy performed with triple lumen and double lumen mitral valvotomy catheters.

BACKGROUND: The triple lumen Inoue balloon is routinely used for balloon mitral valvotomy (BMV) in India. Its major limitation is the high cost. The double lumen Accura balloon is less expensive, making it an attractive alternative in the developing countries. The study was meant to assess the safety, efficacy and midterm results of Accura balloon with respect to the Inoue balloon. PATIENTS AND METHODS: 816 consecutive patients, who underwent elective BMV in this Institute from 1997 to 2003, were included in the study. The data of 487 patients who underwent BMV with Accura balloon was compared with 329 patients who underwent BMV with Inoue balloon. The clinical, echocardiographic, and hemodynamic data of these patients were analyzed retrospectively to assess the safety and efficacy of Accura balloon with respect to the Inoue balloon. RESULTS: Immediate procedural success (93.9% in Inoue group and 91.6% in Accura group p. NS) and complications (6.6% in Inoue group and 5.6% in Accura group p. NS) were comparable between the study groups. The two study population had similar restenosis rate and events at 1 year after BMV. Both balloons could be reused multiple times without compromising on the safety and effectiveness. Accura balloons were less costly than Inoue balloon. The reusability with Accura was slightly more and found to be more cost-effective. CONCLUSIONS: Both Accura and Inoue balloon mitral valvotomy balloons are effective in providing relief from hemodynamically significant mitral stenosis in terms of gain in valve area and reduction in trans mitral gradient. Both groups have similar procedural success and complication rates, restenosis, and follow-up events at 1 year. Both balloons could be reused multiple times and Accura balloon is found to be more cost effective.

Crisscross pulmonary artery with double aortic arch: an unusual association.

Anomalies of pulmonary artery origin are rare. Crisscross pulmonary artery origin is a rare benign anomaly characterized by the left pulmonary artery arising superiorly and to the right side of the right pulmonary artery. The condition is usually accompanied by a conotruncal anomaly. Here, we report a child with crisscross pulmonary arteries and a complete vascular ring formed by a double aortic arch, which was confirmed by computed tomography angiography. The child underwent surgical correction for relief of stridor.

Patient and physician perspectives on treatment burden in end-stage kidney disease: a nominal group technique study.

OBJECTIVES: The treatment workload associated with end-stage kidney disease (ESKD) is high. The treatment burdens experienced by patients with ESKD are not well understood. In this study, we aimed to elucidate the most important areas of treatment burden for discussion in a clinical encounter from the perspectives of patients with ESKD and nephrologists. We sought to explore possible solutions to these high priority treatment burden challenges. DESIGN: Nominal group technique (NGT) sessions. SETTING AND PARTICIPANTS: Three in-person NGT sessions were conducted with 19 patients with dialysis-dependent ESKD from one tertiary treatment centre (mean age 64 years; range 47-82). All patients were either retired or on a disability pension; 74% perceived moderate or severe treatment burden; and 90% spent more than 11 hours on treatment-related activities per week (range 11-30). One online NGT session was conducted with six nephrologists from two Australian states. MAIN OUTCOME MEASURES: The primary outcome was a ranked list of treatment burden priorities. The secondary outcome was potential solutions to these treatment burden challenges. RESULTS: Every patient group ranked health system issues as the most important treatment burden priority. This encompassed lack of continuity and coordination of care, dissatisfaction with frequent healthcare encounters and challenges around healthcare access. Psychosocial burdens on patients and families were perceived to be the most important area of treatment burden by physicians, and were ranked the second highest priority by patients. CONCLUSIONS: Discussing treatment burden in a clinical encounter may lead to a better understanding of patients' capacity to cope with their treatment workload. This could facilitate tailored care, improve health outcomes, treatment sustainability and patients' overall quality of life.

Percutaneous treatment of congenital coronary arteriovenous fistulas.

