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OP-162 Pulmonary Arterial Hypertension in Congenital Heart Defects
MARCH 26e29, 2015
Figure. 49 (31-80), 34 (22-50) mmHg, to 29 (14-63) and 6 (2-27) mmHg after treatment, respectively (p:0,005 for each). The Miller score was significantly reduced from 21 (13-28) to 8 (4-16) (p¼0,005). All patients except one survived to hospital discharge. This patient died of liver failure seven days after USAT. We suspected no recurrent PE at 6 month follow-up. No minor or major bleeding complications occurred in our patients. Conclusions: USAT improved RVD, decreased clot burden without any bleeding complications in intermediate risk PE patients. USAT therapy may be a safe and effective treatment option for intermediate risk PE patients.
- OP-162 Pulmonary Arterial Hypertension in Congenital Heart Defects. Zijo Begic, Senka Mesihovic Dinarevic, Almira Kadic, Mirza Halimic. Paediatric Clinic, CCU Sarajevo, Sarajevo, Bosnia and Herzegovina. Objective: Pulmonary arterial hypertension (PAH) is hemodynamically pathophysiological condition defined as increase of secondary pulmonary arterial pressure, at rest over 25, or after effort over 30 mmHg, measured by right heart catheterization. In everyday pediatric clinical practice, common problem is the estimation of PAH associated with congenital heart defects (CHD), where the increase in pulmonary vascular resistance leads to irreversible changes in pulmonary vasculature (Eisenmenger’s syndrome), which is a contraindication for surgical treatment. The treatment of PAH is full of contradictions, uncertainty and inexperience. The aim of this study was to point out the problem, to show the importance of early diagnosis, assessment and treatment of PAH associated with CHD. Methods: The study included data of registered patients with PAH associated with CHD from PAH register and protocols of catheterized children (311 catheterizations, 63.75% diagnostic) from the period May 1998. -January 2015. in the Clinical Center University of Sarajevo. Results: In mentioned period, from 198 diagnostic catheterization, in 61 patients with congenital heart defects, catheterization was performed in order to evaluate PAH, to decide on its operability. Age of catheterized patients ranged from 3 months to 19 years, with a preponderance of female gender (55.7%). In 18 (29.5%) patients, the existence of fixed (irreversible) pulmonary arterial hypertension was determined, which is a contraindication for correction. There were 8 patients with ventral septal defect, 2 with Atrioventricular septal defect, 2 with aortopulmonary window, 2 with
complex forms of congenital heart defects (Double outlet right ventricle), one with Patent ductus arteriosus (with combination of coarctation of the aorta and subvalvular aortic stenosis). Of these 18 patients, which were monitored and treated, only 4 PAH were not proven by catheterization. Fixed PAH was associated with Down syndrome in 14 patients. Of these patients, 43 patients after catheterization were diagnosed with unfixed (reversible) PAH, 14 children were not operated, and are scheduled for surgical treatment. Conclusions: Prevention in the form of early detection of PAH, prompt diagnosis and operational correction of CHD with left-toright shunt, especially of risk groups (chromosomopathies) appears to be the main task of modern pediatric cardiology. Pediatric catheterization remains to be the gold standard in the assessment of operability, or fixation of pulmonary hypertension. Modern treatment of fixed pulmonary hypertension, which implies the use of prostanoids, inhibitors of phosphodiesterase type-5 and endothelin receptors antagonists, alone or in combination, are partially used in B&H.
- OP-163 Evaluation of First Year Outcomes of First Line Pediatric Pulmonary Arteriel Hypertension Therapy. Ays¸enur Pac, Fatih Atik, Serhat Koca, Vedat Kavurt, Denizhan Bagrul, Ajda Mihcioglu. Department of Pediatric Cardiology, Turkey Education and Research Hospital, Ankara, Turkey. Purpose: Pulmonary arteriel hypertension (PAH) is one of the most important childhood health problem associated with significant morbidity and mortality. Despite improvements, best management of pediatric PAH is still unclear, especially secondary to congenital heart disease (CHD). The aim of our study was to assess the outcomes of various PAH targeted therapies in paediatric patients. Methods: Functional status, six-minute walk test, mean BNP values, haemodynamic and echocardiographic data of 51 paediatric idiopathic PAH and PAH associated with CHD patients admitted between 2007 and 2014 were reviewed at diagnosis and at first year follow-up at our medical center. Patients were groupped according to first line therapy started as mono or combination of PAH spesific agents.
Figure. Results at the diagnosis and first year follow up of non e invasive and invasive assessment of patients according to their prescriptions for PAH spesific treatment. ER: Endothelin receptor, NYHA: New York Heart Association, 6MWT: Six minute walk test, mPAP: mean pulmonary arteriel pressure, PVRI: Pulmonary vascular resistance index, mRAP: Mean right atrial pressure, TAPSE: Tricuspid valve annular plane excursion, RVFWT: Right ventricular free Wall thickness, RVSP: Right ventricular systolic pressure, RVFAC: Right ventricular fractional area change, LV EF: Left ventricular ejection fraction.
