Why industrial methods do not work in healthcare: an analytical approach.

Healthcare professionals and managers in hospitals are frequently suggested to learn from industry and business to improve quality and efficiency. However, evidence that the implementation of industrial techniques and business methods has a meaningful effect on patient outcomes is often lacking. An explanation for this phenomenon is thought to be the complexity of the hospital organisation and the diversity of patients. In this article, we use the practice approach to discuss the application of industrial techniques and business methods in healthcare. We employ a practice model that offers three perspectives to understand professional practices: Identity and intrinsic values, Interests of stakeholders, and Ideals and basic beliefs (Triple I). This model demonstrates that the nature of healthcare practices differs strongly from the nature of industrial and business practices. Healthcare has a moral nature that does not let itself be easily organised along technological or business categories. This may provide a fundamental explanation of why industrial techniques and business methods in general will be less successful in healthcare. At the same time, this model invites hospitals to develop innovative approaches that do justice to the identity and intrinsic values of healthcare. In this process, insights from industry and business cannot be copied but have to be used as sources of inspiration.

Pathogenic effect of a TGFBR1 mutation in a family with Loeys-Dietz syndrome.

BACKGROUND: Thoracic aortic aneurysms and dissections (TAAD) may have a heritable cause in up to 20% of cases. We aimed to investigate the pathogenic effect of a TGFBR1 mutation in relation to TAAD. METHODS: Co-segregation analysis was performed followed by functional investigations, including myogenic transdifferentiation. RESULTS: The c.1043G>A TGFBR1 mutation was found in the index patient, in a deceased brother, and in five presymptomatic family members. Evidence for pathogenicity was found by the predicted damaging effect of this mutation and the co-segregation in the family. Functional analysis with myogenic transdifferentiation of dermal fibroblasts to smooth muscle-like cells, revealed increased myogenic differentiation in patient cells with the TGFBR1 mutation, shown by a higher expression of myogenic markers ACTA2, MYH11 and CNN1 compared to cells from healthy controls. CONCLUSION: Our findings confirm the pathogenic effect of the TGFBR1 mutation in causing TAAD in Loeys-Dietz syndrome and show increased myogenic differentiation of patient fibroblasts.

Ascending Aortic Aneurysm Secondary to Isolated Noninfectious Ascending Aortitis.

Isolated noninfectious ascending aortitis (I-NIAA) is increasingly diagnosed at histopathologic review after resection of an ascending aortic aneurysm. PubMed was searched using the term aortitis; publications addressing the issue were reviewed, and reference lists of selected articles were also reviewed. Eleven major studies investigated the causes of an ascending aortic aneurysm or dissection requiring surgical repair: the prevalence of noninfectious aortitis ranged from 2% to 12%. Among 4 studies of lesions limited to the ascending aorta, 47% to 81% of cases with noninfectious aortitis were I-NIAA, more frequent than Takayasu arteritis or giant cell arteritis. Because of its subclinical nature and the lack of "syndromal signs" as in Takayasu arteritis or giant cell arteritis, I-NIAA is difficult to diagnose before complications occur, such as an aortic aneurysm or dissection. Therefore, surgical specimens of dissected aortic tissue should always be submitted for pathologic review. Diagnostic certainty requires the combination of a standardized histopathologic and clinical investigation. This review summarizes the current knowledge on I-NIAA, followed by a suggested approach to diagnosis, management, and follow-up. An illustrative case of an uncommon presentation is also presented. More follow-up studies on I-NIAA are needed, and diagnosis and follow-up of I-NIAA may benefit from the development of diagnostic biomarkers.

Expert consensus recommendations on the cardiogenetic care for patients with thoracic aortic disease and their first-degree relatives.

BACKGROUND: Thoracic aortic aneurysm (TAA) is a potentially life-threatening disorder with a strong genetic component. The number of genes implicated in TAA has increased exponentially over the last decade. Approximately 20% of patients with TAA have a positive family history. As most TAA remain asymptomatic for a long time, screening of at risk relatives is warranted to prevent complications. Existing international guidelines lack detailed instructions regarding genetic evaluation and family screening of TAA patients. We aimed to develop a consensus document to provide medical guidance for all health care professionals involved in the recognition, diagnosis and treatment of patients with thoracic aortic disease and their relatives. METHODS: A multidisciplinary panel of experts including cardiologists, cardiothoracic surgeons, clinical geneticists and general practitioners, convened to review and discuss the current literature, guidelines and clinical practice on genetic testing and family screening in TAA. RESULTS: There is a lack of high-quality evidence in the literature. This consensus statement, based on the available literature and expert opinions, summarizes our recommendations in order to standardize and optimize the cardiogenetic care for patients and families with thoracic aortic disease. In particular, we provide criteria to identify those patients most likely to have a genetic predisposition, and discuss the preferred modality and frequency of screening in their relatives. CONCLUSIONS: Age, family history, aortic size and syndromic features determine who is advised to have genetic testing as well as screening of first-degree relatives. There is a need for more prospective multicenter studies to optimize current recommendations.

