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. The spectrum of clinical, radiological, and pathological abnormalities is discussed.

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.

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. We believe this is the largest series of cardiac amyloidosis reported in Chinese patients. The 1-year, 3-year and 5-year survival rates were 40%, 25% and 17%, respectively. The major cause of death was cardiac-related. Those patients with overt heart failure or with untreated amyloidosis had a dismal prognosis (mean survival of 2.2 months and 3.5 months, respectively). Those who received specific treatment for the underlying amyloidosis had a better outcome with an average survival of 33.4 months.

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. At a later stage, there may be aneurysmal dilatation of the aorta and rarely dissection or rupture of the aorta. In Takayasu's arteritis, stenosing lesions can occur as well as aneurysmal dilatation of the aorta or arteries. Stenosing lesions may be treated with angioplasty with or without stenting, whereas aneurysmal dilatation of the aorta is treated by aneurys-mectomy with arterial reconstruction or conduit. Severe aortic regurgitation may require aortic valve surgery with or without replacement of the ascending aorta. Irrespective of the interventional procedure undertaken as appropriate for the lesion, control of inflammation with steroid therapy with or without other immunosuppressive agents is of paramount importance. Otherwise, prosthetic valve or graft dehiscence may occur after aortic surgery, and restenosis rate is also higher after percutaneous transluminal angioplasty or stenting.

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. CONCLUSION: Sildenafil increases pulmonary blood flow and improves cyanosis in patients with Eisenmenger syndrome. Efficacy of sildenafil as treatment for idiopathic pulmonary arterial hypertension may be extended to patients with Eisenmenger syndrome.

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%. Strategies to prevent dehiscence of the prosthetic aortic valve, which include institution of immunosuppression, replacement of the diseased aorta, and monitoring inflammatory indices, are discussed.

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. Mechanical circulatory support was required in 50% of the patients. Time from onset of illness to recovery of ventricular function varied from 12 to 17 days. All except one patient were alive at a mean follow-up of 4.4 years after the acute illness. Patients with acute fulminant myocarditis should be treated aggressively with immunosuppression and, if necessary, mechanical circulatory support during the first 2 to 3 weeks of the illness because of the reversible nature of this illness and good long-term prognosis.

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. Incessant ventricular arrhythmia rapidly ensued, which did not respond to anti-arrhythmic therapy and overdrive pacing despite complete surgical revascularization. He eventually died. Autopsy revealed giant cell myocarditis superimposed on coronary artery disease. Acute myocarditis masquerading as myocardial infarction has been well known, but virtually all reported cases had normal coronary arteries. This case illustrated the fact that even in the presence of obvious coronary artery disease the remote possibility of myocarditis should not be entirely disregarded. Although giant cell myocarditis is a rare and frequently fatal disorder, recent studies suggest that combined immunosuppressive therapy may improve the prognosis.