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Query: UMLS:C0036690 (sepsis)
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Thirteen patients with a discordant atrioventricular connexion underwent repair of major associated intracardiac defects. Of the patients, 10 had a discordant ventriculoarterial connexion while 3 had double outlet from the morphologically right ventricle. A ventricular septal defect was the most frequently encountered lesion, present alone or in combination with other lesions in all patients. The other major lesions were pulmonary stenosis in 8, Ebstein's malformation of the left atrioventricular valve in 2, and calcific aortic valve disease in one. The operations performed were closure of the ventricular septal defect in 4 patients, closure of the ventricular septal defect with pulmonary valvotomy in 3 patients (one of whom subsequently underwent replacement of the left atrioventricular valve). Modified Fontan's procedure was performed in 6 patients, one of whom also had replacement of the aortic valve. There was no operative death, although there was one early death on the 40th postoperative day due to septicemia. There has been no late death after an average follow-up of 1.2 years. There was one case of surgically induced complete heart block. All other patients are in normal sinus rhythm in New York Heart Association functional class I or II. Elective repair of major intracardiac anomalies in association with a discordant atrioventricular connexion can now be accomplished safely. The modified Fontan's procedure is a viable alternative in certain cases to the placement of an external valved conduit for relief of pulmonary outflow tract obstruction.
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PMID:Surgical treatment of major intracardiac lesions associated with discordant atrioventricular connexion. 240 9

In patients with pulmonary atresia and ventricular septal defect, hypoplasia of the central pulmonary arteries prevents single-stage complete repair. Over an interval of 8 1/2 years, 105 patients underwent establishment of continuity between the right ventricle and a hypoplastic central pulmonary arterial confluence (first stage). There were 12 hospital deaths (11%) and 11 late deaths before second-stage (complete) repair. Twenty-five patients await late evaluation. The remaining 57 individuals have had follow-up cardiac catheterization a mean of 33 months postoperatively. In 31 of these, final repair was deferred because of insufficient pulmonary arterial enlargement (14), restricted peripheral arborization (nine), or both (eight). The final 26 patients were accepted for second-stage repair, which has been performed in 24. Complete repair included ventricular septal defect closure (24), right ventricular outflow tract reconstruction (18), relief of central pulmonary arterial stenosis (14), and ligation of systemic-pulmonary collateral arteries (10). The mean postrepair peak systolic right ventricular-left ventricular pressure ratio was 0.67 (range 0.32 to 1.0). One of these patients (4%) died in the hospital and there was one late death (4%) from sepsis after tricuspid valve replacement. Three patients were lost to follow-up; the remaining 19 patients are in functional Class I or II. A two-stage surgical approach is highly successful in those patients whose pulmonary arteries are too hypoplastic to allow a single-stage repair.
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PMID:Staged surgical repair of pulmonary atresia, ventricular septal defect, and hypoplastic, confluent pulmonary arteries. 242 10

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

In order to investigate the morbidity and mortality in individuals with Down syndrome(DS), we reviewed 237 cases of DS visiting our hospital in a ten-year period. There were 150 males and 87 females with age range from newborn to 25 years. The major illnesses included: 1) congenital heart disease (CHD), 42.6%; with endocardial cushion defect, ventricular septal defect, atrial septal defect and patent ductus arteriosus as the common types; 2) frequent or major respiratory tract infections, 34.2%; 3) summer fever, 5.5%; 4) major infective episodes other than pneumonia, 5.0%. Other diseases such as seizure disorder, gastrointestinal tract anomalies and thyroid dysfunction were also noted. There were 28 deaths of them and the causes of mortality were 1) CHD with congestive heart failure (CHD with CHF) and pneumonia (46.9%); 2) CHD with CHF and sepsis (10.7%); 3) CHD with CHF (14.3%) and 4) acute leukemia with infection (10.7%) respectively. Most of the mortality occurred during the first year of life, especially in patients with CHD. In our series, the one year survival rate was 93.6%; the result indicates that life expectancy in DS is much better than generally believed.
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PMID:Mortality and survival in Down syndrome in Taiwan. 253 73

