Kidney enlargement and multiple liver cyst formation implicate mutations in PKD1/2 in adult sporadic polycystic kidney disease.

Distinguishing autosomal-dominant polycystic kidney disease (ADPKD) from other inherited renal cystic diseases in patients with adult polycystic kidney disease and no family history is critical for correct treatment and appropriate genetic counseling. However, for patients with no family history, there are no definitive imaging findings that provide an unequivocal ADPKD diagnosis. We analyzed 53 adult polycystic kidney disease patients with no family history. Comprehensive genetic testing was performed using capture-based next-generation sequencing for 69 genes currently known to cause hereditary renal cystic diseases including ADPKD. Through our analysis, 32 patients had PKD1 or PKD2 mutations. Additionally, 3 patients with disease-causing mutations in NPHP4, PKHD1, and OFD1 were diagnosed with an inherited renal cystic disease other than ADPKD. In patients with PKD1 or PKD2 mutations, the prevalence of polycystic liver disease, defined as more than 20 liver cysts, was significantly higher (71.9% vs 33.3%, P = .006), total kidney volume was significantly increased (median, 1580.7 mL vs 791.0 mL, P = .027) and mean arterial pressure was significantly higher (median, 98 mm Hg vs 91 mm Hg, P = .012). The genetic screening approach and clinical features described here are potentially beneficial for optimal management of adult sporadic polycystic kidney disease patients.

Once-weekly teriparatide in hemodialysis patients with hypoparathyroidism and low bone mass: a prospective study.

UNLABELLED: Once-weekly 56.5-µg teriparatide treatment was significantly associated with the increase in lumbar spine bone mineral density at 48 weeks among hemodialysis patients with hypoparathyroidism and low bone mass; however, discontinuation of treatment because of adverse events was frequently observed. Careful monitoring for adverse events should be required. INTRODUCTION: Once-weekly 56.5-µg teriparatide is reportedly effective for treating osteoporotic patients without renal insufficiency. However, little is known about the efficacy and safety of once-weekly teriparatide in hemodialysis patients. METHODS: We conducted a 48-week prospective, observational cohort study including 22 hemodialysis patients aged 20 years or older with hypoparathyroidism and low bone mass who received once-weekly teriparatide at 56.5 µg at a tertiary care hospital between January 2013 and January 2015. Primary outcomes were within-subject percent changes of bone mineral density (BMD) at the lumbar spine, femoral neck, and distal one-third radius at 24 and 48 weeks. Secondary outcomes included percent changes of serum bone turnover markers (osteocalcin, bone-specific alkaline phosphatase (BAP), N-terminal propeptide of procollagen type 1 (P1NP), and tartrate-resistant acid phosphatase 5b (TRAP-5b)). Adverse events were evaluated. RESULTS: The BMD increased at the lumbar spine by 3.3 ± 1.9 % (mean ± SEM) and 3.0 ± 1.8 % at 24 and 48 weeks but not in the femoral neck and distal one-third radius. Serum osteocalcin, BAP, and P1NP increased significantly at 4 weeks, maintaining higher concentrations up to 48 weeks, although TRAP-5b decreased gradually during treatment. The baseline BAP was significantly associated with the 48-week percent change in lumbar spine BMD. Transient hypotension was the most common adverse event. Ten patients discontinued treatment because of adverse events. CONCLUSIONS: Once-weekly teriparatide was associated with increased lumbar spine BMD in hemodialysis patients with hypoparathyroidism and low bone mass. Careful monitoring should be required for treatment of such patients.

Cyst infection in autosomal dominant polycystic kidney disease: causative microorganisms and susceptibility to lipid-soluble antibiotics.

