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A "long-standing" malpositioned pacing lead. Long-term follow-up after extraction.

Monaldi Arch Chest Dis. 2018 Sep 04;88(3):927

Authors: Sarubbi B, Scognamiglio G, Fusco F, Melillo E, D'Alto M, Russo MG

Abstract
Transvenous pacemaker (PM) catheters can be unintentionally placed in the left ventricle (LV) during the implantation procedure. An 8-year-old girl was discovered with a malpositioned pm wire, seven years after the implant. Trans-thoracic echocardiogram revealed the lead traversing the inter-atrial septum, crossing the mitral valve and embedded in the basal lateral wall of the LV. This is a report of a 14-year long follow-up after the surgical extraction of the malpositioned PM lead.

PMID: 30183161 [PubMed - in process]

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Databases for Congenital Heart Defect Public Health Studies Across the Lifespan.

J Am Heart Assoc. 2016 10 26;5(11):

Authors: Riehle-Colarusso TJ, Bergersen L, Broberg CS, Cassell CH, Gray DT, Grosse SD, Jacobs JP, Jacobs ML, Kirby RS, Kochilas L, Krishnaswamy A, Marelli A, Pasquali SK, Wood T, Oster ME, Congenital Heart Public Health Consortium

PMID: 27912209 [PubMed - indexed for MEDLINE]

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Clinical utility and diagnostic accuracy of palm-held, mini-sized ultrasonocardiographic scanner in congenital heart disease.

J Formos Med Assoc. 2018 Feb;117(2):141-146

Authors: Lo MH, Huang CF, Lin IC, Lin YJ, Kuo HC, Hsieh KS

Abstract
BACKGROUND/PURPOSE: To investigate whether a palm-held ultrasonocardiographic scanner would be useful for screening and follow-up in congenital heart disease (CHD).
METHODS: We retrospectively reviewed the echocardiographic images from June 1, 2014 to November 1, 2014. All patients underwent two ultrasonographic examinations including palm-held scanner examination and standard echocardiography. To compare the quality of the two instruments, we developed a diagnostic scoring system ranging from 1 point to 10 points, with 10 points indicating the best quality. Two experienced echocardiographers retrospectively reviewed all recorded images blindedly and gave each examination a score. Comparisons of diagnostic score between two equipments were performed.
RESULTS: A total of 262 patients' images were reviewed. All cardiac lesions could be detected with both instruments. The mean diagnostic score of palm-held scanner and standard echocardiography were 8.20±0.53 versus 9.64±0.37 (p<0.05) in color image and 7.00±1.05 versus 8.56±1.14 (p<0.05) in gray-scale two-dimensional image, respectively. When we compared the score between the two instruments in individual CHDs, we found standard echocardiography had better quality except for detecting muscular ventricular septal defect and pulmonary regurgitation.
CONCLUSION: The diagnostic sensitivity of palm-held scanner in detecting CHD was very good. Despite both instruments having a high diagnostic score in detecting CHD, standard echocardiography had better quality. Traditional echocardiography is still the standard tool for CHD evaluation. However, the palm-held scanner can support physical examination for initial screening and follow-up, and offer cardiologists an opportunity to visualize and listen to the heart at any time.

PMID: 28404481 [PubMed - indexed for MEDLINE]

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Oral findings in Williams-Beuren syndrome.

Med Oral Patol Oral Cir Bucal. 2018 Jan 01;23(1):e1-e6

Authors: Ferreira SB, Viana MM, Maia NG, Leão LL, Machado RA, Coletta RD, de Aguiar MJ, Martelli-Júnior H

Abstract
BACKGROUND: Williams-Beuren syndrome (WBS; OMIM #194050) is a developmental disorder characterized by congenital heart disease, intellectual disability, dysmorphic facial features and ophthalmologic abnormalities. Oral abnormalities are also described in clinical manifestations of the disease. This paper describes orofacial features in patients with WBS.
MATERIAL AND METHODS: Seventeen patients with a confirmed molecular diagnosis of WBS were examined for oral abnormalities through clinical oral evaluations and panoramic radiography.
RESULTS: Malocclusion, specifically with dental midline deviation, and high-arched palate were the most common findings.
CONCLUSIONS: The present results contribute to knowledge on the orofacial manifestations of WBS. Since such patients with WBS may develop severe oral abnormalities, early detection and treatment can help improve their quality of life.

