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Management of Cardiac Involvement Associated With Neuromuscular Diseases: A Scientific Statement From the American Heart Association.

Circulation. 2017 Sep 26;136(13):e200-e231

Authors: Feingold B, Mahle WT, Auerbach S, Clemens P, Domenighetti AA, Jefferies JL, Judge DP, Lal AK, Markham LW, Parks WJ, Tsuda T, Wang PJ, Yoo SJ, American Heart Association Pediatric Heart Failure Committee of the Council on Cardiovascular Disease in the Young; Council on Clinical Cardiology; Council on Cardiovascular Radiology and Intervention; Council on Functional Genomics and Translational Biology; and Stroke Council

For many neuromuscular diseases (NMDs), cardiac disease represents a major cause of morbidity and mortality. The management of cardiac disease in NMDs is made challenging by the broad clinical heterogeneity that exists among many NMDs and by limited knowledge about disease-specific cardiovascular pathogenesis and course-modifying interventions. The overlay of compromise in peripheral muscle function and other organ systems, such as the lungs, also makes the simple application of endorsed adult or pediatric heart failure guidelines to the NMD population problematic. In this statement, we provide background on several NMDs in which there is cardiac involvement, highlighting unique features of NMD-associated myocardial disease that require clinicians to tailor their approach to prevention and treatment of heart failure. Undoubtedly, further investigations are required to best inform future guidelines on NMD-specific cardiovascular health risks, treatments, and outcomes.

PMID: 28838934 [PubMed - indexed for MEDLINE]

Isolated aortic root dilation in homocystinuria.

J Inherit Metab Dis. 2017 Oct 04;:

Authors: Lorenzini M, Guha N, Davison JE, Pitcher A, Pandya B, Kemp H, Lachmann R, Elliott PM, Murphy E

BACKGROUND: Vascular complications in homocystinuria have been known for many years, but there have been no reports to date on involvement of the ascending aorta.
METHODS: We conducted a cross-sectional study of patients with homocystinuria, known to a single metabolic centre, and evaluated in 2016 with a transthoracic echocardiogram. Aortic root dilation was defined as Z-score ≥ 2.0 SD, and graded mild (Z-score 2.0-3.0), moderate (Z-score 3.01-4.0) and severe (Z-score > 4.0).
RESULTS: The study population included 34 patients, median age of 44.3 years (IQR 33.3-52.2), 50% males, 69% diagnosed aged <18 years and 29% pyridoxine-responsive. Eight (24%) had a history of hypertension. Seven patients (21%) were found to have a dilation of the aortic root, mild in two cases (6%), moderate in four (12%) and severe in one (3%). None had dilation of the ascending aorta. Significant aortic regurgitation, secondary to moderate aortic root dilation, was documented in two patients. A single patient had significant mitral regurgitation due to prolapse of both valve leaflets, as well as mild aortic root dilation. Comparing patients with a dilation of the aortic root to those without, there were no significant clinical, laboratory or echocardiographic differences, with the only exception being that the diameter of the ascending aorta was larger in the group with a dilated aortic root, albeit within normal limits.
CONCLUSIONS: A subset of patients with homocystinuria have isolated dilation of the aortic root similar to that observed in Marfan syndrome.

PMID: 28980096 [PubMed - as supplied by publisher]

Do not forget the parents-Parents' concerns during transition to adult care for adolescents with congenital heart disease.

Child Care Health Dev. 2017 Oct 05;:

Authors: Bratt EL, Burström Å, Hanseus K, Rydberg A, Berghammer M, On behalf on the STEPSTONES-CHD consortium

BACKGROUND: Growing up with congenital heart disease (CHD) often means transfer to adult care and lifelong medical follow-up. An optimal transition process usually involves a multipart collaboration between the patient, their parents and other family members, and the healthcare providers. Taking an active role while knowing when it is time to step aside can be difficult for all the concerned parties, even the healthcare professionals. The aim of the present study therefore, was to explore parents' expectations and needs during their adolescent's transition to adult care.
METHOD: Semi-structured interviews were conducted with 18 parents of 16 adolescents (aged 13-18 years) with CHD in 4 pediatric cardiology settings in Sweden. The interviews were analysed with qualitative content analysis.
RESULTS: The analysis resulted in 2 main themes: (a) Feeling secure-the importance of being prepared and informed. This theme focused on the need to be prepared and informed about transition and future transfer to adult care. (b) Recognizing when to hand over at the right time. This theme addressed the process of handing over the responsibility from the parent to the adolescents and contained handing over from pediatric care to adult care.
CONCLUSION: Being prepared and informed about the upcoming transition process was essential. The parents underlined the importance of being involved in the transition planning for gradually handing over responsibility to the adolescent. They also considered establishing contact with the adult healthcare team before transfer as important and needed to be assured that CHD-related information of importance for the young person's daily life would be given.

