Abstract 4553: Pre-Operative Brain Injury in Newborn Infants with Transposition of the Great Arteries Occurs at Similar Rates to other Complex Congenital Heart Disease, and is not Related to Balloon Atrial Septostomy

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Julia Gunn ◽  
Lara Shekerdemian ◽  
Rod Hunt ◽  
Lee Coleman ◽  
Ayton Hope ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Brain Injury ◽  
Ischaemic Stroke ◽  
Congenital Heart ◽  
Pulmonary Atresia ◽  
Complex Congenital Heart Disease ◽  
Balloon Atrial Septostomy ◽  
Increased Risk ◽  
Atrial Septostomy

Introduction: Brain injury (BI) in the form of white matter injury (WMI) or ischaemic stroke, seen on magnetic resonance imaging (MRI), is common in neonates with congenital heart disease (CHD). It has previously been suggested that balloon atrial septostomy (BAS) may be an independent risk factor for pre-operative BI in neonates with transposition of the great arteries (TGA). Hypothesis : We hypothesised that: 1. The incidence of BI is similar in neonates with TGA and other severe CHD 2. The risk of pre-operative BI is not increased by BAS Methods: Neonates with TGA, hypoplastic left heart syndrome (HLHS) or pulmonary atresia (PA) were enrolled in a prospective study of BI related to CHD. Pre-operative brain MRI was performed in all patients. In those with TGA who underwent BAS, MRI occurred after septostomy. T1, T2 and diffusion-weighted images were obtained in axial and coronal planes. Scans were reviewed by blinded assessors, and were classified according to WMI (punctate lesions) or ischaemic stroke. Fisher’s Exact Test was used to compare rates of WMI and stroke in each patient group. Results: Sixty-six neonates (mean weight 3.4±0.5kg) were studied, of whom 44 had TGA, 13 had HLHS and 9 had PA. The median age at MRI was 7 days (range 1–31). Thirty-five of the neonates with TGA underwent BAS a median of 1 day (1–21) followed by MRI 7 days (2–12) later. BI was present in 17 (26%) patients. The table shows the incidence of BI in the sub-groups. Two neonates with a stroke also had WMI. Table showing MRI abnormalities in patient sub-groups. Neonates with TGA were not at increased risk of pre-operative BI compared with those with HLHS or PA (p=1.0). BAS did not increase the risk of pre-operative BI in neonates with TGA (p=1.0). Conclusions: Pre-operative BI occurs at a similar rate in neonates with TGA and other severe CHD. The risk of pre-operative BI in neonates with TGA is not increased by BAS.

scholarly journals Stenting of modified Blalock–Taussig shunt in adult with palliated pulmonary atresia and ventricular septal defect: a case report

2019 ◽  
Vol 3 (4) ◽  
pp. 1-4
Author(s):  
Julia Illner ◽  
Holger Reinecke ◽  
Helmut Baumgartner ◽  
Gerrit Kaleschke
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Congenital Heart ◽  
Septal Defect ◽  
Pulmonary Atresia ◽  
Complex Congenital Heart Disease ◽  
Bare Metal

Abstract Background Adults with complex congenital heart disease palliated with systemic-to-pulmonary artery shunts have become rare and represent a particularly challenging patient group for the cardiologist. One of the complications and causes of severe clinical deterioration during long-term follow-up are progressive obstruction or total occlusion of the shunt. The risk for surgical intervention is frequently high and catheter intervention may be complicated by complex anatomy and shunt calcification. Case summary We report the case of a 47-year-old man with uncorrected (palliated) pulmonary atresia and ventricular septal defect who presented with progressive cyanosis (oxygen saturation 69%) and decreasing exercise capacity. Computed tomography revealed a totally occluded modified left Blalock–Taussig (BT) shunt and a severely stenosed central shunt (Waterston–Cooley) in a patient with confluent but hypoplastic pulmonary arteries and multiple major aortic pulmonary collaterals. Due to a high operative risk, an interventional, percutaneous approach was preferred to re-do surgery. From a radial access the calcified BT shunt could be crossed with a hydrophilic guidewire. Then, a rotational thrombectomy, balloon dilatation, and bare-metal stenting at the proximal and distal anastomoses were performed. Post-interventionally, peripheral oxygen saturation increased from 69% to 82%. Clopidogrel was administered for 1 month after bare-metal stenting. At 1-year follow-up, the BT shunt was still patent on echocardiography and exercise tolerance markedly improved. Discussion This case highlights the benefit of percutaneous rotational thrombectomy followed by stenting of chronically occluded systemic-to-pulmonary artery shunts for further palliation in adult patients with complex congenital heart disease not suitable for surgical repair.

