Mortality among women with ductal carcinoma in situ of the breast in the population-based surveillance, epidemiology and end results program

BACKGROUND: Over 14% of breast cancers diagnosed in the United States annually are ductal carcinomas in situ (DCIS). There are no published population-based reports of the likelihood of breast cancer death among US women with DCIS. METHODS: We used data from the Surveillance, Epidemiology and End Results program to determine the likelihood of breast cancer death at 5 and 10 years among US women aged 40 and older diagnosed with DCIS from 1978 to 1983 (before screening mammography was common; n = 1525) and from 1984 to 1989 (when screening mammography became common; n = 5547). We also calculated standardized mortality ratios (SMRs) to compare observed deaths from breast cancer, cardiovascular disease, and all causes combined among women with DCIS with deaths expected based on general population mortality rates. RESULTS: Among women diagnosed with DCIS from 1978 to 1983, 1.5% died of breast cancer within 5 years and 3.4% within 10 years. Among women diagnosed from 1984 to 1989, 0.7% died of breast cancer within 5 years and 1.9% within 10 years. Relative to the general population, risk of breast cancer death was greater for women diagnosed from 1978 to 1983 (SMR, 3.4; 95% confidence interval [CI], 2.5-4.5) than for women diagnosed from 1984 to 1989 (10-year SMR, 1.9; 95% CI, 1.5-2.3). Women diagnosed from 1984 to 1989 were significantly less likely than women in the general population to have died of cardiovascular diseases (10-year SMR, 0.6; 95% CI, 0.5-0.7) or of all causes combined (SMR, 0.8; 95% CI, 0.7-0.8). CONCLUSIONS: Among women diagnosed with DCIS, risk of death from breast cancer was low, at least within the 10 years following diagnosis. This may reflect the effectiveness of treatment for DCIS, the "benign" nature of DCIS, or both. At 10 years, women diagnosed from 1984 to 1989 were less likely than women diagnosed from 1978 to 1983 to have died of breast cancer, and their risk of dying of all causes combined was lower than that in the general population.     

Prevalence des cardiopathies infantiles symptomatiques au Centre Hospitalier Régional de Louga,Senegal

The management of congenital or acquired infantile heart diseases in sub-Saharan African countries still presents problems, particularly with diagnosis and access to surgical treatment. Our objectives were to describe the heart diseases observed in the paediatric setting of the Louga Regional Hospital (LRH) and report their short-term evolution.In the study period from 1 July 2009 to 31 December 2012, 82 children out of 18 815 presented with heart disease, which was a prevalence of 4.3/1 000. There was a female predominance, with a ratio of 1.2. The most frequent presenting conditions were dyspnoea at 47.5%, followed by heart murmurs at 35.3%, and congestive heart failure at 13.4%. Congenital heart diseases were the most frequent, representing 69.5% of the cases, followed by acquired heart diseases at 29.3%, and mixed-type cases at 1.2%. The most frequently encountered congenital heart diseases were ventricular septal defect (24.4%), followed by atrioventricular septal defect (12.2%), tetralogy of Fallot (9.8%) and patent ductus arteriosus (7.3%). Acquired heart disease was represented by rheumatic heart disease, found in 25.6% of the cases, and tuberculous pericarditis in 3.7%. The mortality rate was high, with 20 children dying (24.4%) during the study period. Only 13 out of 82 patients (15.9%) were operable and surgery was carried out in France, courtesy of the association Humanitarian Mécénat Chirurgie Cardiaque.Infantile heart diseases were therefore not very frequent in the paediatric unit of Louga Regional Hospital. However, congenital heart disease was more frequent than acquired heart disease, with a high mortality rate. Access to surgery remains limited.     

