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First-Trimester Fetal Echocardiography: Identification of Cardiac Structures for Screening from 6 to 13 Weeks' Gestational Age.

J Am Soc Echocardiogr. 2017 Aug;30(8):763-772

Authors: Hutchinson D, McBrien A, Howley L, Yamamoto Y, Sekar P, Motan T, Jain V, Savard W, Hornberger LK

Abstract
BACKGROUND: Early fetal echocardiography (FE), performed at 12 to 16 weeks' gestational age (GA), can be used to screen for fetal heart disease akin to that routinely performed in the second trimester. The efficacy of FE at earlier GAs has not been as well explored, particularly with recent advances in ultrasound technology. The aim of this study was to evaluate the efficacy of early FE in assessing fetal heart structure, and the added benefit of color Doppler (CD), from as early as 6 weeks through to 13+6 weeks' GA.
METHODS: Pregnant women were prospectively recruited for first-trimester FE. All underwent two-dimensional (2D) cardiac imaging combined with CD assessment, and all were offered second-trimester fetal echocardiographic evaluations. Fetal cardiac anatomy was assessed both in real time during FE and additionally offline by two separate reviewers.
RESULTS: Very early FE was performed in 202 pregnancies including a total of 261 fetuses, with 92% (n = 241) being reassessed at ≥18 weeks' GA. Mean GA at FE was 10+6 weeks (range, 6+1 to 13+6 weeks). Transabdominal scanning was used in all cases, and transvaginal scanning was used additionally in most at <11 weeks' GA (n = 103 of 117 [88%]). There was stepwise improvement in image resolution of the fetal heart in those pregnancies that presented at later gestation for assessment. CD assisted with definition of cardiac anatomy at all GAs. A four-chambered heart could be identified in 52% of patients in the eighth week (n = 12 of 23), improving to 80% (n = 36 of 45) in the 10th week and 98% (n = 57 of 58) by the 11th week. The inferior vena cava was visualized by 2D imaging in only 4% (n = 1 of 23) in the eighth week, increasing to 13% (n = 6 of 45) by the 10th week and 80% (n = 25 of 31) by the 13th week. CD improved visualization of the inferior vena cava at earlier GAs to >80% (n = 37 of 45) from 10 weeks. Pulmonary veins were not visualized by either 2D imaging or CD until after the 11th week. Both cardiac outflow tracts could be visualized by 2D imaging in the minority from 8+0 to 10+6 weeks (n = 18 of 109 [16%]) but were imaged in most from 11+0 to 13+6 weeks (n = 114 of 144 [79%]). CD imaging improved visualization of both outflow tracts to 64% (n = 29 of 45) in the 10th week. On 2D imaging alone, both the aortic and ductal arches were seen in only 29% of patients in the 10th week (n = 13 of 45), increasing to 58% when CD was used (58% [n = 26 of 45]) and to >80% (n = 47 of 58) using CD in the 11th week.
CONCLUSIONS: Very early FE, from as early as 8 weeks, can be used to assess cardiac structures. The ability to image fetal heart structures between 6 and 8 weeks is currently nondiagnostic. The use of CD significantly increases the detection of cardiac structures on early FE. The ideal timing of complete early FE, excluding pulmonary vein assessment, appears to be after 11 weeks' GA.

PMID: 28511860 [PubMed - indexed for MEDLINE]

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Late Onset Postcapillary Pulmonary Hypertension in Patients With Transposition of the Great Arteries and Mustard or Senning Baffles.

