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Analysis of circulating microRNAs in patients with repaired Tetralogy of Fallot with and without heart failure.

J Transl Med. 2017 Jul 10;15(1):156

Authors: Abu-Halima M, Meese E, Keller A, Abdul-Khaliq H, Rädle-Hurst T

BACKGROUND: MicroRNAs (miRNAs) are a class of regulatory RNAs that regulate gene expression post-transcriptionally. Little, however, is known on the expression profile of circulating miRNAs in Tetralogy of Fallot (TOF) patients late after surgical repair. In this study, we aimed to identify the specific patterns of circulating miRNAs in blood of patients with repaired, non-syndromic TOF and to assess whether these specific miRNAs may be useful to differentiate patients with and without heart failure.
METHODS: SurePrint™ 8 × 60 K Human v16 miRNA arrays were used to determine miRNA expression profiles in 15 healthy controls and 37 patients after TOF repair of whom 3 had symptomatic right heart failure. The expression levels of selected miRNAs have been validated by quantitative reverse transcription polymerase chain reaction (RT-qPCR). Enrichment analyses of altered miRNA expression were predicted using bioinformatic tools.
RESULTS: Compared with healthy controls, a total of 49, 58 and 77 miRNAs were found to be significantly altered in TOF patients (TOF-all), TOF patients with (TOF-HF) and without symptomatic right heart failure (TOF-noHF) (>2.0-fold change, adjusted P < 0.05), respectively. Three miRNAs namely miR-181d-5p, miR-206 and miR-625-5p were validated by RT-qPCR in all TOF groups. The area under the receiver operating characteristic curve (AUC) for miR-181d-5p, miR-206 and miR-625-5p were 0.987, 0.993 and 0.769 in TOF-all and 0.990, 0.994 and 0.749 in TOF-noHF, respectively. Moreover, expression levels of miR-625-5p, miR-1233-3p and miR-421 were lower in TOF-HF compared to TOF-noHF (P = 0.012).
CONCLUSIONS: Altered expression levels of circulating miRNAs were found in TOF patients late after surgical repair and are different to those seen in the right ventricular myocardium of infants with TOF. Expression levels of miR-421, miR-1233-3p and miR-625-5p are lower in TOF patients with symptomatic right heart failure and thus may indicate disease progression in these patients.

PMID: 28693530 [PubMed - indexed for MEDLINE]

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Risk comparison for prenatal use of analgesics and selected birth defects, National Birth Defects Prevention Study 1997-2011.

Ann Epidemiol. 2017 Oct;27(10):645-653.e2

Authors: Interrante JD, Ailes EC, Lind JN, Anderka M, Feldkamp ML, Werler MM, Taylor LG, Trinidad J, Gilboa SM, Broussard CS, National Birth Defects Prevention Study

PURPOSE: To compare the use of nonsteroidal anti-inflammatory drugs (NSAIDs) and/or opioids to the use of acetaminophen without NSAIDs or opioids with respect to associations with birth defects.
METHODS: We used data from the National Birth Defects Prevention Study (1997-2011). Exposure was self-reported maternal analgesic use from the month before through the third month of pregnancy (periconceptional). Adjusted odds ratios (aORs) were calculated to examine associations with 16 birth defects.
RESULTS: Compared to acetaminophen, mothers reporting NSAIDs were significantly more likely to have offspring with gastroschisis, hypospadias, cleft palate, cleft lip with cleft palate, cleft lip without cleft palate, anencephaly, spina bifida, hypoplastic left heart syndrome, pulmonary valve stenosis, and tetralogy of Fallot (aOR range, 1.2-1.6). Opioids were associated with tetralogy of Fallot, perimembranous ventricular septal defect, and ventricular septal defect with atrial septal defect (aOR range, 1.8-2.3), whereas use of both opioids and NSAIDs was associated with gastroschisis, cleft palate, spina bifida, hypoplastic left heart syndrome, and pulmonary valve stenosis (aOR range, 2.0-2.9).
CONCLUSIONS: Compared to periconceptional use of acetaminophen, selected birth defects occurred more frequently among infants of women using NSAIDs and/or opioids. However, we could not definitely determine whether these risks relate to the drugs or to indications for treatment.

