Training for structural and adult congenital heart disease interventions remains undeveloped. Wit... more Training for structural and adult congenital heart disease interventions remains undeveloped. With the advent of recent percutaneous interventions for the treatment of structural and valvular heart disease, such as transcatheter aortic and pulmonary valve implantation, mitral valve repair, and the expansion of shunt closure procedures, there is a clear need to define the training requirements for this category of procedures. The training needs to be aligned with the goals and priorities of a basic or advanced level and be categorized into acquired and congenital. This document will define the training needs and knowledge base for the developing field of structural heart disease intervention.
We carried out a device-needs survey to evaluate the gaps in device and equipment availability fo... more We carried out a device-needs survey to evaluate the gaps in device and equipment availability for congenital interventional cardiologists. As the complexity and demand for more complete solutions to congenital heart lesions increase, there is a growing need for modification and development of devices and equipment to support this endeavor. The survey was sent out via e-mail to members of the Congenital Cardiovascular Interventional Study Consortium and the Society for Cardiac Angiography and Interventions with a reach of over 350 congenital interventionalists. Responses were received from 68 cardiologists in 8 countries. In terms of the most desired device, 41 % ranked bioresorbable stents as their first choice from a list of 12 possible devices. Similarly, 23 % ranked large covered stents as their first choice. Twenty-seven percent of participants believed bioresorbable stents would have the greatest potential to improve morbidity of their patients, with another 27 % reporting tha...
We sought to report the results of a U.S. registry of device closure of congenital muscular ventr... more We sought to report the results of a U.S. registry of device closure of congenital muscular ventricular septal defects (VSDs) using the new Amplatzer mVSD occluder (AGA Medical Corp., Golden Valley, Minnesota).Muscular VSDs pose a significant surgical challenge with increased morbidity and mortality.Data were prospectively collected from 83 procedures involving 75 patients who underwent an attempt of percutaneous (70 [93.3%] of 75) and/or perventricular (surgical) (6 [8.0%] of 75) device closure of hemodynamically significant muscular VSDs. The patients' median age was 1.4 years (range 0.1 to 54.1 years). Outcome parameters were procedural success, evidence of residual shunts on echocardiography, and occurrence of procedure-related complications. The median follow-up was 211 days (range 1 to 859 days).The median size of the primary VSD was 7 mm (range 3 to 16 mm) and in 34 of 78 (43.6%) procedures, patients had multiple VSDs (range 2 to 7). The device was implanted successfully in 72 of 83 (86.7%) procedures. In 17 of 83 (20.5%) procedures, multiple devices were implanted (range 2 to 3). Procedure-related major complications occurred in 8 of 75 (10.7%) patients. Device embolization occurred in two patients and cardiac perforation in one patient. There were two (2.7%) procedure-related deaths. The 24-h postprocedural complete closure rate was 47.2% (34 of 72 patients), increasing to 69.6% (32 of 46 patients) at 6 months and 92.3% (24 of 26 patients) at 12 months. Six patients underwent successful closure using the perventricular surgical (beating heart) approach, with complete closure at day 1 in three patients and trivial/small residual shunts in the remainder of the patients.The Amplatzer mVSD device (AGA Medical Corp.) offers excellent closure rates and low mortality when used to close congenital muscular VSDs. The device appears to be safe and effective.
