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Variations in Practice Patterns and Consistency With Published Guidelines for Balloon Aortic and Pulmonary Valvuloplasty: An Analysis of Data From the IMPACT Registry.

JACC Cardiovasc Interv. 2018 Mar 26;11(6):529-538

Authors: Glatz AC, Kennedy KF, Rome JJ, O'Byrne ML

OBJECTIVES: The authors sought to study variation in the practice of balloon aortic (BAV) and pulmonary valvuloplasty (BPV).
BACKGROUND: The IMPACT (IMProving Adult and Congenital Treatment) registry provides an opportunity to study practice variation in transcatheter interventions for congenital heart disease.
METHODS: The authors studied BAV and BPV in the IMPACT registry from January 1, 2011, to September 30, 2015, using hierarchical multivariable models to measure hospital-level variation in: 1) the distribution of indications for intervention; and 2) in cases with "high resting gradient" as the indication, consistency with published guidelines.
RESULTS: A total of 1,071 BAV cases at 60 hospitals and 2,207 BPV cases at 75 hospitals were included. The indication for BAV was high resting gradient in 82%, abnormal stress test or electrocardiogram (2%), left ventricular dysfunction (11%), and symptoms (5%). Indications for BPV were high resting gradient in 82%, right-left shunt (6%), right ventricular dysfunction (7%), and symptoms (5%). No association between hospital characteristics and distribution of indications was demonstrated. Among interventions performed for "high resting gradient," there was significant adjusted hospital-level variation in the rates of cases performed consistently with guidelines. For BAV, significant differences were seen across census regions, with hospitals in the East and South more likely to practice consistently than those in the Midwest and West (p = 0.005). For BPV, no association was found between hospital factors and rates of consistent practice, but there was significant interhospital variation (median rate ratio: 1.4; 95% confidence interval: 1.2 to 1.6; p < 0.001).
CONCLUSIONS: There is measurable hospital-level variation in the practice of BAV and BPV. Further research is necessary to determine whether this affects outcomes or resource use.

PMID: 29566797 [PubMed - in process]

Branch Pulmonary Artery Valve Implantation Reduces Pulmonary Regurgitation and Improves Right Ventricular Size/Function in Patients With Large Right Ventricular Outflow Tracts.

JACC Cardiovasc Interv. 2018 Mar 26;11(6):541-550

Authors: Qureshi AM, Bansal N, McElhinney DB, Boudjemline Y, Forbes TJ, Maschietto N, Shahanavaz S, Cheatham JP, Krasuski R, Lamers L, Chessa M, Morray BH, Goldstein BH, Noel CV, Wang Y, Gillespie MJ

OBJECTIVES: The authors sought to assess the intermediate-term effects of percutaneous placed valves in the branch pulmonary artery (PA) position.
BACKGROUND: Most patients with large right ventricular outflow tracts (RVOTs) are excluded from available percutaneous pulmonary valve options. In some of these patients, percutaneous branch PA valve implantation may be feasible. The longer-term effects of valves in the branch PA position is unknown.
METHODS: Retrospective data were collected on patients with significant pulmonary regurgitation who had a percutaneous branch PA valve attempted.
RESULTS: Percutaneous branch PA valve implantation was attempted in 34 patients (18 bilateral and 16 unilateral). One-half of the patients were in New York Heart Association (NHYA) functional class III or IV pre-implantation. There were 2 failed attempts and 6 procedural complications. At follow-up, only 1 patient had more than mild valvar regurgitation. The right ventricular end-diastolic volume index decreased from 147 (range: 103 to 478) ml/m2 to 101 (range: 76 to 429) ml/m2, p < 0.01 (n = 16), and the right ventricular end-systolic volume index decreased from 88.5 (range: 41 to 387) ml/m2 to 55.5 (range: 40.2 to 347) ml/m2, p < 0.01 (n = 13). There were 5 late deaths. At a median follow-up of 2 years, all other patients were in NYHA functional class I or II.
CONCLUSIONS: Percutaneous branch PA valve implantation results in a reduction in right ventricular volume with clinical benefit in the intermediate term. Until percutaneous valve technology for large RVOTs is refined and more widely available, branch PA valve implantation remains an option for select patients.

PMID: 29566799 [PubMed - in process]

Transcatheter Pulmonary Valve Replacement With the Melody Valve in Small Diameter Expandable Right Ventricular Outflow Tract Conduits.

