Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Of 1,193 consecutive pediatric (less than 18 years) patients undergoing intracardiac repair from 1975 to 1984, 38 (3.2%) developed surgically induced complete heart block and were treated by permanent pacemaker implantation. Anomalies included complete atrioventricular septal defect = 9 (24%), simple ventricular septal defect = 9 (24%), atrioventricular discordant connection = 8 (212), tetralogy of Fallot = 7 (182), and other complex anomalies = 5 (13%). There were no hospital deaths. follow-up was 100% complete. There were six late deaths = 16%. Actuarial survival was 79 + 9% at 10 years. None of the late deaths were related to disturbance of cardiac rhythm or pacemaker system failure. Twelve patients (32%), required 27 reoperations for various types of pacemaker system failure. Indications for reoperation included: lead failure (44%). Pulse generator failure (44%), and wound sepsis (12%). Actuarial freedom from any pacemaker related reoperation was 50 + 16% at 48 months and 25 + 15% at 96 months. Only first reoperation was found to be an incremental risk factor for subsequent reoperation (p = 0.03). Surgical heart block has been neutralized as a risk factor for hospital death after repair of congenital cardiac defects. The risk of the development of surgical heart block now approaches zero, as indicated by a decreased incidence (1 of 401 = 0.25%) in our institution from 1985 to 1987, as compared to the era 1975 to 1984 (p = 0.001).
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PMID:Pacemaker system failure and other events in children with surgically induced heart block. 245 71

Size limitations and technical barriers prohibit the use of many conventional mechanical circulatory support systems for postcardiotomy ventricular dysfunction in pediatric populations. Extracorporeal membrane oxygenation (ECMO), frequently used to treat neonatal respiratory failure, can provide cardiac support and is effective treatment of postoperative myocardial failure in children. From 1981 to 1987, 10 patients aged 2 days to 5 years were maintained on ECMO for 15 to 144 hours (mean duration, 92 +/- 16 hours) after cardiotomy. Operative procedures included repair of tetralogy of Fallot (2 patients), closure of a ventricular septal defect (2), the Senning procedure for transposition of the great arteries (1 patient), repair of interrupted aortic arch with closure of a ventricular septal defect (1), repair of a partial atrioventricular septal defect (2), closure of a ventricular septal defect with excision of an anomalous muscle bundle (1), and the Fontan procedure (1). Venoarterial ECMO was established in all 10 children. Six patients underwent transthoracic right atrium-ascending aorta cannulation, 3 had right internal jugular vein-right common carotid artery cannulation through a cervical incision, and 1 had right internal jugular vein-left axillary artery cannulation. Eight of the 10 patients were successfully weaned from ECMO, and 7 are long-term survivors. There were 3 deaths; 1 was caused by cardiac and acute renal failure complicated by sepsis two days after decannulation, another occurred 19 days after atrioventricular septal defect repair, and 1 was caused by massive pulmonary hemorrhage. Major hemorrhage developed in 3 patients while on ECMO; 2 required premature decannulation for mediastinal bleeding from operative sites and ultimately survived, and 1 died of respiratory failure as a result of endobronchial bleeding. We conclude that the use of ECMO in pediatric populations for transient postoperative ventricular dysfunction improves survival with limited overall morbidity.
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PMID:Extracorporeal membrane oxygenation for postcardiotomy cardiogenic shock in children. 275 47

Between July 1979 and October 1984, 61 systemic-pulmonary arterial shunts were created in 45 patients. Clinical and angiographic results with 23 classic Blalock-Taussig shunts (BTSs) and 35 modified Blalock-Taussig shunts (MBTSs) with polytetrafluoroethylene grafts between the subclavian and pulmonary arteries were compared. Forty-three of the patients studied were infants: 28 were less than 1 month old, and 19 were less than 1 week old. Weights were 1.1 to 19 kg (mean 2.9 kg). Diagnoses were complex tetralogy of Fallot (18 patients), transposition of the great arteries with small left ventricle or left ventricular outflow tract obstruction (six patients) pulmonary atresia (seven patients) with intact ventricular septum (three) and ventricular septal defect (four), tricuspid atresia (four patients), univentricular heart (six patients), atrioventricular septal defect (canal) with pulmonary stenosis (three patients), and double-outlet right ventricle (one patient). Comparison groups were concurrent and were equivalent for age, weight, and complexity of anomaly. Patients were removed from the study population at the time of subsequent open heart surgery or at death. All patients were followed for a minimum of 6 months and for up to 5 1/2 years (BTS 1 1/2 to 5 1/2 years, mean 33 months; MBTS 6 months to 3 years, mean 20 months). There were no intraoperative deaths in either group. Among the 23 BTSs, three failed at 1, 3, and 19 days, resulting in two deaths (17%). One premature infant died despite a second shunt, one died during attempted intracardiac repair at 13 days of age, and the other was well after revision of the shunt. Eight patients underwent subsequent intracardiac repair 1 to 5 years (mean 34 months) after the initial procedure. Another four patients died from complex intracardiac anomalies with patent shunts. Three patients are alive 2 to 4 years after receiving shunts and have not undergone subsequent surgery. Late postoperative angiograms demonstrate a disturbing incidence (21%) of stenosis and right pulmonary arterial deformity despite satisfactory immediate postoperative studies and good clinical function. Eight patients required a second shunt, two for anatomic discontinuity of the pulmonary arteries and six because of inadequate blood flow through the first shunt. Among the 35 patients receiving the MBTS, two required early revision (technical error and ductal tissue at the anastomosis). There were no shunt-related deaths, but three (6%) occurred within 30 days of operation from left ventricular infarct, cerebral hemorrhage, sepsis, and severe aortic stenosis with arrhythmias.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Classic versus modified Blalock-Taussig shunts in neonates and infants. 402 66