J Am Heart Assoc. 2017 Oct 12;6(10):

Authors: Chaix MA, Dore A, Mercier LA, Mongeon FP, Marcotte F, Ibrahim R, Asgar AW, Shohoudi A, Labombarda F, Mondésert B, Poirier N, Khairy P

Abstract
BACKGROUND: There is a paucity of data regarding late-onset pulmonary hypertension (PH) in patients with transposition of the great arteries and atrial switch surgery.
METHODS AND RESULTS: A retrospective cohort study was conducted on 140 adults with transposition of the great arteries and atrial switch surgery, age 37.3±7.8, 37.1% female, in order to assess the prevalence and characteristics of late-onset PH and explore associated factors. Patients were followed for a median of 32.3 years after atrial switch surgery and 10.0 years after their first referral visit. PH was detected in 18 of 33 (54.5%) patients who had invasive hemodynamic studies. Average age at diagnosis of PH was 33.9±8.1 years. PH was postcapillary in all, with a mean pulmonary artery pressure of 36±12 mm Hg and mean pulmonary capillary wedge pressure of 28±8 mm Hg. PH was diagnosed in 13 of 17 (76.5%) patients who had cardiac catheterization for heart failure or decreased exercise tolerance. In multivariable analyses, systemic hypertension (odds ratio 9.4, 95% confidence interval 2.2-39.4, P=0.002) and heart failure or New York Heart Association class III or IV symptoms (odds ratio 49.8, 95% confidence interval 8.6-289.0, P<0.001) were independently associated with PH. Patients with PH were more likely to develop cardiovascular comorbidities including atrial (P=0.001) and ventricular (P=0.008) arrhythmias, require hospitalizations for heart failure (P<0.001), and undergo tricuspid valve surgery (P<0.001). Mortality was significantly higher in patients with PH (hazard ratio 9.4, 95% confidence interval 2.1-43.0], P<0.001).
CONCLUSIONS: Late-onset postcapillary PH is highly prevalent in adults with transposition of the great arteries and atrial switch surgery and is associated with an adverse prognosis.

PMID: 29025749 [PubMed - indexed for MEDLINE]

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Health condition and familial factors associated with health-related quality of life in adolescents with congenital heart disease: a cross sectional study.

Health Qual Life Outcomes. 2018 Jan 10;16(1):9

Authors: Im YM, Yun TJ, Lee S

Abstract
BACKGROUND: The focus of clinical care after the repair of congenital heart disease has shifted from saving life of the patient to the patient's quality of life. The purpose of this study was to examine the health condition and familial factors associated with the health related quality of life of adolescents with congenital heart disease.
METHODS: Ninety-eight adolescents aged 13-19 years were collected from a congenital heart clinic from July 22 to August 23, 2013. Perceptions of parental rearing behaviors, health related quality of life of adolescent with congenital heart disease, and general characteristics were measured. We used multiple linear regression analysis to explore factors that are associated with the health related quality of life of adolescents with congenital heart disease.
RESULTS: New York heart association class (Adj R2 = .186, p = .000), presence of siblings (Adj R2 = .240, p = .010), and mother's emotional warmth (Adj R2 = .265, p = .043) were significantly associated with the health related quality of life of adolescents with congenital heart disease.
CONCLUSIONS: Emotionally warm parental rearing behaviors and the presence of siblings were important familial factors that were positively associated with HRQOL in adolescents with CHD. Therefore, it is important for healthcare providers to develop a greater sensitivity to, and awareness of, the familial influences that may be impacting a subject's HRQOL, as well as the exigencies of the CHD, itself.

PMID: 29321028 [PubMed - indexed for MEDLINE]

Left Atrial Appendage Aneurysm Characterized by Multimodal Imaging.

World J Pediatr Congenit Heart Surg. 2018 Jan 01;:2150135118769327

Authors: Brenneman DJ, Pitkin AD, Gupta D, Bleiweis MS, Reyes KM, Chandran A

Abstract
We present a four-year-old female with an incidental finding of a congenital left atrial appendage aneurysm who underwent surgical resection with excellent results. This case highlights the importance of multimodal imaging in the diagnosis and characterization of this rare condition.

PMID: 29888627 [PubMed - as supplied by publisher]

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Clinical and Genetic Diagnosis of Nonischemic Sudden Cardiac Death.

