Jamil Aboulhosn - Academia.edu (original) (raw)

Papers by Jamil Aboulhosn

Research paper thumbnail of A right ventricular diastolic impairment is common in systemic sclerosis and is associated with other target-organ damage

Seminars in arthritis and rheumatism, Jan 4, 2015

Heart involvement in systemic sclerosis (SSc) is a strong prognostic factor. Our aim was to exami... more Heart involvement in systemic sclerosis (SSc) is a strong prognostic factor. Our aim was to examine left ventricle (LV) and right ventricle (RV) involvement. We examined LV and RV, systolic and diastolic functions, using echocardiography and Tissue-Doppler echocardiography (TDE) indexes, in a cohort of 212 consecutive SSc patients seen during a 9-month period at 2 institutions (Paris, France and Los Angeles, USA). They were compared to 50 healthy controls. When compared to controls, SSc patients had consistently impaired RV indices that include reduced RV contractility (p < 0.001), larger right atrial area (p = 0.027) and overall RV diastolic dysfunction (25% of SSc patients versus 0% of controls; p < 0.001). Patients also exhibited alterations in LV contractility and diastolic function (p < 0.001 each). In multivariate analysis, RV contractility as expressed by the TDE ST parameter was associated with TDE LV contractility SM (p = 0.030), DLCO (p = 0.013) whereas RV diastol...

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Research paper thumbnail of The spatial QRS-T angle outperforms the Italian and Seattle ECG-based criteria for detection of hypertrophic cardiomyopathy in pediatric patients

Journal of Electrocardiology, 2015

The spatial peaks QRS-T angle has been shown to differentiate adult patients with hypertrophic ca... more The spatial peaks QRS-T angle has been shown to differentiate adult patients with hypertrophic cardiomyopathy (HCM) from controls. We hypothesized that the spatial peaks QRS-T angle would, in isolation, be more accurate than the Italian 12-lead ECG Pre-participation Screening criteria or the Seattle criteria for detecting hypertrophic cardiomyopathy (HCM) in pediatric patients. A retrospective study of pediatric patients with HCM compared to age and gender-matched control patients was undertaken. Significance, odds ratios, sensitivity and specificity of HCM detection of the visually derived spatial peaks QRS-T angle were compared to those of traditional 12-lead ECG criteria using: 1) Italy&amp;amp;amp;amp;amp;amp;#39;s National Pre-participation Screening Programme criteria; and 2) described criteria from Seattle. ECG results from 130 pediatric HCM patients (14.2±4.4years) were compared to 470 control patients (normal echocardiograms, mean age 13.4±4.6years). Mean±standard deviation (SD) values for spatial peaks QRS-T angles were 120.4±40.7 and 21.3±13.7 degrees for HCM and controls, respectively (P&amp;amp;amp;amp;amp;amp;lt;0.001). A spatial peaks QRS-T angle cutoff value of &amp;amp;amp;amp;amp;amp;gt;54.9 degrees yielded greater sensitivity and specificity (93.1% and 98.7%, respectively) for detecting HCM over ECG criteria from Italy (68.5% and 48.1%, respectively) or Seattle (64.6% and 78.9%, respectively) with odds ratios at 1039.70 (95% CI 363.03 to 2977.67), 2.01 (95% CI 1.33 to 3.04) and 6.84 (4.49-10.44), respectively. In our cohort, a visually derived spatial peaks QRS-T angle has increased sensitivity and specificity for detection of HCM in pediatric patients compared to currently utilized Italian or Seattle ECG criteria.

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Research paper thumbnail of The care of adults with congenital heart disease across the globe: Current assessment and future perspective

International Journal of Cardiology, 2015

The number of adults with congenital heart disease (CHD) has increased markedly over the past few... more The number of adults with congenital heart disease (CHD) has increased markedly over the past few decades as a result of astounding successes in pediatric cardiac care. Nevertheless, it is now well understood that CHD is not cured but palliated, such that life-long expert care is required to optimize outcomes. All countries in the world that experience improved survival in CHD must face new challenges inherent to the emergence of a growing and aging CHD population with changing needs and medical and psychosocial issues. Founded in 1992, the International Society for Adult Congenital Heart Disease (ISACHD) is the leading global organization of professionals dedicated to pursuing excellence in the care of adults with CHD worldwide. Recognizing the unique and varied issues involved in caring for adults with CHD, ISACHD established a task force to assess the current status of care for adults with CHD across the globe, highlight major challenges and priorities, and provide future direction. The writing committee consisted of experts from North America, South America, Europe, South Asia, East Asia, and Oceania. The committee was divided into subgroups to review key aspects of adult CHD (ACHD) care. Regional representatives were tasked with investigating and reporting on relevant local issues as accurately as possible, within the constraints of available data. The resulting ISACHD position statement addresses changing patterns of worldwide epidemiology, models of care and organization of care, education and training, and the global research landscape in ACHD.

