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Atrial Fibrillation Therapy and Heart Failure Hospitalization in Adults With Tetralogy of Fallot.

JACC Clin Electrophysiol. 2019 May;5(5):618-625

Authors: Egbe AC, Miranda WR, Ammash NM, Ananthaneni S, Sandhyavenu H, Farouk Abdelsamid M, Yogeswaran V, Kapa S, Fatola A, Kothapalli S, Connolly HM

OBJECTIVES: This study hypothesized that atrial fibrillation was associated with heart failure (HF) hospitalization, and that patients who received rhythm control therapy had a lower incidence of HF hospitalization and mortality.
BACKGROUND: Atrial fibrillation is a known risk factor for HF hospitalization and mortality in patients with acquired heart disease. Although atrial arrhythmias are common in adults with tetralogy of Fallot (TOF), data about prevalence and outcomes of therapy for atrial fibrillation are very limited.
METHODS: The MACHD (Mayo Adult Congenital Heart Disease) database was queried for adults with repaired TOF and documented atrial fibrillation from 1990 to 2017. Primary endpoint was HF hospitalization defined as admission for volume overload (pulmonary congestion and/or peripheral edema) requiring intravenous diuretics. Secondary endpoint was the effect of rhythm control therapy on HF hospitalization and all-cause mortality. Patients were divided into rhythm control and rate control groups based on the therapy initiated at the time of arrhythmia diagnosis.
RESULTS: Of 415 patients, 27 (7%) had 42 HF hospitalizations. Of these 415 patients, 88 (21%) had atrial fibrillation at age 49 ± 13 years. Atrial fibrillation was an independent risk factor for HF hospitalization (adjusted hazard ratio: 2.67; 95% confidence interval: 1.04 to 7.34; p = 0.045). The 88 patients were divided into the rhythm control group (n = 61, 69%) and the rate control group (n = 27, 31%). The rate control group had higher unadjusted annual incidence of HF hospitalization (13% vs. 3%; p = 0.001) and all-cause mortality (11% vs. 4%; p = 0.002).
CONCLUSIONS: Atrial fibrillation was a risk factor for HF hospitalization and mortality in TOF patients, and rhythm control therapy was protective against these adverse events.

PMID: 31122385 [PubMed - in process]

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Maternal and fetal outcomes in pregnancies complicated by Marfan syndrome.

Heart. 2019 May 25;:

Authors: Cauldwell M, Steer PJ, Curtis SL, Mohan A, Dockree S, Mackillop L, Parry HM, Oliver J, Sterrenberg M, Wallace S, Malin G, Partridge G, Freeman LJ, Bolger AP, Siddiqui F, Wilson D, Simpson M, Walker N, Hodson K, Thomas K, Bredaki F, Mercaldi R, Walker F, Johnson MR

OBJECTIVES: Information to guide counselling and management for pregnancy in women with Marfan syndrome (MFS) is limited. We therefore conducted a UK multicentre study.
METHODS: Retrospective observational study of women with MFS delivering between January 1998 and March 2018 in 12 UK centres reporting data on maternal and neonatal outcomes.
RESULTS: In total, there were 258 pregnancies in 151 women with MFS (19 women had prior aortic root replacements), including 226 pregnancies ≥24 weeks (two sets of twins), 20 miscarriages and 12 pregnancy terminations. Excluding miscarriages and terminations, there were 221 live births in 139 women. Only 50% of women received preconception counselling. There were no deaths, but five women experienced aortic dissection (1.9%; one type A and four type B-one had a type B dissection at 12 weeks and subsequent termination of pregnancy). Five women required cardiac surgery postpartum. No predictors for aortic dissection could be identified. The babies of the 131 (65.8%) women taking beta-blockers were on average 316 g lighter (p<0.001). Caesarean section rates were high (50%), particularly in women with dilated aortic roots. In 55 women, echocardiographic aortic imaging was available prepregnancy and postpregnancy; there was a small but significant average increase in AoR size of 0.84 mm (Median follow-up 2.3 months) CONCLUSION: There were no maternal deaths, and the aortic dissection rate was 1.9% (mainly type B). There with no identifiable factors associated with aortic dissection in our cohort. Preconception counselling rates were low and need improvement. Aortic size measurements increased marginally following pregnancy.

PMID: 31129614 [PubMed - as supplied by publisher]

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Determinants of long-term outcome of repaired pulmonary valve stenosis.