Rev Esp Cardiol (Engl Ed). 2017 Oct;70(10):808-816

Authors: Jiménez-Jáimez J, Alcalde Martínez V, Jiménez Fernández M, Bermúdez Jiménez F, Rodríguez Vázquez Del Rey MDM, Perin F, Oyonarte Ramírez JM, López Fernández S, de la Torre I, García Orta R, González Molina M, Cabrerizo EM, Álvarez Abril B, Álvarez M, Macías Ruiz R, Correa C, Tercedor L

Abstract
INTRODUCTION AND OBJECTIVES: Nonischemic sudden cardiac death (SCD) is predominantly caused by cardiomyopathies and channelopathies. There are many diagnostic tests, including some complex techniques. Our aim was to analyze the diagnostic yield of a systematic diagnostic protocol in a specialized unit.
METHODS: The study included 56 families with at least 1 index case of SCD (resuscitated or not). Survivors were studied with electrocardiogram, advanced cardiac imaging, exercise testing, familial study, genetic testing and, in some cases, pharmacological testing. Families with deceased probands were studied using the postmortem findings, familial evaluation, and molecular autopsy with next-generation sequencing (NGS).
RESULTS: A positive diagnosis was obtained in 80.4% of the cases, with no differences between survivors and nonsurvivors (P=.53). Cardiac channelopathies were more prevalent among survivors than nonsurvivors (66.6% vs 40%, P=.03). Among the 30 deceased probands, the definitive diagnosis was given by autopsy in 7. A diagnosis of cardiomyopathy tended to be associated with a higher event rate in the family. Genetic testing with NGS was performed in 42 index cases, with a positive result in 28 (66.6%), with no differences between survivors and nonsurvivors (P=.21).
CONCLUSIONS: There is a strong likelihood of reaching a diagnosis in SCD after a rigorous protocol, with a more prevalent diagnosis of channelopathy among survivors and a worse familial prognosis in cardiomyopathies. Genetic testing with NGS is useful and its value is increasing with respect to the Sanger method.

PMID: 28566242 [PubMed - indexed for MEDLINE]

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Prevalence and prognostic significance of pulmonary artery aneurysms in adults with congenital heart disease.

Int J Cardiol. 2018 Jun 02;:

Authors: Gallego P, Rodríguez-Puras MJ, Serrano Gotarredona P, Valverde I, Manso B, González-Calle A, Adsuar A, Cubero JM, Díaz de la Llera L, Ordoñez A, Hosseinpour AR

Abstract
BACKGROUND: Prevalence and prognostic significance of pulmonary artery (PA) dilatation in congenital heart disease (CHD) have never been studied systematically.
METHODS: Chest X-rays of 1192 consecutive adults with CHD were reviewed. Major diameter of the PA was determined by imaging techniques in those with PA dilatation. A value >29 mm was considered abnormal. Data on anatomy, hemodynamics, residual lesions and outcomes were retrospectively collected.
RESULTS: Overall prevalence of PA dilatation was 18%. A minority of patients (5.5%) reached 40 mm (aneurysm; PAA) and 1.8% exceeded 50 mm. The most common PAA underlying malformations were pulmonary stenosis (21%), and shunts (55%). Significantly larger diameters were observed in hypertensive shunts (40 mm; IQR 36.7-45 mm vs. 34 mm; IQR 32-36 mm) (p < 0.0001). However, the largest diameters were found in cono-truncal anomalies. There was no significant correlation between PA dimensions and systolic pulmonary pressure (r = -0.196), trans-pulmonary gradient (r = -0.203), pulmonary regurgitation (PR) (r = 0.071) or magnitude of shunt (r = 0.137) (p > 0.05 for all). Over follow-up, 1 sudden death (SD) occurred in one Eisenmenger patient. Complications included coronary (3), recurrent laryngeal nerve (1) and airway (1) compressions, progressive PR (1), and PA thrombosis (1). Coronary compression and SD were strongly associated (univariate analysis) with pulmonary hypertension (120 vs. 55 mm Hg; p = 0.002) but not with extreme PA dilatation (range: 40-65 mm).
CONCLUSIONS: PA dilatation in CHD is common but only a small percentage of patients have PAA. Clinical impact on outcomes is low. Complications occurred almost exclusively in patients with pulmonary hypertension whereas PA diameter alone was not associated with adverse outcomes.