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Research paper thumbnail of Systemic to pulmonary venous collaterals in adults with single ventricle physiology after cavopulmonary palliation

International journal of cardiology, Jan 10, 2015

To assess the frequency, anatomic characteristics, and associations of systemic to pulmonary veno... more To assess the frequency, anatomic characteristics, and associations of systemic to pulmonary venous collaterals in adult patients undergoing cardiac catheterization after a Fontan operation. Additionally, the embryologic basis for the presence of venous collaterals is reviewed. Cardiac catheterization data was reviewed for 66 adults with single ventricle physiology and a Fontan palliation. There were a total of 66 patients that underwent catheterization between 2004 and 2014 at the Ahmanson/UCLA Adult Congenital Heart Disease Center. There were 24 males and 42 females. Systemic venous to pulmonary venous collaterals were present in 38 patients (58%), most commonly originating from the right brachiocephalic vein (35%), azygous vein (20%) and superior vena cava (13%). Trans-catheter interventional closure was performed in 27/38 (71%) of patients with venous collaterals. At baseline these patients had lower oxygen saturation when compared to those not requiring intervention, 85.6%±6.1%...

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Research paper thumbnail of Transcatheter melody valve placement in large diameter bioprostheses and conduits: What is the optimal "Landing zone"?

Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions, Jan 30, 2015

This study sought to elucidate the optimal bioprosthetic valve (BPV) size prior to Melody valve i... more This study sought to elucidate the optimal bioprosthetic valve (BPV) size prior to Melody valve implantation. BPVs provide an ideal "landing zone" for future Melody valve insertion. To guide surgical choice of BPV size, it is important to understand which BPV size can serve consistently as substrates for Melody valve placements. A database of all patients who underwent Melody implantation at UCLA or Kaiser Permanente Los Angeles from 2010 to 2014 was analyzed retrospectively. Patients with an existing BPV were stratified into those with a valve diameter of ≥27 mm or <27 mm. One hundred and sixty patients underwent catheterization with the intention to implant a Melody valve. Melody valve implantation was performed in the pulmonary position in 52 patients with prior BPVs. The immediate procedural success rate was 100%. Immediately post-Melody, the right ventricular to pulmonary artery gradient was significantly higher in the <27 mm group compared to the ≥27 mm group (...

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Research paper thumbnail of Partial anomalous pulmonary venous return: case report and review of the literature

Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions, 2003

Isolated partial anomalous pulmonary venous return (APVR) is an uncommon finding. A patient with ... more Isolated partial anomalous pulmonary venous return (APVR) is an uncommon finding. A patient with isolated APVR had pulmonary hypertension without demonstrable left-to-right shunting prior to anticoagulant treatment of pulmonary emboli. After anticoagulant therapy, with a fall in pulmonary pressures and resistance to near-normal levels, left-to-right shunting was then detected by oximetry and angiography. The anomaly was visualized on electron beam angiography and confirmed by conventional angiography after anticoagulant therapy. Contrary to the expected obligatory drainage of highly saturated blood associated with APVR, lack of detectable shunting was thought to be due to the obstruction of regional blood due to thromboembolism.

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Research paper thumbnail of Percutaneous Angioplasty of Stenotic Outflow Graft Anastomosis of HeartMate II

JACC: Cardiovascular Interventions, 2014

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Research paper thumbnail of The adult with a Fontan operation

Current Cardiology Reports, 2007

Since 1971, the Fontan operation has been performed for the repair of single-ventricle physiology... more Since 1971, the Fontan operation has been performed for the repair of single-ventricle physiology. This ingenious operation commits a single ventricle to the systemic circulation and takes advantage of cardiovascular and respiratory physiology to propel deoxygenated blood to the lungs, thus minimizing right-to-left shunting and cyanosis. Initially performed as a right atrial to pulmonary artery anastomosis, the Fontan operation has gone through evolutionary steps that have resulted in progressive improvements in mortality, morbidity, and outcomes. Inclusion of the right atrium in the slow-flowing Fontan circuit results in progressive dilation and incessant arrhythmias. This spurred forth efforts to create modifications that partially or completely exclude the atrium from the Fontan circuit. The transcatheter completion of the Fontan operation has been performed in a small number of patients and we expect minimally invasive, transcatheter, and hybrid interventions to play an important role in the future management of these patients.

