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Coronary anatomy in children with bicuspid aortic valves and associated congenital heart disease.

Heart. 2018 03;104(5):385-393

Authors: Koenraadt WMC, Bartelings MM, Bökenkamp R, Gittenberger-de Groot AC, DeRuiter MC, Schalij MJ, Jongbloed MR

Abstract
OBJECTIVE: In patients with bicuspid aortic valve (BAV), coronary anatomy is variable. High take-off coronary arteries have been described, but data are scarce, especially when associated with complex congenital heart disease (CHD). The purpose of this study was to describe coronary patterns in these patients.
METHODS: In 84 postmortem heart specimens with BAV and associated CHD, position and height of the coronary ostia were studied and related to BAV morphology.
RESULTS: High take-off right (RCA) and left coronary arteries (LCA) were observed in 23% and 37% of hearts, respectively, most frequently in hearts with hypoplastic left ventricle (HLV) and outflow tract anomalies. In HLV, high take-off was observed in 18/40 (45%) more frequently of LCA (n=14) than RCA (n=6). In hearts with aortic hypoplasia, 8/13 (62%) had high take-off LCA and 6/13 (46%) high take-off RCA. High take-off was seen 19 times in 22 specimens with perimembranous ventricular septal defect (RCA 8, LCA 11). High take-off was associated with type 1A BAV (raphe between right and left coronary leaflets), more outspoken for the RCA. Separate ostia of left anterior descending coronary artery and left circumflex coronary artery were seen in four hearts (5%), not related to specific BAV morphology.
CONCLUSION: High take-off coronary arteries, especially the LCA, occur more frequently in BAV with associated CHD than reported in normal hearts and isolated BAV. Outflow tract defects and HLV are associated with type 1A BAV and high take-off coronary arteries. Although it is unclear whether these findings in infants with detrimental outcome can be related to surviving adults, clinical awareness of variations in coronary anatomy is warranted.

PMID: 28751538 [PubMed - indexed for MEDLINE]

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Intra-atrial re-entrant tachycardia in congenital heart disease: types and relation of isthmus to atrial voltage.

Europace. 2018 02 01;20(2):353-361

Authors: Roca-Luque I, Rivas Gándara N, Dos Subirà L, Pascual JF, Domenech AP, Pérez-Rodon J, Subirana MT, Santos Ortega A, Miranda B, Rosés-Noguer F, Ferreira-Gonzalez I, Ferrer JC, García-Dorado García D, Mitjans AM

Abstract
Background: Intra-atrial re-entrant tachycardia (IART) is a frequent and severe complication in patients with congenital heart disease (CHD). Cavotricuspid isthmus (CTI)-related IART is the most frequent mechanism. However, due to fibrosis and surgical scars, non-CTI-related IART is also frequent.
Objective: The main objective of this study was to describe the types of IART and circuit locations and to define a cut-off value for unhealthy tissue in the atria.
Methods and results: This observational study included all consecutive patients with CHD who underwent a first ablation procedure for IART from January 2009 to December 2015 (94 patients, 39.4% female, age: 36.55 ± 14.9 years, 40.4% with highly complex cardiac disease). During the study, 114 IARTs were ablated (1.21 ± 0.41 IARTs per patient). Cavotricuspid isthmus-related IART was the only arrhythmia in 51% (n = 48) of patients, non-CTI-related IART was the only mechanism in 27.7% (n = 26), and 21.3% of patients (n = 20) presented both types of IART. In cases of non-CTI-related IART, the most frequent location of IART isthmus was the lateral or posterolateral wall of the venous atria, and a voltage cut-off value for unhealthy tissue in the atria of 0.5 mV identified 95.4% of IART isthmus locations.
Conclusion: In our population with a high proportion of complex CHD, CTI-related IART was the most frequent mechanism, although non-CTI-related IART was present in 49% of patients (alone or with concomitant CTI-related IART). A cut-off voltage of 0.5 mV could identify 95.4% of the substrates in non-CTI-related IART.

PMID: 29016802 [PubMed - indexed for MEDLINE]

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Patients' knowledge and attitudes regarding living with implantable electronic devices: results of a multicentre, multinational patient survey conducted by the European Heart Rhythm Association.

