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Characterisation of Endothelial Impairment in Cyanotic Congenital Heart Disease
S290
Abstracts CSANZ 2012 Abstracts
ABSTRACTS
Conclusions: Atrial septal fenestration was well tolerated in our patients. It appeared to prevent collapse in severe idiopathic pulmonary hypertension and improved exercise tolerance as measured by 6MW with the expected fall in exercise saturations. Stent fenestration may allow longer term patency and titratability of the fenestration. Performed earlier in the course of the illness it may be better tolerated, facilitating a gradual tolerance of desaturation. http://dx.doi.org/10.1016/j.hlc.2012.05.709 699 Cardiovascular Follow-Up 10–15 Years Post Kawasaki Disease A. Hohls 1,2 1 University 2 Sydney
of New South Wales, Australia Children’s Hospital, Australia
Background: The cardiovascular health of individuals with a 10–15 year history of Kawasaki Disease (KD) is poorly understood. Objectives: In this pilot study, investigators aimed to determine whether individuals with a 10–15 year history of KD demonstrated physical and/or functional cardiovascular abnormalities and infer whether cardiovascular assessment of all KD patients diagnosed in the past 10–15 years would be of benefit. Methods: We enrolled 14 adolescents (10 male, median age 15.70 years) who were diagnosed and treated with KD at the Sydney Children’s Hospital during the past 10–15 years. A relevant cardiovascular and past medical history was obtained from each patient through review of archived medical records and a questionnaire. Electrocardiogram, echocardiogram and exercise ECG (Dubowy protocol) were used to assess the physical and functional cardiovascular health of adolescents with a 10–15 year history of Kawasaki Disease. Results were reported using descriptive statistics. Results: Overall participants demonstrated excellent physical and functional cardiovascular health. No participants had developed physical signs of KD-associated cardiovascular disease and were able to complete the exercise ECG without developing signs or symptoms of cardiac insufficiency. Conclusion: Our study found that individuals with a 10–15 year history of KD have sound cardiovascular structure and function. Longitudinal studies documenting the development of cardiovascular disease in individuals with a history of KD are needed in order to conclusively define the long-term risks associated with this condition. http://dx.doi.org/10.1016/j.hlc.2012.05.710
Heart, Lung and Circulation 2012;21:S143–S316
700 Causes of Death in Repaired Tetralogy of Fallot in Adults S. O’Meagher 1,2,∗ , J. Duflou 1,3 , P. Celermajer 1,2 , R. Puranik 1,2
Choudhary 1,2 , D.
1 The
University of Sydney, Australia Prince Alfred Hospital, Sydney, Australia 3 The University of New South Wales, Australia 2 Royal
Background: Sudden cardiac death (SCD) is a recognised but rare event after repair of Tetralogy of Fallot (TOF). Only few data are available concerning the causes and circumstances of post-TOF repair deaths in adult subjects. Aims: To understand the causes of death in adults late after TOF repair and provide an estimate of the minimum incidence. Methods: Coroners’ reports from around Australia (1990–2011) were obtained from the National Coroner’s body, where repaired TOF was a recorded diagnosis, in decedents aged ≥16 years. Data from the NSW Coroner were regarded as the most comprehensive. Police reports of the circumstances of death, macroscopic and histological findings were analysed. Results: Sixteen of the 22 deaths were SCD (39 ± 16; 17–71 years of age). Eleven SCDs occurred at rest, three during exertion; circumstances were unclear in two