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Iatrogenic propagation of coronary dissection during diagnostic coronary angiography: an uncommon but important procedural consideration.

BMJ Case Rep. 2017 Dec 14;2017:

Authors: Desai CK, Bhatnagar U, Stys A, Jonsson O

Spontaneous coronary artery dissection is an uncommon cause of acute myocardial infarction in the general population but is relatively more common in the peripartum period. Regardless of clinical setting, the management strategy is individualised, ranging from conservative to invasive. We report a case of peripartum myocardial infarction due to spontaneous coronary dissection that propagated during diagnostic angiography and ultimately required emergent bypass surgery.

PMID: 29246933 [PubMed - indexed for MEDLINE]

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Anticoagulation practices in adults with congenital heart disease and atrial arrhythmias in Switzerland.

Congenit Heart Dis. 2018 Jul 22;:

Authors: Arslani K, Notz L, Zurek M, Greutmann M, Schwerzmann M, Bouchardy J, Engel R, Attenhofer Jost C, Tobler D, SACHER investigators

BACKGROUND: In adults with congenital heart disease (CHD) and atrial arrhythmias, recommendations for thromboprophylaxis are vague and evidence is lacking. We aimed to identify factors that influence decision-making in daily practice.
METHODS: From the Swiss Adult Congenital HEart disease Registry (SACHER) we identified 241 patients with either atrial fibrillation (Afib) or atrial flutter/intraatrial reentrant tachycardia (Aflut/IART). The mode of anticoagulation was reviewed. Logistic regression models were used to assess factors that were associated with oral anticoagulation therapy.
RESULTS: Compared with patients with Aflut/IART, patients with Afib were older (51 ± 16.1 vs 37 ± 16 years, P < .001) and had a higher CHA2 DS2 -VASc (P < .001) and HAS-BLED scores (P = .005). Patients with Afib were more likely on oral anticoagulation than patients with Aflut/IART (67% vs 43%, P < .001). In a multivariate logistic regression model, age [odds ratio (OR) 1.03 per year, 95%CI (1.01-1.05), P = .019], atrial fibrillation [OR 2.75, 95%CI (1.30-5.08), P = .007], non-paroxysmal atrial arrhythmias [OR 5.33, 95%CI (2.21-12.85)], CHA2 DS2 -VASc-Score >1 [OR 2.93, 95%CI (1.87-4.61), P < .001], and Fontan palliation [OR 17.5, 95%CI (5.57-54.97), P < .001] were independently associated with oral anticoagulation treatment, whereas a HAS-BLED score >1 was associated with absence of thromboprophylaxis [OR 0.32, 95%CI (0.17-0.60), P < .001].
CONCLUSIONS: In this multicenter study, age, type, and duration of atrial arrhythmias, CHA2 DS2 -VASc and HAS-BLED scores as well as a Fontan palliation had an impact on the use of thromboprophylaxis in adult CHD patients with atrial arrhythmias. In daily practice, anticoagulation strategies differ between patients with Afib and those with Aflut/IART. Prospective observational studies are necessary to clarify whether this attitude is justified.

PMID: 30033686 [PubMed - as supplied by publisher]

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Fetal echocardiography for early detection of congenital heart diseases.