PMID: 29274148 [PubMed - indexed for MEDLINE]

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Novel Genetic Variants of Sporadic Atrial Septal Defect (ASD) in a Chinese Population Identified by Whole-Exome Sequencing (WES).

Med Sci Monit. 2018 Mar 05;24:1340-1358

Authors: Liu Y, Cao Y, Li Y, Lei D, Li L, Hou ZL, Han S, Meng M, Shi J, Zhang Y, Wang Y, Niu Z, Xie Y, Xiao B, Wang Y, Li X, Yang L, Wang W, Jiang L

Abstract
BACKGROUND Recently, mutations in several genes have been described to be associated with sporadic ASD, but some genetic variants remain to be identified. The aim of this study was to use whole-exome sequencing (WES) combined with bioinformatics analysis to identify novel genetic variants in cases of sporadic congenital ASD, followed by validation by Sanger sequencing. MATERIAL AND METHODS Five Han patients with secundum ASD were recruited, and their tissue samples were analyzed by WES, followed by verification by Sanger sequencing of tissue and blood samples. Further evaluation using blood samples included 452 additional patients with sporadic secundum ASD (212 male and 240 female patients) and 519 healthy subjects (252 male and 267 female subjects) for further verification by a multiplexed MassARRAY system. Bioinformatic analyses were performed to identify novel genetic variants associated with sporadic ASD. RESULTS From five patients with sporadic ASD, a total of 181,762 genomic variants in 33 exon loci, validated by Sanger sequencing, were selected and underwent MassARRAY analysis in 452 patients with ASD and 519 healthy subjects. Three loci with high mutation frequencies, the 138665410 FOXL2 gene variant, the 23862952 MYH6 gene variant, and the 71098693 HYDIN gene variant were found to be significantly associated with sporadic ASD (P<0.05); variants in FOXL2 and MYH6 were found in patients with isolated, sporadic ASD (P<5×10^-4). CONCLUSIONS This was the first study that demonstrated variants in FOXL2 and HYDIN associated with sporadic ASD, and supported the use of WES and bioinformatics analysis to identify disease-associated mutations.

PMID: 29505555 [PubMed - indexed for MEDLINE]

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Evaluation and Management of Maternal Congenital Heart Disease: A Review.

Obstet Gynecol Surv. 2018 Feb;73(2):116-124

Authors: Hopkins MK, Goldstein SA, Ward CC, Kuller JA

Abstract
Objective: Congenital heart defects represent the most common major congenital anomalies. The objective of this review was to define the most common forms of congenital heart disease (CHD) in pregnancy, outline preconception counseling, discuss the associated morbidity and mortality of each lesion, and review current recommendations for management of CHD in pregnancy.
Evidence Acquisition: A MEDLINE search of "congenital heart disease in pregnancy" and specific conditions in pregnancy including "ventricular septal defect," "atrial septal defect," "left outflow obstruction," "right outflow obstruction," "tetralogy of Fallot," and "transposition of the great vessels" was performed.
Results: The evidence included in the review contains 18 retrospective studies, 8 meta-analyses or systematic reviews or expert opinions, 5 case reports including surgical case reports, 2 prospective studies, and 2 clinical texts.
Conclusions: Given advances in surgical and medical management, women with a history of congenital cardiac defects are more frequently reaching childbearing age and requiring obstetric care. Many women with CHD can have successful pregnancies, although there are a few conditions that confer significant maternal risk, and pregnancy may even be contraindicated. Appropriate care for women with CHD requires a knowledge of cardiac physiology in pregnancy, the common lesions of CHD, and coordinated care from cardiology and maternal-fetal medicine specialists.

