Graphical Abstract
Graphical abstract
Pathophysiology of ventricular arrhythmia for repaired tetralogy of Fallot in relation to native right ventricular outflow tract transcatheter pulmonary valve ...replacement. Following repair, progressive degenerative remodelling incurs a risk for sustained monomorphic ventricular tachycardia. With transcatheter pulmonary valve replacement, ventricular tachycardia can become inaccessible to catheter ablation. A and B, trichrome stain demonstrating interstitial and replacement fibrosis in regions between the pulmonary annulus and ventriculotomy incision or ventricular septal defect patch, respectively (courtesy of UCLA pathology archives).
Since 1980, the American College of Cardiology (ACC) and American Heart Association (AHA) have translated scientific evidence into clinical practice guidelines (guidelines) with recommendations to ...improve cardiovascular health. These guidelines, which are based on systematic methods to evaluate and classify evidence, provide a cornerstone for quality cardiovascular care. The ACC and AHA sponsor the development and publication of guidelines without commercial support, and members of each organization volunteer their time to the writing and review efforts. Guidelines are official policy of the ACC and AHA.
The number of patients who require orthotopic heart transplantation (OHT) for failing Fontan physiology continues to grow; however, the methods and tools to evaluate risk of OHT are limited. This ...study aimed to identify a set of preoperative variables and characteristics that were associated with a greater risk of postoperative mortality in patients who received OHT for failing Fontan physiology. Thirty-six Fontan patients were identified as having undergone OHT at University of California-Los Angeles Medical Center from 1991 to 2014. Data were collected retrospectively and analyzed. The primary end point was designated as postoperative mortality. After an average follow-up time of 3.5 years, 17 (44%) patients suffered postoperative mortality. Patient characteristics including (1) age <18 years at the time of OHT, (2) Fontan-OHT interval of <10 years, (3) systemic ventricular ejection fraction <20%, (4) moderate-to-severe atrioventricular valve insufficiency, (5) an elevated Model of End-stage Liver Disease, eXcluding INR score, or (6) need for advanced mechanical support before surgery were associated with an increased incidence of postoperative mortality. Using these risk factors, we present a theoretical framework to stratify risk of postoperative death in failing Fontan patients after OHT. In conclusion, a method such as this may aid in the transplantation evaluation and listing process of patients with failing Fontan physiology.
The corona virus disease -2019 (COVID-19) is a recently described infectious disease caused by the severe acute respiratory syndrome corona virus 2 with significant cardiovascular implications. Given ...the increased risk for severe COVID-19 observed in adults with underlying cardiac involvement, there is concern that patients with pediatric and congenital heart disease (CHD) may likewise be at increased risk for severe infection. The cardiac manifestations of COVID-19 include myocarditis, arrhythmia and myocardial infarction. Importantly, the pandemic has stretched health care systems and many care team members are at risk for contracting and possibly transmitting the disease which may further impact the care of patients with cardiovascular disease. In this review, we describe the effects of COVID-19 in the pediatric and young adult population and review the cardiovascular involvement in COVID-19 focusing on implications for patients with congenital heart disease in particular.
The field of adult congenital interventions is unique in the world of cardiac catheterization, combining the structural concepts commonly employed in pediatric heart disease and applying them to ...adult patients, who are more amenable to device intervention as they no longer experience somatic growth. Rapid advances in the field have been made to match the growing population of adult patients with congenital heart disease, which currently surpasses the number of pediatric patients born with congenital heart disease. Many congenital defects, which once required surgical intervention or reoperation, can now be addressed via the transcatheter approach, minimizing the morbidity and mortality often encountered within adult congenital surgeries. In this paper, we aim to provide a focused review of the more common procedures that are utilized for the treatment of adult congenital heart disease patients in the catheterization laboratory, as examples of current practices in the United States, as well as emerging concepts and devices awaiting approval in the future.
Liver health in adults with Fontan circulation: A multicenter cross-sectional study Wu, Fred M., MD; Kogon, Brian, MD; Earing, Michael G., MD ...
