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Current management aspects in adult congenital heart disease: heading for the future.

Cardiovasc Diagn Ther. 2018 Dec;8(6):696-697

Authors: Kaemmerer H, von Kodolitsch Y

PMID: 30740316 [PubMed]

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Improving medical care and prevention in adults with congenital heart disease-reflections on a global problem-part I: development of congenital cardiology, epidemiology, clinical aspects, heart failure, cardiac arrhythmia.

Cardiovasc Diagn Ther. 2018 Dec;8(6):705-715

Authors: Neidenbach R, Niwa K, Oto O, Oechslin E, Aboulhosn J, Celermajer D, Schelling J, Pieper L, Sanftenberg L, Oberhoffer R, de Haan F, Weyand M, Achenbach S, Schlensak C, Lossnitzer D, Nagdyman N, von Kodolitsch Y, Kallfelz HC, Pittrow D, Bauer UMM, Ewert P, Meinertz T, Kaemmerer H

Abstract
Today most patients with congenital heart defects (CHD) survive into adulthood. Unfortunately, despite relevant residua and sequels, follow-up care of adults with congenital heart disease (ACHD) is not performed in specialized and/or certified physicians or centres. Major problems in the long-term course encompass heart failure, cardiac arrhythmias, heart valve disorders, pulmonary vascular disease, infective endocarditis, aortopathy and non-cardiac comorbidities. Many of them manifest themselves differently from acquired heart disease and therapy regimens from general cardiology cannot be transferred directly to CHD. It should be noted that even simple, postoperative heart defects that were until recently considered to be harmless can lead to problems with age, a fact that had not been expected so far. The treatment of ACHD has many special features and requires special expertise. Thereby, it is important that treatment regimens from acquired heart disease are not necessarily transmitted to CHD. While primary care physicians have the important and responsible task to set the course for adequate diagnosis and treatment early and to refer patients to appropriate care in specialized ACHD-facilities, they should actively encourage ACHD to pursue follow-up care in specialized facilities who can provide responsible and advanced advice. This medical update emphasizes the current data on epidemiology, heart failure and cardiac arrhythmia in ACHD.

PMID: 30740318 [PubMed]

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Improving medical care and prevention in adults with congenital heart disease-reflections on a global problem-part II: infective endocarditis, pulmonary hypertension, pulmonary arterial hypertension and aortopathy.

Cardiovasc Diagn Ther. 2018 Dec;8(6):716-724

Authors: Neidenbach R, Niwa K, Oto O, Oechslin E, Aboulhosn J, Celermajer D, Schelling J, Pieper L, Sanftenberg L, Oberhoffer R, de Haan F, Weyand M, Achenbach S, Schlensak C, Lossnitzer D, Nagdyman N, von Kodolitsch Y, Kallfelz HC, Pittrow D, Bauer UMM, Ewert P, Meinertz T, Kaemmerer H

Abstract
Despite relevant residua and sequels, follow-up care of adults with congenital heart disease (ACHD) is too often not performed by/in specialized and/or certified physicians or centers although major problems in the long-term course may develop. The most relevant encompass heart failure, cardiac arrhythmias, heart valve disorders, pulmonary vascular disease, infective endocarditis (IE), aortopathy and non-cardiac comorbidities. The present publication emphasizes current data on IE, pulmonary and pulmonary arterial hypertension and aortopathy in ACHD and underlines the deep need of an experienced follow-up care by specialized and/or certified physicians or centers, as treatment regimens from acquired heart disease can not be necessarily transmitted to CHD. Moreover, the need of primary and secondary medical prevention becomes increasingly important in order to reduce the burden of disease as well as the socioeconomic burden and costs in this particular patient group.

PMID: 30740319 [PubMed]

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Current management aspects in adult congenital heart disease: non-surgical closure of patent foramen ovale.

