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Hematologic neoplasms that were previously considered fatal are now potentially curable with techniques such as bone marrow transplantation. Such therapies also carry significant morbidity and mortality. With the increasing application of these therapies, a growing number of physicians are using medical decision making regarding critical care for these patients. The process by which ethical decisions are reached for these critically ill patients may be baffling because of several factors: rapidly evolving treatments, uncertain probabilities of the cure of the malignant disorder, the relatively young age of many of these patients, and the poor prognosis with critical illness. I discuss a process to reach acceptable decisions, providing a case example of the application of the process. This process is derived from the ethical principles that drive decision making in general medicine and attempts to maximize patients' autonomy. It involves a consideration of accurate information regarding the disease process and the prognosis, a clear delineation of the goals of the medical care, and communication with patients. Appropriate, ethical, and consistent decisions regarding the critical care of patients with hematologic malignancy can be reached when these considerations are addressed.

With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. They have distinctive forms of heart failure and their cardiac disease can be associated with pulmonary hypertension, thromboemboli, complex arrhythmias and sudden death. Medical aspects that need to be considered relate to the long-term and multisystemic effects of single ventricle physiology, cyanosis, systemic right ventricles, complex intracardiac baffles and failing subpulmonary right ventricles. Since the 2001 Canadian Cardiovascular Society Consensus Conference report on the management of adults with CHD, there have been significant advances in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. Part III of the guidelines includes recommendations for the care of patients with complete transposition of the great arteries, congenitally corrected transposition of the great arteries, Fontan operations and single ventricles, Eisenmenger’s syndrome, and cyanotic heart disease. Topics addressed include genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy risk and follow-up requirements. The complete document consists of four manuscripts, which are published online in the present issue of The Canadian Journal of Cardiology. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.

To test the hypothesis that fecal magnesium excretion is related to fecal volume, fecal specimens were collected from healthy persons who drank 32 single doses of poorly absorbed carbohydrate (mannitol, lactulose or raffinose): the concentration of Mg++ (Y) in fecal water (X) was linearly and inversely related to fecal volume—that is, Y = 42−0.03 X. In contrast, after drinking magnesium sulfate, the concentration of Mg++ in fecal water rose and, except after the lowest ingested dose of 10 mmol, the points relating Mg++ concentration and fecal volume were outside the upper 95% confidence limit of the linear regression line. These findings could be useful when physicians are analyzing stool specimens for supportive evidence of magnesium misuse in baffling cases of diarrhea.

Hemianomalous pulmonary venous drainage with intact atrial septum is a rare congenital anomaly and reports of its surgical repair and the long-term complications related to the correction are only infrequently encountered in the literature. We report the case of a patient with hemianomalous pulmonary venous drainage and intact atrial septum who underwent surgical repair using a pericardial baffle and creation of an “atrial septal defect” aged 15 years. Dyspnoea and recurrent chest infections started 7 months after surgery when he was seen by a respiratory physician without cardiac followup. He presented again aged 28 years with a recurrent pneumonia investigated over 6 weeks and heart pronounced normal from examination and echocardiography. Correct diagnosis was made in Grown Up Congenital Heart (GUCH) clinic stimulating review of data and catheterisation with pulmonary artery angiography which confirmed it. We feel that this case highlights the importance of specialist care and followup for GUCH patients.

A growing number of agents are known to perturb one or more of the interconnected processes of the central nervous system. At the same time, there is an increase in the incidence of neurobehavioral disorders that are confronting clinicians with baffling symptoms and presentations that seem uncommon. Fundamental to the assessment of the environmental-relatedness of the syndromes is a work and exposure history, including information different from that routinely obtained in the clinical setting. Exposure examples are described to suggest the scope of inquiry necessary to differentiate neurotoxic syndromes from nonneurotoxic illness.

