- Email: [email protected]
Pediatric cardiology and adult congenital heart disease
380
Murphy ACC 2003 Annual Session Highlights
JACC Vol. 42, No. 2, 2003 July 16, 2003:375–93
Pediatric Cardiology and Adult Congenital Heart Disease Daniel J. Murphy, JR, MD Palo Alto, California
One hundred nine abstracts were presented as posters or oral presentations covering a wide range of topics encompassing all aspects of congenital cardiology from fetal life to adults with congenital heart disease (ACHD). One study that was presented from two groups in Toronto and Montreal described outcomes for 50 fetuses with complete atrioventricular block in utero. Twenty-two of those had structural heart disease, and 19 of 22 died. On the other hand, of 28 with normal cardiac anatomy, 13 had heart rates ⬍55 beats/min. Seven mothers received treatment beta-sympathomimetics, and only one of the seven treated fetuses died compared with five of six deaths without treatment. Seventeen of 24 pregnancies with autoimmune antibodies were treated with maternal dexamethasone, and only 3 (18%) did not survive the neonatal period, whereas 4 of 7 cases (57%) without steroid therapy died (1). One of the most provocative papers described the experience of a large collaborative group in Boston (2) who performed balloon dilation of aortic stenosis in 12 fetuses. The Boston group evaluated 16 fetuses between 22 and 29 weeks gestation who had aortic stenosis and were expected to develop hypoplastic left heart syndrome. Of the patients who declined to participate, three of four were born with hypoplastic left heart syndrome (HLHS), suggesting that the investigators were able to correctly identify fetuses at risk for the development of HLHS. Balloon aortic valvotomy was attempted in 12 fetuses. Five procedures were technically unsuccessful: one pregnancy was terminated, one fetus died in utero, and three patients were born with HLHS. Of the seven successful aortic valve dilations, three infants were born with HLHS, two are still in utero at 31 and 23 weeks, and one baby was born with mild aortic stenosis and has survived to 15 months without intervention. There was no significant maternal morbidity. This exciting report represents the early results from a large multidisciplinary team and signals the inauguration of fetal cardiac intervention. There were a substantial number of studies in ACHD. A group at McGill University attempted to assess the prevalence of ACHD in Quebec. Using ICD-9 diagnostic codes, the investigators examined provincial health care records to identify all ACHD patients older than 18 years in 2000. They identified 15,339 adults for a total prevalence rate of 2.3/1,000. These data suggest that as many as 500,000 From the Division of Pediatric Cardiology, Stanford University Medical Center, Palo Alto, California.
ACHD are alive in the U.S., 10% of whom are expected to have complex disease (3). A study from Mayo Clinic (4) described outcomes for 112 pregnancies in 43 women with tetralogy of Fallot. All but three had undergone complete surgical repair. There were 29 (26%) spontaneous first trimester miscarriages and one term stillbirth. Six of 43 patients had cardiovascular complications: supraventricular tachycardia in 2, congestive heart failure in 2, pulmonary embolism in 1. There were no peripartum deaths. Two infants had significant congenital cardiac defects. Virtually all individuals with tetralogy of Fallot will face the prospect of pulmonary valve replacement. T. P. Graham presented the results of a multicenter retrospective review sponsored by the International Society for Adult Congenital Heart Disease in which data were collected for 93 patients who underwent pulmonary valve replacement. The average age was 26 years, and follow-up averaged 3 years. There were no early deaths, and the majority of patients experienced reduction in right ventricular size and ability index. Kaplan-Meier estimates of durability showed approximately 50% re-replacement at 11 years (5). Clinical experience with interventional techniques in congenital heart disease is increasing. Reporting early human experience, several reports described successful transcatheter closure of ventricular septal defects. Atrial septal defects (ASD) and patent foramen ovale have been closed via catheter for a number of years. Follow-up studies after transcatheter ASD closure demonstrated favorable right heart remodeling in 50 patients older than 60 years (6), modest improvement in cardiopulmonary exercise capacity (7), and even relief of migraine headaches (8). Septal closure devices can be associated with thrombus formation. Sievert from Frankfurt, Germany (9) performed routine postprocedure transesophageal echocardiograms at two to four weeks and six months. Thrombus was found in 20 patients, associated with three minor strokes and one transient ischemic attack. Most of the thrombi resolved with administration of warfarin, but three patients required surgical thrombectomy. Other midterm complications of ASD devices were reported, including systemic nickel dissemination (10) and mild aortic insufficiency (11). The studies presented at this year’s American College of Cardiology Scientific Sessions demonstrate that short- and long-term results continue to improve for all forms of congenital heart disease. New indications, techniques, and populations provide novel opportunities for intervention and promise to improve outcomes in the future.
