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Prevalence of “silent” pulmonary emboli in adults after the fontan operation
Congenital Heart Disease
Cardiopulmonary Bypass to Repair an Atrial Septal Defect Does not Affect Cognitive Function in Children
Abstracts
Stavinoha PL, Fixler DE, Mahony L. Circulation 2003;107:2722– 5.
Transcatheter Closure of Atrial Septal Defect in Young Children: Results and Follow-up
Study Question: Is there a decline in cognitive function in children after cardiopulmonary bypass (CPB) for atrial septal defect (ASD)? Methods: Prospective study of 18 children, ages 3–17 years (mean 8.5⫾4.3 years) with normal neurocognitive function at baseline who underwent neurocognitive testing using Differential Ability Scales testing a median of 3 days prior to and 5.8 months after surgical repair of ASD using CPB. Outcomes were an overall General Conceptual Ability (GCA) score and separate verbal, nonverbal and spatial factor scores. Results: All patients recovered uneventfully and were discharged from the hospital 2.8⫾0.4 days after surgery. One patient with headaches had a brain MRA with multiple small subacute infarctions felt to be due to air emboli at the time of surgery. There were no focal neurologic findings. CPB time was 42⫾16 minutes with a cross-clamp time of 24⫾16 minutes and a minimal rectal temperature of 31.4⫾1.6°C. The GCA score preoperatively (90.8⫾18.6) was not different from the score after surgery (93.1⫾16.3). There were no differences in verbal, nonverbal or spatial factor scores pre and postoperatively. Conclusion: Relative short periods of mildly hypothermic CPB for ASD repair was not associated with any decline in neurocognitive function in children with normal scores before surgery. Perspective: The relatively short period of follow-up in this study is a limitation. Longer follow-up might reveal late deficits in these children. The findings should not be generalized to children with more complex congenital heart disease or to children with cognitive dysfunction preoperatively. The use of transcatheter closure of isolated secundum ASD obviates the need for surgery in many of these patients. JK
Butera G, De Rosa G, Chessa M, et al. J Am Coll Cardiol 2003; 42:241–5. Study Question: What are the procedural success rates and long-term outcomes of percutaneous transcatheter atrial septal defect (ASD) closure in children ⱕ5 years of age? Methods: Observational study of 48 children ⱕ5 years of age (mean 3.6⫾1.3 years, range 8 months to 5 years) with isolated secundum ASD who underwent transcatheter closure with the Amplatzer septal occluder (ASO) or CardioSEAL/StarFLEX (CS/SF) device. Indications for closure included a pulmonary/systemic flow (Qp/Qs) ratio ⬎1.5:1, signs of right ventricular volume overload, ASD in children with failure to thrive or ⬎6 respiratory infections/year or prophylactic closure of small ASD or patent foramen ovale in children prior to liver transplantation. Residual shunt was defined as the presence of left-to-right flow across the defect by color Doppler on transthoracic or transesophageal echocardiography. Results: During the study period, 99 children were identified with isolated secundum ASD. 52% (51/99) patients had ASD that were too large for percutaneous closure. ASD closure was successful in all of the remaining 48 patients, 10 with the CS/SF and 38 with the ASO device. There were no deaths or major complications. There were technical problems in two patients, both with a successful closure and no clinical sequelae. The total occlusion rate overall was 87% at the time of the procedure, 94% at the time of discharge, 98% at 1 and 6 months, and 100% at 12 and 24 months. There was no difference in closure rates between the CS/SF and the ASO device, but fluoroscopy time and procedure time were less for the ASO device (15⫾11 minutes vs. 26⫾5 min, p⬍0.004 and 60⫾20 minutes vs. 85⫾35 minutes, p⬍0.004, respectively). Within 18⫾14 months, all children with failure to thrive recovered normal growth, and no child with recurrent respiratory infection had another infection. There was late radiologic evidence of fracture of an arm of the device in a 33 mm CS/SF device with no clinical consequences. Conclusion: Percutaneous closure is successful and safe in the short and long term for young children with isolated secundum ASD. Perspective: Transcatheter closure of ASD is a good option for even small children and does not carry the risk of late cognitive decline associated with cardiopulmonary bypass. It is unclear if the procedure will prevent late arrhythmias. JK