Cyst infection is a frequent and serious complication of autosomal dominant polycystic kidney disease (ADPKD). Lipid-soluble antibiotics like fluoroquinolones show good penetration into cysts and are recommended for cyst infection, but causative microorganisms are often resistant to these agents. This study investigated the profile of the microorganisms causing cyst infection in ADPKD, their susceptibility to lipid-soluble antibiotics, and clinical outcomes. This retrospective study reviewed all ADPKD patients admitted to Toranomon Hospital with a diagnosis of cyst infection from January 2004 to March 2014. All patients who underwent cyst drainage and had positive cyst fluid cultures were enrolled. Patients with positive blood cultures who satisfied our criteria for cyst infection or probable infection were also enrolled. There were 99 episodes with positive cyst fluid cultures and 93 episodes with positive blood cultures. The majority of patients were on dialysis. The death rate was high when infection was caused by multiple microorganisms or when there were multiple infected cysts. Gram-negative bacteria accounted for 74-79 % of the isolates in all groups, except for patients with positive hepatic cyst fluid cultures. The susceptibility of Escherichia coli to fluoroquinolones was very low in patients with hepatic cyst infection, especially those with frequent episodes and those with hepatomegaly. Fungi were detected in two episodes. Fluoroquinolone-resistant microorganisms showed a high prevalence in cyst infection. It is important to identify causative microorganisms to avoid the overuse of fluoroquinolones and to improve the outcome of cyst infection in ADPKD.

Impact of tubulointerstitial lesions on anaemia in patients with biopsy-proven diabetic nephropathy.

AIMS: To investigate the relationship between the progression of anaemia and renal pathological findings in patients with diabetic nephropathy. METHODS: A total of 223 patients with diabetes underwent renal biopsy from 1985 to 2010 and were confirmed to have pure diabetic nephropathy according to the recent classification, of whom 113 (baseline haemoglobin ≥ 11 g/dl) were enrolled in the study. Linear regression analysis was used to estimate the changes in haemoglobin levels during the follow-up period. RESULTS: In a multivariate model adjusted for clinical and histopathological variables, higher interstitial fibrosis and tubular atrophy scores were more strongly associated with a decrease in haemoglobin levels than were lower scores. Compared with an interstitial fibrosis and tubular atrophy score of 0, the standardized coefficients for interstitial fibrosis and tubular atrophy scores of 1, 2 and 3 were 0.20 (95% CI -0.31 to 0.93), 0.34 (95% CI -0.22 to 1.34) and 0.47 (95% CI 0.07 to 1.96), respectively, whereas a higher glomerular class, a higher vascular lesion score and the presence of exudative lesions were not strongly correlated with the decrease in haemoglobin. CONCLUSIONS: Tubulointerstitial lesions that are more advanced are significantly associated with the progression of anaemia in patients with diabetic nephropathy after adjustment for numerous covariates. This finding suggests that tubulointerstitial lesions may be a useful prognostic indicator for anaemia in patients with diabetic nephropathy, and that decreased erythropoietin production attributable to the progression of tubulointerstitial lesions is a major cause of anaemia in these patients.

Bone histomorphometry in a long-term hemodialysis patient with hypoparathyroidism and sarcoidosis.

A bone biopsy specimen in a long-term hemodialysis patient with sarcoidosis coexisting with severe hypoparathyroidism has demonstrated that a persistent near physiological level of 1,25-dihydroxyvitamin D3 contributes to the preservation of bone remodeling and has the potential to retard the development of vascular calcification and atherosclerosis. Sarcoidosis-related hypercalcemia and hypoparathyroidism, which is characterized by 1,25-dihydroxyvitamin D3 (1,25(OH)2D3) overproduction, is rarely seen in hemodialysis patients. Herein, we describe a 60-year-old Japanese woman on hemodialysis for 35 years who presented with malaise and hypercalcemia. Severe hypoparathyroidism without parathyroidectomy and a preserved 1,25(OH)2D3 level were detected. Computed tomography showed bilateral axillary lymphadenopathy and minimal aortic and soft tissue calcification. The axillary node biopsy led to a definite diagnosis of sarcoidosis. A bone biopsy specimen obtained from the right iliac crest showed remodeling of normal lamellar bone with scalloped cement lines and clear double labeling by tetracycline on fluorescence microscopy. Histomorphometric analysis revealed that the bone formation rate was preserved (30.0 %/year), together with a decrease of osteoid volume (5.75 %) and fibrous volume (0 %), indicating that the patient did not have adynamic bone disease and only showed mild disease. This is the first documented case of sarcoidosis-related hypercalcemia associated with severe hypoparathyroidism in a long-term hemodialysis patient who underwent bone histomorphometry. Our findings suggest that, in hemodialysis patients with sarcoidosis coexisting with severe hypoparathyroidism, a persistent near physiological level of 1,25(OH)2D3 contributes to the preservation of bone remodeling and has the potential to retard the development of vascular calcification and atherosclerosis.