BACKGROUND: Congenital coronary arteriovenous fistulas (CAVF), although rare, can present as significant hemodynamic lesions necessitating intervention. METHODS AND RESULTS: Six patients (two males) with congenital coronary arteriovenous fistulas (CAVF) underwent percutaneous transcatheter occlusion. The ages ranged from 4 years to 49 years (mean 20.1 years). The fistulas had their origins from the right coronary artery (two), the left anterior descending coronary artery (two), and the left circumflex coronary artery (two). One of the fistulas drained to the right ventricle, four drained to the right atrium, and the remaining one to the left ventricle (LV). The fistulas were closed using the arterial approach with Cook™ coils in two patients and with nitinol ductal occluders (NDOs) using the venous approach in four patients. One patient developed dissection of the wall of the fistula during attempted closure and had spontaneous occlusion of the fistula. Complete occlusion of the fistulas were achieved in all patients. Complications consisted of migration and embolization of the coils in one patient (later closed successfully with NDO) and myocardial infarction occurring two weeks following successful closure in another patient. At mean follow-up of 39.6 +/- 22.9 months, all patients were asymptomatic and echo-Doppler evaluation revealed no residual fistulae. CONCLUSIONS: CAVF are very well amenable to percutaneous closure with acceptable morbidity and high success rates.

SARS-CoV-2 and the kidney.

BACKGROUND: COVID-19 has been at the forefront of public and scientific attention since the initial report in December 2019. The kidney is one of the target organs of the causative SARS-CoV-2 virus. OBJECTIVE: The aim of this article is to discuss the current understanding of COVID-19 renal disease from a primary care perspective, with the caveat that our knowledge of the pathogenesis, clinical course and outcome of the disease is still rapidly evolving. DISCUSSION: The kidney is one of the target organs of the causative SARS-CoV-2 virus, affecting the endothelium, podocytes and renal tubular epithelial cells. Clinical presentation ranges from isolated proteinuria, haematuria to severe acute kidney injury (AKI) requiring renal replacement therapy. Renal dysfunction associated with COVID-19 has a worse prognosis whether it be in the form of AKI or worsening of pre-existing chronic kidney disease, or in patients undergoing renal replacement therapy.

Percutaneous valvuloplasty for mitral valve restenosis: postballoon valvotomy patients fare better than postsurgical closed valvotomy patients.

AIM: To compare the results of percutaneous mitral valvuloplasty (BMV) for mitral restenosis in post-BMV versus postclosed mitral valvotomy (CMV) patients. METHODS AND RESULTS: Ninety-two patients who underwent BMV for mitral restenosis were followed up prospectively. Of these, 28 patients had undergone previous percutaneous mitral valvuloplasty (PRIOR BMV) and 64 patients had undergone previous closed mitral valvotomy (PRIOR CMV). BMV for mitral restenosis was a success in 59% patients (57.1% PRIOR BMV, 59.3% PRIOR CMV, P = 1.0). Incidence of severe mitral regurgitation was 3.25%, all in the PRIOR CMV group. In univariate analysis, the major predictor of successful BMV for mitral restenosis was Wilkins score (P = 0.004). At a follow up of 3.47 + 2.07 years, mitral valve area was similar between groups (1.45 +/- 0.22, 1.46 +/- 0.26, P = 0.35). The combined end points of mitral valve replacement (MVR), need for re-repeat BMV for mitral restenosis or death was higher in the PRIOR CMV group (31.2% PRIOR CMV, 7.1% PRIOR BMV, P = 0.027). Event-free survival at follow up was lower in the PRIOR CMV group (69% PRIOR CMV, 92.8% PRIOR BMV) mainly due to the higher need for MVR (11 vs. 0 patients, P = 0.03). CONCLUSIONS: In conclusion, following BMV for mitral restenosis, patients with PRIOR BMV are found to have lesser event rates on follow-up compared to patients with PRIOR CMV, though procedural success rates are similar.

Natural history of aortic valve disease following intervention for rheumatic mitral valve disease.

