Two cases of Shone syndrome with severe mitral and aortic valve problems and pulmonary hypertension were referred for heart-lung transplantation. Severely elevated pulmonary vascular resistance (PVR) was confirmed as was severe periprosthetic mitral and aortic regurgitation. Based on the severity of the valve lesions in both patients, surgery was decided upon and undertaken. Both experienced ea...

The syndrome of autonomic dysreflexia often occurs in quadriplegic subjects and is characterized by paroxysmal hypertension, headache, vasoconstriction below and flushing of the skin above the level of transection, and bradycardia. These attacks may cause hypertensive encephalopathy, cerebral vascular accidents, and death. In five patients during crises, the mean arterial pressure changed from ...

The ganglioneuroma is a tumor of the sympathetic nervous system and may be associated with hypersecretion of vasoactive substances responsible for various symptoms and signs such as hypertension. The authors report a case of ganglioneuroma and a literature review, focusing on the most important aspects of diagnosis and therapy. The patient had recurrent symptomatic hypertensive crises, having p...

We describe a unique case of a patient with a 'pigmented' cardiac paraganglioma in a multiple paraganglioma syndrome. She was symptomatic for arrhythmias, hypertensive crises and dyspnoea due to a cardiac tumour, which was richly vascularised from the right coronary artery and was partially obstructing the right atrioventricular inflow. She was operated on, but the mass was not completely resec...

BACKGROUND Pulmonary hypertensive crises (PHTC) are a major cause of morbidity and mortality after congenital heart surgery. Inhaled nitric oxide is frequently used as rescue therapy. We did a randomised double-blind study to investigate the role of routinely administered inhaled nitric oxide to prevent pulmonary hypertension in infants at high risk. METHODS We enrolled 124 infants (64 male, ...