cases. Average heart weight was 618 ± 190 g (360 ± 155 g above control values, using normative data). Microscopic RV fibrosis was more frequently identified in the SCD group than in the non-SCD group (p = 0.0012) but heart weight indexed to body surface area was not significantly different. Non-SCD causes included cardiac failure (n = 1), endocarditis (n = 1), and pulmonary artery dissection (n = 1). Using NSW prevalence estimates, this represents a death rate of approximately 1–10 in 10000 cases (recognising the likely significant underestimation resulting from ascertaining coronial cases only). Conclusions: Young adult death after TOF repair has a wide variety of causes; presumed arrhythmia leading to sudden death accounts for approximately two-thirds of cases. http://dx.doi.org/10.1016/j.hlc.2012.05.711 701 Characterisation of Endothelial Impairment in Cyanotic Congenital Heart Disease R. Cordina 1,∗ , J. Leaney 2 , S. O’Meagher 1 , S. Nahkla 3 , S. Graham 2 , D. Celermajer 1 1 Royal
Prince Alfred Hospital, Heart Research Institute and University of Sydney, Australia 2 Australian School of Advanced Medicine, Australia 3 Heart Research Institute, Sydney, Australia Introduction: Cyanotic congenital heart disease (CCHD) is associated with functional limitation and vascular events (e.g. stroke). The role of endothelial dysfunction in cyanosis-related vascular events is poorly
Abstracts CSANZ 2012 Abstracts
understood. In particular, the retinal vasculature is of interest as a window to the cerebral circulation. Methods: Fourteen adults with CCHD (40 ± 13 years) and 14 age and sex matched healthy controls had flowmediated dilatation (FMD) testing of the brachial artery and dynamic vessel analysis of the retina, including arterial flicker responses. Circulating endothelial progenitor cells (EPCs) were assessed by flow cytometry and cell culture. Results: FMD was 44% lower in the cyanosed adults (p = 0.019, n = 11). Retinal arterial response was also significantly impaired (maximal dilatation was 2.9 ± 2.9% versus 5.0 ± 2.1%, p = 0.05, area under the curve (AUC) was 46% lower than controls, p = 0.046, n = 12). Similarly maximal venous dilatation was impaired (3.39 ± 0.95% versus 5.22 ± 2.33%, p = 0.02, AUC was 36% lower, p = 0.02) as was post-stimulation venous constriction (0.67 ± 0.75% versus 0.96 ± 0.52%, p = 0.02). Circulating CD34+45dim133+ cells and CD34+45dim133+VEGF+ progenitor cells were also reduced with cyanosis (p = 0.02, n = 11 for both). The number of EPCs cultured from peripheral blood also tended to be lower with cyanosis (p = 0.07, n = 11). Conclusions: Endothelial function is impaired in the systemic arteries and retinal arteries and veins in adults with cyanotic CHD, suggesting a widespread endotheliopathy. Diminished numbers of endothelial progenitor cells may provide an explanation warranting further study. http://dx.doi.org/10.1016/j.hlc.2012.05.712 702 Comparison of Long-Term Outcomes Following Percutaneous and Surgical Treatment of Congenital Pulmonary Valve Stenosis H. Samardhi ∗ , S. Luis, S. Lee, J. Voityl, C. Mengel, O. Raffel, D. Radford The Prince Charles Hospital, Australia Introduction: The long-term outcome of patients with congenital pulmonary valve stenosis treated by balloon valvuloplasty or surgery is excellent. Late pulmonary regurgitation and consequent right ventricular dysfunction however remain a significant concern. Methods 227 patients who underwent surgical or percutaneous treatment for isolated pulmonary valve stenosis were reviewed. Demographics, pre procedure echocardiographic parameters, re intervention rates, degree of pulmonary regurgitation and mortality were compared. Results: The results are shown below.