Extracardiac malformations associated with congenital heart disease

The incidence of significant extracardiac malformations was determined in a combined clinical and autopsy study comprising 1000 infants and children with congenital heart disease treated and lost at the Children's Hospital in Helsinki. There were 567 boys and 433 girls. 1/4 of the children had a birthweight of 2500 g or less. 850 children were under 1 yr old. Death occurred during the 1st mth of life in 546 cases. Extracardiac malformations were encountered in 439 children. They were more common in girls than in boys. The incidence of associated malformations was comparatively high in infants with a low birthweight. The noncardiac anomalies were considered main causes of death in 1/3 of the cases. Extracardiac organs were involved in the following order of frequency: alimentary, skeletal, urogenital, central nervous and respiratory system. Of the main cardiac malformations, septal defects were associated with the highest and transposition of the great arteries with the lowest incidence of extracardiac anomalies. An accumulation of some defined noncardiac malformations was observed in patients with certain heart lesions.     

The application of a simplified system of criterions for audit to improve paediatric cardiologic and paediatric cardiac surgical care: evidence from a regional approach in Hungary

AIMS: To evaluate the quality of cardiac and surgical care provided for children with congenital cardiac malformations in the eastern county of Hungary. METHODS: We used the method of clinical audit based in selection of criterions, developing five such criterions concerning timely diagnosis, access to treatment, and outcome. To examine compliance with these criterions, we analysed retrospectively the routine data relating to children living in Hajdu-Bihar County. The period of observation lasted from January, 1994, until December, 2001, and was divided into two epochs in order to evaluate any changes over time. RESULTS: In the first epoch, 28 infants, representing 0.1% of all newborns, died of congenital cardiac disease, with one of the malformations being recognised post mortem. In the second epoch, 21 infants died, representing 0.09% of newborns. In this group, each malformation was diagnosed before death. In each period, 6 infants died without having undergone cardiac surgery, and having no other non-cardiac disease. The overall postoperative mortality was 9.2% for the first period, and 4.6% for the second. The number of patients waiting for non-urgent repair had reduced significantly by the end of the second epoch. CONCLUSIONS: The results show that the timely diagnosis of congenital cardiac malformations is in line with accepted international standards. Conversely, the access to invasive treatment was limited over the period of observation, albeit that the waiting list was reduced significantly by the end of the second epoch. The postoperative mortality for those suffering congenital cardiac malformations is also comparable with international standards, except for mortality during infancy for treatment of complex anomalies. Our audit highlights the need for surgical repair of the more complex malformations during infancy.     

Multislice CT scan in congenital heart disease

The management of congenital cardiovascular malformations is a diagnostic challenge. It requires accurate assessment of the intra- and extra-cardiac malformations. Three-dimensional imaging by the multislice CT scanner is now a routine investigation for congenital heart disease, complementary to echocardiography, and, increasingly, an alternative to conventional angiography. Three dimensional imaging is particularly useful in the diagnosis of complex congenital heart disease, in the preparation of complex investigations of interventional cardiac catheterisation and the postoperative evaluation of surgical repair. This report describes the different acquisition protocols adapted for children and illustrates the present role of volumic CT scanner in congenital heart disease by clinical examples of everyday clinical practice.     

Three-dimensional CT scanning: a new diagnostic modality in congenital heart disease

New generation multislice CT technology has changed the approach to non-invasive assessment of congenital heart disease, in both paediatric and adult patients. This is mainly because of rapid advances in spatial and temporal resolution and in post-processing capability. At H?pital Necker-Enfants Malades, CT with multiplanar and three-dimensional reconstruction has become a routine examination in the evaluation of congenital heart disease planning surgery, complex interventional catheterisations and for follow-up. It has proved to be an invaluable diagnostic and decision-aiding methodology in these situations, as a complement to echocardiography and, increasingly, as a substitute for diagnostic angiography (which is usually associated with higher-dose radiation and longer sedation times, as well as occasional morbidity). This review illustrates the current status of 64-slice CT in congenital heart diseases, including assessment of the aorta, the coronary arteries, the pulmonary arteries, the systemic and pulmonary veins, and other intra- and extracardiac malformations.     

Trends in cardiovascular surgery (1961 to 1977): review of the New York City and State experience.

