Thrombotic storm in a 4-year-old boy with a thrombus in the right atrium.

Thrombotic storm (TS) is a rare disease, especially with thrombus in the heart of pediatric patient. We present a case of a 4-year-old boy, who was diagnosed with TS during his first hospitalization due to lower extremity deep venous thrombosis, pulmonary embolism, and thrombosis of the inferior vena cava, cerebral, left internal jugular, portal, renal, and iliac veins. He was eventually prescribed with rivaroxaban to control thrombosis after 30 days of successive use of low-molecular-weight heparin, unfractionated heparin, and warfarin, which were demonstrating little effect on preventing thrombosis, and the patient was intolerant to argatroban. While his lupus anticoagulant ratio was slightly above the normal range and no other potential causes such as congenital thrombophilia, severe infection, malignancy, and trauma were confirmed, we suspected antiphospholipid antibody syndrome and prescribed glucocorticoid and rituximab to control the disease. After 36 days of admission, ultrasonography showed recanalization of the former thrombus. One month after discharge, a tumor embolus resembling a mass emerged in his right atrium under effective anticoagulant therapy. During his second admission, he underwent surgical thrombectomy, and pathological examination confirmed the mass to be a platelet-rich thrombus rather than tumor embolus or infection. Considering the suspected antiphospholipid antibody syndrome as the cause of the TS, we prescribed aspirin combined with rivaroxaban to prevent thrombosis. In this case, surgery and pathology shed light on the type of thrombus that emerged from the inferior vena cava and traveled to the heart, which is the possible potential cause of TS. It also changed our therapeutic strategy to antiplatelet therapy combined with anticoagulant therapy to control the disease.

[Evaluation of coronary artery diameter in normal children by echocardiography and its clinical significance].

OBJECTIVE: To obtain normal range of coronary artery diameter with body surface area (BSA) dynamic changes in normal children at different age. METHOD: The left main coronary artery (LCA), left anterior descending artery (LAD), left circumflex artery (LCX) and the right coronary artery (RCA) diameter were measured in 400 normal subjects from Chinese population aged 0 d to 18 years [(6.43 ± 4.45) years], using HP Sonos 5500 color Doppler ultrasonic system, according to the standard method of measuring the coronary artery diameter. RESULT: (1) The diameters of LCA, LAD, LCX and RCA in different age groups (0 d-12 months, -3 years, -6 years, -9 years, -12 years, -18 years) had significant differences (F = 61.688, 51.343, 46.375, 50.192, P < 0.01,all groups mean differences had significant differences, there was significant difference between every two groups, P < 0.05), there were no significant differences between male and female subjects (P > 0.05). (2) The correlation analyses showed that the diameter of LCA, LAD, LCX and RCA had significant linear correlations with age, height, weight and BSA (r ranged from 0.71 to 0.85, P < 0.01 ). (3) The regression analyses were respectively performed on the diameters of LCA, LAD, LCX and RCA with BSA to establish seven regression models. The coefficients were compared for each model, the best model was chosen to create a Z score calculator, tracing out the Z value curve, through clinical practice,we chose Z score within ± 2 as the coronary artery diameter's normal range for Chinese children. CONCLUSION: Coronary artery diameter's Z score curve is effective and reliable, it provide objective basis for clinicians and sonographers to accurately and quickly diagnose the anomalies in diameter of coronary artery.

Congenital vascular rings: a rare cause of respiratory distress in infants and children.

BACKGROUND: Congenital vascular rings may often cause unexplained respiratory symptoms in infants and young children. Their diagnosis and treatment are often delayed. Few studies of vascular rings have been reported in China. The aim of this study was to describe the clinical presentation, diagnosis and surgical management of infants and children with congenital vascular rings. METHODS: Clinical histories, physical examinations, investigations, image studies and surgical interventions were retrospectively evaluated in 7 children (age range: 2 months-4 years, mean 7 months) with congenital vascular rings. Chest radiography was performed in all patients. Echocardiography and computed tomography (CT) with 3-dimensional (3D) reconstructions were performed in 6 patients. Esophagography, cardiac catheterization and angiography, and bronchoscopy were performed in 1, 1 and 4 children, respectively. RESULTS: Six of the 7 patients had respiratory symptoms, including recurrent cough, stridor and wheeze. Age at onset of symptoms ranged from 1 month to 11 months. Chest X-ray showed nothing important on the vascular rings, besides bronchitis and pneumonia. Contrast-enhanced CT diagnosed vascular rings in 6 patients. Four patients had double aortic arches, two had balanced arches and two were right arch dominant. One patient had a right aortic arch with left ligament and 1 patient had a pulmonary artery sling. Echocardiography failed to diagnose vascular rings in 2 patients. The esophagogram of 1 patient showed esophageal compression. Bronchoscopy of 4 patients showed compression of the distal trachea. Five of the 7 patients underwent surgical division of the vascular rings. Surgical observation confirmed the CT findings in each patient. CONCLUSIONS: Patients, especially infants or young children, with recurrent respiratory symptoms such as chronic cough, stridor and wheeze, should be examined for the possible presence of congenital vascular rings. Contrast-enhanced CT can clearly show the anatomy of vascular rings. As a noninvasive technique, echocardiography is helpful for diagnosis. Early surgical management in symptomatic patients is effective.