Systemic lupus and catastrophic antiphospholipid syndrome manifesting as cardiogenic shock.

In this case series we present two young female patients presenting in the peripartum period with cardiogenic shock. Both patients had underlying autoimmune diseases, one with systemic lupus erythematosus (SLE) and the other with antiphospholipid syndrome (APS). In both cases cardiogenic shock was a direct manifestation of their autoimmune condition, and with prompt diagnosis and management both patients were able to recover. This case series illustrates the importance of early recognition of cardiogenic shock as a rare manifestation both of SLE and APS.

Post-discharge rise in BNP and rehospitalization for heart failure.

BACKGROUND: The B­type natriuretic peptide (BNP) level on discharge of patients hospitalized with decompensated heart failure (HF) is widely considered as the "baseline" value, and treatment should be targeted to maintain this level. The prognostic value of an increase in BNP level from discharge to the 1­month follow-up in predicting rehospitalization has not been previously explored. METHODS: The Evaluation Study of Congestive Heart Failure and Pulmonary Artery Catheterization Effectiveness (ESCAPE) trial data were utilized to determine whether an increase in BNP level from discharge to the 1­month follow-up is associated with a higher risk of rehospitalization. The study endpoints were all-cause rehospitalization up to 6 months following randomization. RESULTS: Among 44 patients (mean age, 56 years, 71% men) who had their BNP levels checked on discharge and at the 1­month follow-up, the average BNP level on discharge of the whole cohort was 467 pg/ml, which increased to 919 pg/ml at 1 month (p = 0.001). The median and interquartile range of the magnitude of rise in BNP level from discharge to 1­month follow-up was higher in rehospitalized compared with non-rehospitalized patients (329 [11, 956] vs. 44 [-90, 316] pg/ml, p = 0.039, in both groups, respectively). Receiver operator characteristic curves showed that the magnitude of the rise in BNP from discharge to the 1­month follow-up had an area under the curve of 0.686 (p = 0.0255) in predicting all-cause rehospitalization. Rehospitalized and non-rehospitalized patients had similar degree of clinical congestion and comparable BNP level on hospital discharge. CONCLUSION: The magnitude of the rise in BNP level from discharge to the 1­month follow-up is a useful prognostic factor that predicts rehospitalization in patients with HF.

Underutilization of Donor Hearts: An Observational Study.

BACKGROUND: Utilization of donor hearts remains insufficient. In this observational study, we explored the rate and reasons of rejection of cardiac donors in 1 organ procurement organization. METHODS: Donors were enrolled in the study for 1 year, from October 5, 2014, through October 4, 2015. Data on demographics, medical history, and diagnostic tests were collected. We compared continuous variables between groups using a Mann-Whitney U test, and categorical variables using the χ2test. Multivariate logistic regression analysis was performed to identify factors predicting transplantation. RESULTS: Of 134 adult hearts, only 39.5% were transplanted. Moreover, almost half (46.9%) of non-transplanted hearts were normal by all data available. In 12 (31.5%) of all hearts, coronary artery disease was discovered by pathology, making them unusable for transplantation. Overall, 26 normal hearts (19.4%) were not accepted for transplantation. The most common abnormality of hearts not suitable for transplantation was a decreased left ventricular ejection fraction (LVEF). In about one-fifth of donors with low LVEF on the first echocardiogram, LVEF improved on a second echocardiogram. In a majority of cases with low LVEF, echocardiogram was never repeated. CONCLUSIONS: Almost 20% of normal donor hearts were not transplanted. Coronary artery disease was the most common abnormality in seemingly normal hearts. Coronary angiography should be performed more liberally in potential donors. Decreased LVEF was the most common reason for not using a heart. Since impaired LVEF may be reversible, repeated echocardiogram is encouraged in order to maximize the rate of transplantation.

Admission-to-discharge temperature reduction in decompensated heart failure is associated with rehospitalization.