Statistical data on surgery for congenital and acquired heart disease in New York City in the 17 years from 1961 through 1977 and in New York State in 1977 document trends in volume and types of operations. The ratio of open heart surgery for congenital versus acquired heart disease of 2:1 in 1961 was reversed to 1:6 by 1977 as the number of procedures for valvular and coronary heart disease increased. The case load for surgery for congenital heart disease using the open heart technique gradually increased until 1974 and then decreased. The number of closed heart operations was relatively constant. Observations suggest a finite number of open and closed heart operations for congenital cardiac malformations; the total of 1,381 cardiac operations in New York State in 1977 may continue to diminish if the decreasing birth rate persists.The number of operations for valvular heart disease has stabilized, but the number of operations for coronary artery disease has increased 7-fold since 1970. In 1977, coronary arterial bypass grafting in New York State accounted for 63 percent of operations for acquired heart disease (6,407) and the mortality rate was less than 3 percent.     

电子束计算机断层摄影术在小儿复杂先天性心脏病诊断中的应用

目的 :评价电子束计算机断层摄影术 (EBCT)在小儿复杂先天性心脏病 (先心病 )诊断中的价值。　　方法 :对 2 6例患者经胸常规超声心动图 (TTE)诊断为复杂先心病 ,同时做 EBCT增强单层容积扫描 ,并由计算机工作站进行图像三维重建。其中 9例又做了常规心血管造影 ,11例经外科手术 ,进行对照研究。　　结果 :全组 11种复杂先心病共 6 1处畸形 ,其中心内畸形 2 1处 ,EBCT与 TTE均作出正确诊断。然而 ,心外大血管畸形及心室—大血管连接异常诊断中两者有明显差别 ,在 40处畸形中 ,EBCT过多诊断 1处 (动脉导管未闭 ) ,手术证实为假阳性 ,EBCT诊断符合率为 97.5 % (39/ 40 ) ;TTE诊断 35处 (误诊 1处 ,漏诊 4处 ) ,TTE诊断符合率 87.5 % (35 / 40 )。EBCT与TTE诊断的正确率有显著差异 (χ2 =3.936 ,P<0 .0 5 )。TTE与 EBCT相结合使诊断的总准确率提高到 98.4% (6 0 / 6 1)。　　结论 :EBCT对复杂先心病中某些畸形的检出优于 TTE。EBCT血管造影与 TTE及心血管造影相结合 ,可提高对复杂先心病诊断的准确率 ,以指导手术。     

Spectrum of congenital heart defects and extracardiac malformations associated with chromosomal abnormalities: results of a seven year necropsy study.

OBJECTIVE: To analyse the spectrum of congenital heart malformations, the frequency of extracardiac malformations, and the proportion of chromosome aberrations among fetuses sent for necropsy. MATERIAL: Necropsies were performed on 815 fetuses-448 induced abortions (55%), 220 spontaneous abortions (27%), and 147 stillbirths (18%)-during a seven year period (1991-97) in the department of pathology of the Charité Medical Centre in Berlin. A congenital heart defect was identified in 129 cases (16%). For all 129 fetuses, karyotyping and an ultrasound examination had been performed. RESULTS: Congenital heart defects were present in 22% of induced abortions (99 cases), 9% of spontaneous abortions (20 cases), and 7% of stillbirths (10 cases). The heart malformations were classified into 13 categories. A fetus with more than one defect was included only in the category of the most serious defect. The malformations in order of frequency were: ventricular septal defect (VSD) (28%), atrioventricular septal defect (AVSD) (16%), hypoplastic left heart (HLH) (16%), double outlet right ventricle (DORV) (12%), coarctation of the aorta (CoA) (6%), transposition of the great arteries (TGA) (4%), aortic valve stenosis (AoVS) (4%), tetralogy of Fallot (TOF) (3%), truncus arteriosus communis (TAC) (3%), pulmonary valve stenosis/pulmonary valve atresia (PaVS/PaVA) (3%), tricuspid atresia (TA) (3%), single ventricle (SV) (1.5%), and atrial septal defect (ASD) (0.5%). The most common congenital heart defects were VSD, AVSD, HLH, and DORV, which made up 72% of all the cases. In 11 cases the heart defect was isolated (no other cardiovascular or extracardiac malformations present), 85 cases (66%) were associated with additional cardiac malformations, 85 cases (66%) were associated with extracardiac malformations, and chromosome anomalies were detected in 43 cases (33%). CONCLUSIONS: Fetal congenital heart malformations are common. These defects are often associated with other cardiovascular and extracardiac malformations, as well as with chromosome anomalies. Complex heart defects such as AVSD, HLH, and DORV are frequent in fetuses, as they often lead to spontaneous abortion or stillbirth or, after prenatal diagnosis, to deliberate termination of pregnancy.     

