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and tiered systems of care in other areas of medicine have been developed and embedded in the complex and large U.S. healthcare system. Often a key question is: what should constitute the designation of a centerwithinasystemasspecializedandcomprehensive? Examples of requirements for such a designation are available in multiple areas of medicine, both for acute problems (stroke, trauma, and myocardial infarction) and selected chronic disorders (bariatric surgery for obesity, adultcongenitaldisease (...) NationalHealthService,congenitalheartdisease care has been redirected; 3 tiers of care, ranging from localtospecialistsurgicalcenters,havebeen organized to provide different levels of care according to patient need (44). In the United States, adultcongenitalheartdisease centers (https://www.achaheart. org/provider-support/accreditation-program)aswellas pulmonary hypertension centers (https://phassociation. org/phcarecenters/medical-professionals/center-criteria/) have been formally designated after meeting
. Physical Inactivity e99 5. Nutrition e119 6. Overweight and Obesity e138 Health Factors and Other Risk Factors 7. High Blood Cholesterol and Other Lipids e161 8. High Blood Pressure e174 9. Diabetes Mellitus e193 10. Metabolic Syndrome e212 11. Kidney Disease e233 12. Sleep e249 Cardiovascular Conditions/Diseases 13. Total Cardiovascular Diseases e257 14. Stroke (Cerebrovascular Disease) e281 15. Congenital Cardiovascular Defects and Kawasaki Disease e327 16. Disorders of Heart Rhythm e346 17. Sudden (...) States and globally. The Statistical Update also presents the latest data on a range of major clinical heart and circulatory diseaseconditions (including stroke, congenitalheartdisease, rhythm disorders, subclinical atherosclerosis, coronaryheartdisease [CHD], heart failure [HF], valvular disease, venous disease, and peripheral arterial disease) and the associated outcomes (including quality of care, procedures, and economic costs). Since 2007, the annual versions of the Statistical Update have
Dilated cardiomyopathy (DCM) is characterized by enlargement of the left ventricle of the heart and systolic impairment, in the absence of abnormal loading conditions or coronary artery disease sufficient to cause global systolic dysfunction (Haas 2015). The symptoms of DCM are similar to heart failure including shortness of breath, chest pain/tightness, fainting episodes and cardiac arrhythmias. The most serious complication of DCM is sudden, irregular heart rhythms that can be life threatening. Some (...) with an isolated type 2 or 3 Brugada pattern on EKG. In most cases, the primary value of genetic testing for Brugada syndrome is to benefit at-risk family members. Short QT Syndrome Short QT syndrome (SQTS) is a congenital, inherited, primary electric disorder of the heart characterized by abnormally short QT intervals on the surface ECG (<360 ms) and an increased proclivity to develop atrial and/or ventricular tachyarrhythmias (Gussak 2005). SQTS is a genetically heterogeneous disease caused by mutations
catheterization, noninvasive imaging, and medical therapies have significantly lowered mortality rates for children and adolescents with complex congenitalheartdisease (CHD). , Survivors are at risk for neurodevelopmental morbidity caused by both biological and environmental risk factors. Biological risk factors include underlying syndromes or genetic/developmental disorders, the circulatory abnormalities specific to the heart defect, and the medical and surgical therapies required. Biological risk factors (...) >2-wk in the hospital) 3.8. Perioperative seizures related to CHD surgery 3.9. Significant abnormalities on neuroimaging or microcephaly 4. Other conditions determined at the discretion of the medical home providers CHD indicates congenitalheartdisease; HLHS, hypoplastic left heartsyndrome; IAA, interrupted aortic arch; PA/IVS, pulmonary atresia with intact ventricular septum; TA, truncus arteriosus; TAPVC, total anomalous pulmonary venous connection; TGA, transposition of the great arteries
needs are transferred or transi- tioned on an ad hoc basis although there are not dedicated clinics for these patient groups in the UK. Until recently, the majority of patients with coeliac disease and other common GI illnesses (con- stipation, GORD), together with simple functional GI disorders, have been transferred from paediatric to adult care or discharged from paediatric care with the expectation that the primary care physician will refer to an appropriate adult consultant if required. However (...) , the most common indication for liver transplant in children is for congenital reasons (biliary atresia) compared with acquired liver disease in adulthood. Recommendations and practice points We recommend that AYP with IBD, coeliac disease and chronic liver disease should be involved in formal transition arrange- ments (GRADE recommendation: strong (C)). Practice points ? AYP with other chronic GI and liver conditions, including complex functional disorders, are likely to bene?tfrom formal transition