BACKGROUND AND AIM OF THE STUDY: A significant proportion of patients who require interventions for rheumatic mitral valve (MV) disease have coexisting aortic valve (AV) disease. To date, little is known of the natural history of AV disease in these patients. METHODS: The details of a cohort of 200 patients (146 females, 54 males; mean age at MV intervention 30.3 +/- 9.9 years) with rheumatic heart disease were retrospectively reviewed. The patients had undergone an index MV intervention (either closed or balloon mitral valvotomy) or MV replacement between 1994 and 1996, and received long-term regular follow up examinations. The clinical and echocardiographic data at entry and at follow up were noted. Patients were allocated to two groups, based on whether the AV disease was absent (group I, n=98) or present (group II, n=102) at baseline. The AV disease was categorized as thickening only (group IIA), isolated aortic regurgitation (AR) (group IIB), or combined aortic stenosis (AS) and AR (group IIC). No patient had isolated AS at baseline. RESULTS: The mean follow up period was 9.3 +/- 1.07 years; during which 10 patients in group I developed new AV disease, which included AV thickening only (n=2), trivial-mild AR (n=7) and mild AS with trivial AR (n=1). Of 16 patients in group IIA, 11 developed isolated AR, and one patient progressed to have mild AS and AR. Among 69 patients in group IIB, 22 (31.9%) developed AS, and all had either mild (n=8) or moderate (n=14) AR with mild AS. Group IIC included 17 patients with mild combined AV disease at baseline, except for moderate AS and moderate AR in one patient each. Among 16 patients with mild AS in group IIC, six progressed to moderate AS and two to severe AS. AR became moderate in 10 patients and severe in one patient. The two patients who progressed to severe AS requiring AV replacement had mild AS at baseline. No patient who developed new combined AV disease had lesions with severity more than mild AS or moderate AR. On logistic regression analysis of the variables predisposing to progression of AV disease, such as age, gender, history of rheumatic fever (RF) and recurrence, and interval from RF episode to symptom onset, only the initial AV gradient was identified as being statistically significant (beta coefficient 0.528, SE = 0.17, p < 0.0001). CONCLUSION: Patients with no or mild AV disease at the time of MV intervention rarely develop severe AV disease, and seldom require AV surgery over the long-term follow up. The presence of mild AS at baseline is predictive in the minority of cases where AV disease will progress relatively more rapidly.

Doppler echocardiographic assessment of TTK Chitra prosthetic heart valve in the mitral position.

AIMS: TTK Chitra heart valve prosthesis (CHVP), a tilting disc mechanical heart valve of low cost and proven efficacy, has been in use for the last 15 years. Although various studies substantiating its long-term safety and efficacy are available, no study had assessed its echocardiographic characteristics. The purpose of this study was to determine the normal Doppler parameters of CHVP in the mitral position and to assess whether derivation of mitral valve area (MVA) using the continuity equation (CE) and more commonly used pressure half-time (PHT) method is comparable in the functional assessment of this tilting disc mitral prosthesis. METHODS AND RESULTS: Doppler echocardiography was performed in 40 consecutive patients with CHVP in the mitral position. All patients were clinically stable, without evidence of prosthetic valve dysfunction such as significant obstruction or regurgitation, endocarditis, left ventricular dysfunction (ejection fraction <40%), or significant aortic regurgitation. Valve sizes studied included 25, 27, and 29 mm. Mitral valve area was derived both by the PHT method and by the CE, using the stroke volume measured in the ventricular outflow tract divided by the time-velocity integral of CHVP jet. The peak Doppler gradient ranged from 5 to 21 (mean 11.0) mmHg, and the mean gradient ranged from 1.7 to 9.2 (mean 4.1) mmHg. Mean gradient negatively correlated with an increase in the actual orifice area (AOA) derived from the valve orifice diameter given by the manufacturer (r = -0.45, P = 0.004). Mitral valve area calculated by both PHT and CE increased significantly with an increase in the AOA (r = 0.42, P = 0.007 and r = 0.32, P = 0.046, respectively). Mitral valve area by the CE averaged 1.55 +/- 0.36 cm(2) (range 0.85 cm(2) for a 25 mm valve to 2.41 cm(2) for a 29 mm valve) and was smaller than by PHT (mean 2.04 +/- 0.41 cm(2), range 1.40-3.14 cm(2); P = 0.0001; t-test), irrespective of whether PHT is less than or >110 ms. CONCLUSION: The Doppler parameters obtained with CHVP in the mitral position are comparable with those obtained with the different prosthetic valves in common use. In the selected group of patients with CHVP, assessment of MVA by the PHT method is comparable with that by the CE. Areas by both methods were smaller than the AOA provided by the manufacturer, as seen in other similar design valves.

Efficacy and safety of combination of extended release niacin and atorvastatin in patients with low levels of high density lipoprotein cholesterol.