JACC Vol. 42, No. 2, 2003 July 16, 2003:375–93 Correspondence: Dr. Daniel J. Murphy, Jr, Stanford University Medical Center, Pediatric Cardiology, 750 Welch Road, Suite 305, Palo Alto, California 94306. E-mail: [email protected].
REFERENCES 1. Jaeggi ET, Fouron J-C, Smallhorn J, Proulx F, Hornberger LK. Prenatally diagnosed complete atrioventricular block with and without structural heart disease in the 1990s: management and impact on outcome (abstr). J Am Coll Cardiol 2003;41 Suppl A:482A. 2. Tworetzky W, Jennings RW, Wilkins-Haug LE, et al. Balloon dilation of severe aortic stenosis in the fetus: technical advances (abstr). J Am Coll Cardiol 2003;41 Suppl A:496A. 3. Marelli AJ, Rhame E, Pilote L. Congenital heart disease: prevalence rates in a population of 5,363,695 adults (abstr). J Am Coll Cardiol 2003;41 Suppl A:490A. 4. Veldman GR Jr., Connolly HM, Grogan M, Ammash NA, Warnes CA. Outcome of pregnancy in women with Fallot’s tetralogy (abstr). J Am Coll Cardiol 2003;41 Suppl A:490A. 5. Graham TP Jr. Outcome of pulmonary valve replacement in adults after tetralogy repair (abstr). J Am Coll Cardiol 2003;41 Suppl A:487A.
Zidar ACC 2003 Annual Session Highlights
381
6. Swan L, Varma C, Yip JW, Warr M, Benson LN, McLaughlin P. Favorable right heart remodeling after atrial septal defect device closure even in the over-60 age group (abstr). J Am Coll Cardiol 2003;41 Suppl A:473A. 7. Weber MW, Neumann T, Rau M, et al. Cardiopulmonary exercise capacity increases in patients with atrial septal defect following transcatheter closure (abstr). J Am Coll Cardiol 2003;41 Suppl A:474A. 8. Reisman M, Gray WA, Olsen JV. Relief of migraine headache associated with closure of patent foramen ovale (abstr). J Am Coll Cardiol 2003;41 Suppl A:474A. 9. Sievert H, Krumsdorf U, Horvath K, Ostermayer S, Billinger K, Thomas T. Thrombus formation on atrial septal defect and patent foramen ovale closure devices: incidence and clinical outcome in 1,000 consecutive patients (abstr). J Am Coll Cardiol 2003;41 Suppl A:473A. 10. Neumann T, Petkovic D, Burian M, et al. Systemic dissemination of nickel in patients with atrial septal defect following the implantation of the Amplatzer septal occluder (abstr). J Am Coll Cardiol 2003;41 Suppl A:488A. 11. Schoen S, Schulze M, Lange S, Bohl S, Braun MU, Strasser RH. Mild aortic valve insufficiency: a mid-term complication of percutaneous closure of atrial septal defects and patent foramen ovale (abstr). J Am Coll Cardiol 2003;41 Suppl A:496A. doi:10.1016/S0735-1097(03)00749-6
Angiography and Interventional Cardiology James P. Zidar, MD, FACC, FSCAI Durham, North Carolina The angiography and interventional cardiology working group reviewed 1,095 abstracts for this year’s scientific sessions and accepted 355. There were five areas of major impact from the meeting: the use of distal projection strategies for vein graft intervention and acute myocardial infarction (AMI), new thromboatherectomy devices, the role of inflammation in percutaneous coronary intervention (PCI), new insights on drug-eluting stents, and peripheral interventions.