INTRODUCTION: Decreasing body temperature on first follow-up visit-relative to discharge-predicts early rehospitalization in heart failure (HF). We studied whether admission-to-discharge temperature reduction was associated with increased HF rehospitalization in the ESCAPE trial. METHODS: We compared patients with or without ≥1 °C decrease in temperature from admission-to-discharge. The study endpoint was rehospitalization due to HF for up to 6 months after discharge. RESULTS: Among 354 patients (average age 57 years, 73% men) with recorded admission and discharge temperature, 22 (6.2%) had an admission-to-discharge temperature reduction ≥1 ºC. Patients with admission-to-discharge temperature reduction ≥1 ºC had higher frequency of rehospitalization for HF (68.2% vs. 44.3%, estimated odds ratio [OR] 2.697, 95% confidence interval [CI] 1.072-6.787, P = 0.029) despite a significantly higher admission temperature. On multivariate analysis, admission-to-discharge temperature reduction ≥1 ºC predicted rehospitalization for HF (OR 2.02, 95% CI 1.028-3.966, P = 0.041) after adjustment for age, BMI, baseline Na, creatinine, ejection fraction and discharge NYHA class. A standard logistic model treating temperature change as a continuous variable, and a model using a restricted cubic spline, did not demonstrate a statistically significant relationship between temperature reduction and HF rehospitalization. Subsequently, an altered logistic model was fit expressing the log odds of HF rehospitalization as a piecewise linear function of temperature decrease; this model did demonstrate statistical significance (P = 0.013) with an estimated odds ratio of 1.140 per 0.1 ºC beyond 0.5 ºC. CONCLUSION: Admission-to-discharge temperature reduction ≥1 ºC is an unfavorable prognostic sign associated with future rehospitalization due to HF.

Longer-than-average length of stay in acute heart failure : Determinants and outcomes.

BACKGROUND: Increased length of stay (LOS) during acute heart failure (HF) hospitalization is associated with readmission and mortality. METHODS: The ESCAPE trial data were utilized to identify determinants and post-discharge outcomes of patients with acute systolic HF requiring longer-than-average LOS (≥7 days). The study endpoints were 6­month all-cause mortality, all-cause rehospitalization, and the composite endpoint of death, cardiac rehospitalization, and cardiac transplant. RESULTS: Among the 424 patients with recorded LOS, 216 (50.9%) and 208 (49.1%) had LOS ≥ or <7 days, respectively. Independent determinants of longer-than-average LOS included older age (OR per 10-year increase: 1.759, 95% CI: 1.120-2.763, p = 0.014), higher blood urea nitrogen (OR per 5 mg/dl increase: 1.202, 95% CI: 1.024-1.410, p = 0.024), greater inferior vena cava diameter (OR per 1 cm increase: 2.453, 95% CI: 1.175-5.121, p = 0.017), and lower sodium (OR per 4 mmol/l increase: 0.494, 95% CI: 0.268-0.911, p = 0.024). We found a significant correlation between right-sided failure (right atrial pressure) and LOS (r = 0.229, p = 0.001) but not left-sided failure (pulmonary capillary wedge pressure, r = 0.099, p = 0.177). Patients with longer-than-average LOS had a significantly higher mortality (25.9% vs. 12%, univariate OR: 2.562, 95% CI: 1.528-4.296, p < 0.001), higher all-cause rehospitalization (63% vs. 53.4%, univariate OR: 1.486, 95% CI: 1.008-2.190, p = 0.046) and higher frequency of the composite endpoint of death, cardiac rehospitalization, and cardiac transplant (61.6% vs. 45.2%, univariate OR: 1.943, 95% CI: 1.320-2.862, p = 0.001) compared with an LOS of <7 days. Cox proportional hazard analysis showed that a longer-than-average LOS was an independent predictor of 6­month all-cause mortality (HR: 1.930, 95% CI: 1.112-3.350, p = 0.019). CONCLUSION: In acute HF, right ventricular failure and renal dysfunction predict longer-than-average LOS, which is a proxy for more severe HF and is associated with worse postdischarge outcomes.

Extent of jugular venous distension and lower extremity edema are the best tools from history and physical examination to identify heart failure exacerbation.

