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Query: UMLS:C0243026 (sepsis)
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Extracorporeal membrane oxygenation (ECMO) is used as circulatory support or bridge to transplantation in patients with severe left ventricular (LV) dysfunction. Left heart decompression is needed to reduce pulmonary edema, prevent pulmonary hemorrhage, and reduce ventricular distention that may aid in recovery of function. We reviewed our experience from November 1993 to December 1997 with 10 patients having severe LV dysfunction (7 myocarditis, 3 dilated cardiomyopathy) who required circulatory support with ECMO and who underwent left heart decompression with blade and balloon atrial septostomy (BBAS). Patients ranged in age from 1 to 24 years (median, 3 years). Indications for BBAS included left atrial/left ventricular distension (10), pulmonary edema/hemorrhage (9), or severe mitral regurgitation (2). BBAS was performed electively in eight patients and urgently in two patients. BBAS was performed while on ECMO in seven patients and pre-ECMO in three. A femoral venous approach was used in all patients. ECMO patients were fully heparinized. Transseptal puncture was required in nine patients while one patient had a patent foramen ovale. Blade septostomy was performed in all patients. Enlargement of the defect was then performed by stationary balloon dilation in nine and Rashkind balloon atrial septostomy in one. Balloon diameters ranged from 10 to 20 mm. Sequential balloon inflations were performed in some patients. Adequacy of the atrial septal defect (ASD) was confirmed by pressure measurement and echocardiography. Adequate left heart decompression was achieved in all patients. Pulmonary edema improved in nine of nine patients. Left atrial mean pressure fell from a mean of 30.5 mm Hg, (range, 12-50 mm Hg) to 16 mm Hg (range, 9-24 mm Hg). Left atrial to right atrial pressure gradient fell from a mean of 20 mm Hg pre-BBAS to 3 mm Hg post-BBAS. ASDs ranged in size from 2.5 to 8 mm (mean, 5.9 mm). Complications included needle perforation of the left atrium without hemodynamic compromise (one), ventricular fibrillation requiring defibrillation (one), and hypotension following BBAS which responded to volume infusion (two). Duration of ECMO ranged from 41 hr to 704 hr (mean, 294 hr). Seven patients survived and four patients had recovery of normal LV function. Of those who recovered, two had no ASD at follow-up while two ASDs are patent 14 days and 3 months post-BBAS. Three patients underwent successful cardiac transplantation. Three patients died, all of whom had multisystem organ failure with or without sepsis. A patent ASD was noted at transplant (three) or autopsy (two). No patient required a second BBAS. BBAS alleviates severe left atrial hypertension and pulmonary edema. In addition, BBAS avoids the potential bleeding complications of surgical left heart decompression. Stationary balloon dilation of the atrial septum is an effective alternative to Rashkind balloon septostomy in older patients. BBAS achieves left heart decompression that may permit recovery of LV function or allow extended ECMO support as a bridge to transplant.
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PMID:Blade and balloon atrial septostomy for left heart decompression in patients with severe ventricular dysfunction on extracorporeal membrane oxygenation. 1034 39

The patient is 61-year-old woman who underwent partial left ventriculectomy, (Batista procedure) due to dilated cardiomyopathy and multiple thromboembolism. Although postoperative course was uneventful, she has had clinical symptoms of the left heart failure due to the increased mitral valve regurgitation at the early postoperative period, gradually. Even though mitral valve regurgitation was severe, it was not apt to re-dilatate the left ventricular capacity evaluated by echocardiography. She underwent the mitral valve replacement on the 92nd postoperative day, and was once possible for weaning from cardiopulmonary bypass under the support of IABP. However, she died on the 19th postoperative day caused by sepsis. It is important to evaluate the accurate mitral valve regurgitation preoperatively for Batista procedure. Although there was the mild mitral valve regurgitation, it is essential to repair or replace the mitral valve for Batista procedure.
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PMID:[Progressive mitral regurgitation which is necessitated the mitral valve replacement after partial left ventriculectomy (Batista procedure): a case report]. 1047 46

