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S28-1 Pathophysiology and Clinical Presentation
S40
Abstracts of the 17th Asian Pacific Congress of Cardiology
Symposium 28. Pediatric Cardiology 1: Diagnosis and Management of Cardiac Failure in Adults with Congenital Heart Disease S28-1 Pathophysiology and Clinical Presentation Koichiro Niwa. Department of Adult Congenital Heart Disease, Chiba Cardiovascular Center, Chiba, Japan Any form of heart disease including congenital heart disease (CHD) can lead to the development of heart failure, which is characterized by symptoms, exercise limitation, ventricular dysfunction and neurohormonal activation. In patients with CHD, specific hemodynamics of background CHD can modify the heart failure. Myocardial dysfunction can be induced by several factors: change in contractility, metabolism and growth in myocyte and decrease in cardiac output. Few other factors are further included such as neurohormonal activation, change in gene expression, upregulation of fibroblast, collagen deposition and chronic effect of fibrosis, infarction, operative insult and cyanosis. In adult with CHD, subpulmonary or especially subaortc (systemic) right ventriclar physiology can be affecting heart failure and need the special management. Finally, heart failure in adult with CHD is characterised as follows: 1. Chronic heart failure syndrome is widespread. RV failure is common and specific in this patient population. 2. Chronotropic incompetence and exercise intolerance are present even in asymptomatic patients. Autonomic nervous dysfunction is common. These are observed in adult CHD patients regardless of morphology in CHD. 3. Atrial/ventricular chamber dilatation, fibrosis and dysfunction may become risk factors for cardiac failure, sustained arrhythmia and sudden death. 4. However, true clinical implication of these abnormalities remains unknown. S28-2 Heart Failure in Adults with Congenital Heart Disease: Defining the Problem Daniel J. Murphy Jr.. Stanford University, Palo Alto, California, USA Surgical treatment of congenital heart disease (CHD) ensures adult survival in the majority of patients, but is rarely curative. Life expectancy is significantly shortened and the major causes of death are congestive heart failure and sudden death. Heart failure is defined by: (1) a background of cardiac disease, (2) exercise intolerance, and (3) activation of neurohormones and other substances. Based on this definition, CHD results in a chronic heart failure state. Nearly every form of CHD contains structural and functional elements that produce congestive heart failure. These features include valvar abnormalities, volume overload, pressure overload, and damaged myocardium. There is evidence that exercise intolerance, although frequently unrecognized by the patient or the care provider, is ubiquitous in adults with CHD. However, the presence of symptoms is not required to define heart failure and the onset of symptoms may not represent a pathological event. Neurohormonal activation in adult CHD relates to symptom severity and ventricular dysfunction, not necessarily to anatomic substrate. In addition, levels of other substances, such as cytokines, correlate with functional status. The population of adults with CHD and heart failure is younger and more complex than other heart failure populations. We have limited tools to assess ventricular function in this population and are frequently limited to observing the effects of impaired cardiovascular performance on other organs.
