A spuriously 'normal' haemoglobin A1c result.

We report a case of spuriously 'normal' haemoglobin A1c (HbA1c) result due to misidentification of HbG Taipei as HbAo by the Variant II built-in retention time algorithm. The defect was circumvented effectively by the implementation of a chromatographic system specific internal quality control mechanism for peak identity verification. HbA1c and estimated average glucose results were corrected from 4.

Isolated left ventricular non-compaction: an unusual indication for heart transplantation.

We report a patient with isolated left ventricular non-compaction diagnosed by echocardiography and cardiac magnetic resonance imaging. She developed refractory congestive heart failure and subsequently underwent successful heart transplantation. This type of cardiomyopathy is thought to be caused by the arrest of normal embryogenesis of the endocardium and myocardium.

Spontaneous atraumatic rupture of a normal-sized spleen due to AL amyloid angiopathy.

We report an unusual cause of hemoperitoneum in an AL amyloid patient on peritoneal dialysis due to spontaneous rupture of a normal-sized spleen not related to any trauma. The rupture was not due to amyloid deposition within the spleen pulp but rather due to amyloid angiopathy causing hemorrhage within the spleen and capsular tear.

Aortitis.

Inflammatory or noninfectious aortitis may be idiopathic or it may be part of a systemic autoimmune disease, such as Takayasu's arteritis, Behçet's disease, or giant cell arteritis. At the acute stage, there is thickening of the aortic wall with dilatation of the aorta, more commonly in the thoracic aorta. If it involves the aortic root, there may be annuloaortic ectasia or aortic regurgitation.

Cardiac amyloidosis - experience in a tertiary cardiac referral centre.

Amyloidosis is an uncommon systemic disease characterized by deposition of insoluble fibrillar protein in different organs and the prognosis is poor if the heart is involved. Experience with management of cardiac amyloidosis is difficult because of its rare occurrence, late presentation and ineffective treatment. Since 1995, we have encountered and prospectively followed up 16 cases of cardiac amyloidosis in our cardiac centre.

Effects of chronic sildenafil in patients with Eisenmenger syndrome versus idiopathic pulmonary arterial hypertension.

Background: To test the hypothesis that chronic sildenafil treatment has similar functional and hemodynamic effects in patients with severe pulmonary arterial hypertension due to Eisenmenger syndrome as those due to idiopathic pulmonary arterial hypertension without intracardiac shunts.

Methods: A prospective open-label study was carried out to compare the effects of sildenafil on the pulmonary hemodynamics between two groups of patients with severe pulmonary hypertension and similar baseline functional capacity--Eisenmenger syndrome (ES group) (n=7) versus idiopathic pulmonary arterial hypertension (IPAH group) (n=6).

Results: After 6 months of sildenafil, there was a significant improvement in the functional capacity, the arterial saturation and the pulmonary hemodynamics in the ES group, as shown by significant reduction in the systolic and mean pulmonary artery pressures and the pulmonary vascular resistance.

Non-infectious aortitis: an important cause of severe aortic regurgitation.

From 1995 to 2004, 13 patients with severe aortic regurgitation due to non-infectious aortitis underwent aortic valve surgery at our center. Twenty-eight operations (18 aortic valve replacements and 10 Bentall procedures including 1 aortic root replacement with a homograft) were performed due to a high incidence of prosthetic valve or valved conduit dehiscence (54%). Steroid therapy reduced the incidence of re-operation for anastomotic dehiscence within one year from 90% to 18%.

Treatment and outcome of biopsy-proven fulminant myocarditis in adults.

Fulminant myocarditis may be rapidly fatal with severe haemodynamic compromise in previously healthy patients. We reviewed our experience with eight cases of biopsy-proven fulminant myocarditis (seven cases with lymphocytic myocarditis and one with eosinophilic myocarditis). Immunosuppression was given in seven out of eight cases.

Coronary artery disease obscuring giant cell myocarditis--a case report.

A case in which the diagnosis of idiopathic giant cell myocarditis was obscured by the presence of severe coronary artery disease is described. A 47-year-old man presented with recurrent inferior myocardial infarction and complete heart block. Cardiac catheterization confirmed severe 2-vessel disease and left ventricular dysfunction.