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Transcatheter closure of unroofed coronary sinus using covered stents in an adult with drainage of the coronary sinus to the right ventricle after supra-annular tricuspid valve replacement.

Catheter Cardiovasc Interv. 2017 Dec 01;90(7):1154-1157

Authors: Mohammad Nijres B, Kenny D, Kazmouz S, Hijazi ZM

We present a rare case of unroofed coronary sinus in a patient who underwent supra-annular tricuspid valve replacement with consequent drainage of the coronary sinus to the right ventricle. It is unclear whether the coronary sinus was unroofed congenitally or iatrogenically. This rare setup resulted in significant cyanosis. The abnormal drainage was successfully closed via trans-catheter delivery of covered stents with resolution of the cyanosis. © 2017 Wiley Periodicals, Inc.

PMID: 28296217 [PubMed - indexed for MEDLINE]

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Postmortem therapy from a subcutaneous ICD: What is the mechanism?

Pacing Clin Electrophysiol. 2017 06;40(6):735-737

Authors: Wiles BM, Fitzsimmons SJ, Roberts PR

PMID: 28374449 [PubMed - indexed for MEDLINE]

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Anomalous origin of coronary arteries from the "wrong" sinus in athletes: Diagnosis and management strategies.

Int J Cardiol. 2018 Feb 01;252:13-20

Authors: Palmieri V, Gervasi S, Bianco M, Cogliani R, Poscolieri B, Cuccaro F, Marano R, Mazzari M, Basso C, Zeppilli P

AIMS: Although anomalous origin of left (AOLCA) and right coronary artery (AORCA) from the wrong sinus may cause sudden death (SD) in athletes, early diagnosis and management of these anomalies are still challenging. We analysed clinical/instrumental profiles of athletes identified with AOLCA/AORCA focusing our attention on diagnosis, management and follow-up.
METHODS AND RESULTS: We report 23 athletes (17 males, mean age 27±17yrs.), 6 with AOLCA and 17 with AORCA. Diagnosis was made by trans-thoracic echocardiography (TTE) in 21/23(91%). Symptoms were present only in 10(41%). Only 3 had an abnormal rest-ECG and 9(39%) an abnormal stress test ECG (3 ST-depression, 4 ventricular arrhythmias, 1 supraventricular arrhythmias, 1 rate-dependent left-bundle-branch-block). Anatomy of the anomalous coronary artery showed no significant correlation with clinical presentation, except for a tendency to higher occurrence of proximal hypoplasia in symptomatic athletes (83% vs 40%, p=0.09). All athletes were disqualified from competitive-sports and advised to avoid strenuous effort. Surgery was recommended to all athletes with AOLCA and 6 with AORCA, but only 6 underwent surgery. No major cardiac events or ischemic symptoms/signs occurred during a mean follow-up of 65±70months.
CONCLUSIONS: Early diagnosis of AOLCA/AORCA in athletes is feasible by TTE. Typical symptoms/signs of myocardial ischemia are present only in one third of cases thus underlying the need of a high index of clinical suspicion to achieve the diagnosis. After exercise restriction, none had major cardiac events or ischemia symptoms/signs recurrence. There was no correlation between anatomical characteristics and clinical presentation with the possible exception of coronary hypoplasia.

PMID: 29146296 [PubMed - indexed for MEDLINE]

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Targeted next-generation sequencing identified ADAMTS5 as novel genetic substrate in patients with bicuspid aortic valve.

Int J Cardiol. 2018 Feb 01;252:150-155

Authors: Lin X, Liu X, Wang L, Jiang J, Sun Y, Zhu Q, Chen Z, He Y, Hu P, Xu Q, Gao F, Lin Y, Jaiswal S, Xiang M, Wang J

BACKGROUND: Bicuspid Aortic Valve (BAV) is the most common congenital heart disease, affecting >1% of the general population. Up to date, three genes, NOTCH1, GATA5 and SMAD6, have been linked to the isolated form of BAV. However, potential genetic determinants remain largely unknown in most BAV patients.
MATERIAL AND METHODS: Targeted next-generation sequencing of 7 BAV candidate genes (NOTCH1, GATA5, SMAD6, NOS3, ADAMTS5, Alk2 and SMAD2) was performed in 32 BAV patients. Additional 35 BAV patients and 238 tricuspid aortic valve (TAV) patients, consisting of 107 patients from the transcatheter aortic valve implantation (TAVI) registry and 131 patients from the coronary artery disease (CAD) registry, were selected for further genotyping.
RESULTS: We found 2 rare non-synonymous variants in 2/7 genes in 3 BAV patients: one was NOTCH1:c.4297G>A and the other one was ADMTS5:c.935C>A that shared by two patients. NOTCH1:c.4297G>A has not been reported previously. ADMTS5:c.935C>A was predicted to be pathogenic by all applied algorithms. Alignment of protein sequences from all available species revealed that ADMTS5:p.Arg312Leu, produced by ADMTS5:c.935C>A, is located in a highly conserved region. The minor allele frequency of ADMTS5:c.935C>A in BAV patients was significantly higher than the matched population in TAV group (0.015 vs. 0, P=0.048).
CONCLUSION: Our results suggested that ADMTS5:c.935C>A are potentially associated with BAV. Further studies, such as large sample case-control replication test and functional research, are needed to explore the role of this rare variant in the development of BAV.