Prevalence of “Silent” Pulmonary Emboli in Adults After the Fontan Operation Varma C, Warr MR, Hendler AL, Paul NS, Webb GD, Therrien J. J Am Coll Cardiol 2003;41:2252– 8. Study Questions: What is the prevalence of clinically silent pulmonary embolism (PE) in adults after the Fontan procedure? What are the risk factors associated with silent PE? Methods: Prospective study of 30 adults, mean age 26⫾7 years, 57% men, who underwent last Fontan operation at a mean age of 12⫾7 years. All patients underwent evaluation for a prothrombotic state and nuclear ventilation/perfusion (VQ) scan utilizing a technique specific for Fontan patients, which were interpreted using standard PIOPED criteria. If
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the VQ scan was intermediate or high probability, the patient underwent a CT pulmonary angiogram. All patients underwent transthoracic echocardiography. Results: All patients were NYHA functional class 1 or 2, and 80% had systemic ventricular function graded as normal or mildly depressed. Atrial fibrillation or flutter was present in 9/30 patients. Four patients had a past or current history of right atrial thrombus. Nine subjects were taking warfarin and two were taking aspirin. The VQ scan was normal in 18 patients, low probability in seven patients and intermediate or high probability in five (17%) patients. All five of these patients had PE confirmed by CT pulmonary angiography. No patient had an abnormal prothrombotic evaluation. Silent PE was associated with older age at time of last Fontan operation (19⫾6 vs. 11⫾6 years, p⫽0.012) and type of Fontan anatomy (0/18 with direct atriopulmonary connection, 2/6 with right atrium to right ventricle conduit and 3⁄4 with a lateral tunnel, p⫽0.001). No patients taking warfarin had PE, but one patient taking aspirin had PE. Prevalence of silent PE in patients not on warfarin was 24% (5/21). Over 50% patients had traditional indications for anticoagulation (atrial dysrhtymias, atrial thrombus or PE). Conclusion: Clinically silent PE was found in at least 17% of adults after Fontan procedure. Perspective: The presence of right atrial thrombus was undoubtedly underestimated because of lack of performance of transesophageal echocardiography. In addition, the presence of clinically silent PE was probably underestimated since CT pulmonary angiography was not performed on patients with low probability VQ scans. Although the authors note that over 50% of the original cohort would have indications for warfarin therapy, the actual number is probably considerably higher. The authors call for a randomized, prospective trial of prophylactic warfarin therapy in all patients after Fontan procedure. JK
thelium-dependent vasodilation (flow-mediated dilation, FMD), vasodilator response to glyceryl trinitrate (GTND) and measurement of the brachial-radial pulse wave velocity (PWV) as an assessment of vascular stiffness. No patient had recurrent CoA. Results: Resting systolic blood pressure (SBP), mean BP, 24-hour SBP and diastolic BP, pulse pressure and heart rate were all higher in CoA patients than in controls. Resting systolic hypertension was present in 21% of CoA patients. Ambulatory systolic hypertension was found in one control and in 31 CoA patients (54%), 22 of whom had normal BP at rest. LV mass and LV mass index were higher in CoA patients than in controls (141.7⫾48.7 vs. 114.1⫾41.5 g, p⫽0.002, and 87.9⫾19.8 vs. 68.1⫾18.4 g, p⫽0.0001, respectively). Six CoA patients (9.7%) had LV hypertrophy, all with an eccentric geometric pattern. Vascular stiffness was higher in CoA than controls (PWV 9.3⫾2.6 vs. 8.1⫾1.8 m/s, p⫽0.05), and FMD and GTND were significantly reduced in CoA patients. Predictors of 24-hour BP were reduced GTND, weight and the presence of repaired CoA. In CoA patients, LV mass index correlated with age, age at time of repair, body mass index, resting brachial artery diameter, resting SBP, 24-hour and daytime SBP, and 24-hour pulse pressure. On multivariable analysis, independent predictors of LV mass were weight, 24-hour SBP and prior CoA repair. Conclusions: Late hypertension is common in patients after repair of CoA and is related to abnormalities of upper extremity conduit artery function. Reduced vasodilator response to GTN is a strong predictor of arterial hypertension. Ambulatory 24-hour SBP, and presence of CoA predicted LV mass independent of age, weight and resting BP, suggesting that patients with CoA have a greater LV response to a given BP and weight than do healthy subjects. Perspective: Patients who underwent successful CoA repair at a very young age require careful and long-term follow-up since ambulatory hypertension is common even in patients with normal resting BP. JK
