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CVIR Endovascular Jun 2021To describe technical details of modifying four different Cook Zenith devices to treat complex aortic aneurysms.
PURPOSE
To describe technical details of modifying four different Cook Zenith devices to treat complex aortic aneurysms.
MATERIAL
In the first three cases, the modification process involved complete stent graft deployment on a sterile back table. Fenestrations were created using an ophthalmologic cautery and reinforced with a radiopaque snare using a double-armed 4-0 Ethibond locking suture based on measurements obtained on centerline of flow. In each instance, a nitinol wire was withdrawn and redirected through and through the fabric and used as a constraining wire. In the fourth patient, modification involved partial stent graft deployment and creation of additional two fenestrations to accommodate renal arteries. The devices are resheathed and implanted in the standard fashion.
RESULTS
Four patients underwent exclusion of their aneurysms, including thoracoabdominal aneurysms (n = 2), a contained ruptured juxtarenal aneurysm (n = 1), and a ruptured failed previous endovascular repair (n = 1). Fifteen fenestrations were successfully bridged with Atrium iCAST stent grafts. Average graft modification time, operative time, contrast volume, radiation dose, estimated blood loss, and hospital length of stay were 89 min, 155.25 min, 58.8 mL, 2451 mGy, 175 mL, and 4.3 days, respectively. One patient required a secondary intervention to treat a type Ib endoleak. During an average follow-up of 25 months, aneurysm sacs progressively shrank without additional intervention.
CONCLUSION
Physician-modified fenestrated/branched endografts are a safe alternative to custom made devices, especially in urgent cases and should be part of the armamentarium of any complex aortic program.
PubMed: 34061297
DOI: 10.1186/s42155-021-00233-7 -
Global Heart Apr 2021Recent advances in the diagnosis and management of pulmonary arterial hypertension (PAH) have led to a significant improvement in the outcomes for patients with PAH.... (Review)
Review
UNLABELLED
Recent advances in the diagnosis and management of pulmonary arterial hypertension (PAH) have led to a significant improvement in the outcomes for patients with PAH. However, prompt and accurate diagnosis of PAH remains an unmet challenge due to lack of awareness and lack of meticulous data to profile the etiology and pathophysiology of this rare progressive disease, especially in low- and middle-income country. In Indonesia, the true prevalence and incidence of different subtypes of PAH in general population is still unknown. The Congenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry was the first single-center prospective registry in Indonesia, which indicated that almost 80% of adult patients with congenital heart disease (CHD) had experienced PAH and even Eisenmenger syndrome due to delayed diagnosis. Screening for early detection of asymptomatic CHD in children is yet to be systematically established in Indonesia, leading to undiagnosed and uncorrected CHD in adulthood. There are no specific national guidelines focusing on diagnostic workup and treatment of PAH in Indonesia. Furthermore, the lack of adequate diagnostic facilities, limited treatment availability, and limited drug coverage under the National Health Insurance Scheme are key issues that remain unaddressed. This review focuses on the diagnosis, treatment, and management of PAH associated with CHD in Indonesia as per international guidelines. We have proposed recommendations to effectively control and prevent PAH associated with CHD in Indonesia. The paper should be of interest to readers in the area of medical management and policy makers especially in low- and middle-income countries.
KEY HIGHLIGHTS
Pulmonary arterial hypertension (PAH) is a rare progressive subtype of pulmonary hypertension with poor overall prognosis and outcomes.Prompt and accurate diagnosis of PAH remains an unmet challenge in low- and middle-income countries due to poor knowledge about the etiology and pathophysiology of this syndrome. Also, the symptoms and signs of early-stage PAH are usually nonspecific or undetectable in newborn and infants, thus presenting a challenge for physicians to establish early diagnoses of PAH.The challenging factors in low- and middle-income countries, especially Indonesia archipelago are limitations of healthcare infrastructure, limited expertise, lack of awareness, lack of timely PAH screening strategies, poor antenatal care and unpredictable availability of PAH medications.There are no specific national guidelines focusing on diagnostic workup and treatment of PAH in Indonesia. Under-utilization of treatment guidelines and lack of adequate diagnostic treatment facilities have resulted in sub-optimal management of PAH patients in Indonesia.Adherence to international guidelines is an important aspect of PAH management in Indonesia. Updated disease and functional classifications of PAH as per international guidelines along with new research findings on prognostic factors can help in making better management decisions for PAH patients at different stages of the disease.