The American Journal of Cardiologyâ MARCH 26e29, 2015 11th INTERNATIONAL CONGRESS OF UPDATE IN CARDIOLOGY AND CARDIOVASCULAR SURGERY ABSTRACTS / Oral
S71
O R A L A B S T R A C T S
Figure. 49 (31-80), 34 (22-50) mmHg, to 29 (14-63) and 6 (2-27) mmHg after treatment, respectively (p:0,005 for each). The Miller score was significantly reduced from 21 (13-28) to 8 (4-16) (p¼0,005). All patients except one survived to hospital discharge. This patient died of liver failure seven days after USAT. We suspected no recurrent PE at 6 month follow-up. No minor or major bleeding complications occurred in our patients. Conclusions: USAT improved RVD, decreased clot burden without any bleeding complications in intermediate risk PE patients. USAT therapy may be a safe and effective treatment option for intermediate risk PE patients.
- OP-162 Pulmonary Arterial Hypertension in Congenital Heart Defects. Zijo Begic, Senka Mesihovic Dinarevic, Almira Kadic, Mirza Halimic. Paediatric Clinic, CCU Sarajevo, Sarajevo, Bosnia and Herzegovina. Objective: Pulmonary arterial hypertension (PAH) is hemodynamically pathophysiological condition defined as increase of secondary pulmonary arterial pressure, at rest over 25, or after effort over 30 mmHg, measured by right heart catheterization. In everyday pediatric clinical practice, common problem is the estimation of PAH associated with congenital heart defects (CHD), where the increase in pulmonary vascular resistance leads to irreversible changes in pulmonary vasculature (Eisenmenger’s syndrome), which is a contraindication for surgical treatment. The treatment of PAH is full of contradictions, uncertainty and inexperience. The aim of this study was to point out the problem, to show the importance of early diagnosis, assessment and treatment of PAH associated with CHD. Methods: The study included data of registered patients with PAH associated with CHD from PAH register and protocols of catheterized children (311 catheterizations, 63.75% diagnostic) from the period May 1998. -January 2015. in the Clinical Center University of Sarajevo. Results: In mentioned period, from 198 diagnostic catheterization, in 61 patients with congenital heart defects, catheterization was performed in order to evaluate PAH, to decide on its operability. Age of catheterized patients ranged from 3 months to 19 years, with a preponderance of female gender (55.7%). In 18 (29.5%) patients, the existence of fixed (irreversible) pulmonary arterial hypertension was determined, which is a contraindication for correction. There were 8 patients with ventral septal defect, 2 with Atrioventricular septal defect, 2 with aortopulmonary window, 2 with
complex forms of congenital heart defects (Double outlet right ventricle), one with Patent ductus arteriosus (with combination of coarctation of the aorta and subvalvular aortic stenosis). Of these 18 patients, which were monitored and treated, only 4 PAH were not proven by catheterization. Fixed PAH was associated with Down syndrome in 14 patients. Of these patients, 43 patients after catheterization were diagnosed with unfixed (reversible) PAH, 14 children were not operated, and are scheduled for surgical treatment. Conclusions: Prevention in the form of early detection of PAH, prompt diagnosis and operational correction of CHD with left-toright shunt, especially of risk groups (chromosomopathies) appears to be the main task of modern pediatric cardiology. Pediatric catheterization remains to be the gold standard in the assessment of operability, or fixation of pulmonary hypertension. Modern treatment of fixed pulmonary hypertension, which implies the use of prostanoids, inhibitors of phosphodiesterase type-5 and endothelin receptors antagonists, alone or in combination, are partially used in B&H.
- OP-163 Evaluation of First Year Outcomes of First Line Pediatric Pulmonary Arteriel Hypertension Therapy. Ays¸enur Pac, Fatih Atik, Serhat Koca, Vedat Kavurt, Denizhan Bagrul, Ajda Mihcioglu. Department of Pediatric Cardiology, Turkey Education and Research Hospital, Ankara, Turkey. Purpose: Pulmonary arteriel hypertension (PAH) is one of the most important childhood health problem associated with significant morbidity and mortality. Despite improvements, best management of pediatric PAH is still unclear, especially secondary to congenital heart disease (CHD). The aim of our study was to assess the outcomes of various PAH targeted therapies in paediatric patients. Methods: Functional status, six-minute walk test, mean BNP values, haemodynamic and echocardiographic data of 51 paediatric idiopathic PAH and PAH associated with CHD patients admitted between 2007 and 2014 were reviewed at diagnosis and at first year follow-up at our medical center. Patients were groupped according to first line therapy started as mono or combination of PAH spesific agents.
Figure. Results at the diagnosis and first year follow up of non e invasive and invasive assessment of patients according to their prescriptions for PAH spesific treatment. ER: Endothelin receptor, NYHA: New York Heart Association, 6MWT: Six minute walk test, mPAP: mean pulmonary arteriel pressure, PVRI: Pulmonary vascular resistance index, mRAP: Mean right atrial pressure, TAPSE: Tricuspid valve annular plane excursion, RVFWT: Right ventricular free Wall thickness, RVSP: Right ventricular systolic pressure, RVFAC: Right ventricular fractional area change, LV EF: Left ventricular ejection fraction.
The American Journal of Cardiologyâ MARCH 26e29, 2015 11th INTERNATIONAL CONGRESS OF UPDATE IN CARDIOLOGY AND CARDIOVASCULAR SURGERY ABSTRACTS / Oral
S71
O R A L A B S T R A C T S