The spectrum of patients operated on without preoperative catheterization and angiography, the accuracy of echocardiographic diagnosis and its impact on the results of surgical treatment were prospectively assessed in 2,788 children consecutively operated for congenital heart defects (CHD) between 1986 and 1992. The overall percentage of surgery based solely on noninvasive preoperative examination increased from 63% in 1986 to 81% in 1990 and decreased to 72% in 1992. There were no differences in the preoperative diagnostic approach between groups of newborn, infants and children. A high percentage of patients with patent ductus arteriosus (96.5%), atrial septal defect (94%), incomplete atrioventricular septal defect (88.6%), ventricular septal defect (86.3%), coarctation of the aorta (80.2%) and total anomalous pulmonary venous connection (79.3%) was referred for surgery without prior invasive examination, while a lower percentage was found in univentricular heart (48.4%), pulmonary atresia (34.6%) and double outlet right ventricle (27.7%). More patients with pulmonary and tricuspid atresia were catheterized before complete repair compared to those who underwent palliative surgery (p < 0.01 and p < 0.0001, respectively). The echocardiographic diagnosis was correct in 96% of patients. Two patients of those with incomplete preoperative diagnosis died early postoperatively, both with missed apical ventricular septal defect. One with tetralogy of Fallot died after reoperation, the other with persistent truncus arteriosus due to sepsis. When the echocardiographic findings are in full agreement with the clinical status, physical examination, ECG and chest X-ray, we recommend cardiac surgery without prior catheterization in many patients with CHD.
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PMID:Seven-year experience of noninvasive preoperative diagnostics in children with congenital heart defects: comprehensive analysis of 2,788 consecutive patients. 758 60

Aortic root infection remains a challenging problem in the surgical treatment of both native and prosthetic valve endocarditis. Between 1980 and 1991, 73 patients with active aortic valve endocarditis and paravalvular infection underwent operation. Indications for operation included congestive heart failure and uncontrolled sepsis. Aortic root abscesses were located in the noncoronary anulus or in the aorticomitral junction in 45% of cases, followed by the subannular interventricular septum in 23%. Two patients had an aorticoatrial fistula, seven an interventricular septal defect. Total or partial left ventricular-aortic dehiscence was observed in 27 patients. All patients underwent aortic valve replacement, nine with simultaneous mitral valve operations. Two of the latter required patch reconstruction of the destroyed aorticomitral septum with double valve replacement. Reconstruction of the aortic base was possible in 16 patients, whereas in 12 total replacement of the aortic root was necessary. In one patient, supracoronary aortic valve replacement was used. Recently, topical application of antibiotics in fibrin sealant was used in 25 patients. The operative mortality rate was 21% and correlated to preoperative uncontrolled sepsis and the presence of extensive root destruction. Operation for active endocarditis of the aortic root requires radical, individualized techniques and results in an acceptable operative and long-term risk. The use of an antibiotic fibrin compound appears to be a useful prophylactic tool to prevent postoperative residual endocarditis.
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PMID:Surgical treatment of active infective endocarditis with paravalvular involvement. 828 80

We treated 40 children, aged between 15 d and 17 y, diagnosed with acute respiratory distress syndrome and/or pulmonary hypertension, with inhaled nitric oxide. The most frequent underlying diagnosis associated with ARDS were bronchopneumonia (eight), cardiac surgery (five), and sepsis (three). Pulmonary hypertension was secondary to cardiomyopathy in 2 patients and occurred in the postoperative period of cardiac surgery in 17 patients--the most frequent were ventricular septal defect (5), transposition of great arteries (4), and atrioventricular septal defect (3). In 11 patients, sudden discontinuation of nitric oxide induced a decrease in oxygenation associated in some of the patients with an increase in pulmonary artery pressure. In two patients discontinuation of nitric oxide induced severe pulmonary hypertension, extreme bradycardia and hypoxaemia, which required cardiopulmonary resuscitation. When exogenous nitric oxide is abruptly interrupted, hypoxaemia and pulmonary hypertension are found in some patients, due to a decrease in the nitric oxide concentration in the pulmonary circulation. This may be caused by the exogenous nitric oxide administration that may have inhibited endogenous production. We recommend making a progressive withdrawal of inhaled nitric oxide to avoid the side effects observed in the sudden discontinuation.
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PMID:Life-threatening effects of discontinuing inhaled nitric oxide in children. 947 12