PMID: 28993061 [PubMed - indexed for MEDLINE]

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Diffuse fibrosis is common in the left, but not in the right ventricle in patients with transposition of the great arteries late after atrial switch operation.

Int J Cardiovasc Imaging. 2018 Mar 29;:

Authors: Shehu N, Meierhofer C, Messroghli D, Mkrtchyan N, Martinoff S, Ewert P, Stern H

In adult patients with transposition of the great arteries (TGA) late after atrial switch operation (AtSO), each of the ventricles is faced with a profoundly different pressure regimen from the one they are meant to support in normal conditions. The extent of diffuse fibrosis of the right ventricle (RV) and left ventricle (LV) in these patients remains incompletely investigated. Aim of this study was to quantify the degree of fibrosis of the unloaded LV and of the overloaded RV by determining the myocardial extracellular volume (ECV) with non-invasive techniques as T1 mapping. We determined ECV by cardiac magnetic resonance (CMR) in 10 patients (36.8 ± 5.3 years old) with TGA late after AtSO, without relevant pulmonary stenosis, by acquiring T1-maps of the myocardium before and 10 min after injection of Gadolinium-based contrast agent. ECV of the inferior wall (36% (33-41%)) and of the lateral wall (37% (35-39%)) of the LV was significantly increased compared to the ECV of the RV (27% (25-29%)), in both comparisons P < 0.0001. Long-time LV unloading following atrial switch procedures leads to severe myocardial fibrosis of the subpulmonary LV. T1 mapping CMR might be useful for selection of patients with atrial switch operation, in whom reestablishment of the LV as a systemic ventricle by staged arterial switch operation is planned. However larger studies and newer higher resolution methods for T1-mapping are needed to determine the role of ECV in the decision of a surgical intervention in this kind of population.

PMID: 29600485 [PubMed - as supplied by publisher]

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Pulmonary hypertension: Barrier or just a bump in the road in transplanting adults with congenital heart disease.

Congenit Heart Dis. 2018 Mar 30;:

Authors: Menachem JN, Birati EY, Zamani P, Owens AT, Atluri P, Bermudez CA, Drajpuch D, Fuller S, Kim YY, Mascio CE, Palanivel V, Rame JE, Wald J, Acker MA, Mazurek JA

BACKGROUND: Heart failure (HF) is the leading cause of death in adults with congenital heart disease (ACHD). Identification of disease progression and timing of referral for advanced therapies is often delayed. However, increased awareness and understanding of ACHD and improvements in the approach to treatment have led to improved outcomes. Pulmonary hypertension (PH) is a common barrier to HT. In ACHD, the approach to PH and HT is quite complicated, given the anatomic heterogeneity and lower prevalence and experience. However, in some cases, PH is a result of elevated systemic filling pressures and low output.
METHODS: We describe the approach used to successfully transplant an ACHD patient with severe pre-HT PH performing HT alone. We review the literature and describe the one patient's journey from primarily palliative, to a combined heart-lung transplant candidate, to successful HT patient.
RESULTS: We discuss the methodology used to successfully transplant a patient, with significantly elevated pulmonary pressures and an initial pulmonary vascular resistance (PVR) > 13 Wood units.
CONCLUSIONS: There are a number of complexities associated with the ACHD population and it is of utmost importance to carefully identify the underlying hemodynamic milieu and inform the appropriate treatment course in order to have successful transplant outcomes.

PMID: 29603626 [PubMed - as supplied by publisher]

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A Translational Model of Incomplete Catch-Up Growth: Early-Life Hypoxia and the Effect of Physical Activity.