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

A material of 87 patients who underwent cardiac surgery for active infective endocarditis from 1975 to 1987 is analyzed retrospectively. 91 emergency operations were performed in 19 women and 68 men with a mean age of 48 years. 72 native valves and 19 prosthetic valves were involved. Streptococci (41%) and staphylococci (27%) were the most frequent bacteriological isolates, whereas 19% of the cultures remained negative. Heart failure (52%), embolism (21%), uncontrolled infection (11%), prosthetic valve endocarditis (10%), atrioventricular block (4%) and ventricular septal defect (2%) were the indications for surgery an average of 22 days after diagnosis. 17 patients (19%) died, 9 during hospitalization from heart failure or septicemia and 8 in the later course. 16 patients required reoperation for valvular incompetence (5), paravalvular leak (4) or prosthesis infection (7). Five relapses (5.5%) and two reinfections (2.5%) were treated surgically while two reinfections responded to medical therapy alone. Postoperatively, 34 patients (39%) suffered severe complications such as neurological deficits, prosthetic valve endocarditis or anticoagulant haemorrhage. After a mean observation period of 52 months (range 1-147 months) 64 (91%) of the surviving patients were in NYHA classes I + II and 6 (9%) in NYHA classes III + IV.
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PMID:[Heart valve replacement in active infectious endocarditis]. 279 30

The natural history of hypertrophic obstructive cardiomyopathy (HOCM) is usually characterized by development of mitral insufficiency, congestive heart failure (CHF) and sudden death. In patients (pts) belonging to at least clinical class III (NYHA) after failed medical therapy (beta-blocking agents and calcium-antagonists) surgery should be considered (by means of transaortic subvalvular myectomy). The history and development of different surgical techniques and procedures has been described in detail since 1958, when Cleland performed the first transaortic subvalvular myotomy. Our surgical series (1963-May 31, 1986) consists of 212 pts (mean age 40 years, range 6-73 years) with typical and atypical HOCM. The total hospital mortality rate was 6.6% (n = 14), which was reduced to 3.8% (n = 6), if only transaortic subvalvular myectomy (TSM) was performed (n = 160). In the group of 52 pts with additional surgical procedures the mortality rate was 15.4% (n = 8). The main problems occurred in pts with additional mitral valve replacement (MVR) (n = 15, three deaths). The rate of HOCM-related complications (secondary VSD, total AV-block, cerebral embolism, intraoperative re-myectomy) and those related to surgery (bleeding, pulmonary embolism, wound dehiscence, septicemia) was low. Therefore TSM for HOCM is a low-risk surgical procedure with a good long-term prognosis. However, in pts with a need for additional surgical procedures, the risk is considerably increased. Subjective impression of the pts and hemodynamic data indicate a clear clinical improvement postoperatively. Concerning long-term survival and reduction of the sudden death rate, our data do not allow a final judgement at the moment.
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PMID:Techniques and complications of transaortic subvalvular myectomy in patients with hypertrophic obstructive cardiomyopathy (HOCM). 343 68

A review of 13 autopsy specimens and of 13 patients who were operated on for complete atrioventricular (AV) canal and tetralogy of Fallot between 1975 and 1985 revealed a number of anatomical details that are important in the successful repair of this combined lesion. A bridging anterior leaflet was present in 25 hearts. A septum primum was present and attached to leaflet tissue in 13. In all 26, a ventricular septal communication was present beneath the bridging anterior leaflet and extended anteriorly, but in 14 there was no ventricular septal defect underneath the posterior leaflet. Additional pathological features included the following: leaflet tissue deficiency (4 hearts), single left papillary muscle (3), accessory valve orifice (4), and left ventricular (4) or right ventricular (RV) (1) dominance. All 26 had infundibular stenosis, and 10 had hypoplastic pulmonary annuli. One had pulmonary atresia, and 6 had branch pulmonary artery stenosis. Surgical technique was modified to include incision of the septum primum in 7. Because of rightward displacement of the anterior ventricular septum and also to minimize the risk of causing subaortic stenosis, the bridging anterior leaflet was divided more toward the tricuspid orifice so as to parallel the crest of the ventricular septum. Transannular RV outflow patches were used in 10 patients, and a right ventricle-pulmonary artery conduit was placed in 1 patient. Three required repair of branch pulmonary artery stenosis. There were no hospital deaths. Three patients died late of residual AV valve regurgitation and branch pulmonary artery stenosis (2) and sepsis (1).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Complete atrioventricular canal and tetralogy of Fallot: surgical considerations. 375 74

Corynebacterium diphtheriae usually produces an infection limited to the respiratory tract and the organisms rarely invade the blood stream. We report the case of a 6-year-old girl who, 2 months after an unsuccessful repair of a ventricular septal defect, developed septicaemia with non-toxigenic C. diphtheriae. The organism appeared resistant to penicillin in vitro and failed to respond to a course of trimethoprim-sulfamethoxazole to which it was susceptible in the laboratory. A cure was finally achieved using cephalothin and gentamicin, followed by an additional course of ampicillin and amoxicillin. Twelve previously recorded cases of diphtheritic sepsis and endocarditis are reviewed.
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PMID:Diphtheritic septicaemia and probable endocarditis: a case report and review of the literature. 387 95

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


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