We report the first case of a girl born to a diabetic mother who was found to have Down syndrome and prune-belly anomalies (bilateral gross hydronephrosis, megaureter, and megacystis with abdominal muscle deficiency). The girl also had an atrioventricular septal defect. Diagnoses were confirmed with a cytogenetic study and micturating cystourethrography. She died at 29 days of age with a sudden collapse, most likely due to sepsis.
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PMID:Prune-belly anomalies in a girl with Down syndrome. 1368 Mar 26

To define risk and outcome of surgery in adults with congenital valve disease (CVD), experience between 2002 and 2005 with 371 CVD operations (288 males, aged 56 +/- 9 years) was compared with 2102 for acquired valve disease (AVD) (69 +/- 22 years, p = 0.02). Diagnosis included: bicuspid aortic valve (BAV), 337, s/p ToF repair, 11; atrio-ventricular valve dysfunction, 10; other, 13. Associated lesions were present in 259 patients (70% vs. 17%, p = 0.001): ascending aorta, 205; right ventricular outflow tract obstruction, 40; coronary artery, 34; mitral/tricuspid valve, 27; septal defect, 17; subaortic stenosis, 4; aortic arch, 4; other, 3. Fifty-two patients (14% vs. 2.5% AVD, p = 0.001) had undergone 75 prior operations (1.4/patient) and 14 (3.8% vs. 1.9% AVD, p = 0.04) required urgent/emergent surgery (endocarditis, dissection). Valve repair was done in 36 (10% vs. 3% AVD, p = 0.02) and replacement in 335: stentless solution (native, autograft, xenograft) was offered to 101 (29%) patients. In BAV, partial root replacement was associated in 63, complete in 77 and ascending aorta in 92. Three (0.8%) hospital deaths occurred (vs. 1.9% AVD, p = 0.2) due to endocarditis. Twenty-six patients (7.0% vs. 10.8% AVD, p = 0.003) experienced complications (cardiac, 7; neurologic, 6; respiratory, 5; renal, 3; sepsis/multiple organ failure (MOF), 2; hemorrhage, 8). Urgent/emergent surgery predicted hospital mortality (p = 0.001). During 5-year follow-up (average 2.6 +/- 1.8 years), there was one late cardiac death and three reoperations (98% free). Despite higher prevalence of associated procedures, reoperation and emergent indication, operative risk in CVD is lower than in AVD, possibly because of younger age. Stentless valve surgery, allowing normal life-style (e.g., exercise, pregnancy), is increasingly preferred.
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PMID:Operative risk and outcome of surgery in adults with congenital valve disease. 1881 32

Coxa vara is an abnormality of the proximal femur with a decreased neck-shaft angle and resulting leg-length discrepancy, has been associated with conditions such as congenital coxa vara, traumatic injury, sepsis, rickets, vascular damage, or metabolic disorders; however its possible relationship with extracorporeal membrane oxygenation has been recently reported.A full term girl was born with a total infradiaphragmatic anomalous pulmonary venous drainage, at the age of 12 days, an anastomosis of the pulmonary venous trunk with the left auricle and closure of the auricular septal defect was performed; during this procedure extracorporeal membrane oxygenation was used during 104 minutes, no neonatal sepsis was developed. She had no orthopedic issues until she was 3 years and 10 months old, when she presented with limp related to the right lower limb, with no pain. She had a leg-length discrepancy of 2 cm (right - left), limited right hip abduction to 25 degrees , and internal rotation to 5 degrees , also had a positive Trendelenburg test. No flexion/extension abnormalities. Anteroposterior radiographs and magnetic resonance revealed coxa vara with proximal femoral growth arrest. A valgus osteotomy with greater trochanteric epiphysiodesis was performed. At the eight month follow-up, she had no hip pain, better hip range of motion, no difficulties with recreational activities and the osteotomy healed.Another four similar cases had been reported with similar outcome, we think that it will be recommendable to take images in patients with this background and limb leg-length discrepancy or abnormal range of motion.
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PMID:Coxa vara with proximal femoral growth arrest as a possible consequence of extracorporeal membrane oxygenation: a case report. 1991 57