Europace. 2018 02 01;20(2):386-391

Authors: Haugaa KH, Potpara TS, Boveda S, Deharo JC, Chen J, Dobreanu D, Fumagalli S, Lenarczyk R, Hernandez Madrid A, Larsen TB, Sciarrafia E, Taborsky M, Tilz RR, Pieragnoli P, Przybylski A, Dagres N

Abstract
The purpose of this patient survey was to analyse the knowledge, experiences, and attitudes regarding cardiac implantable electronic devices (CIED) in patients with pacemakers, implantable cardioverter-defibrillators (ICDs), or cardiac resynchronization devices. Of the 1644 patients with CIEDs from seven European countries, 88% were over 50 years of age. Most patients (90%) knew what device they were implanted with and felt sufficiently informed about the indications for therapy. As many as 42% of patients needed additional information on the battery replacement and limitations in physical activity. The self-reported incidence of complications was 9%, and among these, a quarter of the respondents felt insufficiently informed about the possibility of complications and their management. The majority of patients (83%) were followed by face-to-face visits, which was the most commonly preferred follow-up strategy by the patients. Nearly 75% of the patients reported improved quality of life after device implantation, but about 40% had worries about their device. Less than 20% had discussed with their physician or thought about device handling in the end-of-life circumstances or end-stage disease. Notably, almost 20% of the ICD patients did not wish to answer the question regarding what they wanted to be done with their ICD in case of end-stage disease, indicating the challenges in approaching these issues.

PMID: 29300970 [PubMed - indexed for MEDLINE]

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The effects of supraphysiological oestrogen levels on ventricular repolarisation parameters.

Kardiol Pol. 2018;76(6):974-979

Authors: Yiginer O, Dogan M, Gun I, Kutlu HT, Degirmencioglu G, Guliyev İ, Tokatli A, Kilicaslan F

Abstract
BACKGROUND: The frequency of arrhythmic death developing without a structural cardiac disease is higher in women. Also, female sex is an independent risk factor regarding development of torsades de pointes. Several studies have been conducted on the physiological and therapeutic effects of sex hormones on the cardiac conduction system.
AIM: In this study we aim to examine the effect of hormonal changes, especially supraphysiological E2 level changes occurring during in vitro fertilisation treatment, on ventricular repolarisation parameters.
METHODS: The study included female patients aged between 23 and 39 years, who were administered controlled ovarian hyperstimulation treatment. Patients' electrocardiograms and blood samples were obtained and analysed before and after the ovarian hyperstimulation treatment.
RESULTS: Mean QTc intervals before ovarian hyperstimulation were 411.9 ± 23.7 ms. Measurements during oestradiol peak were calculated as 420.7 ± 23.3 ms, and the QTc interval increase was significant (p = 0.007). Corrected QT dispersion averages were not significant before or after hyperstimulation (53 ± 17 ms vs. 54.5 ± 18.2 ms, respectively, p > 0.05). Tp-e, J-T peak, and PR dispersion changes were not significant after the ovarian hyperstimulation therapy.
CONCLUSIONS: Supraphysiological oestradiol levels that occur during controlled ovarian hyperstimulation cause prolongation of QTc intervals, but not to a pathological level. Although this prolongation is not significant in healthy individuals, it might increase ventricular arrhythmia risk in patients with congenital long QT syndrome and in patients taking medication that prolongs QT.

PMID: 29399762 [PubMed - indexed for MEDLINE]

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A novel double snare technique to retrieve embolized septal and left atrial appendage occluders.

J Interv Cardiol. 2018 Oct;31(5):685-692

Authors: Ha KS, Choi JY, Jung SY, Kim JS, Byun KH, Akagi T, Zufarov MM

Abstract
BACKGROUND: Device embolization is the most frequent procedural complication during transcatheter closure of congenital cardiac defects. Retrieval of an embolized device may often be complicated by failure to introduce the right atrial (RA) disk hub into the sheath or difficulty in securely grasping the hub pin of RA disk. We aimed to evaluate the efficiency and success rate of device retrieval using a novel double snare technique.
METHODS: We reviewed retrieval procedures of embolized atrial septal defect (ASD) or left atrial appendage (LAA) occluder using double snare technique reported from five tertiary referral centers in Korea, Japan, and Uzbekistan. A total of 16 retrieval procedures in 15 patients were reported, including 14 patients who were planned for ASD device closure while 1 patient was planned for LA appendage occlusion.
RESULTS: Retrieved devices included 15 ASD occluders from six different manufacturers and one Amplantzer cardiac plug. Success rate of retrieval procedure was 100% using the double snare technique. There were no complications related to device retrieval. Most (15/16, 93.8%) of these devices could be retrieved through their original delivery sheaths. In six patients for whom retrieval was unsuccessful with conventional single snare technique and switched to double snare technique, the retrieval time was shortened significantly (P = 0.004*) by using the double snare technique.
CONCLUSIONS: The double snare technique enables effective retrieval of various embolized devices. It abolishes the need of changing the sheath to a larger one in most patients.