Rev Esp Cardiol (Engl Ed). 2019 May 24;:

Authors: Galian-Gay L, Gordon B, Marsal JR, Rafecas A, Pijuan Domènech A, Castro MA, Subirana Domènech MT, Sureda C, Miranda B, Martí G, García-Dorado D, Dos Subirà L

INTRODUCTION AND OBJECTIVES: Although pulmonary valve stenosis (PVS) is considered a low risk congenital heart disease, there have been reports of complications and the need for reintervention throughout follow-up. The aims of this study were to evaluate the long-term outcome of repaired PVS and to identify predictors of cardiovascular complications and reintervention.
METHODS: We studied 158 adult patients with repaired PVS (repair procedures performed from 1957 to 2010) receiving active follow-up in a tertiary referral center.
RESULTS: A total of 95 patients (60%) received surgical treatment, and 63 patients (40%) received percutaneous pulmonary balloon valvuloplasty. At the end of follow-up (27 years, IQR, 20-33 years), most patients (n=134, 84.8%) were in New York Heart Association functional class I, but 61 patients (38.6%) required a reintervention, mainly pulmonary valve replacement (17.7%, n=28), and 19 patients (12%) had at least one cardiovascular complication: 13 (8.2%) supraventricular arrhythmias, 6 (3.8%) heart failure, 5 (3.2%) stroke, 1 (0.6%) death, 1 (0.6%) thromboembolism, and 1 (0.6%) ventricular arrhythmia. Multivariate analysis showed that age at PVS repair (HR, 1.08; 95%CI, 1.04-1.12; P <.001) and the presence of cyanosis before PVS repair (HR, 5.23; 95%CI, 1.99-13.78; P=.001) were independent predictors for cardiovascular complications.
CONCLUSIONS: Good long-term outcome can be expected after PVS repair, but complications and the need for reintervention may appear. Older age and the presence of cyanosis at PVS repair emerged as predictors of cardiovascular complications and identified a population that may merit stricter control.

PMID: 31130344 [PubMed - as supplied by publisher]

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A derived and validated score to predict prolonged mechanical ventilation in patients undergoing cardiac surgery.

J Thorac Cardiovasc Surg. 2017 01;153(1):108-115

Authors: Sharma V, Rao V, Manlhiot C, Boruvka A, Fremes S, Wąsowicz M

OBJECTIVES: Prolonged mechanical ventilation after cardiac surgery imposes a significant burden on the patient in terms of morbidity as well as a financial burden on the hospital. We undertook a retrospective analysis of 2 prospectively collected databases developed in tertiary cardiac care centers to derive and validate a risk index predicting prolonged mechanical ventilation after cardiac surgery.
METHODS: We studied a retrospective cohort of 32,045 patients undergoing cardiac surgery in 2 hospitals in Toronto, Canada. The development cohort consisted of 21,661 patients at Toronto General Hospital. Data Sunnybrook Health Sciences Centre, Toronto, Canada, with 10,384 patients, served as an institutional validation cohort. We operationally characterized prolonged mechanical ventilation as the duration from surgery completion to extubation exceeding 48 hours.
RESULTS: Prolonged postoperative mechanical ventilation rates in the development and validation cohort were 6% and 7%, respectively. Multivariable regression in the development cohort showed that the following factors were strong predictors of prolonged mechanical ventilation after cardiac surgery: previous cardiac surgery, lower left ventricular ejection fraction, shock, surgery involving repair of congenital heart disease, and cardiopulmonary bypass time. The intraoperative multivariable model retained good discrimination in the validation cohort, achieving a c statistic of 0.787.
CONCLUSIONS: Prolonged mechanical ventilation after cardiac surgery can be accurately predicted by readily available pre- and intraoperative information.

PMID: 27665221 [PubMed - indexed for MEDLINE]

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Tricuspid regurgitation is uncommon after mitral valve repair for degenerative diseases.

J Thorac Cardiovasc Surg. 2017 07;154(1):110-122.e1

Authors: David TE, David CM, Fan CS, Manlhiot C

OBJECTIVE: To determine the incidence and effects of tricuspid regurgitation (TR) after surgery for mitral valve (MV) repair for mitral regurgitation (MR) due to degenerative disease.
PATIENTS AND METHODS: We examined 1171 patients who had MV repair and were followed prospectively with periodical clinical and echocardiographic assessments during a mean of 9.1 ± 5.3 years. Patients' mean age was 58.2 ± 12.7 years, and 70.5% were men. Preoperatively, 44.6% were in functional classes III and IV, 20.1% had atrial fibrillation, and 34.2% had ejection fraction <60%. In addition to MV repair, 13.8% had coronary artery bypass, 11.4% had the maze procedure, and 4.7% had tricuspid annuloplasty.
RESULTS: Moderate and severe TR was present in 138 patients before surgery and associated with older age, preoperative atrial fibrillation, preoperative congestive heart failure, congenital heart septal defects, lower preoperative left ventricular ejection fraction, and female sex by multivariable analysis. TR resolved postoperatively but recurrent or new isolated TR occurred in 45 patients postoperatively (13.6% at 15 years in all patients). Factors associated with isolated postoperative TR by multivariable analysis included older age at operation, unrepaired preoperative moderate/severe TR, and the development of postoperative MR. Patients with preoperative TR had reduced long-term survival and tricuspid annuloplasty did not restore lifespan.
CONCLUSIONS: Preoperative TR in patients with MR due to degenerative diseases was associated with longstanding MV disease and adversely affected long-term survival after MV repair. New postoperative TR was uncommon. The findings of this study are compelling reasons to repair the MV before the development of TR.