DISTAL PROTECTION In the setting of AMI, Drs. Gibson and Stone have previously demonstrated that distal emboli and inflammation in the distal coronary bed predict 30-day and 1-year mortality, even in the presence of Thrombolysis In Myocardial Infarction grade 3 (TIMI-3) flow. Advancing this work, Dr. Limbruno presented the Filter Wire Distal Protection Trial in AMI. This 100-patient study enrolled patients who presented with ST-segment elevation within six hours and ⬍TIMI-3 flow in a native coronary vessel ⬎3 mm. Patients were randomized to stent with or without the protection of the distal filter wire. An improvement in TIMI-3 flow and myocardial blush scores, more rapid resolution of ST-segment changes, and improvement in regional wall motion recovery and less distal emboli were demonstrated in the treated group relative to the control From Duke University Medical Center, Durham, North Carolina.
group. Dr. Stone presented the FIRE trial, comparing the Filter Wire to the Percusurge Guard wire in saphenous vein graft (SVG) intervention. This trial was designed as a non-inferiority trial to the approved Guard Wire System. Major adverse cardiac events (MACE) at 30 days with the Filter Wire were equivalent to the Guard Wire, with respect to death, myocardial infarction (MI), and urgent target vessel revascularization. Data were presented on the X-SIZER catheter, a thromboatherectomy device in AMI. Dr. Reimers and colleagues randomized 92 patients with ST-segment elevation MI and thrombus to the X-SIZER or control. Although there were no differences in TIMI-3 flow, improvements in the more sensitive score of myocardial blush were evident, along with improvements in ST-segment elevation resolution and significant reduction in angiographic complications. There are also trends seen in the recovery of left ventricular function at discharge and at 30 days. These two trials suggest that distal protection offers a beneficial role to our SVG and AMI angioplasty patients.
INFLAMMATION AND PCI Turning to inflammation and its role in the angioplasty laboratory, a group at the Cleveland Clinic randomized 75 patients who had elective angioplasty to pretreatment with 300 mg of clopidogrel versus no pretreatment. Dr. Frank Zidar presented data on the inflammatory markers: C-reactive protein, CD-40 ligand, P-selectin, and interleukin-6. Plate-
Murphy ACC 2003 Annual Session Highlights
JACC Vol. 42, No. 2, 2003 July 16, 2003:375–93
Pediatric Cardiology and Adult Congenital Heart Disease Daniel J. Murphy, JR, MD Palo Alto, California
One hundred nine abstracts were presented as posters or oral presentations covering a wide range of topics encompassing all aspects of congenital cardiology from fetal life to adults with congenital heart disease (ACHD). One study that was presented from two groups in Toronto and Montreal described outcomes for 50 fetuses with complete atrioventricular block in utero. Twenty-two of those had structural heart disease, and 19 of 22 died. On the other hand, of 28 with normal cardiac anatomy, 13 had heart rates ⬍55 beats/min. Seven mothers received treatment beta-sympathomimetics, and only one of the seven treated fetuses died compared with five of six deaths without treatment. Seventeen of 24 pregnancies with autoimmune antibodies were treated with maternal dexamethasone, and only 3 (18%) did not survive the neonatal period, whereas 4 of 7 cases (57%) without steroid therapy died (1). One of the most provocative papers described the experience of a large collaborative group in Boston (2) who performed balloon dilation of aortic stenosis in 12 fetuses. The Boston group evaluated 16 fetuses between 22 and 29 weeks gestation who had aortic stenosis and were expected to develop hypoplastic left heart syndrome. Of the patients who declined to participate, three of four were born with hypoplastic left heart syndrome (HLHS), suggesting that the investigators were able to correctly identify fetuses at risk for the development of HLHS. Balloon aortic valvotomy was attempted in 12 fetuses. Five procedures were technically unsuccessful: one pregnancy was terminated, one fetus died in utero, and three patients were born with HLHS. Of the seven successful aortic valve dilations, three infants were born with HLHS, two are still in utero at 31 and 23 weeks, and one baby was born with mild aortic stenosis and has survived to 15 months without intervention. There was no significant maternal morbidity. This exciting report represents the early results from a large multidisciplinary team and signals the inauguration of fetal cardiac intervention. There were a substantial number of studies in ACHD. A group at McGill University attempted to assess the prevalence of ACHD in Quebec. Using ICD-9 diagnostic codes, the investigators examined provincial health care records to identify all ACHD patients older than 18 years in 2000. They identified 15,339 adults for a total prevalence rate of 2.3/1,000. These data suggest that as many as 500,000 From the Division of Pediatric Cardiology, Stanford University Medical Center, Palo Alto, California.
