Alan Nugent - Academia.edu (original) (raw)

Papers by Alan Nugent

Research paper thumbnail of Pulmonary artery stents: Long-term follow-up

Catheterization and Cardiovascular Interventions, 2010

Objectives: Determine the long-term outcomes of branch pulmonary artery (PA) stents. Background: ... more Objectives: Determine the long-term outcomes of branch pulmonary artery (PA) stents. Background: PA stents in congenital heart disease effectively relieve stenoses in the short-term. Published long-term data are limited. Methods: Patients enrolled in an FDA IDE protocol from 1989-92 were included. Clinical follow-up and catheterization data were evaluated. Patients were included if >5 year follow-up data was available or if mortality occurred following the initial procedure. Results: There were five deaths: four due to progression of their underlying heart disease, and one from a complication during a follow-up catheterization. Clinical data for 43 surviving patients demonstrated 39 patients (91%) are in NYHA class I or II. Seven patients underwent surgical intervention during the follow-up period (five RV-PA conduit, two Fontan revisions), but none addressed PA stenosis. Final repeat catheterizations were performed in 36 patients (55 stents) 7.2 6 4.3 years post stent insertion with 1.2 6 0.9 further procedures with stent dilations. In this subgroup, the minimum vessel diameter increased from 4.7 6 1.8 to 13.4 6 2.4 mm (P < 0.001), and the pressure gradient improved from 41 6 25 to 9 6 11 mm Hg (P < 0.001). Higher initial gradient and smaller balloons were associated with a final stent diameter of <14 mm (P 5 0.030 and 0.046). Jailed vessels occurred in 49% of stents with abnormal angiographic flow in 18/55. Six repeat catheterizations resulted in complications, including the one procedural death. Conclusion: Stents implants for PA stenoses provide effective improvement in vessel caliber in the long-term. Although repeat interventions are necessary, this procedure reduces RV pressure and provides an important alternative to surgery for residual PA obstruction. V C 2009 Wiley-Liss, Inc.

Research paper thumbnail of Focal seizure and cerebral contrast retention after cardiac catheterization

Pediatric Neurology, 2005

Although rare, ionic and nonionic contrast-induced seizures occur as a complication of enhanced c... more Although rare, ionic and nonionic contrast-induced seizures occur as a complication of enhanced cranial computed tomography for both adults and children. However, contrast-induced seizures after cardiac catheterization has only been reported in adults. This report describes an 18-month-old male who developed a new-onset focal seizure 12 hours after cardiac catheterization. Unenhanced cranial computed tomography 1 hour after the seizure demonstrated general cerebral edema and unilateral focal cerebral contrast retention with sparing of the area supplied by the middle cerebral artery. The contrast was reabsorbed from the subarachnoid space over a 48-hour period, the cerebral edema resolved over several days, and the child returned to his baseline state 4 days after the seizure episode. This study documents the evolution of computed tomographic findings after contrast-induced seizures in a child. Contrast toxicity should be considered in any case of a new-onset neurologic deficit arising after angiography or enhanced computed tomography.

Research paper thumbnail of Transcatheter Delivery of a Replacement Heart Valve

Research paper thumbnail of Optimization of Preoperative Status in Hypoplastic Left Heart Syndrome With Intact Atrial Septum by Left Atrial Decompression and Bilateral Pulmonary Artery Bands

Pediatric Cardiology, 2014

Hypoplastic left heart syndrome (HLHS) with intact (IAS) or highly restrictive atrial septum (RAS... more Hypoplastic left heart syndrome (HLHS) with intact (IAS) or highly restrictive atrial septum (RAS) has extremely poor outcomes largely related to pulmonary pathology. At birth, immediate left atrial (LA) decompression is required to remain viable, but there is a tradeoff between residual increase in LA pressure and pulmonary overcirculation, either of which exacerbates the pulmonary status. From August 2010 to April 2013, a retrospective chart review was performed on consecutive patients with a prenatal diagnosis of HLHS with IAS/RAS presenting to a single center. The management strategy was immediate LA decompression followed by placement of bilateral pulmonary artery bands (bPAB) and subsequent conventional Norwood procedure. Six patients were born with HLHS with IAS/RAS during this time period with this planned management strategy. Four patients underwent LA decompression and subsequently developed low cardiac output with pulmonary overcirculation. bPAB were used with improvement in cardiac output and pronounced diuresis. These patients all survived the Norwood and subsequent Glenn procedures and remain alive [median follow-up 2.2 years (range 11 months-2.7 years)]. Two patients did not survive with therapy being withdrawn before the Norwood procedure. It is hypothesized that a strategy of total LA decompression followed by bPAB maximizes preoperative systemic perfusion and minimizes ongoing injury to the pulmonary system. This may enhance patient candidacy for the Norwood procedure and long-term survival.

