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Improved Outcomes of Heart Transplantation in Adults With Congenital Heart Disease Receiving Regionalized Care.

J Am Coll Cardiol. 2019 Dec 10;74(23):2908-2918

Authors: Nguyen VP, Dolgner SJ, Dardas TF, Verrier ED, McMullan DM, Krieger EV

BACKGROUND: The number of adult congenital heart disease (CHD) patients undergoing heart transplantation is increasing rapidly. CHD patients have higher surgical risk at transplantation. High-volume adult CHD transplant centers may have better transplant outcomes.
OBJECTIVES: This study aimed to evaluate the effect of center CHD transplant volume and expertise on transplant outcomes in CHD patients.
METHODS: The authors studied heart transplantations in CHD patients age ≥18 years using the United Network of Organ Sharing (UNOS) database for the primary outcomes of waitlist mortality and post-transplant outcomes at 30 days and 1 year. Transplant centers were assessed by status as the highest CHD transplant volume center in a UNOS region versus all others, presence of Adult Congenital Heart Association accreditation, and adult versus pediatric hospital designation.
RESULTS: Between January of 2000 and June of 2018, 1,746 adult CHD patients were listed for transplant; 1,006 (57.6%) of these underwent heart transplantation. After adjusting for age, sex, listing status, and inotrope requirement, waitlist mortality risk was lower at Adult Congenital Heart Association accredited centers (hazard ratio: 0.730; p = 0.020). Post-transplant 30-day mortality was lower at the highest volume CHD transplant center in each UNOS region (hazard ratio: 0.706; p = 0.014).
CONCLUSIONS: Designated expertise in CHD care is associated with improved waitlist outcomes for CHD patients listed for transplantation. Post-transplant survival was improved at the highest volume regional center. These findings suggest a possible advantage of regionalization of CHD transplantation.

PMID: 31806135 [PubMed - in process]

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More Evidence for Regionalization.

J Am Coll Cardiol. 2019 Dec 10;74(23):2919-2920

Authors: Backer CL, Karamlou T, Welke KF

PMID: 31806136 [PubMed - in process]

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Retrosternal Clots After Fontan Surgery by Systematic Evaluation With Transthoracic Ultrasound.

J Cardiothorac Vasc Anesth. 2019 Nov 18;:

Authors: Cantinotti M, Giordano R, Marchese P, Franchi E, Viacava C, Pak V, Murzi B, Arcieri L, Poli V, Federici D, Koestenberger M, Assanta N

OBJECTIVES: Bleeding is a common, serious, and often subtle complication after total cavopulmonary connection surgery. The aim of the present study was to assess the incidence of retrosternal clots after surgery, which were searched for systematically with transthoracic ultrasound.
DESIGN: Retrospective study.
SETTING: Single center.
PARTICIPANTS: Total cavopulmonary surgeries were reviewed from January 2016 to May 2019.
INTERVENTIONS: Thoracic ultrasound with careful evaluation of the retrosternal area was performed at different postoperative times (12-36 hours, 5-7 days, and before discharge) as completion of routine echocardiography.
MEASUREMENTS AND MAIN RESULTS: Among 37 children undergoing total cavopulmonary connection (mean age 5.5 ± 1.8 years [range 2.4-11.7]; mean body surface area 0.7 ± 0.1 m2 [range 0.3-1.6 m2]), retrosternal clots were detected in 18 (48.6%). Of these, 7 (13.5%) had small clots (<1 cm), 2 (5.4%) small to moderate sized clots (>1 cm-<2 cm), 3 (8.1%) moderate sized clots (>2-<3 cm), and 6 (16.2%) large clots (>3 cm). Four of the 6 detected large clots required surgical revision, and in the other 2 patients, the clots were not treated because the patients' conditions were clinically stable. When 3 major groups (group 1-no or small clots, group gropu 2 are small to moderate or moderate, group 3-large clots) were evaluated, no significant differences were noted in age, body surface area, CPB time, conduit type, or the number of previous surgeries.
CONCLUSIONS: With thoracic ultrasound diagnosis, existence of retrosternal clots was found to be very common after total cavopulmonary connection. Most clots were small or moderate with no clinical effect; however, large clots that required redo surgery also were detected.