ACHD are alive in the U.S., 10% of whom are expected to have complex disease (3). A study from Mayo Clinic (4) described outcomes for 112 pregnancies in 43 women with tetralogy of Fallot. All but three had undergone complete surgical repair. There were 29 (26%) spontaneous first trimester miscarriages and one term stillbirth. Six of 43 patients had cardiovascular complications: supraventricular tachycardia in 2, congestive heart failure in 2, pulmonary embolism in 1. There were no peripartum deaths. Two infants had significant congenital cardiac defects. Virtually all individuals with tetralogy of Fallot will face the prospect of pulmonary valve replacement. T. P. Graham presented the results of a multicenter retrospective review sponsored by the International Society for Adult Congenital Heart Disease in which data were collected for 93 patients who underwent pulmonary valve replacement. The average age was 26 years, and follow-up averaged 3 years. There were no early deaths, and the majority of patients experienced reduction in right ventricular size and ability index. Kaplan-Meier estimates of durability showed approximately 50% re-replacement at 11 years (5). Clinical experience with interventional techniques in congenital heart disease is increasing. Reporting early human experience, several reports described successful transcatheter closure of ventricular septal defects. Atrial septal defects (ASD) and patent foramen ovale have been closed via catheter for a number of years. Follow-up studies after transcatheter ASD closure demonstrated favorable right heart remodeling in 50 patients older than 60 years (6), modest improvement in cardiopulmonary exercise capacity (7), and even relief of migraine headaches (8). Septal closure devices can be associated with thrombus formation. Sievert from Frankfurt, Germany (9) performed routine postprocedure transesophageal echocardiograms at two to four weeks and six months. Thrombus was found in 20 patients, associated with three minor strokes and one transient ischemic attack. Most of the thrombi resolved with administration of warfarin, but three patients required surgical thrombectomy. Other midterm complications of ASD devices were reported, including systemic nickel dissemination (10) and mild aortic insufficiency (11). The studies presented at this year’s American College of Cardiology Scientific Sessions demonstrate that short- and long-term results continue to improve for all forms of congenital heart disease. New indications, techniques, and populations provide novel opportunities for intervention and promise to improve outcomes in the future.
JACC Vol. 42, No. 2, 2003 July 16, 2003:375–93 Correspondence: Dr. Daniel J. Murphy, Jr, Stanford University Medical Center, Pediatric Cardiology, 750 Welch Road, Suite 305, Palo Alto, California 94306. E-mail: [email protected].
REFERENCES 1. Jaeggi ET, Fouron J-C, Smallhorn J, Proulx F, Hornberger LK. Prenatally diagnosed complete atrioventricular block with and without structural heart disease in the 1990s: management and impact on outcome (abstr). J Am Coll Cardiol 2003;41 Suppl A:482A. 2. Tworetzky W, Jennings RW, Wilkins-Haug LE, et al. Balloon dilation of severe aortic stenosis in the fetus: technical advances (abstr). J Am Coll Cardiol 2003;41 Suppl A:496A. 3. Marelli AJ, Rhame E, Pilote L. Congenital heart disease: prevalence rates in a population of 5,363,695 adults (abstr). J Am Coll Cardiol 2003;41 Suppl A:490A. 4. Veldman GR Jr., Connolly HM, Grogan M, Ammash NA, Warnes CA. Outcome of pregnancy in women with Fallot’s tetralogy (abstr). J Am Coll Cardiol 2003;41 Suppl A:490A. 5. Graham TP Jr. Outcome of pulmonary valve replacement in adults after tetralogy repair (abstr). J Am Coll Cardiol 2003;41 Suppl A:487A.
