Hypertrophic cardiomyopathy (HCM) FAQs

Unless stated otherwise the information below is based on a review in Circulation. It’s a little dated but good, and available as free full text.

What are the differences between HCM as it classically presents in younger patients and HCM of the elderly? Is there such a thing as acquired HCM of the elderly?

Genetic HCM can have its presentation delayed until old age but genetic testing, as opposed to young patients, has a low yield in HCM of the elderly lacking a positive family history.

Certain mutations are known to predispose to later onset disease.

Later onset disease is associated with milder hypertrophy and a more benign course attributable to the disease itself.

Upper septal hypertrophy (USH), aka disproportionate upper septal thickening (DUST) (overlapping with the concept of sigmoid septum) is characteristic though not exclusively seen in the elderly. Its relationship to genetic HCM is unclear but when seen in isolation it is not associated with a bad prognosis.

It is currently believed that an acquired form of HCM, including “IHSS physiology”, can be seen in the elderly, arising from LVH secondary to hypertension or valvular aortic stenosis. The exception is that these patients generally don’t have asymmetric septal hypertrophy (ASH) but rather concentric LVH.

What about the genetics?

A genetic abnormality can be found in most younger patients with HCM and a positive family history.

Up to Date says many mutations have been found in 11 genes.

According to the ACC/AHA guidelines genetic screening has a class IIa recommendation for an index case. Screening of first degree relatives can be clinical (phenotypic) or genetic. Genetic screening to assess the risk of SCD carries only a IIb recommendation. Cost considerations apply in the real world.

What are the anatomic and physiologic variants?

HCM does not always present with the classic “IHSS” phenotype. From my reading of Up to Date, other variants include DUST (which may occur in isolation and not represent HCM, see above), mid septum and free wall hypertrophy resulting in an intracavitay gradient, free wall greater then septal hypertrophy (rare), varying degrees of concentric LVH, and apical HCM (Yamaguchi syndrome).

What are the risk factors for SCD and how should they be taken into account in deciding whether to recommend an AICD?

From the Circulation article:

Accepted risk factors for SCD are unexplained syncope; family history of SCD due to HCM or occurring with no other explanation before 50 years of age; extreme (greater than 30 mm) LVH; ventricular tachycardia, as detected by Holter monitoring; and an abnormal blood pressure response to exercise. Young and middle-aged patients at risk of SCD are usually offered an implantable cardioverter-defibrillator. The risk of SCD may be lower in elderly patients with HCM, but there is uncertainty with regard to how to adapt the conventional risk factors to this population.

These factors are taken together, and a clinical scoring tool is available. Genetic testing to decide on device implantation is not highly recommended.

What are the medical treatments? Where do surgical treatments and septal ablation fit in?

A nice algorithm from the Circulation article is here.

Your patient wants to leave AMA. Now what?

There’s an interesting article in the Journal of Hospital Medicine on what to do when a patient wants to leave the hospital against medical advice. After reading and rereading it I had to disagree with the conclusion but it took me a bit to get there because the article, with its confusing use of terms, is a masterpiece of obfuscation. The most obvious example is the oxymoronic use of the term “AMA discharge” in the title and throughout the article. If a patient leaves AMA it's not your decision. How is that a discharge? Put another way, if you discharge the patient you are making a statement that the patient is medically ready to leave the hospital. A discharge order can only mean the patient is leaving in accordance with, not against, your advice. Why, after all, would you enter an order for something that is against your own judgment?

Another example of language confusion is the authors’ statement that leaving the hospital AMA can be a process of informed consent. Quoting directly from the article:

Because all competent patients have the right to decline recommended inpatient treatment, the ethical and legal standard is that the physician obtain the patient’s informed consent to leave…

Consent to leave? That’s an inappropriate use of the word. Consent leads to adherence with the physician’s recommendation, which in this case would be to remain in the hospital. In the AMA situation the patient’s decision to leave is a demand, not a consent.

Getting past all the confusion, there were a few good points. When the patient leaves against your advice you don’t have to destroy the rapport. It doesn’t have to be an adversarial transaction. But the authors go beyond that principle by stating that when the patient leaves AMA not only should it be handled as a regular discharge but that you should not even document that the departure is against your advice. Again, form the article:

The solution to improve quality is straightforward—avoid designating discharges as AMA…

Treat all discharges similarly. Avoid designating an inpatient discharge as AMA.

