Marcus Haw - Academia.edu (original) (raw)

Papers by Marcus Haw

Research paper thumbnail of Abstract 222: Combination of Blood Cardioplegic and Strategic Reperfusion Period Leucodepletion During Cardiopulmonary Bypass Preserves the Cardiac and Pulmonary Function: A Prospective Randomized Controlled Study

Circulation Research, 2012

Objectives: Leucocytes activation during cardiopulmonary bypass (CPB) contributes to postoperativ... more Objectives: Leucocytes activation during cardiopulmonary bypass (CPB) contributes to postoperative organ dysfunction. We compared the outcomes of various leucodepletion strategies in patients undergoing coronary artery bypass grafting (CABG). Methods: One hundred-twenty low-risk patients undergoing first time CABG were prospectively randomized to six groups: 1 non-leucodepletion arterial filtration; 2 continuous arterial leucodepletion; 3 reperfusion period leucodepletion; 4 leucodepletion of blood cardioplegia; 5 combination of continuous arterial and blood cardioplegic leucodepletion; 6 combination of blood cardioplegic and reperfusion period leucodepletion. Blood samples were taken 5 min before CPB, 5 and 30 min on CPB, 5, 60 min after aortic X-clamp removal and 6 h post-CPB. Activated leucocytes were identified with Nitroblue Tetrazolium staining. Exhaled nitro oxide (NO) was measured pre- and post-CPB using real-time chemiluminescense analyzer. Respiratory index (alveolar-arter...

Research paper thumbnail of Viral Induced Genetics Revealed by Multi-Dimensional Precision Medicine Transcriptional Workflow

Precision medicine requires the translation of basic biological understanding to medical insights... more Precision medicine requires the translation of basic biological understanding to medical insights, mainly applied to characterization of each unique patient. In many clinical settings, this requires tools that can be broadly used to identify pathology and risks. Patients often present to the intensive care unit with broad phenotypes, including multiple organ dysfunction syndrome (MODS) resulting from infection, trauma, or other disease processes. Etiology and outcomes are unique to individuals, making it difficult to cohort patients with MODS, but presenting a prime target for testing/developing tools for precision medicine. Using multi-time point whole blood (cellular/acellular) total transcriptomics in 27 patients, we highlight the promise of simultaneously mapping viral/bacterial load, cell composition, tissue damage biomarkers, balance between syndromic biology vs. environmental response, and unique biological insights in each patient using a single platform measurement. Integra...

Research paper thumbnail of Virus-induced genetics revealed by multidimensional precision medicine transcriptional workflow applicable to COVID-19

Physiological Genomics

Precision medicine requires the translation of basic biological understanding to medical insights... more Precision medicine requires the translation of basic biological understanding to medical insights, mainly applied to characterization of each unique patient. In many clinical settings, this requires tools that can be broadly used to identify pathology and risks. Patients often present to the intensive care unit with broad phenotypes, including multiple organ dysfunction syndrome (MODS) resulting from infection, trauma, or other disease processes. Etiology and outcomes are unique to individuals, making it difficult to cohort patients with MODS, but presenting a prime target for testing/developing tools for precision medicine. Using multitime point whole blood (cellular/acellular) total transcriptomics in 27 patients, we highlight the promise of simultaneously mapping viral/bacterial load, cell composition, tissue damage biomarkers, balance between syndromic biology versus environmental response, and unique biological insights in each patient using a single platform measurement. Integ...

Research paper thumbnail of Three‐dimensional printing for surgical planning in complex congenital heart disease

Journal of Cardiac Surgery

Research paper thumbnail of Circulating Microrna Signatures as Potential Markers of Tissue Specific Stress in Children Undergoing Cardiopulmonary Bypass

Journal of the American College of Cardiology

Research paper thumbnail of Stenting and Reimplanting Disconnected Pulmonary Artery in Tetralogy of Fallot

The Annals of thoracic surgery, 2017

Tetralogy of Fallot with absent pulmonary valve syndrome (TOF/APV) is a rare congenital malformat... more Tetralogy of Fallot with absent pulmonary valve syndrome (TOF/APV) is a rare congenital malformation. Although pulmonary artery (PA) anomalies have been observed in TOF, its association with disconnected PA is extremely rare. We report successful stenting of the disconnected left PA in a 3-year-old boy with TOF/APV followed by surgical reimplantation. The significance of this transcatheter intervention for guidance during surgery and the importance of visualizing a ductal stump on angiography as an indicator of disconnected PA are discussed.