INTRODUCTION: We aimed to identify the best tools from history and physical examination that predict severity of heart failure (HF) exacerbation among patients with an ejection fraction (EF) ≤ 30%. METHODS: Patients enrolled in the ESCAPE trial were divided into tertiles according to the combined value of pulmonary capillary wedge pressure (PCWP) and right atrial pressure (RAP) which we used as a marker of volume loading of both pulmonary and systemic compartments. Variables of congestion from history and physical examination were examined across tertiles. RESULTS: There were significant differences across tertiles (tertile 1: PCWP + RAP < 31 mm Hg, tertile 2: PCWP + RAP 31-42 mm Hg and tertile 3: PCWP + RAP > 42 mm Hg) with respect to baseline B­type natriuretic peptide (P = 0.016), blood urea nitrogen (P = 0.022), sodium (P = 0.015), left ventricular ejection fraction (P = 0.005), and inferior vena cava diameter during inspiration (P < 0.001) and expiration (P < 0.001). With respect to variables of congestion from history and physical examination, we found significant differences across tertiles predominantly in signs of right sided failure, specifically, the frequency of jugular venous distension (JVD, P < 0.001) and JVD > 12 cmH2O (p < 0.001), lower extremity edema (P = 0.001) and lower extremity edema of at least grade 2 + (P = 0.029), and positive hepatojugular reflux (HJR, P = 0.022) but no differences in patients' symptoms such as degree of dyspnea, orthopnea or fatigue. With regards to post-discharge outcomes, there was a significant difference across tertiles in all-cause mortality (P = 0.029) and rehospitalization for HF (P = 0.031) at 6 months following randomization. Receiver operator characteristic curves showed that admission PCWP + RAP had an area under the curve of 0.623 (P = 0.0075) and 0.617 (P = 0.0048), respectively, in predicting 6­month all-cause mortality and rehospitalization for HF. CONCLUSION: The presence and extent of JVD and lower extremity edema, and a positive HJR are better than other signs and symptoms in identifying severity of HF exacerbation among patients with EF ≤ 30%.

Successful Liver Transplantation From a Donor With a Continuous-Flow Left Ventricular Assist Device for 9 Months.

Organ donor criteria continue to be extended in an attempt to meet growing demands. Patients with continuous-flow left ventricular assist devices are one group of potential donors being considered. One concern with this group is the effect of continuous flow for a prolonged duration, as opposed to normal pulsatile flow, on end-organ function. We report the 1st case of a liver transplantation from a donor who had a continuous-flow left ventricular assist device for 9 months. The recipient was a 69-year-old woman with a history of cryptogenic cirrhosis and hepatocellular carcinoma. The transplantation was complicated by moderate acute cellular rejection as well as biliary obstruction requiring sphincterotomy and stent placement. After management of those complications, the patient's liver function tests returned to normal values and remained stable at her 6-month post-transplantation follow-up. This case shows that organ transplantation from a donor with a continuous-flow left ventricular assist device for a prolonged period can be performed successfully.

Rise in BNP despite appropriate acute decompensated heart failure treatment : Patient characteristics and outcomes.

INTRODUCTION: B-type natriuretic peptide (BNP) tends to decrease in response to successful treatment of decompensated heart failure (HF). We identified characteristics and outcomes of patients whose BNP levels rise during admission despite appropriate decongestive therapy. METHODS: Patients enrolled in the ESCAPE trial admitted with acute systolic HF were divided into two groups according to whether or not BNP increased during the period from admission (T0) to discharge (T1). The study endpoint was 6­month all-cause mortality. RESULTS: Of 245 patients (age 56 years, 75 % men) admitted with acute systolic HF, 67 (27.3 %) had a higher BNP at T1 relative to T0. Despite similar degrees of congestion at T0, patients with BNP rise at T1 had less degree of decongestion from T0 to T1 as evident in the lower frequency of patients who had resolution of jugular venous distension (39.7 vs. 59.5 %, P = 0.01) and orthopnea (32.2 vs. 48.8 %, P = 0.029) at T1, in addition to lower reduction in IVC diameter during inspiration (P = 0.001) and expiration (P = 0.002) and less weight loss (P = 0.04). Patients with BNP rise at T1 were more likely to die (29.9 vs. 15.7 %, univariate OR 2.28, 95 % CI 1.177-4.414, P = 0.015) despite a lower BNP at T0 (492 vs. 1260 pg/ml, P < 0.001). Cox proportional hazard analysis revealed that a higher BNP at T1 independently predicts 6­month mortality (hazard ratio 1.95, 95 % CI 1.067-3.578, P = 0.03) after adjustment for age, sodium, creatinine, and NYHA class-all at discharge. Kaplan-Meier analysis comparing survival in patients with or without BNP rise on discharge showed a significant intergroup difference (log-rank P value = 0.017). CONCLUSION: Higher BNP levels on discharge identifies a subset of patients with lower degree of decongestion from T0 to T1 and higher 6­month mortality.