PMID: 31812566 [PubMed - as supplied by publisher]

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Corrigendum to "Management of Adults with Congenital Heart Disease and Pulmonary Arterial Hypertension in the UK: Survey of Current Practice, Unmet Needs and Expert Commentary" [Heart Lung Circ 2018; 27: 1018-1027].

Heart Lung Circ. 2019 Dec;28(12):1915

Authors: Tulloh R, Dimopoulos K, Condliffe R, Clift P, CHAMPION Steering Committee

PMID: 31813481 [PubMed - in process]

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The Future of Adult Congenital Heart Disease Research: Precision Health Services Delivery for the Next Decade.

Can J Cardiol. 2019 Dec;35(12):1609-1619

Authors: Marelli A

PMID: 31813496 [PubMed - in process]

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Misperception of Survival in Adult Congenital Heart Disease and Importance of Both Anatomic and Functional Indices: Educate Your Patients!

Can J Cardiol. 2019 Dec;35(12):1635-1639

Authors: Wichert-Schmitt B, Oechslin E

PMID: 31813500 [PubMed - in process]

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Transition and Transfer From Pediatric to Adult Congenital Heart Disease Care in Canada: Call For Strategic Implementation.

Can J Cardiol. 2019 Dec;35(12):1640-1651

Authors: Mackie AS, Fournier A, Swan L, Marelli AJ, Kovacs AH

Dramatic increases in survival to adulthood for persons born with congenital heart disease (CHD) have led rise to a corresponding need to provide age-appropriate and developmentally appropriate care across the lifespan. Health care transition is a multidimensional process that ideally begins in early adolescence in the pediatric setting and continues through young adulthood with input from both pediatric and adult CHD providers. Preparation for transition includes the fostering of adolescents' knowledge of their CHD and of self-management and self-advocacy skills needed for lifelong management of chronic disease. Transfer is the event in time when a patient's care and ownership of health records is taken over by the adult health care team; this is just one element of the broader transition process. Transfer typically occurs by age 18 throughout much of Canada. Successful transition is a shared responsibility, requiring engaged pediatric and adult providers and partnership with both young adults and their parents, all of whom may struggle with this process. An interdisciplinary approach to transition is recommended, given that health care transition is a complex process that occurs within the broader context of young adults' lives. This review summarizes existing evidence regarding transition and transfer, offers perspectives from multiple stakeholders, and proposes a transition curriculum of development of CHD education and self-management and self-advocacy skills. Specific recommendations to improve implementation of transition and transfer care within the Canadian context are provided. This review sheds light on the current capacity and challenges of adult CHD providers and proposes directions to move this field forward.

PMID: 31813501 [PubMed - in process]

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Eisenmenger Syndrome: A Multisystem Disorder-Do Not Destabilize the Balanced but Fragile Physiology.

Can J Cardiol. 2019 Dec;35(12):1664-1674

Authors: Chaix MA, Gatzoulis MA, Diller GP, Khairy P, Oechslin EN

Eisenmenger syndrome is the most severe and extreme phenotype of pulmonary arterial hypertension associated with congenital heart disease. A large nonrestrictive systemic left-to-right shunt triggers the development of pulmonary vascular disease, progressive pulmonary arterial hypertension, and increasing pulmonary vascular resistance at the systemic level, which ultimately results in shunt reversal. Herein, we review the changing epidemiological patterns and pathophysiology of Eisenmenger syndrome. Multiorgan disease is an integral manifestation of Eisenmenger syndrome and includes involvement of the cardiac, hematological, neurological, respiratory, gastrointestinal, urinary, immunological, musculoskeletal, and endocrinological systems. Standardized practical guidelines for the assessment, management, risk stratification, and follow-up of this very fragile and vulnerable population are discussed. Multidisciplinary care is the best clinical practice. An approach to the prevention and management of a broad spectrum of complications is provided. Relevant therapeutic questions are discussed, including anticoagulation, noncardiac surgery, physical activity, transplantation, and advanced-care planning (palliative care). Advanced pulmonary arterial hypertension therapies are indicated in patients with Eisenmenger syndrome and World Health Organization functional class II or higher symptoms to improve functional capacity, quality of life, and-less well documented-survival. Specific recommendations regarding monotherapy or combination therapy are provided according to functional class and clinical response. The ultimate challenge for all care providers remains early detection and management of intracardiac and extracardiac shunts, considering that Eisenmenger syndrome is a preventable condition.