PMID: 29891239 [PubMed - as supplied by publisher]

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Undifferentiated connective tissue diseases and adverse pregnancy outcomes. An undervalued association?

Am J Reprod Immunol. 2017 Dec;78(6):

Authors: Spinillo A, Beneventi F, Caporali R, Ramoni V, Montecucco C

Abstract
Undifferentiated connective tissue diseases (UCTDs) are a heterogeneous group of disorders characterized by symptoms and signs suggestive of systemic autoimmune rheumatic disease (ARD), but which do not fulfill all the established criteria for definite diagnosis of a condition. Although a third of UCTDs can progress to a definite ARD within months or years, most UCTDs can remain stable for years with minimal disease activity. The annual incidence of UCTD in the general population ranges from 14 to 140 per 100 000 people. UCTDs are associated with the persistence of several circulating autoantibodies including antinuclear, antiphospholipid or antithyroid antibodies. Immunological evaluation of subjects with UCTDs suggests a proinflammatory state and dysregulation of the Th1/Th2 balance. Autoantibodies have well-known deleterious effects on placentation and have been associated with an increased risk of prematurity, fetal growth restriction (FGR), preeclampsia, and congenital atrioventricular heart block. Although epidemiological and biological data suggest a potential negative impact on reproductive outcomes, the relationship between UCTD and pregnancy outcomes has not been adequately studied. While awaiting definitive data from large studies, obstetricians should be aware that rheumatic disorders in their early, incomplete, or undifferentiated phases can adversely affect pregnancy outcomes, increasing the likelihood of pregnancy loss, FGR, preeclampsia, and prematurity.

PMID: 28921728 [PubMed - indexed for MEDLINE]

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Age is not a good predictor of irreversibility of pulmonary hypertension in congenital cardiac malformations with left-to-right shunt.

Congenit Heart Dis. 2018 Mar;13(2):210-216

Authors: Hosseinpour AR, Perez MH, Longchamp D, Cotting J, Sekarski N, Hurni M, Prêtre R, Di Bernardo S

Abstract
OBJECTIVE: Congenital cardiac malformations with high pulmonary blood flow and pressure due to left-to-right shunts are usually repaired in early infancy for both the benefits of early relief of heart failure and the fear that the concomitant pulmonary hypertension may become irreversible unless these defects are corrected at an early age. Age, however, has been a poor predictor of irreversibility of pulmonary hypertension in our experience, which is presented here.
DESIGN: A retrospective observational study. We defined "late" as age ≥2 years. We examined clinical, echocardiographic, and hemodynamic data from all patients aged ≥2 years with such malformations referred to us from 2004 untill 2015.
SETTING: Department of Pediatric Cardiology and Cardiac Surgery, University Hospital of Vaud, Lausanne, Switzerland.
PATIENTS: There were 39 patients, aged 2-35 years (median: 5 years), without chromosomal abnormalities. All had malformations amenable to biventricular repair, and all had high systolic right ventricular pressures by echocardiography prior to referral.
INTERVENTIONS: All patients underwent catheterization for assessment of pulmonary hypertension. If this was reversible, surgical correction was offered.
OUTCOME MEASURES: (1) Operability based on reversibility of pulmonary hypertension. (2) When surgery was offered, mortality and evidence of persisting postoperative pulmonary hypertension were examined.
RESULTS: Eighteen patients had no pulmonary hypertension, 5 of variable ages were inoperable due to irreversible pulmonary hypertension, and 16 had reversible pulmonary hypertension. Therefore, 34 patients underwent corrective surgery, with no immediate or late mortality. Pulmonary arterial and right ventricular pressures decreased noticeably in all operated patients. This is sustained to date; they are all asymptomatic with no echocardiographic evidence of pulmonary hypertension at a median follow-up of 7 years (range 2-13 years).
CONCLUSIONS: Pulmonary hypertension may still be reversible in many surprisingly old patients with left-to-right shunt lesions, who may therefore still be operable.