Impact of the presence and duration of donor-specific antibodies on renal function.

BACKGROUND: Although anti-human leukocyte antigen (HLA) antibodies (DSA) is associated with graft loss, 3 things remain unclear: whether the duration and strength of DSA affect renal function; what mean fluorescence intensity (MFI) cut-off should be used; and whether the DSA effect is additive in case of multiple DSAs. METHODS: A study was made of 63 patients who received living donor kidney transplants with clonal deletion protocol and were followed up for 18 months with reduced doses of immunosuppressants. DSA was tested for monthly, using Luminex Mixed and Single Antigen beads (One Lambda, Inc., Canoga Park, CA, USA). Decrease of estimated glomerular filtration rate (eGFR) was obtained at baseline and 18 months after transplantation. Association of renal damage and DSAs was compared using several DSA models with several MFI cut-offs. RESULTS: Additive DSA models always showed better association with renal damage than comprehensive models. When calculating the DSA effect in additive models, "proxy-area under the curve" (AUC)-a triangular approximation of the actual AUC-showed better association with renal damage than did DSA duration (R(2) = 0.105 vs 0.087). Adjusting for other factors, 27% of the variation of GFR change was explained by proxy-AUC. No significant change of association occurred if the MFI cut-off level changed from 1000 to 3000. CONCLUSION: Our results support the association of DSA with development of longitudinal renal damage. The clinical interpretation may be similar at MFI cut-offs of 1000, 2000, and 3000. An additive DSA effect may be expected in patients with multiple DSAs. Our study suggests the importance of frequently checking for DSA and reducing their MFI value to minimize renal damage by the antibodies.

A case of familial lecithin-cholesterol acyltransferase deficiency on hemodialysis for over 20 years.

We trace the 34-year history of a member of the first Japanese family in which lecithin-cholesterol acyltransferase (LCAT) deficiency was diagnosed. Marriage between cousins with low LCAT activity was responsible for familial LCAT deficiency (FLD). In 1976, a 27-year-old Japanese man was noted to have FLD based on proteinuria, hematuria, grayish corneal opacity and low LCAT activity (9.83%). Genetic analysis showed insertion of G-G-C coding glycine at codon 141. Total cholesterol (C) was low at 108 mg/dl and the ratio of C-ester to total C was very low (12%), while the lecithin (phosphatidylcholine) level was very high (97.3%). When his serum creatinine reached 2.6 mg/dl at the age of 41 years (in 1991), renal biopsy was performed. This showed expansion of the mesangial matrix and irregularly thickened capillary walls with a bubble-like appearance because of lipid deposits consisting of two components (partly lucent vacuolated areas and partly deeply osmiophilic areas). Magnification of the latter deposits showed curvilinear and serpiginous striated membranous structure. Hemodialysis was started in 1990 and has been continued for over 20 years until August 2010. Clinical problems have included AV shunt failure requiring 4 operations and 13 percutaneous transcatheter angioplasty procedures, as well as episodes of hemolytic anemia that subsided after infusion of fresh frozen plasma. Cardiovascular events have not yet occurred, although severe calcification of abdominal aorta has been detected by computed tomography.

[Redo-operation for the cusp perforation 5 years after aortic valve replacement with stentless bioprosthesis; report of a case].

A 31-year-old male presented with increase of aortic valve regurgitation 5 years after implantation of Prima Plus Stentless bioprosthesis in a bicuspid aortic valve. He underwent redo aortic valve replacement with a mechanical valve concomitant with replacement of the ascending aorta. Pathological examination of the explanted stentless valve presented no inflammatory cell infiltration. The prosthetic valve regurgitation was considered to be due to small injury at the 1st operation.

Hepatitis C virus-related kidney disease: various histological patterns.

