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Development of Quality Metrics in Ambulatory Pediatric Cardiology.

J Am Coll Cardiol. 2017 Feb 07;69(5):541-555

Authors: Chowdhury D, Gurvitz M, Marelli A, Anderson J, Baker-Smith C, Diab KA, Edwards TC, Hougen T, Jedeikin R, Johnson JN, Karpawich P, Lai W, Lu JC, Mitchell S, Newburger JW, Penny DJ, Portman MA, Satou G, Teitel D, Villafane J, Williams R, Jenkins K, American College of Cardiology’s Adult Congenital and Pediatric Cardiology Section’s Ambulatory Pediatric Cardiology Quality Metrics Working Group

The American College of Cardiology Adult Congenital and Pediatric Cardiology (ACPC) Section had attempted to create quality metrics (QM) for ambulatory pediatric practice, but limited evidence made the process difficult. The ACPC sought to develop QMs for ambulatory pediatric cardiology practice. Five areas of interest were identified, and QMs were developed in a 2-step review process. In the first step, an expert panel, using the modified RAND-UCLA methodology, rated each QM for feasibility and validity. The second step sought input from ACPC Section members; final approval was by a vote of the ACPC Council. Work groups proposed a total of 44 QMs. Thirty-one metrics passed the RAND process and, after the open comment period, the ACPC council approved 18 metrics. The project resulted in successful development of QMs in ambulatory pediatric cardiology for a range of ambulatory domains.

PMID: 28153110 [PubMed - in process]

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Factors Associated with Readmission of Patients with Congenital Heart Disease in a Swiss University Hospital.

Pediatr Cardiol. 2017 Feb 02;:

Authors: Chave M, Marques-Vidal P

Congenital heart defects (CHD) lead to extensive use of healthcare resources. Still, there is little information available regarding readmission rates or associated factors. We sought to evaluate readmission rates and their determinants among patients with CHD hospitalized in a Swiss university hospital. We conducted a retrospective study using data from all non-adult (<18 years) patients hospitalized between 2002 and 2014 at the University Hospital of Lausanne with an International Classification of Diseases version 10 code Q20-Q25. Overall, 996 patients (460 girls, 332 undergoing surgery, mean age 2.7 years) were assessed, 96 of whom (9.6%) were readmitted within 30 days after discharge. Among the 96 readmissions, 83 (86.5%) were related to the CHD. Median time to readmission was 10 days (interquartile range 6-20) and median length of readmission was 12 days (interquartile range 6-20). After multivariate adjustment, foreign nationality, greater distance to hospital and length of index hospitalization <14 days predisposed to readmission. Patients who underwent surgery were less likely to be readmitted (8.7%). We conclude that readmissions were frequent, almost 1 in 10 patients, and associated with several socio-clinical factors. Providing patients who live far from hospital with specialized care closer to home may help reduce the rate of readmission.

PMID: 28154912 [PubMed - as supplied by publisher]

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Update on the Management of Adults With Arterial Switch Procedure for Transposition of the Great Arteries.

Curr Treat Options Cardiovasc Med. 2017 Jan;19(1):4

Authors: Safi LM, Bhatt AB

OPINION STATEMENT: The arterial switch operation (ASO) is now the most frequently performed surgical correction in individuals with dextro-transposition of the great arteries (D-TGA). Patients who undergo this procedure as neonates have overall good clinical outcomes yet continued clinical follow-up is important to evaluate for postoperative complications. In this group, the highest mortality is in the immediate postoperative period and is generally associated with reimplantation of the coronary arteries. As these patients live into adulthood, longitudinal follow-up for other ASO complications including neo-pulmonary stenosis, right ventricular outflow tract (RVOT) obstruction, or neo-aortic root dilation and resulting aortic insufficiency should be performed. In adults, extra care should be taken to identify and treat traditional cardiovascular risk factors as individuals with coronary obstruction may not present with typical anginal symptoms. Management of these patients should be performed in collaboration with an adult congenital heart center of excellence. This population offers a unique opportunity to provide timely feedback to adult congenital heart community of providers regarding late outcomes from surgical intervention and in the next decade will hopefully demonstrate a model for clinical feedback cycles in lifelong congenital care.

