Life-threatening tumors of the heart in fetal and postnatal age.

OBJECTIVES: To evaluate the role of histology in diagnosis and management of biologically benign heart tumors causing life-threatening symptoms and even death in children and fetuses. The clinical impact of a multidisciplinary approach including 2-D echocardiography, histology, genetics, and cardiac surgery has not yet been fully elucidated. STUDY DESIGN: Forty-one consecutive antenatal (n = 17) or postnatal (n = 24) detected cardiac masses were evaluated by 2-D echocardiography (in alive patients) or at autopsy, and 12/41 cases with definite histologic diagnosis of primary and benign cardiac tumor were entered in this study. RESULTS: Rhabdomyomas (n = 6), hemangiomas (n = 3), central fibrous body chondroma (n = 1), fibroma (n = 1), or left atrial myxoma (n = 1) were histologically diagnosed in 4 fetuses and in 8 children. Death occurred in 6 patients showing diffuse or infiltrative tumors, 2/6 experiencing intrauterine death or sudden and unexpected infant death. Seven patients underwent surgery, 4/7 are alive and well at >5 years follow-up, whereas 3 deaths followed partial tumor resection. Two fetuses with extensive tumor/s were aborted. Tuberous sclerosis complex gene mutations were seen in patients with rhabdomyomas. CONCLUSIONS: Histology represents the best diagnostic approach in life-threatening pediatric cardiac tumors allowing definite diagnosis in cases other than rhabdomyoma and in sudden deaths, influencing clinical management and counselling. 2-D echocardiography remains the main tool for early clinical diagnosis and follow-up. A multidisciplinary approach is advisable because of rarity, difficult management, and possible associations with inheritable diseases.

Quantitative coronary angiography in the current era: principles and applications.

Coronary angiography, despite its inherent invasiveness and need for contrast media and radiographic exposure, is still routinely employed every year for the diagnostic and therapeutic management of millions of patients with or at risk for coronary artery disease. Whereas approximate visual estimation is the most common way to evaluate coronary angiography findings, since the late 1980s a number of investigators have developed and investigated methods of quantitative coronary angiography (QCA) analysis exploiting automated or semi-automated edge detection. Despite the inherent drawback of QCA due to its focus on the contrast-filled lumen of the vessel, QCA has offered and continues to offer important insights for clinical research and, in selected cases, clinical practice. This review aims thus to provide a comprehensive and updated viewpoint on the actual role of QCA.