Research paper thumbnail of Risk of reoperation for structural failure of aortic and mitral tissue valves

The Journal of Heart Valve Disease, May 1, 2002

The study aim was to assess the risk of reoperation for patients with a failing stented tissue va... more The study aim was to assess the risk of reoperation for patients with a failing stented tissue valve. Between 1980 and 1999, 259 patients (118 males, 141 females; mean age 60.1+/-15.4 years) underwent redo valve replacement to replace a failing stented tissue valve. Of these patients, 94 (36.3%) underwent redo aortic valve replacement (AVR), 105 (40.5%) redo mitral valve replacement (MVR), and 60 (23.2%) redo aortic and mitral valve replacement (DVR). Twenty patients (7.7%) had previous coronary artery bypass grafting (CABG); further CABG were performed in 32 cases (12.4%). Preoperatively, 216 patients (83.3%) were in NYHA functional class III or IV. The early mortality was (6.5%; n = 17), including three patients who had AVR, five DVR, and nine MVR. A higher preoperative NHYA status (p <0.0004) and emergency surgery (p <0.0001) were significantly associated with an increased risk of operative death (univariate analysis). Age at surgery (p = 0.45), previous CABG (p = 0.45), position of the valve replaced (p = 0.2), type of implant (p = 0.06) and presence of coronary artery disease (p = 0.51) were not associated with a significant risk of operative mortality. Including those patients who died, 88 (34.0%) experienced a peri- or postoperative complication, seven of which (2.7%) were permanent. A failing tissue valve can be replaced, with acceptable operative mortality and morbidity. The choice of valve is a balance of its advantages and disadvantages, and these must be discussed with the patient. It appears, however, that the trend towards reducing the age at which tissue valve implantation is performed may be justified.

Research paper thumbnail of Hepatic fibrosis and cirrhosis in the Fontan circulation: a detailed morphological study

Journal of Clinical Pathology, Apr 1, 2008

Aims:To describe the histological features of the liver in patients with a Fontan circulation.Met... more Aims:To describe the histological features of the liver in patients with a Fontan circulation.Methods:Specimens from liver biopsies carried out as part of preoperative assessment prior to extracardiac cavopulmonary conversion of an older style Fontan were examined and scored semi-quantitatively for pertinent histological features. To support the use of the scoring, biopsy specimens were also ranked by eye for severity to allow correlation with assigned scores.Results:Liver biopsy specimens from 18 patients with a Fontan circulation were assessed. All specimens showed sinusoidal fibrosis. In 17 cases there was at least fibrous spur formation, with 14 showing bridging fibrosis and 2 showing frank cirrhosis. In 17 cases at least some of the dense or sinusoidal fibrosis was orcein positive, although a larger proportion of the dense fibrous bands were orcein positive compared with the sinusoidal component. All specimens showed marked sinusoidal dilatation, and 14 showed bile ductular proliferation; 1 showed minimal iron deposition, and 1 showed mild lobular lymphocytic inflammation. There was no cholestasis or evidence of hepatocellular damage. Similar appearances were observed in 2 patients with severe tricuspid regurgitation.Discussion:The histological features of the liver in patients with a Fontan circulation are similar to those described in cardiac sclerosis. Sinusoidal dilatation and sinusoidal fibrosis are marked in the Fontan series. The presence of a significant amount of orcein negative sinusoidal fibrosis suggests there may be a remediable component, although the dense fibrous bands are predominantly orcein positive, suggesting chronicity and permanence. No inflammation or hepatocellular damage is evident, suggesting that fibrosis may be mediated by a non-inflammatory mechanism.