BACKGROUND: Although hepatitis C virus (HCV) infection is known to be associated with Type 2 cryoglobulinemic glomerulopathy (CG), only a few reports about other types of nephropathy have been published. METHODS: 68 HCV antibody positive patients in whom renal biopsy had been performed for persistent proteinuria, hematuria, and/or renal dysfunction between 1992 and 2008 at our institute were included. The histological, clinical and laboratory characteristics including the age, gender, hypertension, diabetes mellitus, liver histology (chronic hepatitis or liver cirrhosis), HCV-RNA, HCV genotype, splenomegaly, gastroesophageal varices, serum creatinine, hemoglobin, platelet count, rheumatoid factor, cryoglobulin, IgG, IgA, IgM, CH50, C3, C4, creatinine clearance, 24-h protein excretion, and hematuria, between their nephropathy with and without immune deposition were compared. RESULTS: Nephropathy was classified into two groups based on the detection of immune deposits by immunofluorescence microscopy: i.e., a positive group (n = 39) and a negative group (n = 29). The former group was further classified into three types of nephropathy: IgG dominant group (n = 10) (including membranous nephropathy (MN)), IgA dominant group (n = 20) (including IgA nephropathy (IgAN)), membranoproliferative glomerulonephritis (MPGN) (IgA type)), and IgM dominant group (n = 9) (MPGN apart from the IgA type). The latter group included diabetic nephropathy (n = 13), focal glomerular sclerosis (n = 4), and benign nephrosclerosis (n = 3), malignant nephrosclerosis (n = 1), tubulointerstitial nephritis (TIN) (n = 2), minimal change nephrotic syndrome (n = 1), cast nephropathy (n = 1), granulomatous TIN (n = 1), and others (n = 3). An increased serum IgM level, hypocomplementemia, splenomegaly, thrombocytopenia, liver cirrhosis, hematuria, and a high HCV RNA level were features of patients with MPGN of IgM dominant group (consistent with "CG"). CONCLUSIONS: Our results showed various histological patterns of HCV-related kidney disease and the specificity of CG, and revealed that a minority of HCV patients (n = 7) presented typical CG, while IgAN, MN, and diabetic nephropathy were more frequent.

The long-term survival rate of catecholamine-resistant septic shock in Japanese patients who received vasopressin therapy.

BACKGROUND: Septic shock is associated with vasopressin deficiency and hypersensitivity to its exogenous administration. The aim of this study is to review the 28-day survival rate, hemodynamic and renal effects of vasopressin therapy in refractory septic shock Japanese patients. METHODS: 55 Japanese patients experiencing catecholamine-resistant septic shock were treated with vasopressin. Hemodynamic alterations and the serum concentrations of aspartate aminotransferase, total bilirubin and creatinine clearance were evaluated following vasopressin treatment. RESULTS: In both, survivors and non-surviving patients, treatment with vasopressin resulted in a significantly increase in mean arterial pressure, hourly urine output, and a significant decrease in heart rate and total pressor dosage requirements. Creatinine clearance was significantly increased only in survivors. There were no significant changes in the serum concentrations of aspartate aminotransferase and total bilirubin. The 28-day survival rate was 45% (25 patients). CONCLUSIONS: In Japanese septic shock patients, vasopressin infusion improved hemodynamic status and reduced catecholamine requirement, and 28-day survival rate was 45%.

A patient with pregnancy-related acute abdomen after hemodialysis for over 18 years.

Because pregnancy is rare in women with end-stage renal disease, dialysis patients have not been reported to present with acute abdominal symptoms related to pregnancy including ectopic pregnancy. A 41-year-old woman treated with hemodialysis for over 18 years was brought to the emergency room at our institution because of acute abdominal pain. Ultrasonography detected an abdominal fluid collection, and her anemia had worsened (hematocrit 18%). Emergency laparoscopic exploration disclosed a hemorrhagic corpus luteum of pregnancy, causing ovarian bleeding on the left. Coagulation of bleeding points was carried out. At this time, pregnancy at 7 weeks of gestation was discovered. After the procedures, hemodialysis frequency was increased to 5 times weekly, and an erythropoietin derivative was administered to maintain a hematocrit above 30%. The patient developed no hypertension. At 33 weeks of gestation, cesarean section was performed because of a decrease in amniotic fluid and frequent late deceleration of the fetal heart rate. A live baby girl weighing 1,422 g was born. The successful pregnancy reflects remarkable progress in dialysis technology. Pregnancy, then, can underlie an acute abdomen in childbearing-age women (14 - 44 years old) undergoing long-term dialysis.

[Surgical treatment for ischemic heart disease].