PMID: 28262288 [PubMed - indexed for MEDLINE]

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Risk of Dementia in Adults With Congenital Heart Disease: Population-Based Cohort Study.

Circulation. 2018 05 01;137(18):1912-1920

Authors: Bagge CN, Henderson VW, Laursen HB, Adelborg K, Olsen M, Madsen NL

BACKGROUND: More children with congenital heart disease (CHD) are surviving to adulthood, and CHD is associated with risk factors for dementia. We compared the risk of dementia in CHD adults to that of the general population.
METHODS: In this cohort study, we used medical registries and a medical record review covering all Danish hospitals to identify adults with CHD diagnosed between 1963 and 2012. These individuals with CHD were followed from January 1, 1981, 30 years of age, or date of first CHD registration (index date for matched members of the general population cohort) until hospital diagnosis of dementia, death, emigration, or end of study (December 31, 2012). For each individual with CHD, we identified 10 members of the general population utilizing the Danish Civil Registration System matched on sex and birth year. We computed cumulative incidences and hazard ratios (HRs) of dementia, adjusting for sex and birth year.
RESULTS: The cumulative incidence of dementia was 4% by 80 years of age in 10 632 adults with CHD (46% male). The overall HR comparing adults with CHD with the general population cohort was 1.6 (95% confidence interval [CI], 1.3-2.0). The HR among individuals with CHD without extracardiac defects was 1.4 (95% CI, 1.1-1.8). Adults with mild-to-moderate CHD had an HR of 1.5 (95% CI, 1.1-2.0), whereas the HR was 2.0 (95% CI, 1.2-3.3) for severe CHD, including univentricular hearts. The HR for early onset dementia (<65 years of age) was 2.6 (95% CI, 1.8-3.8), whereas the late-onset HR was 1.3 (95% CI, 1.0-1.8).
CONCLUSIONS: CHD was associated with an increased risk of dementia compared with the general population, in particular for early onset dementia. Further understanding of dementia risk in the population with CHD is a potential target for future investigation.

PMID: 29440121 [PubMed - indexed for MEDLINE]

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Spontaneous Coronary Artery Dissection: Current State of the Science: A Scientific Statement From the American Heart Association.

Circulation. 2018 05 08;137(19):e523-e557

Authors: Hayes SN, Kim ESH, Saw J, Adlam D, Arslanian-Engoren C, Economy KE, Ganesh SK, Gulati R, Lindsay ME, Mieres JH, Naderi S, Shah S, Thaler DE, Tweet MS, Wood MJ, American Heart Association Council on Peripheral Vascular Disease; Council on Clinical Cardiology; Council on Cardiovascular and Stroke Nursing; Council on Genomic and Precision Medicine; and Stroke Council

Spontaneous coronary artery dissection (SCAD) has emerged as an important cause of acute coronary syndrome, myocardial infarction, and sudden death, particularly among young women and individuals with few conventional atherosclerotic risk factors. Patient-initiated research has spurred increased awareness of SCAD, and improved diagnostic capabilities and findings from large case series have led to changes in approaches to initial and long-term management and increasing evidence that SCAD not only is more common than previously believed but also must be evaluated and treated differently from atherosclerotic myocardial infarction. High rates of recurrent SCAD; its association with female sex, pregnancy, and physical and emotional stress triggers; and concurrent systemic arteriopathies, particularly fibromuscular dysplasia, highlight the differences in clinical characteristics of SCAD compared with atherosclerotic disease. Recent insights into the causes of, clinical course of, treatment options for, outcomes of, and associated conditions of SCAD and the many persistent knowledge gaps are presented.

PMID: 29472380 [PubMed - indexed for MEDLINE]

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Analysis of gene copy number variations in patients with congenital heart disease using multiplex ligation-dependent probe amplification.