JACC Cardiovasc Interv. 2018 Mar 26;11(6):554-564

Authors: Shahanavaz S, Qureshi AM, Levi DS, Boudjemline Y, Peng LF, Martin MH, Bauser-Heaton H, Keeshan B, Asnes JD, Jones TK, Justino H, Aboulhosn JA, Gray RG, Nguyen H, Balzer DT, McElhinney DB

OBJECTIVES: This study sought to evaluate the safety, feasibility, and outcomes of transcatheter pulmonary valve replacement (TPVR) in conduits ≤16 mm in diameter.
BACKGROUND: The Melody valve (Medtronic, Minneapolis, Minnesota) is approved for the treatment of dysfunctional right ventricular outflow tract (RVOT) conduits ≥16 mm in diameter at the time of implant. Limited data are available regarding the use of this device in smaller conduits.
METHODS: The study retrospectively evaluated patients from 9 centers who underwent percutaneous TPVR into a conduit that was ≤16 mm in diameter at the time of implant, and reported procedural characteristics and outcomes.
RESULTS: A total of 140 patients were included and 117 patients (78%; median age and weight 11 years of age and 35 kg, respectively) underwent successful TPVR. The median original conduit diameter was 15 (range: 9 to 16) mm, and the median narrowest conduit diameter was 11 (range: 4 to 23) mm. Conduits were enlarged to a median diameter of 19 mm (29% larger than the implanted diameter), with no difference between conduits. There was significant hemodynamic improvement post-implant, with a residual peak RVOT pressure gradient of 7 mm Hg (p < 0.001) and no significant pulmonary regurgitation. During a median follow-up of 2.0 years, freedom from RVOT reintervention was 97% and 89% at 2 and 4 years, respectively, and there were no deaths and 5 cases of endocarditis (incidence rate 2.0% per patient-year).
CONCLUSIONS: In this preliminary experience, TPVR with the Melody valve into expandable small diameter conduits was feasible and safe, with favorable early and long-term procedural and hemodynamic outcomes.

PMID: 29566801 [PubMed - in process]

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Innominate artery aneurysm, how to solve it?

J Int Med Res. 2017 Jun;45(3):1279-1284

Authors: Wang XL, Guan XL, Jiang WJ, Liu O, Zhang HJ

We herein describe our *These authors contributed equally to this work. experience with a congenital innominate artery aneurysm (IAA) that was managed with a simple surgical procedure. A 44-year-old woman was admitted for chest distress. Computed tomography angiography showed a 3.6-cm IAA arising from the aortic arch and compressing the trachea. A median sternotomy was performed with the patient under general anesthesia, and the IAA was found to involve the origin of the innominate artery and the bifurcation of the right subclavian artery and common carotid artery; however, the aorta was intact. An 8-mm Dacron graft was anastomosed to the ascending aorta and distal end of the IAA without cardiopulmonary bypass. The postoperative course was uneventful, and repeat computed tomography angiography revealed no evidence of recurrence 6 months postoperatively. We also herein present a literature review of this rare clinical condition.

PMID: 28553761 [PubMed - indexed for MEDLINE]

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"Not such a kid thing anymore": Young adults' perspectives on transfer from paediatric to adult cardiology care.

Child Care Health Dev. 2018 Mar 25;:

Authors: Catena G, Rempel GR, Kovacs AH, Rankin KN, Muhll IV, Mackie AS

BACKGROUND: Transfer of adolescents with congenital heart disease from paediatric cardiology providers to specialized adult congenital heart disease (ACHD) care providers is becoming a standard practice. However, some paediatric cardiologists continue to provide care for their patients into adult life. Little is known about the perspectives of young adult patients who have been transferred to ACHD clinics versus those who continue to receive their cardiology care in paediatric settings.
METHODS: Content and thematic analysis of structured telephone interviews with 21 young adults age 18-25 (13 transferred to ACHD clinic and 8 who had not transferred) was conducted to identify similarities and differences in patient characteristics of those in ACHD versus paediatric settings.
RESULTS: There were no appreciable differences in gender, age, heart disease type, and independence between those transferred to ACHD care versus those not transferred. Participants in both groups were aware of differences between the paediatric and ACHD care settings and providers, with some favouring the familiarity offered by the paediatric setting and providers. Participants had varying views on parental involvement in their care; most of them had attended clinic appointments on their own. Those who had transferred to ACHD care acknowledged that it would take time to adjust to new relationships. Positive perspectives on actual or anticipated transfer to ACHD care included a growing sense of autonomy and responsibility, as well as access to reproductive information relevant to ACHD patients.
CONCLUSIONS: The absence of patient characteristics distinguishing those in ACHD care versus those still followed in paediatric care suggests that system, provider, and parent factors, rather than patient factors, may account for patients' perspectives on transfer to ACHD care.