Topics: Adult; Eisenmenger Complex; Female; Heart Defects, Congenital; Humans; Hypertension, Pulmonary; Indonesia; Pregnancy; Pulmonary Arterial Hypertension
PubMed: 34040936
DOI: 10.5334/gh.944 -
Open Heart Apr 2021Adults with congenital heart disease (ACHD) may be at a higher risk of a fatal outcome in case of COVID-19. Current risk stratification among these patients relies on...
OBJECTIVE
Adults with congenital heart disease (ACHD) may be at a higher risk of a fatal outcome in case of COVID-19. Current risk stratification among these patients relies on personal experience and extrapolation from patients with acquired heart disease. We aimed to provide an expert view on risk stratification while awaiting results from observational studies.
METHODS
This study was an initiative of the EPOCH (European Collaboration for Prospective Outcome Research in Congenital Heart disease). Among nine European countries (Austria, Belgium, Denmark, France, Germany, Italy, the Netherlands, Spain and Switzerland), 24 experts from 23 tertiary ACHD centres participated in the survey. ACHD experts were asked to identify ACHD-specific COVID-19 risk factors from a list of potential outcome predictors and to estimate the risk of adverse COVID-19 outcomes in seven commonly seen patient scenarios.
RESULTS
82% of participants did not consider all ACHD patients at risk of COVID-19 related complications. There was a consensus on pulmonary arterial hypertension, Fontan physiology and cyanotic heart disease as risk factors for adverse outcomes. Among different ACHD scenarios, a patient with Eisenmenger syndrome was considered to be at the highest risk. There was a marked variability in risk estimation among the other potential outcome predictors and ACHD scenarios.
CONCLUSIONS
Pulmonary arterial hypertension, Fontan palliation and cyanotic heart disease were widely considered as risk factors for poor outcome in COVID-19. However, there was a marked disparity in risk estimation for other clinical scenarios. We are in urgent need of outcome studies in ACHD suffering from COVID-19.
Topics: COVID-19; Eisenmenger Complex; Europe; Fontan Procedure; Global Burden of Disease; Heart Defects, Congenital; Humans; Outcome Assessment, Health Care; Prognosis; Pulmonary Arterial Hypertension; Risk Assessment; Risk Factors; SARS-CoV-2; Societies, Medical; Surveys and Questionnaires
PubMed: 33883228
DOI: 10.1136/openhrt-2020-001455 -
ESC Heart Failure Apr 2021Adult patients with uncorrected congenital heart diseases and chronic intracardiac shunt may develop Eisenmenger syndrome (ES) due to progressive increase of pulmonary...
Adult patients with uncorrected congenital heart diseases and chronic intracardiac shunt may develop Eisenmenger syndrome (ES) due to progressive increase of pulmonary vascular resistance, with significant morbidity and mortality. Acute decompensation of ES in conditions promoting a further increase of pulmonary vascular resistance, such as pulmonary embolism or pneumonia, can precipitate major arterial hypoxia and death. In such conditions, increasing systemic oxygenation with veno-venous extracorporeal membrane oxygenation (VV-ECMO) could be life-saving, serving as a bridge to treat a potential reversible cause for the decompensation, or to urgent lung transplantation. Anticipating the effects of VV-ECMO in this setting could ease the clinical decision to initiate such therapeutic strategy. Here, we present a series of equations to accurately predict the effects of VV-ECMO on arterial oxygenation in ES and illustrate this point by a case of ES decompensation with refractory hypoxaemia consecutive to an acute respiratory failure due to viral pneumonia.