Research paper thumbnail of Morphology of Interatrial Defects Created by Interventional Techniques in a Neonatal Animal Model

Pediatric Cardiology, 2014

Pathological details are lacking of various techniques used for interatrial defect (IAD) creation... more Pathological details are lacking of various techniques used for interatrial defect (IAD) creation in lesions requiring mixing or with left atrial hypertension. Therefore, the morphology of the IAD created by different interventional techniques is described. The atrial septa of ten euthanized piglets (seven at &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;3 days and three at 2 weeks of age) were surgically exposed. In pigs of both ages, a needle-created communication was dilated with angioplasty, cutting, and cryoplasty balloons. A stent was also implanted in a newborn pig. By way of a patent foramen ovale (PFO) in newborns, angioplasty and septostomy balloons and a stent created IADs. The morphology of the IAD was directly imaged and the size measured. Newborn piglets had noticeably thinner atrial septa, and a PFO was still present. Static balloon dilation created a circular IAD equivalent to the balloon diameter (3 and 6 mm) in both the created defects of pigs of both ages and the neonatal PFO. A 3-mm cutting balloon (CB) produced a 2-mm triangular IAD (corresponding to atherotomes) in pigs of both ages. Premounted stents, by way of a created defect or PFO, led to circular IADs equivalent to balloon diameter. The 3-mm cryoplasty balloon created a large 5 × 4-mm IAD in the newborn pig; however, the IAD measured only 2 × 1-mm at 2 weeks. By way of a neonatal PFO, a septostomy balloon (inflated to 2 cc) created a 3-mm circular IAD. In neonatal piglet hearts, static balloon angioplasty, CBs, and stents created a predictable IAD. Cryoplasty balloons created highly variable defects.

Research paper thumbnail of Abstract 15508: Animal Model of Fetal Aortic Valvuloplasty: Technical Success Maximized With Combination of Perventricular and Transhepatic Access

Circulation, Nov 26, 2013

Research paper thumbnail of The Epidemiology of Childhood Cardiomyopathy in Australia

New England Journal of Medicine, 2003

gions. New England and the Central Southwest region of the United States. Methods: All children w... more gions. New England and the Central Southwest region of the United States. Methods: All children with newly diagnosed cardiomyopathy in New England and the Central Southwest region of the United States between 1996 and 1999 were entered into a registry. Data was abstracted from medical records at all participating centers and including race, sex, age and type of cardiomyopathy. Incidence rates are calculated with 1990 Census data serving as the denominator. Results: 467 cases of cardiomyopathy were placed in the registry with an overall annual incidence of 1.13/100,000 children. Dilated cardiomyopathy accounted for 51% of cases, hypertrophic cardiomyopathy for 42%, and restrictive cardiomyopathy for 3% (4% unspecified). There was a greater incidence rate in New England (1.44/100,000) compared to the Central Southwest (.98/100,000). Black children had a higher incidence rate than white children and infants had a higher incidence rate then children Ͼ1 year of age. Conclusions: These data demonstrate an average cardiomyopathy incidence rate of 1.13/100,000, with some regional variation. Infants and Black children were at increased risk of being diagnosed with cardiomyopathy. Perspective: The summation of this large prospective registry from two geographic regions in the US is likely to provide more accurate incidence rates then retrospective data collection. The comparative data detailed in this study may be extremely helpful in evaluating future early detection, treatment, and prevention strategies for various pediatric cardiomyopathies. CG Data were acquired from all of Australia by site visits to all of the pediatric cardiac centers in Australia and contact with all adult cardiologists, rural pediatricians and cardiac transplantation centers. A single observer reviewed all available cardiac information to determine the classification of each case. The overall incidence rate and specific incidence rates for demographic subgroups were calculated using Australian age-specific population data. Results: There were 314 new cases of cardiomyopathy identified over the 10-year study period resulting in an overall annual incidence of 1.24/100,000 children. Dilated cardiomyopathy accounted for 58.6% of cases, hypertrophic cardiomyopathy 25.5% of cases and restrictive cardiomyopathy for 2.5% of cases. In addition, 9.2% of cases were defined as left ventricular non-compaction. Indigenous children had a higher incidence rate of cardiomyopathy (1.52/100,000 children) than non-indigenous children .57/ 100,000 children. Conclusions: The overall annual incidence rate of childhood cardiomyopathy is 1.24/100,000 children with the rate in indigenous children higher than in non-indigenous children. The majority of cases are consistent with a dilated cardiomyopathy. Left ventricular non-compaction accounted for a significant number of pediatric cardiomyopathy cases (9.2%). Perspective: While these data were collected retrospectively, the search for pertinent patient data seems to have been quite rigorous and the resulting sample population large. The relatively increased rate of non-compaction syndrome is surprising. It remains unclear if the echo phenotype of non-compaction stems from multiple etiologies. Large epidemiological studies like this one and that reviewed above by Lipshultz et al will help to improve the understanding of the etiologies of cardiomyopathy in children. CG