We undertook a retrospective study of the risk factors determining outcome of nontraumatic patients with shock in the pediatric emergency service. From October 1992 through September 1997, 22 patients with the diagnosis of shock were identified, including 11 with septic shock (50%), 7 with hypovolemic shock (32%) and 4 with cardiogenic shock (18%). Their age ranged from 2 months to 19 years old. Among the cases, 14 patients (64%) had other underlying diseases. Gram-negative bacterial sepsis (6/11, 55%), dilated cardiomyopathy (2/4, 50%) and acute gastroenteritis (7/7, 100%) were the most frequent causes of septic, cardiogenic and hypovolemic shock, respectively. In total, 12 patients (55%) died. The mortality rate was high in septic shock (9/11, 82%) and cardiogenic shock patients (3/4, 75%), but low in hypovolemic shock patients (0/7, 0%). The risk factors of poor outcome in patients with shock included thrombocytopenia, prolonged prothrombin time and partial thromboplastin time. Patients with leukopenia, a higher level of C-reactive protein, or under 2 years of age tended to have poor outcome.
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PMID:Shock in the pediatric emergency service: five years' experience. 1091 May 75

In children with dilated cardiomyopathy the disease may progress so rapidly that they die during the waiting period before a suitable donor organ is found. Fifteen children of 4 months to 15 years of age had been in congestive heart failure with multiorgan failure due to dilated cardiomyopathy, where intensive medical treatment had failed. After resuscitation, a miniaturized pulsatile ventricular assist device for the mechanical replacement of heart function was implanted. The biventricular assist device ("Berlin Heart") consists of two extracorporeal pneumatically driven polyurethane blood pumps, with a multi-layer flexible membrane that separates a blood and an air chamber. Four silicon cannulae connect the blood pumps to the patient. A three-leaflet valve prevents blood reflux. The pumps are driven by a pulsatile electropneumatic system. In 12 of the 15 children the bridging to transplantation was successful with a support time of 1 to 98 days (mean 24 days). Nine of them were extubated and mobilized while assisted. Three children died during the support time due to hemorrhage, sepsis, and pulmonary failure. In addition, there was one infarction of the arteria cerebri media. To date seven of the transplanted children are in good condition on follow-up. The beneficial effects of ventricular assist device use are well known in adult patients and with special devices it can be transferred to infants and children in whom longer need for support is anticipated. Even in small infants it is an effective method for bridging to cardiac transplantation.
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PMID:[Artificial heart in terminal stage of dilated cardiomyopathy in childhood]. 1114 71

Anthracycline cardiotoxicity can induce dilated cardiomyopathy (DCM). Nine patients (four men) experienced postchemotherapy DCM: age at time of tumour diagnosis ranged from 1-45 years (mean 13.5 +/- 19 years); interval time between tumour and HT was 3-23 years (mean 10.8 +/- 6.6) and age at HT ranged from 10-65 years (30.8 +/- 20.1). Interval between end of chemotherapy and beginning of cardiac symptoms was 5.71 +/- 4.6 years. Mean age at DCM diagnosis was 19.2 +/- 19.7 (range 1-50 years). Interval between start of chemotherapy and DCM ranged from 1 month to 10 years (mean 3.15 +/- 3.6 years). Tumours were Ewing sarcoma (7-year-old boy), paratesticular rabdomyosarcoma (1-year-old boy), Wilms tumor with pulmonary metastasis (3-year-old girl), bilateral breast carcinoma (45-year-old woman), uterine leiomyosarcoma (44-year-old woman), acute myelocytic leukemia (1.5-year-old boy and 17-year-old girl), and chronic myelocytic leukemia (5-year-old boy). All patients had high pulmonary resistance values. One patient with chronic myelocytic leukemia (14 year-old at HT) died due to graft failure on the first postoperative day. At follow-up (mean, 80.4 +/- 69.3 months) two patients died: a 32-year-old woman (acute myelocytic leukemia) 1 year after HT for sepsis and a 68-year-old woman who had breast adenocarcinoma recurrence 81 months after HT. The remaining patients are alive, in good condition with no difference in survival from other transplanted patients (P =.757). Patients with end-stage postchemotherapy DCM without evidence of tumour recurrence can safely undergo HT.
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PMID:Heart transplantation in chemotherapeutic dilated cardiomyopathy. 1282 9