PMID: 29696697 [PubMed - indexed for MEDLINE]

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Coronary Artery-Left Ventricular Fistula and Takotsubo Cardiomyopathy - An Association or an Incidental Finding? A Case Report.

Am J Case Rep. 2018 May 29;19:614-618

Authors: Taha ME, Al-Khafaji J, Abdalla AO, Wilson CR

Abstract
BACKGROUND A coronary artery-left ventricular fistula is an anomalous communication between the coronary arteries and the cardiac chambers and is a rare congenital coronary anomaly that is often small and asymptomatic. Takotsubo cardiomyopathy, on the other hand, is a syndrome characterized by transient regional systolic dysfunction of the left ventricle, mimicking myocardial infarction, but in the absence of angiographic evidence of obstructive coronary artery disease or acute plaque rupture. We present the case of an elderly woman who presented with Takotsubo cardiomyopathy and who was incidentally discovered to have an associated coronary artery-left ventricular fistula. CASE REPORT We report the case of a 68-years-old woman with a family history of premature cardiac diseases who presented with ischemic chest pain and elevated troponin levels. Her EKG and troponins were suggestive of non-ST-elevation myocardial infarction (NSTEMI), for which she was initially treated medically and later underwent coronary angiography. Unexpectedly, the angiography revealed patent coronary arteries, and we discovered evidence of coronary artery to left ventricular fistula in the addition to angiographic evidence of Takotsubo cardiomyopathy. A working diagnosis of Takotsubo was made, for which she was treated medically with resulting improvement of her symptoms and later in the imaging findings. CONCLUSIONS This described case illustrates a rare association between coronary artery fistulas and Takotsubo cardiomyopathy. It is unclear if this association has played a role in the pathogenesis or perhaps is just an incidental finding. More similar cases are needed to expand the clinical presentation of both conditions and add to the literature.

PMID: 29807977 [PubMed - indexed for MEDLINE]

Related Articles

Impact of Percutaneous Pulmonary Valve Implantation on the Timing of Reintervention for Right Ventricular Outflow Tract Dysfunction.

Rev Esp Cardiol (Engl Ed). 2018 Oct;71(10):838-846

Authors: de Torres-Alba F, Kaleschke G, Baumgartner H

Abstract
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. Early surgical repair has dramatically improved the outcome of this condition. However, despite the success of contemporary approaches with early complete repair, these are far from being curative and late complications are frequent. The most common complication is right ventricle outflow tract (RVOT) dysfunction, affecting most patients in the form of pulmonary regurgitation, pulmonary stenosis, or both, and can lead to development of symptoms of exercise intolerance, arrhythmias, and sudden cardiac death. Optimal timing of restoration of RVOT functionality in asymptomatic patients with RVOT dysfunction after TOF repair is still a matter of debate. Percutaneous pulmonary valve implantation, introduced almost 2 decades ago, has become a major game-changer in the treatment of RVOT dysfunction. In this article we review the pathophysiology, the current indications, and treatment options for RVOT dysfunction in patients after TOF repair with a focus on the role of percutaneous pulmonary valve implantation in the therapeutic approach to these patients.

PMID: 29859895 [PubMed - indexed for MEDLINE]

Durability of pulmonary homografts for reconstruction of the right ventricular outflow tract: how relevant are donor-related factors?