Our responsibilities include: (1) to provide lifetime care for individuals born with CHD, (2) to assess such patients longitudinally, (3) to intervene in a timely fashion, and finally, (4) to develop interdisciplinary and inter-institutional alliances for clinical care and research. S28-3 Management of Late RV failure from Un-Operated Congenital Heart Disease in Adults with Pulmonary Arterial Hypertension Kritvikrom Durongpisitkul. Division of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand Pulmonary Arterial Hypertension (PAH) and right ventricular (RV) failure are the crucial problem in patients with congenital heart disease (CHD) in Thailand. Our database at Siriraj hospital has shown that the number of PAH associated with CHD tend to be increasing every year. From 2005 to 2008, the annual percentage of the admitted patients who were diagnosed with PAH in CHD increased from 13% to 14% and 22% respectively. Compared to studies in European countries, there is only 10 15% of PAH associated with CHD. Several of our adult congenital heart patients presented first time in our hospital with severe PAH and RV failure. The managements included careful evaluation with imaging studies, acute pulmonary vasodilator testing with or without balloon occlusion of lesions during cardiac catheterization. Majority of patients can have their lesion elective closed and continue taking selective pulmonary vasodilator therapies. Previously, we reported the effect of 12 months combination therapy of oral beraprost and twice daily inhaled iloprost in 23 CHD patients with severe PAH. There were 12 patients who had PAH post cardiac catheterization interventional procedure and 11 patients with Eisenmenger physiology (EP). There was a significant improvement of 6MWT from an average of 268±70 meters to 308±57 meters at the end of 12 months. However, there was no significant difference in oxygen saturation. It appeared that there was some limitation, primarily due to side effects, regarding using combination therapy (beraprost or iloprost). In 2003, Barst reported an improvement of 6MWT in PAH patients using beraprost. The improvement in 6MWT occurring during early phases of treatment in WHO functional class II or III patients, but this effect attenuated with time. The effect of beraprost appeared to be effective in the short term, but not over time in patients with WHO functional class II or III. We have also recently reported the efficacy and safety of bosentan in Thai PAH patients associated with CHD (6 patients with Eisenmenger physiology; EP; and 5 patients had PAH post cardiac intervention). We are currently evaluate the effect of 1 year therapy of EP patients on right ventricle mass index and the change in Septal Delay Contrast enhancement using cardiac MRI. S28-4 MRI Assessment of Biventricular Function and Pulmonary Regurgitation in Patients with Tetralogy of Fallot After Surgical Treatment Yeon Hyeon Choe. Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea Surgical management of tetralogy of Fallot (TOF) results in anatomic and functional abnormalities in the majority of patients. Of all the residual lesions, pulmonary regurgitation (PR) is the most important. Moderate or severe left ventricle (LV) or RV systolic dysfunction is independently associated with impaired clinical status in long-term survivors of TOF repair. The close relationship between LV ejection fraction
Abstracts of the 17th Asian Pacific Congress of Cardiology
Symposium 28. Pediatric Cardiology 1: Diagnosis and Management of Cardiac Failure in Adults with Congenital Heart Disease S28-1 Pathophysiology and Clinical Presentation Koichiro Niwa. Department of Adult Congenital Heart Disease, Chiba Cardiovascular Center, Chiba, Japan Any form of heart disease including congenital heart disease (CHD) can lead to the development of heart failure, which is characterized by symptoms, exercise limitation, ventricular dysfunction and neurohormonal activation. In patients with CHD, specific hemodynamics of background CHD can modify the heart failure. Myocardial dysfunction can be induced by several factors: change in contractility, metabolism and growth in myocyte and decrease in cardiac output. Few other factors are further included such as neurohormonal activation, change in gene expression, upregulation of fibroblast, collagen deposition and chronic effect of fibrosis, infarction, operative insult and cyanosis. In adult with CHD, subpulmonary or especially subaortc (systemic) right ventriclar physiology can be affecting heart failure and need the special management. Finally, heart failure in adult with CHD is characterised as follows: 1. Chronic heart failure syndrome is widespread. RV failure is common and specific in this patient population. 2. Chronotropic incompetence and exercise intolerance are present even in asymptomatic patients. Autonomic nervous dysfunction is common. These are observed in adult CHD patients regardless of morphology in CHD. 3. Atrial/ventricular chamber dilatation, fibrosis and dysfunction may become risk factors for cardiac failure, sustained arrhythmia and sudden death. 4. However, true clinical implication of these abnormalities remains unknown. S28-2 Heart Failure in Adults with Congenital Heart Disease: Defining the Problem Daniel J. Murphy Jr.. Stanford University, Palo Alto, California, USA Surgical treatment of congenital heart disease (CHD) ensures adult survival in the majority of patients, but is rarely curative. Life expectancy is significantly shortened and the major causes of death are congestive heart failure and sudden death. Heart failure is defined by: (1) a background of cardiac disease, (2) exercise intolerance, and (3) activation of neurohormones and other substances. Based on this definition, CHD results in a chronic heart failure state. Nearly every form of CHD contains structural and functional elements that produce congestive heart failure. These features include valvar abnormalities, volume overload, pressure overload, and damaged myocardium. There is evidence that exercise intolerance, although frequently unrecognized by the patient or the care provider, is ubiquitous in adults with CHD. However, the presence of symptoms is not required to define heart failure and the onset of symptoms may not represent a pathological event. Neurohormonal activation in adult CHD relates to symptom severity and ventricular dysfunction, not necessarily to anatomic substrate. In addition, levels of other substances, such as cytokines, correlate with functional status. The population of adults with CHD and heart failure is younger and more complex than other heart failure populations. We have limited tools to assess ventricular function in this population and are frequently limited to observing the effects of impaired cardiovascular performance on other organs.