Yield of family screening in patients with isolated bicuspid aortic valve in a general hospital.

AIM: To determine the prevalence of unidentified bicuspid aortic valve (BAV) or aortic dilatation (>40mm) in first degree relatives (FDR) of patients with isolated BAV in a general hospital. METHODS AND RESULTS: Patients with isolated BAV received information advising cardiac screening of their FDR. Referred and screened were 134 FDR of 54 adult index patients with isolated BAV (median 2 per index patient). FDR's mean age was 49years (range 16-83years) and 41% were male. They comprised 5 parents (3.7%), 52 siblings (39%) and 77 offspring (57%). Among these FDR, the prevalence of BAV was 6.0% (8 patients). In FDR without BAV, 10 (7.5%) had aortic dilatation. 'Familial BAV' was present in 9/54 families (17%). CONCLUSION: In a general hospital, screening of FDR of patients with isolated BAV resulted in a substantial yield of 13% new cases with BAV or aortic dilatation without BAV.

Effect of Using the HEART Score in Patients With Chest Pain in the Emergency Department: A Stepped-Wedge, Cluster Randomized Trial.

BACKGROUND: The HEART (History, Electrocardiogram, Age, Risk factors, and initial Troponin) score is an easy-to-apply instrument to stratify patients with chest pain according to their short-term risk for major adverse cardiac events (MACEs), but its effect on daily practice is unknown. OBJECTIVE: To measure the effect of use of the HEART score on patient outcomes and use of health care resources. DESIGN: Stepped-wedge, cluster randomized trial. (ClinicalTrials.gov: NCT01756846). SETTING: Emergency departments in 9 Dutch hospitals. PATIENTS: Unselected patients with chest pain presenting at emergency departments in 2013 and 2014. INTERVENTION: All hospitals started with usual care. Every 6 weeks, 1 hospital was randomly assigned to switch to "HEART care," during which physicians calculated the HEART score to guide patient management. MEASUREMENTS: For safety, a noninferiority margin of a 3.0% absolute increase in MACEs within 6 weeks was set. Other outcomes included use of health care resources, quality of life, and cost-effectiveness. RESULTS: A total of 3648 patients were included (1827 receiving usual care and 1821 receiving HEART care). Six-week incidence of MACEs during HEART care was 1.3% lower than during usual care (upper limit of the 1-sided 95% CI, 2.1% [within the noninferiority margin of 3.0%]). In low-risk patients, incidence of MACEs was 2.0% (95% CI, 1.2% to 3.3%). No statistically significant differences in early discharge, readmissions, recurrent emergency department visits, outpatient visits, or visits to general practitioners were observed. LIMITATION: Physicians were hesitant to refrain from admission and diagnostic tests in patients classified as low risk by the HEART score. CONCLUSION: Using the HEART score during initial assessment of patients with chest pain is safe, but the effect on health care resources is limited, possibly due to nonadherence to management recommendations. PRIMARY FUNDING SOURCE: Netherlands Organisation for Health Research and Development.

Knowledge of native valve anatomy is essential in follow-up of patients after aortic valve replacement.

BACKGROUND: After aortic valve replacement (AVR), bicuspid aortic valve (BAV) patients continue to be at risk of aortic complications. Therefore, knowledge of native valve anatomy is important for follow-up. We aimed to determine the extent of which the presence of BAV disease is known in a regional post-AVR population. METHODS: The Electronical Medical Record system was used to collect all patients under follow-up after AVR. We documented their clinical data and used the operative report to determine valve phenotype; lacking reports were retrieved. RESULTS: We identified 560 patients who underwent AVR between 1971 and 2012, with a median of 6.2years follow-up postoperatively. Mean age at surgery was 66years (SD13.2years), and 319 patients (57%) were male. In 29 cases (5%), an operative report was not available and in 85 patients (16%) the report lacked a description of valve phenotype. In 446 patients, a surgeon's description of native valve was available: 299 patients (67%) had tricuspid aortic valve, 140 (31%) BAV, and 3 (1%) quadricuspid aortic valve. In 4 patients (1%) the description was non-conclusive. In 66/140 BAV patients the surgeon's diagnosis was not reported back to the referring cardiologist, which corresponded with 12% of all 560 AVR patients. Another 21% of these 560 lacked a clear description of native valve anatomy: no report, no native valve description or an unclear valve description. CONCLUSIONS: Native valve anatomy was not known in one-third of AVR patients under follow-up, which included almost half of the BAV patients. This lack of knowledge withholds patients from appropriate ascending aorta surveillance.

Contemporary prevalence of pulmonary arterial hypertension in adult congenital heart disease following the updated clinical classification.