Number of patients Male gender Median follow up (IQR) (months) Peak pre-procedure gradient (IQR) Redo procedure Pulmonary regurgitation (≥Grade 2/4) Mortality
Percutaneous
Surgical
106 45% 61.5 (91) 24 (29) mmHg 19% 35% 0%
121 54% 141.8 (171.6) 80 (35) mmHg 26% 46% 5%
All patients in the percutaneous group and >95% of patients in the surgical group were in NYHA class I at fol-
S291
low up. There was no statistically significant difference in redo procedure rate (p = 0.31) or pulmonary regurgitation (p = 0.15) at follow up between both groups. Conclusions: The long-term incidence of pulmonary regurgitation following both surgical and percutaneous treatment of isolated pulmonary stenosis is significant. This is associated with the need for further intervention by pulmonary valve replacement. The majority of patients are asymptomatic with low overall mortality and a good long-term prognosis. http://dx.doi.org/10.1016/j.hlc.2012.05.713 703 Derivation of Transthoracic Echocardiographic Reference Ranges for Left Ventricular, Left Atrial and Aortic Root M-Mode Dimensions in Preterm Infants S. Farrell 1,2,∗ , S. Hope 1,2 1 Monash Cardiovascular Research Centre, Department of Medicine (SCS), Monash University, Australia 2 MonashHEART, Southern Health, Melbourne, Australia
Background: M-mode measurement of left heart dimensions is routine in transthoracic echocardiography. Reference ranges are well defined in adults and paediatric patients. No such data have been published in preterm neonates. Methods: All echocardiographic studies performed in Monash Newborn between 3rd December 2007 and 2nd December 2010 were identified. Infants with congenital heart disease, a greater than small sized patent ductus arteriosus or technically inadequate M-mode images were excluded. M-mode left ventricular, left atrial and aortic root measurements were made retrospectively by an experienced cardiac sonographer. Analysis was limited to infants with a birth or current weight ≤3.5 kg and ≤45 completed weeks of corrected gestational age (cGA) at the time of echocardiography. Analysis was performed in SPSS. Regression curves were modelled with respect to weight and cGA and reference curves for ±1 and ±2 standard deviations calculated using the method of Altman (Statistics in Medicine, 1993). Results: 234 studies were identified, mean GA at birth 30 weeks, birth weight 1.5 ± 0.9 kg. All measured parameters increased with weight and cGA (all P < 0.001). Standard deviation increased, but did not reach statistical significance over the given weight and cGA range. Examples for Left ventricular end diastolic dimension (LVEDd), posterior wall and aortic root dimensions below. Conclusions: The derivation of reference ranges for left ventricular dimensions in preterm infants will permit better recognition of abnormalities and better description and documentation of change with the opportunity to calculate Z scores.
ABSTRACTS
Heart, Lung and Circulation 2012;21:S143–S316
Abstracts CSANZ 2012 Abstracts
ABSTRACTS
Conclusions: Atrial septal fenestration was well tolerated in our patients. It appeared to prevent collapse in severe idiopathic pulmonary hypertension and improved exercise tolerance as measured by 6MW with the expected fall in exercise saturations. Stent fenestration may allow longer term patency and titratability of the fenestration. Performed earlier in the course of the illness it may be better tolerated, facilitating a gradual tolerance of desaturation. http://dx.doi.org/10.1016/j.hlc.2012.05.709 699 Cardiovascular Follow-Up 10–15 Years Post Kawasaki Disease A. Hohls 1,2 1 University 2 Sydney
of New South Wales, Australia Children’s Hospital, Australia
Background: The cardiovascular health of individuals with a 10–15 year history of Kawasaki Disease (KD) is poorly understood. Objectives: In this pilot study, investigators aimed to determine whether individuals with a 10–15 year history of KD demonstrated physical and/or functional cardiovascular abnormalities and infer whether cardiovascular assessment of all KD patients diagnosed in the past 10–15 years would be of benefit. Methods: We enrolled 14 adolescents (10 male, median age 15.70 years) who were diagnosed and treated with KD at the Sydney Children’s Hospital during the past 10–15 years. A relevant cardiovascular and past medical history was obtained from each patient through review of archived medical records and a questionnaire. Electrocardiogram, echocardiogram and exercise ECG (Dubowy protocol) were used to assess the physical and functional cardiovascular health of adolescents with a 10–15 year history of Kawasaki Disease. Results were reported using descriptive statistics. Results: Overall participants demonstrated excellent physical and functional cardiovascular health. No participants had developed physical signs of KD-associated cardiovascular disease and were able to complete the exercise ECG without developing signs or symptoms of cardiac insufficiency. Conclusion: Our study found that individuals with a 10–15 year history of KD have sound cardiovascular structure and function. Longitudinal studies documenting the development of cardiovascular disease in individuals with a history of KD are needed in order to conclusively define the long-term risks associated with this condition. http://dx.doi.org/10.1016/j.hlc.2012.05.710
Heart, Lung and Circulation 2012;21:S143–S316
700 Causes of Death in Repaired Tetralogy of Fallot in Adults S. O’Meagher 1,2,∗ , J. Duflou 1,3 , P. Celermajer 1,2 , R. Puranik 1,2
Choudhary 1,2 , D.
