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At the Heart of the Pregnancy: What Prenatal and Cardiovascular Genetic Counselors Need to Know about Maternal Heart Disease.

J Genet Couns. 2017 Aug;26(4):669-688

Authors: Morales A, Allain DC, Arscott P, James E, MacCarrick G, Murray B, Tichnell C, Shikany AR, Spencer S, Fitzgerald-Butt SM, Kushner JD, Munn C, Smith E, Spoonamore KG, Tandri HS, Kay WA

In the last decade, an increasing number of cardiac conditions have been shown to have a genetic basis. Cardiovascular genetic counseling has emerged as a subspecialty aiming to identify unaffected at-risk individuals. An important sector of this at-risk population also includes expectant mothers, in whom unique clinical challenges may arise. Genetic counselors, especially those in cardiovascular and prenatal settings, have an opportunity to identify and assist women who may benefit from cardiovascular care during pregnancy. This paper provides basic management and genetic evaluation principles for affected women, as well as guidance on identifying those who are at risk. We provide considerations for cardiac surveillance in pregnancy and the post-partum period. Finally, key psychosocial issues that appraise how to best provide support to at risk women as they make informed decisions are discussed. We propose that a team approach including cardiology, maternal fetal medicine, and genetic counseling best serves this patient population. Ongoing questions addressing an evidence based approach to cardiovascular genetic conditions in pregnancy still remain. Thus, well-designed research protocols are essential to mark progress in this area.

PMID: 28283918 [PubMed - indexed for MEDLINE]

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Idiopathic pulmonary arterial hypertension with coexisting CHD.

Cardiol Young. 2018 May;28(5):743-746

Authors: Day TG, Hughes M, Pandya B

Sinus venosus atrial septal defect can result in an increase in pulmonary blood flow and vascular resistance, leading to pulmonary hypertension. Rarely, the degree of pulmonary hypertension is out of proportion to the degree of intra-cardiac shunting. This case outlines the differences between pulmonary hypertension secondary to CHD and idiopathic pulmonary hypertension, and illustrates the investigation and management strategy used in a patient with features of both.

PMID: 29316988 [PubMed - indexed for MEDLINE]

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Percutaneous retrieval of a partially flared Melody valve.

Cardiol Young. 2018 May;28(5):753-755

Authors: Gendera K, Ewert P, Eicken A

We report on successful endovascular retrieval of an accidentally flared Melody valve in an adult patient with an indication for percutaneous pulmonary valve implantation. The Melody valve was removed through a 24 F sheath, introduced via the right jugular vein, and the urgent open-heart surgery was avoided.

PMID: 29409567 [PubMed - indexed for MEDLINE]

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Three-dimensional printing models in congenital heart disease education for medical students: a controlled comparative study.

BMC Med Educ. 2018 Aug 02;18(1):178

Authors: Su W, Xiao Y, He S, Huang P, Deng X

BACKGROUND: This study sought to assess, using subjective (self-assessment) and objective (MCQ) methods, the efficacy of using heart models with ventricular septal defect lesions produced with three-dimensional printing technology in a congenital heart disease curriculum for medical students.
METHODS: Three computed tomography datasets of three subtypes of ventricular septal defects (perimembranous, subarterial and muscular, one for each) were obtained and processed for building into and printing out 3D models. Then a total of 63 medical students in one class were randomly allocated to two groups (32 students in the experimental, and 31 the control). The two groups participated in a seminar with or without a 3D heart model, respectively. Assessment of this curriculum was carried out using Likert-type questionnaires as well as an objective multiple choice question test assessing both knowledge acquisition, and structural conceptualization. Open-ended questions were also provided for getting advice and suggestion on 3D model utilization in CHD education.
RESULTS: With these 3D models, feedback shown in the questionnaires from students in experimental group was significantly more positive than their classmates in the control. And the test results also showed a significant difference in structural conceptualization in favor of the experimental group.
CONCLUSION: It is effective to use heart models created using current 3D printing technology for congenital heart disease education. It stimulates students' interest in congenital heart disease and improves the outcomes of medical education.

PMID: 30068323 [PubMed - indexed for MEDLINE]

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Development and effects of a self-management efficacy promotion program for adult patients with congenital heart disease.

Eur J Cardiovasc Nurs. 2018 Sep 10;:1474515118800099

Authors: Lee MJ, Jung D

BACKGROUND: With the recent advances in medicine, patients with congenital heart disease are surviving to adulthood. Adults with congenital heart disease must practice self-management to recognize the symptoms of complications and the appropriate response.
AIMS: The purpose of this study was to develop a self-management efficacy promotion program for adults with congenital heart disease and to test the effects of the developed program on disease-related knowledge, self-management implementation and health-related quality of life.
METHODS: A non-equivalent, control group, pre-post test design was used. The intervention group received the self-management efficacy promotion program for six weeks. The control group received only the usual care.
RESULTS: The study results showed that disease-related knowledge ( F=91.095, p<0.001) was significantly different between the two groups, as was the self-management performance ( F=11.846, p<0.001). However, health-related quality of life (generic core scale: F=0.023, p=0.881, cardiac module scale: F=0.174, p=0.678) was not significantly different between groups.
CONCLUSION: The self-management efficacy promotion program for adults with congenital heart disease had a significant effect on disease-related knowledge and self-management implementation, but did not affect health-related quality of life.