Retrobulbar Hematoma After Heart Transplantation: Case Report and Literature Review.

Spontaneous retrobulbar hemorrhage is a rare phenomenon observed after surgery not directly involving the orbit. When it occurs, presentation is usually obvious, and it carries high morbidity unless emergent and timely surgical treatment is instituted. There are only a handful of cases associated with cardiac surgery, and to our knowledge no cases with heart transplantation. We present a case of a 35-year-old woman who underwent orthotopic heart transplantation for peripartum cardiomyopathy and developed spontaneous retrobulbar hemorrhage.

Treatment options for patients with mobile left ventricular thrombus and ventricular dysfunction: a case series.

INTRODUCTION: Little is known about the optimal management of large, mobile, pedunculated left ventricular clots. The management is particularly challenging in patients with advanced heart failure considered for left ventricular assist device implantation, because the clot may cause pump thrombosis. METHODS: We retrospectively reviewed the records of patients with left ventricular thrombi identified by echocardiography, and found three cases with large protruding mobile clots. RESULTS: In this paper, we are presenting three challenging cases where the clots were successfully treated surgically. In two cases, the removal of clot was performed simultaneously with the implantation of ventricular assist devices. In the third case, the patient underwent only thrombectomy. Overall, the early outcomes were good in all three patients, but one subsequently died from unrelated causes. CONCLUSIONS: These clinical cases give evidence for surgical treatment of large mobile clots without systemic embolism, even if ventricular assist device is implanted during the same operation.

How much atrial fibrillation causes symptoms of heart failure?

INTRODUCTION: Patients with atrial fibrillation (AF) are more symptomatic than patients with sinus rhythm. However, it is unknown what per cent of time spent in AF is associated with symptoms. METHODS: We used a limited access dataset from the Atrial Fibrillation Follow-up Investigation of Rhythm Management trial. Patients had their current rhythm and New York Heart Association (NYHA) class recorded at baseline and at every follow-up visit. The ratio of number of visits when patients were in AF to the total number of visits was used as a surrogate measure of AF burden. The median number of visits was 12 per patient. We grouped patients labelled as class 0 and I by NYHA as having no symptoms and NYHA II or III as having symptoms. Furthermore, we calculated mortality and the prevalence of symptoms depending on the per cent of visits when they had AF. RESULTS: Of 4060 patients enrolled in the trial, 74 had no follow-up visits and were excluded; the remaining 3986 patients were analysed. Patients who had no or little AF throughout the study (0-20%) had the lowest prevalence of symptoms. Prevalence of symptoms increased with greater per cent of time spent in AF. Specifically, symptoms became more prevalent when AF burden reached 20-40%. Mortality was similar regardless of proportion of visits when patients were in AF. CONCLUSIONS: Higher AF burden is associated with higher prevalence of symptoms. The increment became significant when patients were in AF at 20-40% of visits.

Outcomes of adult orthotopic heart transplantation with extended allograft ischemic time.

BACKGROUND: Heart procurement for orthotopic heart transplant (OHT) is limited by the conventional 4 hours of ischemic time (IT). Based on a recent report from our center showing that extended IT from a young donor group is safe, we widened our geographical reach, resulting in almost 40% of our transplants having an IT > 4 hours. METHODS: We retrospectively reviewed records of adult patients who underwent OHT from January 2006 to December 2011. The primary outcome was survival, and secondary outcomes included resource utilization, end-organ dysfunction, and acute cellular rejection. Overall survival was analyzed using Kaplan-Meier curves and log-rank tests. Secondary outcomes were compared with a combination of parametric and nonparametric statistics. RESULTS: A total of 323 patients underwent OHT. There was a significant difference in overall survival between the standard and extended IT groups (85.7% vs 76.4%, P = .03). There were no significant differences between the groups for secondary outcomes except a higher incidence of liver dysfunction in the extended IT group (84.9% vs 73%, P = .01). Further analysis revealed that mortality remains similar if IT is below 4 hours and between 4 and 5 hours, but begins to climb after 5 hours, driving the difference between our standard and extended IT. CONCLUSIONS: Limited donor availability for OHT dictates alternative strategies to enlarge the donor pool. Although there is an overall increasing risk with extended IT beyond 4 hours, it may be possible to safely increase the threshold to at least 5 hours without compromising the outcomes.