That is where I have to disagree.

There's more to unpack. The authors make frequent mention of shared decision-making. Indeed shared decision-making is a is a core principle of evidence-based medicine but it is just one component. The AMA departure sometimes pushes shared decision making to the level of absurdity. How does it apply, for example, if the patient with an actively evolving myocardial infarction wants to leave the emergency room? What if the patient just swallowed antifreeze because he ran out of his beverage of choice? Where does shared decision making come in when the patient’s choice means almost certain harm? Some patient preferences and choices are simply wrong.

Finally there's the matter of legal protection. The authors make this statement:

Although clinicians may presume that the AMA designation provides protection from liability, the claim is not supported by the available literature.14,15 In these studies, which reviewed relevant case law, defendants prevailed not because of the physician’s AMA designation, but because the plaintiff was not able to prove negligence.

That’s a misrepresentation of the cited articles. Both articles (see here and here) contain statements to the effect that the AMA designation may indeed afford some legal protection.

So what should we do? Why not consider each case on its individual merits? If the patient wants to leave prematurely but the risk is low it may be reasonable to capitulate and enter a discharge order. In other situations where the patient's choice is clearly ill-advised and the risk is high a discharge order may be inappropriate and the departure should be documented as being against medical advice. Even in such cases try to work with the patient to help formulate a follow-up plan and, if appropriate, provide medication prescriptions. Assure the patient that you are not angry, respectfully ask that they reconsider and assure them that they are welcome to return. Clearly advise them about the danger of leaving but don’t threaten them or imply adverse insurance consequences.

Hepatocellular carcinoma (HCC) with a focus on diagnosis and screening

Here is a free full text review.

Recommendations are that all patients with cirrhosis be screened as well as certain hepatitis B patients regardless of cirrhosis if certain risk factors are present. Some guidelines make similar recommendations for hepatitis C. The screening modality of choice is ultrasound. Imaging is favored over tumor markers.

Calcium pyrophosphate crystal deposition disease

Below are some key points from a couple of free full text reviews [1] [2].

Terminology has changed and can be confusing

The current official term is calcium pyrophosphate crystal deposition (CPPD). Pseudogout, a term which historically referred to the acute attacks of CPPD, has been replaced by “acute calcium pyrophosphate crystal arthritis.” The plain radiographic finding known as chondrocalcinosis is frequently replaced by the term “cartridge calcification.” CC is not universal in patients with attacks nor is it entirely specific for CPPD.

Crystal identification is important in diagnosis

As opposed to the needle like negatively birefringent crystals of gout, the crystals of CPPD are variable in shape, often rectangular, and if birefringent at all, only weakly positively birefringent.

There are some disease associations

From the second reference above:

Metabolic disease associations of CPPD include haemochromatosis (18), hyperparathyroidism (19, 20), hypomagnesemia (21) and hypophosphatasia (22). Other diseases such as diabetes mellitus and hypothyroidism do not associate with CPPD once adjusted for age (22, 23). Haemochromatosis is the only metabolic disease associated with CPPD that results in structural arthropathy, and this commonly affects the knees, wrists, hips, MCPJs, and ankles (18, 24).

There are some genetic determinants as well.

The importance of nutrition in cystic fibrosis

From a recent review:

Recent findings

Evidence-based and expert-based guidelines emphasize the need for adequate nutritional intake to improve nutritional status. For infants and young children, the aim is to achieve the 50th percentile of weight and length for a healthy same-age population up to age 2 years. For older children and adolescents 2–18 years, the target is a BMI of at or above the 50th percentile for healthy children. For CF adults of at least 18 years, the target is a BMI of at or above 22 kg/m 2 for women and at or above 23 kg/m 2 for men. Recently, new drugs are developed with the aim to treat the malfunction of the cystic fibrosis transmembrane conductance regulator gene. This potentiator/corrector therapy improves lung function and nutritional status and decreases the number of infective exacerbations. As survival is improving and the CF population is aging, it is important to focus on micronutrient and macronutrient intake of CF patients in different age and disease stages.