Research paper thumbnail of Bypass Rate of Alveolar Production of Exhaled Nitric Oxide During Cardiopulmonary A Prospective Randomized Study to Evaluate the Effect of Leukodepletion on the

Research paper thumbnail of Case report - Congenital Use of extra corporeal membrane oxygenation in the management of sepsis secondary to an infected right ventricle-to-pulmonary artery Contegra conduit in an adult patient

This is the first report in the cardiac surgical literature in a grown-up congenital heart male p... more This is the first report in the cardiac surgical literature in a grown-up congenital heart male patient with endocarditis of the Contegra conduit who developed septic shock with cardio-respiratory failure and required treatment with extra corporeal membrane oxygenation (ECMO) in order to stabilize his clinical condition preoperatively.

Research paper thumbnail of Arterialised hepatic nodules in the Fontan circulation: Hepatico-cardiac interactions

International Journal of Cardiology, Jun 1, 2010

Hypervascular nodules occur commonly when there is hepatic venous outlet obstruction. Their natur... more Hypervascular nodules occur commonly when there is hepatic venous outlet obstruction. Their nature and determinants in the Fontan circulation is poorly understood. We reviewed the records of 27 consecutive Fontan patients who had computerized tomography scan (CT) over a 4 year period for arterialised nodules and alterations in hepatic flow patterns during contrast enhanced CT scans and related these findings to cardiac characteristics. Mean patient age was 24 ± 5.8 years, (range 16.7-39.8) and mean Fontan duration was 16.8 ± 4.8 years (range 7.3-28.7). Twenty-two patients demonstrated a reticular pattern of enhancement, 4 a zonal pattern and only 1 demonstrated normal enhancement pattern. Seven (26%) patients had a median of 4 (range 1-22) arterialised nodules, mean size 1.8 cm (range 0.5 to 3.2 cm). All nodules were located in the liver periphery, their outer aspect lying within 2 cm of the liver margin. Patients with nodules had higher mean RA pressures (18 mmHg ± 5.6 vs. 13 mmHg ± 4, p = 0.025), whereas their mixed venous saturation and aortic saturation was not significantly different (70%± 11 vs. 67% ± 9 and 92% ± 10 vs. 94% ± 4, p N 0.05). Post-mortem histology suggests focal nodular hyperplasia is the underlying pathology. ConclusionsAbnormalities of hepatic blood flow and the presence of arterialised nodules are common in the failing Fontan circulation. They occur especially when central venous pressures are high, and very likely indicate arterialisation of hepatic blood flow and reciprocal portal venous deprivation. The underlying pathology is most likely focal nodular hyperplasia.

Research paper thumbnail of With Severe Cardiac Dysfunction After Ravitch Procedure Use of Extracorporeal Membrane Oxygenation in the Management of Septic Shock

Research paper thumbnail of Biventricular Repair of Pulmonary Atresia After Fontan Palliation

The Annals of thoracic surgery, 2016

Fontan palliation is used when biventricular repair (BVR) is not possible. Early outcomes are acc... more Fontan palliation is used when biventricular repair (BVR) is not possible. Early outcomes are acceptable; however, the long-term sequelae include protein-losing enteropathy, declining functional status, increased pulmonary vascular resistance, heart failure, and hepatic and renal dysfunction. These adverse events are characteristic of persistent venous hypertension and may be avoided if restoring biventricular circulation is possible. Arrhythmias are a common adverse event, particularly in patients with an atriopulmonary connection, which may lead to acute decompensation and early death. We describe a 30-year-old woman who underwent successful BVR for pulmonary atresia with intact ventricular septum and demonstrate that where favorable anatomy exists with a failing Fontan, BVR should be considered.

Research paper thumbnail of Integration of Computed Tomography and Three-Dimensional Echocardiography for Hybrid Three-Dimensional Printing in Congenital Heart Disease

Journal of digital imaging, Dec 12, 2016

Three-dimensional (3D) printing is an emerging technology aiding diagnostics, education, and inte... more Three-dimensional (3D) printing is an emerging technology aiding diagnostics, education, and interventional, and surgical planning in congenital heart disease (CHD). Three-dimensional printing has been derived from computed tomography, cardiac magnetic resonance, and 3D echocardiography. However, individually the imaging modalities may not provide adequate visualization of complex CHD. The integration of the strengths of two or more imaging modalities has the potential to enhance visualization of cardiac pathomorphology. We describe the feasibility of hybrid 3D printing from two imaging modalities in a patient with congenitally corrected transposition of the great arteries (L-TGA). Hybrid 3D printing may be useful as an additional tool for cardiologists and cardiothoracic surgeons in planning interventions in children and adults with CHD.