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Research paper thumbnail of Echocardiographic Evaluation of Congenital Left Ventricular Outflow Obstruction

Echocardiography, 2014

Left ventricular outflow obstructions (LVOTO) encompass a series of stenotic lesions beginning wi... more Left ventricular outflow obstructions (LVOTO) encompass a series of stenotic lesions beginning within the anatomic left ventricular outflow tract and ending in the descending thoracic aorta, occurring alone or in series. The degree of stenosis and hemodynamic consequences may vary from mild and well tolerated to severe and functionally limiting. The majority of LVOTO (with the exception of calcific and rheumatic aortic stenosis) are congenital in etiology. Echocardiography has become an integral tool in the assessment of patients with congenital heart disease. This article is a contemporary review of the clinical features of LVOTO and the role of echocardiography in this patient population.

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Research paper thumbnail of Congenital Heart Disease and Computed Tomography

Cardiac CT Imaging, 2006

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Research paper thumbnail of Applications of cardiac CT in the tetralogy of Fallot patient

JACC. Cardiovascular imaging, 2014

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Research paper thumbnail of Congenital Heart Disease in Adults Lower Extremity Systemic Venous Health in Fontan Patients Study

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Research paper thumbnail of Novel techniques of mechanical circulatory support for the right heart and Fontan circulation

International journal of cardiology, Jan 20, 2014

Currently available ventricular assist devices are designed primarily for use in patients with le... more Currently available ventricular assist devices are designed primarily for use in patients with left sided heart failure. This study evaluated the efficacy of the Jarvik 2000 ventricular assist device (VAD) as a pulmonary pump to power a Fontan circuit in a large animal model. Without the use of cardiopulmonary bypass, Fontan circulations were surgically created in 4 pigs (50 kg) using synthetic grafts from the inferior and superior vena cavas to the main pulmonary artery. Subsequently, the VAD was implanted within the common Fontan graft to provide a pulmonary pump. Direct chamber pressures and epicardial Doppler images were taken during the various phases of the experiment. Heart rate, femoral artery blood pressure, oxygen saturation, and aortic flow rate were continuously recorded. The outflow cannula of the VAD was then partially banded by 50% and then 75% to mimic increased afterload. Fontan and VAD implantation was successfully performed in all 4 animals. Arterial pressure and ...

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Research paper thumbnail of Long-term outcome of off-pump palliation for hypoplastic left ventricle with glutaraldehyde fixation of the ductus arteriosus

The Journal of thoracic and cardiovascular surgery, 2014

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Research paper thumbnail of Evaluation of Peak Pressure Gradients in Patients after Melody Valve Implantation: A Comparison of Cardiac Catheterization and Doppler Echocardiography

Echocardiography, 2014

The aim of this study was to determine and quantify the relationship between Doppler echocardiogr... more The aim of this study was to determine and quantify the relationship between Doppler echocardiography and cardiac catheterization measurements of the peak right ventricle-pulmonary artery (RV-PA) pressure gradient in patients within 24 hours of Melody valve implantation for treatment of a dysfunctional RV outflow tract (RVOT) conduit or bioprosthetic valve (BPV). Patients with a dysfunctional RVOT conduit or BPV are now routinely treated percutaneously with implantation of a Melody valve. However, often the postimplantation catheter measurements of the RV-PA peak gradient do not match the postimplant echo-derived gradients obtained after completion of the procedure. Importantly, these echo gradients are commonly used to monitor patients after implantation over time. Medical records of 42 patients with Melody valve implantation were reviewed (men: 25/42; mean age: 22.4 ± 11.2 years; RVOT conduit: 23/42; BPV: 18/42; pulmonic stenosis: 6/42, pulmonic regurgitation: 3/42, both: 33/42). The postimplantation RV-PA peak gradient measured by Doppler echocardiography (within 24 hours of valve implantation) was significantly higher than the measurements acquired by catheterization immediately following implantation (24.2 ± 16.3 mmHg vs. 11.6 ± 8.5 mmHg, P &amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001). The relationship showed a moderate-strong correlation (r = 0.65, P &amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001) with regression analysis suggesting a linear association in both directions (echo gradient = 1.24 × cath gradient + 9.8, P&amp;amp;amp;amp;amp;amp;amp;amp;lt;0.0001 vs. cath gradient = 0.34 × echo gradient + 3.4, P&amp;amp;amp;amp;amp;amp;amp;amp;lt;0.0001). The Doppler echocardiography-derived RV-PA peak gradient within 24 hours of valve implantation is predictably higher than the catheter measured peak gradient immediately following valve implantation. A regression equation was derived to define this important relationship.