1 The
University of Sydney, Australia Prince Alfred Hospital, Sydney, Australia 3 The University of New South Wales, Australia 2 Royal
Background: Sudden cardiac death (SCD) is a recognised but rare event after repair of Tetralogy of Fallot (TOF). Only few data are available concerning the causes and circumstances of post-TOF repair deaths in adult subjects. Aims: To understand the causes of death in adults late after TOF repair and provide an estimate of the minimum incidence. Methods: Coroners’ reports from around Australia (1990–2011) were obtained from the National Coroner’s body, where repaired TOF was a recorded diagnosis, in decedents aged ≥16 years. Data from the NSW Coroner were regarded as the most comprehensive. Police reports of the circumstances of death, macroscopic and histological findings were analysed. Results: Sixteen of the 22 deaths were SCD (39 ± 16; 17–71 years of age). Eleven SCDs occurred at rest, three during exertion; circumstances were unclear in two cases. Average heart weight was 618 ± 190 g (360 ± 155 g above control values, using normative data). Microscopic RV fibrosis was more frequently identified in the SCD group than in the non-SCD group (p = 0.0012) but heart weight indexed to body surface area was not significantly different. Non-SCD causes included cardiac failure (n = 1), endocarditis (n = 1), and pulmonary artery dissection (n = 1). Using NSW prevalence estimates, this represents a death rate of approximately 1–10 in 10000 cases (recognising the likely significant underestimation resulting from ascertaining coronial cases only). Conclusions: Young adult death after TOF repair has a wide variety of causes; presumed arrhythmia leading to sudden death accounts for approximately two-thirds of cases. http://dx.doi.org/10.1016/j.hlc.2012.05.711 701 Characterisation of Endothelial Impairment in Cyanotic Congenital Heart Disease R. Cordina 1,∗ , J. Leaney 2 , S. O’Meagher 1 , S. Nahkla 3 , S. Graham 2 , D. Celermajer 1 1 Royal
Prince Alfred Hospital, Heart Research Institute and University of Sydney, Australia 2 Australian School of Advanced Medicine, Australia 3 Heart Research Institute, Sydney, Australia Introduction: Cyanotic congenital heart disease (CCHD) is associated with functional limitation and vascular events (e.g. stroke). The role of endothelial dysfunction in cyanosis-related vascular events is poorly
Abstracts CSANZ 2012 Abstracts
understood. In particular, the retinal vasculature is of interest as a window to the cerebral circulation. Methods: Fourteen adults with CCHD (40 ± 13 years) and 14 age and sex matched healthy controls had flowmediated dilatation (FMD) testing of the brachial artery and dynamic vessel analysis of the retina, including arterial flicker responses. Circulating endothelial progenitor cells (EPCs) were assessed by flow cytometry and cell culture. Results: FMD was 44% lower in the cyanosed adults (p = 0.019, n = 11). Retinal arterial response was also significantly impaired (maximal dilatation was 2.9 ± 2.9% versus 5.0 ± 2.1%, p = 0.05, area under the curve (AUC) was 46% lower than controls, p = 0.046, n = 12). Similarly maximal venous dilatation was impaired (3.39 ± 0.95% versus 5.22 ± 2.33%, p = 0.02, AUC was 36% lower, p = 0.02) as was post-stimulation venous constriction (0.67 ± 0.75% versus 0.96 ± 0.52%, p = 0.02). Circulating CD34+45dim133+ cells and CD34+45dim133+VEGF+ progenitor cells were also reduced with cyanosis (p = 0.02, n = 11 for both). The number of EPCs cultured from peripheral blood also tended to be lower with cyanosis (p = 0.07, n = 11). Conclusions: Endothelial function is impaired in the systemic arteries and retinal arteries and veins in adults with cyanotic CHD, suggesting a widespread endotheliopathy. Diminished numbers of endothelial progenitor cells may provide an explanation warranting further study. http://dx.doi.org/10.1016/j.hlc.2012.05.712 702 Comparison of Long-Term Outcomes Following Percutaneous and Surgical Treatment of Congenital Pulmonary Valve Stenosis H. Samardhi ∗ , S. Luis, S. Lee, J. Voityl, C. Mengel, O. Raffel, D. Radford The Prince Charles Hospital, Australia Introduction: The long-term outcome of patients with congenital pulmonary valve stenosis treated by balloon valvuloplasty or surgery is excellent. Late pulmonary regurgitation and consequent right ventricular dysfunction however remain a significant concern. Methods 227 patients who underwent surgical or percutaneous treatment for isolated pulmonary valve stenosis were reviewed. Demographics, pre procedure echocardiographic parameters, re intervention rates, degree of pulmonary regurgitation and mortality were compared. Results: The results are shown below.