Summary

Recent evidence-based nutritional guidelines and improved medical treatment support the nutritional monitoring and interventions in CF patients. Nutritional care should be personalized and provided by a specialized CF dietitian because patients’ care needs may change dramatically during their disease progress.

Insomnia as a cardiovascular disease risk factor

From a review in Chest:

Insomnia is the most prevalent sleep disorder in the United States and has high comorbidity with a number of cardiovascular diseases (CVDs). In the past decade, a number of observational studies have demonstrated an association between insomnia and incident cardiovascular disease (CVD) morbidity and mortality, including hypertension (HTN), coronary heart disease (CHD), and heart failure (HF). Despite some inconsistencies in the literature, likely due to variations in how insomnia is defined and measured, the existing data suggest that insomnia, especially when accompanied by short sleep duration, is associated with increased risk for HTN, CHD and recurrent acute coronary syndrome, and HF. Purported mechanisms likely relate to dysregulation of the hypothalamic-pituitary axis, increased sympathetic nervous system activity, and increased inflammation. This paper reviews the most recent studies of insomnia and CVD and the potential pathophysiological mechanisms underlying this relationship and highlights the need for randomized trials to further elucidate the nature of the relationship between insomnia and CVD.

Insulin in type 2 diabetes: are we pushing too hard?

From a recent meta-analysis:

Conclusions

There is no significant evidence of long term efficacy of insulin on any clinical outcome in T2D. However, there is a trend to clinically harmful adverse effects such as hypoglycaemia and weight gain. The only benefit could be limited to reducing short term hyperglycemia. This needs to be confirmed with further studies.

Inhaled steroid/beta agonist treatments improved oxygenation in patients at risk for ARDS

From the report:

Objectives: Effective pharmacologic treatments directly targeting lung injury in patients with the acute respiratory distress syndrome are lacking. Early treatment with inhaled corticosteroids and beta agonists may reduce progression to acute respiratory distress syndrome by reducing lung inflammation and enhancing alveolar fluid clearance.

Design: Double-blind, randomized clinical trial (ClinicalTrials.gov: NCT01783821). The primary outcome was longitudinal change in oxygen saturation divided by the FIO2 (S/F) through day 5. We also analyzed categorical change in S/F by greater than 20%. Other outcomes included need for mechanical ventilation and development of acute respiratory distress syndrome.

Setting: Five academic centers in the United States.

Patients: Adult patients admitted through the emergency department at risk for acute respiratory distress syndrome.

Interventions: Aerosolized budesonide/formoterol versus placebo bid for up to 5 days.

Measurements and Main Results: Sixty-one patients were enrolled from September 3, 2013, to June 9, 2015. Median time from presentation to first study drug was less than 9 hours. More patients in the control group had shock at enrollment (14 vs 3 patients). The longitudinal increase in S/F was greater in the treatment group (p = 0.02) and independent of shock (p = 0.04). Categorical change in S/F improved (p = 0.01) but not after adjustment for shock (p = 0.15). More patients in the placebo group developed acute respiratory distress syndrome (7 vs 0) and required mechanical ventilation (53% vs 21%).

Conclusions: Early treatment with inhaled budesonide/formoterol in patients at risk for acute respiratory distress syndrome is feasible and improved oxygenation as assessed by S/F. These results support further study to test the efficacy of inhaled corticosteroids and beta agonists for prevention of acute respiratory distress syndrome.

Reperfused STEMI and intramyocardial hemorrhage

This is interesting:

Abstract

Background Findings from recent studies show that microvascular injury consists of microvascular destruction and intramyocardial hemorrhage (IMH). Patients with ST‐segment elevation myocardial infarction (STEMI) with IMH show poorer prognoses than patients without IMH. Knowledge on predictors for the occurrence of IMH after STEMI is lacking. The current study aimed to investigate the prevalence and extent of IMH in patients with STEMI and its relation with periprocedural and clinical variables.