PMID: 28155117 [PubMed - in process]

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Exome Sequencing Identifies Compound Heterozygous Mutations in SCN5A Associated with Congenital Complete Heart Block in the Thai Population.

Dis Markers. 2016;2016:3684965

Authors: Thongnak C, Limprasert P, Tangviriyapaiboon D, Silvilairat S, Puangpetch A, Pasomsub E, Sukasem C, Chantratita W

Background. Congenital heart block is characterized by blockage of electrical impulses from the atrioventricular node (AV node) to the ventricles. This blockage can be caused by ion channel impairment that is the result of genetic variation. This study aimed to investigate the possible causative variants in a Thai family with complete heart block by using whole exome sequencing. Methods. Genomic DNA was collected from a family consisting of five family members in three generations in which one of three children in generation III had complete heart block. Whole exome sequencing was performed on one complete heart block affected child and one unaffected sibling. Bioinformatics was used to identify annotated and filtered variants. Candidate variants were validated and the segregation analysis of other family members was performed. Results. This study identified compound heterozygous variants, c.101G>A and c.3832G>A, in the SCN5A gene and c.28730C>T in the TTN gene. Conclusions. Compound heterozygous variants in the SCN5A gene were found in the complete heart block affected child but these two variants were found only in the this affected sibling and were not found in other unaffected family members. Hence, these variants in the SCN5A gene were the most possible disease-causing variants in this family.

PMID: 28018021 [PubMed - indexed for MEDLINE]

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Intrapericardial rupture of a mitral subannular aneurysm: A case report and review of the literature.

Ann Cardiol Angeiol (Paris). 2016 Apr;65(2):99-102

Authors: Diop IB, Leye M, Diallo AD, Sarr EH, Manga SJ, Diene LL, Jobe M

Mitral subannular aneurysm is a rare heart disease that can have many different forms of clinical presentations. It was first described in young men of African descent and was later reported in other geographical areas of the world. The etiopathogenesis as per data from the literature can be congenital, acquired or idiopathic. We report the case of a 19-year-old male in whom we made the diagnosis of mitral subannular aneurysm. The evolution was fatal following a rupture of the aneurysm into the pericardium. Through this case report, we stress the importance of echocardiography in the diagnosis of this condition. In resource-limited countries, the prognosis is unfortunately often unfavorable especially the ruptured forms. Therefore, a high degree of suspicion is needed to make a prompt diagnosis and timely surgical intervention.

PMID: 25702237 [PubMed - indexed for MEDLINE]

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Medium-term follow-up of renal function in hypoxaemic congenital heart disease patients.