Journal of Cardiovascular Electrophysiology, Dec 1, 1997
Late Sudden Death Risk in Postoperative TOF. Following surgery for tetralogy of Fallot (TOP), chi... more Late Sudden Death Risk in Postoperative TOF. Following surgery for tetralogy of Fallot (TOP), children may develop late onset ventritular arrhythmia.s. Many patients have both depolarization and repolarization abnormalities, including right bundle branch block (RBBB) and QT prolongation. The goal of thi.s .study was to improve prospective risk-assessment screening for late onset sudden death. Resting ECG markers including QRS duration, QTc, JTc, and interlead QT and JT dispersion were statistically analyzed to identify those patients at risk for ventricular arrhythmias and sudden cardiac death. To determine predictive markers for future development of arrhythmia, we examined 101 resting ECGs in patients (age 12 ± 6 years) with postoperative TOF and RBBB, 14 of whom developed late ventricular tachycardia (VT) or sudden death. These ECGs were also compared with an additional control group of 1000 age-and gender-matched normal ECGs. The mean QRS (± SD) in the VT group was 0.18 ± 0.02 seconds versus 0.14 ± 0.02 seconds in the non-VT group {P < 0.01). QTc and JTc in the VT group was 0.53 ± 0.05 seconds and 0.33 ± 0.03 seconds compared with 0.50 ± 0.03 seconds and 0.32 ± 0.03 seconds in the non-VT group (P = NS). There was no increase in QT dispersion among TOF patients with VT or sudden death compared with control patients or TOF patients without VT, although JT dispersion was more common in the TOF groups. A prolonged QRS duration in postoperative TOF with RBBB is more predictive than QTc, JTc, or dispersion indexes for identifying vulnerahility to ventricular arrhythmias in this population, while retaining high specificity. The combination of hoth QRS prolongation and increased JT dispersion had very good positive and negative predictive values. These results suggest that arrhythmogenesis in children following TOF surgery might involve depolarization in addition to repolarization abnormalities. Prospective identification of high-risk children may he accomplished using these ECG criteria.
It has been suggested that hibernation, a condition of chronic pedusinn-COntraction down-regulati... more It has been suggested that hibernation, a condition of chronic pedusinn-COntraction down-regulation with preserved viability, results from recurrent episodes of reversible ischemia and "chronic" postischemic dysfunction. We examined the interrelation between flow, metalxdism, and function in an acute model of repetitive stunning. Nine dogs underwent four 5 rain balloon occlusions of the LAD or circumflex arteries, each separated by 5 rain of reperfusion. Regional blood flow (BF), metabolism and function were evaluated 2 hourS after reperfusion in 5 dogs, and 2 hours, 24 hours, and 1 week post-reperfual0n in 4 dogs, Regional wall motion 0NM) was evaluated with 2-D echo and 8F with radiotsbalod micraspheres. Measurements of oxidative metabolism (MVO2) and glucose uptake (during hypednsulinemioeuglycemic ¢tsmp) were derived with PET imaging. Regional WM was severely decreased after the 4 Cycles of ischemia, remained severely impaired 2 hours after repertusion, and normalized after 1 week. Dudng reflow, BF in stunned regions was restored to near-normal levels (0.89 • 0,2 vs 0.95:1:0.2 ml/g/min, P < 0.01). Glucose uptake in stunned regions was depressed at 2 hours (73 • 15% of control, P < 0.001) and 24 hours after retiow (80 4-7% of ~ontrel, P = 0.04), but recovered after I week (100 • 26°/= of control, P = NS), Similarly, MVO2 in stunned regions was decreased at 2 hours (84 :E 7% of COntrol, P < 0.001 ) and at 24 hours (8~) :E 6% of COntrol, P = 0.02), and recovered 1 week after reperfusion (97 • 3% of COntrol, P = NS). Thus, repetitive stunned myocardium demonal~tod a persistent reduction of both MVO2 and glucose uptake which recovered as regional wall motion improved. The resu~ may have important implk;ations for detecting stunning in patients with CAD and further suggest a unique metabolic adaptation in "chronic stunning ~, different from that typically seen in hibemalion. ~ Tc-99m.Labeled Ap4A for Early Gamma Scintigrephlc Visualization of Experimental Athemscleroti¢ Lesions Substances related to ATP such as diadenosine tetraphosphate (Ap4A) bind to purina receptors in vascular smooth muscle o~IL Since proliferating smooth muscle cells are an obligatory COmponent of atherosolerotic lesions, T¢-99m-Ap4A was used to assess whether puflne receptors are upregutsted and can detect atherosclerottc lesions in vive.