Methods and Results A multicenter observational cohort study was performed in patients with successfully reperfused STEMI with cardiovascular magnetic resonance examination 5.5±1.8 days after percutaneous coronary intervention. Microvascular injury was visualized using late gadolinium enhancement and T2‐weighted cardiovascular magnetic resonance imaging for microvascular obstruction and IMH, respectively. The median was used as the cutoff value to divide the study population with presence of IMH into mild or extensive IMH. Clinical and periprocedural parameters were studied in relation to occurrence of IMH and extensive IMH, respectively. Of the 410 patients, 54% had IMH. The presence of IMH was independently associated with anterior infarction (odds ratio, 2.96; 95% CI, 1.73–5.06 [P less than 0.001]) and periprocedural glycoprotein IIb/IIIa inhibitor treatment (odds ratio, 2.67; 95% CI, 1.49–4.80 [P less than 0.001]). Extensive IMH was independently associated with anterior infarction (odds ratio, 3.76; 95% CI, 1.91–7.43 [P less than 0.001]). Presence and extent of IMH was associated with larger infarct size, greater extent of microvascular obstruction, larger left ventricular dimensions, and lower left ventricular ejection fraction (all P less than 0.001).

Conclusions Occurrence of IMH was associated with anterior infarction and glycoprotein IIb/IIIa inhibitor treatment. Extensive IMH was associated with anterior infarction. IMH was associated with more severe infarction and worse short‐term left ventricular function in patients with STEMI.

Clinical Perspective

What is New?

This is the first study to link periprocedural additional glycoprotein IIb/IIIa inhibitor treatment to higher occurrence of intramyocardial hemorrhage in patients with reperfused ST‐segment elevation myocardial infarction.

What are the Clinical Implications?

The optimal application of aggressive antithrombotic therapies in patients with ST‐segment elevation myocardial infarction undergoing percutaneous coronary intervention remains to be studied, especially in the era of adequate double antiplatelet preloading.

Anterior infarct location predicted presence and severity of intramyocardial hemorrhage and may prove useful in direct risk stratification.

What the hospitalist needs to know about interstitial lung disease

Here are some key points from a review in the Journal of Hospital Medicine:

What is the classification?

Categories are exposure related (eg hypersensitivity pneumonitis, drugs, occupational), connective tissue disease related, idiopathic and miscellaneous (including sarcoid, Langerhans cell histiocytosis, eosiniphilic pneumonia and vasculitis (including diffuse alveolar hemorrhage).

What labs should be ordered initially on a patient suspected for the first time to have ILD?

According to the review, ANA, RA, anti cyclic citrullinated peptide, CK and aldolase. (Aldolase may be elevated while the CK is normal in some cases of inflammatory myopathy). (I would wonder if synthetase antibody testing should be added to this list). After this round of testing, subsequent tests can be added based on initial results and the ensuing clinical course.

What imaging studies should initially be done?

CXR and HRCT according to the article, which also mentioned that if clinical conditions warrant, instead of just doing a HRCT, get a CT PA gram with simultaneous high resolution images of the lung parenchyma.

What about bronchoscopy?

This is not considered routine and what I get from the review is that the diagnostic confidence derived from HRCT and the anticipation of how the results might change treatment will influence this decision. Circumstances that might favor doing bronchoscopy include suspected acute eosinophilic pneumonia (AEP), suspected acute hypersensitivity pneumonitis, suspected sarcoid and suspected unusual infection.

When should antibiotics be given?

According to the review, onset or exacerbation of ILD can be difficult to distinguish from infection, so always at least consider them. (Given the all too often undifferentiated nature of the presentation in critically ill patients, I suspect the threshold would be low). The review did not comment on the specific type of coverage. However, many such patients are immunosuppressed, have had frequent hospitalizations, or are critically ill and those factors would guide antibiotic choices.

Should corticosteroids be used?

In cases of clinical deterioration or respiratory failure, which is the case with most patients who require hospitalization, yes. The etiology is often unknown, and it must be kept in mind that some etiologies are known to be highly steroid responsive, particularly AEP and COP. Recommendations also support the use of steroids for CTD related ILD, acute HP and drug induced ILD. IPF by comparison is poorly steroid responsive but current guidelines conditionally recommend their use.

Cautions that would apply include the ever present risk of unusual infection and the potential for steroids to increase the risk of renal crisis in patients with systemic sclerosis.