PMID: 29162281 [PubMed - indexed for MEDLINE]

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Prognostic power of cardiopulmonary exercise testing in Fontan patients: a systematic review.

Open Heart. 2018;5(1):e000812

Authors: Udholm S, Aldweib N, Hjortdal VE, Veldtman GR

Objective: Exercise impairment is common in Fontan patients. Our aim is to systematically review previous literature to determine the prognostic value of exercise capacity in older adolescent and adult Fontan patients with respect to late outcome. Additionally, we reviewed the determinants of exercise capacity in Fontan patients and changes in exercise capacity over time.
Methods: PubMed, CINAHL, Embase, The Cochrane Library and Scopus were searched systematically for studies reporting exercise capacity and late outcome such as mortality, cardiac transplantation and hospitalisation. Studies were eligible for inclusion if more than 30 patients were included and mean age was ≥16 years.
Results: Four thousand and seven hundred and twenty-two studies were identified by the systematic search. Seven studies fulfilled the inclusion and exclusion criteria. The total number of patients was 1664 adult Fontan patients. There were 149 deaths and 35 heart transplantations. All eligible studies were retrospective cohort studies. The correlation between exercise capacity and late outcome was identified, and HRs were reported.
Conclusion: In Fontan patients, the best predictors of death and transplantation were a decline in peak VO2, heart rate variables and exercise oscillatory ventilation. Peak VO2 was not strongly predictive of mortality or hospitalisation in Fontan patients. Several variables were strong and independent predictors of hospitalisation and morbidity.

PMID: 30057765 [PubMed]

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Congenital pulmonary vascular anomalies.

Cardiovasc Diagn Ther. 2018 Jun;8(3):214-224

Authors: Dimas VV, Dillenbeck J, Josephs S

Congenital pulmonary vascular anomalies are typically found in infancy or early childhood however, some may remain silent and present in adult patients. Anomalies may be separated into anatomic categories based on involvement of the pulmonary arteries, pulmonary veins or both with or without involvement of the lung parenchyma. Association with congenital heart disease and other syndromes is very common. Computed tomography (CT) and magnetic resonance imaging (MRI) are both invaluable at assessment of these anomalies allowing for both diagnosis and detailed treatment planning. This article will focus primarily on the use of CT, as the high resolution evaluation of the lung parenchyma is also important in many of these conditions. In young patients especially, rapid heart rate and concerns of radiation exposure are important considerations when performing CT. This article will discuss scan techniques as well as clinical diagnostic considerations and basic endovascular treatment of congenital pulmonary vascular anomalies.

PMID: 30057871 [PubMed]

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Characteristics of hospital admissions associated with implantable cardioverter defibrillator placement among adults with congenital heart disease.

Int J Cardiol. 2018 Jul 24;:

Authors: Baskar S, Veldtman GR, Khoury PR, Opotowsky AR, Cedars AM

BACKGROUND: Characteristics of hospitalizations including healthcare utilization for adult patients with congenital heart disease (ACHD) at the time of implantable cardioverter defibrillator (ICD) placement has not been well studied.
METHODS: We analyzed data from the 2002-2014 United States National Inpatient Sample (NIS). ICD implantation, CHD, complications, and indications for admissions were determined based on diagnostic codes among adults. Propensity score matching was performed, based on age, sex and in-hospital mortality index with a 10:1 ratio between adults without CHD and those with CHD, to determine relative healthcare utilization attributable to CHD.
RESULTS: ACHD accounted for 136,509 ± 3488 admissions of which 1451 ± 121 admissions (1.1 ± 0.06%) were associated with an ICD placement. ICD placement occurred most frequently among patients with TOF, VSD, and transposition complexes usually in the context of a dysrhythmia. Compared to those without CHD, ACHD patients had higher adjusted total hospital charges ($147,002 ± 5516 vs $132,455 ± 2182; p < 0.001), length of stay (6.2 ± 0.5 vs 5.2 ± 0.1 days; p < 0.001), lower readmission score (5.5 ± 0.5 vs 9.7 ± 0.1; p = 0.04) and a higher complication rate (13.4% vs 8.3%; p < 0.001). Dysrhythmias were more frequently the primary diagnosis for admission in the ACHD cohort (63% vs 38%; p < 0.001).
CONCLUSION: Compared to a matched non-CHD population, ACHD patients had greater healthcare utilization and had more frequent complications. The reasons underlying this difference bear investigation to improve care quality.

