Two patients with ascending aortic aneurysms due to cystic medical necrosis are described. One of them was phenotypically normal while the other had features of Marfan's syndrome. Both were disabled by dyspnoea and angina which required corrective surgery.
Bacterial arteritis is relatively uncommon and management of this condition, which carries high morbidity and mortality, is difficult and time-consuming. Common organisms implicated include Salmonella and Staphylococcus. Arteritis as a result of infection by Burkholderia pseudomallei (formerly Pseudomonas pseudomallei) has been rarely reported in the English literature. This organism, which is endemic in our part of the world, is well known to cause a wide spectrum of septic conditions. A review of cases managed at Hospital Kuala Lumpur revealed that bacterial arteritis due to melioidosis is not such a rare entity. We share our experience in the management of this condition using three cases as examples.
FBN1 gene encodes for the connective tissue protein fibrillin-1 which can also regulate the profibrotic cytokine transforming growth factor (TGF)-ß1. Mutations in the FBN1 gene cause Marfan syndrome (MFS), a genetic condition with defective connective tissues. FBN1 haplotypes and single nucleotide polymorphisms have also been reported to be associated with systemic sclerosis (SSc), a connective tissue disease characterized by fibrosis of multiple organs. Furthermore, the duplication of the Fbn1 gene causes a SSc-like disease in the TsK1 mouse model. To the best of our knowledge, there are no reports of MFS and SSc co-existing in a patient. Here, we describe a 46-year-old woman who presented with cardiac failure. She had a family history of MFS. Physical examination revealed marfanoid habitus and scleroderma features. Echocardiography demonstrated dilated cardiomyopathy with aortic root dilatation, aortic regurgitation and mitral regurgitation. Cardiac magnetic resonance imaging was consistent with dilated cardiomyopathy, mid-wall fibrosis at basal septal wall and dilated aortic root. Extractable nuclear antigen panel detected anti-Scl 70. She fulfilled Ghent criteria for MFS and satisfied American College of Rheumatology/ European League Against Rheumatism classification criteria for SSc. Although we do not have the FBN1 sequence in our patient, the co-existence of MFS and SSc in this patient raises the possibility of co-existence of distinct mutations in the FBN1 gene that could affect TGF-β signaling differently, resulting in divergent pathologic consequences - loss of structural integrity in MFS versus increased extracellular matrix deposition in SSc, and different clinical manifestations.
Delayed ascending aortic dissection following coronary artery bypass surgery is a rare but lethal complication. We present the case of a 54-year-old man with a delayed acute Stanford A aortic dissection following an off-pump coronary artery bypass surgery in preexisting chronic type B disease. Such a case of an iatrogenic acute aortic dissection poses a significant challenge and dilemma in choosing the best technique for coronary revascularization in this group of patients. The pathophysiology and technical options are discussed.