Symptoms in heart failure correlate poorly with objective haemodynamic parameters.

INTRODUCTION: Even though heart failure (HF) is a very common condition, surprisingly little is known regarding association between patient's symptoms and objective data. The purpose of this study was to evaluate for any correlations between haemodynamic, echocardiographic and laboratory data of presenting symptoms in HF patients. METHODS: This study is a retrospective analysis of the limited access dataset from the Evaluation Study of Congestive Heart Failure and Pulmonary Artery Catheterization Effectiveness (ESCAPE) trial provided by the National Heart, Lung and Blood Institute. Symptoms including dyspnoea, orthopnoea, fatigue and gastrointestinal (GI) discomfort were graded by their severity from minimal (0) to maximal (3) on admission, at discharge, at 3 months and at 6 months from the admission. Results of Minnesota Living with Heart Failure (MLHF) score and assigned New York Heart Association (NYHA) functional class were available at the same time points. RESULTS: A total of 433 patients with decompensated HF and decreased systolic function (ejection fraction < 30%) were included in this trial. Orthopnoea, dyspnoea and fatigue had weak correlation with invasive pulmonary artery systolic and diastolic pressure and negative correlation with serum creatinine, albumin, sodium, total bilirubin, haemoglobin and haematocrit; fatigue showed positive correlation to pulmonary artery pressures. Abdominal discomfort had no correlation to symptoms. There was no correlation of symptoms, NYHA class, or MLHF scores with age, gender, peak VO(2) on cardiopulmonary stress test, body mass index, either right or left ventricular systolic function, B-type natriuretic peptide, cardiac output or cardiac index, troponin level, velocity of tricuspid regurgitation and multiple other factors predicting morbidity and mortality in HF. CONCLUSION: Overall, the correlation between symptoms and objective parameters was weak. Because of low magnitude of relationship between symptoms to objective parameters, it was concluded that there are likely other factors determining the perception of symptoms in HF patients.

Understanding hypertrophic cardiomyopathy: implications of diagnosis for the patient and family.

Hypertrophic cardiomyopathy (HCM) may be a challenging condition for an internist. The algorithm for work-up and treatment is fairly straight forward when the presentation is classic. However, in the real world, subtle and moderate forms of the disease occur more commonly. We analyse, step by step, diagnosis and management of a patient with mild HCM and review the literature on pertinent questions regarding diagnosis, risk stratification, treatment options and implications for patient's lifestyle and for his family.

Pulmonary edema after transfusion in a patient with end-stage renal disease.

AIMS: To describe a patient with end-stage renal disease who developed non-cardiogenic pulmonary edema after transfusion of packed red blood cells. DESIGN: Case report and literature review. RESULTS: The patient under consideration is a 60-year-old woman who developed acute pulmonary edema after transfusion of packed red blood cells without concomitant dialysis. The initial diagnosis of fluid overload was managed by isolated ultrafiltration. Minimal fluid removal led to significant hypotension that was resistant to vasopressors. Subsequent pulmonary artery catheter readings were consistent with non-cardiogenic pulmonary edema. The patient improved spontaneously over the next few days with supportive care only. Plasma from the donors was checked for granulocyte antibodies and antibodies to HLA class I antigens. No granulocyte antibodies were detected in donor plasma but of one the HLA antibodies detected in donor plasma had specificity for a recipient HLA-A antigen. These characteristics supported a final diagnosis of transfusion-related acute lung injury (TRALI). CONCLUSIONS: Acute pulmonary edema following blood transfusion in a dialysis-dependent patient does not always signify fluid overload and nephrologists should be aware of the alternative diagnosis of TRALI. Proper awareness of TRALI will lead to prompt diagnosis and appropriate management.