Our responsibilities include: (1) to provide lifetime care for individuals born with CHD, (2) to assess such patients longitudinally, (3) to intervene in a timely fashion, and finally, (4) to develop interdisciplinary and inter-institutional alliances for clinical care and research. S28-3 Management of Late RV failure from Un-Operated Congenital Heart Disease in Adults with Pulmonary Arterial Hypertension Kritvikrom Durongpisitkul. Division of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand Pulmonary Arterial Hypertension (PAH) and right ventricular (RV) failure are the crucial problem in patients with congenital heart disease (CHD) in Thailand. Our database at Siriraj hospital has shown that the number of PAH associated with CHD tend to be increasing every year. From 2005 to 2008, the annual percentage of the admitted patients who were diagnosed with PAH in CHD increased from 13% to 14% and 22% respectively. Compared to studies in European countries, there is only 10 15% of PAH associated with CHD. Several of our adult congenital heart patients presented first time in our hospital with severe PAH and RV failure. The managements included careful evaluation with imaging studies, acute pulmonary vasodilator testing with or without balloon occlusion of lesions during cardiac catheterization. Majority of patients can have their lesion elective closed and continue taking selective pulmonary vasodilator therapies. Previously, we reported the effect of 12 months combination therapy of oral beraprost and twice daily inhaled iloprost in 23 CHD patients with severe PAH. There were 12 patients who had PAH post cardiac catheterization interventional procedure and 11 patients with Eisenmenger physiology (EP). There was a significant improvement of 6MWT from an average of 268±70 meters to 308±57 meters at the end of 12 months. However, there was no significant difference in oxygen saturation. It appeared that there was some limitation, primarily due to side effects, regarding using combination therapy (beraprost or iloprost). In 2003, Barst reported an improvement of 6MWT in PAH patients using beraprost. The improvement in 6MWT occurring during early phases of treatment in WHO functional class II or III patients, but this effect attenuated with time. The effect of beraprost appeared to be effective in the short term, but not over time in patients with WHO functional class II or III. We have also recently reported the efficacy and safety of bosentan in Thai PAH patients associated with CHD (6 patients with Eisenmenger physiology; EP; and 5 patients had PAH post cardiac intervention). We are currently evaluate the effect of 1 year therapy of EP patients on right ventricle mass index and the change in Septal Delay Contrast enhancement using cardiac MRI. S28-4 MRI Assessment of Biventricular Function and Pulmonary Regurgitation in Patients with Tetralogy of Fallot After Surgical Treatment Yeon Hyeon Choe. Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea Surgical management of tetralogy of Fallot (TOF) results in anatomic and functional abnormalities in the majority of patients. Of all the residual lesions, pulmonary regurgitation (PR) is the most important. Moderate or severe left ventricle (LV) or RV systolic dysfunction is independently associated with impaired clinical status in long-term survivors of TOF repair. The close relationship between LV ejection fraction