PMID: 28980341 [PubMed - as supplied by publisher]

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Incidence and predictors of obstetric and fetal complications in women with structural heart disease.

Heart. 2017 Oct;103(20):1610-1618

Authors: van Hagen IM, Roos-Hesselink JW, Donvito V, Liptai C, Morissens M, Murphy DJ, Galian L, Bazargani NM, Cornette J, Hall R, Johnson MR

OBJECTIVE: Women with cardiac disease becoming pregnant have an increased risk of obstetric and fetal events. The aim of this study was to study the incidence of events, to validate the modified WHO (mWHO) risk classification and to search for event-specific predictors.
METHODS: The Registry Of Pregnancy And Cardiac disease is a worldwide ongoing prospective registry that has enrolled 2742 pregnancies in women with known cardiac disease (mainly congenital and valvular disease) before pregnancy, from January 2008 up to April 2014.
RESULTS: Mean age was 28.2±5.5 years, 45% were nulliparous and 33.3% came from emerging countries. Obstetric events occurred in 231 pregnancies (8.4%). Fetal events occurred in 651 pregnancies (23.7%). The mWHO classification performed poorly in predicting obstetric (c-statistic=0.601) and fetal events (c-statistic=0.561). In multivariable analysis, aortic valve disease was associated with pre-eclampsia (OR=2.6, 95%CI=1.3 to 5.5). Congenital heart disease (CHD) was associated with spontaneous preterm birth (OR=1.8, 95%CI=1.2 to 2.7). Complex CHD was associated with small-for-gestational-age neonates (OR=2.3, 95%CI=1.5 to 3.5). Multiple gestation was the strongest predictor of fetal events: fetal/neonatal death (OR=6.4, 95%CI=2.5 to 16), spontaneous preterm birth (OR=5.3, 95%CI=2.5 to 11) and small-for-gestational age (OR=5.0, 95%CI=2.5 to 9.8).
CONCLUSION: The mWHO classification is not suitable for prediction of obstetric and fetal events in women with cardiac disease. Maternal complex CHD was independently associated with fetal growth restriction and aortic valve disease with pre-eclampsia, potentially offering an insight into the pathophysiology of these pregnancy complications. The increased rates of adverse obstetric and fetal outcomes in women with pre-existing heart disease should be highlighted during counselling.

PMID: 28377476 [PubMed - indexed for MEDLINE]

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Cardiovascular disease in patients with inflammatory bowel disease: An issue in no guidelines land.

Int J Cardiol. 2016 Nov 01;222:984-5

Authors: Zuin M, Rigatelli G, Del Favero G, Andreotti AN, Picariello C, Zuliani G, Carraro M, Galasso MP, Roncon L

PMID: 27526375 [PubMed - indexed for MEDLINE]

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Rare congenital mitral valve malformations assessed by real-time three-dimensional echocardiography.

Int J Cardiol. 2016 Nov 01;222:1027-30

Authors: Sun F, Chen Y, Huang L, Ren W, Yu X, Ni C

PMID: 27533917 [PubMed - indexed for MEDLINE]

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Could we consider diverticular disease as a non-traditional risk factor for coronary artery disease?

Int J Cardiol. 2016 Nov 15;223:649-650

Authors: Zuin M, Rigatelli G, Andreotti AN, Fogato L, Scaranello F, Zuliani G, Faggian G, Roncon L

PMID: 27567232 [PubMed - indexed for MEDLINE]

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Association of cardiac cachexia and atrial fibrillation in heart failure patients.

Int J Cardiol. 2016 Nov 15;223:863-866

Authors: Arámbula-Garza E, Castillo-Martínez L, González-Islas D, Orea-Tejeda A, Santellano-Juárez B, Keirns-Davies C, Peláez-Hernández V, Sánchez-Santillán R, Pineda-Juárez J, Cintora-Martínez C, Pablo-Santiago R

BACKGROUND: Cachexia is a common complication in patients with advanced heart failure (HF) associated with inflammatory response activation. Atrial fibrillation (AF) is the most frequent arrhythmia (26%), probably both exacerbate the cardiac cachexia (CC).
OBJECTIVES: Evaluate the association of cardiac cachexia and atrial fibrillation in heart failure patients.
MATERIAL AND METHODS: In a case control study, CC was diagnosed by electrical bioimpedance with vectorial analysis (BIVA). Subjects with congenital heart disease, cancer, HIV, drug use and other causes than HF were excluded.
RESULTS: Of the 359 subjects analyzed (men: 52.9%) median age 65years (55-74). Those with CC were older [72 (61-67)] vs. without [62 (52-70) years old, p<0.01]. During follow-up 47.8% of subjects developed CC and 17.27% AF, this was significantly more frequent in cachectic patients CC (23% vs 12.11%, OR: 2.17, 95% CI: 1.19-4.01, p=0.006). Subjects, with AF had lower left ventricular ejection fraction (25.49±12.96 vs. 32.01±15.02, p=0.08), lower posterior wall thickness (10.03±2.12 vs. 11.00±2.47, p=0.007), larger diameter of the left atrium (49.87±9.84 vs. 42.66±7.56, p<0.001), and a higher prevalence of CC (85.42% vs. 69.77%, p=0.028). The 50.58% of was in NYHA class I. In NYHA III, 22.95% were in AF vs. 12.10% with not AF (p=0.027).
CONCLUSION: The frequent coexistence of CC and AF as HF complications indicate greater severity of HF, regardless of its type of HF.