J Echocardiogr. 2017 Mar;15(1):13-17

Authors: Chitra N, Vijayalakshmi IB

BACKGROUND: Fetal echocardiography is a complete two-dimensional and Doppler ultrasound evaluation of the human fetal cardiovascular system. It is completely noninvasive, harmless, and also serves as the fetal electrocardiogram.
AIM: To analyze the fetal echocardiographic cases referred to a tertiary cardiac center.
MATERIALS, METHODS, AND RESULTS: A total of 478 cases of fetal echocardiograms performed over a period of 5.5 years were compiled and analyzed. Details regarding gestational age, maternal, family history, exposure to teratogens, and reason for referral were recorded. The average gestational age at referral was 24.8 ± 4.6 weeks and maternal age was 24.7 ± 4.3 years. Indications for referrals were abnormal fetal cardiac scan in 128 (26.8 %), previous sibling with congenital heart disease (CHD) in 99 (20.7 %), maternal indications in 87 (18.2 %), echogenic intracardiac focus (EIF) in 87 (18.2 %), high risk in 50 (10.5 %), rhythm problems in 21 (4.4 %), and others (extracardiac malformations) in 6 (1.2 %). In the 87 CHD cases diagnosed, nearly 70 % had complex CHD and 66.7 % were referred only after 22 weeks of gestation. A further 103 cases had EIF, 17 cases had fetal arrhythmia, 3 cases had cardiac masses, and the remaining 268 cases had normal fetal echocardiograms.
CONCLUSION: Fetal echocardiography can effectively identify abnormal hearts and has enhanced prenatal detection of CHD. The concern in the Indian scenario is the late referrals, lack of follow-up, and financial difficulties, all of which conspire against the chance of the fetus with heart disease getting appropriate treatment.

PMID: 27530200 [PubMed - indexed for MEDLINE]

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Familial Ebstein Anomaly: Whole Exome Sequencing Identifies Novel Phenotype Associated With FLNA.

Circ Cardiovasc Genet. 2017 Dec;10(6):

Authors: Mercer CL, Andreoletti G, Carroll A, Salmon AP, Temple IK, Ennis S

BACKGROUND: Familial Ebstein anomaly is a rare form of congenital heart disease. We report 7 individuals among 2 generations of 1 family with Ebstein anomaly. This family was first reported in 1991 by Balaji et al in which family members were also reported to have a mild skeletal phenotype. The most likely mechanism of inheritance was concluded to be autosomal dominant. We sought to identify the genetic pathogenesis in this family using a next generation sequencing approach.
METHODS AND RESULTS: Whole exome sequencing was performed in 2 cousins in this family using the Agilent SureSelect Human all Exon 51 Mb version 5 capture kit. Data were processed through an analytic in-house pipeline. Whole exome sequencing identified a missense mutation in FLNA (Filamin A), an actin-binding protein located at Xq28, mutations in which are associated with the skeletal phenotypes Frontometaphyseal dysplasia, Otopalatodigital, and Melnick-Needles syndrome, with X-linked periventricular nodular heterotopia and FG syndrome (Omim, 305450). Review of the phenotypes of those with the mutation in this family shows increased severity of the cardiac phenotype and associated skeletal features in affected males, consistent with X-linked inheritance.
CONCLUSIONS: Although congenital heart disease is reported in families with mutations in FLNA, this is the first report of individuals being affected by Ebstein anomaly because of a mutation in this gene and details the concurrent skeletal phenotype observed in this family.

PMID: 29237676 [PubMed - indexed for MEDLINE]

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Multidisciplinary approach and treatment options in right ventricular outflow tract malformations.

J Multidiscip Healthc. 2018;11:333-338

Authors: Skoglund K, Clase L, Dellborg M

Background: Among patients with congenital heart disease, implantation of a valved conduit is common practice for surgical reconstruction of malformations involving the right ventricular outflow tract (RVOT). The conduit has limited durability, and treatments with surgical replacement and transcatheter pulmonary valve replacement (TPVR) are common. Previous studies indicate that TPVR, despite being a less invasive alternative, is not used for the majority of these patients.
Methods and results: This is a descriptive study of the medical records of 100 consecutive adult patients with RVOT malformations who were evaluated a total of 118 times between January 1, 2008 and December 31, 2015, at meetings of the hospital's multidisciplinary heart patient review board, in which relevant specialists make all treatment decisions on each case through a consensus process. The most common overall outcome decision was surgical conduit implantation. In 51 cases, the patient had a pre-existing conduit and, of those, 16 cases were recommended for TPVR. In seven of those 16, TPVR could not be performed, most commonly due to the risk of coronary compression or unfavorable conduit anatomy.
Conclusion: Among patients with congenital heart disease involving the RVOT, surgical conduit implantation was the main treatment both in native RVOT malformations and in the case of a pre-existing dysfunctional conduit, despite the introduction of TPVR. Although the hospital's multidisciplinary heart patient review board often recommended TPVR, it was found to be unfeasible in many cases. The main reasons were risk of coronary compression and unfavorable conduit anatomy.