Cardiol Young. 2016 Aug;26(6):1137-43

Authors: Martínez-Quintana E, Rodríguez-González F

UNLABELLED: Introduction Hypoxaemic congenital heart disease (CHD) patients are at higher risk of complications. The aim of this study was to compare and follow-up blood and 24-hour urine analytical data in hypoxaemic and non-hypoxaemic CHD patients.
METHODS: The inclusion criteria for this study were as follows: patients older than 14 years of age with a structural CHD with or without associated hypoxaemia.
RESULTS: In total, 27 hypoxaemic and 48 non-hypoxaemic CHD patients were included in order to compare blood and 24-hour urine analytical data. Among hypoxaemic patients, 13 (48.1%) were male, two (7.4%) had diabetes mellitus, one of whom was a smoker, one (3.7%) had systemic arterial hypertension, and 11 (40.7%) showed pulmonary arterial hypertension. The mean follow-up time was 3.1±1.9 years. Hypoxaemic CHD patients showed higher proteinuria concentrations (g/24 hours) (0.09 (0.07; 0.46) versus 0.08 (0.07; 0.1), p=0.054) and 24-hour albumin excretion rate (µg/min) (16.5 (11.2; 143.5) versus 4.4 (0.0; 7.6), p<0.001) compared with non-hypoxaemic CHD patients; however, no significant differences were found in the proteinuria levels and in the 24-hour albumin excretion rate in CHD patients with associated hypoxaemia, both at baseline and at follow-up. When divided into groups, hypoxaemic patients with palliative shunts showed significantly higher proteinuria concentrations compared with hypoxaemic patients not operated on or with Fontan procedures (p=0.01). No significant differences were seen in 24-hour proteinuria and 24-hour albumin excretion rate during the follow-up of patients with palliative shunts.
CONCLUSIONS: Hypoxaemic CHD patients have significant higher 24-hour proteinuria concentration and 24-hour albumin excretion rate compared with non-hypoxaemic CHD patients. Among hypoxaemic CHD patients, those with palliative shunts showed the highest 24-hour proteinuria concentrations.

PMID: 26381329 [PubMed - indexed for MEDLINE]

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Transcatheter closure of perimembranous ventricular septal defects with the Amplatzer Vascular Plug-II.

Cardiol Young. 2016 Aug;26(6):1194-201

Authors: Hua N, Aquino P, Owada CY

OBJECTIVE: The aim of this study was to evaluate the safety and efficacy of the Amplatzer Vascular Plug-II used for the closure of perimembranous ventricular septal defects.
BACKGROUND: There are no FDA-approved transcatheter devices for the closure of perimembranous ventricular septal defects. Several studies have reported on the use of various devices either off-label or under clinical trial protocols. However these reports have described significant adverse events including residual shunts, complete heart block, arrhythmia, and new valve regurgitations. Thus far, no study on the Amplatzer Vascular Plug-II has been reported.
METHODS: We conducted a 4-year retrospective chart review from August, 2010 to August, 2014, of patients with perimembranous ventricular septal defects associated with ventricular septal aneurysm who underwent transcatheter closure using the Amplatzer Vascular Plug-II.
RESULTS: A total of 16 patients underwent Amplatzer Vascular Plug-II transcatheter closure of their perimembranous ventricular septal defects. The median age was 2.56 years (range: 0.5-27.3). Their median weight was 13.0 kg (range: 6.9-71.6). The left ventricular median defect size was 9.3 mm (range: 5.9-14.4). The right ventricular median defect size was 3.6 mm (range: 2.3-5.8). All the patients underwent successful device implantation with 83% of the patients having complete echocardiographic closure at the 1-year follow-up; however, one procedure was complicated by early device embolisation. The device was successfully retrieved and replaced with a larger device. There were no device-related outflow tract obstructions, rhythm abnormalities, or haemolysis.
CONCLUSION: Application of the Amplatzer Vascular Plug-II for closure of perimembranous ventricular septal defects appears to be a safe and effective treatment option. Prospective clinical trials and longer follow-up periods are warranted.

PMID: 26498904 [PubMed - indexed for MEDLINE]

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Factors associated with the internal jugular venous approach for Melody™ Transcatheter Pulmonary Valve implantation.

Cardiol Young. 2016 Jun;26(5):948-56

Authors: Zampi JD, Berman DP, Bocks ML, Yu S, Zahn EM, Lu JC, Shaya JA, Armstrong AK