PMID: 27580222 [PubMed - indexed for MEDLINE]

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Systemic right ventricular morphology in the early postoperative course after extracardiac Fontan operation: is there still a need for special care?

Eur J Cardiothorac Surg. 2017 03 01;51(3):483-489

Authors: Nordmeyer S, Rohder M, Nordmeyer J, Miera O, Peters B, Cho MY, Photiadis J, Berger F, Ovroutski S

Objectives: We aimed to compare early postoperative outcome after extracardiac (EC) Fontan operation between patients with right (RV) or left (LV) systemic ventricles.
Methods: In total, 173 consecutive patients (median age 4 years, median weight 14 kg) underwent EC Fontan between 1995 and 2013. Pre- and intraoperative data as well as detailed postoperative haemodynamic variables were compared between patients with LV [ n  = 109 (63%)] and RV [ n  = 64 (37%)].
Results: : RV patients showed significantly lower mean arterial (median 55 vs 59 mmHg, P  = 0.04), higher atrial (median 8 vs 6 mmHg, P  = 0.03) and comparable pulmonary pressure (median 14 vs 14 mmHg, P  = 0.7) as well as lower mean systemic perfusion pressure (median 39 vs 43 mmHg, P  = 0.03) on Day 0 after EC Fontan. They suffered from longer intubation time (median 18 vs 12 h, P  = 0.008), higher incidence of ascites (46% vs 28%, P  = 0.04) and need for dialysis (21% vs 4%, P  = 0.003). Prolonged inotropic support (25% vs 8%, P  = 0.02) and pharmacological treatment to reduce pulmonary vascular resistance (71% vs 53%, P  = 0.002) were more often used in RV patients and they showed more often supraventricular tachyarrhythmia (27% vs 5%, P  < 0.001) and a longer intensive care unit-stay (median 4 vs 3 days, P  = 0.03). However, early mortality, need for Fontan takedown, use of mechanical circulatory support, pleural effusions and hospital stay were not significantly different between both groups.
Conclusions: Patients with systemic RV demonstrate higher morbidity in the early postoperative course compared with patients with systemic LV anatomy and require intensified postoperative management to avoid postoperative Fontan failure.

PMID: 28007868 [PubMed - indexed for MEDLINE]

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Perventricular device closure of perimembranous ventricular septal defect: effectiveness of symmetric and asymmetric occluders.

Eur J Cardiothorac Surg. 2017 03 01;51(3):478-482

Authors: Ou-Yang WB, Wang SZ, Hu SS, Zhang FW, Zhang DW, Liu Y, Meng H, Pang KJ, Meng LK, Pan XB

Objectives: To assess safety and effectiveness of symmetric and asymmetric occluders in perventricular device closure without cardiopulmonary bypass of perimembranous ventricular septal defects (pmVSDs).
Methods: The present retrospective study enrolled 581 patients who underwent perventricular device closure of pmVSDs under transoesophageal echocardiography guidance from May 2011 to April 2016, and outpatient electrocardiography and transthoracic echocardiography assessments at 1, 3, 6 and 12 months, and yearly thereafter.
Results: The overall success rate of device implantation was 92.6% (43 surgical conversions immediately). Between patients receiving symmetric ( n  = 353) and asymmetric ( n  = 185) occluders, there were no significant differences in age, weight and defect diameter distributions; however, both before discharge and at mean 28.6 ± 21.2 (range, 1-60)-month follow-up, the symmetric group had lower rates of trivial residual shunt (5.7% vs 11.4%, P  = 0.018; and 0.8% vs 5.9%, P  = 0.001) and bundle branch block (0.8% vs 5.4%, P  = 0.002; and 0.6% vs 3.8%, P  = 0.009); and at follow-up, the asymmetric group had lower residual shunt (47.6% vs 85.0%, P  = 0.020) and similar branch block (30.0% vs 33.3%, P  = 1.000) disappearance rates. There were no severe complications, i.e. aortic regurgitation, malignant arrhythmias, haemolysis or device dislocation.
Conclusions: Perventricular device closure of pmVSDs appears safe and effective with symmetric and asymmetric occluders. However, the lower residual shunt disappearance and higher branch block incidence rates for asymmetric occluders would favour more proactive conversion to surgical repair immediately when residual shunt is present intraoperatively.

PMID: 28082474 [PubMed - indexed for MEDLINE]