Bland-White-Garland syndrome and atrial septal defect—: Rare Association and diagnostic challenge

Summary: We report on a 40-year-old woman referred for evaluation of a cardiac murmur and dyspnea on exertion. The electrocardiogram (ECG) showed incomplete right bundle branch block, and echocardiography revealed a large atrial septal defect (ASD, ostium secundum type) with dilated right-sided heart chambers. At cardiac catheterization, a large left-to-right shunt (78% of the pulmonary blood flow) was found, and surprisingly, the additional diagnosis of anomalous origin of the left coronary artery from pulmonary artery (ALCAPA) was established. After ASD closure and left coronary artery ligation with implantation of a vein graft to the left anterior descending artery, she had an uneventful 18-years follow-up. We discuss the interaction of the two associated conditions, and based on the herein reported unusual combination, we highlight typical features of non-invasive examinations including auscultation, ECG, and echocardiography in adult patients with ALCAP

Influence of drugs and comorbidity on serum potassium in 15 000 consecutive hospital admissions

Background. Drug trials often exclude subjects with relevant comorbidity or comedication. Nevertheless, after approval, these drugs will be prescribed to a much broader collective. Our goal was to quantify the impact of drugs and comorbidity on serum potassium in unselected patients admitted to the hospital. Methods. This was a retrospective pharmacoepidemiologic study in 15 000 consecutive patients admitted to the medical department of the Kantonsspital St. Gallen, a 700-bed tertiary hospital in eastern Switzerland. Patients with ‘haemolytic' plasma and patients on dialysis or with an estimated glomerular filtration rate (GFR) <10 mL/min/1.73 m2 were excluded. For the remaining 14 146 patients, drug history on admission, age, sex, body weight, physical findings, comorbidity (ICD-10 diagnoses) and laboratory information (potassium and creatinine) were extracted from electronic sources. Results. Estimated GFR was the strongest predictor of serum potassium (P < 0.0001). Angiotensin-converting enzyme inhibitors, cyclosporine, loop diuretics and potassium-sparing diuretics all showed a significant effect modification with decreasing GFR (P < 0.001). Similarly, in patients with liver cirrhosis a significantly stronger effect on potassium was found for angiotensin receptor blockers, betablockers and loop diuretics (P < 0.01). Several significant drug-drug interactions were identified. Diabetes, male sex, older age, lower blood pressure and higher body weight were all independently associated with higher serum potassium levels (P < 0.001). The model explained 14% of the variation of serum potassium. Conclusions. The effects of various drugs on serum potassium are highly influenced by comorbidity and comedication. Although the presented model cannot be used to predict potassium in individual patients, we demonstrate that clinical databases could evolve as a powerful tool for industry-independent analysis of postmarketing drug safet

N-terminal pro-B-type natriuretic peptide and functional capacity in patients with obstructive sleep apnea

The obstructive sleep apnea syndrome (OSAS) is associated with cardiovascular abnormalities including left ventricular hypertrophy, left ventricular diastolic dysfunction, and endothelial dysfunction. The present study evaluated whether N-terminal pro-B-type natriuretic peptide (NT-proBNP) and peak oxygen consumption (peak VO2), both integral markers of cardiovascular function, are related to OSAS severity. In addition, we tested whether NT-proBNP levels depend on body composition in OSAS patients, similar to what has been reported in patients without OSAS. Eighty-nine patients with untreated OSAS underwent NT-proBNP measurement, dual X-ray absorptiometry, and cardiopulmonary exercise testing. In a representative subgroup (n = 32), transthoracic echocardiography was performed. The severity of OSAS was classified based on apnea-hypopnea index (AHI) values as mild (AHI 5-15h−1), moderate (AHI 15-30h−1), and severe (AHI >30h−1). OSAS was mild in 19 (21%), moderate in 21 (24%), and severe in 49 (55%) patients. NT-proBNP levels did not differ among patients with mild [30 (10-57)], moderate [37 (14-55)], and severe [24 (13-49) pg/ml; p = 0.8] OSAS and were not related to body mass index (r = 0.07; p = 0.5), percent lean body mass (r = −0.17; p = 0.1), and percent fat mass (r = 0.18; p = 0.1). Percent predicted peak VO2 was on average normal and did not differ among patients with mild (115 ± 26), moderate (112 ± 23), and severe OSAS (106 ± 29%; p = 0.4). Body weight-indexed peak VO2 did not differ among patients with mild (31.9 ± 10.3), moderate (32.1 ± 7.9), and severe OSAS (30.0 ± 9.9ml kg−1 min−1; p = 0.6) either. Lower NT-proBNP (β = −0.2; p = 0.02) was independently but weakly associated with higher body weight-indexed peak VO2. In the echocardiography subgroup, NT-proBNP was not significantly related to left ventricular mass index (r = 0.26; p = 0.2). In conclusion, NT-proBNP and peak VO2 are not related to OSAS severity, and NT-proBNP poorly reflects left ventricular hypertrophy in OSAS. The lack of a relationship between NT-proBNP and OSAS severity is not due to a significant influence of body composition on NT-proBNP. There is an association between higher NT-proBNP and lower peak VO2, indicating that NT-proBNP is a marker of cardiorespiratory fitness in patients with OSAS. However, the association is too weak to be clinically usefu