Coarctation of the aorta was once viewed as a simple discrete narrowing of the aortic isthmus tha... more Coarctation of the aorta was once viewed as a simple discrete narrowing of the aortic isthmus that could be 'cured' by surgical intervention. It is now clear that this condition may: (1) affect the aortic arch in a highly variable manner; (2) be associated with a host of other left sided heart lesions; (3) represent a wider vasculopathy within the pre-coarctation arterial tree, leading to significant prevalence of hypertension by adolescence, and subsequent risk of early morbidity and death. This review outlines the evaluation and treatment of this disease from pre-natal to adult life. (Cardiol J 2011; 18, 5: 487-495)
The hybrid approach to management of hypoplastic left heart syndrome (HLHS) was developed as an a... more The hybrid approach to management of hypoplastic left heart syndrome (HLHS) was developed as an alternative to neonatal Norwood surgery, providing a less invasive initial palliation for HLHS. We describe our experience in extending the concept of the hybrid procedure to palliate neonates with anatomically compromised systemic arterial blood flow in a variety of congenital cardiac anomalies and supporting its application as first-line palliation in centers developing their HLHS programs. Retrospective review of patients undergoing therapy for HLHS at a single institution from June 2008 to December 2014 was performed. Subject demographics, clinical and procedural data, along with follow-up, were collected. Thirteen patients had initial hybrid palliation for HLHS during the time frame indicated at a median age of 8 days (range 1-29 days) and median weight of 3.4 kg (range 2.4-4.6 kg). Diagnoses included typical HLHS (n = 6), right-dominant unbalanced atrioventricular septal defect with arch hypoplasia (n = 4), double outlet right ventricle [subpulmonic VSD (n = 1) and intact ventricular septum (n = 1)] with hypoplastic transverse aortic arch and borderline left ventricular dimensions. Standard approach with bilateral pulmonary artery banding and ductal stenting was carried out in all thirteen patients. Two patients required two ductal stents at the time of index procedure. There were no intraprocedural complications. Median intubation length post-procedure was 4 days (range 1-74 days). Median hospital stay post-procedure was 47 days (range 15-270 days). The overall mortality rate on follow-up through comprehensive stage 2 over the 6-year experience was 38 % (5 out of 13). Of note, the mortality rate was significantly lower in the latter 3 years of the study period when the procedure was adopted as a primary palliation for HLHS (14 % or 1 out of 7) compared to the initial 3-year period when it was reserved for higher risk cohorts (67 % or 4 out of 6). Median time to subsequent surgery was 3 months (range 1-4 months). One patient required further ductal stenting on follow-up and developed subsequently airway compression. On median follow-up of 24 months, two patients required pulmonary artery arterioplasty. The hybrid procedure may be used for palliation for a variety of cardiac lesions to avoid high-risk surgery in the neonatal period. This approach may be also an alternative in centers performing lower number of Norwood surgery, which has been associated with higher mortality.
Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions, Jan 8, 2015
Distortion of transcatheter heart valve (THV) stent shape and morphology has been shown to impact... more Distortion of transcatheter heart valve (THV) stent shape and morphology has been shown to impact on THV function. This study sought to evaluate the relationship between geometrical CT follow-up data and consequent valve function in patients undergoing transcatheter pulmonary valve replacement with the Edwards SAPIEN THV. All patients were enrolled in the COMPASSION trial. Multidetector computed tomography (MDCT) was performed as part of the study protocol at 6 months and yearly thereafter following valve implantation. Prosthesis eccentricity indices, circularity ratios, and expansion ratios (ER) were calculated. Valve function and reintervention rates were correlated with MDCT findings. Twenty consecutive patients undergoing 58 CT scans were included. Maximum Doppler gradients across the right ventricular outflow tract (RVOT) were significantly reduced following valve implantation (P < 0.001). Geometrical indices and maximum RVOT gradients were compared between the reinterventio...
Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography, Jan 24, 2015
Speckle-tracking echocardiographic (STE) measures of right ventricular (RV) function appear to im... more Speckle-tracking echocardiographic (STE) measures of right ventricular (RV) function appear to improve after transcatheter pulmonary valve implantation (TPVI). Measures of exercise function, such as ventilatory efficiency (the minute ventilation [VE]/carbon dioxide production [Vco2] slope), have been shown to be prognostic of mortality in patients who may require TPVI. The aim of this study was to evaluate the correlation between STE measures of RV function and changes in VE/Vco2 after TPVI. Speckle-tracking echocardiography and cardiopulmonary exercise testing were performed at baseline and 6 months after TPVI in 24 patients from four centers. Conventional echocardiographic measures of RV function were also assessed. Echocardiographic and exercise stress test results were interpreted by single blinded observers at separate core laboratories. All patients demonstrated relief of pulmonary regurgitation and stenosis after TPVI. Improvements in RV longitudinal strain (-16.9 ± 3.5% vs -...
Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions, 2011
We describe our experience with self-fabricated covered stents in the setting of coarctation of t... more We describe our experience with self-fabricated covered stents in the setting of coarctation of the aorta (CoA). Balloon-expandable covered stents are increasingly being utilized to treat CoA in older children and adults. These stents however, are not available in the United States limiting the interventionalist's ability to treat this condition safely and effectively. Retrospective analysis and follow-up data review of our complete experience with self-fabricated covered stents for CoA. Stents were fashioned by suturing an appropriate length of tubular polytetraflouroethylene to a bare metal stent and deploying this stent across the coarctation in a standardized fashion. Over a 9-year period we implanted 53 balloon-expandable stents in 49 patients with CoA. Of these 13 were self-fabricated covered stents deployed in 13 patients (7 male). Median age at implantation was 25.4 years (range, 8.7-49.5 years) with median weight of 65.5 kg (range, 28-168 kg). Indications for stent plac...
The evolution of congenital cardiac surgery has seen significant innovative advances in collabora... more The evolution of congenital cardiac surgery has seen significant innovative advances in collaborative efforts between congenital cardiac surgeons and interventionalists to provide the least invasive intervention with the greatest hemodynamic benefit for patients with congenital heart disease. This review looks at how this collaborative approach has evolved and is being applied to treat a number of congenital conditions across the age ranges.
Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions, 2014
Balloon expandable transcatheter pulmonary valve systems are not applicable to the large majority... more Balloon expandable transcatheter pulmonary valve systems are not applicable to the large majority of patients with chronic severe pulmonary regurgitation (PR) following surgical right ventricular outflow tract (RVOT) rehabilitation. This report describes the clinical use and short-term follow-up of a novel transcatheter self-expanding pulmonary valve system (Venus P Valve) for rehabilitation of the RVOT in patients with chronic severe PR. Patients with native RVOT and severe PR were selected on a case-by-case basis as part of early clinical experience with this valve. Patient demographics and pre-procedural, intra-procedural, and follow-up data were reviewed. Five patients (four females) with a mean weight of 54.8 ± 11.4 kg were selected for attempted valve deployment. Patients were either NYHA class II (n = 3) or class III (n = 2) at baseline. PR was grade 4 in all cases with mean right ventricular end-diastolic volumes of 155.0 ± 16.6 ml/m(2) on cardiac MRI. Mean minimum "ann...
Coarctation of the aorta was once viewed as a simple discrete narrowing of the aortic isthmus tha... more Coarctation of the aorta was once viewed as a simple discrete narrowing of the aortic isthmus that could be 'cured' by surgical intervention. It is now clear that this condition may: (1) affect the aortic arch in a highly variable manner; (2) be associated with a host of other left sided heart lesions; (3) represent a wider vasculopathy within the pre-coarctation arterial tree, leading to significant prevalence of hypertension by adolescence, and subsequent risk of early morbidity and death. This review outlines the evaluation and treatment of this disease from pre-natal to adult life.
Catheterization and cardiovascular diagnosis, 1996
Within the field of pediatric cardiology, a number of subspecialty fields are generally recognize... more Within the field of pediatric cardiology, a number of subspecialty fields are generally recognized. Some of these overlap. For example, most electrophysiologists also would consider themselves to be clinical cardiologists. Some fields, however, are relatively mutually exclusive. For example, most clinical pediatric cardiologists would not consider themselves to be electrophysiologists and would admit that there is a different knowledge, skill, and experience base that separates an electrophysiologist from other specialists within the broader field of pediatric cardiology. Likewise, it is our opinion that a separate knowledge, skill, and experience base exists among pediatric Invasive/Interventional cardiologists. The purpose of this report is to define the unique knowledge and skill base required for the training of an invasive pediatric cardiologist. The scope of this report is limited to the training of Invasive/Interventional cardiologists dealing with the treatment of pediatric ...
