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While severe vasculopathic manifestations of systemic sclerosis (SSc) are well-recognized, characterization of subclinical progressive vasculopathy contributing to cardiac involvement remains an unmet clinical need. This review highlights the evolving understanding of SSc heart involvement (SHI), including current standard clinical cardiac evaluation methods, prevalence of various cardiac manifestations of SHI, and advances at the forefront of precision medicine. Informed by this growing body of literature, we describe the development of a novel interdisciplinary cardio-rheumatology clinic at the Vanderbilt University Medical Center. Utilizing advances in imaging techniques and systemic retrieval and analysis of complex data sets, our dedicated cardio-rheumatology clinic offers opportunities for therapeutic advances and personalized medicine through mechanistic disease phenotyping in SSc. Nailfold capillaroscopy, thermography, and hand ultrasound with Doppler are acquired to characterize small vessel vasculopathy, while echocardiogram, ambulatory cardiac rhythm monitoring, cardiac magnetic resonance imaging, and cardiac positron emission tomography/computed tomography are utilized to characterize cardiac disease. By correlating vasculopathy imaging with cardiac manifestations, our cardio-rheumatology clinic aims to identify patients with SSc who would benefit from additional cardiac investigation even in the absence of cardiac symptomatology. This interdisciplinary collaboration may allow earlier detection of primary SHI, which is a common cause of death in SSc patients, resulting from both morpho-functional and electrical cardiac abnormalities. Our shared model of care and robust data acquisition facilitate clinical investigation by utilizing technological advances in data management. Using deep learning and pattern recognition, artificial intelligence (AI) offers opportunities to integrate data from imaging and monitoring techniques outlined in this report to provide quantifiable markers of disease progression and treatment efficacy. Given the potential for extensive AI data processing but the low prevalence of SSc, developing a multicenter cloud-based image sharing platform would accelerate clinical investigation in the field. Ultimately, we aim to tailor therapeutic decisions and risk mitigation strategies to improve SSc patient outcomes.

Cardiac amyloidosis is an acquired heart disease secondary to the deposition of β-pleated amyloid proteins in heart tissue. Amyloid light chain (AL) amyloidosis is usually secondary to multiple myeloma and can rapidly deteriorate cardiac function, with high mortality. Up to 50% of AL patients have cardiac involvement presenting as heart failure, conduction abnormalities, and cardiomyopathies. One of the rare presentations is the likely simulation of disease with hypertrophic cardiomyopathies like left ventricular outflow tract (LVOT) obstruction due to the systolic anterior motion of the mitral valve and irregular septal hypertrophy secondary to amyloid deposits. We present a case of cardiac amyloidosis secondary to multiple myeloma who presented with dynamic LVOT obstruction resembling hypertrophic obstructive cardiomyopathy and complicated by acute pulmonary edema. These complicated cases can be initially treated for pulmonary edema with an elevation of the head of the bed, furosemide, and nitroglycerin intravenously. For multiple myeloma, chemotherapy was continued. Beta-blockers, calcium channel blockers and angiotensin-converting enzyme inhibitors, and aldosterone receptor blocker were avoided due to poor tolerability. After symptomatic control, the patient can likely be scheduled for septal myotomy and the placement of a pacemaker or implantable cardiac defibrillator to prevent any arrhythmias causing sudden cardiac death in these subsets of patients.

We present a case of azathioprine hypersensitivity presenting as sepsis with elevated procalcitonin in a 68-year-old man with myasthenia gravis. The patient presented with fever, chills, nausea, vomiting, and headache. He developed numerous 1 cm erythematous papules over his upper torso. Infectious workup including bacteriological tests and microbial cultures was negative and a skin biopsy was performed which revealed suppurative folliculitis with eosinophils, consistent with drug hypersensitivity. Notably, acute phase reactants including C-reactive protein and procalcitonin were elevated upon presentation, likely secondary to drug hypersensitivity.