scholarly journals Acute ischaemic stroke in secondary polycythaemia due to complex congenital cyanotic heart disease

2019 ◽  
Vol 12 (10) ◽  
pp. e231261
Author(s):  
Jeyaraj Durai Pandian ◽  
Ivy Anne Sebastian ◽  
Ashlee Sidhu
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Ischaemic Stroke ◽  
Congenital Heart ◽  
Septal Defect ◽  
Brain Mri ◽  
Neurological Deficits ◽  
Complex Congenital Heart Disease ◽  
Haematocrit Level ◽  
Polycythaemia Vera

A 65-year-old woman presented to the emergency department with an acute onset of left-sided hemiparesis and slurred speech for 6 hours. Physical examination was notable for clubbing and peripheral cyanosis. Brain MRI showed an acute infarct involving the right gangliocapsular region. Investigations showed an elevated haemoglobin level, a haematocrit level of >65% and thrombocytopaenia. Transthoracic echocardiography revealed tricuspid atresia, ventricular septal defect and atrial septal defect. A diagnosis of cyanotic congenital heart disease was made. Studies have indicated an association between raised haematocrit and vascular occlusive episodes in patients with polycythaemia vera. However, a relationship between raised haematocrit due to polycythaemia secondary to complex congenital heart disease and vascular thrombosis has not been reported. The improvement of neurological deficits in our patient coincided with a decline in haematocrit. The objective of this report was to highlight an association between elevated haematocrit and ischaemic stroke in patients with secondary polycythaemia due to complex congenital heart disease.

scholarly journals Balloon Atrial Septostomy in Congenital Heart Disease　– Reply –

2016 ◽  
Vol 80 (4) ◽  
pp. 1051
Author(s):  
Hisashi Sugiyama ◽  
Kazuto Fujimoto ◽  
Tetsuko Ishii ◽  
Toshio Nakanishi ◽  
Hideshi Tomita
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Balloon Atrial Septostomy ◽  
Atrial Septostomy

scholarly journals Cardiopatias Congénitas Complexas: Influência do Diagnóstico Pré-Natal

2015 ◽  
Vol 28 (2) ◽  
pp. 158 ◽  
Author(s):  
Marta Correia ◽  
Fabiana Fortunato ◽  
Duarte Martins ◽  
Ana Teixeira ◽  
Graça Nogueira ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Prenatal Diagnosis ◽  
Congenital Heart ◽  
Pulmonary Atresia ◽  
Complex Congenital Heart Disease ◽  
Significant Relation ◽  
Transposition Of Great Arteries ◽  
Birth Place ◽  
Tertiary Center