Research paper thumbnail of Surgical Treatment of Neonate With Congenital Left Main Coronary Artery Atresia

The Annals of Thoracic Surgery, 2016

Left main coronary artery atresia (LMCAA) is a rare congenital malformation with a nonspecific an... more Left main coronary artery atresia (LMCAA) is a rare congenital malformation with a nonspecific and varied clinical presentation. Ventricular dysfunction and mitral insufficiency are expected ischemic consequences in the neonatal period. Left internal mammary artery (LIMA) bypass grafting (CABG) is uncommon because of the technical difficulties in performing this procedure in neonates. We describe LMCAA revascularization with a LIMA graft and mitral valve repair in a 7-week-old neonate with successful outcome 1 year postoperatively.

Research paper thumbnail of Implantable cardioverter defibrillator therapy in paediatric practice: a single-centre UK experience with focus on subcutaneous defibrillation

Europace European Pacing Arrhythmias and Cardiac Electrophysiology Journal of the Working Groups on Cardiac Pacing Arrhythmias and Cardiac Cellular Electrophysiology of the European Society of Cardiology, Jan 20, 2013

Research paper thumbnail of Methylenetetrahydrofolate Reductase C677T: Hypoplastic Left Heart and Thrombosis

World journal for pediatric & congenital heart surgery, 2015

The incidence of congenital heart defects is higher in infants with mutation of methylenetetrahyd... more The incidence of congenital heart defects is higher in infants with mutation of methylenetetrahydrofolate reductase (MTHFR) gene. The MTHFR C677T gene decreases the bioavailability of folate and increases plasma homocysteine, a risk factor for thrombosis. There have been no reported cases in the literature on the clinical implications of this procoagulable state in the setting of cyanotic heart disease, which itself has prothrombotic predisposition. Two patients with hypoplastic left heart syndrome developed postoperative thrombotic complications, both were homozygous for MTHFR C677T. We present these cases and highlight the implications of MTHFR mutation in the management of complex congenital heart disease.

Research paper thumbnail of 14. Biventricular Repair of Palliated Congenital Heart Disease: A Single Center Experience

Objective: Complex congenital heart disease presenting early in life may not be considered amenab... more Objective: Complex congenital heart disease presenting early in life may not be considered amenable for complete repair. These patients undergo palliative procedures most commonly utilizing the functioning ventricle to pump blood into the systemic and pulmonary circulations and staged rerouting of systemic venous blood to the pulmonary arteries. However, single ventricle physiology is associated with a number of well recognized complications. We describe successful palliation in 3 patients with complex congenital heart disease previously deemed irreparable and discuss its implications. Methods: Three patients with the following diagnosis – heterotaxy with complete atrioventricular canal defect, pulmonary atresia with intact ventricular septum, and pulmonary atresia with ventricular septal defect and major aortopulmonary connections (MAPCAs) – underwent a comprehensive assessment using two- and three-dimensional echocardiography, cardiac magnetic resonance imaging and cardiac cathete...

Research paper thumbnail of P.33 Reversal of pulmonary arteriovenous malformations following biventricular repair of patient with complex heterotaxy syndrome

Background & Objective  Patients with heterotaxy syndrome and interruption of inferior vena cava... more Background & Objective  Patients with heterotaxy syndrome and interruption of inferior vena cava (IVC) are commonly palliated with the Kawashima procedure or bidirectional cavopulmonary anastomosis.

Research paper thumbnail of P.32 Surgical treatment of a neonate with left main coronary artery atresia

Background & Objective  Congenital left main coronary artery atresia (LMCAA) is an extremely rar... more Background & Objective  Congenital left main coronary artery atresia (LMCAA) is an extremely rare abnormality where the proximal mainstem ends blindly and blood flows from the right coronary artery (RCA) to the left via collaterals.  We report successful LMCAA revascularization and continued patency in a neonate using a left internal mammary artery (LIMA) graft one-year after coronary artery bypass grafting (CABG).