Objective To assess the effectiveness of a traditional birth attendant (TBA) referral program on increasing the number of deliveries overseen by skilled birth attendants (SBA) in rural Kenyan health facilities before and after the implementation of a free maternity care policy. Methods In a rural region of Kenya, TBAs were recruited to educate pregnant women about the importance of delivering in healthcare facilities and were offered a stipend for every pregnant woman whom they brought to the healthcare facility. We evaluated the percentage of prenatal care (PNC) patients who delivered at the intervention site compared with the percentage of PNC patients who delivered at rural control facilities, before and after the referral program was implemented, and before and after the Kenya government implemented a policy of free maternity care. The window period of the study was from July of 2011 through September 2013, with a TBA referral intervention conducted from March to September 2013. Results The absolute increases from the pre-intervention period to the TBA referral intervention period in SBA deliveries were 5.7 and 24.0 % in the control and intervention groups, respectively ( p  < 0.001). The absolute increases in SBA delivery rates from the pre-intervention period to the intervention period before the implementation of the free maternity care policy were 4.7 and 17.2 % in the control and intervention groups, respectively ( p  < 0.001). After the policy implementation the absolute increases from pre-intervention to post-intervention were 1.8 and 11.6 % in the control and intervention groups, respectively ( p  < 0.001). Conclusion The percentage of SBA deliveries at the intervention health facility significantly increased compared to control health facilities when TBAs educated women about the need to deliver with a SBA and when TBAs received a stipend for bringing women to local health facilities to deliver. Furthermore, this TBA referral program proved to be far more effective in the target region of Kenya than a policy change to provide free obstetric care.

Purpose of Review Diastolic dysfunction is an important, though often underappreciated, cause for exertional dyspnea. Echocardiography enables noninvasive evaluation of diastolic function and filling pressure, but images acquired at rest may be insensitive for detection of exertional abnormalities. This review focuses on stress echocardiography to assess diastolic function, including traditional and novel techniques, with emphasis on specific patient sub-groups in whom this testing may be valuable. Recent Findings Emerging data informs patient selection for diastolic stress testing. Further, increasing literature provides considerations for performance and interpretation of diastolic metrics relevant to patients with heart failure with preserved ejection fraction, hypertrophic cardiomyopathy, athletes, and those with microvascular coronary dysfunction. Methods, such as speckle-tracking and multi-modality imaging, provide additional and complementary information for non-invasive diastolic assessment. Summary This review serves as a guide to optimally utilize existing and novel techniques of stress echocardiography for diastolic assessment across a broad range of patients.

Objective: To assess the effectiveness of a traditional birth attendant (TBA) referral program on increasing the number of deliveries overseen by skilled birth attendants (SBA) in rural Kenyan health facilities before and after the implementation of a free maternity care policy. Methods: In a rural region of Kenya, TBAs were recruited to educate pregnant women about the importance of delivering in healthcare facilities and were offered a stipend for every pregnant woman whom they brought to the healthcare facility. We evaluated the percentage of prenatal care (PNC) patients who delivered at the intervention site compared with the percentage of PNC patients who delivered at rural control facilities, before and after the referral program was implemented, and before and after the Kenya government implemented a policy of free maternity care. The window period of the study was from July of 2011 through September 2013, with a TBA referral intervention conducted from March to September 2013. Results: The absolute increases from the pre-intervention period to the TBA referral intervention period in SBA deliveries were 5.7 and 24.0 % in the control and intervention groups, respectively (p < 0.001). The absolute increases in SBA delivery rates from the pre-intervention period to the intervention period before the implementation of the free maternity care policy were 4.7 and 17.2 % in the control and intervention groups, respectively (p < 0.001). After the policy implementation the absolute increases from pre-intervention to post-intervention were 1.8 and 11.6 % in the control and intervention groups, respectively (p < 0.001). Conclusion: The percentage of SBA deliveries at the intervention health facility significantly increased compared to control health facilities when TBAs educated women about the need to deliver with a SBA and when TBAs received a stipend for bringing women to local health facilities to deliver. Furthermore, this TBA referral program proved to be far more effective in the target region of Kenya than a policy change to provide free obstetric care.