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Research paper thumbnail of Catheter ablation of tachycardia arising from the pulmonary venous atrium after surgical repair of congenital heart disease

Heart rhythm : the official journal of the Heart Rhythm Society, 2015

Tachycardia arising from the pulmonary venous atrium (PVA) has not been adequately characterized ... more Tachycardia arising from the pulmonary venous atrium (PVA) has not been adequately characterized in the setting of surgically repaired congenital heart disease (CHD). The purpose of this study was to determine the mechanisms, approach, and outcomes of catheter ablation of PVA tachycardia after CHD repair. The adult CHD procedural database was searched for consecutive ablation procedures over a 4-year period. Procedural characteristics of the population with tachycardia arising from the PVA were compared to those without PVA tachycardia. Groups were classified as (1) biventricular CHD, (2) single ventricle, or (3) d-transposition of the great arteries (DTGA)-baffle. Complete 3-dimensional mapping was possible for 113 of 124 sustained tachycardias during 81 procedures. Of these, 31 (19%) arose from the PVA, including 11 (15%) tachycardias in biventricular CHD, 8 (31%) in single ventricle, and 12 (80%) in DTGA-baffle procedures. Intra-atrial reentrant tachycardia was less frequently ob...

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Research paper thumbnail of Successful tricuspid valve replacement in a patient with severe pulmonary arterial hypertension and preserved right ventricular systolic function

Case reports in medicine, 2009

A 56-year-old patient with severe pulmonary hypertension developed severe tricuspid regurgitation... more A 56-year-old patient with severe pulmonary hypertension developed severe tricuspid regurgitation, right-sided heart failure, and congestive hepatopathy. She was transferred for possible lung transplant and/or tricuspid valve surgery. Clinical and echocardiographic assessment provided confidence that acute tricuspid valve failure was responsible for the decompensation and that tricuspid valve replacement despite pulmonary hypertension could be performed.

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Research paper thumbnail of Pheochromocytoma and Paraganglioma in Cyanotic Congenital Heart Disease

The Journal of Clinical Endocrinology & Metabolism, 2015

Aberrant cellular oxygen sensing (pseudo-hypoxia) is a leading theory for development of pheochro... more Aberrant cellular oxygen sensing (pseudo-hypoxia) is a leading theory for development of pheochromocytoma (PHEO) and paraganglioma (PGL). To test the hypothesis that chronic hypoxia in patients with cyanotic congenital heart disease (CCHD, CHD) increases the risk for PHEO-PGL. We investigated the association between CCHD and PHEO-PGL with 2 complementary studies: Study 1) An international consortium was established to identify patients with PHEO-PGL diagnosis confirmed by pathology, or biochemistry and imaging. Study 2) The 2000-2009 Nationwide Inpatient Survey, a nationally representative discharge database, was used to determine population-based cross-sectional PHEO-PGL frequency in hospitalized CCHD patients compared with non-cyanotic CHD and those without CHD using multivariable logistic regression adjusted for age, sex, and genetic PHEO-PGL syndromes. Study 1) We identified 20 PHEO-PGL cases, of which 18 had CCHD. Most presented with cardiovascular or psychiatric symptoms. Median cyanosis duration for the CCHD PHEO-PGL cases was 20y (range 1-57y). Cases were young at diagnosis (median 31.5y, range 15-57) and 7/18 had multiple tumors (2 bilateral PHEO; 6 multi-focal or recurrent PGL) while 11 had single tumors (7 PHEO; 4 PGL). PGL were abdominal (13/17) or head/neck (4/17). Cases displayed a noradrenergic biochemical phenotype similar to reported hypoxia-related PHEO-PGL genetic syndromes, but without clinical signs of such syndromes. Study 2) Hospitalized CCHD patients had increased likelihood of PHEO-PGL (adjusted OR=6.0, 95%CI [2.6-13.7], p&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;0.0001) compared with those without CHD; patients with non-cyanotic CHD had no increased risk (OR=0.9, p=0.48). There is a strong link between CCHD and PHEO-PGL. Whether these rare diseases co-associate due to hypoxic stress, common genetic or developmental factors, or some combination, requires further investigation.

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Research paper thumbnail of Diagnosis and Hemodynamic Assessment of Pulmonary Arterial Hypertension

Seminars in Respiratory and Critical Care Medicine, 2009

Pulmonary hypertension (PH) can occur as either a primary or a secondary process, and in general,... more Pulmonary hypertension (PH) can occur as either a primary or a secondary process, and in general, its presence increases overall morbidity and mortality. Importantly, the majority of prior studies have been in the setting of idiopathic pulmonary arterial hypertension (IPAH); thus the following discussion focuses on IPAH. Because the majority of available diagnostic strategies lack sensitivity and specificity, the physician must maintain a high index of suspicion in considering PAH. This article provides an overview of the available diagnostic studies for PAH with a particular focus on hemodynamic assessment. Novel approaches to the often delayed diagnosis of PAH are being studied and are also discussed here.