PMID: 30038501 [PubMed]

Coronary Disease and Modifying Cardiovascular Risk in Adult Congenital Heart Disease Patients: Should General Guidelines Apply?

Prog Cardiovasc Dis. 2018 Jul 21;:

Authors: Awerbach JD, Krasuski RA, Camitta MGW

There are more than 1.4 million adult congenital heart disease (CHD;ACHD) patients living in the United States. Coronary artery disease (CAD) is at least as prevalent in ACHD patients as in the general population and has become a leading cause of their mortality. In the majority of cases, CAD in the ACHD population is driven by the presence of traditional cardiovascular disease (CVD) risk factors. 80% of ACHD patients have at least one CVD risk factor. Hypertension (HTN), obesity and physical inactivity are frequently seen in both pediatric and adult patients with CHD. Many ACHD patients demonstrate abnormal glucose metabolism and are at an increased risk for developing diabetes. Current guidelines for CVD risk assessment and prevention do not specifically mention patients with CHD but are likely applicable to most of these patients. Specific CHD populations have "high-risk" lesions that are associated with an increased risk of CVD complications and may warrant intensified screening and treatment. These include patients with a history of coarctation of the aorta or with prior coronary artery ostial manipulation (patients with a history of d-transposition of the great arteries or anomalous aortic origin of a coronary artery). The physiology of single ventricle patients is also poorly suited for the effects of superimposed CVD; these patients may benefit from intensified treatment of CVD risk factors, particularly HTN and obesity.

PMID: 30041020 [PubMed - as supplied by publisher]

When the Heart is Not to Blame: Managing Lung Disease in Adult Congenital Heart Disease.

Prog Cardiovasc Dis. 2018 Jul 21;:

Authors: Ginde S, Earing MG

It is well-recognized now that adult survivors with congenital heart disease (CHD) are at risk for non-cardiac co-morbidities and complications that can impact symptoms and clinical outcomes. Lung disease, in particular, is common in this population, but likely an under-recognized and undertreated cause for long-term morbidity. Abnormal lung function contributes to exercise intolerance and is associated with a higher risk for mortality in this population. The exact mechanisms that contribute to abnormal measurements of lung function are not entirely known, and are likely multifactorial and variable depending on the underlying CHD. Nevertheless, lung disease is a potentially modifiable risk factor in this patient population, the management of which may result in improved clinical outcomes. This review summarizes our current understanding of the prevalence, impact and management of lung disease in adults with CHD.

PMID: 30041022 [PubMed - as supplied by publisher]

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Maternal Stress and Anxiety in the Pediatric Cardiac Intensive Care Unit.

Am J Crit Care. 2017 Mar;26(2):118-125

Authors: Lisanti AJ, Allen LR, Kelly L, Medoff-Cooper B

BACKGROUND: Mothers whose infants are born with complex congenital heart disease (CCHD) experience stress during their infant's hospitalization in a pediatric cardiac intensive care unit (PCICU).
OBJECTIVES: This study addressed 2 research questions: (1) What are the parental stressors for mothers whose infants with CCHD are in the PCICU? And (2) What are the relationships of trait anxiety and 3 parental stressors to the parental stress response of state anxiety in mothers whose infants with CCHD are in the PCICU?
METHODS: This descriptive correlational study included 62 biological mothers of infants admitted to a PCICU within 1 month of birth who had undergone cardiac surgery for CCHD. Maternal and infant demographics and responses to the Parental Stressor Scale: Infant Hospitalization and the State-Trait Anxiety Inventory were collected at 3 major PCICUs across the United States.
RESULTS: Mothers' scores revealed that infant appearance and behavior was the greatest stressor, followed by parental role alteration, then sights and sounds. The combination of trait anxiety and parental role alteration explained 26% of the variance in maternal state anxiety. Mothers with other children at home had significantly higher state anxiety than did mothers with only the hospitalized infant.
CONCLUSIONS: Results from this study revealed factors that contribute to the stress of mothers whose infants are born with CCHD and are hospitalized in a PCICU. Nurses are in a critical position to provide education and influence care to reduce maternal stressors in the PCICU, enhance mothers' parental role, and mitigate maternal state anxiety.