PMID: 29480926 [PubMed - indexed for MEDLINE]

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Pregnancy Outcomes in Women With Transposition of the Great Arteries After an Arterial Switch Operation.

JAMA Cardiol. 2018 Sep 05;:

Authors: Stoll VM, Drury NE, Thorne S, Selman T, Clift P, Chong H, Thompson PJ, Morris RK, Hudsmith LE

Abstract
Importance: A growing number of women are approaching childbearing age after arterial switch surgery for transposition of the great arteries. Prepregnancy counseling requires updated knowledge of the additional cardiovascular risks pregnancy poses for this cohort of women and the potential effect on their offspring; however, to our knowledge, this information is currently unknown.
Objective: To determine the pregnancy outcomes of women with transposition of the great arteries after an arterial switch operation, as well as the outcomes of their offspring.
Design, Setting, and Participants: This cohort study assessed women who had had arterial switch surgery from 1985 to the present and who were 16 years or older as of January 2018. All women with a previous arterial switch surgery for transposition of the great arteries with completed or ongoing pregnancy were included. Data were collected in a level 1 congenital cardiology center and joint obstetrics-cardiology clinic in Birmingham, United Kingdom.
Exposures: Patients were assessed before, during, and after pregnancy.
Main Outcomes and Measures: Adverse maternal cardiac events (arrhythmia, heart failure, aortic dissection, or acute coronary syndrome) and aortic root dilatation, aortic regurgitation, and left ventricular function before and after pregnancy were the main outcomes. Mode of delivery and fetal outcomes were considered secondary outcomes.
Results: A total of 25 pregnancies were identified in 15 women; 8 women had had 1 pregnancy, while 7 were multiparous. There were no adverse maternal cardiac events. Before pregnancy, 8 women (53%) had no aortic regurgitation, 1 (7%) had a trivial degree of regurgitation, 4 (26%) had mild regurgitation, and 2 (14%) had moderate regurgitation. After pregnancies, 1 woman (7%) had minor progression of aortic regurgitation. Five women (36%) had mild neoaortic root dilatation prepregnancy, but none developed progressive dilatation in the first year post-partum. A total of 24 pregnancies were completed by the end of the study, with all infants born alive and well. Nineteen modes of delivery were known; there were 7 cesarean deliveries (37%), of which 2 (11%) were recommended for aortic dilatation and 5 (26%) for obstetric indications or maternal choice.
Conclusions and Relevance: Pregnancy is well tolerated after arterial switch operation; no adverse maternal cardiac events or early progression of neoaortic root dilatation or aortic regurgitation were observed in this study. These results provide evidence to allow reassurance of women with previous arterial switch surgery who are planning pregnancies.

PMID: 30193342 [PubMed - as supplied by publisher]

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Contemporary ACHD training and the reality of the field in the United States.

Int J Cardiol. 2018 Sep 01;:

Authors: Ephrem G, Alshawabkeh L

Abstract
BACKGROUND: Care delivery for the growing population of adults living with congenital heart disease (CHD) has been met with challenges due to a shortage of physicians trained to care for this population. To meet this urgent need, restructuring and standardization of the training programs were implemented in 2015. The consequences of such a system on the graduating fellows have not been examined.
METHODS: A 25-question electronic survey was distributed to early career physicians who graduated following training in adult CHD (ACHD) care between 2015 and 2017 and are currently practicing in the United States. The survey results were anonymous.
RESULTS: Of the 30 physicians who trained in ACHD between 2015 and 2017 in the U.S., 21 (70%) responded to the survey. The majority completed a 2-year ACHD program, practice at an adult hospital, are happy with their current job, spend most of their time in ACHD-related activities, make on average around 250,000 USD for entry level positions, and prioritize supportive leadership and colleagues. Their training was adequate for their job requirements. However, the acquisition of an additional skill, in addition to clinical ACHD care, allowed them to secure a more ideal job. A sizeable number of jobs required program building or expansion with only 9.5% of trainees comfortable doing so immediately after graduation.
CONCLUSIONS: The new ACHD training curriculum successfully meets most of the needs for ACHD jobs. Integration of specialty tracks, ensuring uniformity in the quality of training between programs, and promoting leadership skills may improve career prospects.