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Research paper thumbnail of Patients With Single-Ventricle Physiology: Prognostic Implications of Stress Testing

Pediatric Cardiology, 2011

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Research paper thumbnail of A right ventricular diastolic impairment is common in systemic sclerosis and is associated with other target-organ damage

Seminars in arthritis and rheumatism, Jan 4, 2015

Heart involvement in systemic sclerosis (SSc) is a strong prognostic factor. Our aim was to exami... more Heart involvement in systemic sclerosis (SSc) is a strong prognostic factor. Our aim was to examine left ventricle (LV) and right ventricle (RV) involvement. We examined LV and RV, systolic and diastolic functions, using echocardiography and Tissue-Doppler echocardiography (TDE) indexes, in a cohort of 212 consecutive SSc patients seen during a 9-month period at 2 institutions (Paris, France and Los Angeles, USA). They were compared to 50 healthy controls. When compared to controls, SSc patients had consistently impaired RV indices that include reduced RV contractility (p < 0.001), larger right atrial area (p = 0.027) and overall RV diastolic dysfunction (25% of SSc patients versus 0% of controls; p < 0.001). Patients also exhibited alterations in LV contractility and diastolic function (p < 0.001 each). In multivariate analysis, RV contractility as expressed by the TDE ST parameter was associated with TDE LV contractility SM (p = 0.030), DLCO (p = 0.013) whereas RV diastol...

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Research paper thumbnail of The spatial QRS-T angle outperforms the Italian and Seattle ECG-based criteria for detection of hypertrophic cardiomyopathy in pediatric patients

Journal of Electrocardiology, 2015

The spatial peaks QRS-T angle has been shown to differentiate adult patients with hypertrophic ca... more The spatial peaks QRS-T angle has been shown to differentiate adult patients with hypertrophic cardiomyopathy (HCM) from controls. We hypothesized that the spatial peaks QRS-T angle would, in isolation, be more accurate than the Italian 12-lead ECG Pre-participation Screening criteria or the Seattle criteria for detecting hypertrophic cardiomyopathy (HCM) in pediatric patients. A retrospective study of pediatric patients with HCM compared to age and gender-matched control patients was undertaken. Significance, odds ratios, sensitivity and specificity of HCM detection of the visually derived spatial peaks QRS-T angle were compared to those of traditional 12-lead ECG criteria using: 1) Italy&amp;amp;amp;amp;amp;amp;#39;s National Pre-participation Screening Programme criteria; and 2) described criteria from Seattle. ECG results from 130 pediatric HCM patients (14.2±4.4years) were compared to 470 control patients (normal echocardiograms, mean age 13.4±4.6years). Mean±standard deviation (SD) values for spatial peaks QRS-T angles were 120.4±40.7 and 21.3±13.7 degrees for HCM and controls, respectively (P&amp;amp;amp;amp;amp;amp;lt;0.001). A spatial peaks QRS-T angle cutoff value of &amp;amp;amp;amp;amp;amp;gt;54.9 degrees yielded greater sensitivity and specificity (93.1% and 98.7%, respectively) for detecting HCM over ECG criteria from Italy (68.5% and 48.1%, respectively) or Seattle (64.6% and 78.9%, respectively) with odds ratios at 1039.70 (95% CI 363.03 to 2977.67), 2.01 (95% CI 1.33 to 3.04) and 6.84 (4.49-10.44), respectively. In our cohort, a visually derived spatial peaks QRS-T angle has increased sensitivity and specificity for detection of HCM in pediatric patients compared to currently utilized Italian or Seattle ECG criteria.

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Research paper thumbnail of The care of adults with congenital heart disease across the globe: Current assessment and future perspective

International Journal of Cardiology, 2015

The number of adults with congenital heart disease (CHD) has increased markedly over the past few... more The number of adults with congenital heart disease (CHD) has increased markedly over the past few decades as a result of astounding successes in pediatric cardiac care. Nevertheless, it is now well understood that CHD is not cured but palliated, such that life-long expert care is required to optimize outcomes. All countries in the world that experience improved survival in CHD must face new challenges inherent to the emergence of a growing and aging CHD population with changing needs and medical and psychosocial issues. Founded in 1992, the International Society for Adult Congenital Heart Disease (ISACHD) is the leading global organization of professionals dedicated to pursuing excellence in the care of adults with CHD worldwide. Recognizing the unique and varied issues involved in caring for adults with CHD, ISACHD established a task force to assess the current status of care for adults with CHD across the globe, highlight major challenges and priorities, and provide future direction. The writing committee consisted of experts from North America, South America, Europe, South Asia, East Asia, and Oceania. The committee was divided into subgroups to review key aspects of adult CHD (ACHD) care. Regional representatives were tasked with investigating and reporting on relevant local issues as accurately as possible, within the constraints of available data. The resulting ISACHD position statement addresses changing patterns of worldwide epidemiology, models of care and organization of care, education and training, and the global research landscape in ACHD.