Myocardial factor revisited: The importance of myocardial fibrosis in adults with congenitalheartdisease. Pioneers in congenitalheart surgery observed that exercise capacity did not return to normal levels despite successful surgical repair, leading some to cite a "myocardial factor" playing a role. They conjectured that residual alterations in myocardial function would be significant for patients' long-term outlook. In fulfillment of their early observations, today's adultcongenitalheart (...) disease (ACHD) population shows well-recognized features of heart failure, even among patients without clear residual anatomic or hemodynamic abnormalities, demonstrating the vital role of the myocardium in their morbidity and mortality. Whereas the 'myocardial factor' was an elusive concept in the early history of congenitalheart care, we now have imaging techniques to detect and quantify one such factor--myocardial fibrosis. Understanding the importance of myocardial fibrosis as a final common
Free breathing contrast-enhanced time-resolved magnetic resonance angiography in pediatric and adultcongenitalheartdisease. Contrast enhanced magnetic resonance angiography (MRA) is generally performed during a long breath-hold (BH), limiting its utility in infants and small children. This study proposes a free-breathing (FB) time resolved MRA (TRA) technique for use in pediatric and adultcongenitalheartdisease (CHD).A TRA sequence was developed by combining spiral trajectories (...) with sensitivity encoding (SENSE, x4 kx-ky and x2 kz) and partial Fourier (75% in kz). As no temporal data sharing is used, an independent 3D data set was acquired every ~1.3s, with acceptable spatial resolution (~2.3x2.3x2.3 mm). The technique was tested during FB over 50 consecutive volumes. Conventional BH-MRA and FB-TRA data was acquired in 45 adults and children with CHD. We calculated quantitative image quality for both sequences. Diagnostic accuracy was assessed in all patients from both sequences
Structural and congenitalheartdisease interventions: the role of three-dimensional printing Advances in catheter-based interventions in structural and congenitalheartdisease have mandated an increased demand for three-dimensional (3D) visualisation of complex cardiac anatomy. Despite progress in 3D imaging modalities, the pre- and periprocedural visualisation of spatial anatomy is relegated to two-dimensional flat screen representations. 3D printing is an evolving technology based (...) in the catheter-based treatment of adult patients with structural and congenitalheartdisease while highlighting the current limitations of this technology in terms of segmentation, model accuracy and dynamic capabilities. Furthermore, we provide information on the resources needed to establish a hospital-based 3D printing laboratory.
Acyanotic CongenitalHeartDisease and Transesophageal Echocardiography The spectrum of congenitalheartdisease (CHD) seen in the adult varies widely. Malformations range from mild anomalies requiring no intervention to extremely complex pathologies characterized by the presence of multiple coexistent defects. Echocardiography represents the primary noninvasive imaging modality in the assessment of these lesions. The transesophageal approach expands the applications of echocardiography (...) by allowing the acquisition of anatomic and functional information that may not be obtainable by transthoracic imaging.
Multimodality imaging in congenitalheartdisease-related pulmonary arterial hypertension. Pulmonary arterial hypertension (PAH) in adult patients with congenitalheartdisease (CHD) is associated with increased morbidity and mortality. The present review aims to discuss the clinical applications of invasive and non-invasive diagnostic modalities and to describe the strengths and weaknesses of each technique. Chest radiograph is an inexpensive investigation providing information on pulmonary (...) and pulmonary circulation, may represent an ideal target for evaluation with cardiac magnetic resonance. This non-invasive imaging modality has a low biological impact. CT plays an important role for patients with limited echocardiographic windows and those who are unable to undergo MRI (claustrophobia, poor compliance, presence of a pacemaker/implantable cardioverter defibrillator). It is the modality of choice for detailed assessment of pulmonary vessel obstruction or thrombosis. Finally, heart
An adult case of Bland-White-Garland syndrome with collaterals from giant right coronary artery and bronchial artery Bland-White-Garland syndrome (BWG) is a congenitalheartdisease characterized by anomalous origin of left coronary artery from main pulmonary artery. The anomaly causes significant myocardial ischemia, mitral insufficiency, and high infant mortality. Several investigators have reported that the subjects who survived to adulthood showed extensive collateral flow from right (...) coronary artery (RCA). We report a case of newly diagnosed BWG in an elderly patient who had well-developed collaterals from aneurysmal giant RCA and bronchial artery, which were identified by coronary angiography with multi-detector computed tomography. heart disease is occasionally hard to assess with invasive angiography. In this case, multi-detector computed tomography demonstrated complicated structure of anomalous coronary morphology including collateral sources
navigation 21 September 2017 Article Contents Article Navigation 2017 ESC/EACTS Guidelines for the management of valvular heartdisease Helmut Baumgartner Corresponding authors: Helmut Baumgartner, Division of AdultCongenital and Valvular HeartDisease, Department of Cardiovascular Medicine, University Hospital Muenster, Albert Schweitzer Campus 1, Building A1, 48149 Muenster, Germany. Tel: +49 251 834 6110, Fax: +49 251 834 6109, E-mail: . Volkmar Falk, Department of Cardiothoracic and Vascular Surgery (...) , German Heart Center, Augustenburger Platz 1, D-133353 Berlin, Germany and Department of Cardiovascular Surgery, Charite Berlin, Charite platz 1, D-10117 Berlin, Germany. Tel: +49 30 4593 2000, Fax: +49 30 4593 2100, E-mail: . Search for other works by this author on: Volkmar Falk Corresponding authors: Helmut Baumgartner, Division of AdultCongenital and Valvular HeartDisease, Department of Cardiovascular Medicine, University Hospital Muenster, Albert Schweitzer Campus 1, Building A1, 48149 Muenster