Cardiovasc Diagn Ther. 2018 Dec;8(6):739-753

Authors: Vaidya K, Khandkar C, Celermajer D

Abstract
A patent foramen ovale (PFO) is a remnant interatrial communication, best diagnosed with transoesophageal echocardiography (TOE) and bubble study. Although quite common and often asymptomatic, PFO is associated with cryptogenic stroke and migraine. Approximately one-half of patients with a cryptogenic stroke have a PFO, and the dilemma regarding whether or not to proceed with percutaneous device closure, to reduce the risk of future recurrent events due to paradoxical embolism, has been subject to debate for nearly two decades. Despite promising observational data, initial randomised clinical trials failed to demonstrate superiority of closure over medical therapy. However, long-term follow-up data from one of these early trials, combined with two new randomised trials, have provided more evidence for the benefits of closure in selected patients. This new evidence suggests that younger patients with high-risk features such as an atrial septal aneurysm (ASA) or large interatrial shunt are more likely to benefit from PFO closure, after fastidious exclusion of an alternative cause for the index stroke. However, issues which require further clarification include whether anticoagulant therapy is preferable to antiplatelet therapy for medical management, and which particular type of closure device is optimal. Finally, despite promising retrospective observational data suggesting improvement in migraine attacks after PFO closure, high quality evidence is lacking in this regard.

PMID: 30740321 [PubMed]

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Current spectrum, challenges and new developments in the surgical care of adults with congenital heart disease.

Cardiovasc Diagn Ther. 2018 Dec;8(6):754-764

Authors: Hörer J

Abstract
Today, more than two thirds of patients with congenital heart disease (CHD) are adults. Cardiac surgery plays an essential role in restoring and maintaining cardiac function, aside from evolving medical treatment and catheter-based interventions. The aim of the present publication was to describe the spectrum of operations performed on adults with CHD (ACHD) by reviewing current literature. Currently, surgery for ACHD is predominantly valve surgery, since valvular pathologies are often either a part of the basic heart defect or develop as sequelae of corrective or palliative surgery. Surgical techniques for valve repair, established in patients with acquired heart disease (non-ACHD), can often be transferred to ACHD. New valve substitutes may help to reduce the number of redo operations. Most of valve operations yield good results in terms of survival and quality of life, with the precondition that the ventricular function is preserved. Heart failure due to end-stage CHD is the most frequent cause of mortality in ACHD. However, surgical treatment by means of mechanical circulatory support (MCS) is still uncommon and the mortality exceeds the one following other operations in ACHD. Currently, different devices are used and new technical developments are in progress. However, there still is no ideal assist device available. Therefore, heart transplantation remains the only valid option for end-stage CHD. Despite higher early mortality following heart transplantation in ACHD compared to non-ACHD, the long-term survival compares favorably to non-ACHD. There is room for improvement by refining the indications, the time of listing, and the perioperative care of ACHD transplant patients. Sudden death is the second most frequent cause of mortality in ACHD. Ventricular tachycardia is the most frequent cause of sudden death followed by coronary artery anomaly. Due to the increasing awareness of physicians and the improved imaging techniques, coronary artery anomalies are coming more into the focus of cardiac surgeons. However, the reported experience is limited and it is currently difficult to provide a standardized and generally applicable recommendation for the indication and the adequate surgical technique. With the increasing age and complexity of ACHD, treatment of rhythm disturbances by surgical ablation, pacemaker or implantable cardioverter defibrillator (ICD) implantation and resynchronisation gains importance. A risk score specifically designed for surgery in ACHD is among the newest developments in predicting the outcome of surgical treatment of ACHD. This evidence-based score, derived from and validated with data from the Society of Thoracic Surgeons Congenital Heart Surgery Database, enables comparison of risk-adjusted performance of the whole spectrum of procedures performed in ACHD and helps in understanding the differences in surgical outcomes. The score is thus a powerful tool for quality control and quality improvement. In conclusion, new developments in surgery for ACHD are currently made with regard to valve surgery, which comprises more than half of all operations in ACHD and in treatment of end-stage CHD, which still yields high mortality and morbidity.

PMID: 30740322 [PubMed]

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The role of echocardiography in the management of adult patients with congenital heart disease following operative treatment.

Cardiovasc Diagn Ther. 2018 Dec;8(6):771-779

Authors: Havasi K, Ambrus N, Kalapos A, Forster T, Nemes A

Abstract
Treatment of congenital heart diseases has significantly advanced over the last few decades. Due to the continuously increasing survival rate, there are more and more adult patients with congenital heart diseases and these patients present at the adult cardiologist from the paediatric cardiology care. The aim of the present review is to demonstrate the role of echocardiography in some significant congenital heart diseases.

PMID: 30740324 [PubMed]

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Aortic valve repair in adult congenital heart disease.