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Research paper thumbnail of Systemic to pulmonary venous collaterals in adults with single ventricle physiology after cavopulmonary palliation

International journal of cardiology, Jan 10, 2015

To assess the frequency, anatomic characteristics, and associations of systemic to pulmonary veno... more To assess the frequency, anatomic characteristics, and associations of systemic to pulmonary venous collaterals in adult patients undergoing cardiac catheterization after a Fontan operation. Additionally, the embryologic basis for the presence of venous collaterals is reviewed. Cardiac catheterization data was reviewed for 66 adults with single ventricle physiology and a Fontan palliation. There were a total of 66 patients that underwent catheterization between 2004 and 2014 at the Ahmanson/UCLA Adult Congenital Heart Disease Center. There were 24 males and 42 females. Systemic venous to pulmonary venous collaterals were present in 38 patients (58%), most commonly originating from the right brachiocephalic vein (35%), azygous vein (20%) and superior vena cava (13%). Trans-catheter interventional closure was performed in 27/38 (71%) of patients with venous collaterals. At baseline these patients had lower oxygen saturation when compared to those not requiring intervention, 85.6%±6.1%...

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Research paper thumbnail of Transcatheter melody valve placement in large diameter bioprostheses and conduits: What is the optimal "Landing zone"?

Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions, Jan 30, 2015

This study sought to elucidate the optimal bioprosthetic valve (BPV) size prior to Melody valve i... more This study sought to elucidate the optimal bioprosthetic valve (BPV) size prior to Melody valve implantation. BPVs provide an ideal "landing zone" for future Melody valve insertion. To guide surgical choice of BPV size, it is important to understand which BPV size can serve consistently as substrates for Melody valve placements. A database of all patients who underwent Melody implantation at UCLA or Kaiser Permanente Los Angeles from 2010 to 2014 was analyzed retrospectively. Patients with an existing BPV were stratified into those with a valve diameter of ≥27 mm or <27 mm. One hundred and sixty patients underwent catheterization with the intention to implant a Melody valve. Melody valve implantation was performed in the pulmonary position in 52 patients with prior BPVs. The immediate procedural success rate was 100%. Immediately post-Melody, the right ventricular to pulmonary artery gradient was significantly higher in the <27 mm group compared to the ≥27 mm group (...

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Research paper thumbnail of Partial anomalous pulmonary venous return: case report and review of the literature

Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions, 2003

Isolated partial anomalous pulmonary venous return (APVR) is an uncommon finding. A patient with ... more Isolated partial anomalous pulmonary venous return (APVR) is an uncommon finding. A patient with isolated APVR had pulmonary hypertension without demonstrable left-to-right shunting prior to anticoagulant treatment of pulmonary emboli. After anticoagulant therapy, with a fall in pulmonary pressures and resistance to near-normal levels, left-to-right shunting was then detected by oximetry and angiography. The anomaly was visualized on electron beam angiography and confirmed by conventional angiography after anticoagulant therapy. Contrary to the expected obligatory drainage of highly saturated blood associated with APVR, lack of detectable shunting was thought to be due to the obstruction of regional blood due to thromboembolism.

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Research paper thumbnail of Percutaneous Angioplasty of Stenotic Outflow Graft Anastomosis of HeartMate II

JACC: Cardiovascular Interventions, 2014

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Research paper thumbnail of The adult with a Fontan operation

Current Cardiology Reports, 2007

Since 1971, the Fontan operation has been performed for the repair of single-ventricle physiology... more Since 1971, the Fontan operation has been performed for the repair of single-ventricle physiology. This ingenious operation commits a single ventricle to the systemic circulation and takes advantage of cardiovascular and respiratory physiology to propel deoxygenated blood to the lungs, thus minimizing right-to-left shunting and cyanosis. Initially performed as a right atrial to pulmonary artery anastomosis, the Fontan operation has gone through evolutionary steps that have resulted in progressive improvements in mortality, morbidity, and outcomes. Inclusion of the right atrium in the slow-flowing Fontan circuit results in progressive dilation and incessant arrhythmias. This spurred forth efforts to create modifications that partially or completely exclude the atrium from the Fontan circuit. The transcatheter completion of the Fontan operation has been performed in a small number of patients and we expect minimally invasive, transcatheter, and hybrid interventions to play an important role in the future management of these patients.