PMID: 30060972 [PubMed - as supplied by publisher]

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The Challenge of Timing Surgery in Degenerative Mitral Regurgitation: Is B-Type Natriuretic Peptide the Solution?

J Am Coll Cardiol. 2016 09 20;68(12):1308-11

Authors: Baumgartner H

PMID: 27634122 [PubMed - indexed for MEDLINE]

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Folate, vitamin B12, homocysteine and polymorphisms in folate metabolizing genes in children with congenital heart disease and their mothers.

Eur J Clin Nutr. 2017 Dec;71(12):1437-1441

Authors: Elizabeth KE, Praveen SL, Preethi NR, Jissa VT, Pillai MR

BACKGROUND/OBJECTIVES: The objective of the study was to assess the role of variations in serum folate, vitamin B12, homocysteine and the presence of genetic polymorphisms as risk factors for congenital heart disease (CHD) in children.
SUBJECTS/METHODS: A total of 32 children with CHD, and their mothers and 32 normal children and their mothers formed the study and control groups, respectively. Serum folate, vitamin B12 and homocysteine as well as genetic polymorphisms MTHFR C677→︀T, MTHFR A1298→︀C, MTR A2756→︀G and MTRR A66→︀G were assessed.
RESULTS: Low serum folate and genetic polymorphisms MTHFR C677→︀T and MTRR A66→︀G among children and their mothers and high homocysteine among mothers were noted as risk factors for CHD (P<0.05). Vitamin B12 levels were normal and showed no association. Presence of MTHFR C677→︀T and MTRR A66→︀G, both concurrently among children as well as mothers and simultaneously among mother-child pairs, showed several fold increase in the risk for CHD. On multivariate analysis, the risk factors noted for CHD were presence of MTHFR C677→︀T among children and their mothers and MTRR A66→︀G among mothers. Analyses for nutrient-gene interaction revealed significant associations between low serum folate and high serum homocysteine levels, and the presence of selected genetic polymorphisms.
CONCLUSIONS: Low serum folate, high homocysteine and presence of selected genetic polymorphisms among children and their mothers were noted as risk factors for CHD. Nutrient-gene interaction being a modifiable risk factor, the study recommends the use of peri-conceptional folate supplementation with vitamin B12 sufficiency for primary prevention of CHD.

PMID: 28876333 [PubMed - indexed for MEDLINE]

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Chemotherapy and echocardiographic indices in patients with non-Hodgkin lymphoma: the ONCO-ECHO study.

Med Oncol. 2017 Dec 22;35(1):14

Authors: Mizia-Stec K, Elżbieciak M, Wybraniec MT, Różewicz M, Bodys A, Braksator W, Gąsior Z, Gościniak P, Hryniewiecki T, Kasprzak J, Wojtarowicz A, Zdziarska B, Płońska-Gościniak E

The cardiotoxicity of chemotherapy (CTx) for non-Hodgkin's lymphomas is not well recognized. In order to facilitate individual risk counseling for patients, we analyzed the effect of CTx on echocardiographic indices in regard to clinical data in patients treated for non-Hodgkin's lymphoma (NHL). A prospective multicenter ONCO-ECHO trial included 67 patients with NHL (45 patients with DLBCL (diffuse large B cell lymphoma) and 22 with non-DLBCL). Patients received standard CTx, primarily R-CHOP, CHOP, R-COP and COP regimens. Clinical data and echocardiographic indices were obtained at baseline, 3-, 6- and 12-month follow-up. The primary end point representing CTx cardiotoxicity was defined as a ≥ 10% decrease in the left ventricular ejection fraction (LVEF) during 12-month observation. In a 12-month follow-up five (7.5%) deaths occurred, while no clinical manifestations of heart failure were reported. There was an increase in left ventricular end-systolic diameter (p = 0.002) and E/e' index (p = 0.036) in 12-month observation. Preexisting coronary artery disease was associated with significant decrease in the ΔLVEF (p = 0.008), increase in ΔLVEDV (p = 0.03) and ΔLVESV (p = 0.02) and increase in the Δ left atrium diameter (p = 0.02); while history of arterial hypertension was related to significant decrease in the ΔLVEF (p = 0.039), diabetes mellitus was related to significant increase in the ΔE/e' index (p = 0.002). The primary end point was reported in ten (14.9%) patients. There were no independent risk factors for cardiotoxicity in the study population. Chemotherapy administered to NHL patients may induce dilatation and impaired LV diastolic function. Standard cardiovascular risk factors may predispose patients to negative LV remodeling.

PMID: 29274027 [PubMed - indexed for MEDLINE]