Research paper thumbnail of Abstract 222: Combination of Blood Cardioplegic and Strategic Reperfusion Period Leucodepletion During Cardiopulmonary Bypass Preserves the Cardiac and Pulmonary Function: A Prospective Randomized Controlled Study

Circulation Research, 2012

Objectives: Leucocytes activation during cardiopulmonary bypass (CPB) contributes to postoperativ... more Objectives: Leucocytes activation during cardiopulmonary bypass (CPB) contributes to postoperative organ dysfunction. We compared the outcomes of various leucodepletion strategies in patients undergoing coronary artery bypass grafting (CABG). Methods: One hundred-twenty low-risk patients undergoing first time CABG were prospectively randomized to six groups: 1 non-leucodepletion arterial filtration; 2 continuous arterial leucodepletion; 3 reperfusion period leucodepletion; 4 leucodepletion of blood cardioplegia; 5 combination of continuous arterial and blood cardioplegic leucodepletion; 6 combination of blood cardioplegic and reperfusion period leucodepletion. Blood samples were taken 5 min before CPB, 5 and 30 min on CPB, 5, 60 min after aortic X-clamp removal and 6 h post-CPB. Activated leucocytes were identified with Nitroblue Tetrazolium staining. Exhaled nitro oxide (NO) was measured pre- and post-CPB using real-time chemiluminescense analyzer. Respiratory index (alveolar-arter...

Research paper thumbnail of Viral Induced Genetics Revealed by Multi-Dimensional Precision Medicine Transcriptional Workflow

Precision medicine requires the translation of basic biological understanding to medical insights... more Precision medicine requires the translation of basic biological understanding to medical insights, mainly applied to characterization of each unique patient. In many clinical settings, this requires tools that can be broadly used to identify pathology and risks. Patients often present to the intensive care unit with broad phenotypes, including multiple organ dysfunction syndrome (MODS) resulting from infection, trauma, or other disease processes. Etiology and outcomes are unique to individuals, making it difficult to cohort patients with MODS, but presenting a prime target for testing/developing tools for precision medicine. Using multi-time point whole blood (cellular/acellular) total transcriptomics in 27 patients, we highlight the promise of simultaneously mapping viral/bacterial load, cell composition, tissue damage biomarkers, balance between syndromic biology vs. environmental response, and unique biological insights in each patient using a single platform measurement. Integra...

Research paper thumbnail of Virus-induced genetics revealed by multidimensional precision medicine transcriptional workflow applicable to COVID-19

Physiological Genomics

Precision medicine requires the translation of basic biological understanding to medical insights... more Precision medicine requires the translation of basic biological understanding to medical insights, mainly applied to characterization of each unique patient. In many clinical settings, this requires tools that can be broadly used to identify pathology and risks. Patients often present to the intensive care unit with broad phenotypes, including multiple organ dysfunction syndrome (MODS) resulting from infection, trauma, or other disease processes. Etiology and outcomes are unique to individuals, making it difficult to cohort patients with MODS, but presenting a prime target for testing/developing tools for precision medicine. Using multitime point whole blood (cellular/acellular) total transcriptomics in 27 patients, we highlight the promise of simultaneously mapping viral/bacterial load, cell composition, tissue damage biomarkers, balance between syndromic biology versus environmental response, and unique biological insights in each patient using a single platform measurement. Integ...

Research paper thumbnail of Three‐dimensional printing for surgical planning in complex congenital heart disease

Journal of Cardiac Surgery

Research paper thumbnail of Circulating Microrna Signatures as Potential Markers of Tissue Specific Stress in Children Undergoing Cardiopulmonary Bypass

Journal of the American College of Cardiology

Research paper thumbnail of Stenting and Reimplanting Disconnected Pulmonary Artery in Tetralogy of Fallot

The Annals of thoracic surgery, 2017

Tetralogy of Fallot with absent pulmonary valve syndrome (TOF/APV) is a rare congenital malformat... more Tetralogy of Fallot with absent pulmonary valve syndrome (TOF/APV) is a rare congenital malformation. Although pulmonary artery (PA) anomalies have been observed in TOF, its association with disconnected PA is extremely rare. We report successful stenting of the disconnected left PA in a 3-year-old boy with TOF/APV followed by surgical reimplantation. The significance of this transcatheter intervention for guidance during surgery and the importance of visualizing a ductal stump on angiography as an indicator of disconnected PA are discussed.

