Measurement of left atrial size as a predictor of severity of illness in sickle cell disease.

BACKGROUND: Sickle cell disease (SCD) is characterized by microvascular occlusion which leads to multiorgan damage, including left ventricular diastolic dysfunction. Left ventricular diastolic dysfunction has been shown to be an independent risk factor for death in SCD patients. Left atrial dilation (LAD) has been used as a surrogate marker for identification of left ventricular diastolic dysfunction. OBJECTIVE: Investigate the association of LAD, as determined by echocardiography, with increased disease burden in SCD as reflected by increased emergency department (ED) utilization, increased hemolysis markers, and worsening anemia. METHODS: A retrospective cohort study of patients from a single university hospital were selected from a national registry. Age, sickle cell phenotype, echocardiogram findings, ED utilization, baseline hemoglobin, and lab values needed for calculation of hemolytic index were recorded for each patient. Patients were then stratified into two distinct groups based on the presence or absence of LAD to compare ED utilization, baseline hemoglobin and hemolytic index between the two groups. RESULTS: 129 patients met the criteria for inclusion with 88 having normal left atrial volume and 41 with LAD. There was a higher percentage of high ED utilizers in the LAD group compared to the normal left atrial volume group [34% vs. 17%, p = 0.03]. Average hemoglobin was lower in the LAD group compared with the normal left atrial volume group [mean 8.57 g/dL vs. 9.47 g/dL, p = 0.011]. The mean hemolytic index was higher in the LAD group when compared with the normal left atrial volume group [0.44 vs. -0.21, p < 0.001]. CONCLUSIONS: LAD was associated with higher ED utilization, lower hemoglobin level, and more hemolysis in patients with SCD.

A rare case of type 2 Kounis syndrome secondary to iodinated contrast.

Immune-mediated acute coronary syndrome, also known as Kounis syndrome (KS), is an underrecognized and challenging diagnosis. In this case report, we present a case of cardiac arrest secondary to iodinated contrast allergy requiring emergent cardiac catheterization and hemodynamic support secondary to type 2 KS. KS necessitates a high index of clinical suspicion by the treating physician in order to address both the hypersensitivity reaction and its cardiac implications. Learning objectives: Kounis syndrome (KS) is a clinically distinct entity from anaphylaxis; managing KS in the same way as anaphylaxis can worsen cardiac demand and ischemia. In addition, KS may present as coronary vasospasm or plaque rupture; regardless, percutaneous coronary intervention (PCI) should be performed as worse outcomes have been described in cases where PCI is not performed or delayed.