Topics: Adult; Eisenmenger Complex; Extracorporeal Membrane Oxygenation; Humans; Pneumonia, Viral; Respiratory Distress Syndrome
PubMed: 33630406
DOI: 10.1002/ehf2.13253 -
The Journal of International Medical... Jan 2021Aortopulmonary window is a rare congenital heart disease that can increase pulmonary vascular resistance, exacerbate left-to-right shunt and lead to heart failure and...
Aortopulmonary window is a rare congenital heart disease that can increase pulmonary vascular resistance, exacerbate left-to-right shunt and lead to heart failure and respiratory tract infections. Most patients die during childhood. We report a 53-year-old male patient with a large aortopulmonary window combined with anomalous origin of the right pulmonary artery from the aorta, with Eisenmenger syndrome and without surgery.
Topics: Adult; Aorta; Aortopulmonary Septal Defect; Eisenmenger Complex; Humans; Male; Middle Aged; Pulmonary Artery; Vascular Resistance
PubMed: 33472471
DOI: 10.1177/0300060520984656 -
BMC Cardiovascular Disorders Dec 2020Patent ductus arteriosus (PDA) complicated by Eisenmenger syndrome (ES) remains to be a major cause of morbidity and mortality worldwide. Giving increasing evidences of...
BACKGROUND
Patent ductus arteriosus (PDA) complicated by Eisenmenger syndrome (ES) remains to be a major cause of morbidity and mortality worldwide. Giving increasing evidences of benefit from targeted therapies, ES patients once thought to be inoperable may have increasing options for management. This study aims to explore whether PDA in patients with ES can be treated with transcatheter closure (TCC).
METHODS
Between August 2014 and July 2016, four of fifteen PDA-ES patients whose Qp/Qs improved significantly and Qp/Qs > 1.5 after acute vasodilator testing with 100% oxygen were selected to receive TCC and pulmonary vasodilator therapy. PAH-targeted drugs were prescribed before and after occlusion for all. Trial occlusion was performed before permanent closure.
RESULTS
The first TCC failed after initiation of PAH-targeted drugs for 6 months in four patients. After the medication was adjusted and extended to 12 months, TCC was performed for all without hemodynamic intolerances during perioperative period. Pulmonary artery systolic pressure (PASP) was significantly decreased (≥ 40%) immediately after TCC. During a mean follow-up of 48 ± 14.70 months, there were a further decrease of PASPs in two patients, the other two showed improved pulmonary vascular resistance, WHO functional class and six-minute walking distance despite deteriorated PASP.
CONCLUSION
Some selected PDA-ES patients might benefit from TCC and combined PAH-targeted drugs play a crucial role.
Topics: Adult; Antihypertensive Agents; Arterial Pressure; Cardiac Catheterization; Combined Modality Therapy; Drug Therapy, Combination; Ductus Arteriosus, Patent; Eisenmenger Complex; Female; Humans; Male; Pulmonary Artery; Recovery of Function; Retrospective Studies; Time Factors; Treatment Outcome; Vasodilator Agents; Young Adult
PubMed: 33261574
DOI: 10.1186/s12872-020-01795-5 -
Tidsskrift For Den Norske Laegeforening... Nov 2020The combination of jaundice and acute abdominal pain is a common clinical problem associated with a broad array of aetiologies.
BACKGROUND
The combination of jaundice and acute abdominal pain is a common clinical problem associated with a broad array of aetiologies.
CASE PRESENTATION
A 36-year-old male with Down's syndrome and Eisenmenger's syndrome presented with abdominal pain, jaundice and acute liver failure. Initial transabdominal ultrasound and subsequent magnetic resonance cholangiopancreatography (MRCP) revealed gallbladder stones, but no common bile duct stones. During the course of the patient's hospital admission, his liver chemistries were consistently elevated. Thus, endoscopic retrograde cholangiography (ERC) with sphincterotomy was performed, despite the anaesthesiological risk associated with his chronic heart failure. However, the ERC and sphincterotomy did not relieve the patient's symptoms and had no apparent effect on his abnormal liver chemistries. By the end of his hospital stay, the patient recovered spontaneously and was discharged with no final conclusion having been reached. An unexpected turn of events led us to conclude upon a diagnosis a few weeks later.