Lung transplantation is a consideration for certain non responding patients

Form the article:

In these cases, lung transplantation may be the only remaining treatment option. This is particularly true for patients presenting with IPF, and it is 1 of the most common indications for lung transplantation. Patients with respiratory failure and ILD should be evaluated early in the hospital course for transplantation or considered for transfer to a transplant center. General contraindications to transplant are age older than 70 years, underweight or elevated BMI (generally higher than 30), malignancy within the last 2 years (with the exception of cutaneous squamous and basal cell tumors), untreatable major organ dysfunction other than the lung, noncurable chronic extrapulmonary infection (chronic active viral hepatitis B, hepatitis C, human immunodeficiency virus), significant chest wall deformity, untreatable psychiatric or psychologic disease, substance addiction within the last 6 months, or lack of dependable social support.4

Another excellent review, free full text, is here. Though a couple of years old it is still relevant and has lots of pearls.

Trends in ICU care, 2009-2013

From a recent study in Chest:

Background

Longitudinal analyses of large, detailed adult critical care data

sets provide insights into practice trends and generate useful outcome and process benchmarks.

Methods

Data representing 991,571 consecutive critical care visits to 160 US adult ICUs from 2009 to 2013 from the eICU Research Institute clinical practice database were used to quantitate patient characteristics, APACHE IV–based acuity predictions, treatments, and outcomes. Analyses for changes over time were performed for patient characteristics, entry and discharge locations, primary admission diagnosis, treatments, adherence to consensus ICU best practices, length of stay (LOS), and inpatient mortality.

Results

We detected significant trends for increasing age, BMI, and risk of mortality, higher frequency of admission from an ED and stepdown unit, and more frequent hospital discharge to substance abuse centers and skilled nursing facilities. Significantly more patients were admitted for sepsis, emphysema, coma, congestive heart failure, diabetic ketoacidosis, and fewer were admitted for asthma, unspecified chest pain, coronary artery bypass graft, and stroke care. The frequency of noninvasive mechanical ventilation and adherence to critical care best practices significantly increased, whereas the duration of renal replacement therapies, frequency of transfusions, antimicrobial use, critical care complications, LOS, and inpatient mortality decreased.

Conclusions

Analyses of patients, practices, and outcomes from a large geographically dispersed sample of adult ICUs revealed trends of increasing age and acuity, higher rates of adherence to best practice, use of noninvasive mechanical ventilation, and decreased use of antimicrobials, transfusions, and duration of renal replacement therapies. Acuity-adjusted LOS and in hospital mortality decreased.

Hypertrophic cardiomyopathy

Here’s a good review, with a focus on HCM of the elderly.

Intravascular hemolysis before and after TAVR

Interesting, form the American Journal of Cardiology:

Intravascular hemolysis (IVH) has been identified in patients with surgical prosthetic valves, but few have been reported after transcatheter aortic valve implantation (TAVI). We conducted a prospective analysis of 64 TAVI patients. The hemolysis profiles were collected at baseline and 6 months after TAVI. The echocardiography was performed at baseline and 6 months after TAVI. There are 14 patients (21.9%) with IVH before and 24(37.5%) after TAVI. The serum haptoglobin values before and 6 months after TAVI are 126.7 ± 75.1 vs 86.3 ± 57.1 mg/dl (p less than 0.001). More greater than or equal to moderate paravalvular leakage (PVL) (50% vs 7.5%, p less than 0.001), bicuspid aortic valve (BAV) (33.3% vs 5.0%, p = 0.004), use of 23 mm prosthesis (29.2% vs 7.5%, p = 0.03), higher residual valvular pressure gradient (17.9 ± 6.8 mm Hg vs 14.7 ± 5.7 mm Hg, p = 0.05), and lower effective orifice area index (1.05 ± 0.21 vs 1.21 ± 0.29, p = 0.03) were observed in patients with post-TAVI IVH. On multivariate regression analysis, BAV and greater than or equal to moderate PVL are independently related to post-TAVI IVH. With log-rank test, 1-year rates of readmission due to cardiovascular cause were significantly higher in patients with post-TAVI IVH (odds ratio 4.5; 95% confidence interval 1.3 to 15.6; p = 0.02), after adjusting age and gender. In conclusion, greater than or equal to moderate PVL and BAV are predictors of post-TAVI IVH, which is associated with increased cardiovascular readmission in 1-year follow-up.

