A successful endovascular treatment for complicated acute type B aortic dissection.

A young male patient with Marfan syndrome suffered from acute type B aortic dissection with visceral organ malperfusion. The thoracic stent grafting was urgently performed with a successful outcome. This study reports a potential endovascular approach to treat complicated acute type B aortic dissection in a Marfan syndrome patient.

A retrospective study of bosentan in pulmonary arterial hypertension associated with congenital heart disease.

BACKGROUND: Pulmonary Arterial Hypertension (PAH) plays a significant role in morbidity and mortality of patients with congenital heart disease (CHD). Bosentan, a dual endothelin receptor antagonist has been approved for PAH patients with Eisenmenger physiology (EP). The authors retrospectively reviewed the efficacy and safety of bosentan in Thai PAH patients associated with CHD. MATERIAL AND METHOD: The study population was obtained from the databases of the CHD patients at Siriraj Hospital from October 2004 to April 2007 who received 6 months of bosentan treatment. Inclusion criteria are: CHD with Eisenmenger physiology (EP) or those with severe PAH after surgical repair or interventional cardiac catheterization. Clinical characteristics including the 6-- minute walk test (6MWT) distances, oxygen saturation (O2 sat), New York Heart Association (NYHA) functional class, and right ventricular systolic pressure (RVSP) at baseline were compared with those at 1, 3, and 6 months post bosentan treatment. Signs and symptoms of adverse events were also recorded. RESULTS: There were 11 patients from among those who fitted the inclusion criteria and whose records were examined. Their average age was 51.1 +/- 10.1 years old (13-61 years old). Patients were divided into 2 groups; Group A (6 patients) was PAH with EP and Group B (5 patients) was PAH post intervention. In group A, the 6MWT increased from 151 +/- 69 meters to 293 +/- 61 meters (p = 0.001) with the average increase of 38 +/- 61 meters. The 2O sat increased from 83 +/- 12.7% to 91.8 +/- 5.6% (p = 0.038) with an average increase of 1.4 +/- 0.07%. There was no significant change in right ventricular systolic pressure (RVSP). In group B, there was a trend in 6MWT improvement from 274 +/- 69 meters to 312 +/- 38 meters but this was not statistically different. There were improvements in the NYHA functional class in both groups. There was no significant increase in serum aminotransferase at the end of 6 months in each patient. CONCLUSION: There are benefits of bosentan for treatment of severe PAH in CHD, especially in patients with Eisenmenger physiology. Obvious benefits are an improvement of 6MWT and O2 sat.

Dobutamine stress echocardiography for the evaluation of coronary artery disease in Thai population: Siriraj experience.

OBJECTIVE: No previous report of dobutamine stress echocardiography in the Thai population has been available. The present study seeks to document the protocol, indications, results and safety of dobutamine stress echocardiography performed at Siriraj Hospital. MATERIAL AND METHOD: The authors studied 421 [mean age 67.7 +/- 11.0 years, 179 (43%) men] consecutive Thai patients undergoing dobutamine stress echocardiography at Siriraj Hospital. The protocol, indications and echocardiographic analysis were described. Clinical characteristics, hemodymanics, results and adverse effects were recorded at the time of dobutamine stress echocardiography. RESULTS: Dobutamine stress echocardiography was performed for preoperative assessment before non-cardiac surgery in 212 patients (50%), for the diagnosis of suspected Coronary Artery Disease (CAD) in 179 patients (43%), for risk stratification of CAD in 24 patients (6%), and for other reasons in six patients (1%). The results were normal and positive for inducible ischemia in 276 (66%) and 80 (19%) patients, respectively. Limiting side effects were observed in 3%. No death, myocardial infarction or life-threatening arrhythmias occurred. Transient stress-associated tachyarrhythmias, such as atrialfibrillation, nonsustained ventricular tachycardia or supraventricular tachycardia, occurred in 3.5% of patients. CONCLUSION: Dobutamine stress echocardiography was considered a safe and tolerable technique for the evaluation of CAD in Thai population.