PMID: 29574895 [PubMed - as supplied by publisher]

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Novel oral anticoagulant use in adult Fontan patients: A single center experience.

Congenit Heart Dis. 2018 Mar 24;:

Authors: Georgekutty J, Kazerouninia A, Wang Y, Ermis PR, Parekh DR, Franklin WJ, Lam WW

OBJECTIVE: Adult Fontan patients are at increased risk for thrombosis and thromboembolic complications leading to increased morbidity and mortality. Most are prescribed antiplatelet or anticoagulant therapy for thromboprophylaxis; novel oral anticoagulants (NOACs) are uncommonly used given lack of data on their use in this population and generalized concerns regarding Fontan patients' abnormal coagulation. We report the largest single-center experience with the use of NOACs for treatment and prophylaxis of thrombosis and thromboembolism in adult Fontan patients.
RESULTS: A retrospective chart review identified 21 patients (11 female, 10 male), median age 33 years (18-50) at first initiation, who were prescribed a NOAC on 27 different occasions. The main indications for anticoagulation were arrhythmia (N = 12), thrombosis (N = 8), and persistent right to left shunts (N = 2); one patient was initially on anticoagulation for arrhythmia but restarted for thrombosis. The most common indications for initiation of a NOAC over warfarin were patient/provider preference (N = 11), labile international normalized ratio (INR) (N = 5), initiation of therapy elsewhere (N = 3), and history of poor clinical follow-up (N = 2). Over a cumulative 316 months of patient therapy, one new thrombotic event was noted. No major or nonmajor bleeding events occurred, and 10 patients experienced minor bleeding that did not require the cessation of therapy. One patient died from multiorgan system failure following an unwitnessed, out of hospital arrest. At present, 10 patients remain on NOAC therapy in the setting of ongoing arrhythmia (N = 4), history of stroke (N = 2), history of pulmonary embolism (N = 2), history of deep vein thrombosis (N = 1), and history of right ventricle thrombus (N = 1).
CONCLUSIONS: While our study is limited by size, our results suggest that NOACs may be a non-inferior alternative to traditional anticoagulation and that further study is warranted.

PMID: 29575675 [PubMed - as supplied by publisher]

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Echocardiographic and clinical markers of left ventricular ejection fraction and moderate or greater systolic dysfunction in left ventricular noncompaction cardiomyopathy.

Echocardiography. 2018 Mar 25;:

Authors: Arenas IA, Mihos CG, DeFaria Yeh D, Yucel E, Elmahdy HM, Santana O

BACKGROUND: Left ventricular noncompaction (LVNC) is associated with progressive LV systolic dysfunction and dilated cardiomyopathy. We aimed to investigate the echocardiographic and clinical characteristics associated with LV ejection fraction (LVEF) and moderate or greater systolic dysfunction in patients with LVNC.
METHODS: Our institutional echocardiography database was retrospectively reviewed between 2008 and 2014, and 62 patients with LVNC were identified. Forty-three (69%) had moderate or greater LV systolic dysfunction (LVEF ≤ 40%) and were compared with 19 (31%) patients with preserved or mildly reduced LVEF (>40%). Linear regression analyses were utilized to identify markers associated with LVEF.
RESULTS: The mean age was 63 ± 17 years and noncompacted-to-compacted ratio was 2.3 ± 0.5, and was larger in patients with LVEF ≤ 40% (2.4 vs 2.1; P = .02). Patients with LVEF ≤ 40% were older, had more congestive heart failure, significant QRS interval prolongation, and greater LV remodeling and worse mean global longitudinal strain (GLS). Multivariate regression analysis revealed increased age (standardized regression coefficient (β) = -0.17; P = .04) and QRS duration (β = -0.13; P = .08), congestive heart failure (β = -0.18; P = .04), and worsened GLS (β = -0.40; P = .001) were independently associated with decreased LVEF in the cohort (overall model fit R2  = 0.71; P < .0001). Increased age (β = -0.49; P = .01) and QRS duration (β = -0.50; P = .002), and worsened GLS (β = -0.33; P = .04), were also associated with a lower LVEF in patients with LVEF > 40%.
CONCLUSIONS: The independent markers associated with LVEF and moderate or greater LV systolic dysfunction in patients with LVNC, in particular GLS and QRS duration, may detect high-risk candidates for more aggressive clinical surveillance and medical therapy.

PMID: 29577407 [PubMed - as supplied by publisher]

Arrhythmias in congenital heart disease: a position paper of the European Heart Rhythm Association (EHRA), Association for European Paediatric and Congenital Cardiology (AEPC), and the European Society of Cardiology (ESC) Working Group on Grown-up Congenital heart disease, endorsed by HRS, PACES, APHRS, and SOLAECE.