OBJECTIVE: We investigated the safety and efficacy of combination therapy of extended release (ER) niacin and atorvastatin in patients with low HDL-C and compared the results with atorvastatin monotherapy. METHODS: This open label study recruited consecutive men and women who had coronary artery disease with HDL-C levels <35 mg/dL. These patients were already on atorvastatin therapy targeted to lower low density lipoprotein cholesterol (LDL-C), for a minimum period of 6 months. Group 1, n = 104 (mean age 52.7 years) received ER niacin in addition to atorvastatin and group 2 (n = 106) continued on atorvastatin (mean age 52.3 years). ER niacin dose was built up to a maximum of 1.5 g and atorvastatin dose titrated according to LDL levels in both the groups. The lipoprotein levels at baseline were similar (p = NS). RESULTS: At 9 +/- 1.8 months of follow-up, the mean dose of ER niacin was 1.3 g and atorvastatin 13.2 mg in group 1. In comparison, group 2 patients had mean atorvastatin dose of 15.9 mg. Patients in group 1 had significant elevation in HDL-C cholesterol (39.5 +/- 5.5 vs 35.7 +/- 4.5 mg/dL), reduction in total cholesterol (156.4 +/- 31 vs 164.5 +/- 39.3 mg/dL) and also LDL-C (88.9 +/- 28.3 vs 99.8 +/- 35.4 mg/dL) compared to group 2 (all p < 0.05). The magnitude of reduction in triglyceride levels was not significant between the groups (140.1 +/- 40.4 vs 145.2 +/- 46.5 mg/dL) (p = NS). No major adverse events or clinical myopathy occurred in either groups. Four patients (4%) discontinued ER niacin (2 due to gastro-intestinal symptoms and 2 due to worsening of diabetes). Flushing occurred in 3% patients, but none felt it to be troublesome. CONCLUSION: Adding ER niacin to atorvastatin exhibited beneficial effects on lipid profile with significant elevation of HDL-C cholesterol and further lowering of LDL-C compared to monotherapy. This treatment offered better targeted therapy and was well tolerated with proper monitoring in Indian patients.

Left ventricular regression after balloon atrial septostomy in d-transposition of the great arteries.

OBJECTIVES: Balloon atrial septostomy (BAS) is an effective palliative procedure in children with transposition of the great arteries and poor intercirculatory mixing. While the subpulmonary left ventricle (LV) is known to regress with time in these newborns due to the declining afterload, it has not been studied how it behaves following BAS and a consequent decrease in preload. The study was designed to examine the effects of BAS on the LV in infants with simple d-transposition of the great arteries by serial 2D echocardiographic monitoring. METHODS: This was a prospective echocardiographic follow-up study of all consecutive children with simple d-transposition of the great arteries who underwent BAS for restrictive interatrial communication and oxygen saturation below 75% between January 2014 and June 2015. Left ventricular mass estimation was done by M-mode transthoracic echocardiography before balloon septostomy and serially on follow-up till surgery. RESULTS: The median age of the 25 children studied was 4 days (1-95 days) when they underwent BAS. Twenty patients underwent arterial switch operation at a mean of 9 days from balloon septostomy. The mean baseline left ventricular mass was 47.9 g/m2, which decreased to 38.5, 36.2, 32.1, 32.4, 25.7 and 25.2 g/m2 on Days 1, 3, 6, 9, 12 and 15, respectively. The left ventricular mass decreased by 1.5 g/m2 every day during the first 2 weeks following balloon septostomy adjusted for the age of the child in days. Children who underwent BAS beyond 3 weeks of life had faster LV regression than those who underwent the procedure earlier (unstandardized regression coefficient ß 0.892, P < 0.001). CONCLUSIONS: BAS is associated with accelerated regression of the LV in infants with simple d-transposition of the great arteries in the first 2 weeks after the procedure. Regression of the LV is faster in children who underwent BAS after 3 weeks of age.

Long-term outcome of patients undergoing balloon mitral valvotomy in pregnancy.

The outcome of 36 patients (mean age 25.8 +/- 4.3 years) who underwent balloon mitral valvuloplasty during pregnancy is described in this report. The procedure was successful in 35 patients (97.2%), with no maternal mortality, and all patients subsequently had symptomatic improvement and uneventful deliveries. The children had normal growth and development at a follow up of 2.8 +/- 3.3 years. Thus, in patients with symptomatic severe mitral stenosis, balloon mitral valvuloplasty is feasible, safe, and effective with good long-term outcomes for both the mother and the fetus.

Ventricular septal rupture following myocardial infarction. Long-term survival of patients who did not undergo surgery. Single-centre experience.