Clin Transl Sci. 2018 Mar 30;:

Authors: Radom-Aizik S, Zaldivar FP, Nance DM, Haddad F, Cooper DM, Adams GR

Advances in therapies have led to prolonged survival from many previously lethal health threats in children, notably among prematurely born babies and those with congenital heart disease. Evidence for catch-up growth is common in these children, but in many cases the adult phenotype is never achieved. A translational animal model is required in which specific tissues can be studied over a reasonable time interval. We investigated the impact of postnatal hypoxia (HY) (12%O2 (HY12) or 10% O2 (HY10)) on growth in rats relative to animals raised in room air. Subgroups had access to running wheels following the HY period. Growth was fully compensated in adult HY12 rats but not HY10 rats. The results of this study indicate that neonatal hypoxia can be a useful model for the elucidation of mechanisms that mediate successful catch-up growth following neonatal insults and identify the critical factors that prevent successful catch-up growth.

PMID: 29603633 [PubMed - as supplied by publisher]

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Anaesthetic management of breast surgery in a patient with Eisenmenger syndrome.

Rev Esp Anestesiol Reanim. 2017 Jan;64(1):41-45

Authors: Galán Gutiérrez JC, Fernández Suárez FE, Miranda García P, Sopena Zubiria LA

Eisenmenger syndrome (ES) is a complex combination of cardiovascular abnormalities defined as pulmonary hypertension with investment or bidirectional flow through an intracardiac or aortopulmonary communication, usually secondary to a congenital heart disease not resolved promptly. It carries a significant risk of perioperative mortality, with an incidence close to 30% for non-cardiac surgery. We report the anaesthetic management in a ES patient undergoing breast surgery, which was successfully performed under general anaesthesia combined with thoracic analgesic blocks. The main pathophysiological implications of this syndrome are discussed, emphasizing the importance of appropriate preoperative evaluation with thorough assessment of associated risks, careful intraoperative management, and postoperative care, which should be initially performed in a critical care unit. The need to individualize and tailor the choice of drugs and anesthetic technique to the hemodynamic condition of the patient and the surgical procedure is highlighted.

PMID: 27554331 [PubMed - indexed for MEDLINE]

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Pulmonary vascular volume ratio measured by cardiac computed tomography in children and young adults with congenital heart disease: comparison with lung perfusion scintigraphy.

Pediatr Radiol. 2017 Nov;47(12):1580-1587

Authors: Goo HW, Park SH

BACKGROUND: Lung perfusion scintigraphy is regarded as the gold standard for evaluating differential lung perfusion ratio in congenital heart disease.
OBJECTIVE: To compare cardiac CT with lung perfusion scintigraphy for estimated pulmonary vascular volume ratio in patients with congenital heart disease.
MATERIALS AND METHODS: We included 52 children and young adults (median age 4 years, range 2 months to 28 years; 31 males) with congenital heart disease who underwent cardiac CT and lung perfusion scintigraphy without an interim surgical or transcatheter intervention and within 1 year. We calculated the right and left pulmonary vascular volumes using threshold-based CT volumetry. Then we compared right pulmonary vascular volume percentages at cardiac CT with right lung perfusion percentages at lung perfusion scintigraphy by using paired t-test and Bland-Altman analysis.
RESULTS: The right pulmonary vascular volume percentages at cardiac CT (66.3 ± 14.0%) were significantly smaller than the right lung perfusion percentages at lung perfusion scintigraphy (69.1 ± 15.0%; P=0.001). Bland-Altman analysis showed a mean difference of -2.8 ± 5.8% and 95% limits of agreement (-14.1%, 8.5%) between these two variables.
CONCLUSION: Cardiac CT, in a single examination, can offer pulmonary vascular volume ratio in addition to pulmonary artery anatomy essential for evaluating peripheral pulmonary artery stenosis in patients with congenital heart disease. However there is a wide range of agreement between cardiac CT and lung perfusion scintigraphy.

PMID: 28646273 [PubMed - indexed for MEDLINE]

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Is having a job a protective factor? Employment status and state of medical care as subjectively perceived by adults with CHD in Germany.