Two infants were referred to the emergency ward: a 3.5-month-old male infant in whom sweating and shortness of breath were seen during drinking and crying, and a 5-week-old girl that had drunk less the past day, moaned while breathing for the past few hours and had become increasingly drowsy. They were not suffering from an infection but from a dilated cardiomyopathy based on an anomalous origin of the left coronary artery from the pulmonary artery. The left coronary artery was then implanted in the aorta surgically. Both patients recovered well. Airway tract infection and sepsis are obvious common differential diagnostic considerations when confronted with a tachy-dyspnoeic infant. However, a cardiac cause, such as a dilated cardiomyopathy with cardiac decompensation, must also be considered as the presentation can be similar. The abnormal origin of the left coronary artery from the pulmonary artery is one of the few readily well treatable causes.
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PMID:[Tachypnoea and dyspnoea in two infants due to dilated cardiomyopathy associated with an anomalous origin of the left coronary artery]. 1562 12

The aim of this study was to describe the clinical spectrum of chronic renal failure (CRF) in the elderly. The diagnosis of CRF was made using standard clinical criteria. The elderly was defined as person with over 60 years of age. In total, 200 elderly patients with CRF were evaluated between July 2002 and February 2004. Their age (male: 146; female: 54) ranged between 60 and 90 (mean 64.31+/-4.18) years. Diabetic nephropathy was the most common (46%) cause of CRF. Hypertensive nephrosclerosis, chronic interstitial nephritis and obstructive uropathy were responsible for CRF in 18%, 14% and 13% of patients, respectively. We observed chronic glomerulonephritis in 7% of elderly CRF. Urinary tract infection (55.5%), hypovolemia (22.2%), accelerated hypertension (11.1%) and sepsis (11.1%) were responsible for acute exacerbation of renal failure in 36 (18%) patients. Associated co-morbid conditions were noted in 93 (46.5%) patients. They included; coronary artery disease 46 (49.46%), cerebrovascular disease 20 (21.50%), osteoarthritis 13 (13.97%), chronic obstructive pulmonary disease 6 (6.45%), dilated cardiomyopathy 5 (5.37%), and malignancy in 3 (3.22%) patients. Acute dialytic support was required in 164 (82%) cases and remaining 36 (18%) patients received conservative management. Mortality was noted in 25 (12.5%) cases. The coronary artery disease (48%), acute pulmonary edema (20%) and hyperkalemia (12%) were the main causes of death. Subsequent evaluation revealed that 102 (51%) patients had ESRD of which only 3 (2.94%) patients could afford CAPD. A total of 11 (10.7%) patients underwent chronic maintenance hemodialysis for 3-4 months and then discontinue dialysis mainly because of financial constraints. Remaining 88 (86.27 %) patients with ESRD were discharged from hospital after symptomatic improvement with acute dialysis. Thus, diabetic nephropathy related to type-2 diabetes was the commonest cause of CRF in our elderly patients. Chronic renal failure in elderly was associated with a number of co-morbid conditions, which contributed significantly to morbidity and mortality. Acute on chronic renal failure with severe uremic complications were an important cause of hospitalization. The financial constraint was the major limiting factor for the management of elderly ESRD patients.
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PMID:Clinical spectrum of chronic renal failure in the elderly: a hospital based study from eastern India. 1709 77