Congenital cardiopathies in Arab children

661 consecutive children below the age of 14 years underwent open and closed heart surgery at the Saudi Heart Centre during a 4-year period for congenital cardiopathies of simple and complex nature with an average mortality rate of 10.5%. Congenital cardiopathies in Saudi Arabia differ from those seen in the Western world by the relative frequency of cyanotic heart disease, the presence of multiple complex congenital anomalies together, the advanced pulmonary hypertension, a rather aggressive nature of the cardiac diseases, and late referral of the children who often suffer also from congestive heart failure, reduced general condition and a malnutritional state.     

纤维支气管镜在先天性心脏病患儿围手术期中的临床应用

目的:探讨柔韧纤维支气管镜(FFB)在先天性心脏病(CHD)患儿术前、术中、术后(即围手术期)的临床应用。方法:总结采用FFB对2009年11月~2012年6月期间235例CHD患儿进行的诊断和治疗。结果:FFB对CHD患儿围手术期的肺不张、肺部感染和肺出血的治疗具有明显效果,还可以作为CHD并发气道结构异常病变的辅助诊断手段。所有病例均无与FFB相关的死亡、大出血、气胸和心律失常等严重并发症发生,部分患儿有一过性氧饱和度下降。结论:FFB检查是小儿气道病变诊断的金标准,能够较安全地用于CHD患儿术前、术中、术后的诊断与治疗。     

胎儿右主动脉弓畸形合并多脏器畸形尸解病理分析

目的:通过研究胎儿右主动脉弓畸形的血管走行及其合并多脏器畸形,并进行文献复习,提高对此畸形的认识。方法:在我院近10年胎儿尸解1 400余例中,发现4例右主动脉弓发育畸形,对其进行详细解剖和观察,同时复习文献。结果:4例右主动脉弓病例中,男性2例,女性2例;I型2例,Ⅱ型2例,4例均有复杂先天性心脏病,3例合并心外多脏器畸形。文献所见,右主动脉弓存活者不少,有症状者多系血管环压迫所致。结论:右主动脉弓并不罕见,可为单发畸形,亦常合并多种心脏及或心外畸形,其非致死性畸形,伴血管环者可通过手术矫正。B超、MIR、CT等可在孕中期发现此畸形。染色体检查亦多有异常。加深对其认识具有重要的临床实践意义。     

Declining mortality from congenital heart disease related to innovations in diagnosis and treatment: a population-based study.

The majority of live births with severe congenital cardiac malformations that do not undergo intervention of some form die prematurely. The population of Malta provides an ideal closed cohort from which to monitor trends. We have observed a significant declining specific mortality from congenital heart disease, and circumstantial evidence relates this decline to the introduction of new techniques used in the diagnosis and treatment of these malformations.     