Interact Cardiovasc Thorac Surg. 2018 Nov 23;:

Authors: Dekens E, Van Damme E, Jashari R, Van Hoeck B, François K, Bové T

Abstract
OBJECTIVES: Our goal was to evaluate the influence of donor-related factors on the durability of pulmonary homografts (PHGs) for right ventricular outflow tract reconstruction of congenital heart defects.
METHODS: Between 1990 and 2016, 223 PHGs were used in 197 patients for right ventricular outflow tract reconstruction. Long-term durability was investigated in relation to patient- and disease-specific as well as to donor-related factors, based on the PHG replacement rate. To minimize the effect of outgrowth, a subgroup analysis was performed on patients with PHG size >22 mm, as the discriminant cut-off identified by the classification tree method.
RESULTS: During a median follow-up of 8.5 years [interquartile range (IQR) 12.3], 47 (21%) PHGs were explanted within a mean interval of 9.5 ± 5.3 years, resulting in a freedom from PHG replacement of 82 ± 6% at 10 years. The risk factors for PHG explantation determined by univariable analysis were predominantly patient-related, including younger age (P = 0.003), extra-anatomic implantation (P = 0.006), bicuspidalization (P = 0.002) and younger donor age (P = 0.032). PHG size [hazard ratio (HR) 0.80, 95% confidence interval 0.73-0.88; P < 0.001] was the only independent determinant in multivariable analysis. The subgroup analysis comprised 119 PHG >22 mm, implanted at a median age of 15 years (IQR 7). A significant beneficial effect of ABO matching on the explantation rate was only identified with univariable analysis (HR 0.24, 95% confidence interval 0.12-4.68; P = 0.010).
CONCLUSIONS: Cryopreserved PHGs provide a durable substitute for right ventricular outflow tract reconstruction in congenital heart disease. PHG size at the time of implantation remains the principal determinant of PHG explantation during late follow-up. However, once an adult-sized homograft is required, matching for ABO blood group compatibility between host and donor might help to improve homograft durability.

PMID: 30476047 [PubMed - as supplied by publisher]

Isolated Congenital Dysgenesia of a Pulmonary Valve Cusp Presenting as Pulmonary Artery Aneurysm.

Ann Thorac Surg. 2018 Nov 23;:

Authors: Saedi S, Rezaei-Kalantari K, Parsaee M, Behjati M, Hosseini S

Abstract
Pulmonary artery aneurysm is a rare disorder with the underlying pathology remaining obscure in many instances. More common causes include pulmonary hypertension, post stenotic dilation or vasculitis . In this case series we report three patients presenting with isolated pulmonary artery aneurysms in whom multi-modality imaging revealed the dysgenesia of a single pulmonary valve cusp as the cause of the huge pulmonary aneurysm.

PMID: 30476478 [PubMed - as supplied by publisher]

Related Articles

The outcome of adults born with pulmonary atresia: High morbidity and mortality irrespective of repair.

Int J Cardiol. 2018 Nov 07;:

Authors: Montanaro C, Merola A, Kempny A, Alvarez-Alvarez B, Alonso-Gonzalez R, Swan L, Uebing A, Li W, Babu-Narayan SV, Gatzoulis MA, Dimopoulos K

Abstract
OBJECTIVES: To describe the characteristics and long-term outcome of a large adult cohort with pulmonary atresia.
BACKGROUND: Patients with pulmonary atresia (PA) are a heterogeneous population in terms of anatomy, physiology and surgical history, and their management during adulthood remains challenging.
METHODS: Data on all patients with PA followed in our center between January 2000 and March 2015 were recorded. Patients were classified into the following groups: PA with ventricular septal defect (PA-VSD, 1), PA with intact ventricular septum (PA-IVS, 2) and other miscellaneous PA (PA-other, 3).
RESULTS: Two-hundred twenty-seven patients with PA were identified, 66.1% female, mean age 25.5 ± 8.7 years. Over a median follow-up of 8.8 years, 49 (21.6%) patients had died: heart failure (n = 21, 42.8%) and sudden cardiac death (n = 8, 16.3%) were the main causes. There was no significant difference in mortality between the 3 Groups (p = 0.12) or between repaired and unrepaired patients in Group 1 (p = 0.16). Systemic ventricular dysfunction and resting oxygen saturations were the strongest predictors of mortality. Additionally, 116 (51%) patients were hospitalized, driven mainly by the need for invasive procedures, heart failure and arrhythmias.
CONCLUSIONS: Adult survivors with pulmonary atresia have a high morbidity and mortality irrespective of underlying cardiac anatomy and previous reparative or palliative surgery. We present herewith predictors of outcome in adult life that may assist with their tertiary adult congenital care.

PMID: 30477927 [PubMed - as supplied by publisher]

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