of English-language articles from 1966 to 2015, cross-referencing congenital and acquired heartdisease with pertinent MESH search terms, as follows: acute cardiac tamponade; acute kidney injury; adoles- cent; adult; amiodarone; aortic valve insufficiency/sur - gery; aortic valve stenosis/congenital; aortic valve steno- sis/surgery; aortic valve stenosis/therapy; arrhythmias, cardiac; atropine; bicarbonates; biomarkers; calcium; cardiac arrest; cardiac catheterization; cardiac output, low; cardiac (...) Chitra Ravishankar, MD Ricardo A. Samson, MD Ravi R. Thiagarajan, MBBS, MPH Rune Toms, MD James Tweddell, MD, FAHA Peter C. Laussen, MBBS, Co-Chair On behalf of the American Heart Association CongenitalCardiac Defects Committee of the Council on Cardiovascular Disease in the Young; Council on Clinical Cardiology; Council on Cardiovascular and Stroke Nursing; Council on Cardio- vascular Surgery and Anesthe- sia; and Emergency Cardiovas- cular Care Committee Cardiopulmonary Resuscitation in Infants
Computed tomography imaging in patients with congenitalheartdisease, part I: Rationale and utility. An expert consensus document of the Society of Cardiovascular Computed Tomography (SCCT). This is an expert consensus document created to provide information about the current use of cardiovascular computed tomography (CT) in patients of all ages with proven or suspected congenitalheartdisease (CHD). The discussion and recommendations are based on available literature and the judgment (...) background of the cardiacimager. This is reflected by a writing committee that consists of pediatric and adult radiologists and cardiologists, all whom have extensive experience in performing CT in this patient population. Cardiovascular CT is complementary to other imaging modalities and its optimal use will be in centers where all diagnostic modalities are available. The choice of modality for an individual patient should be determined by age, diagnosis, clinical condition, clinical question
Mirror image atrial dilatation in adult patients with atrial fibrillation and congenitalheartdisease. Atrial fibrillation (AF) is largely regarded to be initiated from left atrial (LA) dilatation, with subsequent dilatation of the right atrium (RA) in those who progress to chronic AF. We hypothesized that in adult patients with right-sided congenitalheartdisease (CHD) and AF, RA dilatation will predominate with subsequent dilatation of the left atrium, as a mirror image.Adult patients (...) . Multivariate analysis showed that age (p=0.0001), RA (p=0.025) and LA area (p=0.0016) were significantly related to AF. In patients with pure left-sided pathologies, there was progressive and predominant LA dilatation that paralleled the development of AF from none to paroxysmal to chronic AF. In patients with pure right-sided pathologies, there was a mirror image of progressive and predominant RA dilatation with the development of AF.We observed a mirror image atrial dilatation in patients with right
solid organ transplantation, adultcongenitalheartdisease, cardiac amyloidosis, high sensitization, and post-transplantation management of antibodies to human leukocyte antigen, rejection, cardiac allograft vasculopathy, and long-term noncardiac care. Attention is also given to Canadian-specific management strategies including the prioritization of highly sensitized transplant candidates (status 4S) and heart organ allocation algorithms. The focus topics in this position statement highlight (...) year). Radovancevic B. McGiffin D.C. Kobashigawa J.A. et al. Retransplantation in 7,290 primary transplant patients: a 10-year multi-institutional study. J Heart Lung Transplant. 2003; 22 : 862-868 14. We recommend cardiac retransplantation for highly selected patients with severe chronic allograft dysfunction (Strong Recommendation, Moderate-Quality Evidence). Values and preferences. Retransplantation should be avoided in patients with AR. Adultcongenitalheartdisease There are no published
that is not urgent or emergent; (v) ablation of arrhythmias thought to be contributing to cardiomyopathy (Domain 4). • More detailed classification of elective EP and CIED procedures that could be considered as “non-urgent (Domain 4). • Addition of Domain 6, with recommendations for EP and CIED implications for children and adults with congenitalheartdisease. HRC COVID-19 9/4/2020, version 2 3 EXECUTIVE SUMMARY The COVID-19 pandemic poses a significant stress on health resources in Australia. The Heart Rhythm (...) a pneumonic respiratory illness, with the potential for severe cardiovascular damage. Its high infectivity rate has led to rapid escalation of affected cases around the world. It demonstrates a higher mortality rate amongst patients with pre- existing illness, in particular, those with cardiovascular disease. This has prompted a rapid evaluation of routine cardiac electrophysiology (EP) and Cardiac Implantable Electronic Devices (CIED) services within Australia. Major international societies (Heart Rhythm