Anatol J Cardiol. 2018 Jul;20(1):9-15

Authors: Mutlu ET, Aykan HH, Karagöz T

OBJECTIVE: At the molecular and cellular levels, heart development entails the precise orchestration of genetic events such as the interplay of master transcriptional regulators, signaling pathways, and chromatin remodeling. Recent studies among patients with congenital heart disease (CHD) have shown the importance of recurrent copy number variations (CNVs) in a significant number of patients. Recurrent CNVs that span several genes may affect other important organs, besides the heart. Because CHD may be the first presenting symptom in such patients, the analysis of recurrent CNVs in the genomic regions containing genes associated with CHD in patients referring to cardiology clinics may lead to an early diagnosis and the treatment of extracardiac symptoms in these patients. In this study, we aimed to screen CNVs of genomic regions including GATA4, NKX2-5, TBX5, BMP4, and CRELD1 genes and to analyse the 22q11.2 chromosomal region in apparently nonsyndromic patients with cardiac septal defects.
METHODS: Genomic regions including GATA4, NKX2-5, TBX5, BMP4, and CRELD1 genes and the 22q11.2 chromosomal region were analyzed in apparently nonsyndromic 45 patients with cardiac septal defects using the MLPA P-311 A2 Congenital Heart Disease kit. Multiplex ligationdependent probe amplification (MLPA) is an established technique for the detection of known CNVs. MLPA is substantially less expensive than array CGH and is relatively simple to use for clinicians without specific expertise in genomic technology; thus, MLPA could be used as a first-tier screening assay.
RESULTS: We screened 45 patients with cardiac septal defects for CNVs using the MLPA P-311 A2 kit. We identified three CNVs (n=3/45, 6.66%) and three 22q11 deletions. The CNVs were confirmed using fluorescence in situ hybridization.
CONCLUSION: Our study confirmed that the analysis of recurrent CNVs using the MLPA assay within pediatric cardiology clinics can led to an early syndrome diagnosis in nonsyndromic patients with CHD.

PMID: 29952356 [PubMed - indexed for MEDLINE]

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Electrocardiographic left atrial abnormalities predict cardiovascular mortality.

J Electrocardiol. 2018 Jul - Aug;51(4):652-657

Authors: Ha LD, Grober AF, Hock J, Wheeler M, Elbadawi A, Biniwale N, Baig B, Froelicher V

OBJECTIVE: Clinical utilization of electrocardiography for diagnosis of left atrial abnormalities is hampered by variable P-wave morphologies, multiple empiric criteria, and lack of an imaging "gold standard". Our aim was to determine the prevalence of P-wave patterns and demonstrate which components have associations with cardiovascular death (CVD).
METHODS: This is a retrospective analysis of 20,827 veterans <56 years of age who underwent electrocardiograms at a Veteran's Affairs Medical Center from 1987 to 1999, followed for a median duration of 17.8 years for CVD. Receiver Operating Characteristic, Kaplan-Meier and Cox Hazard analyses were applied, the latter with adjustment for age, gender and electrocardiography abnormalities.
RESULTS: The mean age was 43.3 ± 8 years, and 888 CVD (4.3%) occurred. A single positive deflection of the P-wave (Pattern 1) was present in 29% for V1 and 81% for V2. A singular negative P-wave (Pattern 2) was present in 4.6% for V1 and 1.6% in V2. A P-wave with an upward component followed by downward component (Pattern 3) was present in 64.5% for V1 and 17.5% for V2. When the downward component in Patterns 2 and/or 3 is at least -100 μV, a significant association is observed with CVD (adjusted hazard ratios [HRs] 2.9-4.1, P < 0.001). Total P-wave duration ≥140 ms was also associated with CVD (adjusted HR 2.2, P < 0.001).
CONCLUSIONS: A negative P-wave in V1 or V2 ≤-100 μV, and P-wave with a duration of ≥140 ms, all have independent and significant associations with CVD, with HRs comparable to other electrocardiography abnormalities.

PMID: 29997006 [PubMed - indexed for MEDLINE]

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Management of high risk cardiac conditions in pregnancy: Anticoagulation, severe stenotic valvular disease and cardiomyopathy.

Trends Cardiovasc Med. 2019 04;29(3):155-161

Authors: Lau E, DeFaria Yeh D

Cardiovascular disease contributes to approximately one third of all maternal mortality and remains a significant source of peri‑ and postpartum morbidity. As more women at risk for and with cardiovascular disease are desiring pregnancy, it is imperative that general cardiologists and obstetricians participate collaboratively in preconception counseling and are more facile with management of these lesions during peri‑ and postpartum periods. This review aims to address this growing need and highlights the management strategies for some of the major high risk cardiac conditions encountered during pregnancy including anticoagulation, cardiomyopathies as well as severe mitral and aortic stenosis; aortopathy, pulmonary hypertension, and severe congenital heart lesions will not be addressed.

PMID: 30104111 [PubMed - indexed for MEDLINE]