Hypoplastic coronary artery disease, as a cause of sudden death

ABSTRACT Hypoplastic coronary artery disease (HCAD) is a rare coronary artery anomaly that may be the cause of sudden death. It can involve a single or all coronary arteries. This anomaly may cause circulatory insufficiency leading to myocardial infarction. HCAD has no symptoms or may exhibit cardiovascular signs like syncope, dyspnea, chest discomfort, or dizziness. It is often diagnosed at autopsy, and early diagnosis is made with a coronary angiogram. We report HCAD as the cause of the sudden death of a 25-year-old female with a history of loss of consciousness following exertion. On autopsy, all the coronary arteries' lumen was narrowed with thin vessel walls. Histopathological examination shows an underdeveloped and missing muscular layer of the left anterior descending and circumflex coronary arteries' vascular wall. Many cases of HCAD diagnosed by radiographic imaging in living patients have been reported in the literature, but a structural anomaly of coronaries leading to HCAD has not been reported. We report a case of HCAD describing the histopathological examination findings of the vascular wall of coronary vessels illustrating the structural difference.

Acute Promyelocytic Leukemia Patients Receiving ATRA with and without Voriconazole Prophylaxis: Effect on Incidence and Outcomes of Differentiation Syndrome.

Background: The combination of all trans-retinoic acid (ATRA) and voriconazole may lead to increased ATRA exposure resulting in a higher incidence of differentiation syndrome (DS). Patients and Methods: This single center analysis evaluated the incidence and outcomes of ATRA-induced DS in 46 adult patients with acute promyelocytic leukemia (APL) undergoing induction chemotherapy. Results: Thirty-one patients (69% by day 60) received a chemotherapy regimen including ATRA coinciding with voriconazole administration and 15 patients underwent treatment without voriconazole. The overall incidence of DS was 36% (n=16) by day 60, with patients receiving voriconazole showing a trend towards a higher incidence (HR 2.31, CI 0.78-6.847, p=0.1308). This trend persisted after adjusting for BMI (HR 1.96, CI 0.65-5.94, p=0.23); however, a small number of DS events precluded statistical significance. Conclusion: A trend towards an increased incidence and severity of ATRA-mediated DS was seen in adult APL patients receiving voriconazole prophylaxis during induction chemotherapy. This important finding warrants validation in larger studies.

Thrombotic microangiopathies presenting as an obstetric emergency.

Thrombotic microangiopathies are disorders that arise due to a diffuse endothelial damage. They predominantly manifest either as thrombotic trombocytopenic purpura (TTP) or hemolytic uremic syndrome (HUS). When they arise as a complication of pregnancy and associated disorders they are associated with a high mortality and morbidity.