BACKGROUND: Surgical treatment for ischemic heart disease (IHD) has changed after the administration of off-pump coronary artery bypass grafting (CABG) [OPCAB] and left ventricular restoration (LVR). We studied the development of the treatment and the surgical results. PATIENTS AND METHODS: Since May 2000 when the indication for OPCAB and LVR was defined, surgical treatment for IHD has been performed in 1,251 patients. The age ranged from 32 to 91 (mean 66 +/- 10) years and there were 977 men and 274 women. The elective operation was 1,130 and emergency 121. Definite indication for OPCAB was calcified ascending aorta, significant cerebrovascular disease, hemorrhagic tendency, and single vessel lesion. Conventional CABG (C-CAB) was the first choice and morbidity and surgical results were examined. RESULTS: OPCAB was performed in 297 (29.9%) and combined operation with CABG was required in 258 patients (20.6%). In elective operation, hospital mortality was one in OPCAB and one in C-CAB. In OPCAB and C-CAB, stroke was none and one, and mediastinitis was 0 and 0, respectively. CONCLUSION: The technique for OPCAB is necessary for CABG; however, it is not appropriate to persist with only OPCAB for CABG. Combined operation is often required with CABG and it is essential to perform precise C-CAB.

Multicentric Castleman disease with secondary AA renal amyloidosis, nephrotic syndrome and chronic renal failure, remission after high-dose melphalan and autologous stem cell transplantation.

Multicentric Castleman disease is a systemic lymphoproliferative disease with incomplete understood etiology. The various renal complications of this disease may include minimal change disease, mesangial proliferative glomerulonephritis, membranous glomerulonephritis and nephrotic syndrome, caused by secondary amyloidosis. In several reported cases of localized Castleman disease associated with renal amyloidosis and nephrotic syndrome, resection of organs involved by lymphoid proliferation resulted in complete remission. However, therapy of multicentric Castleman disease with renal amyloidosis is not well-established. We treated a case of a 39-year-old woman with multicentric Castleman disease complicated by nephrotic syndrome caused by secondary AA amyloidosis. The patient underwent autologous peripheral blood stem cell transplantation (auto-PBSCT), achieving complete remission. Autologous stem cell transplantation may be an attractive choice in therapy for refractory multicentric Castleman disease.

A pediatric occurrence of crescentic glomerulonephritis associated with antineutrophil cytoplasmic antibodies and mesangial IgA deposits.

Antineutrophil cytoplasmic antibody-(ANCA) associated glomerulonephritis usually shows histopathologic features of pauciimmune crescentic glomerulonephritis and occurs late in life. We report a 14-year-old Japanese girl presenting with proteinuria, hematuria and mildly elevated serum creatinine. A renal biopsy specimen demonstrated crescentic glomerulonephritis, immunofluorescence showed mesangial IgA staining. Electron microscopic examination disclosed paramesangial deposits. Serum ANCA against myeloperoxidase (MPO) were detected at high titers. Myeloperoxidase-ANCA-related nephritis accompanied by IgA nephropathy is considered rare in childhood and teen years. Yet, if ANCA assays and detailed electron microscopic examination of renal specimens were performed routinely in patients with rapidly progressive glomerulonephritis, the diagnosis might be more frequent in young patients.

Lecithinized Cu, Zn-superoxide dismutase limits the infarct size following ischemia-reperfusion injury in rat hearts in vivo.

Covalent binding of 4 molecules of phosphatidylcholine palmitoyl to human recombinant superoxide dismutase (SOD) results in a compound (lecithinized SOD) that has a longer half-life and greater affinity to the cell membrane than unmodified SOD. We investigated whether lecithinized SOD played a protective role against myocardial ischemia-reperfusion injuries in rats. Rats underwent 45 min of myocardial ischemia by occluding the left coronary artery followed by 120 min of reperfusion. They were randomly assigned to receive either lecithinized SOD, polyethylene glycol conjugated SOD (PEG-SOD), unmodified SOD, free lecithin derivative, or PBS intravenously at 5 min prior to reperfusion. Myocardial infarct area assessed by TTC staining was smaller in lecithinized SOD group than PEG-SOD, unmodified SOD, free lecithin derivative or control group. Blood pressure and heart rate was similar in each group. ELISA demonstrated SOD level in the heart was significantly high in lecithinized SOD group, especially in the heart of ischemia at risk. Although serum SOD level of PEG-SOD was as high as lecithinized SOD, SOD level of the heart was low. These data suggested lecithinized SOD had a protective effect in myocardial ischemia-reperfusion injuries through its increased bioavailability.