Research paper thumbnail of Presenting features and outcomes for children with dilated cardiomyopathy who develop persisting cardiac dysfunction

Journal of the American College of Cardiology, 2003

Research paper thumbnail of Abstract 16010: A Novel Biodegradable Stent Design Applicable for Pediatric Use in Congenital Heart Disease : Bench Testing and Initial Results in a Rabbit Model

Circulation, Nov 22, 2011

Research paper thumbnail of Long-Term Outcomes of Hypertrophic Cardiomyopathy Presenting During Childhood: Results From a National Population-Based Study of Childhood Cardiomyopathy

Journal of the American College of Cardiology, 2010

Background: Population based studies have changed the perception of hypertrophic cardiomyopathy (... more Background: Population based studies have changed the perception of hypertrophic cardiomyopathy (HCM) in affected adults. Long-term outcomes for children with HCM have not been well defined.

Research paper thumbnail of Long-Term Outcomes of Dilated Cardiomyopathy Presenting During Childhood: Results From a National Population-Based Study of Childhood Cardiomyopathy

Journal of the American College of Cardiology, 2010

Category: Pediatric Cardiology

Research paper thumbnail of Outcomes after stent implantation for the treatment of congenital and postoperative pulmonary vein stenosis in children

Circulation. Cardiovascular interventions, 2012

Pulmonary vein stenosis (PVS) is a rare condition that can lead to worsening pulmonary hypertensi... more Pulmonary vein stenosis (PVS) is a rare condition that can lead to worsening pulmonary hypertension and cardiac failure in children, and it is frequently lethal. Surgical and transcatheter approaches are acutely successful but restenosis is common and rapid. We reviewed outcomes among patients who underwent transcatheter pulmonary vein stent implantation for congenital or postoperative PVS at <18 years of age. A total of 74 pulmonary veins were stented with bare metal, drug-eluting, or covered stents in 47 patients. Primary diagnoses included PVS associated with anomalous venous return in 51%, PVS associated with other congenital cardiovascular defects in 36%, and congenital ("de novo") PVS in 13% of patients. Median age at the time of pulmonary vein stent implantation was 1.4 years. During a median cross-sectional follow-up of 3.1 years, 21 patients died. Estimated survival was 62±8% at 1 year and 50±8% at 5 years after pulmonary vein stent implantation. Stent placemen...

Research paper thumbnail of Survival and Reverse Cardiac Remodelling in Childhood Dilated Cardiomyopathy (DCM): Results From a Long-Term National Population-Based Study

Journal of the American College of Cardiology, 2012

Background: DCM in childhood is characterised by varied causes and heterogeneous outcomes. The in... more Background: DCM in childhood is characterised by varied causes and heterogeneous outcomes. The incidence of normalisation of cardiac function over time has not been well deined.

Research paper thumbnail of Atherotome Dislodgement and Successful Retrieval During Cutting Balloon Pulmonary Angioplasty

Circulation: Cardiovascular Interventions, 2012

Research paper thumbnail of Outcome for children with lymphocytic myocarditis enrolled in the National Australian Childhood Cardiomyopathy study

Journal of the American College of Cardiology, 2002

Background: 12-lead ECG has traditionally been an iniegral part of the noninvasive diagnostic eva... more Background: 12-lead ECG has traditionally been an iniegral part of the noninvasive diagnostic evaluation of patients with hypertrophic cardiomyopathy (HCM), and may reflect the degree of left ventdcular hypertrophy (LVH). Echocardiographic studies have recently shown magnitude of LVH to be directly related to prognosis, specifically as a predictor of sudden death risk (based on maximum [max.] wall thickness _> 30 ram).

