An Intriguing Presentation of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis.

Hemophagocytic lymphohistiocytosis (HLH) is an immune related clinical syndrome with protean manifestations, varying presentation, clinically complex, with diverse causes, and is an under-recognized entity which carries high morbidity and mortality. It is precipitated by an immunological trigger in a susceptible host resulting in immune activation and dysregulation leading to disruption of immune homeostasis, cytokine storm and multi-organ failure. We describe a case of Epstein-Barr virus (EBV) associated HLH with its typical diagnostic challenges and associated high mortality rate. Certain diagnostic criteria and online tools may help to arrive at an earlier presumptive diagnosis which, in turn, may expedite treatment and lead to better clinical outcomes.

How effective are continuous flow left ventricular assist devices in lowering high pulmonary artery pressures in heart transplant candidates?

BACKGROUND: Pulmonary hypertension (PH) is considered a risk factor for morbidity and mortality in patients undergoing heart transplantation. Medical therapy with oral and pharmacologic agents is not always effective in reducing pulmonary artery (PA) pressures. Left ventricular assist devices (LVADs) have been used to reduce PA pressures in cases of PH unresponsive to medical therapy. METHODS AND RESULTS: Our study sought to evaluate the effectiveness of axial- and centrifugal- continuous flow LVADs in reversing PH in heart transplant candidates. Hemodynamics were assessed pre- and post-operatively in nine patients undergoing HeartMate II and six patients undergoing HeartWare continuous flow LVADs. Mean PA pressures were reduced from 31.9 ± 10.6 mm Hg to 22.1 ± 6.6 mm Hg (p = 0.001), and pulmonary vascular resistance was reduced from 3.08 ± 1.6 mm Hg to 1.8 ± 1.0 mm Hg (p = 0.007). This improvement was seen within seven days of LVAD implantation. Three of 15 patients were successfully transplanted, with 100% survival at an average of 199 days post-transplant. CONCLUSIONS: The results of this study suggest that both axial- and centrifugal-continuous flow LVADs are effective in immediately lowering PA pressures in heart transplant candidates with PH.

Anomalous origin of all three coronary arteries from right sinus of Valsalva in a patient with angina pectoris.

Anomalous origin of coronary arteries is uncommon, and all 3 coronary arteries arising from a single sinus of Valsalva is very rare. The anomaly has been associated with myocardial infarction and sudden cardiac death in particular when the anomalous left coronary artery courses between the great vessels. Imaging modalities have a complementary role to angiography in the diagnosis. Percutaneous interventions are technically difficult. Surgical options like direct repair of anomalous artery or coronary artery bypass graft can be helpful. We report a case of a patient who presented with chest pain and was found to have all 3 coronary arteries arising from the right sinus of Valsalva.

Anomalous origin of right coronary artery from pulmonary artery and severe mitral regurgitation due to myxomatous mitral valve disease: a case report and literature review.

Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is an uncommon congenital coronary artery anomaly. In contrast to anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), most of the patients with ARCAPA remain asymptomatic. However, few cases of angina, heart failure and sudden cardiac death depicting the malignant nature of the disease are described in the literature. We report an unusual case of ARCAPA with severe mitral valve regurgitation and pulmonary hypertension. Echocardiography and angiography were utilized for the diagnosis. Surgical correction was provided to our patient and should be considered for all patients with this potentially fatal disease.