Europace. 2018 Mar 20;:

Authors: Hernández-Madrid A, Paul T, Abrams D, Aziz PF, Blom NA, Chen J, Chessa M, Combes N, Dagres N, Diller G, Ernst S, Giamberti A, Hebe J, Janousek J, Kriebel T, Moltedo J, Moreno J, Peinado R, Pison L, Rosenthal E, Skinner JR, Zeppenfeld K, ESC Scientific Document Group

The population of patients with congenital heart disease (CHD) is continuously increasing with more and more patients reaching adulthood. A significant portion of these young adults will suffer from arrhythmias due to the underlying congenital heart defect itself or as a sequela of interventional or surgical treatment. The medical community will encounter an increasing challenge as even most of the individuals with complex congenital heart defects nowadays become young adults. Within the past 20 years, management of patients with arrhythmias has gained remarkable progress including pharmacological treatment, catheter ablation, and device therapy. Catheter ablation in patients with CHD has paralleled the advances of this technology in pediatric and adult patients with structurally normal hearts. Growing experience and introduction of new techniques like the 3D mapping systems into clinical practice have been particularly beneficial for this growing population of patients with abnormal cardiac anatomy and physiology. Finally, device therapies allowing maintanence of chronotropic competence and AV conduction, improving haemodynamics by cardiac resynchronization, and preventing sudden death are increasingly used. For pharmacological therapy, ablation procedures, and device therapy decision making requires a deep understanding of the individual pathological anatomy and physiology as well as detailed knowledge on natural history and long-term prognosis of our patients. Composing expert opinions from cardiology and paediatric cardiology as well as from non-invasive and invasive electrophysiology this position paper was designed to state the art in management of young individuals with congenital heart defects and arrhythmias.

PMID: 29579186 [PubMed - as supplied by publisher]

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Egypt: coronary and structural heart interventions from 2010 to 2015.

EuroIntervention. 2017 May 15;13(Z):Z21-Z24

Authors: Magdy A, Shawky A, Mohanad A, Shaheen S

Interventional cardiology procedures are constantly increasing in numbers and in quality, especially in developing countries such as Egypt. The numbers and types of procedure now available have driven development in the field and in its accompanying services. The aim of this short report is to present a review of the development of interventional cardiology in Egypt during the period 2010-2015 and the demographic, economic and educational factors that have affected this process. We collected and analysed data provided by different centres and from the distributors of intervention tools for the years 2010 to 2015. Analysis of these data showed a steady growth of primary PCI, amounting to a threefold increase over the six-year period. There are increasing numbers of PCI-capable centres, especially in Cairo. Almost 55,000 PCIs are performed yearly in Egypt utilising around 100,000 stents; the percentage of drug-eluting stents (DES) used has increased to 65-70% (90% in private and insured patients). Structural intervention is growing fast for congenital heart disease, and balloon mitral valvuloplasty has become the default strategy. The numbers of TAVI and EVAR are also increasing, although cost remains the greatest challenge. These changes can be attributed to the increasing numbers of ischaemic heart disease patients over the last 25 years, involving improved education and awareness, patients presenting at a younger age, and improved practice in intervention. In Egypt, there has been a steady growth in PCI and intervention tools which has been faster in coronary and congenital heart disease than in structural heart disease during the period from 2010 to 2015.

PMID: 28504225 [PubMed - indexed for MEDLINE]

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[Progressive pulmonary hypertension in a patient with type 1 Gaucher disease].

Ter Arkh. 2017;89(10):71-74

Authors: Ponomarev RV, Model SV, Averbukh OM, Gavrilov AM, Galstyan GM, Lukina EA

Gaucher disease is the most common form of hereditary enzymopathies combined into a group of lysosomal storage diseases. The basis for the disease is a hereditary deficiency of the activity of acid β-glucosidase, a lysosomal enzyme involved in the catabolism of lipids, which results in the accumulation of nonutilized cellular metabolism products in the macrophage lysosomes. The main clinical manifestations of type 1 Gaucher disease are cytopenia, hepatomegaly, and splenomegaly, and bone lesion. One of the atypical clinical manifestations of Gaucher disease is damage to the lungs with the development of pulmonary hypertension, which is usually considered within the underlying disease - the development of pneumosclerosis due to macrophage dysfunction. The paper describes a case of progressive pulmonary hypertension in a patient with type 1 Gaucher disease.

PMID: 29171474 [PubMed - indexed for MEDLINE]