Research paper thumbnail of Model of magnetically guided fetal cardiac intervention: potential to avoid direct cardiac puncture

Journal of Maternal-Fetal and Neonatal Medicine, 2013

Fetal cardiac interventions are performed via direct cardiac puncture and are associated with sig... more Fetal cardiac interventions are performed via direct cardiac puncture and are associated with significant fetal morbidity. The feasibility of utilizing magnetic navigation to maneuver a guide wire and balloon across a fetal aortic valve without direct cardiac puncture is tested. A fetal heart model was manufactured and placed in a catheterization laboratory equipped with magnetic navigation. Magnetically steerable guide wires along with commercially available coronary balloons were inserted into the model at a site mimicking a hepatic vein. Passage of the wire and balloon was achieved on every attempt. The model was suitable for testing although the structural characteristics of the model made wire passage from the right to the left atrium the most challenging aspect. Once the wire was positioned in the left ventricle, it was easily maneuvered 180 degrees towards the left ventricular outflow tract and then the descending aorta. Advancement of a coronary balloon over this wire was uncomplicated. In a fetal heart model, it is feasible to deliver a wire and balloon from abdominal venous access antegrade across the aortic valve. Progression to fetal lamb models is planned and may advance fetal cardiac interventions by reduction of fetal morbidity.

Research paper thumbnail of The natural history of restrictive cardiomyopathy presenting during childhood

Journal of the American College of Cardiology, 2002

ventricular septum (n=l), right ventricular free wail (n=l) and coronary cusp (n=l). Acutely 16/2... more ventricular septum (n=l), right ventricular free wail (n=l) and coronary cusp (n=l). Acutely 16/26 (62%) ablations were successful, 3i26 (12%) partially successful (arrhythmia became non sustained or there were other arrhythmias that were not targeted and 7/26 (27%) unsuccessful. Three of the unsuccessful ablation sessions were repeated within 3-20 days. There were 3 complications (1 respiratory distress, 1 cardiac perforation and 1 heart block). There was no recurrence at follow-up in 13/21 (62%) patients (mean follow up time 304.0±268.3 days). Time to recurrence (documented ventricular arrhythmia) in 8/21 (38%) patients was 137±158 days. There were 2 deaths at 3 and 34 days (1 due to ventricular arrhythmia and 1 due to carebrovascular accident). CONCLUSION: Although infrequent catheter based ablation for ventricular tachycardia in patients with NIDCM is relatively safe and has good short and long term success rates.

Research paper thumbnail of 34 Pediatric Interventional Catheterization: Development of a Risk Adjustment Model for Preventable Complications

Pediatric Research, 2005

Background Group B streptococcus (GBS) is the leading cause of early-onset infections (EOI) in th... more Background Group B streptococcus (GBS) is the leading cause of early-onset infections (EOI) in the developed world. Few data are available in Europe about incidence and clinical findings of neonatal disease. We reviewed cases occurred in the last 2 years in Emilia-Romagna (an area with about 4 500 000 million people).

Research paper thumbnail of 1152-199 Thromboembolic events among children with cardiomyopathy: Results from the National Australian childhood cardiomyopathy study

Journal of the American College of Cardiology, 2004

Results: Indications for PVR were valvular regurgitation (n=6), obstruction (n=3), and both (n=1)... more Results: Indications for PVR were valvular regurgitation (n=6), obstruction (n=3), and both (n=1). Valve types were St. Jude (n=7), Starr-Edwards (n=2), and Bjork-Shiley (n=1). In 5 pts, the mechanical PVR was placed within a conduit. Concomitant mechanical tricuspid valve replacement (St. Jude) was performed in 6 pts. Postoperatively, all but 1 were anticoagulated with warfarin. During late follow-up (8.3 +/-7.7 yrs, max 25 yrs), 1 pt required repeat PVR for outgrowth of prosthesis 25 yrs postoperatively, and 1 pt with normal prosthetic pulmonary valve function underwent heart transplantation for severe biventricular failure 3.5 yrs postoperatively. The average mean gradient across the mechanical PVR by echocardiography in the remaining 8 pts was 11 +/-7 mmHg. There were no perivalvular leaks or vegetations, and no evidence of pannus formation or prosthesis dysfunction. There were 3 late minor bleeding events (epistaxis in 2 pts and menorrhagia in 1). There was 1 sudden late death 15 yrs after PVR, and 1 death from unknown causes in the aforementioned pt with outgrowth of the mechanical PVR, 5.5 yrs post re-PVR with a bioprosthesis. There were no cases of pulmonary emboli or PVR thrombosis. Conclusions: Mechanical PVR appears to provide excellent durability and hemodynamic results without mechanical valve failure or valve thrombosis in this small series. Mechanical prostheses should be considered for PVR in selected pts, particularly those who require chronic warfarin anticoagulation for other reasons. 11:45 a.m.

Research paper thumbnail of 33 Neonatal Group B Streptococcal Infections in a North Italian Area

Pediatric Research, 2005

Background Group B streptococcus (GBS) is the leading cause of early-onset infections (EOI) in th... more Background Group B streptococcus (GBS) is the leading cause of early-onset infections (EOI) in the developed world. Few data are available in Europe about incidence and clinical findings of neonatal disease. We reviewed cases occurred in the last 2 years in Emilia-Romagna (an area with about 4 500 000 million people).