PMID: 28249863 [PubMed - indexed for MEDLINE]

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Color and power Doppler combined with Fetal Intelligent Navigation Echocardiography (FINE) to evaluate the fetal heart.

Ultrasound Obstet Gynecol. 2017 Oct;50(4):476-491

Authors: Yeo L, Romero R

OBJECTIVE: To evaluate the performance of color and bidirectional power Doppler ultrasound combined with Fetal Intelligent Navigation Echocardiography (FINE) in examining the fetal heart.
METHODS: A prospective cohort study was conducted of fetuses in the second and third trimesters with a normal heart or with congenital heart disease (CHD). One or more spatiotemporal image correlation (STIC) volume datasets, combined with color or bidirectional power Doppler (S-flow) imaging, were acquired in the apical four-chamber view. Each successfully obtained STIC volume was evaluated by STICLoop™ to determine its appropriateness before applying the FINE method. Visualization rates for standard fetal echocardiography views using diagnostic planes and/or Virtual Intelligent Sonographer Assistance (VIS-Assistance®) were calculated for grayscale (removal of Doppler signal), color Doppler and S-flow Doppler. In four cases with CHD (one case each of tetralogy of Fallot, hypoplastic left heart and coarctation of the aorta, interrupted inferior vena cava with azygos vein continuation and asplenia, and coarctation of the aorta with tricuspid regurgitation and hydrops), the diagnostic potential of this new technology was presented.
RESULTS: A total of 169 STIC volume datasets of the normal fetal heart (color Doppler, n = 78; S-flow Doppler, n = 91) were obtained from 37 patients. Only a single STIC volume of color Doppler and/or a single volume of S-flow Doppler per patient were analyzed using FINE. Therefore, 60 STIC volumes (color Doppler, n = 27; S-flow Doppler, n = 33) comprised the final study group. Median gestational age at sonographic examination was 23 (interquartile range, 21-27.5) weeks. Color Doppler FINE generated nine fetal echocardiography views (grayscale) using (1) diagnostic planes in 73-100% of cases, (2) VIS-Assistance in 100% of cases, and (3) a combination of diagnostic planes and/or VIS-Assistance in 100% of cases. The rate of generating successfully eight fetal echocardiography views with appropriate color and S-flow Doppler information was 89-100% and 91-100% of cases, respectively, using a combination of diagnostic planes and/or VIS-Assistance. However, the success rate for the ninth echocardiography view (i.e. superior and inferior venae cavae) was 33% and 30% for color and S-flow Doppler, respectively. In all four cases of CHD, color Doppler FINE demonstrated evidence of abnormal fetal cardiac anatomy and/or hemodynamic flow.
CONCLUSIONS: The FINE method applied to STIC volumes of normal fetal hearts acquired with color or bidirectional power Doppler information can generate successfully eight to nine standard fetal echocardiography views (via grayscale, color Doppler or power Doppler) in the second and third trimesters. In cases of CHD, color Doppler FINE demonstrates successfully abnormal anatomy and/or Doppler flow characteristics. Published 2017. This article is a U.S. Government work and is in the public domain in the USA. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of the International Society of Ultrasound in Obstetrics and Gynecology.

PMID: 28809063 [PubMed - indexed for MEDLINE]

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Response to Letter to 'Pregnancy and delivery outcomes from patients with repaired anomalous origin of the left coronary artery from the pulmonary artery'.

J Obstet Gynaecol Res. 2018 Jul 25;:

Authors: Kanoh M, Inai K

PMID: 30043497 [PubMed - as supplied by publisher]