Cardiovasc Diagn Ther. 2018 Dec;8(6):789-798

Authors: Girdauskas E, Petersen J, Sachweh J, Kozlik-Feldmann R, Sinning C, Rickers C, von Kodolitsch Y, Reichenspurner H

Abstract
Aortic valve repair in adult congenital heart disease (ACHD) went through a major development during the last two decades to become an increasingly established treatment option in experienced heart valve repair centers. This mini-review addresses valve-sparing treatment strategies in the two most common clinical entities of patients with adult congenital aortic valve disease, namely those presenting with bicuspid (BAV) and unicuspid (UAV) aortic valve disease. Both diseases are integral components of the continuum of congenital aortic valve diseases and represent one of the most common reasons of cardiovascular morbidity in young and otherwise healthy adult patients. The review will highlight the most important advantages of aortic valve sparing procedures as compared to the conventional valve replacement strategy. New treatment aspects will be reviewed including minimally-invasive surgical approaches for aortic valve repair as well as modern protocols of enhanced perioperative recovery which will potentially improve the perioperative recovery and quality of life of the patients undergoing valve-sparing surgical procedures in the future.

PMID: 30740326 [PubMed]

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Extra-cardiac comorbidities or complications in adults with congenital heart disease: a nationwide inpatient experience in the United States.

Cardiovasc Diagn Ther. 2018 Dec;8(6):814-819

Authors: Singh S, Desai R, Fong HK, Sadolikar A, Samani S, Goyal H

Abstract
With a great interest, we read the article by Neidenbach et al. on non-cardiac comorbidities in German adults with congenital heart disease (ACHD). ACHD always bear an increased risk of developing concomitant non-cardiac comorbidities and complications and impose a great healthcare burden. Limited large-scale data from the United States (US) on this focus incited us to write this brief report. Gilboa et al. estimated nearly 2.4 million people living with CHD (1.4 million adults, 1 million children) in the US in 2010. To have a better nationwide prospect of the current scenario, we looked at the extra-cardiac comorbidities among ACHD patients hospitalized in the US using the National Inpatient Sample database (NIS) for years 2013-2014. The burden of extracardiac comorbidities among the NIS cohort in the US was diverse as compared to the German outpatient ACHD cohort. Our study reports a higher burden of endocrinological, hematological, metabolic, pulmonary, psychiatric, renal and rheumatological comorbidities as compared to the German cohort. However, the burden of gastrointestinal and hepatological comorbidities was higher in the German outpatient cohort. In addition, ACHD patients with non-cardiac comorbidities were older except for those suffering from the psychiatric illnesses as compared to ACHD hospitalizations without comorbidities. It is imperative for the clinicians to understand the non-cardiac complications which a patient might encounter during a lifetime, and which could further complicate the management of ACHD and increases the risk of mortality.

PMID: 30740330 [PubMed]

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"Tailored" endovascular pulmonary valve and root replacement for rupture of a dilated homograft.

Cardiovasc Diagn Ther. 2018 Dec;8(6):820-824

Authors: Müller GC, Sinning C, Zengin-Sahm E, Stark V, Mir TS, Rickers C, Kozlik-Feldmann R, Kölbel T, Schäfer U

Abstract
Current balloon expandable and self-expanding valves have limitations for the treatment of the enlarged right ventricular outflow tract. We report the first use of a tailored Zenith graft in composition with an Edwards Sapien S3 valve as an alternative to high-risk surgery for the treatment of a spontaneously ruptured homograft in an adult congenital heart disease patient.

PMID: 30740331 [PubMed]

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Using speckle tracking echocardiography to assess fetal myocardial deformation: are we there yet?

Ultrasound Obstet Gynecol. 2019 Feb 10;:

Authors: Day TG, Charakida M, Simpson JM

Abstract
Speckle tracking echocardiography (STE) is now in widespread use in adult and paediatric cardiology practice. Interest is growing in the use of STE in the assessment of fetal cardiac function. There are potential advantages to the use of STE compared to other methods of myocardial functional assessment, being less affected by fetal and maternal movement, and potentially by angle of insonation. It has been shown that use of STE is feasible and reproducible in fetal life. However, there are currently several limitations and potential pitfalls, especially regarding frame-rate and spatial resolution, and these are explored in this review. Before STE can enter mainstream clinical use in fetal cardiology, there are some questions that require answering and this will require further research. The aim of this review is to present an overview of the techniques used to assess cardiac function, with an emphasis on STE, including a discussion of the current limitations of this technique. This article is protected by copyright. All rights reserved.

PMID: 30740804 [PubMed - as supplied by publisher]

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