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Research paper thumbnail of Echocardiographic Evaluation of Congenital Left Ventricular Outflow Obstruction

Echocardiography, 2014

Left ventricular outflow obstructions (LVOTO) encompass a series of stenotic lesions beginning wi... more Left ventricular outflow obstructions (LVOTO) encompass a series of stenotic lesions beginning within the anatomic left ventricular outflow tract and ending in the descending thoracic aorta, occurring alone or in series. The degree of stenosis and hemodynamic consequences may vary from mild and well tolerated to severe and functionally limiting. The majority of LVOTO (with the exception of calcific and rheumatic aortic stenosis) are congenital in etiology. Echocardiography has become an integral tool in the assessment of patients with congenital heart disease. This article is a contemporary review of the clinical features of LVOTO and the role of echocardiography in this patient population.

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Research paper thumbnail of Congenital Heart Disease and Computed Tomography

Cardiac CT Imaging, 2006

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Research paper thumbnail of Applications of cardiac CT in the tetralogy of Fallot patient

JACC. Cardiovascular imaging, 2014

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Research paper thumbnail of Congenital Heart Disease in Adults Lower Extremity Systemic Venous Health in Fontan Patients Study

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Research paper thumbnail of Novel techniques of mechanical circulatory support for the right heart and Fontan circulation

International journal of cardiology, Jan 20, 2014

Currently available ventricular assist devices are designed primarily for use in patients with le... more Currently available ventricular assist devices are designed primarily for use in patients with left sided heart failure. This study evaluated the efficacy of the Jarvik 2000 ventricular assist device (VAD) as a pulmonary pump to power a Fontan circuit in a large animal model. Without the use of cardiopulmonary bypass, Fontan circulations were surgically created in 4 pigs (50 kg) using synthetic grafts from the inferior and superior vena cavas to the main pulmonary artery. Subsequently, the VAD was implanted within the common Fontan graft to provide a pulmonary pump. Direct chamber pressures and epicardial Doppler images were taken during the various phases of the experiment. Heart rate, femoral artery blood pressure, oxygen saturation, and aortic flow rate were continuously recorded. The outflow cannula of the VAD was then partially banded by 50% and then 75% to mimic increased afterload. Fontan and VAD implantation was successfully performed in all 4 animals. Arterial pressure and ...

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Research paper thumbnail of Long-term outcome of off-pump palliation for hypoplastic left ventricle with glutaraldehyde fixation of the ductus arteriosus

The Journal of thoracic and cardiovascular surgery, 2014

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Research paper thumbnail of Evaluation of Peak Pressure Gradients in Patients after Melody Valve Implantation: A Comparison of Cardiac Catheterization and Doppler Echocardiography

Echocardiography, 2014

The aim of this study was to determine and quantify the relationship between Doppler echocardiogr... more The aim of this study was to determine and quantify the relationship between Doppler echocardiography and cardiac catheterization measurements of the peak right ventricle-pulmonary artery (RV-PA) pressure gradient in patients within 24 hours of Melody valve implantation for treatment of a dysfunctional RV outflow tract (RVOT) conduit or bioprosthetic valve (BPV). Patients with a dysfunctional RVOT conduit or BPV are now routinely treated percutaneously with implantation of a Melody valve. However, often the postimplantation catheter measurements of the RV-PA peak gradient do not match the postimplant echo-derived gradients obtained after completion of the procedure. Importantly, these echo gradients are commonly used to monitor patients after implantation over time. Medical records of 42 patients with Melody valve implantation were reviewed (men: 25/42; mean age: 22.4 ± 11.2 years; RVOT conduit: 23/42; BPV: 18/42; pulmonic stenosis: 6/42, pulmonic regurgitation: 3/42, both: 33/42). The postimplantation RV-PA peak gradient measured by Doppler echocardiography (within 24 hours of valve implantation) was significantly higher than the measurements acquired by catheterization immediately following implantation (24.2 ± 16.3 mmHg vs. 11.6 ± 8.5 mmHg, P &amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001). The relationship showed a moderate-strong correlation (r = 0.65, P &amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001) with regression analysis suggesting a linear association in both directions (echo gradient = 1.24 × cath gradient + 9.8, P&amp;amp;amp;amp;amp;amp;amp;amp;lt;0.0001 vs. cath gradient = 0.34 × echo gradient + 3.4, P&amp;amp;amp;amp;amp;amp;amp;amp;lt;0.0001). The Doppler echocardiography-derived RV-PA peak gradient within 24 hours of valve implantation is predictably higher than the catheter measured peak gradient immediately following valve implantation. A regression equation was derived to define this important relationship.