Cardiorespiratory fitness and the risk for atrial fibrillation, stroke and all cause mortality

From a recent study:

Benefits of cardiorespiratory fitness on cardiovascular health are well recognized, but the impact on incidence of atrial fibrillation (AF) and stroke, and, particularly, risk of stroke and mortality in patients with AF is less clear. From 1993 to 2010, patients referred for a treadmill exercise test (TMET) at the Mayo Clinic Rochester, MN, were retrospectively identified (N = 76,857). From this, 14,094 local residents were selected. Exclusions were age less than 18 years; history of heart failure, structural or valvular heart disease, AF or flutter, or stroke. Subjects were divided into 4 groups at baseline based on quartiles of functional aerobic capacity (FAC) and followed through January 2016. The final study cohort included 12,043 patients. During median follow-up of 14 (9 to 17) years, 1,222 patients developed incident AF, 1,128 developed stroke, and 1,590 patients died. Each 10% increase in FAC was associated with decreased risk of incident AF, stroke, and mortality by 7% (0.93 [0.91 to 0.96, p less than 0.001]), 8% (0.92 [0.89 to 0.94, p less than 0.001]), and 16% (0.84 [0.82 to 0.86, p less than 0.001]), respectively. In patients who developed incident AF with baseline FAC less than 75% versus greater than or equal to 105%, risks of both stroke (1.40 [1.04 to 1.90, p = 0.01]) and mortality (3.20 [2.11 to 4.58, p less than 0.001]) were significantly higher. In conclusion, better cardiorespiratory fitness is associated with lower risk of incident AF, stroke, and mortality. Similarly, risk of stroke and mortality in patients with AF is also inversely associated with cardiorespiratory fitness.

Eating speed is a risk factor for metabolic syndrome

From the AHA meeting abstracts:

Results: During the 5-year follow-up, 84 people were diagnosed with metabolic syndrome. The incidence rates of metabolic syndrome among slow, normal and fast-eating participants were 2.3, 6.5 and 11.6%, respectively. The multivariate-adjusted hazard ratio for incidence of metabolic syndrome in the fast-eating group compared to the normal and slow group was 1.89 (95% confidence interval [CI], 1.21-2.98, p less than 0.05), 5.49 (95% confidence interval [CI], 1.30-23.3, p less than 0.05). Eating speed was significantly correlated with weight gain, triglyceride (TG) and high-density lipoprotein cholesterol (HDL-C) components of metabolic risk factors. Multivariable logistic analysis revealed that weight gain and TG and HDL-C was significantly associated with Mets cumulative incidence (OR 3.59: 95% CI: 2.12-6.09, p less than 0.001, OR 1.003: 95% CI: 1.001-1.004, p less than 0.001, OR 0.96: 95% CI:0.935-0.980, p less than 0.005).

Conclusions: Eating speed was associated with obesity and future prevalence of Metabolic syndrome. Eating slowly may therefore indicated to be a crucial lifestyle factor for preventing metabolic syndrome among the Japanese.

Excessive supraventricular ectopy in the elderly associated with lower cognition, and only partly explained by stroke incidence

From an abstract presentation at AHA:

Abstract

Introduction: Supraventricular ectopic beats (SVE) are common and considered benign. However, excessive supraventricular ectopy activity (ESVEA) has been associated with incident atrial fibrillation (AF) and stroke. While AF has been associated with lower cognitive function independent of stroke, the association between ESVEA and cognitive function has not yet been characterized.

Hypothesis: ESVEA would be cross-sectionally associated with lower cognitive test scores.

Methods: We included N=1,116 ARIC study participants [age mean±SD 80±5 years, 55% female, 23% non-white race] who underwent cognitive testing and wore a leadless, ambulatory ECG-monitoring device (Zio®Patch by iRhythm Technologies Inc) for greater than or equal to 2 days between 6/2016-2/2017. Cognitive domain-specific factor scores for memory, executive function and language were estimated using standardized z-scores. SVE burden was calculated as mean number per day and ESVEA was defined as greater than or equal to 75th percentile of mean number per day. Multiple linear regression was used to evaluate the association between ESVEA and cognitive test scores.