Training for structural and adult congenital heart disease interventions remains undeveloped. Wit... more Training for structural and adult congenital heart disease interventions remains undeveloped. With the advent of recent percutaneous interventions for the treatment of structural and valvular heart disease, such as transcatheter aortic and pulmonary valve implantation, mitral valve repair, and the expansion of shunt closure procedures, there is a clear need to define the training requirements for this category of procedures. The training needs to be aligned with the goals and priorities of a basic or advanced level and be categorized into acquired and congenital. This document will define the training needs and knowledge base for the developing field of structural heart disease intervention.
We carried out a device-needs survey to evaluate the gaps in device and equipment availability fo... more We carried out a device-needs survey to evaluate the gaps in device and equipment availability for congenital interventional cardiologists. As the complexity and demand for more complete solutions to congenital heart lesions increase, there is a growing need for modification and development of devices and equipment to support this endeavor. The survey was sent out via e-mail to members of the Congenital Cardiovascular Interventional Study Consortium and the Society for Cardiac Angiography and Interventions with a reach of over 350 congenital interventionalists. Responses were received from 68 cardiologists in 8 countries. In terms of the most desired device, 41 % ranked bioresorbable stents as their first choice from a list of 12 possible devices. Similarly, 23 % ranked large covered stents as their first choice. Twenty-seven percent of participants believed bioresorbable stents would have the greatest potential to improve morbidity of their patients, with another 27 % reporting tha...
We sought to report the results of a U.S. registry of device closure of congenital muscular ventr... more We sought to report the results of a U.S. registry of device closure of congenital muscular ventricular septal defects (VSDs) using the new Amplatzer mVSD occluder (AGA Medical Corp., Golden Valley, Minnesota).Muscular VSDs pose a significant surgical challenge with increased morbidity and mortality.Data were prospectively collected from 83 procedures involving 75 patients who underwent an attempt of percutaneous (70 [93.3%] of 75) and/or perventricular (surgical) (6 [8.0%] of 75) device closure of hemodynamically significant muscular VSDs. The patients' median age was 1.4 years (range 0.1 to 54.1 years). Outcome parameters were procedural success, evidence of residual shunts on echocardiography, and occurrence of procedure-related complications. The median follow-up was 211 days (range 1 to 859 days).The median size of the primary VSD was 7 mm (range 3 to 16 mm) and in 34 of 78 (43.6%) procedures, patients had multiple VSDs (range 2 to 7). The device was implanted successfully in 72 of 83 (86.7%) procedures. In 17 of 83 (20.5%) procedures, multiple devices were implanted (range 2 to 3). Procedure-related major complications occurred in 8 of 75 (10.7%) patients. Device embolization occurred in two patients and cardiac perforation in one patient. There were two (2.7%) procedure-related deaths. The 24-h postprocedural complete closure rate was 47.2% (34 of 72 patients), increasing to 69.6% (32 of 46 patients) at 6 months and 92.3% (24 of 26 patients) at 12 months. Six patients underwent successful closure using the perventricular surgical (beating heart) approach, with complete closure at day 1 in three patients and trivial/small residual shunts in the remainder of the patients.The Amplatzer mVSD device (AGA Medical Corp.) offers excellent closure rates and low mortality when used to close congenital muscular VSDs. The device appears to be safe and effective.