BACKGROUND: The aging congenital heart disease (CHD) population is prone to develop a variety of sequelae, including pulmonary arterial hypertension (PAH). Previous prevalence estimates are limited in applicability due to the use of tertiary centers, or database encoding only. We aimed to investigate the contemporary prevalence of PAH in adult CHD patients, using a nationwide population. METHODS: A cross-sectional study was performed, using the population-based Dutch CONgenital CORvitia (CONCOR) registry. All patients born with a systemic-to-pulmonary shunt, thereby at risk of developing PAH, were identified. From this cohort, a random sample was obtained and carefully reviewed. RESULTS: Of 12,624 registered adults with CHD alive in 2011, 5,487 (44%) were at risk of PAH. The random sample consisted of 1,814 patients (mean age 40 ± 15 years) and 135 PAH cases were observed. PAH prevalence in patients born with a systemic-to-pulmonary shunt was 7.4%. The prevalence of PAH after corrective cardiac surgery was remarkably high (5.7%). Furthermore, PAH prevalence increased with age, from 2.5% under 30 years until 35% in the eldest. PAH prevalence in the entire CHD population was 3.2%. Based on 3000 per million adult CHD patients in the general population, we can assume that PAH-CHD is present in 100 per million. CONCLUSIONS: This new approach using a nationwide CHD population reports a PAH prevalence of 3.2% in CHD patients, and 100 per million in the general adult population. Especially in patients after shunt closure and the elderly, physicians should be aware of PAH-CHD, to provide optimal therapeutic and clinical care.

Infectious aortitis with multiple mycotic aneurysms caused by Streptococcus agalactiae.

We report a case of a 66-year-old man with multiple thoracoabdominal mycotic aortic aneurysms caused by Streptococcus agalactiae (S agalactiae). The infectious aortitis (IA) was diagnosed by transesophageal echocardiography and computed tomography and confirmed by positive blood cultures. The patient was treated with antibiotics, but, after worsening of the aortitis, a successful surgical procedure was performed. A review of the literature is presented together with a series of 7 other cases of IA caused by S agalactiae.

The predictive value of the exercise ECG for major adverse cardiac events in patients who presented with chest pain in the emergency department.

BACKGROUND: To improve early diagnostic and therapeutic decision making, we designed the HEART score for chest pain patients in the emergency department (ED). HEART is an acronym of its components: History, ECG, Age, Risk factors and Troponin. Currently, many chest pain patients undergo exercise testing on the consecutive days after presentation. However, it may be questioned how much diagnostic value the exercise ECG adds when the HEART score is already known. METHODS: A subanalysis was performed of a multicenter prospective validation study of the HEART score, consisting of 248 patients who underwent exercise testing within 7 days after presentation in the ED. Outcome is the predictive value of exercise testing in terms of major adverse cardiac events (MACE) within 6 weeks after presentation. RESULTS: In low-risk patients (HEART score ≤ 3), 63.1 % were negative tests, 28.6 % non-conclusive and 8.3 % positive; the latter were all false positives. In the intermediate-risk group (HEART score 4-6), 30.9 % were negative tests, 60.3 % non-conclusive and 8.8 % positive, half of these positives were false positives. In the high-risk patients (HEART score ≥ 7), 14.3 % were negative tests, 57.1 % non-conclusive and 28.6 % positive, of which half were false positives. CONCLUSION: In a chest pain population risk stratified with HEART, exercise testing has only a modest contribution to clinical decision making. 50 % of all tests are non-conclusive, with high rates of false positive tests in all three risk groups. In intermediate-risk patients, negative exercise tests may contribute to the exclusion of disease. Clinicians should rather go for sensitive tests, in particular in patients with low HEART scores.

Circumstances of death in adult congenital heart disease.

BACKGROUND: Circumstances of death have been described for various cardiovascular diseases, but this study is the first for adults with congenital heart disease (CHD). METHODS: Review of medical records and additional information from treating cardiologists and general practitioners, for circumstances of all deaths in a national registry of over 8000 adults with CHD. RESULTS: Of 8595 patients, 231 (2.7%) patients died over 26,500 patient years. Main causes of death were progressive heart failure (26%) and sudden cardiac death (22%). Mortality was highest in the northern, most rural region of the country (p ≤ 0.05). Overall, death occurred out-of-hospital in approximately 35%, but more frequently in rural than in urban areas (55% versus 32%, p ≤ 0.05). Mortality was almost equally distributed throughout the seasons, although fall showed a slightly higher mortality rate. Cardiovascular death occurred suddenly in nearly 40%. Sudden cardiovascular death occurred in 8% during exercise, and most often out-of-hospital (62%). Of non-sudden cardiovascular deaths 18% had occurred out-of-hospital. CONCLUSION: In adult patients with congenital heart disease, mortality shows substantial regional and subtle seasonal variation. Death usually occurs at rest; approximately 1 of 10 sudden cardiovascular deaths occur during exercise.