Closing ASDs with pulmonary hypertension Shakeel A Qureshi Evelina Children s Hospital London Ho Chi Minh, Vietnam, January 2012
ACC/AHA 2008 Guidelines ASD closure Closure is indicated for right atrial and right ventricular enlargement Paradoxical embolism Documented orthodeoxia-platypnoea Net L-to-R shunt, PAP < 2/3 rd systemic and PVR less than 2/3 rd SVR or responsive to pulmonary vasodilator therapy Surgical closure in patients who have concomitant repairable defects or when anatomy of the defect precludes the use of available devices
ASDs with pulmonary hypertension PA pressure > systemic pressure Right-to-left shunt Net left-to-right shunt PA pressure between ½ to ¾ systemic PA pressure < ½ systemic PA pressure moderately elevated with LV diastolic dysfunction
ASDs and pulmonary hypertension Pulmonary hypertension may be unrelated to size of ASD PA pressure normal PA pressure >1/2 systemic
Natural history of ASDs Survival up to 94 years of age reported, so normal life span is possible During the first decade of life most patients with ASDs are asymptomatic Around 50% begin to complain of exertional dyspnoea by age of 20 yrs, and nearly 100% do so by 6 th decade Natural history of ASDs shows 75% mortality in patients over 50 years old and almost 90% mortality in those with an uncorrected defect over the age of 60
ASDs with pulmonary hypertension Knight and Lenox, 1972 Moderate increase in pulmonary artery pressure before operation (up to 65 mm Hg systolic) did not increase overall hospital mortality Operating on patients with severe pulmonary hypertension was too hazardous Later studies also showed that pts in NYHA III or IV had higher mortality with surgery
Natural history of ASDs Comparison of ASDs treated medically vs surgically when diagnosed age > 25 yrs All patients with ASD followed up since 1955, who had reached a current age of over 45 years 34 medical and 48 surgical patients with a mean follow up of 25 years Shah et al, 1994
Natural history of ASDs Medical treatment (Gp I) in 34, surgery (Gp II) in 48 Age mean 38.6 (range 25-54 yrs) in Gp I, 36.2 (range 26-51 yrs) in Gp II 74% in Gp I and 71% in Gp II in NYHA I Qp:Qs 2.5:1 in both groups Mean PA pressure: 34 (range 22-42) mmhg in Gp I, 30 (range 20-44) mmhg in Gp II Shah et al, 1994
Natural history of ASDs - incidence of atrial fibrillation Incidence of atrial fibrillation is similar in both medical and surgical pts So development of AF was neither reduced nor delayed by surgery Shah et al, 1994
Natural history of ASDs No difference in survival or symptoms between the two groups No difference in the incidence of new arrhythmias, stroke or other embolic phenomena, or cardiac failure No patient in either group developed progressive pulmonary vascular disease Shah et al, 1994
Natural history of ASDs Delayed closure of ASD in adult life does not alter natural history Neither survival nor the incidence or timing of development of atrial arrhythmias, embolic stroke, pulmonary vascular disease, or heart failure were affected by surgery in patients whose defects had not been detected until adulthood Outcome in adults with ASD was not improved by surgical closure Because progressive pulmonary vascular disease did not develop in any of these patients, its prevention is not a reason for advising closure of ASD in adults Shah et al, 1994
Why close ASDs Prevent impairment of lung function Studies have shown abnormal lung function in patients with ASDs in all age groups Lung volume restriction commonest abnormality Central and peripheral airway obstruction also present These abnormalities persist even a few years after correction
Why close ASDs Prevent impairment of lung function: Study of 46 patients who had surgery (20) or device closure (26) age matched patients No patients had pulmonary hypertension before procedure Lung function studied mean of 5.8 years later Zaqout et al, 2010
Why close ASDs Prevent impairment of lung function: No difference in functional residual capacity, total lung capacity, and residual volume between groups Surgical group showed a significant decrease in expiratory reserve volume (p<0.04) and forced vital capacity (p<0.03) So device closure may minimise effects of surgery on lung function Zaqout et al, 2010
Surgical closure of ASD in patients > 40 years of age 76 patients (63 women, 13 men) had surgical repair of ASD Age range 40 62 years (mean 45.8 years) Follow up between 1 and 17 years 1 operative and 1 late death occurred 62% in NYHA III and IV preoperatively 82% in NYHA I and II postoperatively 4 had AF before and 9 after surgery Jemielity et al, 2001