Objective—Obstruction of the venous pathways after Mustard repair for transposition of the great arteries is associated with an increased risk of arrhythmia and sudden death. The purpose of this study was to assess the effectiveness of the largest (tracheal 22 × 40 mm) Wallstents in treating baffle obstructions.
Design—Retrospective analysis of patients with stented venous pathways.
Subjects—Eleven patients with baffle obstruction after Mustard repair for transposition of the great arteries.
Interventions—Stenoses were dilated with an 18 or 20 mm balloon. However, recoil was noticed in 11 patients: immediately (n = 7) or on repeat angiography (n = 4). Eighteen stents were implanted (mean (SD)) 18 (3.3) years postoperatively. After dilatation a tracheal Wallstent (11.5 F) was deployed.
Main outcome measures—Relief of obstruction, haemodynamic improvement.
Results—In the inferior vena cava, 10 stents were deployed in seven baffle obstructions with an increase in diameter from 9.8 (2.4) mm to 16.5 (1.4) mm (p < 0.01) and a mean (SD) pressure gradient decrease from 5.1 (3.6) mm Hg to 1.4 (2.0) mm Hg; in the superior vena cava, eight stents were implanted increasing the diameter from 9.1 (3.7) mm to 15.6 (3.8) mm (p < 0.001) with a decrease in mean pressure gradient from 5.1 (2.7) mm Hg to 1.9 (1.5) mm Hg. No complications were experienced during implantation. No anticoagulation was prescribed. During follow up (1.7 (0.6) years; range, 0.9-2.6) no problems were noted; five patients were re-catheterised without change in measurements. There was no evidence of peal formation in any of the stents.
Conclusion—It is concluded that Wallstents are safe, easy to use, and effective in relieving baffle obstruction. Anticoagulation does not seem neccessary.

 Keywords: Mustard procedure;  venous baffle obstruction;  stent

The Research Committee of the British Thoracic Association conducted a confidential inquiry into death from asthma in adults aged 15-64 years resident in the West Midland and Mersey Regions during 1979. Death certificates recording the word asthma were received for 153 persons. The International Classification of Diseases code for the cause of death was obtained from the Office of Population Censuses and Surveys. Information about the patients, their illness, and their death was obtained by interviews, questionnaires, and inspection of patients' records. A panel of three physicians assisted by a pathologist assessed the clinical and, where applicable, the necropsy findings to ascertain whether bronchial asthma was the true cause of death. Of 147 assessable patients, 89 were considered by the panel to have died from asthma. In 77 of these cases the death certificates were correctly coded, whereas in 12 (13%) death was considered to have been wrongly attributed to another cause (falsely negative). Twenty four deaths on the other hand were considered to have been wrongly attributed to asthma (falsely positive). From this it appears that the total number of 101 certificates recording death from asthma represents a net overestimate of 13%. Accuracy was highest in the youngest age group. There were few discrepancies between the assessment of the panel and certified cause of death when a necropsy had been performed. The most common error (17% of all certificates) was failure to follow the procedure advised for completion of death certificates. This usually occurred when patients suffered from two or more conditions, or when death was sudden and necropsy was not performed.

A 28-year-old woman, who as an infant had undergone the Mustard atrial switch procedure for dextro-transposition of the great arteries, presented with a baffle leak and consequent intermittent cyanosis. In addition, an occlusive thrombus had formed in the systemic venous baffle after a failed attempt to remove infected pacemaker leads. Corrective surgery was successful. In addition to the case of our patient, we discuss long-term sequelae of the atrial switch procedure that present challenges in patient care.

There is a high prevalence of consanguinity and bronchial asthma in Saudi Arabia. The objective of this study is to explore the effect of parental consanguinity on the occurrence of bronchial asthma in children. The study sample was determined by multistage random probability sampling of Saudi households. The families with at least one child with asthma were matched with an equal number of families randomly selected from a list of families with healthy children, the latter families being designated as controls. There were 103 families with children having physician-diagnosed bronchial asthma, matched with an equal number of families with no children with asthma. This resulted in 140 children with bronchial asthma and 295 children from controls. The age and gender distribution of the children with bronchial asthma and children from controls were similar. There were 54/103 (52.4%) and 61/103 (59.2%) cases of positive parental consanguinity in asthmatic children and children from controls respectively (P = 0.40). Analysis of consanguinity status of the parents of children with asthma and parents among controls indicates that 71/140 (51%) of the children with asthma and 163/295 (55.3%) of the children from controls had positive parental overall consanguinity (P = 0.43). The results of this study suggest that parental consanguinity does not increase the risk of bronchial asthma in children.

Background

Brazil still does not have a national program to combat asthma. Isolated initiatives have been developed in a non-standardized fashion. The Program for Control of Asthma in Bahia (ProAR) was established in Salvador, Bahia, in 2003, aiming for the control of the most severe cases.

Objective

To analyze time trends in mortality from asthma and its distribution in the districts of Salvador (2000–2009) and to correlate mortality rates with social indicators.