Ambulatory Blood Pressure, Left Ventricular Mass, and Conduit Artery Function Late After Successful Repair of Coarctation of the Aorta
Prospective Echocardiographic Diagnosis and Surgical Repair of Anomalous Origin of a Coronary Artery From the Opposite Sinus with an Interarterial Course
de Divitiis M, Pilla C, Kattenhorn M, et al. J Am Coll Cardiol 2003;41:2259 – 65. Study Question: Are there associations between arterial hypertension, left ventricular (LV) mass and abnormalities of arterial vasodilator response and stiffness in patients after successful repair of coarctation of the aorta? Methods: 72 patients (median age 19.8 years, 50 males, 22 females) who underwent surgical repair of coarctation of the aorta (CoA) at a median age of 3.1 months (range 0.1– 480 months, 60% repaired prior to age 1 year) were studied and compared to 53 healthy age-matched controls with similar body surface area and body mass index. Patients underwent echocardiography for determination of LV mass and wall thickness, resting and ambulatory blood pressure measurement and studies of brachial artery endo-
Crommelt PC, Frommelt MA, Tweddell JS, Jaquiss RDB. J Am Coll Cardiol 2003;42:148 –54. Study Objective: The authors described the clinical presentation, anatomic features, diagnostic techniques and surgical outcome of patients with anomalous origin of a coronary artery (AOCA) from the opposite coronary sinus with an interarterial course between the aorta and pulmonary artery. Methods: Retrospective record view of patients with AOCA identified between September 1997 and August 2002.
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Cardiopulmonary Bypass to Repair an Atrial Septal Defect Does not Affect Cognitive Function in Children
Abstracts
Stavinoha PL, Fixler DE, Mahony L. Circulation 2003;107:2722– 5.
Transcatheter Closure of Atrial Septal Defect in Young Children: Results and Follow-up
Study Question: Is there a decline in cognitive function in children after cardiopulmonary bypass (CPB) for atrial septal defect (ASD)? Methods: Prospective study of 18 children, ages 3–17 years (mean 8.5⫾4.3 years) with normal neurocognitive function at baseline who underwent neurocognitive testing using Differential Ability Scales testing a median of 3 days prior to and 5.8 months after surgical repair of ASD using CPB. Outcomes were an overall General Conceptual Ability (GCA) score and separate verbal, nonverbal and spatial factor scores. Results: All patients recovered uneventfully and were discharged from the hospital 2.8⫾0.4 days after surgery. One patient with headaches had a brain MRA with multiple small subacute infarctions felt to be due to air emboli at the time of surgery. There were no focal neurologic findings. CPB time was 42⫾16 minutes with a cross-clamp time of 24⫾16 minutes and a minimal rectal temperature of 31.4⫾1.6°C. The GCA score preoperatively (90.8⫾18.6) was not different from the score after surgery (93.1⫾16.3). There were no differences in verbal, nonverbal or spatial factor scores pre and postoperatively. Conclusion: Relative short periods of mildly hypothermic CPB for ASD repair was not associated with any decline in neurocognitive function in children with normal scores before surgery. Perspective: The relatively short period of follow-up in this study is a limitation. Longer follow-up might reveal late deficits in these children. The findings should not be generalized to children with more complex congenital heart disease or to children with cognitive dysfunction preoperatively. The use of transcatheter closure of isolated secundum ASD obviates the need for surgery in many of these patients. JK