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Research paper thumbnail of Catheter ablation of tachycardia arising from the pulmonary venous atrium after surgical repair of congenital heart disease

Heart rhythm : the official journal of the Heart Rhythm Society, 2015

Tachycardia arising from the pulmonary venous atrium (PVA) has not been adequately characterized ... more Tachycardia arising from the pulmonary venous atrium (PVA) has not been adequately characterized in the setting of surgically repaired congenital heart disease (CHD). The purpose of this study was to determine the mechanisms, approach, and outcomes of catheter ablation of PVA tachycardia after CHD repair. The adult CHD procedural database was searched for consecutive ablation procedures over a 4-year period. Procedural characteristics of the population with tachycardia arising from the PVA were compared to those without PVA tachycardia. Groups were classified as (1) biventricular CHD, (2) single ventricle, or (3) d-transposition of the great arteries (DTGA)-baffle. Complete 3-dimensional mapping was possible for 113 of 124 sustained tachycardias during 81 procedures. Of these, 31 (19%) arose from the PVA, including 11 (15%) tachycardias in biventricular CHD, 8 (31%) in single ventricle, and 12 (80%) in DTGA-baffle procedures. Intra-atrial reentrant tachycardia was less frequently ob...

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Research paper thumbnail of Successful tricuspid valve replacement in a patient with severe pulmonary arterial hypertension and preserved right ventricular systolic function

Case reports in medicine, 2009

A 56-year-old patient with severe pulmonary hypertension developed severe tricuspid regurgitation... more A 56-year-old patient with severe pulmonary hypertension developed severe tricuspid regurgitation, right-sided heart failure, and congestive hepatopathy. She was transferred for possible lung transplant and/or tricuspid valve surgery. Clinical and echocardiographic assessment provided confidence that acute tricuspid valve failure was responsible for the decompensation and that tricuspid valve replacement despite pulmonary hypertension could be performed.

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Research paper thumbnail of Pheochromocytoma and Paraganglioma in Cyanotic Congenital Heart Disease

The Journal of Clinical Endocrinology & Metabolism, 2015

Aberrant cellular oxygen sensing (pseudo-hypoxia) is a leading theory for development of pheochro... more Aberrant cellular oxygen sensing (pseudo-hypoxia) is a leading theory for development of pheochromocytoma (PHEO) and paraganglioma (PGL). To test the hypothesis that chronic hypoxia in patients with cyanotic congenital heart disease (CCHD, CHD) increases the risk for PHEO-PGL. We investigated the association between CCHD and PHEO-PGL with 2 complementary studies: Study 1) An international consortium was established to identify patients with PHEO-PGL diagnosis confirmed by pathology, or biochemistry and imaging. Study 2) The 2000-2009 Nationwide Inpatient Survey, a nationally representative discharge database, was used to determine population-based cross-sectional PHEO-PGL frequency in hospitalized CCHD patients compared with non-cyanotic CHD and those without CHD using multivariable logistic regression adjusted for age, sex, and genetic PHEO-PGL syndromes. Study 1) We identified 20 PHEO-PGL cases, of which 18 had CCHD. Most presented with cardiovascular or psychiatric symptoms. Median cyanosis duration for the CCHD PHEO-PGL cases was 20y (range 1-57y). Cases were young at diagnosis (median 31.5y, range 15-57) and 7/18 had multiple tumors (2 bilateral PHEO; 6 multi-focal or recurrent PGL) while 11 had single tumors (7 PHEO; 4 PGL). PGL were abdominal (13/17) or head/neck (4/17). Cases displayed a noradrenergic biochemical phenotype similar to reported hypoxia-related PHEO-PGL genetic syndromes, but without clinical signs of such syndromes. Study 2) Hospitalized CCHD patients had increased likelihood of PHEO-PGL (adjusted OR=6.0, 95%CI [2.6-13.7], p&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;0.0001) compared with those without CHD; patients with non-cyanotic CHD had no increased risk (OR=0.9, p=0.48). There is a strong link between CCHD and PHEO-PGL. Whether these rare diseases co-associate due to hypoxic stress, common genetic or developmental factors, or some combination, requires further investigation.

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Research paper thumbnail of Diagnosis and Hemodynamic Assessment of Pulmonary Arterial Hypertension

Seminars in Respiratory and Critical Care Medicine, 2009

Pulmonary hypertension (PH) can occur as either a primary or a secondary process, and in general,... more Pulmonary hypertension (PH) can occur as either a primary or a secondary process, and in general, its presence increases overall morbidity and mortality. Importantly, the majority of prior studies have been in the setting of idiopathic pulmonary arterial hypertension (IPAH); thus the following discussion focuses on IPAH. Because the majority of available diagnostic strategies lack sensitivity and specificity, the physician must maintain a high index of suspicion in considering PAH. This article provides an overview of the available diagnostic studies for PAH with a particular focus on hemodynamic assessment. Novel approaches to the often delayed diagnosis of PAH are being studied and are also discussed here.

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Research paper thumbnail of Patients With Single-Ventricle Physiology: Prognostic Implications of Stress Testing

Pediatric Cardiology, 2011

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