Methods

Observational study of deaths from asthma registered by the National Database of Mortality according to ICD-10. Mortality rates were calculated per 100,000 inhabitants and analyzed by simple linear regression. The distribution of mortality for asthma in the period was mapped into the 12 health districts of Salvador. The correlation of the number of deaths in Salvador with GDP per capita, HDI and Index Gini was evaluated.

Results

The average asthma mortality in Salvador between 2000 and 2009 was 1.542/100.000 inhabitants, with a declining trend (R2 = 0.539, b = –11.1, P = 0.016). Deaths occurred more frequently in women than men (66% vs 34%). Asthma mortality rates were higher in subjects > 35 years. There was a reduction at ages younger than 1 year, 5 to 14 years, 25 to 34 years, and 45 to 54 years with a sharp decline between 55 and 64 (–8.14/100,000). The mortality rate (19.68/100,000 inhabitants in 2009) was higher for individuals > 75 years. The highest mortality rates were noted in more populated and poorer areas with less infrastructure and access to health services. It was observed that 78% of the deaths occurred in hospitals or health facilities. Deaths rates for asthma correlated directly with the district Gini index (rho = 0.400, P = 0.505) and inversely with HDI (rho = –0.300, P = 0.624), though not statistically significant.

Conclusions

Asthma mortality in Salvador is concentrated in the poorest areas with less infrastructure and access to health services, most commonly affecting women and the elderly. There was a reduction in mortality during the study period, possibly related to interventions for asthma control in the municipality. Mortality from asthma behaves differently in each district of the city.

Background:

Asthma is a common health problem whose prevalence in Saudi Arabia has risen over the last few decades. Brick factories in the city of Bahrah have exacerbated the problem, and increasing numbers of asthma cases are attending local primary health care centers.

Objective:

Determine the risk factors of asthma in Bahrah.

Materials and Methods:

The study was comprised of 110 cases of bronchial asthma resident in Bahrah who were diagnosed by the treating physicians and 110 healthy controls matched in age and sex. A questionnaire was completed from cases and controls, consisting of data regarding personal, familial, indoor and outdoor environmental factors that may be potential risk factors to asthma. Bivariate and multivariate logistic regression analyses were done to assess risk factors.

Results:

The mean ages of cases and controls were 22.4 (± 16.7) and 22.8 (± 16.1) years, respectively. Each group consisted of 64 males (58%) and 46 (42%) females. There was a significant association between distance from houses to brick factories and bronchial asthma (Chi square for linear trend = 26.6, p<0.001). Multivariate logistic regression analysis showed that living within one kilometer of a brick factory (OR=5.1, CI=2.33-11.16), family history (OR=4.6, CI=2.16-9.78), allergic rhinitis (OR=3.39, CI=1.49-7.69), skin atopy (OR=4.6, CI=1.53-13.87) and recurrent respiratory tract infections (OR=4.1, CI=1.79-9.22) were independent risk factors for asthma in Bahrah.

Conclusions and recommendations:

Brick factories, family history and history of rhinitis, skin atopy, or recurrent respiratory tract infections are risk factors of bronchial asthma. An in-depth study to asses air pollution in Bahrah is recom-mended. People are advised not to live near brick factories.

Asthma has emerged as a major public health problem in the United States over the past 20 years. Currently, nearly 15 million Americans have asthma, including almost 5 million children. The number of asthma cases has more than doubled since 1980. Approximately 5,500 persons die from asthma each year, and rates have increased over the past 20 years. Rates of death, hospitalization, and emergency department visits are 2-3 times higher among African Americans than among white Americans. The costs of asthma have also increased to 12.7 billion dollars in 1998. Both lifestyle and environmental hypotheses have been invoked to explain the increase in asthma prevalence. Several studies have examined the relationship of obesity and asthma and found associations suggesting that obesity predisposes to the development of asthma. Some studies have found that day care attendance and having older siblings protect against the development of asthma. This observation has led investigators to hypothesize that increased exposure to microbial agents might protect against asthma (the hygiene hypothesis). Environmental exposures found to predispose to asthma include house dust mite allergen and environmental tobacco smoke. Although current knowledge does not permit definitive conclusions about the causes of asthma onset, better adherence to current recommendations for medical therapy and environmental management of asthma would reduce the burden of this disease.