The congenital disorders of glycosylation (CDG) are a recently described group of inherited multisystem disorders characterized by defects predominantly of N- and O-glycosylation of proteins. Cardiomyopathy in CDG has previously been described in several subtypes; it is usually associated with high morbidity and mortality and the majority of cases present in the first 2 years of life. This is the first case with presentation in late childhood and the article reviews current literature. An 11-year-old female with a background of learning difficulties presented in cardiac failure secondary to severe dilated cardiomyopathy. Prior to the diagnosis of CDG, her condition deteriorated; she required mechanical support (Excor Berlin Heart) and was listed for cardiac transplant. Investigations included screening for glycosylation disorders, and isoelectric focusing of transferrin revealed an abnormal type 1 pattern. Analysis of phosphomannomutase and phosphomannose isomerase showed normal enzyme activity, excluding PMM2 (CDG Ia) and MPI (CDG Ib). Lipid-linked oligosaccharide and mutational studies have not yet defined the defect. Despite aggressive therapy there were persistent difficulties achieving adequate anticoagulation and she developed multiple life-threatening thrombotic complications. She was removed from the transplant list and died from overwhelming sepsis 5 weeks following admission. This case emphasizes the need to screen all children with an undiagnosed cardiomyopathy for CDG, regardless of age, and where possible to exclude CDG before the use of cardiac bridging devices. It highlights the many practical and ethical challenges that may be encountered where clinical knowledge and experience are still evolving.
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PMID:Cardiomyopathy in the congenital disorders of glycosylation (CDG): a case of late presentation and literature review. 1975 45

Since the introduction of cardiac plasma troponin measurements, a significant number of patients were seen with chest pain, elevated troponin levels but no significant coronary artery disease. Pulmonary embolism, aortic valve disease, myocarditis, sepsis, trauma, arrythmias, stress cardiomyopathy and dilated cardiomyopathy stand among possible causes for this syndrome. In some cases, myocardial strain could be the mechanism underlying this phenomenon, since it is known that the stimulation of stretch-responsive integrins may lead to the release of cardiac troponin I. In the present text, a case is made in favour of classifying this syndrome, of chest pain with increased values for plasma cardiac troponin, with or without ECG changes, in the absence of definite myocardial infarction or coronary artery disease, as pseudo myocardial infarction (PMI). This constitutes a new definition for a concept with decades, formerly centered on clinical and electrocardiographic changes mimicking infarct. The case is based on the search of scientific truth, on avoidance of unnecessary cardiac examinations, on avoidance of unnecessary drug therapy and on avoidance of unnecessary legal liability. PMI should be seen as a working diagnosis, since a more definitive diagnosis can be reached at all time. It should also be seen as a heterogeneous group of patients - several different diseases and conditions can lead to this phenomenon. But it must certainly not be seen as a benign condition, since published studies point in a totally different direction.
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PMID:Pseudo myocardial infarction - a condition in need to be redefined? 1985 81

Prolonged support with left ventricular assist system (LVAS) increases the risk of device-related infection. We experienced a rare complication of LVAS: an infectious aneurysm at the apical cannula, which appeared with atypical presentation. A 27-year-old male, who developed acute aggravation of dilated cardiomyopathy, was placed on extra-corporeal type LVAS. Six months later, the patient suffered from methicillin-resistant Staphylococcus aureus (MRSA) sepsis that lasted for as long as three months despite intensive antibiotic therapy. At 17 months after the implantation, he presented with obstructive ileus. Monthly assessment with transthoracic echocardiography (TTE) did not document any abnormalities around the ventricle. A contrast computed tomographic (CT) scan revealed a huge apical aneurysm protruding into the preperitoneal space. The aneurysm oppressed the transverse colon, resulting in obstructive ileus. Aneurysmectomy was carried out and MRSA was identified from the resected tissue. We reached the precise diagnosis with a CT-scan, although routine assessment with TTE failed to reveal abnormalities. Knowledge of this complication is essential in LVAS management. This is certainly rare, but possibly occurs in all the patients on prolonged LVAS support. Early and accurate diagnosis together with aggressive intervention would bring favorable outcome in such serious cases.
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PMID:Atypical presentation of an apical pseudoaneurysm in a patient on prolonged left ventricular mechanical support. 1993 5


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