Cardiol Young. 2017 Aug;27(6):1110-1117

Authors: Helm PC, Sticker EJ, Keuchen R, Koerten MA, Diller GP, Tutarel O, Bauer UMM

BACKGROUND: Most patients born with CHD nowadays reach adulthood, and thus quality of life, life situation, and state of medical care aspects are gaining importance in the current era. The present study aimed to investigate whether patients' assessment depends on their means of occupation. The findings are expected to be helpful in optimising care and for developing individual treatment plans.
METHODS: The present study was based on an online survey conducted in cooperation with patient organisations. Participants were recruited from the database of the German National Register for Congenital Heart Defects. In total, 1828 individuals (777 males, 1051 females) took part. Participants were asked to rate aspects such their state of health on a six-tier scale (1=worst specification). Response behaviour was measured against the background of occupational details.
RESULTS: Training for or pursuing a profession was found to be significantly associated with participants' rating of five of the six examined aspects (p<0.05). Sex seemed to play an important part in four of the six aspects.
CONCLUSIONS: An optimal treatment plan for adults with CHD should always consider aspects such as sex and employment status. To work out such an optimal and individual treatment plan for each adult CHD patient, an objective tool to measure patients' actual CHD-specific knowledge precluding socially accepted response bias would be very useful.

PMID: 27830637 [PubMed - indexed for MEDLINE]

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Congenital coronary artery anomalies in adults: review of 111 cases from a single-centre experience.

Cardiol Young. 2017 Aug;27(6):1041-1050

Authors: Çanga Y, Güvenç TS, Karataş MB, Çalık AN, Onuk T, Tanık VO, Güngör B, Bolca O

BACKGROUND: Coronary artery anomalies are a heterogeneous group of congenital disorders presenting with a wide spectrum of symptoms, ranging from vague chest pain to sudden cardiac death. Despite available data, there is no consensus about the classification, nomenclature, and outcomes of coronary anomalies in the normally connected heart. In this study, we aimed to investigate clinical and angiographic characteristics of coronary arterial anomalies, as well as the frequency of atherosclerotic involvement in anomalous coronaries, diagnosed at a tertiary referral centre.
METHODS: We retrospectively reviewed coronary angiograms performed between 2011 and 2015 for the presence of a coronary anomaly. A total of 111 patients with a final diagnosis of coronary anomaly were included in the study group. We also recruited 110 age- and sex-matched patients who underwent coronary angiography because of symptomatic coronary artery disease as controls.
RESULTS: Among 36,893 coronary angiograms, 111 (0.30%) major coronary anomalies were found. Compared with controls, the prevalence of significant atherosclerotic coronary disease was lower in patients with coronary anomalies and stable symptoms (p=0.02); however, the prevalence of significant coronary atherosclerosis was similar among patients admitted with unstable angina or myocardial infarction (p>0.05). Compared with controls, patients with an anomalous left anterior descending coronary artery had significantly less atherosclerotic involvement than those in whom the left anterior descending artery was not anomalous (p=0.005).
CONCLUSIONS: Although coronary artery anomalies are cited as a cause for myocardial ischaemia, atherosclerotic coronary artery disease is also frequent and may offer an alternative explanation to ischaemic symptoms. No predisposition to accelerated atherosclerosis was found, however, and atherosclerotic involvement was less frequent in some anomalous vessels.

PMID: 27842619 [PubMed - indexed for MEDLINE]

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Transthoracic echocardiography study of congenitally corrected transposition of the great arteries.

J Clin Ultrasound. 2017 Jul 08;45(6):375-379

Authors: Singh A, Singh G, Dhaliwal TS, Singh M

Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart defect, occurring in only 0.5% of patients exhibiting congenital heart defects. Because it is not seen in a routine transthoracic echocardiography (TTE) examination, CCTGA may be missed. The echocardiography diagnosis of CCTGA is difficult and not well explained in most textbooks. The present report provides key sonographic images that contribute to the diagnosis. In the present case, the patient had no prior history of heart disease. TTE revealed that the patient had CCTGA and early tricuspid regurgitation with right ventricle enlargement and hypertrophy. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 45:375-379, 2017.

PMID: 28182287 [PubMed - indexed for MEDLINE]