BACKGROUND: Transcatheter pulmonary valve implantation is usually performed from a femoral venous - transfemoral - approach, but this may not be the optimal vascular access option in some patients. This study aimed to determine which group of patients might benefit from an internal jugular - transjugular - approach for transcatheter pulmonary valve implantation.
METHODS: This multicentre retrospective study included all patients who underwent attempted transcatheter pulmonary valve placement in the right ventricular outflow tract between April 2010 and June 2012 at two large congenital heart centres. Patients were divided into two groups based on venous access site - transfemoral or transjugular. Patient characteristics, procedural outcomes, and complications were compared between groups.
RESULTS: Of 81 patients meeting the inclusion criteria (median age 16.4 years), the transjugular approach was used in 14 patients (17%). The transjugular group was younger (median age 11.9 versus 17.3 years), had lower body surface area (mean 1.33 versus 1.61 m2), more often had moderate or greater tricuspid regurgitation (29% versus 7%), and had a higher ratio of right ventricle-to-systemic systolic pressure (mean 82.4 versus 64.7). Patients requiring a transjugular approach after an unsuccessful transfemoral approach had longer fluoroscopic times and procedure duration.
CONCLUSIONS: The transjugular approach for transcatheter pulmonary valve implantation is used infrequently but is more often used in younger and smaller patients. Technical limitations from a transfemoral approach may be anticipated if there is moderate or greater tricuspid regurgitation or higher right ventricular pressures. In these patients, a transjugular approach should be considered early.

PMID: 26521753 [PubMed - indexed for MEDLINE]

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Replacement of Regurgitant Bicuspid Aortic Valve in a Dilated, Non-Compacted Left Ventricle.

World J Pediatr Congenit Heart Surg. 2016 May;7(3):389-93

Authors: Schenone AL, Cohen A, Pettersson G, Majdalany D

Bicuspid aortic valve (BAV) is the most common form of congenital heart disease, with 20% of asymptomatic adults with BAV presenting with significant valve insufficiency. Yet, limited data exist regarding surgical indications and outcomes when BAV is accompanied by left ventricular dilation, systolic dysfunction, or left ventricle non-compaction (LVNC) syndrome. We present a case of dilated cardiomyopathy due to severe BAV regurgitation and partial LVNC syndrome and the decision to undergo aortic valve replacement. Our patient represents the most extreme documented case of regurgitant BAV with dilated, dysfunctional, and partially non-compacted left ventricle. Yet, surgical intervention provided improvement in systolic performance and ventricular dimensions.

PMID: 26701622 [PubMed - indexed for MEDLINE]

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Infective endocarditis prophylaxis: current practice trend among paediatric cardiologists: are we following the 2007 guidelines?

Cardiol Young. 2016 Aug;26(6):1176-82

Authors: Naik RJ, Patel NR, Wang M, Shah NC

BACKGROUND: In 2007, the American Heart Association modified the infective endocarditis prophylaxis guidelines by limiting the use of antibiotics in patients with cardiac conditions associated with the highest risk of adverse outcomes after infective endocarditis. Our objective was to evaluate current practice for infective endocarditis prophylaxis among paediatric cardiologists.
METHODS: A web-based survey focussing on current practice, describing the use of antibiotics for infective endocarditis prophylaxis in various congenital and acquired heart diseases, was distributed via e-mail to paediatric cardiologists. The survey was kept anonymous and was distributed twice.
RESULTS: Data from 253 participants were analysed. Most paediatric cardiologists discontinued infective endocarditis prophylaxis in patients with simple lesions such as small ventricular septal defect, patent ductus arteriosus, and bicuspid aortic valve without stenosis or regurgitation; however, significant disagreement persists in prescribing infective endocarditis prophylaxis in certain conditions such as rheumatic heart disease, Fontan palliation without fenestration, and the Ross procedure. Use of antibiotic prophylaxis in certain selected conditions for which infective endocarditis prophylaxis has been indicated as per the current guidelines varies from 44 to 83%. Only 44% follow the current guidelines exclusively, and 34% regularly discuss the importance of oral hygiene with their patients at risk for infective endocarditis.
CONCLUSION: Significant heterogeneity still persists in recommending infective endocarditis prophylaxis for several cardiac lesions among paediatric cardiologists. More than half of the participants (56%) do not follow the current guidelines exclusively in their practice. Counselling for optimal oral health in patients at risk for infective endocarditis needs to be optimised in the current practice.

PMID: 26715655 [PubMed - indexed for MEDLINE]