Long-term survival is rare in patients not undergoing surgery after post-myocardial infarction ventricular septal rupture. We report our experience of seven patients out of 27, who did not undergo surgery and were followed up for a mean period of 2.8 years. They were evaluated after a mean period of 2.2 months after infarction in our centre. The septal defects measured 9.8 mms on average and the mean left-to-right shunt ratio was 1.98: 1. The mean pulmonary artery, right atrial and left ventricular end diastolic pressures were 28.3 +/- 10.6, 4 +/- 3 and 15.8 +/- 4.8 mm Hg, respectively. Only three out of seven patients had LV aneurysm and all patients had single-vessel disease. Smaller defect size, minimal left-to-right shunt and preserved right ventricular function may be the factors responsible for long-term survival.

Adverse outcome of human islet-allogeneic blood interaction.

BACKGROUND: A report of inflammatory damage when islets come into contact with allogeneic blood prompted us to confirm the finding. METHODS: Fresh handpicked human islets were incubated in blood group matched, nonsensitized allogeneic blood. Destruction was quantified by assaying the supernatants for proinsulin release and by blood clot histology. The effect on global hemostasis was assessed by thromboelastography (TEG), and heparin-bonded tubing was used to assess the effect on blood cellular counts. In separate experiments, islets were incubated in allogeneic blood with heparin or Reopro (monoclonal anti-GpIIbIIIa). Islets were also incubated in serum, and cryosections were stained for C1q, C4, C3, C5b-9, immunoglobulin (Ig)M, and IgG binding using immunohistochemistry. RESULTS: Histologic assessment showed severe destruction in 37% of islets in contact with allogeneic blood versus none in controls and a sevenfold increase in proinsulin release from controls (n = 6)(P < 0.005). TEG (n = 11) showed accelerated coagulation in the presence of islets (P < 0.001). Analysis of blood cellular counts (n = 3) showed consumption of platelets, neutrophils, and monocytes in the presence of islets (P < 0.001). Inhibition of coagulation with heparin (n = 3) or inhibition of platelet aggregation with Reopro (n = 3), separately or together (n = 3), did not make a substantial improvement in the destruction in terms of histology or proinsulin release. Immunohistochemical staining (n = 4) revealed C1q, C4, C3, and C5b-9 deposition along with IgG binding. IgM binding was weak if anything. CONCLUSION: This study confirms and extends the finding that human islet-allogeneic blood interaction results in significant destruction of islet tissue with activation of the coagulation cascade and platelet, neutrophil, and monocyte consumption. There was evidence for activation of complement by the classical pathway along with IgG binding.

Usefulness of transthoracic echocardiography for identification of left atrial thrombus before balloon mitral valvuloplasty.

The objectives of this study were (1) to identify clinical and transthoracic echocardiographic features of patients with mitral stenosis who have thrombus recognized on transesophageal echocardiography, and (2) to define a clinical and echocardiographic model to predict the presence of left atrial thrombus in these patients.

Supravalvar aortic stenosis: clinical and hemodynamic profile, and surgical outcome.

BACKGROUND: Supravalvar aortic stenosis is the rarest of left ventricular outflow obstructions. Data on this rare entity from India are scarce. METHODS AND RESULTS: We retrospectively analyzed the data of 15 patients (13 males, mean age 15.5+/-10.18 years) with a diagnosis of supravalvar aortic stenosis confirmed by cardiac catheterization. Five patients had morphological features of Williams' syndrome. One patient had diffuse while the rest had discrete type of supravalvar aortic stenosis. Five patients did not have any associated lesions. A 9-year-old male had an ascending aortic aneurysm, and 3 patients had associated peripheral pulmonary artery stenosis. One child had a subaortic ventricular septal defect, and another had severe mitral regurgitation. Twelve patients had electrocardiographic evidence of left ventricular hypertrophy. Three patients had mild aortic valvar stenosis while 2 had aortic regurgitation. Six patients had dilated coronary arteries. Two patients with supravalvar aortic gradients of 20 and 40 mmHg were kept on close follow-up. One patient was not willing to undergo surgery while the other is awaiting surgery. Eleven patients underwent surgical correction. Dacron or pericardial patch aortoplasty was done in all the patients. In addition, one patient each underwent pulmonary artery plasty, ventricular septal defect closure, repair of ascending aortic aneurysm, and mitral valve replacement. The patient with diffuse type of supravalvar aortic stenosis underwent augmentation aortoplasty. Two patients died perioperatively. One was lost to follow-up. Two had moderate residual gradients. The rest of the patients were in New York Heart Association functional class I on follow-up of 6.3+/-4.7 years. CONCLUSIONS: Repair of supravalvar aortic stenosis by single sinus aortoplasty is safe and produces good results.