<strong>Introduction:</strong> Complex congenital heart disease is a group of severe conditions. Prenatal diagnosis has implications on morbidity and mortality for most severe conditions. The purpose of this work was to evaluate the influence of prenatal diagnosis and distance of residence and birth place to a reference center, on immediate morbidity and early mortality of complex congenital heart disease.<br /><strong>Material and Methods::</strong> Retrospective study of complex congenital heart disease patients of our Hospital, born between 2007 and 2012.<br /><strong>Results::</strong> There were 126 patients born with complex congenital heart disease. In 95%, pregnancy was followed since the first trimester, with prenatal diagnosis in 42%. There was a statistically significant relation between birth place and prenatal diagnosis. Transposition of great arteries was the most frequent complex congenital heart disease (45.2%), followed by pulmonary atresia with ventricular septal defect (17.5%) and hypoplastic left ventricle (9.5%). Eighty-two patients (65.1%) had prostaglandin infusion and 38 (30.2%)were ventilated before an intervention. Surgery took place in the neonatal period in 73%. Actuarial survival rate at 30 days, 12 and 24 months was 85%, 80% and 75%, respectively. There was no statistically significant relation between prenatal diagnosis and mortality.<br /><strong>Discussion::</strong> Most patients with complex congenital heart disease did not have prenatal diagnosis. All cases with prenatal diagnosis were born in a tertiary center. Prenatal diagnosis did not influence significantly neonatal mortality, as already described in other studies with heterogeneous complex heart disease.<br /><strong>Conclusion::</strong> prenatal diagnosis of complex congenital heart disease allowed an adequate referral. Most patients with complex congenital heart disease weren’t diagnosed prenatally. This data should be considered when planning prenatal diagnosis of congenital heart disease.

scholarly journals Heart Transplantation in Congenital Heart Disease: In Whom to Consider and When?

2013 ◽  
Vol 2013 ◽  
pp. 1-12 ◽  
Author(s):  
Christine H. Attenhofer Jost ◽  
Dörthe Schmidt ◽  
Michael Huebler ◽  
Christian Balmer ◽  
Georg Noll ◽  
...  
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Heart Transplantation ◽  
Lung Transplantation ◽  
Congenital Heart ◽  
Complex Congenital Heart Disease ◽  
Eisenmenger’S Syndrome ◽  
Number Of Patients ◽  
Increased Risk

Due to impressive improvements in surgical repair options, even patients with complex congenital heart disease (CHD) may survive into adulthood and have a high risk of end-stage heart failure. Thus, the number of patients with CHD needing heart transplantation (HTx) has been increasing in the last decades. This paper summarizes the changing etiology of causes of death in heart failure in CHD. The main reasons, contraindications, and risks of heart transplantation in CHD are discussed and underlined with three case vignettes. Compared to HTx in acquired heart disease, HTx in CHD has an increased risk of perioperative death and rejection. However, outcome of HTx for complex CHD has improved over the past 20 years. Additionally, mechanical support options might decrease the waiting list mortality in the future. The number of patients needing heart-lung transplantation (especially for Eisenmenger’s syndrome) has decreased in the last years. Lung transplantation with intracardiac repair of a cardiac defect is another possibility especially for patients with interatrial shunts. Overall, HTx will remain an important treatment option for CHD in the near future.

Abstract 18813: Outcomes of Patients with Congenital Heart Disease following Heart Transplantation: the Impact of Disease Type, Prior Thoracic Surgeries and End-Organ Dysfunction

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Matthew J Lewis ◽  
Jonathan Ginns ◽  
P. C Schulze ◽  
Matt Lippel ◽  
Paul Chai ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Heart Transplantation ◽  
Organ Dysfunction ◽  
Congenital Heart ◽  
Fontan Operation ◽  
Pulmonary Atresia ◽  
Patient Specific ◽  
Increased Risk ◽  
The Impact