Combination therapy of prostacyclin for pulmonary hypertension in congenital heart disease.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a recognized complication of congenital heart disease. Despite differences in etiology and pathophysiology, successful therapy for idiopathic PAH may benefit in patients with congenital heart disease. We theorized that combination of oral and aerosolization prostacyclin will benefit this group of patients in long-term. MATERIAL AND METHOD: The study design was single group and open label study with intention to treat for patients with congenital heart disease with pulmonary artery (PA pressure) more than 50% of systemic pressure. All patients were given a combination of orally given beraprost sodium and inhalation of iloprost for 12 months. Data were collected prospectively consisting of functional class, O2 saturation, 6-minute walk test and right ventricular systolic pressure (RVSP). RESULTS: There were 23 patients with an average right ventricular systolic pressure (+/- SD) of 94.8 +/- 14.5 mmHg and with average age of 27.8 +/- 14.9 years (2.5 to 50 years). The average oxygen saturation was 87.9 +/- 7 %. There were 12 patients with post surgical repair or cardiac catheterization interventional procedure and 11 with and Eisenmenger's syndrome. There were significant improvement of 6-minute-walk test from an average of 268 +/- 70 meters to 308 +/- 57 meters at the end of 12 months. The functional class of patients was also improving. However, there were no significant different in oxygen saturation. CONCLUSION: Combination therapy of oral and inhalation of aerosolized vasodilators is a fascinating concept in the therapy of pulmonary hypertension. Treated patients showed an improvement in exercise capacity and right ventricular systolic pressure without a worsening in oxygen saturation.

Changes in hematologic markers in patients with mitral stenosis after successful percutaneous balloon mitral valvuloplasty.

Systemic embolism is a major complication of mitral stenosis which is usually related to a presence of left atrial thrombus. Percutaneous balloon mitral valvuloplasty (PBMV) was previously reported to reduce the incidence of this complication. However, the mechanisms of this beneficial procedure was under investigated. The aim of this study was to investigate the changes in coagulation activity, platelet activity and endocardial function in 29 patients with mitral stenosis after successful PBMV. All subjects had good left ventricular systolic function and 48.3% had atrial fibrillation. There was a significant reduction in thrombin-antithrombin complex (TAT) after a successful procedure and the level of thrombomodulin was also significantly higher one month after successful procedure. However, the level of platelet factor 4 (PF4) and beta-thromboglobulin (beta-TG) were increased after this procedure but not achieved the statistical significance. In conclusion, successful PBMV can reduce the prethrombotic state in patients with mitral stenosis. In addition, it may improve endocardial function of the left atrium in those without atrial fibrillation.

An abnormal systemic and regional hypercoagulable state in patients with mitral stenosis.

Mitral stenosis still remains a major problem in Southeast Asia including Thailand. It contributes to the morbidity and mortality related to thromboembolism which was associated with the left atrial thrombus. However, the pathogenesis of left atrial thrombus in these patients is not completely understood. Therefore, the objective of this study was to investigate the coagulation and platelet activity including the function of the endocardium in the left atrium and peripheral circulation in patients with mitral stenosis who were free of left atrial thrombus and to compare those hematologic markers activity in the peripheral venous blood between the patients with mitral stenosis and the control. Thirty-six patients with moderate to severe mitral stenosis were included in the study. Most of the patients were in functional class II and 50 per cent had atrial fibrillation. Blood was obtained from the femoral vein, femoral artery, pulmonary artery and left atrium of these patients before heparin was administered to determine the value of various hematologic markers. In the control group, blood for determining the hematologic markers was collected only from the antecubital vein. The results of this study demonstrated that the levels of prothrombin activation fragment 1+2 (F1+2), thrombin-antithrombin III complex (TAT) and Beta-thromboglobulin (beta-TG) in the left atrium of the patients with mitral stenosis were significantly higher than those in the femoral vein and femoral artery, whereas the level of thrombomodulin was significantly lower in the left atrium compared with the femoral artery and vein. When comparing with the control group, the levels of TAT, plasminogen activator inhibitors-1 (PAI-1) from the peripheral vein were significantly higher and the level of thrombomodulin was also significantly lower in the patients with mitral stenosis. In conclusion, the present study demonstrated an abnormal hypercoagulable state of the left atrium and systemic circulation related to the abnormalities of coagulation, platelets and the endocardium which may cause the formation of left atrial thrombus in patients with mitral stenosis.

Efficacy and safety of percutaneous metallic mitral valvuloplasty in rheumatic mitral stenosis at Siriraj Hospital.

Mitral stenosis is an important problem that leads to heart failure and stroke in Thailand. The options of treatment at present are either surgical or balloon mitral commissurotomy. However, the cost of balloon is very expensive. To reduce the expense of the procedure, the authors prospectively did a study using a new device called the metallic valvulotome in symptomatic severe mitral stenosis to assess the safety, feasibility and immediate outcomes. Fifty-seven patients were included in the study. The successful outcome achieved by the metallic valvulotome was 96.2 per cent in patients in whom the procedure was actually performed. The mean transmitral gradient, left atrial pressure and pulmonary artery pressure were significantly decreased and the mitral valve area was also significantly increased. Three cases failed the procedure due to inappropriate position of the septal puncture. No death occurred in the study and complications of the procedure included only two cases of hemopericardium. In the future, it is believed that this new innovative device will provide improvement and reduce the cost of the procedure in patients with severe mitral stenosis.