Research paper thumbnail of Risk of reoperation for structural failure of aortic and mitral tissue valves

The Journal of Heart Valve Disease, May 1, 2002

The study aim was to assess the risk of reoperation for patients with a failing stented tissue va... more The study aim was to assess the risk of reoperation for patients with a failing stented tissue valve. Between 1980 and 1999, 259 patients (118 males, 141 females; mean age 60.1+/-15.4 years) underwent redo valve replacement to replace a failing stented tissue valve. Of these patients, 94 (36.3%) underwent redo aortic valve replacement (AVR), 105 (40.5%) redo mitral valve replacement (MVR), and 60 (23.2%) redo aortic and mitral valve replacement (DVR). Twenty patients (7.7%) had previous coronary artery bypass grafting (CABG); further CABG were performed in 32 cases (12.4%). Preoperatively, 216 patients (83.3%) were in NYHA functional class III or IV. The early mortality was (6.5%; n = 17), including three patients who had AVR, five DVR, and nine MVR. A higher preoperative NHYA status (p <0.0004) and emergency surgery (p <0.0001) were significantly associated with an increased risk of operative death (univariate analysis). Age at surgery (p = 0.45), previous CABG (p = 0.45), position of the valve replaced (p = 0.2), type of implant (p = 0.06) and presence of coronary artery disease (p = 0.51) were not associated with a significant risk of operative mortality. Including those patients who died, 88 (34.0%) experienced a peri- or postoperative complication, seven of which (2.7%) were permanent. A failing tissue valve can be replaced, with acceptable operative mortality and morbidity. The choice of valve is a balance of its advantages and disadvantages, and these must be discussed with the patient. It appears, however, that the trend towards reducing the age at which tissue valve implantation is performed may be justified.

Research paper thumbnail of Hepatic fibrosis and cirrhosis in the Fontan circulation: a detailed morphological study

Journal of Clinical Pathology, Apr 1, 2008

Aims:To describe the histological features of the liver in patients with a Fontan circulation.Met... more Aims:To describe the histological features of the liver in patients with a Fontan circulation.Methods:Specimens from liver biopsies carried out as part of preoperative assessment prior to extracardiac cavopulmonary conversion of an older style Fontan were examined and scored semi-quantitatively for pertinent histological features. To support the use of the scoring, biopsy specimens were also ranked by eye for severity to allow correlation with assigned scores.Results:Liver biopsy specimens from 18 patients with a Fontan circulation were assessed. All specimens showed sinusoidal fibrosis. In 17 cases there was at least fibrous spur formation, with 14 showing bridging fibrosis and 2 showing frank cirrhosis. In 17 cases at least some of the dense or sinusoidal fibrosis was orcein positive, although a larger proportion of the dense fibrous bands were orcein positive compared with the sinusoidal component. All specimens showed marked sinusoidal dilatation, and 14 showed bile ductular proliferation; 1 showed minimal iron deposition, and 1 showed mild lobular lymphocytic inflammation. There was no cholestasis or evidence of hepatocellular damage. Similar appearances were observed in 2 patients with severe tricuspid regurgitation.Discussion:The histological features of the liver in patients with a Fontan circulation are similar to those described in cardiac sclerosis. Sinusoidal dilatation and sinusoidal fibrosis are marked in the Fontan series. The presence of a significant amount of orcein negative sinusoidal fibrosis suggests there may be a remediable component, although the dense fibrous bands are predominantly orcein positive, suggesting chronicity and permanence. No inflammation or hepatocellular damage is evident, suggesting that fibrosis may be mediated by a non-inflammatory mechanism.