Journal of Cardiovascular Electrophysiology, Dec 1, 1997
Late Sudden Death Risk in Postoperative TOF. Following surgery for tetralogy of Fallot (TOP), chi... more Late Sudden Death Risk in Postoperative TOF. Following surgery for tetralogy of Fallot (TOP), children may develop late onset ventritular arrhythmia.s. Many patients have both depolarization and repolarization abnormalities, including right bundle branch block (RBBB) and QT prolongation. The goal of thi.s .study was to improve prospective risk-assessment screening for late onset sudden death. Resting ECG markers including QRS duration, QTc, JTc, and interlead QT and JT dispersion were statistically analyzed to identify those patients at risk for ventricular arrhythmias and sudden cardiac death. To determine predictive markers for future development of arrhythmia, we examined 101 resting ECGs in patients (age 12 ± 6 years) with postoperative TOF and RBBB, 14 of whom developed late ventricular tachycardia (VT) or sudden death. These ECGs were also compared with an additional control group of 1000 age-and gender-matched normal ECGs. The mean QRS (± SD) in the VT group was 0.18 ± 0.02 seconds versus 0.14 ± 0.02 seconds in the non-VT group {P < 0.01). QTc and JTc in the VT group was 0.53 ± 0.05 seconds and 0.33 ± 0.03 seconds compared with 0.50 ± 0.03 seconds and 0.32 ± 0.03 seconds in the non-VT group (P = NS). There was no increase in QT dispersion among TOF patients with VT or sudden death compared with control patients or TOF patients without VT, although JT dispersion was more common in the TOF groups. A prolonged QRS duration in postoperative TOF with RBBB is more predictive than QTc, JTc, or dispersion indexes for identifying vulnerahility to ventricular arrhythmias in this population, while retaining high specificity. The combination of hoth QRS prolongation and increased JT dispersion had very good positive and negative predictive values. These results suggest that arrhythmogenesis in children following TOF surgery might involve depolarization in addition to repolarization abnormalities. Prospective identification of high-risk children may he accomplished using these ECG criteria.
It has been suggested that hibernation, a condition of chronic pedusinn-COntraction down-regulati... more It has been suggested that hibernation, a condition of chronic pedusinn-COntraction down-regulation with preserved viability, results from recurrent episodes of reversible ischemia and "chronic" postischemic dysfunction. We examined the interrelation between flow, metalxdism, and function in an acute model of repetitive stunning. Nine dogs underwent four 5 rain balloon occlusions of the LAD or circumflex arteries, each separated by 5 rain of reperfusion. Regional blood flow (BF), metabolism and function were evaluated 2 hourS after reperfusion in 5 dogs, and 2 hours, 24 hours, and 1 week post-reperfual0n in 4 dogs, Regional wall motion 0NM) was evaluated with 2-D echo and 8F with radiotsbalod micraspheres. Measurements of oxidative metabolism (MVO2) and glucose uptake (during hypednsulinemioeuglycemic ¢tsmp) were derived with PET imaging. Regional WM was severely decreased after the 4 Cycles of ischemia, remained severely impaired 2 hours after repertusion, and normalized after 1 week. Dudng reflow, BF in stunned regions was restored to near-normal levels (0.89 • 0,2 vs 0.95:1:0.2 ml/g/min, P < 0.01). Glucose uptake in stunned regions was depressed at 2 hours (73 • 15% of control, P < 0.001) and 24 hours after retiow (80 4-7% of ~ontrel, P = 0.04), but recovered after I week (100 • 26°/= of control, P = NS), Similarly, MVO2 in stunned regions was decreased at 2 hours (84 :E 7% of COntrol, P < 0.001 ) and at 24 hours (8~) :E 6% of COntrol, P = 0.02), and recovered 1 week after reperfusion (97 • 3% of COntrol, P = NS). Thus, repetitive stunned myocardium demonal~tod a persistent reduction of both MVO2 and glucose uptake which recovered as regional wall motion improved. The resu~ may have important implk;ations for detecting stunning in patients with CAD and further suggest a unique metabolic adaptation in "chronic stunning ~, different from that typically seen in hibemalion. ~ Tc-99m.Labeled Ap4A for Early Gamma Scintigrephlc Visualization of Experimental Athemscleroti¢ Lesions Substances related to ATP such as diadenosine tetraphosphate (Ap4A) bind to purina receptors in vascular smooth muscle o~IL Since proliferating smooth muscle cells are an obligatory COmponent of atherosolerotic lesions, T¢-99m-Ap4A was used to assess whether puflne receptors are upregutsted and can detect atherosclerottc lesions in vive.