PMID: 31813503 [PubMed - in process]

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Technological Advances in Arrhythmia Management Applied to Adults With Congenital Heart Disease.

Can J Cardiol. 2019 Dec;35(12):1708-1722

Authors: Mondésert B, Nair K, McLeod CJ, Khairy P

Arrhythmias are a leading cause of morbidity in adults with congenital heart disease. Numerous challenges to managing arrhythmias include vascular access issues, intracardiac shunts, unconventional locations of the conduction system, and anatomical complexities. Technological advances are improving our ability to diagnose and treat arrhythmias. Implantable loop recorders and various technologies developed for consumers to record electrocardiographic tracings using smartphone applications, watches, and dedicated portable devices are expanding our armamentarium of diagnostic tools. Remote magnetic-guided catheter ablation is enhancing access to otherwise difficult to reach arrhythmia substrates. Cryoablation allows perinodal arrhythmias to be safely treated in patients with displaced or difficult to predict locations of the atrioventricular conduction system. Interventions that minimize radiation exposure to near 0 levels are gaining interest and pulmonary vein isolation procedures to treat atrial fibrillation are increasingly performed. Cardiac implantable electronic devices compatible with magnetic resonance imaging have become the norm. Subcutaneous defibrillators and leadless pacemakers are providing effective solutions to patients in whom transvenous leads are contraindicated or not desired. His-bundle pacing is emerging as a viable option to provide more physiological pacing. Progressive advances in multicomponent communicating leadless devices carry the promise of providing leadless dual chamber pacing and cardiac resynchronization therapy in the near future. The safety of transvenous lead extraction procedures is likely to improve with advances such as the low-pressure balloon to manage superior vena cava lacerations. Awareness of these developments and referral to sites with dedicated expertise could contribute to further improving outcomes in adults with congenital heart disease.

PMID: 31813504 [PubMed - in process]

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Heart Failure in Adult Congenital Heart Disease: From Advanced Therapies to End-of-Life Care.

Can J Cardiol. 2019 Dec;35(12):1723-1739

Authors: Crossland DS, Van De Bruaene A, Silversides CK, Hickey EJ, Roche SL

There is mounting recognition that some of the most urgent problems of adult congenital heart disease (ACHD) are the prevention, diagnosis, and management of heart failure (HF). Recent expert consensus and position statements not only emphasize a specific and pressing need to tackle HF in ACHD (ACHD-HF) but also highlight the difficulty of doing so given a current sparsity of data. Some of the challenges will be addressed by this review. The authors are from 3 different centres; each centre has an established subspeciality ACHD-HF clinic and is able to provide heart transplant, multiorgan transplant, and mechanical support for patients with ACHD. Appropriate care of this complex population requires multidisciplinary ACHD-HF teams evaluate all possible treatment options. The risks and benefits of nontransplant ACHD surgery, percutaneous structural and electrophysiological intervention, and ongoing conservative management must be considered alongside those of transplant strategies. In our approach, advanced care planning and palliative care coexist with the consideration of advanced therapies. An ethos of shared decision making, guided by the patient's values and preferences, strengthens clinical care, but requires investment of time as well as skilled communication. In this review, we aim to offer practical real-world advice for managing these patients, supported by scientific data where it exists.

PMID: 31813505 [PubMed - in process]