Research paper thumbnail of Bypass Rate of Alveolar Production of Exhaled Nitric Oxide During Cardiopulmonary A Prospective Randomized Study to Evaluate the Effect of Leukodepletion on the

Research paper thumbnail of Case report - Congenital Use of extra corporeal membrane oxygenation in the management of sepsis secondary to an infected right ventricle-to-pulmonary artery Contegra conduit in an adult patient

This is the first report in the cardiac surgical literature in a grown-up congenital heart male p... more This is the first report in the cardiac surgical literature in a grown-up congenital heart male patient with endocarditis of the Contegra conduit who developed septic shock with cardio-respiratory failure and required treatment with extra corporeal membrane oxygenation (ECMO) in order to stabilize his clinical condition preoperatively.

Research paper thumbnail of Arterialised hepatic nodules in the Fontan circulation: Hepatico-cardiac interactions

International Journal of Cardiology, Jun 1, 2010

Hypervascular nodules occur commonly when there is hepatic venous outlet obstruction. Their natur... more Hypervascular nodules occur commonly when there is hepatic venous outlet obstruction. Their nature and determinants in the Fontan circulation is poorly understood. We reviewed the records of 27 consecutive Fontan patients who had computerized tomography scan (CT) over a 4 year period for arterialised nodules and alterations in hepatic flow patterns during contrast enhanced CT scans and related these findings to cardiac characteristics. Mean patient age was 24 ± 5.8 years, (range 16.7-39.8) and mean Fontan duration was 16.8 ± 4.8 years (range 7.3-28.7). Twenty-two patients demonstrated a reticular pattern of enhancement, 4 a zonal pattern and only 1 demonstrated normal enhancement pattern. Seven (26%) patients had a median of 4 (range 1-22) arterialised nodules, mean size 1.8 cm (range 0.5 to 3.2 cm). All nodules were located in the liver periphery, their outer aspect lying within 2 cm of the liver margin. Patients with nodules had higher mean RA pressures (18 mmHg ± 5.6 vs. 13 mmHg ± 4, p = 0.025), whereas their mixed venous saturation and aortic saturation was not significantly different (70%± 11 vs. 67% ± 9 and 92% ± 10 vs. 94% ± 4, p N 0.05). Post-mortem histology suggests focal nodular hyperplasia is the underlying pathology. ConclusionsAbnormalities of hepatic blood flow and the presence of arterialised nodules are common in the failing Fontan circulation. They occur especially when central venous pressures are high, and very likely indicate arterialisation of hepatic blood flow and reciprocal portal venous deprivation. The underlying pathology is most likely focal nodular hyperplasia.

Research paper thumbnail of With Severe Cardiac Dysfunction After Ravitch Procedure Use of Extracorporeal Membrane Oxygenation in the Management of Septic Shock

Research paper thumbnail of Biventricular Repair of Pulmonary Atresia After Fontan Palliation

The Annals of thoracic surgery, 2016

Fontan palliation is used when biventricular repair (BVR) is not possible. Early outcomes are acc... more Fontan palliation is used when biventricular repair (BVR) is not possible. Early outcomes are acceptable; however, the long-term sequelae include protein-losing enteropathy, declining functional status, increased pulmonary vascular resistance, heart failure, and hepatic and renal dysfunction. These adverse events are characteristic of persistent venous hypertension and may be avoided if restoring biventricular circulation is possible. Arrhythmias are a common adverse event, particularly in patients with an atriopulmonary connection, which may lead to acute decompensation and early death. We describe a 30-year-old woman who underwent successful BVR for pulmonary atresia with intact ventricular septum and demonstrate that where favorable anatomy exists with a failing Fontan, BVR should be considered.

Research paper thumbnail of Integration of Computed Tomography and Three-Dimensional Echocardiography for Hybrid Three-Dimensional Printing in Congenital Heart Disease

Journal of digital imaging, Dec 12, 2016

Three-dimensional (3D) printing is an emerging technology aiding diagnostics, education, and inte... more Three-dimensional (3D) printing is an emerging technology aiding diagnostics, education, and interventional, and surgical planning in congenital heart disease (CHD). Three-dimensional printing has been derived from computed tomography, cardiac magnetic resonance, and 3D echocardiography. However, individually the imaging modalities may not provide adequate visualization of complex CHD. The integration of the strengths of two or more imaging modalities has the potential to enhance visualization of cardiac pathomorphology. We describe the feasibility of hybrid 3D printing from two imaging modalities in a patient with congenitally corrected transposition of the great arteries (L-TGA). Hybrid 3D printing may be useful as an additional tool for cardiologists and cardiothoracic surgeons in planning interventions in children and adults with CHD.

