Case Report: Postpartum Purtscher-like Retinopathy.

SIGNIFICANCE: This case highlights various clinical features and resolution of Purtscher-like retinopathy after childbirth. PURPOSE: This study aimed to present a case report of postpartum Purtscher-like retinopathy and subsequent resolution after oral and intravitreal treatment. CASE REPORT: A 30-year-old woman presented with subjective visual disturbance and transient scotoma in her right eye 7 days after giving birth. Vision measured 20/20 in her right eye and 20/20 in her left eye. Clinical examination revealed optic disc edema, retinal ischemia, and retinal hemorrhage in the right eye with a grossly normal appearance of the left eye. A diagnosis of Purtscher-like retinopathy was made. Neuroimaging and bloodwork were noncontributory to the diagnosis. Approximately 1 month after treatment with rapidly tapered oral methylprednisolone and a single intravitreal bevacizumab (Avastin; Genentech, San Francisco, CA) injection, the retinopathy had resolved with subjective visual improvement. CONCLUSIONS: Purtscher-like retinopathy can occur after childbirth, usually within 1 to 2 days postpartum and occasionally associated with preeclampsia. Clinical course and visual improvement vary, but novel treatments may aid in visual recovery.

Cardiac Amyloidosis: The Importance of Surveillance in a Patient With an Initial Negative Cardiac Biopsy.

Cardiac amyloidosis is a progressive disorder and is sometimes difficult to diagnose even when suspected in the appropriate clinical setting. We present an interesting case of rapidly progressive light-chain cardiac amyloidosis and highlights the importance of close monitoring even when the initial biopsy and imaging findings are not pathognomonic for amyloidosis. (Level of Difficulty: Beginner.).

Assessment of myocardial oxygenation, strain, and diastology in MYBPC3-related hypertrophic cardiomyopathy: a cardiovascular magnetic resonance and echocardiography study.

AIMS: Myocardial oxygenation is impaired in hypertrophic cardiomyopathy (HCM) patients with left ventricular hypertrophy (LVH), and possibly also in HCM gene carriers without LVH. Whether these oxygenation changes are also associated with abnormalities in diastolic function or left ventricular (LV) strain are unknown. METHODS AND RESULTS: We evaluated 60 subjects: 20 MYBPC3 gene positive patients with LVH (G+LVH+), 18 MYBPC3 gene positive without LVH (G+LVH-), 11 gene negative siblings (G-), and 11 normal controls (NC). All subjects underwent 2D transthoracic echocardiography and cardiovascular magnetic resonance imaging for assessment of ventricular volumes, mass, and myocardial oxygenation at rest and adenosine stress using the blood oxygen level dependent (BOLD) technique. Maximal septal thickness was 20 mm in the G+LVH+ group, vs. 9 mm for the G+LVH- group. As expected, the G+LVH+ group had a more blunted myocardial oxygenation response to stress when compared with the G+LVH- group (-5% ± 3% vs. 2% ± 4%, P < 0.05), G- siblings (-5% ± 3% vs. 11% ± 4%, P < 0.0001) and NC (-5% ± 3% vs. 15% ± 4%, P < 0.0001). A blunted BOLD response to stress was also seen in G+LVH- subjects when compared with gene negative siblings (2% ± 4% vs. 11% ± 4%, P < 0.05) and NC (15% ± 4%, P < 0.050). G+LVH+ patients exhibited abnormal diastolic function including lower E', higher E to E' ratio and greater left atrial area compared with the G+LVH- subjects who all had normal values for these indices. CONCLUSION: Myocardial deoxygenation during stress is observed in MYBPC3 HCM patients, even in the presence of normal LV diastolic function, LV global longitudinal strain, and LV wall thickness.