Introduction: Adults with congenital heart disease (ACHD) are at increased risk for early adverse outcomes following heart transplantation (Htx). Despite the need for improved risk stratification, small cohorts have constrained identification of patient-specific factors associated with poor prognosis. We hypothesized that type of CHD, number of sternotomies and prior end-organ dysfunction would be associated with an increased risk for mortality post-HTx. Methods: We performed a retrospective, observational cohort study of all patients with ACHD who underwent HTx at our institution from 1/1997 to 1/2014. The primary endpoint was death. Exposures of interest included type of CHD, number of sternotomies and hepatic dysfunction secondary to passive congestion, measured as high Models for End-Stage Liver Disease Excluding INR (MELD-XI) score. Results: 48 ACHD patients were followed (mean age at HTx: 36±1.7 years). Diagnoses included: Tetralogy of Fallot (TOF)/pulmonary atresia/double outlet right ventricle in 15 (31%), D-transposition of the great arteries in 10 (21 %), tricuspid atresia/double inlet left ventricle in 9 (19%), VSD/ASD in 4 (8%), heterotaxy in 3 (6%), congenitally corrected transposition of the great arteries in 2 (4%), and 5 (10%) with other diagnoses. Pre-transplant cardiac surgeries included: Fontan operation in 12 (25%), Mustard/Senning repair in 9 (19%), TOF repair in 5 (11%), Rastelli repair in 5 (11%), VSD/ASD closures in 3 (6%), other another congenital heart surgery in 8 (17%) and no prior cardiac surgery in 5 (11%). Over a median follow-up time of 3.9 years, 14 patients died (29%) and 10 (71%) deaths occurred within 30 days of HTx. Survival at 1 and 5 years was 77%. Deaths within 30 days of surgery were secondary to hemorrhage in 4 (40%), graft failure in 3 (30%) and multi-system organ failure in 3 (30%). By multivariate analysis, ≥3 sternotomies (HR 8.5, p=0.02), MELD-XI score >18 (HR 6.2, p=0.009) and heterotaxy (HR 9.8, p=0.01), were significant predictors of mortality. Conclusion: In our cohort of patients with CHD undergoing Htx, ≥3 sternotomies, MELD-XI score >18 and heterotaxy syndrome were significantly associated with death. These findings may be important in patient selection and timing of Htx in this population.

scholarly journals Novel JAG1 Deletion Variant in Patient with Atypical Alagille Syndrome

2019 ◽  
Vol 20 (24) ◽  
pp. 6247 ◽  
Author(s):  
Emanuele Micaglio ◽  
Andreea Alina Andronache ◽  
Paola Carrera ◽  
Michelle M. Monasky ◽  
Emanuela T. Locati ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Neural Tube ◽  
Neural Tube Defect ◽  
Congenital Heart ◽  
Ductus Arteriosus ◽  
Pulmonary Atresia ◽  
Alagille Syndrome ◽  
Collateral Arteries ◽  
Increased Risk

Alagille syndrome (AGS) is an autosomal-dominant disorder characterized by various degrees of abnormalities in the liver, heart, eyes, vertebrae, kidneys, face, vasculature, skeleton, and pancreas. This case report describes a newborn child exhibiting a congenital neural tube defect and peculiar craniofacial appearance characterized by a prominent forehead, deep-set eyes, bulbous nasal tip, and subtle upper lip. Just a few hours after birth, congenital heart disease was suspected for cyanosis and confirmed by heart evaluation. In particular, echocardiography indicated pulmonary atresia with ventricular septal defect with severe hypoplasia of the pulmonary branches (1.5 mm), large patent ductus arteriosus and several major aortopulmonary collateral arteries. Due to the association of peculiar craniofacial appearance and congenital heart disease, a form of Alagille syndrome was suspected. In addition, on the fifth day after birth, the patient developed jaundice, had acholic stools, and high levels of conjugated bilirubin and gamma-glutamyltransferase (GGT) were detected in the blood. Genetic testing revealed the novel variant c.802del in a single copy of the JAG1 gene. No variants in the NOTCH2 gene were detected. To the best of our knowledge, this is the first clinical description of a congenital neural tube defect in a molecularly confirmed Alagille patient. This work demonstrates a novel pathogenic heterozygous JAG1 mutation is associated with an atypical form of Alagille syndrome, suggesting an increased risk for neural tube defects compared to other Alagille patients.

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