Journal of thoracic and cardiovascular surgery/The Journal of thoracic and cardiovascular surgery/The journal of thoracic and cardiovascular surgery,
03/2017, Letnik:
153, Številka:
3
Journal Article
Recenzirano
Odprti dostop
Abstract Objectives Liver disease is an important contributor to morbidity and mortality in patients after Fontan surgery. There has been no large-scale survey of liver health in this population. We ...sought to explore the prevalence and predictors of liver disease in a multicenter cohort of adults with Fontan physiology. Methods Subjects were recruited from 6 adult congenital heart centers. Demographics; clinical history; and laboratory, imaging, and histopathology data were obtained. Results Of 241 subjects (median age 25.8 years 11.8-59.4, median time since Fontan 20.3 years 5.4-34.5), more than 94% of those who underwent testing (208 of 221) had at least 1 abnormal liver-related finding. All hepatic imaging (n = 54) and liver histology (n = 68) was abnormal. Subjects with abnormal laboratory values had higher sinusoidal fibrosis stage (2 vs 1, P = .007) and higher portal fibrosis stage (3 vs 1, P = .003) compared with those with all normal values. Low albumin correlated with lower sinusoidal fibrosis stage (1 vs 2; P = .02) and portal fibrosis stage (0 vs 3, P = .002); no other liver studies correlated with fibrosis. Regenerative nodules were seen on 33% of histology specimens. Conclusions Regardless of modality, findings of liver disease are common among adults with Fontan circulation, even those appearing clinically well. Cirrhosis is present in up to one-third of subjects. Correlations between hepatic fibrosis stage and clinical history or findings on noninvasive testing are few. Further research is needed to identify patients at risk for more severe liver disease and to determine the best methods for assessing liver health in this population.
Currently in the United States, more than 1 million adults have congenital heart disease. Therefore, it will be inevitable for providers performing cardiac catheterization to be faced with adults ...with repaired congenital heart abnormalities. Moreover, a meticulous approach to every cardiac catheterization procedure is mandatory to avoid missing unrecognized congenital lesions presenting in adulthood. The authors provide a primer for cardiac catheterization procedures in adults with congenital heart disease, reviewing key procedural components: flow calculation, detection of shunts according to different lesions, and, when indicated, ventricular angiography and coronary angiography.
Hepatic fibrosis is highly prevalent in individuals with Fontan circulation. FibroSure (LabCorp, Burlington, NC) and hyaluronic acid (HA) have been validated for assessment of hepatic fibrosis in ...several forms of liver disease. We sought to determine whether these tests could identify Fontan patients with advanced hepatic fibrosis or cirrhosis.
Subjects who had liver biopsy and FibroSure or HA testing within 6 months of biopsy were identified from the Alliance for Adult Research in Congenital Cardiology Fontan Liver Health study. Biopsy specimens were scored for degree of sinusoidal and portal fibrosis on a 3- and 5-point scale, respectively. Histologic findings were correlated with FibroSure and HA results.
The study included 27 subjects. Median age was 26.8 years (range, 17.4-59.8 years), and the median time since the Fontan surgery was 20.4 years (range, 12.0-31.3 years). FibroSure scores were elevated (>0.21) in 21 of 23 subjects (91%), and the scores for 3 (13%) suggested cirrhosis (>0.74). HA suggested cirrhosis (>46 ng/mL) in 3 of 17 subjects (18%). One subject died during the collection period. Eleven of 26 subjects (42%) had 4/5 or 5/5 portal fibrosis, consistent with cirrhosis; 17 (63%) had 3/3 sinusoidal fibrosis involving >66% of sinusoids. The FibroSure score and HA levels did not correlate with the degree of hepatic fibrosis and did not predict cirrhosis.
Abnormal biomarkers of hepatic fibrosis and specimen-proven hepatic fibrosis are common in adults with Fontan circulation. However, FibroSure and HA do not accurately predict the degree of histologic hepatic fibrosis. Further studies are needed to guide strategies for surveillance of liver disease in this population.
Objectives
To describe a multicenter experience of percutaneous transcatheter pulmonary valve replacement (TPVR) using the Edwards SAPIEN S3 valve without the use of a prior stent (“pre‐stenting”).
...Background
The SAPIEN S3 and XT valves have durable cobalt–chromium stent frames which may allow for TPVR in large diameter dysfunctional right ventricular outflow tracts (RVOTs) without pre‐stenting the landing zone.
Methods
A retrospective review was performed of all patients with Congenital Heart Disease and dysfunctional RVOT who underwent TPVR using the SAPIEN valve without the use of a pre‐stent. Imaging data, procedural elements, and clinical follow‐up data were collected to evaluate short and intermediate‐term results.
Results
Fifty‐seven patients underwent percutaneous placement of the SAPIEN valve in the pulmonary position without the use of pre‐stenting. The anatomical substrate varied: native RVOTs (n = 41), conduits (n = 10), and bioprosthetic valves (n = 6). There were no cases in which the valve could not be implanted and no cases of valve embolization or misplacement. On follow‐up (range 1 month to 2.2 years, median 5.3 months), no patients had significant obstruction or regurgitation around the valve. There were no frame fractures. There were no procedural deaths. Major complications included severe aortic compression (n = 1) requiring surgical explantation and tricuspid valve injury requiring surgical intervention (n = 2).
Conclusions
This limited multi‐institutional experience demonstrates that the SAPIEN valve can be used for TPVR without the use of a pre‐stent without medium‐term risk of frame fracture, paravalvar leak, or embolization. Longer term follow‐up is required to fully assess this method.
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