PMID: 30195839 [PubMed - as supplied by publisher]

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Pulmonary hypertension in adults with congenital heart disease: Updated recommendations from the Cologne Consensus Conference 2018.

Int J Cardiol. 2018 Aug 25;:

Authors: Kaemmerer H, Apitz C, Brockmeier K, Eicken A, Gorenflo M, Hager A, de Haan F, Huntgeburth M, Kozlik-Feldmann RG, Miera O, Diller GP

Abstract
In the summer of 2016, delegates from the German Respiratory Society (DGP), the German Society of Cardiology (DGK) and the German Society of Pediatric Cardiology (DGPK) met in Cologne, Germany, to define consensus-based practice recommendations for the management of patients with pulmonary hypertension (PH). These recommendations were built on the 2015 European Pulmonary Hypertension guidelines, aiming at their practical implementation, considering country-specific issues, and including new evidence, where available. To this end, a number of working groups was initiated, one of which was specifically dedicated to PH in adults associated with congenital heart disease (CHD). As such patients are often complex and require special attention, and the general PAH treatment algorithm in the ESC/ERS guidelines appears too unspecific for CHD, the working group proposes an analogous algorithm for the management of PH-CHD which takes the special features of this patient group into consideration, and includes general measures, supportive therapy, targeted PAH drug therapy as well as interventional and surgical procedures. The detailed results and recommendations of the working group on PH in adults with CHD, which were last updated in the spring of 2018, are summarized in this article.

PMID: 30195841 [PubMed - as supplied by publisher]

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Contrast volume to estimated glomerular filtration rate ratio for prediction of contrast-induced acute kidney injury after cardiac catheterization in adults with congenital heart disease.

Catheter Cardiovasc Interv. 2018 Sep 09;:

Authors: Gellis L, Gauvreau K, Ferguson M, Bergersen L, Shafer K, Porras D

Abstract
BACKGROUND: Adults with congenital heart disease (ACHD) are vulnerable to contrast-induced acute kidney injury (CI-AKI) after cardiac catheterization. The aim of this study was to identify risk factors for clinically significant CI-AKI and evaluate the predictive value of contrast volume to estimated glomerular filtration rate ratio (V/eGFR) for the risk of CI-AKI following catheterization in the ACHD population.
METHODS: ACHD patients who underwent catheterization at Boston Children's hospital between 1/2011 and 1/2017 were retrospectively analyzed. CI-AKI was defined as an increase in serum creatinine ≥0.3 mg/dL within 48 hr or ≥1.5 times baseline within 7 days of procedure. Controls without CI-AKI were matched for calendar year of catheterization with cases using a 3:1 ratio.
RESULTS: Of 453 catheterizations meeting inclusion criteria, 27 catheterizations (5.9%) were complicated by CI-AKI, with dialysis being used to manage renal dysfunction in five of these events. Older age, male gender, admission prior to catheterization, and V/eGFR ratio were found to be related to risk of CI-AKI. Patients with a V/eGFR ≥ 2.6 had a significantly higher risk of CI-AKI (OR = 6.4; 95% CI = 2.0-20.4; P = 0.002). Survival at 3 years post-catheterization, was significantly shorter for CI-AKI cases compared to controls (49% versus 97%; P < 0.001) even in those with return to baseline renal function prior to discharge (60% versus 97%, P < 0.001).
CONCLUSION: In ACHD patients undergoing cardiac catheterization, a higher V/eGFR ratio is a strong predictor of clinically significant CI-AKI. Development of CI-AKI is a poor prognostic indicator and is associated with decreased survival in this population.

PMID: 30196559 [PubMed - as supplied by publisher]

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