Results: During a mean recording time of 12.8±2.4 days, 95% of participants had greater than or equal to 1 SVE, and a median 205 SVE/day (IQR=66-823). ESVEA was associated with lower executive function z-scores by 0.19 (95% CI: -0.37, -0.02) after adjustment for demographics (Table). After multivariable adjustment, this association was slightly attenuated [-0.16 (-0.33, 0.01)]. ESVEA was not associated with memory or language scores. In sensitivity analysis, the ESVEA-executive function association was weakened but followed a similar pattern after excluding those with AF and/or stroke [Model 1: -0.17 (-0.35, 0.01); Model 2: -0.13 (-0.31, 0.04)].

Conclusions: In this elderly population, ESVEA was associated with lower executive function and this association was only partly explained by AF or stroke prevalence.

What’s wrong with leaders in health care?

Dr. Suneel Dhand says it’s the fact that they’re non-physicians.

That’s partially true. There are docs in health care leadership but their problem is a shift away from the attitude of a clinician: a passion for doing your best for one patient at a time.

Serving up the kool-aid

Hospitalists as the leaders in a global shift in health care?

Whatever happened to the original idea of the hospitalist as a expert clinician for the care of the individual patient?

Eosinophilic granulomatosis with polyangiitis (EGPA) aka Churg-Strauss syndrome: clinical presentations

This small case series contains a number of pearls. The typical presentation was that of neuropathic symptoms, usually in the lower extremity (mononeuritis and usually multiplex) in a patient with a history of asthma for years. Most had a rash resembling Janeway lesions. Consistent with what we have traditionally been taught, only about half had a positive ANCA, mainly P. Think of it if your asthmatic patient inexplicably begins complaining of foot pain or numbness.

Update on diffuse cystic lung diseases and spontaneous pneumothorax as a presenting manifestation

From a recent review:

Purpose of review

Diffuse cystic lung diseases (DCLDs) are a heterogeneous group of disorders with varying pathophysiologic mechanisms that are characterized by the presence of air-filled lung cysts. These cysts are prone to rupture, leading to the development of recurrent spontaneous pneumothoraces. In this article, we review the epidemiology, clinical features, and management DCLD-associated spontaneous pneumothorax, with a focus on lymphangioleiomyomatosis, Birt–Hogg–Dubé syndrome, and pulmonary Langerhans cell histiocytosis.

Recent findings

DCLDs are responsible for approximately 10% of apparent primary spontaneous pneumothoraces. Computed tomography screening for DCLDs (Birt–Hogg–Dubé syndrome, lymphangioleiomyomatosis, and pulmonary Langerhans cell histiocytosis) following the first spontaneous pneumothorax has recently been shown to be cost-effective and can help facilitate early diagnosis of the underlying disorders. Patients with DCLD-associated spontaneous pneumothorax have a very high rate of recurrence, and thus pleurodesis should be considered following the first episode of spontaneous pneumothorax in these patients, rather than waiting for a recurrent episode. Prior pleurodesis is not a contraindication to future lung transplant.

Summary

Although DCLDs are uncommon, spontaneous pneumothorax is often the sentinel event that provides an opportunity for diagnosis. By understanding the burden and implications of pneumothoraces in DCLDs, clinicians can facilitate early diagnosis and appropriate management of the underlying disorders.

Is this quality?

A recent Medscape piece is titled In Search of a Better Way to Measure Quality Primary Care. I agree with the premise of the article but would strike the word “better.” No one has found a way to measure quality at all. Quality is mentioned multiple times throughout the article but I would call this what it really is: performance. Performance is a very poor surrogate for quality. From the article:

Although the intentions are good, the results of these measures are less clear. Measuring and reporting quality, as currently accomplished, has effects and consequences that negatively influence quality of care, patient outcomes, and clinician job satisfaction.[2]

Note the confusion around the notion of quality. In the quote above there is one inappropriate and one appropriate use of the term. The footnote was in reference to this JAMA paper. From that paper:

Despite these plausible mechanisms of quality improvement, the value of publicly reporting quality information is largely undemonstrated and public reporting may have unintended and negative consequences on health care. These unintended consequences include causing physicians to avoid sick patients in an attempt to improve their quality ranking, encouraging physicians to achieve “target rates” for health care interventions even when it may be inappropriate among some patients, and discounting patient preferences and clinical judgment. Public reporting of quality information promotes a spirit of openness that may be valuable for enhancing trust of the health professions, but its ability to improve health remains undemonstrated, and public reporting may inadvertently reduce, rather than improve, quality.