Left ventricle restoration in patients with non-ischemic dilated cardiomyopathy: risk factors and predictors of outcome and change of mid-term ventricular function.

OBJECTIVE: The partial left ventriculectomy (PLV) for end-stage dilated cardiomyopathy (DCM) which worked in some patients has been reported, although the hospital mortality is high. To reduce hospital mortality, we selected operative procedures of left ventricular (LV) restoration to improve the operative results. We analyzed the risk factors and predictors of outcome, and the mid-term changes of the LV function were determined. PATIENTS AND METHODS: Between December 1996 and September 2000, 74 patients with non-ischemic DCM received LV restoration. The age ranged from 14 to 76 years (mean, 49.0+/-14.0 years), and there were 63 men and 11 women. The etiology of the DCM was idiopathic DCM in 49 patients, and dilated hypertrophic cardiomyopathy in seven patients and others in 18. The preoperative New York Heart Association (NYHA) functional class was 29 in class III and 45 in class IV, in which 32 patients depended on inotropic support. PLV or septal anterior ventricular exclusion (SAVE) was selected depending on the akinetic lesion of the LV based on the intraoperative echo-test. Fifty-six patients received elective operations, and emergency operations were performed in 18 patients. The risk factors and predictors of outcome were analyzed in 74 patients, and in 35 patients who survived more than 1 year after receiving LV restoration, the mid-term cardiac function was examined by cardiac echogram and catheterization. RESULTS: PLV was performed in 62 patients and SAVE in 12 patients. Concomitant mitral surgery was performed in 66 patients (89%) and tricuspid annuloplasty in 42 patients (57%). There were 15 hospital deaths and 13 patients died after discharge from the hospital (cardiac deaths in nine and non-cardiac deaths in four). In the 46 late survivors, the NYHA class was I or II in 42 patients and III in four patients. Selection of the procedure of LV restoration (P<0.01), elective operation (P<0.05), and the preoperative volume of LV (endodiastolic volume index of <180 ml/m(2); P<0.05) were risk factors and predictors influencing hospital and late death. After the operation, the LV function improved significantly and the improvement was maintained at the mid-term period; the LV ejection fraction was 31.8+/-7.9% (P<0.01) at 1 year from 23.0+/-7.3% preoperatively, left ventricular diastolic diameter was 62.8+/-10.9 (P<0.01) from 81.7+/-8.2 mm and the LV endosystolic volume index was 88.5+/-45.8 (P<0.05) from 162.6+/-41.6 ml/m(2). CONCLUSIONS: The operative results improved with the selection of the procedures, with elective operation, and mitral plasty for less cardiac dilatation. The mid-term results of clinical status and LV function showed the effectiveness of the operation.

Effects of chondroitin sulfate on colitis induced by dextran sulfate sodium in rats.

Chondroitin sulfate (CS) is currently marketed as a therapeutic drug for neurodynia, lumbago and arthrodynia. Recently, many clinical studies have demonstrated the therapeutic effects of orally administered CS against diseases with inflammation. Furthermore, these reports suggest CS plays an important role in the protection of the base of ulcers and has anti-inflammatory activity. We investigated the effects of CS against dextran sulfate sodium (DSS)-induced rat colitis. Rats were given 3% DSS solution for 10 days ad libitum. CS and 5-aminosalicylic acid (5-ASA) were orally administered daily. The doses of the CS groups were 20 or 100 mg/kg and that for the 5-ASA group was 100 mg/kg. Evaluations were made of bloody stools, areas of erosion and hematological data. CS improved the symptoms of bloody stools, erosion and increase of white blood cells. Especially, CS (100 mg/kg) group showed markedly more improvement than the 5-ASA group. We think that the major mechanism of the therapeutic effects of CS are the prevention of tissue damage by the protection of digestive mucosa and anti-inflammatory effects. Therefore, CS may have therapeutic value for alimentary tract diseases such as inflammatory bowel disease or ulcer.

[Left ventriculoplasty for non-ischemic cardiomyopathy with severe heart failure in 70 patients].