Surgical closure of ASD in patients > 40 years of age Symptoms after surgery Jemielity et al, 2001
Surgical closure of ASD in patients > 40 years of age Can improve clinical status and prevent RV dilatation Surgical treatment is recommended for older patients, even those over 50, who are in NYHA classes III and IV Jemielity et al, 2001
Closure of ASD with pulmonary hypertension 215 adults, some with pulmonary hypertension had device closure Systolic PA pressure no PHT (<40 mm Hg) Gp I (107 pts) mild PHT (40 to 49 mm Hg) Gp II (62 pts) moderate PHT (50 to 59 mm Hg) Gp III (27 pts) severe PHT (>60 mm Hg) Gp IV (19 pts) Yong et al, 2009
Closure of ASD with pulmonary hypertension Patients with higher baseline pressures were more likely to experience a >5 mmhg decrease (33.7%, 73.9%, 79.2%, and 100.0% in groups I to IV) Normalization of pressures (<40 mm Hg) occurred less frequently in patients with more advanced PAH (90.2%, 71.7%, 66.7%, and 23.5% in groups I to IV) Yong et al, 2009
Balloon occlusion and PA pressures In borderline cases, balloon occlusion gives valuable information Sanchez et al, 2010
Effect of balloon occlusion, 100% O2, Nitric Oxide Pt did not have surgery Pt had surgery Sanchez et al, 2010
LV diastolic dysfunction May occur with increasing age So if pt has this and moderate pulmonary hypertension, fenestrated device closure should be considered
Closure of ASD with pulmonary hypertension 15 pts with ASDs and pulmonary hypertension ASDs closed with fenestrated (5-8mm) ASO 5 male, 10 female, mean age 66 years (range 48 77) On TOE, ASD was 9 34mm (22.7± 7.0). By balloon stretch sizing 18 38 mm (28.6 ± 4.8) PA systolic mean 58 mmhg pre and 50mmHg post closure, mean PA of 35 mmhg pre and 31 mmhg post closure Bruch et al, 2008
Haemodynamics of ASD closure 55 year old, long standing history of asthma (on inhalers) Recent increase in breathlessness Chest x-ray showed cardiomegaly Echocardiogram: confirmed RV dilation and large secundum ASD and LV diastolic dysfunction
Haemodynamics of ASD closure TOE showed secundum ASD of 24 mm diameter with thin septum, with LV diastolic dysfunction
Haemodynamics of ASD closure Assessment of suitability of ASD closure by balloon occlusion
Haemodynamics of ASD closure Qp:Qs = 2.5:1, PA pressure 1/2 systemic Balloon occlusion of ASD with a sizing balloon and measurement of LA pressures: Baseline LVEDP = 20 mmhg, LA = mean 22 mmhg With balloon LVEDP = 26 mmhg, LA = mean 30 mmhg (PA pressure 2/3 systemic) Defect was not closed Treated with diuretics and Captopril for 6 months and brought to catheter lab again
Haemodynamics of ASD closure Repeat balloon occlusion of ASD with a sizing balloon and measurement of LA pressures: Baseline LVEDP = 18 mmhg, LA = mean 14 mmhg With balloon, LVEDP = 20, LA = mean 18 mmhg PA pressure <1/2 systemic
Haemodynamics of ASD closure Repeat balloon occlusion of ASD with a sizing balloon and measurement of LA pressures: Baseline LVEDP = 18 mmhg, LA = mean 14 mmhg Balloon LVEDP = 20, LA = mean 18 mmhg After 30 minutes of balloon occlusion, LA mean to 14 mm Hg ASD successfully closed with 26 mm ASO with a 5 mm fenestration
ASD closure in the elderly with risk factors Mean LA pressure >18 mm Hg, balloon occlusion for 15 mins. If mean LA pressure increases > 5mm Hg, then diuretics + afterload reduction for several days/weeks Repeat balloon occlusion for 15 mins. If LA pressure still increases by > 5mm Hg, then fenestrated device If LA pressure does not increase, in either case, then regular ASD device closure Kim et al, 2011
ASD closure in the elderly with risk factors Balloon occlusion for 10 mins. If mean LA pressure > 10mm Hg, then diuretics + iv inotropes for 48-60 hours Repeat balloon occlusion for 10 mins. If LA pressure still > 10mm Hg, then fenestrated device If LA pressure < 10mm Hg, in either case, then regular ASD device closure Kim et al, 2011
Patients with moderate or severe PAH may benefit from substantial reductions in PA pressures after catheter ASD closure Closure of ASD with pulmonary hypertension PA pressures may remain elevated in a sizeable proportion of patients Quality of life may improve even in 7 th of 8 th decades Caution needed in patients in 3 rd or 4 th decades with severe PAH. These may be 2 separate diseases