Research paper thumbnail of Pulmonary artery stents: Long-term follow-up

Catheterization and Cardiovascular Interventions, 2010

Objectives: Determine the long-term outcomes of branch pulmonary artery (PA) stents. Background: ... more Objectives: Determine the long-term outcomes of branch pulmonary artery (PA) stents. Background: PA stents in congenital heart disease effectively relieve stenoses in the short-term. Published long-term data are limited. Methods: Patients enrolled in an FDA IDE protocol from 1989-92 were included. Clinical follow-up and catheterization data were evaluated. Patients were included if >5 year follow-up data was available or if mortality occurred following the initial procedure. Results: There were five deaths: four due to progression of their underlying heart disease, and one from a complication during a follow-up catheterization. Clinical data for 43 surviving patients demonstrated 39 patients (91%) are in NYHA class I or II. Seven patients underwent surgical intervention during the follow-up period (five RV-PA conduit, two Fontan revisions), but none addressed PA stenosis. Final repeat catheterizations were performed in 36 patients (55 stents) 7.2 6 4.3 years post stent insertion with 1.2 6 0.9 further procedures with stent dilations. In this subgroup, the minimum vessel diameter increased from 4.7 6 1.8 to 13.4 6 2.4 mm (P < 0.001), and the pressure gradient improved from 41 6 25 to 9 6 11 mm Hg (P < 0.001). Higher initial gradient and smaller balloons were associated with a final stent diameter of <14 mm (P 5 0.030 and 0.046). Jailed vessels occurred in 49% of stents with abnormal angiographic flow in 18/55. Six repeat catheterizations resulted in complications, including the one procedural death. Conclusion: Stents implants for PA stenoses provide effective improvement in vessel caliber in the long-term. Although repeat interventions are necessary, this procedure reduces RV pressure and provides an important alternative to surgery for residual PA obstruction. V C 2009 Wiley-Liss, Inc.

Research paper thumbnail of Focal seizure and cerebral contrast retention after cardiac catheterization

Pediatric Neurology, 2005

Although rare, ionic and nonionic contrast-induced seizures occur as a complication of enhanced c... more Although rare, ionic and nonionic contrast-induced seizures occur as a complication of enhanced cranial computed tomography for both adults and children. However, contrast-induced seizures after cardiac catheterization has only been reported in adults. This report describes an 18-month-old male who developed a new-onset focal seizure 12 hours after cardiac catheterization. Unenhanced cranial computed tomography 1 hour after the seizure demonstrated general cerebral edema and unilateral focal cerebral contrast retention with sparing of the area supplied by the middle cerebral artery. The contrast was reabsorbed from the subarachnoid space over a 48-hour period, the cerebral edema resolved over several days, and the child returned to his baseline state 4 days after the seizure episode. This study documents the evolution of computed tomographic findings after contrast-induced seizures in a child. Contrast toxicity should be considered in any case of a new-onset neurologic deficit arising after angiography or enhanced computed tomography.

Research paper thumbnail of Transcatheter Delivery of a Replacement Heart Valve

Research paper thumbnail of Optimization of Preoperative Status in Hypoplastic Left Heart Syndrome With Intact Atrial Septum by Left Atrial Decompression and Bilateral Pulmonary Artery Bands

Pediatric Cardiology, 2014

Hypoplastic left heart syndrome (HLHS) with intact (IAS) or highly restrictive atrial septum (RAS... more Hypoplastic left heart syndrome (HLHS) with intact (IAS) or highly restrictive atrial septum (RAS) has extremely poor outcomes largely related to pulmonary pathology. At birth, immediate left atrial (LA) decompression is required to remain viable, but there is a tradeoff between residual increase in LA pressure and pulmonary overcirculation, either of which exacerbates the pulmonary status. From August 2010 to April 2013, a retrospective chart review was performed on consecutive patients with a prenatal diagnosis of HLHS with IAS/RAS presenting to a single center. The management strategy was immediate LA decompression followed by placement of bilateral pulmonary artery bands (bPAB) and subsequent conventional Norwood procedure. Six patients were born with HLHS with IAS/RAS during this time period with this planned management strategy. Four patients underwent LA decompression and subsequently developed low cardiac output with pulmonary overcirculation. bPAB were used with improvement in cardiac output and pronounced diuresis. These patients all survived the Norwood and subsequent Glenn procedures and remain alive [median follow-up 2.2 years (range 11 months-2.7 years)]. Two patients did not survive with therapy being withdrawn before the Norwood procedure. It is hypothesized that a strategy of total LA decompression followed by bPAB maximizes preoperative systemic perfusion and minimizes ongoing injury to the pulmonary system. This may enhance patient candidacy for the Norwood procedure and long-term survival.