Number of patients Male gender Median follow up (IQR) (months) Peak pre-procedure gradient (IQR) Redo procedure Pulmonary regurgitation (≥Grade 2/4) Mortality
Percutaneous
Surgical
106 45% 61.5 (91) 24 (29) mmHg 19% 35% 0%
121 54% 141.8 (171.6) 80 (35) mmHg 26% 46% 5%
All patients in the percutaneous group and >95% of patients in the surgical group were in NYHA class I at fol-
S291
low up. There was no statistically significant difference in redo procedure rate (p = 0.31) or pulmonary regurgitation (p = 0.15) at follow up between both groups. Conclusions: The long-term incidence of pulmonary regurgitation following both surgical and percutaneous treatment of isolated pulmonary stenosis is significant. This is associated with the need for further intervention by pulmonary valve replacement. The majority of patients are asymptomatic with low overall mortality and a good long-term prognosis. http://dx.doi.org/10.1016/j.hlc.2012.05.713 703 Derivation of Transthoracic Echocardiographic Reference Ranges for Left Ventricular, Left Atrial and Aortic Root M-Mode Dimensions in Preterm Infants S. Farrell 1,2,∗ , S. Hope 1,2 1 Monash Cardiovascular Research Centre, Department of Medicine (SCS), Monash University, Australia 2 MonashHEART, Southern Health, Melbourne, Australia
Background: M-mode measurement of left heart dimensions is routine in transthoracic echocardiography. Reference ranges are well defined in adults and paediatric patients. No such data have been published in preterm neonates. Methods: All echocardiographic studies performed in Monash Newborn between 3rd December 2007 and 2nd December 2010 were identified. Infants with congenital heart disease, a greater than small sized patent ductus arteriosus or technically inadequate M-mode images were excluded. M-mode left ventricular, left atrial and aortic root measurements were made retrospectively by an experienced cardiac sonographer. Analysis was limited to infants with a birth or current weight ≤3.5 kg and ≤45 completed weeks of corrected gestational age (cGA) at the time of echocardiography. Analysis was performed in SPSS. Regression curves were modelled with respect to weight and cGA and reference curves for ±1 and ±2 standard deviations calculated using the method of Altman (Statistics in Medicine, 1993). Results: 234 studies were identified, mean GA at birth 30 weeks, birth weight 1.5 ± 0.9 kg. All measured parameters increased with weight and cGA (all P < 0.001). Standard deviation increased, but did not reach statistical significance over the given weight and cGA range. Examples for Left ventricular end diastolic dimension (LVEDd), posterior wall and aortic root dimensions below. Conclusions: The derivation of reference ranges for left ventricular dimensions in preterm infants will permit better recognition of abnormalities and better description and documentation of change with the opportunity to calculate Z scores.
ABSTRACTS
Heart, Lung and Circulation 2012;21:S143–S316