Butera G, De Rosa G, Chessa M, et al. J Am Coll Cardiol 2003; 42:241–5. Study Question: What are the procedural success rates and long-term outcomes of percutaneous transcatheter atrial septal defect (ASD) closure in children ⱕ5 years of age? Methods: Observational study of 48 children ⱕ5 years of age (mean 3.6⫾1.3 years, range 8 months to 5 years) with isolated secundum ASD who underwent transcatheter closure with the Amplatzer septal occluder (ASO) or CardioSEAL/StarFLEX (CS/SF) device. Indications for closure included a pulmonary/systemic flow (Qp/Qs) ratio ⬎1.5:1, signs of right ventricular volume overload, ASD in children with failure to thrive or ⬎6 respiratory infections/year or prophylactic closure of small ASD or patent foramen ovale in children prior to liver transplantation. Residual shunt was defined as the presence of left-to-right flow across the defect by color Doppler on transthoracic or transesophageal echocardiography. Results: During the study period, 99 children were identified with isolated secundum ASD. 52% (51/99) patients had ASD that were too large for percutaneous closure. ASD closure was successful in all of the remaining 48 patients, 10 with the CS/SF and 38 with the ASO device. There were no deaths or major complications. There were technical problems in two patients, both with a successful closure and no clinical sequelae. The total occlusion rate overall was 87% at the time of the procedure, 94% at the time of discharge, 98% at 1 and 6 months, and 100% at 12 and 24 months. There was no difference in closure rates between the CS/SF and the ASO device, but fluoroscopy time and procedure time were less for the ASO device (15⫾11 minutes vs. 26⫾5 min, p⬍0.004 and 60⫾20 minutes vs. 85⫾35 minutes, p⬍0.004, respectively). Within 18⫾14 months, all children with failure to thrive recovered normal growth, and no child with recurrent respiratory infection had another infection. There was late radiologic evidence of fracture of an arm of the device in a 33 mm CS/SF device with no clinical consequences. Conclusion: Percutaneous closure is successful and safe in the short and long term for young children with isolated secundum ASD. Perspective: Transcatheter closure of ASD is a good option for even small children and does not carry the risk of late cognitive decline associated with cardiopulmonary bypass. It is unclear if the procedure will prevent late arrhythmias. JK
Prevalence of “Silent” Pulmonary Emboli in Adults After the Fontan Operation Varma C, Warr MR, Hendler AL, Paul NS, Webb GD, Therrien J. J Am Coll Cardiol 2003;41:2252– 8. Study Questions: What is the prevalence of clinically silent pulmonary embolism (PE) in adults after the Fontan procedure? What are the risk factors associated with silent PE? Methods: Prospective study of 30 adults, mean age 26⫾7 years, 57% men, who underwent last Fontan operation at a mean age of 12⫾7 years. All patients underwent evaluation for a prothrombotic state and nuclear ventilation/perfusion (VQ) scan utilizing a technique specific for Fontan patients, which were interpreted using standard PIOPED criteria. If
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the VQ scan was intermediate or high probability, the patient underwent a CT pulmonary angiogram. All patients underwent transthoracic echocardiography. Results: All patients were NYHA functional class 1 or 2, and 80% had systemic ventricular function graded as normal or mildly depressed. Atrial fibrillation or flutter was present in 9/30 patients. Four patients had a past or current history of right atrial thrombus. Nine subjects were taking warfarin and two were taking aspirin. The VQ scan was normal in 18 patients, low probability in seven patients and intermediate or high probability in five (17%) patients. All five of these patients had PE confirmed by CT pulmonary angiography. No patient had an abnormal prothrombotic evaluation. Silent PE was associated with older age at time of last Fontan operation (19⫾6 vs. 11⫾6 years, p⫽0.012) and type of Fontan anatomy (0/18 with direct atriopulmonary connection, 2/6 with right atrium to right ventricle conduit and 3⁄4 with a lateral tunnel, p⫽0.001). No patients taking warfarin had PE, but one patient taking aspirin had PE. Prevalence of silent PE in patients not on warfarin was 24% (5/21). Over 50% patients had traditional indications for anticoagulation (atrial dysrhtymias, atrial thrombus or PE). Conclusion: Clinically silent PE was found in at least 17% of adults after Fontan procedure. Perspective: The presence of right atrial thrombus was undoubtedly underestimated because of lack of performance of transesophageal echocardiography. In addition, the presence of clinically silent PE was probably underestimated since CT pulmonary angiography was not performed on patients with low probability VQ scans. Although the authors note that over 50% of the original cohort would have indications for warfarin therapy, the actual number is probably considerably higher. The authors call for a randomized, prospective trial of prophylactic warfarin therapy in all patients after Fontan procedure. JK
thelium-dependent vasodilation (flow-mediated dilation, FMD), vasodilator response to glyceryl trinitrate (GTND) and measurement of the brachial-radial pulse wave velocity (PWV) as an assessment of vascular stiffness. No patient had recurrent CoA. Results: Resting systolic blood pressure (SBP), mean BP, 24-hour SBP and diastolic BP, pulse pressure and heart rate were all higher in CoA patients than in controls. Resting systolic hypertension was present in 21% of CoA patients. Ambulatory systolic hypertension was found in one control and in 31 CoA patients (54%), 22 of whom had normal BP at rest. LV mass and LV mass index were higher in CoA patients than in controls (141.7⫾48.7 vs. 114.1⫾41.5 g, p⫽0.002, and 87.9⫾19.8 vs. 68.1⫾18.4 g, p⫽0.0001, respectively). Six CoA patients (9.7%) had LV hypertrophy, all with an eccentric geometric pattern. Vascular stiffness was higher in CoA than controls (PWV 9.3⫾2.6 vs. 8.1⫾1.8 m/s, p⫽0.05), and FMD and GTND were significantly reduced in CoA patients. Predictors of 24-hour BP were reduced GTND, weight and the presence of repaired CoA. In CoA patients, LV mass index correlated with age, age at time of repair, body mass index, resting brachial artery diameter, resting SBP, 24-hour and daytime SBP, and 24-hour pulse pressure. On multivariable analysis, independent predictors of LV mass were weight, 24-hour SBP and prior CoA repair. Conclusions: Late hypertension is common in patients after repair of CoA and is related to abnormalities of upper extremity conduit artery function. Reduced vasodilator response to GTN is a strong predictor of arterial hypertension. Ambulatory 24-hour SBP, and presence of CoA predicted LV mass independent of age, weight and resting BP, suggesting that patients with CoA have a greater LV response to a given BP and weight than do healthy subjects. Perspective: Patients who underwent successful CoA repair at a very young age require careful and long-term follow-up since ambulatory hypertension is common even in patients with normal resting BP. JK
Ambulatory Blood Pressure, Left Ventricular Mass, and Conduit Artery Function Late After Successful Repair of Coarctation of the Aorta
Prospective Echocardiographic Diagnosis and Surgical Repair of Anomalous Origin of a Coronary Artery From the Opposite Sinus with an Interarterial Course
de Divitiis M, Pilla C, Kattenhorn M, et al. J Am Coll Cardiol 2003;41:2259 – 65. Study Question: Are there associations between arterial hypertension, left ventricular (LV) mass and abnormalities of arterial vasodilator response and stiffness in patients after successful repair of coarctation of the aorta? Methods: 72 patients (median age 19.8 years, 50 males, 22 females) who underwent surgical repair of coarctation of the aorta (CoA) at a median age of 3.1 months (range 0.1– 480 months, 60% repaired prior to age 1 year) were studied and compared to 53 healthy age-matched controls with similar body surface area and body mass index. Patients underwent echocardiography for determination of LV mass and wall thickness, resting and ambulatory blood pressure measurement and studies of brachial artery endo-
Crommelt PC, Frommelt MA, Tweddell JS, Jaquiss RDB. J Am Coll Cardiol 2003;42:148 –54. Study Objective: The authors described the clinical presentation, anatomic features, diagnostic techniques and surgical outcome of patients with anomalous origin of a coronary artery (AOCA) from the opposite coronary sinus with an interarterial course between the aorta and pulmonary artery. Methods: Retrospective record view of patients with AOCA identified between September 1997 and August 2002.
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