The factors associated with the deaths of 31 asthma patients were examined. The subjects, whose deaths occurred in the period 1967 through 1979, had all received some care at the Hospital for Sick Children in Toronto, but only nine died there. The greatest single cause of death was the inappropriate use of beta-agonists, with or without the concurrent use of epinephrine. In seven patients an asthma attack that occurred outside hospital progressed so rapidly that there was insufficient time for them to obtain adequate therapy. In five cases the assessment of the patient's condition or the therapy recommended by the attending physician appeared to have been inadequate. Two patients suffered an acute attack in hopital and did not respond to treatment that appeared to have been adequate. In six cases the available information was insufficient to indicate the cause of death. Over half (18) of the deaths occurred in teenagers. Various ways of preventing death from asthma are discussed, including better education of physicians and patients, adequate management of factors that provoke bronchospasm, sufficient follow-up -- especially in teenagers -- and the use of approaches with teenagers that encourage better compliance.

Many patients with asthma are treated in the primary care setting. The primary care physician is therefore in a key position to recognize poorly controlled asthma and to improve asthma management for these patients. However, current evidence continues to show that, for a substantial number of patients, asthma control is inadequate for a wide variety of reasons, both physician-related and patient-related. The most recently updated treatment guidelines from the National Asthma Education and Prevention Program were designed to help clinicians, including primary care physicians, manage asthma more effectively with an increased focus on achieving and maintaining good asthma control over time. The current review is intended to assist primary care physicians in improving asthma control among their patients; this review clarifies the new guidelines and provides a specialist's perspective on diagnosis, appropriate therapy, disease control surveillance, and appropriate referral when necessary. This discussion is based primarily on the new guidelines and the references cited therein, supplemented by the author's own clinical experience.

Introduction

Asthma mortality rates are based on deaths for which asthma is coded as the underlying cause on the death certificate. We conducted an asthma mortality review to evaluate this surveillance measure for Minnesota residents who were aged 55 years or older.

Methods

We enlisted an expert panel to review transcribed interviews from the next-of-kin and case histories for decedents whose deaths were attributed to asthma. In addition, we examined death certificates to determine whether the certifier had intended asthma to be the underlying cause.

Results

In the age group of Minnesotans we examined, 55 deaths were attributed to asthma during the 1-year study period. Of the 35 deaths for which adequate information was available for review, 2 were determined to be due to asthma. On 33 of the 55 death certificates, the certifier had chosen asthma as the underlying cause; on the rest, the certifier had not chosen asthma, but inconsistencies in death certificate completion had resulted in "asthma" automatically overriding the underlying cause that had been chosen.

Conclusion

Asthma mortality rates for older Minnesotans may be overestimated because of inaccurate reporting of the underlying cause of death on death certificates.

Research has shown that access to an asthma specialist improves asthma outcomes. We hypothesized that we could improve access to expert asthma care through a telemedicine link between an asthma specialist and a school-based asthma program. We conducted a prospective cohort study in 3 urban schools to ascertain the feasibility of using an asthma-focused telemedicine solution. Each subject was seen by an asthma expert at 0, 8, and 32 weeks. The assessment and recommendations for care were sent to the primary care physician (PCP) and parents were told to contact their physician for follow-up care. Eighty three subjects participated in the study. Subjects experienced improvement (P < .05) in family social activities and the number of asthma attacks. Ninety four percent of subjects rated the program as good or excellent. This study demonstrates the feasibility and acceptance of a school-based asthma program using a telemedicine link to an asthma specialist.

OBJECTIVE

Population-based asthma detection is a potential strategy to reduce asthma morbidity in children; however, the burden of respiratory symptoms and health care use among children identified by case detection is not well known.

METHODS

Data come from a school-based asthma case detection validation study of 3539 children. Respiratory symptoms, emergency department (ED) visits, and hospitalizations were assessed by questionnaire for children whose case detection result and physician study diagnosis agreed.

RESULTS

Physician evaluation of 530 case detection results yielded 420 cases of agreement (168 children with previously diagnosed asthma, 39 with undiagnosed asthma, and 213 without asthma). Children with previously diagnosed asthma were more likely to be male (P < .0001). No differences in severity were observed in children with previously and undiagnosed asthma (P = .31). Children with undiagnosed asthma reported less frequent daytime and nighttime symptoms than children with previously diagnosed asthma but more than those without asthma (P < .0001). The proportion of children with at least 1 respiratory-related ED visit in the past year was 32%, 3%, and 3% for those with previously diagnosed, undiagnosed, and no asthma, respectively (P < .0001). The proportion with at least 1 respiratory-related hospitalization was 8%, 0%, and 0%, respectively (P < .0001). There were no differences in nonrespiratory ED visits (P = .93).