Research paper thumbnail of Morphology of Interatrial Defects Created by Interventional Techniques in a Neonatal Animal Model

Pediatric Cardiology, 2014

Pathological details are lacking of various techniques used for interatrial defect (IAD) creation... more Pathological details are lacking of various techniques used for interatrial defect (IAD) creation in lesions requiring mixing or with left atrial hypertension. Therefore, the morphology of the IAD created by different interventional techniques is described. The atrial septa of ten euthanized piglets (seven at &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;3 days and three at 2 weeks of age) were surgically exposed. In pigs of both ages, a needle-created communication was dilated with angioplasty, cutting, and cryoplasty balloons. A stent was also implanted in a newborn pig. By way of a patent foramen ovale (PFO) in newborns, angioplasty and septostomy balloons and a stent created IADs. The morphology of the IAD was directly imaged and the size measured. Newborn piglets had noticeably thinner atrial septa, and a PFO was still present. Static balloon dilation created a circular IAD equivalent to the balloon diameter (3 and 6 mm) in both the created defects of pigs of both ages and the neonatal PFO. A 3-mm cutting balloon (CB) produced a 2-mm triangular IAD (corresponding to atherotomes) in pigs of both ages. Premounted stents, by way of a created defect or PFO, led to circular IADs equivalent to balloon diameter. The 3-mm cryoplasty balloon created a large 5 × 4-mm IAD in the newborn pig; however, the IAD measured only 2 × 1-mm at 2 weeks. By way of a neonatal PFO, a septostomy balloon (inflated to 2 cc) created a 3-mm circular IAD. In neonatal piglet hearts, static balloon angioplasty, CBs, and stents created a predictable IAD. Cryoplasty balloons created highly variable defects.

Research paper thumbnail of Abstract 15508: Animal Model of Fetal Aortic Valvuloplasty: Technical Success Maximized With Combination of Perventricular and Transhepatic Access

Circulation, Nov 26, 2013

Research paper thumbnail of The Epidemiology of Childhood Cardiomyopathy in Australia

New England Journal of Medicine, 2003

gions. New England and the Central Southwest region of the United States. Methods: All children w... more gions. New England and the Central Southwest region of the United States. Methods: All children with newly diagnosed cardiomyopathy in New England and the Central Southwest region of the United States between 1996 and 1999 were entered into a registry. Data was abstracted from medical records at all participating centers and including race, sex, age and type of cardiomyopathy. Incidence rates are calculated with 1990 Census data serving as the denominator. Results: 467 cases of cardiomyopathy were placed in the registry with an overall annual incidence of 1.13/100,000 children. Dilated cardiomyopathy accounted for 51% of cases, hypertrophic cardiomyopathy for 42%, and restrictive cardiomyopathy for 3% (4% unspecified). There was a greater incidence rate in New England (1.44/100,000) compared to the Central Southwest (.98/100,000). Black children had a higher incidence rate than white children and infants had a higher incidence rate then children Ͼ1 year of age. Conclusions: These data demonstrate an average cardiomyopathy incidence rate of 1.13/100,000, with some regional variation. Infants and Black children were at increased risk of being diagnosed with cardiomyopathy. Perspective: The summation of this large prospective registry from two geographic regions in the US is likely to provide more accurate incidence rates then retrospective data collection. The comparative data detailed in this study may be extremely helpful in evaluating future early detection, treatment, and prevention strategies for various pediatric cardiomyopathies. CG Data were acquired from all of Australia by site visits to all of the pediatric cardiac centers in Australia and contact with all adult cardiologists, rural pediatricians and cardiac transplantation centers. A single observer reviewed all available cardiac information to determine the classification of each case. The overall incidence rate and specific incidence rates for demographic subgroups were calculated using Australian age-specific population data. Results: There were 314 new cases of cardiomyopathy identified over the 10-year study period resulting in an overall annual incidence of 1.24/100,000 children. Dilated cardiomyopathy accounted for 58.6% of cases, hypertrophic cardiomyopathy 25.5% of cases and restrictive cardiomyopathy for 2.5% of cases. In addition, 9.2% of cases were defined as left ventricular non-compaction. Indigenous children had a higher incidence rate of cardiomyopathy (1.52/100,000 children) than non-indigenous children .57/ 100,000 children. Conclusions: The overall annual incidence rate of childhood cardiomyopathy is 1.24/100,000 children with the rate in indigenous children higher than in non-indigenous children. The majority of cases are consistent with a dilated cardiomyopathy. Left ventricular non-compaction accounted for a significant number of pediatric cardiomyopathy cases (9.2%). Perspective: While these data were collected retrospectively, the search for pertinent patient data seems to have been quite rigorous and the resulting sample population large. The relatively increased rate of non-compaction syndrome is surprising. It remains unclear if the echo phenotype of non-compaction stems from multiple etiologies. Large epidemiological studies like this one and that reviewed above by Lipshultz et al will help to improve the understanding of the etiologies of cardiomyopathy in children. CG