Results of surgical treatment for infective endocarditis in children

OBJECTIVE: Infective endocarditis is uncommon condition, with a high degree of morbidity and mortality. It is less common in children, albeit tending to be associated with congenital cardiac malformations. We describe our experience of the need for surgical treatment in children with infective endocarditis. PATIENTS AND METHODS: We analyzed retrospectively the records of 9 children aged below 16 years seen between May 2003 and March 2005 with infective endocarditis, reviewing the demographic details, clinical presentation, microbiological and echocardiographic data, operative findings, and outcome. RESULTS: Apart from pre-existing renal insufficiency in 1 patient, congenital cardiac malformations were the predisposing factors. Blood cultures were positive in 3, but remained negative in the other 6 patients. The indications for surgical treatment included uncontrolled sepsis, congestive heart failure, recurrent endocarditis, patch or graft dehiscence, and pseudoaneursymal formation. Death due to uncontrolled sepsis resulting in multiorgan failure occurred in 1 patient, who had tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. Another patient died late postoperatively due to cardiac failure after relapse of the endocarditis in the setting of negative blood cultures. CONCLUSION: Despite advances in antimicrobial therapy, diagnosis, and measures of treatment for infective endocarditis, complications continue to be responsible for substantial morbidity and mortality. Since blood cultures are frequently negative, clinical and echocardiographic findings should be the major determinants of strategies used for treatment. We believe that our small series of patients seen over the past two years in which surgical treatment was performed will be helpful in guiding the clinical perspectives for children with infective endocarditis.     

Outcome of prenatally detected cardiac malformations.

Reliable, prenatal detection of congenital heart disease has become possible over the past decade with the evolution of fetal echocardiography. We have documented the outcome of 170 cardiac defects diagnosed prenatally since 1984. Of 170 cases, 55 (32%) had major extracardiac malformations and 45 (28%) chromosomal abnormalities (16 had both). Elective termination was chosen in 77 (45%) pregnancies. Of 93 continuing pregnancies 15 were stillborn and 43 died postnatally (48% of these fetuses and infants had extracardiac or chromosomal anomalies, or both). Thirty-five patients survive at 1 to 80 months (mean 36). Aneuploidy or extracardiac defects are present in 20% of survivors. Nonimmune hydrops secondary to cardiac failure was present in 7 continuing pregnancies and none of these patients survived. The prognosis of prenatally diagnosed cardiac lesions is negatively influenced by the presence of cardiac failure, aneuploidy or extracardiac malformations, or a combination of these. Optimal counseling and management requires the identification of these conditions when present.     

Contrast-enhanced MR angiography of thoracic vascular malformations in children

To plan an effective management of thoracic vascular malformations, clinicians must have a clear understanding of the anatomy. Although echocardiography and angiography are the leading imaging modalities in patients with congenital cardiovascular anomalies, magnetic resonance (MR) imaging and computed tomography (CT) are valuable noninvasive adjuncts. MR imaging and CT are effective in demonstrating the complex extracardiac morphology and yield helpful information that can change the treatment plan. Although recent reports state the usefulness of multidetector CT (MDCT), in terms of pediatric population, the significance of radiation exposure should be taken into account. Thus, contrast enhanced MR angiography, as a guide in planning surgery, seems to be the best alternative to conventional angiography in the diagnosis of congenital vascular malformations. In this review, the diagnostic features of thoracic vascular malformations in pediatric population are discussed, and, the potential uses of contrast-enhanced MR angiography are emphasized with the retrospective evaluation of imaging findings in 114 examinations.     

Comparison of echocardiography and 320-row multidetector computed tomography for the diagnosis of congenital heart disease in children