PMID: 30198757 [PubMed - as supplied by publisher]

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A Study of Practice Behavior for Endotracheal Intubation Site for Children With Congenital Heart Disease Undergoing Surgery: Impact of Endotracheal Intubation Site on Perioperative Outcomes-An Analysis of the Society of Thoracic Surgeons Congenital Cardiac Anesthesia Society Database.

Anesth Analg. 2018 Sep 05;:

Authors: Greene NH, Jooste EH, Thibault DP, Wallace AS, Wang A, Vener DF, Matsouaka RA, Jacobs ML, Jacobs JP, Hill KD, Ames WA

BACKGROUND: In adults undergoing cardiopulmonary bypass surgery, oral intubation is typically preferred over nasal intubation due to reduced risk of sinusitis and infection. In children, nasal intubation is more common and sometimes preferred due to perceived benefits of less postoperative sedation and a lower risk for accidental extubation. This study sought to describe the practice of nasal intubation in the pediatric population undergoing cardiopulmonary bypass surgery and assess the risks/benefits of a nasal route against an oral one.
METHODS: Patients <18 years of age in the Society of Thoracic Surgeons Congenital Heart Surgery Database between January 2010 and December 2015 were included. Patients with a preoperative endotracheal tube, tracheostomy, or known airway anomalies were excluded. Multivariable modeling was used to assess the association between route of tracheal intubation and a composite measure of infection risk (wound infection, mediastinitis, septicemia, pneumonia, and endocarditis). Covariates were included to adjust for important patient characteristics (eg, weight, age, comorbidities), case complexity, and center effects. Secondary outcomes included length of intubation, hospital length of stay, and airway complications including accidental extubations. We also performed a subanalysis in children <12 months of age in high-volume centers (>100 cases/y) examining how infection risk may change with age at the time of surgery.
RESULTS: Nasal intubation was used in 41% of operations in neonates, 38% in infants, 15% in school-aged children, and 2% in adolescents. Nasal intubation appeared protective for accidental extubation only in neonates (P = .02). Multivariable analysis in infants and neonates showed that the nasal route of intubation was not associated with the infection composite (relative risk [RR], 0.84; 95% CI, 0.59-1.18) or a shorter length of stay (RR, 0.992; 95% CI, 0.947-1.039), but was associated with a shorter intubation length (RR, 0.929; 95% CI, 0.869-0.992). Restricting to high-volume centers showed a significant interaction between age and intubation route with a risk change for infection occurring between approximately 6-12 months of age (P = .003).
CONCLUSIONS: While older children undergoing nasal intubation trend similar to the adult population with an increased risk of infection, nasal intubation in neonates and infants does not appear to carry a similar risk. Nasal intubation in neonates and infants may also be associated with a shorter intubation length but not a shorter length of stay. Prospective studies are required to better understand these complex associations.

PMID: 30198928 [PubMed - as supplied by publisher]

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Efficacy and Safety of Low-Dose Amiodarone Therapy for Tachyarrhythmia in Congenital Heart Disease.

Pediatr Cardiol. 2018 Jun;39(5):1016-1022

Authors: Iwasawa S, Uyeda T, Saito M, Ishii T, Inage A, Hamamichi Y, Yazaki S, Yoshikawa T

Amiodarone (AMD) is a class III anti-arrhythmic drug that is highly effective for tachyarrhythmia treatment. AMD is widely used in adults with congenital heart disease (CHD); however, higher doses of AMD (> 200 mg/day) can cause various non-cardiac side effects. The purpose of this study was to assess the efficacy, safety, and adverse events of low-dose AMD (≤ 200 mg/day) for tachyarrhythmia in patients with CHD. We retrospectively studied 80 patients with CHD and tachyarrhythmia who received oral low-dose AMD (≤ 200 mg/day) from January 2004 to March 2016. Low-dose AMD therapy was used to treat supraventricular tachycardia (SVT) in 51 patients and ventricular tachycardia (VT) in 29 patients. After a mean follow-up of 2.9 years for SVT and 3.2 years for VT, 36% and 65% of the patients with SVT and VT, respectively, were free from a first tachyarrhythmia recurrence for 3 years. The incidence of AMD-induced side effects was 23%, and all these cases consisted of thyroid dysfunction. Low-dose AMD was effective for the treatment of tachyarrhythmia in patients with CHD and had a relatively low incidence of side effects. These findings suggest that low-dose AMD is useful and effective for decreasing the frequency of tachyarrhythmia in patients with CHD and has a low incidence of side effects.