CONCLUSIONS

Despite similar physician-rated severity, children with undiagnosed asthma reported significantly less frequent respiratory symptoms and health care use than children with previously diagnosed asthma. These findings suggest that the potential health gains from case detection may be smaller than expected.

BACKGROUND—It is important to diagnose asthma at an early stage as early treatment may improve the prognosis in the long term. However, many patients do not present at an early stage of the condition so the physician may have difficulty with the diagnosis. A study was therefore undertaken to compare the proportion of patients who underpresented their respiratory symptoms with the proportion of underdiagnosed cases of asthma by the general practitioner (GP). A secondary aim was to investigate whether bad perception of dyspnoea by the patient was a determining factor in the underpresentation of asthma symptoms to the GP.
METHODS—A random sample of 1155 adult subjects from the general population in the eastern part of the Netherlands was screened for respiratory symptoms and lung function and the results were compared with the numbers of asthma related consultations registered in the medical files of the GP. In subjects with reduced lung function the ability to perceive dyspnoea was investigated during a histamine provocation test in subjects who did and did not report their symptoms to their GP.
RESULTS—Of the random sample of 1155 subjects 86 (7%) had objective airflow obstruction (forced expiratory volume in one second (FEV1) below the reference value corrected for age, length, and sex minus 1.64SD on two occasions) and had symptoms suggestive of asthma. Of these 86 subjects only 29 (34%) consulted the GP, which indicates underpresentation by 66% of patients. Of all subjects with objective airflow obstruction who presented to their GP with respiratory symptoms, 23 (79%) were recorded in the medical files as having asthma, indicating underdiagnosis by the GP in 21% of cases. Of the subjects with objective airflow obstruction who visited the GP with respiratory symptoms 6% had bad perception of dyspnoea compared with 26% of those who did not present to the GP in spite of airflow obstruction (χ2 = 3.02, p = 0.08).
CONCLUSIONS—Underpresentation to GPs of respiratory symptoms by asthmatic patients contributes significantly to the problem of underdiagnosis of asthma. Underdiagnosis by the GP seems to play a smaller role. Furthermore, there are indications that underpresentation of symptoms by the patient is at least partly explained by a worse perception of dyspnoea.



Regulated upon activation, normal T-cell expressed and secreted (RANTES) is one of the most extensively studied C-C chemokines in allergic inflammation. A growing body of evidence suggests that many cell types present in asthmatic airways have the capacity to generate RANTES, which directly supported the potential role of RANTES in asthma. A number of studies have evaluated the functional polymorphism -28C/G in the RANTES promoter region, which had been found to affect the transcription of the RANTES gene, in relation to asthma susceptibility. However, the results remain conflicting rather than conclusive. This meta-analysis on 1894 asthma cases and 1766 controls for -28C/G from 9 published case-control studies showed that the variant allele -28G was associated with significantly increased risk of asthma (GG+CG vs CC: OR=1.24, 95%CI=1.08-1.41) without any between-study heterogeneity.

In the stratified analysis by asthma type, age and ethnicity, we found that the increased asthma risk associated with -28G/C polymorphism was more evident in children (OR=1.24, 95%CI=1.06-1.45), Asian group (OR=1.27, 95%CI=1.04-1.56) and African group (OR=1.72, 95%CI=1.07-2.78). These results suggest that RANTES -28G/C polymorphism may contribute to asthma development, especially in children and in Asian population. Additional well-designed large studies were required for the validation of this association.

Formaldehyde is but one of many chemicals capable of causing the tight building syndrome or environmentally induced illness (EI). The spectrum of symptoms it may induce includes attacks of headache, flushing, laryngitis, dizziness, nausea, extreme weakness, arthralgia, unwarranted depression, dysphonia, exhaustion, inability to think clearly, arrhythmia or muscle spasms. The nonspecificity of such symptoms can baffle physicians from many specialties. Presented herein is a simple office method for demonstrating that formaldehyde is among the etiologic agents triggering these symptoms. The very symptoms that patients complain of can be provoked within minutes, and subsequently abolished, with an intradermal injection of the appropriate strength of formaldehyde. This injection aids in convincing the patient of the cause of the symptoms so he can initiate measures to bring his disease under control.