Elevated Cardiac Troponin I in Sepsis and Septic Shock: No Evidence for Thrombus Associated Myocardial Necrosis

Elevated cardiac troponin I (cTnI) is frequently observed in patients with severe sepsis and septic shock. However, the mechanisms underlying cTnI release in these patients are still unknown. To date no data regarding coagulation disturbances as a possible mechanism for cTnI release during sepsis are available.Consecutive patients with systemic inflammatory response syndrome (SIRS), sepsis or septic shock without evidence of an acute coronary syndrome were analyzed. Coagulation parameters (clotting time (CT), clot formation time (CFT), maximum clot firmness (MCF), alpha-angle) were assessed in native whole blood samples, and using specific activators to evaluate the extrinsic and intrinsic as well as the fibrin component of the coagulation pathway with the use of rotational thrombelastometry (ROTEM). Thirty-eight patients were included and 22 (58%) were cTnI-positive. Baseline characteristics between TnI-positive and -negative patients were similar. The CT, CFT, MCF and the alpha-angle were similar between the groups with trends towards shorter CT in the extrinsic and fibrin activation.We found no differences in coagulation parameters analyzed with rotational thrombelastometry between cTnI-positive and -negative patients with SIRS, severe sepsis, and septic shock. These findings suggest that pathophysiological mechanisms other than thrombus-associated myocardial damage might play a major role, including reversible myocardial membrane leakage and/or cytokine mediated apoptosis in these patients

End-of-life preferences of elderly patients with chronic heart failure

Aims Elderly heart failure (HF) patients are assumed to prefer improved quality of life over longevity, but sufficient data are lacking. Therefore, we assessed the willingness to trade survival time for quality-of-life (QoL) and the preferences for resuscitation. Methods and results At baseline and after 12 and 18 months, 622 HF patients aged ≥60 years (77 ± 8 years, 74% NYHA-class ≥III) participating in the Trial of Intensified vs. standard Medical therapy in Elderly patients with Congestive Heart Failure had prospective evaluation of end-of-life preferences by answering trade-off questions (willingness to accept a shorter life span in return for living without symptoms) and preferences for resuscitation if necessary. The time trade-off question was answered by 555 patients (89%), 74% of whom were not willing to trade survival time for improved QoL. This proportion increased over time (Month 12: 85%, Month 18: 87%, P < 0.001). In multivariable analysis, willingness to trade survival time increased with age, female sex, a reduced Duke Activity Status Index, Geriatric Depression Score, and history of gout, exercise intolerance, constipation and oedema, but even combining these variables did not result in reliable prediction. Of 603 (97%) patients expressing their resuscitation preference, 51% wished resuscitation, 39% did not, and 10% were undecided, with little changes over time. In 430 patients resuscitation orders were known; they differed from patients' preferences 32% of the time. End-of-life preferences were not correlated to 18-month outcome. Conclusion Elderly HF patients are willing to address their end-of-life preferences. The majority prefers longevity over QoL and half wished resuscitation if necessary. Prediction of individual preferences was inaccurate. Trial Registration: isrctn.org Identifier: ISRCTN4359647