OBJECTIVES: Treatment of cardiac failure due to non-ischemic cardiomyopathy by left ventriculoplasty using partial left ventriculectomy (Batista operation) or septal anterior ventricular exclusion was evaluated. METHODS: Left ventriculoplasty was performed in 70 patients (59 men and 11 women with a mean age of 51 years) from December 1996 to June 2000. Preoperative New York Heart Association (NYHA) functional class was IV in 43 patients including 29 receiving inotropic support, and class III in 27 patients. Nineteen patients required emergency surgery because of on-going shock and 51 patients were operated electively. Combined cardiac procedures were mitral valve reconstruction in 62 patients (45 replacements, 17 repairs), tricuspid annuloplasty in 37, and aortic valve replacement in 4. The initial 24 patients underwent typical Batista operation regardless of myocardial viability. The other 46 patients underwent selective ventriculoplasty to excise or exclude the weakest part according to the findings of the intraoperative echo-guided volume reduction test. RESULTS: The intraaortic balloon pump was used in 12 patients and the left ventricular assist device in 2 patients. Three (5.9%) of the 51 patients who underwent elective operation and 12 (63.2%) of the 19 patients with emergency operation died in the hospital, giving an overall hospital mortality of 21.4% (15/70). Hospital mortality was reduced from 33.3% (8/24) in the initial 24 patients to 15.2% (7/46) in the recent 46 patients with the volume reduction test. Mean ejection fraction increased from 22.2 +/- 6.7% to 29.6 +/- 6.0%. Diastolic dimension decreased from 81.1 +/- 9.5 to 69.8 +/- 19.2 mm. End-diastolic and systolic volume indices decreased from 199.0 +/- 47.9 to 124.1 +/- 34.9 ml/m2 and from 154.0 +/- 41.2 to 89.3 +/- 31.7 ml/m2, respectively, at one postoperative month in the 55 hospital survivors. The mean pulmonary capillary wedge pressure decreased from 25.6 +/- 7.8 to 13.6 +/- 4.5 mmHg. Serum brain natriuretic peptide decreased from 999 +/- 647 preoperatively to 547 +/- 362 pg/ml one month after the operation. Thirteen patients (18.6%) died in the late period mainly due to heart failure. Among the 42 survivors, 37 patients returned to NYHA functional class I-II, and cardiac events were rare after one postoperative year. Actuarial survivals at 3 years in elective and emergency operations were 71.9% and 33.3%, respectively. CONCLUSIONS: Left ventriculoplasty is acceptably safe for elective operation and clinical improvement can be obtained by proper surgical procedures and careful postoperative medical treatment.

Effects of antihypertensive therapy on blood pressure and left ventricular hypertrophy in patients with severe hypertension.

The mechanisms responsible for regression of left ventricular (LV) mass with antihypertensive therapy in patients with severe hypertension remain unclear. This study was designed to examine whether systolic and diastolic blood pressures are associated with changes in LV mass. Eighteen patients with essential hypertension whose average seated diastolic blood pressure was >or = 110 mm Hg were enrolled in the study. All patients were administered antihypertensive therapy and underwent M-mode echocardiography before and after 6 months of treatment. In all patients, antihypertensive treatment significantly reduced systolic blood pressure from 175 +/- 21 mm Hg at baseline to 143 +/- 22 mm Hg at 6 months (p < 0.001), and diastolic blood pressure from 116 +/- 7 mm Hg at baseline to 92 +/- 20 mm Hg at 6 months (p < 0.001). LV mass index at 6 months was significantly reduced compared to its baseline value (p < 0.05). Change (value at 6 months-value at baseline) in systolic and diastolic blood pressures correlated positively with the change in LV mass index (r = 0.61, p < 0.01 and r = 0.71, p < 0.001, respectively). The patients were divided into responders. whose LV mass regressed by > or = 10% (n = 9), and nonresponders, whose LV mass regressed by < 10% (n = 9). Systolic (p < 0.001) and diastolic (p < 0.001) blood pressures. interventricular septal thickness (p< 0.05), posterior wall thickness (p < 0.001), and LV mass index (p < 0.001) were significantly decreased in the responders, but not in the nonresponders, at 6 months compared with those at baseline. Systolic (p < 0.05) and diastolic (p < 0.05) blood pressures in nonresponders were significantly higher than those in the responders at 6 months. The changes in systolic and diastolic blood pressures did not correlate with the change in LV mass index in the responders or the nonresponders. The regression of LV mass is strongly affected by reducing blood pressure. This is the first study using antihypertensive therapy to demonstrate that a change in blood pressure correlates positively with changes in LV mass index in severely hypertensive patients.