Primary Atrophic Rhinitis (PAR) which has baffled the physicians of the past and the present ENT surgeons, till now, is found to have strong clinical evidence to suggest it to be of Hanseniatic origin. A rational review of literature, elicitation of the cardinal signs of leprosy — in its indeterminate paucibacillary form, and the similarity of the symptomatology between these two diseases corroborate this view. This is further sustained by observing it cured with antileprotic drugs which brings a new hope for these patients. It also unfolds a new horizon on further research on this disease.

Thirty children aged from 7 weeks to 14 years were examined by echocardiography after Mustard's operation for transposition of the great arteries. Discrete and persitent echoes were noted within the original left atrial cavity and contrast echocardiography was used to establish that these originated from the interatrial baffle. In the presence of caval channel obstruction, caused by malposition or shrinkage of the baffle, significant differences were seen in the echocardiographic appearances of the baffle, namely limitation of baffle motion, thickening, and multiplicity of the baffle echoes. These findings suggest that the technique may be of value in the postoperative assessment of patients with transposition of the great arteries.

Images

Nurses often encounter patients with chronic pelvic pain associated with endometriosis, which is a puzzling and problematic gynecologic condition that has continued to plague women and baffle doctors and researchers worldwide since it was first identified by Dr. J. Sampson in the 1920s (Sampson, 1940). Endometriosis is defined as the growth, adhesion and progression of endometrial glands and stroma outside of the uterine cavity, with cellular activity evident in lesions, nodules, cysts or endometriomas (Audebert et al., 1992). Although it typically appears benign on histopathology, endometriosis has been likened to a malignant tumor since the lesions grow, infiltrate and adhere to adjacent tissues and interfere with physiologic processes (Kitawaki et al., 2002; Noble, Simpson, Johns, & Bulun, 1996). Ectopic endometriotic growths respond to cyclic changes of estrogen and proliferate and shed in a manner similar to eutopic endometrium. This cyclic ectopic activity results in internal bleeding, formation of scar tissue, inflammation and sometimes debilitating chronic pain (Kitawaki et al.).

Objective To investigate the association between bronchodilator treatment and death from asthma.

Design Case-control study.

Setting 33 health authorities or health boards in Great Britain.

Participants 532 patients under age 65 who died from asthma and 532 controls with a hospital admission for asthma matched for period, age, and area.

Main outcome measures Odds ratios for deaths from asthma associated with prescription of bronchodilators and other treatment, with sensitivity analyses adjusting for age at onset, previous hospital admissions, associated chronic obstructive lung disease, and number of other drug categories.

Results After full adjustment, there were no significant associations with drugs prescribed in the 4-12 months before the index date. For prescriptions in the 1-5 years before, mortality was positively associated with inhaled short acting β2 agonists (odds ratio 2.05, 95% confidence interval 1.26 to 3.33) and inversely associated with antibiotics (0.59, 0.39 to 0.89). The former association seemed to be confined to those aged 45-64, and the association with antibiotics was more pronounced in those under 45. Significant age interactions across all periods suggested inverse associations with oral steroids confined to the under 45 age group. An inverse association with long acting β2 agonists and a positive association with methylxanthines in the 1-5 year period were non-significant.

Conclusion There was no evidence of adverse effects on mortality with medium to long term use of inhaled long acting β2 agonist drugs. The association with short acting β2 agonists has several explanations, only one of which may be a direct adverse effect.

With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. They have distinctive forms of heart failure, and their cardiac disease can be associated with pulmonary hypertension, thromboemboli, complex arrhythmias and sudden death. Medical aspects that need to be considered relate to the long-term and multisystemic effects of single-ventricle physiology, cyanosis, systemic right ventricles, complex intracardiac baffles and failing subpulmonary right ventricles. Since the 2001 Canadian Cardiovascular Society Consensus Conference report on the management of adults with CHD, there have been significant advances in the understanding of the late outcomes, genetics, medical therapy and interventional approaches in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. The present executive summary is a brief overview of the new guidelines and includes the recommendations for interventions. The complete document consists of four manuscripts that are published online in the present issue of The Canadian Journal of Cardiology, including sections on genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy and contraception risks, and follow-up requirements. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.