INTERPRETATION
This case illustrates the challenges of a multidisciplinary approach in a complex patient, and an overlooked detail that became a lesson to learn from.
Topics: Abdominal Pain; Adult; Cholangiopancreatography, Endoscopic Retrograde; Gallstones; Heart Failure; Humans; Jaundice; Male
PubMed: 33231388
DOI: 10.4045/tidsskr.20.0191 -
Medicina (Kaunas, Lithuania) Aug 2020Pulmonary hypertensive vascular disease (PHVD), and pulmonary hypertension (PH), which is a broader term, are severe conditions associated with high morbidity and... (Review)
Review
Pulmonary hypertensive vascular disease (PHVD), and pulmonary hypertension (PH), which is a broader term, are severe conditions associated with high morbidity and mortality at all ages. Treatment guidelines in childhood are widely adopted from adult data and experience, though big differences may exist regarding aetiology, concomitant conditions and presentation. Over the past few years, paediatric aspects have been incorporated into the common guidelines, which currently address both children and adults with pulmonary hypertension (PH). There are multiple facets of PH in the context of cardiac conditions in childhood. Apart from Eisenmenger syndrome (ES), the broad spectrum of congenital heart disease (CHD) comprises PH in failing Fontan physiology, as well as segmental PH. In this review we provide current data and novel aspects on the pathophysiological background and individual management concepts of these conditions. Moreover, we focus on paediatric left heart failure with PH and its challenging issues, including end stage treatment options, such as mechanical support and paediatric transplantation. PH in the context of rare congenital disorders, such as Scimitar Syndrome and sickle cell disease is discussed. Based on current data, we provide an overview on multiple underlying mechanisms of PH involved in these conditions, and different management strategies in children and adulthood. In addition, we summarize the paediatric aspects and the pros and cons of the recently updated definitions of PH. This review provides deeper insights into some challenging conditions of paediatric PH in order to improve current knowledge and care for children and young adults.
Topics: Anemia, Sickle Cell; Antihypertensive Agents; Bronchopulmonary Dysplasia; Child; Down Syndrome; Eisenmenger Complex; Heart Failure; Heart Transplantation; Heart-Lung Transplantation; Hemodynamics; Humans; Hypertension, Pulmonary; Scimitar Syndrome; Thromboembolism
PubMed: 32825190
DOI: 10.3390/medicina56090420 -
Methodist DeBakey Cardiovascular Journal 2020
Topics: Adult; Cor Triatriatum; Eisenmenger Complex; Female; Heart Septal Defects, Ventricular; Humans
PubMed: 32670480
DOI: 10.14797/mdcj-16-2-168 -
Journal of Investigative Medicine High... 2020Tetralogy of Fallot is the most common cyanotic congenital heart defect consisting of an overriding aorta, right ventricular outflow obstruction, ventricular septal...
Tetralogy of Fallot is the most common cyanotic congenital heart defect consisting of an overriding aorta, right ventricular outflow obstruction, ventricular septal defect, and right ventricular hypertrophy. Without surgical management, approximately only 3% of patients survive past the age of 40 years. Cases of unoperated patients reaching adulthood have been reported; however, few studies describe treatment guidelines for surgical or therapeutic management. In this article, we report the case of a 59-year-old Hispanic male with unoperated tetralogy of Fallot presenting to our cardiology clinic for initial workup and management.
Topics: Anticoagulants; Atrial Fibrillation; Cardiac Catheterization; Disease Management; Eisenmenger Complex; Electrocardiography; Humans; Hypertension, Pulmonary; Hypertrophy, Right Ventricular; Male; Middle Aged; Radiography, Thoracic; Survivors; Tetralogy of Fallot
PubMed: 32462941
DOI: 10.1177/2324709620926908