PMID: 29523919 [PubMed - indexed for MEDLINE]

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Silencing mutations in JAG1 gene may play crucial roles in the pathogenesis of Tetralogy of Fallot.

Cell Mol Biol (Noisy-le-grand). 2018 Mar 31;64(4):103-107

Authors: Safari-Arababadi A, Behjati-Ardakani M, Kalantar SM, Jaafarinia M

JAG1 gene through Notch signaling is implicated in cell fate decisions in early cardiac development, and mutations in several proteins in the pathway have been involved in various disorders. Tetralogy of Fallot (TOF) is the most frequent form of complicated congenital heart disease. The abnormality of TOF begins through the first eight weeks of fetal growth and is confused with ventricular septal defects, obstruction to right ventricular outflow tract, aortic dextroposition, and right ventricular hypertrophy. Hence the existence of mutations in JAG1 gene in Iranian patients with TOF is evaluated. The clinical data and peripheral blood samples were collected from 44 sporadic nonsyndromic patients with TOF and compared to 44 healthy individuals. DNA was extracted, and the exon 6 of the JAG1 gene was amplified by PCR then the PCR products were purified and sequenced. The age range in patients and the control group was 2-36 years, and the mean and standard deviation (SD) of the age in patients was (11.69 ± 7.85 years) and in control group (11.63 ± 7.99 years).  Finally, the samples were successfully sequenced, then analyzed and one synonymous variant (c.765C&gt;T; p.Y255Y) was observed in 38 patients with frequency (86.4%) and three controls with frequency (6.8%). The c.765C&gt;T variant is significantly associated with the pathogenesis of TOF in Iranian population.

PMID: 29631691 [PubMed - indexed for MEDLINE]

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Symptomatic Subclavian Steal During Pregnancy in a Woman Status Post Coarctation Repair in Infancy.

World J Pediatr Congenit Heart Surg. 2018 Sep 10;:2150135118783638

Authors: Lubert AM, Cotts TB, Henke PK

A 24-year-old woman with a history of coarctation repair by subclavian flap aortoplasty presented at 15 weeks' gestation with transient episodes of vision loss. She was diagnosed with subclavian steal syndrome and underwent left carotid artery to subclavian artery bypass at 17 weeks' gestation. She has had no recurrence of symptoms at ten months of postoperative follow-up. Despite the anatomic substrate for subclavian steal in patients with this type of surgical repair, neurologic symptoms are uncommon. It is possible that the pregnancy-induced fall in systemic vascular resistance triggered symptoms in this previously asymptomatic patient.

PMID: 30200813 [PubMed - as supplied by publisher]

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Mode of death and predictors of mortality in adult Fontan survivors: A Japanese multicenter observational study.

Int J Cardiol. 2018 Sep 05;:

Authors: Ohuchi H, Inai K, Nakamura M, Park IS, Watanabe M, Hiroshi O, Kim KS, Sakazaki H, Waki K, Yamagishi H, Yamamura K, Kuraishi K, Miura M, Nakai M, Nishimura K, Niwa K, JSACHD Fontan Investigators

BACKGROUND: Mortality rates may be high in adult Fontan patients; however, the clinical determinants remain unclear.
PURPOSE: We conducted a prospective multicenter study of adult Fontan survivors to determine the 5-year mortality rate and clarify the determinants.
METHOD AND RESULTS: We followed 600 adult Fontan survivors from 40 Japanese institutions (307 men, 28 ± 7 years old, follow-up: 18 ± 6 years). The New York Heart Association (NYHA) functional class I and II was 51% and 42%, respectively. During the follow-up period of 4.1 ± 1.6 years, 33 patients died, and the 5-year survival rate was 93.5%. The mode of death was heart failure in 11 patients (34%), arrhythmia or sudden death in 8 (24%), cancer in 5 (15%), perioperative problems and hemostatic problems in 4 each (12% for each), and infection in 1 (3%). Left isomerism, prior hospitalization, protein losing enteropathy (PLE), pulmonary arteriovenous fistulae, NYHA functional class, impaired hemodynamics, hyponatremia, hepatorenal dysfunction, and use of diuretics were associated with a high mortality rate (p < 0.05-0.0001). Further, PLE (hazard ratio [HR]: 14.4), left isomerism (HR: 3.5), and NYHA (HR: 2.4) independently predicted a high 5-year high mortality (p < 0.05 for all). The incidence of cancer-related mortality increased markedly with age >40 years.
CONCLUSIONS: Majority of the Japanese adult Fontan survivors had good functional status, with an acceptable 5-year survival rate. However, the significant prevalence of non-cardiac mortality highlights Fontan pathophysiology as a multi-organ disease that requires a multidisciplinary management strategy to improve the long-term outcome.

PMID: 30201381 [PubMed - as supplied by publisher]