Research paper thumbnail of Presenting features and outcomes for children with dilated cardiomyopathy who develop persisting cardiac dysfunction

Journal of the American College of Cardiology, 2003

Research paper thumbnail of Abstract 16010: A Novel Biodegradable Stent Design Applicable for Pediatric Use in Congenital Heart Disease : Bench Testing and Initial Results in a Rabbit Model

Circulation, Nov 22, 2011

Research paper thumbnail of Long-Term Outcomes of Hypertrophic Cardiomyopathy Presenting During Childhood: Results From a National Population-Based Study of Childhood Cardiomyopathy

Journal of the American College of Cardiology, 2010

Background: Population based studies have changed the perception of hypertrophic cardiomyopathy (... more Background: Population based studies have changed the perception of hypertrophic cardiomyopathy (HCM) in affected adults. Long-term outcomes for children with HCM have not been well defined.

Research paper thumbnail of Long-Term Outcomes of Dilated Cardiomyopathy Presenting During Childhood: Results From a National Population-Based Study of Childhood Cardiomyopathy

Journal of the American College of Cardiology, 2010

Category: Pediatric Cardiology

Research paper thumbnail of Outcomes after stent implantation for the treatment of congenital and postoperative pulmonary vein stenosis in children

Circulation. Cardiovascular interventions, 2012

Pulmonary vein stenosis (PVS) is a rare condition that can lead to worsening pulmonary hypertensi... more Pulmonary vein stenosis (PVS) is a rare condition that can lead to worsening pulmonary hypertension and cardiac failure in children, and it is frequently lethal. Surgical and transcatheter approaches are acutely successful but restenosis is common and rapid. We reviewed outcomes among patients who underwent transcatheter pulmonary vein stent implantation for congenital or postoperative PVS at <18 years of age. A total of 74 pulmonary veins were stented with bare metal, drug-eluting, or covered stents in 47 patients. Primary diagnoses included PVS associated with anomalous venous return in 51%, PVS associated with other congenital cardiovascular defects in 36%, and congenital ("de novo") PVS in 13% of patients. Median age at the time of pulmonary vein stent implantation was 1.4 years. During a median cross-sectional follow-up of 3.1 years, 21 patients died. Estimated survival was 62±8% at 1 year and 50±8% at 5 years after pulmonary vein stent implantation. Stent placemen...

Research paper thumbnail of Survival and Reverse Cardiac Remodelling in Childhood Dilated Cardiomyopathy (DCM): Results From a Long-Term National Population-Based Study

Journal of the American College of Cardiology, 2012

Background: DCM in childhood is characterised by varied causes and heterogeneous outcomes. The in... more Background: DCM in childhood is characterised by varied causes and heterogeneous outcomes. The incidence of normalisation of cardiac function over time has not been well deined.

Research paper thumbnail of Atherotome Dislodgement and Successful Retrieval During Cutting Balloon Pulmonary Angioplasty

Circulation: Cardiovascular Interventions, 2012

Research paper thumbnail of Outcome for children with lymphocytic myocarditis enrolled in the National Australian Childhood Cardiomyopathy study

Journal of the American College of Cardiology, 2002

Background: 12-lead ECG has traditionally been an iniegral part of the noninvasive diagnostic eva... more Background: 12-lead ECG has traditionally been an iniegral part of the noninvasive diagnostic evaluation of patients with hypertrophic cardiomyopathy (HCM), and may reflect the degree of left ventdcular hypertrophy (LVH). Echocardiographic studies have recently shown magnitude of LVH to be directly related to prognosis, specifically as a predictor of sudden death risk (based on maximum [max.] wall thickness _> 30 ram).

Research paper thumbnail of Model of magnetically guided fetal cardiac intervention: potential to avoid direct cardiac puncture

Journal of Maternal-Fetal and Neonatal Medicine, 2013

Fetal cardiac interventions are performed via direct cardiac puncture and are associated with sig... more Fetal cardiac interventions are performed via direct cardiac puncture and are associated with significant fetal morbidity. The feasibility of utilizing magnetic navigation to maneuver a guide wire and balloon across a fetal aortic valve without direct cardiac puncture is tested. A fetal heart model was manufactured and placed in a catheterization laboratory equipped with magnetic navigation. Magnetically steerable guide wires along with commercially available coronary balloons were inserted into the model at a site mimicking a hepatic vein. Passage of the wire and balloon was achieved on every attempt. The model was suitable for testing although the structural characteristics of the model made wire passage from the right to the left atrium the most challenging aspect. Once the wire was positioned in the left ventricle, it was easily maneuvered 180 degrees towards the left ventricular outflow tract and then the descending aorta. Advancement of a coronary balloon over this wire was uncomplicated. In a fetal heart model, it is feasible to deliver a wire and balloon from abdominal venous access antegrade across the aortic valve. Progression to fetal lamb models is planned and may advance fetal cardiac interventions by reduction of fetal morbidity.