Coarctation of the aorta was once viewed as a simple discrete narrowing of the aortic isthmus tha... more Coarctation of the aorta was once viewed as a simple discrete narrowing of the aortic isthmus that could be 'cured' by surgical intervention. It is now clear that this condition may: (1) affect the aortic arch in a highly variable manner; (2) be associated with a host of other left sided heart lesions; (3) represent a wider vasculopathy within the pre-coarctation arterial tree, leading to significant prevalence of hypertension by adolescence, and subsequent risk of early morbidity and death. This review outlines the evaluation and treatment of this disease from pre-natal to adult life. (Cardiol J 2011; 18, 5: 487-495)
The hybrid approach to management of hypoplastic left heart syndrome (HLHS) was developed as an a... more The hybrid approach to management of hypoplastic left heart syndrome (HLHS) was developed as an alternative to neonatal Norwood surgery, providing a less invasive initial palliation for HLHS. We describe our experience in extending the concept of the hybrid procedure to palliate neonates with anatomically compromised systemic arterial blood flow in a variety of congenital cardiac anomalies and supporting its application as first-line palliation in centers developing their HLHS programs. Retrospective review of patients undergoing therapy for HLHS at a single institution from June 2008 to December 2014 was performed. Subject demographics, clinical and procedural data, along with follow-up, were collected. Thirteen patients had initial hybrid palliation for HLHS during the time frame indicated at a median age of 8 days (range 1-29 days) and median weight of 3.4 kg (range 2.4-4.6 kg). Diagnoses included typical HLHS (n = 6), right-dominant unbalanced atrioventricular septal defect with arch hypoplasia (n = 4), double outlet right ventricle [subpulmonic VSD (n = 1) and intact ventricular septum (n = 1)] with hypoplastic transverse aortic arch and borderline left ventricular dimensions. Standard approach with bilateral pulmonary artery banding and ductal stenting was carried out in all thirteen patients. Two patients required two ductal stents at the time of index procedure. There were no intraprocedural complications. Median intubation length post-procedure was 4 days (range 1-74 days). Median hospital stay post-procedure was 47 days (range 15-270 days). The overall mortality rate on follow-up through comprehensive stage 2 over the 6-year experience was 38 % (5 out of 13). Of note, the mortality rate was significantly lower in the latter 3 years of the study period when the procedure was adopted as a primary palliation for HLHS (14 % or 1 out of 7) compared to the initial 3-year period when it was reserved for higher risk cohorts (67 % or 4 out of 6). Median time to subsequent surgery was 3 months (range 1-4 months). One patient required further ductal stenting on follow-up and developed subsequently airway compression. On median follow-up of 24 months, two patients required pulmonary artery arterioplasty. The hybrid procedure may be used for palliation for a variety of cardiac lesions to avoid high-risk surgery in the neonatal period. This approach may be also an alternative in centers performing lower number of Norwood surgery, which has been associated with higher mortality.
Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions, Jan 8, 2015
Distortion of transcatheter heart valve (THV) stent shape and morphology has been shown to impact... more Distortion of transcatheter heart valve (THV) stent shape and morphology has been shown to impact on THV function. This study sought to evaluate the relationship between geometrical CT follow-up data and consequent valve function in patients undergoing transcatheter pulmonary valve replacement with the Edwards SAPIEN THV. All patients were enrolled in the COMPASSION trial. Multidetector computed tomography (MDCT) was performed as part of the study protocol at 6 months and yearly thereafter following valve implantation. Prosthesis eccentricity indices, circularity ratios, and expansion ratios (ER) were calculated. Valve function and reintervention rates were correlated with MDCT findings. Twenty consecutive patients undergoing 58 CT scans were included. Maximum Doppler gradients across the right ventricular outflow tract (RVOT) were significantly reduced following valve implantation (P < 0.001). Geometrical indices and maximum RVOT gradients were compared between the reinterventio...
Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography, Jan 24, 2015
Speckle-tracking echocardiographic (STE) measures of right ventricular (RV) function appear to im... more Speckle-tracking echocardiographic (STE) measures of right ventricular (RV) function appear to improve after transcatheter pulmonary valve implantation (TPVI). Measures of exercise function, such as ventilatory efficiency (the minute ventilation [VE]/carbon dioxide production [Vco2] slope), have been shown to be prognostic of mortality in patients who may require TPVI. The aim of this study was to evaluate the correlation between STE measures of RV function and changes in VE/Vco2 after TPVI. Speckle-tracking echocardiography and cardiopulmonary exercise testing were performed at baseline and 6 months after TPVI in 24 patients from four centers. Conventional echocardiographic measures of RV function were also assessed. Echocardiographic and exercise stress test results were interpreted by single blinded observers at separate core laboratories. All patients demonstrated relief of pulmonary regurgitation and stenosis after TPVI. Improvements in RV longitudinal strain (-16.9 ± 3.5% vs -...
Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions, 2011
We describe our experience with self-fabricated covered stents in the setting of coarctation of t... more We describe our experience with self-fabricated covered stents in the setting of coarctation of the aorta (CoA). Balloon-expandable covered stents are increasingly being utilized to treat CoA in older children and adults. These stents however, are not available in the United States limiting the interventionalist's ability to treat this condition safely and effectively. Retrospective analysis and follow-up data review of our complete experience with self-fabricated covered stents for CoA. Stents were fashioned by suturing an appropriate length of tubular polytetraflouroethylene to a bare metal stent and deploying this stent across the coarctation in a standardized fashion. Over a 9-year period we implanted 53 balloon-expandable stents in 49 patients with CoA. Of these 13 were self-fabricated covered stents deployed in 13 patients (7 male). Median age at implantation was 25.4 years (range, 8.7-49.5 years) with median weight of 65.5 kg (range, 28-168 kg). Indications for stent plac...
The evolution of congenital cardiac surgery has seen significant innovative advances in collabora... more The evolution of congenital cardiac surgery has seen significant innovative advances in collaborative efforts between congenital cardiac surgeons and interventionalists to provide the least invasive intervention with the greatest hemodynamic benefit for patients with congenital heart disease. This review looks at how this collaborative approach has evolved and is being applied to treat a number of congenital conditions across the age ranges.
Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions, 2014
Balloon expandable transcatheter pulmonary valve systems are not applicable to the large majority... more Balloon expandable transcatheter pulmonary valve systems are not applicable to the large majority of patients with chronic severe pulmonary regurgitation (PR) following surgical right ventricular outflow tract (RVOT) rehabilitation. This report describes the clinical use and short-term follow-up of a novel transcatheter self-expanding pulmonary valve system (Venus P Valve) for rehabilitation of the RVOT in patients with chronic severe PR. Patients with native RVOT and severe PR were selected on a case-by-case basis as part of early clinical experience with this valve. Patient demographics and pre-procedural, intra-procedural, and follow-up data were reviewed. Five patients (four females) with a mean weight of 54.8 ± 11.4 kg were selected for attempted valve deployment. Patients were either NYHA class II (n = 3) or class III (n = 2) at baseline. PR was grade 4 in all cases with mean right ventricular end-diastolic volumes of 155.0 ± 16.6 ml/m(2) on cardiac MRI. Mean minimum "ann...
Coarctation of the aorta was once viewed as a simple discrete narrowing of the aortic isthmus tha... more Coarctation of the aorta was once viewed as a simple discrete narrowing of the aortic isthmus that could be 'cured' by surgical intervention. It is now clear that this condition may: (1) affect the aortic arch in a highly variable manner; (2) be associated with a host of other left sided heart lesions; (3) represent a wider vasculopathy within the pre-coarctation arterial tree, leading to significant prevalence of hypertension by adolescence, and subsequent risk of early morbidity and death. This review outlines the evaluation and treatment of this disease from pre-natal to adult life.
Catheterization and cardiovascular diagnosis, 1996
Within the field of pediatric cardiology, a number of subspecialty fields are generally recognize... more Within the field of pediatric cardiology, a number of subspecialty fields are generally recognized. Some of these overlap. For example, most electrophysiologists also would consider themselves to be clinical cardiologists. Some fields, however, are relatively mutually exclusive. For example, most clinical pediatric cardiologists would not consider themselves to be electrophysiologists and would admit that there is a different knowledge, skill, and experience base that separates an electrophysiologist from other specialists within the broader field of pediatric cardiology. Likewise, it is our opinion that a separate knowledge, skill, and experience base exists among pediatric Invasive/Interventional cardiologists. The purpose of this report is to define the unique knowledge and skill base required for the training of an invasive pediatric cardiologist. The scope of this report is limited to the training of Invasive/Interventional cardiologists dealing with the treatment of pediatric ...
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Papers by Ziyad Hijazi