PMID: 29071783 [PubMed - indexed for MEDLINE]

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Health-related quality of life of mothers of children with congenital heart disease in a sub-Saharan setting: cross-sectional comparative study.

BMC Res Notes. 2017 Oct 26;10(1):513

Authors: Sileshi L, Tefera E

Abstract
BACKGROUND: While the Health Related Quality of Life of the children with congenital heart defects is primarily affected, caring for a child with birth defect has an impact on the family's quality of life as well. Understanding the level of quality of life of the parents, which is likely to vary in different cultural settings, beliefs and parental educational status may help to implement educational programs and other interventional measures that may improve the HRQOL of parents of such children. This cross-sectional comparative study reports the health-related quality of life of mothers of children with congenital heart diseases in a sub-Saharan setting.
RESULTS: Mean age of the mothers in the study group was 32.2 ± 7.1 years where as that of the control group was 30.5 ± 6.5 years (p = .054). One hundred-four children had congenital cardiac lesions classified as mild to moderate while 31 patients had severe lesions. On average, mothers in the study group showed poor performance on the Short Form-36 (SF-36) with statistically significant differences on all sub-scales including general health perception, physical functioning, role physical, role emotional, social functioning, bodily pain, vitality and mental health. Severity of the congenital heart defect was not associated with statistically significant difference in the health-related quality of life of the mothers.
CONCLUSIONS: Mothers of children with congenital heart disease in our study have significantly lower quality of life in all domains of SF-36 compared to the control group. Planning and devising a strategy to support these mothers may need to be part of management and clinical care of children with congenital heart diseases.

PMID: 29073929 [PubMed - indexed for MEDLINE]

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Impact of pregnancy on autograft dilatation and aortic valve function following the Ross procedure.

Congenit Heart Dis. 2018 Mar;13(2):217-221

Authors: Carvajal HG, Lindley KJ, Shah T, Brar AK, Barger PM, Billadello JJ, Eghtesady P

Abstract
OBJECTIVE: The effects of pregnancy on autograft dilatation and neoaortic valve function in patients with a Ross procedure have not been studied. We sought to evaluate the effect of pregnancy on autograft dilatation and valve function in these patients with the goal of determining whether pregnancy is safe after the Ross procedure.
DESIGN: A retrospective chart review of female patients who underwent a Ross procedure was conducted.
PATIENTS: Medical records for 51 patients were reviewed. Among the 33 patients who met inclusion criteria, 11 became pregnant after surgery and 22 did not.
OUTCOME MEASURES: Echocardiographic reports were used to record aortic root diameter and aortic insufficiency before, during, and after pregnancy. Patient's charts were reviewed for reinterventions and complications. Primary endpoints included reinterventions, aortic root dilation of ≥5 cm, aortic insufficiency degree ≥ moderate, and death.
RESULTS: There were 18 pregnancies carried beyond 20 weeks in 11 patients. There was no significant difference in aortic root diameter between nulliparous patients and parous patients prior to their first pregnancy (3.53 ± 0.44 vs 3.57 ± 0.69 cm, P = .74). There was no significant change in aortic root diameter after first pregnancy (3.7 ± 0.4 cm, P = .056) although there was significant dilatation after the second (4.3 ± 0.7 cm, P = .009) and third (4.5 ± 0.7 cm, P = .009) pregnancies. Freedom from combined endpoints was significantly higher for patients in the pregnancy group than those in the nonpregnancy group (P = .002).
CONCLUSIONS: Pregnancy was not associated with significantly increased adverse events in patients following the Ross procedure. Special care should be taken after the first pregnancy, as multiparity may lead to increased neoaortic dilatation.

PMID: 29250922 [PubMed - indexed for MEDLINE]

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