Research paper thumbnail of The natural history of restrictive cardiomyopathy presenting during childhood

Journal of the American College of Cardiology, 2002

ventricular septum (n=l), right ventricular free wail (n=l) and coronary cusp (n=l). Acutely 16/2... more ventricular septum (n=l), right ventricular free wail (n=l) and coronary cusp (n=l). Acutely 16/26 (62%) ablations were successful, 3i26 (12%) partially successful (arrhythmia became non sustained or there were other arrhythmias that were not targeted and 7/26 (27%) unsuccessful. Three of the unsuccessful ablation sessions were repeated within 3-20 days. There were 3 complications (1 respiratory distress, 1 cardiac perforation and 1 heart block). There was no recurrence at follow-up in 13/21 (62%) patients (mean follow up time 304.0±268.3 days). Time to recurrence (documented ventricular arrhythmia) in 8/21 (38%) patients was 137±158 days. There were 2 deaths at 3 and 34 days (1 due to ventricular arrhythmia and 1 due to carebrovascular accident). CONCLUSION: Although infrequent catheter based ablation for ventricular tachycardia in patients with NIDCM is relatively safe and has good short and long term success rates.

Research paper thumbnail of 34 Pediatric Interventional Catheterization: Development of a Risk Adjustment Model for Preventable Complications

Pediatric Research, 2005

Background Group B streptococcus (GBS) is the leading cause of early-onset infections (EOI) in th... more Background Group B streptococcus (GBS) is the leading cause of early-onset infections (EOI) in the developed world. Few data are available in Europe about incidence and clinical findings of neonatal disease. We reviewed cases occurred in the last 2 years in Emilia-Romagna (an area with about 4 500 000 million people).

Research paper thumbnail of 1152-199 Thromboembolic events among children with cardiomyopathy: Results from the National Australian childhood cardiomyopathy study

Journal of the American College of Cardiology, 2004

Results: Indications for PVR were valvular regurgitation (n=6), obstruction (n=3), and both (n=1)... more Results: Indications for PVR were valvular regurgitation (n=6), obstruction (n=3), and both (n=1). Valve types were St. Jude (n=7), Starr-Edwards (n=2), and Bjork-Shiley (n=1). In 5 pts, the mechanical PVR was placed within a conduit. Concomitant mechanical tricuspid valve replacement (St. Jude) was performed in 6 pts. Postoperatively, all but 1 were anticoagulated with warfarin. During late follow-up (8.3 +/-7.7 yrs, max 25 yrs), 1 pt required repeat PVR for outgrowth of prosthesis 25 yrs postoperatively, and 1 pt with normal prosthetic pulmonary valve function underwent heart transplantation for severe biventricular failure 3.5 yrs postoperatively. The average mean gradient across the mechanical PVR by echocardiography in the remaining 8 pts was 11 +/-7 mmHg. There were no perivalvular leaks or vegetations, and no evidence of pannus formation or prosthesis dysfunction. There were 3 late minor bleeding events (epistaxis in 2 pts and menorrhagia in 1). There was 1 sudden late death 15 yrs after PVR, and 1 death from unknown causes in the aforementioned pt with outgrowth of the mechanical PVR, 5.5 yrs post re-PVR with a bioprosthesis. There were no cases of pulmonary emboli or PVR thrombosis. Conclusions: Mechanical PVR appears to provide excellent durability and hemodynamic results without mechanical valve failure or valve thrombosis in this small series. Mechanical prostheses should be considered for PVR in selected pts, particularly those who require chronic warfarin anticoagulation for other reasons. 11:45 a.m.

Research paper thumbnail of 33 Neonatal Group B Streptococcal Infections in a North Italian Area

Pediatric Research, 2005

Background Group B streptococcus (GBS) is the leading cause of early-onset infections (EOI) in th... more Background Group B streptococcus (GBS) is the leading cause of early-onset infections (EOI) in the developed world. Few data are available in Europe about incidence and clinical findings of neonatal disease. We reviewed cases occurred in the last 2 years in Emilia-Romagna (an area with about 4 500 000 million people).