Research paper thumbnail of Surgical Treatment of Neonate With Congenital Left Main Coronary Artery Atresia

The Annals of Thoracic Surgery, 2016

Left main coronary artery atresia (LMCAA) is a rare congenital malformation with a nonspecific an... more Left main coronary artery atresia (LMCAA) is a rare congenital malformation with a nonspecific and varied clinical presentation. Ventricular dysfunction and mitral insufficiency are expected ischemic consequences in the neonatal period. Left internal mammary artery (LIMA) bypass grafting (CABG) is uncommon because of the technical difficulties in performing this procedure in neonates. We describe LMCAA revascularization with a LIMA graft and mitral valve repair in a 7-week-old neonate with successful outcome 1 year postoperatively.

Research paper thumbnail of Implantable cardioverter defibrillator therapy in paediatric practice: a single-centre UK experience with focus on subcutaneous defibrillation

Europace European Pacing Arrhythmias and Cardiac Electrophysiology Journal of the Working Groups on Cardiac Pacing Arrhythmias and Cardiac Cellular Electrophysiology of the European Society of Cardiology, Jan 20, 2013

Research paper thumbnail of Methylenetetrahydrofolate Reductase C677T: Hypoplastic Left Heart and Thrombosis

World journal for pediatric & congenital heart surgery, 2015

The incidence of congenital heart defects is higher in infants with mutation of methylenetetrahyd... more The incidence of congenital heart defects is higher in infants with mutation of methylenetetrahydrofolate reductase (MTHFR) gene. The MTHFR C677T gene decreases the bioavailability of folate and increases plasma homocysteine, a risk factor for thrombosis. There have been no reported cases in the literature on the clinical implications of this procoagulable state in the setting of cyanotic heart disease, which itself has prothrombotic predisposition. Two patients with hypoplastic left heart syndrome developed postoperative thrombotic complications, both were homozygous for MTHFR C677T. We present these cases and highlight the implications of MTHFR mutation in the management of complex congenital heart disease.

Research paper thumbnail of 14. Biventricular Repair of Palliated Congenital Heart Disease: A Single Center Experience

Objective: Complex congenital heart disease presenting early in life may not be considered amenab... more Objective: Complex congenital heart disease presenting early in life may not be considered amenable for complete repair. These patients undergo palliative procedures most commonly utilizing the functioning ventricle to pump blood into the systemic and pulmonary circulations and staged rerouting of systemic venous blood to the pulmonary arteries. However, single ventricle physiology is associated with a number of well recognized complications. We describe successful palliation in 3 patients with complex congenital heart disease previously deemed irreparable and discuss its implications. Methods: Three patients with the following diagnosis – heterotaxy with complete atrioventricular canal defect, pulmonary atresia with intact ventricular septum, and pulmonary atresia with ventricular septal defect and major aortopulmonary connections (MAPCAs) – underwent a comprehensive assessment using two- and three-dimensional echocardiography, cardiac magnetic resonance imaging and cardiac cathete...

Research paper thumbnail of P.33 Reversal of pulmonary arteriovenous malformations following biventricular repair of patient with complex heterotaxy syndrome

Background & Objective  Patients with heterotaxy syndrome and interruption of inferior vena cava... more Background & Objective  Patients with heterotaxy syndrome and interruption of inferior vena cava (IVC) are commonly palliated with the Kawashima procedure or bidirectional cavopulmonary anastomosis.

Research paper thumbnail of P.32 Surgical treatment of a neonate with left main coronary artery atresia

Background & Objective  Congenital left main coronary artery atresia (LMCAA) is an extremely rar... more Background & Objective  Congenital left main coronary artery atresia (LMCAA) is an extremely rare abnormality where the proximal mainstem ends blindly and blood flows from the right coronary artery (RCA) to the left via collaterals.  We report successful LMCAA revascularization and continued patency in a neonate using a left internal mammary artery (LIMA) graft one-year after coronary artery bypass grafting (CABG).