Again, somewhat confusing use of the word but we get the idea. That was in 2005. I’ve seen nothing in the way of fundamental change since then that would undermine the premise of that article. There’s a lot to unpack from the text of the JAMA article quoted above. Avoiding sick patients is a way to get around not just process, but also outcome reports. Outcome reporting has been considered as a way to get around the pervasive gaming of process report cards. However, there are ways to game outcome metrics as well as processes metrics. Finally, the JAMA piece correctly points out that artificial incentives to adhere to metric targets discount the judgment of the clinician and the preferences of the patient, two of the three key elements of evidence based medicine. Thus these incentives actually oppose rather than promote EBM.

Update on cystic fibrosis related liver disease

From a recent review:

Recent findings

The variable pathophysiology of CFLD complicates its diagnosis and treatment. A ‘gold standard’ for CFLD diagnosis is lacking. Over the past years, new techniques to diagnose features of CFLD, such as transient elastography, have been investigated. Although most of these tests confirm cystic fibrosis-related liver involvement (CFLI), they are, however, not suitable to distinguish various phenotypical presentations or predict progression to clinically relevant cirrhosis or portal hypertension. A combined initiative from the European and the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition has been started, aimed to obtain consensus on CFLD criteria and definitions. Currently, only ursodeoxycholic acid is used in CFLD treatment, although it has not been convincingly demonstrated to change the natural course of the disease. Drugs that directly target cystic fibrosis transmembrane conductance regulator protein dysfunction show promising results; however, more long-term follow-up and validation studies are needed.

Summary

CFLD is an umbrella term referring to a wide variety of liver manifestations with variable clinical needs and consequences. CFLD with portal hypertension is the most severe form of CFLD due to its significant implications on morbidity and mortality. The clinical relevance of other CFLI is uncertain. Consensus on CFLD definitions is essential to validate new diagnostic tools and therapeutic outcome measures.

Hyponatremia in heart failure

In this study most heart failure patients with hyponatremia on admission were discharged hyponatremic. Among treatment approaches fluid restriction was most common. No deliberate attempt to lower serum sodium ranked second. Despite heavy promotion hardly anybody used tolvaptan.

From the Clinical Perspective portion of the paper:

Clinical Perspective

What Is New?

Hyponatremia (HN) is common in patients hospitalized with acute heart failure and is associated with worse outcomes.

We examined current practices for the management of HN in 762 patients with acute heart failure.

Fluid restriction was the most commonly used strategy for correcting HN; however, nearly one quarter received no specific therapy.

What Are the Clinical Implications?

Most patients with HN remained hyponatremic at discharge.

Further studies are needed to determine optimal approaches to effectively correct HN the inpatient setting.

The last sentence above reflects the wrong perspective. Hyponatremia is indeed a predictor of worse outcomes but not as a result of the sodium concentration itself. Rather, it is because it is a marker of neurohumeral activation. Antagonizing the neurohumeral response has been the focus of treatment of systolic heart failure for decades. One class of neurohumeral antagonists, the aldostone receptor antagonists, has the effect of lowering serum sodium.

Treating HIT? Go off label!

Fondaparinux is associated with better HIT outcomes when compared to the approved anticoagulants.

The quality of discharge summaries at two Michigan hospitals

From a recent report in BMJ Quality and Safety:

RESULTS:

Clinical summaries averaged six pages (range 3-12). Several content elements were universally auto-populated into clinical summaries (eg, medication lists); others were not (eg, care team). Eighty-five per cent of clinical summaries contained discharge instructions, more often generated from third-party sources than manually entered by clinicians. Clinical summaries contained an average of 14 unique messages, including non-clinical elements irrelevant to postdischarge care. Medication list organisation reflected reconciliation mandates, and dosing charts, when present, did not carry column headings over to subsequent pages. Summaries were written at the 8th-12th grade reading level and scored poorly on assessments of understandability and actionability.

Not good.