Zidar ACC 2003 Annual Session Highlights
381
6. Swan L, Varma C, Yip JW, Warr M, Benson LN, McLaughlin P. Favorable right heart remodeling after atrial septal defect device closure even in the over-60 age group (abstr). J Am Coll Cardiol 2003;41 Suppl A:473A. 7. Weber MW, Neumann T, Rau M, et al. Cardiopulmonary exercise capacity increases in patients with atrial septal defect following transcatheter closure (abstr). J Am Coll Cardiol 2003;41 Suppl A:474A. 8. Reisman M, Gray WA, Olsen JV. Relief of migraine headache associated with closure of patent foramen ovale (abstr). J Am Coll Cardiol 2003;41 Suppl A:474A. 9. Sievert H, Krumsdorf U, Horvath K, Ostermayer S, Billinger K, Thomas T. Thrombus formation on atrial septal defect and patent foramen ovale closure devices: incidence and clinical outcome in 1,000 consecutive patients (abstr). J Am Coll Cardiol 2003;41 Suppl A:473A. 10. Neumann T, Petkovic D, Burian M, et al. Systemic dissemination of nickel in patients with atrial septal defect following the implantation of the Amplatzer septal occluder (abstr). J Am Coll Cardiol 2003;41 Suppl A:488A. 11. Schoen S, Schulze M, Lange S, Bohl S, Braun MU, Strasser RH. Mild aortic valve insufficiency: a mid-term complication of percutaneous closure of atrial septal defects and patent foramen ovale (abstr). J Am Coll Cardiol 2003;41 Suppl A:496A. doi:10.1016/S0735-1097(03)00749-6
Angiography and Interventional Cardiology James P. Zidar, MD, FACC, FSCAI Durham, North Carolina The angiography and interventional cardiology working group reviewed 1,095 abstracts for this year’s scientific sessions and accepted 355. There were five areas of major impact from the meeting: the use of distal projection strategies for vein graft intervention and acute myocardial infarction (AMI), new thromboatherectomy devices, the role of inflammation in percutaneous coronary intervention (PCI), new insights on drug-eluting stents, and peripheral interventions.
DISTAL PROTECTION In the setting of AMI, Drs. Gibson and Stone have previously demonstrated that distal emboli and inflammation in the distal coronary bed predict 30-day and 1-year mortality, even in the presence of Thrombolysis In Myocardial Infarction grade 3 (TIMI-3) flow. Advancing this work, Dr. Limbruno presented the Filter Wire Distal Protection Trial in AMI. This 100-patient study enrolled patients who presented with ST-segment elevation within six hours and ⬍TIMI-3 flow in a native coronary vessel ⬎3 mm. Patients were randomized to stent with or without the protection of the distal filter wire. An improvement in TIMI-3 flow and myocardial blush scores, more rapid resolution of ST-segment changes, and improvement in regional wall motion recovery and less distal emboli were demonstrated in the treated group relative to the control From Duke University Medical Center, Durham, North Carolina.
group. Dr. Stone presented the FIRE trial, comparing the Filter Wire to the Percusurge Guard wire in saphenous vein graft (SVG) intervention. This trial was designed as a non-inferiority trial to the approved Guard Wire System. Major adverse cardiac events (MACE) at 30 days with the Filter Wire were equivalent to the Guard Wire, with respect to death, myocardial infarction (MI), and urgent target vessel revascularization. Data were presented on the X-SIZER catheter, a thromboatherectomy device in AMI. Dr. Reimers and colleagues randomized 92 patients with ST-segment elevation MI and thrombus to the X-SIZER or control. Although there were no differences in TIMI-3 flow, improvements in the more sensitive score of myocardial blush were evident, along with improvements in ST-segment elevation resolution and significant reduction in angiographic complications. There are also trends seen in the recovery of left ventricular function at discharge and at 30 days. These two trials suggest that distal protection offers a beneficial role to our SVG and AMI angioplasty patients.
INFLAMMATION AND PCI Turning to inflammation and its role in the angioplasty laboratory, a group at the Cleveland Clinic randomized 75 patients who had elective angioplasty to pretreatment with 300 mg of clopidogrel versus no pretreatment. Dr. Frank Zidar presented data on the inflammatory markers: C-reactive protein, CD-40 ligand, P-selectin, and interleukin-6. Plate-