IntroductionEchocardiography (echo) is the primary non-invasive imaging modality for the assessment of congenital heart disease (CHD). Computed tomography angiography (CTA) also has potential to examine the anatomy of complex heart anomalies as well as extracardiac involvement.ObjectivesThe aim of this study is to determine the impact of new CTA technology in the diagnosis of CHD and to compare echo and CTA in terms of diagnostic accuracy.MethodsForty-five patients who underwent preoperative echo and CTA assessment in the intensive care unit were included in this study. The results were assessed for three main types of CHD (cardiac malformations, cardiac-major vessel connections and major vessels). The main groups were also divided into subgroups according to surgical features in order to assess them more objectively. Imaging methods were compared for diagnostic accuracy, sensitivity and specificity, while surgical findings were accepted as the gold standard.ResultsPatients’ median age and weight were two months (three days-eight years) and 12 kg (2.5-60 kg), respectively. In 45 operated cases, 205 subgroup malformations were assessed. Diagnostic accuracy was significantly greater in echo (echo vs. CTA: 98.4% and 96.2% [chi-square=6.4, p=0.011]). During surgery, 84 cardiac malformations (echo vs. CTA: 97.4% and 95.1% [chi-square=4.9, p=0.03]), 47 cardiac-major vessel connections (echo vs. CTA: 98.3% and 95.4% [chi-square=7.5, p=0.03]), and 74 major vessel malformations (echo vs. CTA: 96% and 98% [chi-square=1.8, p=0.48]) were confirmed.ConclusionEchocardiography and CTA are imaging methods with high diagnostic accuracy in children with CHD. The use of echocardiography together with CTA, especially for the visualization of extracardiac anatomy, provides additional information for clinicians.     

Preoperative Evaluation of Adult Congenital Heart Disease Patients for Non-cardiac Surgery

Purpose of Review

Highlight the extracardiac comorbidities that adult congenital heart disease patients and summarize the current recommendations for non-cardiac surgery.

Recent Findings

Adult congenital heart disease patients are living longer, becoming more complex and developing adult comorbidities as they age. These patients have multiorgan involvement including higher prevalence of kidney disease and decreased lung function. Non-cardiac comorbidities can complicate surgery in this patient population. Most patients have non-cardiac surgery in community settings. These settings may increase adverse events during and in the post-operative period. Survival is improved when moderate and complex patients are seen in regional referral centers.

Summary

Improved awareness of long-term complications of congenital heart disease and extracardiac comorbidities for adult congenital heart disease patients is needed. Appropriate care settings with both congenital heart disease expertise and adult subspecialty care reduces morbidity and mortality in these complex patients.

     

Results of surgery for coarctation of the aorta in children under one year of age

The aim of this study was to determine the surgical results of repair of coarcta tion of the aorta in children under 1 year of age and to assess the risk of recoarctation. Between 1984 and 2004, 206 children under 1 were operated for coarctation of the aorta. Three groups of patients were identified: Group I (isolated coarctation, N=99), Group II (associated ventricular septal defect, N=63), and Group III (complex congenital heart disease, N=44). The transverse aorta was hypoplastic in 51% of cases. Uni- and multivariate analysis of the risk factors for mortality and recoarctation was performed. An extended resection anastomosis was performed in 206 patients. Reconstruction of the transverse aorta was performed in 32 cases. The hospital mortality was 3.9% (N=8). The presence of a complex intracardiac anomaly was a risk factor for mortality on multivariate analysis (p=0.023). In univariate analysis, a two-step management of patients in Group III was a significant risk factor for mortality (p=0.036). Thirty patients (14.6%) had recoarctation (gradient > 20 mmHg, follow-up 40 +/- 44 months). On multivariate analysis the severity of the immediate postoperative gradient was the only risk factor for recoarctation. The authors conclude that surgery for coarctation of the aorta is associated with excellent results in children under 1 year of age. The prognosis is related to the presence of associated severe cardiac malformations. Survival seems to be better if treatment is carried out in a single stage in this group. The risk of recoarctation is related to the immediate postoperative transisthmic gradient which justifies extensive repair of hypoplastic aortic arches.     

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??Abstract??Congenital heart disease is common among the newborns??and the incidence rate is about 0.8%.Over the past 30 years??great progress has been made in the diagnosis and treatment of congenital heart disease in children.As a result??many children with such diseases now survive to adulthood.In the United States alone??the population of adults with congenital heart disease??either surgically corrected or uncorrected??is estimated to be increasing at a rate of about 5 percent every year??in 2008 there had be almost 1 million such patients.Currently in China??although there is no statistical data of incidence of adult congenital heart disease??the number of such patients must be huge.This article discusses congenital heart disease from two aspects??acyanotic and cyanotic??and clinical countermeasures against each type will be described.