BackgroundThe Protein Kinase AMP-Activated Non-Catalytic Subunit Gamma 2 (PRKAG2) cardiac syndrome is characterized by glycogen accumulation in the cardiac tissue. The disease presents clinically with hypertrophic cardiomyopathy (HCM), and it is often associated with conduction abnormalities.Case presentationA 23 year-old female with history of Wolff-Parkinson-White (WPW) and HCM presented for evaluation after an episode of Non-ST Elevation Myocardial Infarction (NSTEMI). The patient was found to have severe coronary bridging on angiography and underwent an unroofing of the left anterior descending artery (LAD). Due to the constellation of symptoms, the patient underwent genetic testing and a cardiac muscle biopsy. Genetic testing was significant for an Arg302Gln mutation in the PRKAG2 gene. Cardiac tissue biopsy revealed significant myocyte hypertrophy and large vacuoles with glycogen stores.ConclusionThe pathologic and genetics findings of our patient are consistent with PRKAG2 syndrome. Patients presenting with conduction abnormalities and suspected HCM should be considered for genetic testing to identify possible underlying genetic etiologies.

Objectives To evaluate how a comprehensive evidence‐based clinical review by a multidisciplinary revascularization heart team on treatment decisions for revascularization in patients with complex coronary artery disease using SYNTAX scores combined with Society of Thoracic Surgeons‐derived clinical variables can be additive to the utilization of Appropriate Use Criteria for coronary revascularization. Background Decision‐making regarding the use of revascularization for coronary artery disease has come under major scrutiny due to inappropriate overuse of revascularization. There is little data in routine clinical practice evaluating how a structured, multidisciplinary heart team approach may be used in combination with the Appropriate Use Criteria for revascularization. Methods From May 1, 2012 to January 1, 2015, multidisciplinary revascularization heart team meetings were convened to discuss evidence‐based management of 301 patients with complex coronary artery disease. Heart team recommendations were adjudicated with the Appropriate Use Criteria for coronary revascularization for each clinical scenario using the Society for Cardiovascular Angiography and Interventions' Quality Improvement Toolkit (SCAI‐QIT) Appropriate Use Criteria App. Results Concordance of the Heart Team to Appropriate Use Criteria had a 99.3% appropriate primary indication for coronary revascularization. Among patients who underwent percutaneous revascularization, 34.9% had an inappropriate or uncertain indication as recommended by the Heart Team. Patients with uncertain or inappropriate percutaneous coronary interventions had significantly higher SYNTAX score (27.3 ± 6.6; 28.5 ± 5.5; 19.2 ± 6; P < 0.0001) and Society of Thoracic Surgeons‐Predicted Risk of Mortality (6.1% ± 4.7%; 8.1% ± 6.3%; 3.7% ± 4.1%; P < 0.0081) compared to appropriate indications, frequently had concomitant forms of advanced comorbidities and frailty in the setting of symptomatic coronary artery disease. Conclusions A formal, multidisciplinary revascularization heart team can provide proper validation for clinical decisions and should be considered in combination with the Appropriate Use Criteria for coronary revascularization to formulate revascularization strategies for individuals in a patient‐centered fashion. © 2015 Wiley Periodicals, Inc.

Multidisciplinary decision making has been shown to be highly effective in various aspects of medicine, most notably with the concept of tumor boards and transplant committees. ,2The most updated guidelines for percutaneous coronary intervention (PCI), published jointly by the American College of Cardiology Foundation, American Heart Association, and the Society for Cardiovascular Angiography and Interventions, assign a class IC recommendation for the use of a collaborative Heart Team approach in the treatment of patients with complex coronary artery disease (CAD). 3 The guidelines assert that this recommendation is based on retrospective analyses showing that patients with complex CAD referred for revascularization based on a Heart Team consensus have improved mortality compared with patients merely assigned to a particular strategy in the context of their trial enrollment. Despite the suggestion of improved mortality in this retrospective comparison, the Heart Team approach has not been adopted widely in the current clinical practice of cardiovascular medicine. This multidisciplinary innovation remains in its infancy, and numerous questions remain about its practicality, feasibility, and efficacy.For several reasons, there remains significant variability in the care delivered to patients with complex CAD. 4 Numerous reports show that although differences in patient characteristics may explain some of the variability in revascularization decisions, much of this variance is physician driven, such as practicing in a fee-for-service model or high-risk anatomy for low-volume operators. 4,5 As emphasis grows on informed decision making and patient-centered care, a critical evaluation of these difficult questions will be essential to discovering whether there is a clinically meaningful effect of the Heart Team approach on patients with complex CAD.Although the longstanding use of tumor boards in the field of oncology represents a functioning model of interdisciplinary care on which the Heart Team may be based, it is critical to gather data on the types of patients who could be discussed in a Heart Team. Although the convening of tumor boards to provide prospective care in oncology is supported by the American College of Surgeons guidelines, recent data suggest that the use of tumor boards may have little impact on quality of care and survival across a large sample of patients in the Veterans Affairs Healthcare System. 6 Accordingly, if the Heart Team model of care is to be adopted, more evidence needs to be established in the literature on the details of how to implement such a system. Challenges in ImplementationSubstantial barriers do exist and have been identified previously. 7 The most significant barriers include the challenge of integrating multiple opinions into a final treatment decision systematically, engaging patients and families in decision making, communicating recommendations accurately and efficiently, and, finally, addressing the issue of remuneration for services. Also, time is a frequently cited li...

Cardiovascular disease is the leading cause of global mortality, yet its early detection remains a vexing problem of modern medicine. Although the computed tomography (CT) calcium score predicts cardiovascular risk, relatively high cost ($250-400) and radiation dose (1-3 mSv) limit its universal utility as a screening tool. Dual-energy digital subtraction radiography (DE; <$60, 0.07 mSv) enables detection of calcified structures with high sensitivity. In this pilot study, we examined DE radiography's ability to quantify coronary artery calcification (CAC). We identified 25 patients who underwent non-contrast CT and DE chest imaging performed within 12 months using documented CAC as the major inclusion criteria. A DE calcium score was developed based on pixel intensity multiplied by the area of the calcified plaque. DE scores were plotted against CT scores. Subsequently, a validation cohort of 14 additional patients was independently evaluated to confirm the accuracy and precision of CAC quantification, yielding a total of 39 subjects. Among all subjects (n = 39), the DE score demonstrated a correlation coefficient of 0.87 (p < 0.0001) when compared with the CT score. For the 13 patients with CT scores of <400, the correlation coefficient was -0.26. For the 26 patients with CT scores of ≥400, the correlation coefficient yielded 0.86. This pilot study demonstrates the feasibility of DE radiography to identify patients at the highest cardiovascular risk. DE radiography's accuracy at lower scores remains unclear. Further evaluation of DE radiography as an inexpensive and low-radiation imaging tool to diagnose cardiovascular disease appears warranted.