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Fetal heart examination at the time of 13 weeks scan: a 5 years' prospective study.

J Perinat Med. 2019 Oct 25;47(8):871-878

Authors: Ebrashy A, Aboulghar M, Elhodiby M, El-Dessouky SH, Elsirgany S, Gaafar HM, Sheta SS, Kamal R, Negm S, El Sheikhah A, Idris O, Abd-El-Kader M, Ehab M, Momtaz M

Objective To evaluate our ability in classifying the fetal heart as normal or abnormal during the 1st trimester scan through fetal cardiac examination and determining the best time for this examination. Methods This was a prospective study performed on 3240 pregnant women to examine the fetal heart. Four chambers view and ventricular outflow tracts were mainly examined during the scan. We used grayscale and color mapping in the diagnosis. Color Doppler was used if additional information was needed, and all patients were rescanned during the 2nd trimester to confirm or negate our diagnosis. Results The cardiac findings were normal at both scans in 3108 pregnancies. The same cardiac abnormality was detected at both scans in 79 cases. In 36 cases there was false-positive diagnosis at the early scan; in 20 of these cases, there were mildly abnormal functional findings early in pregnancy with no abnormality found later. In 17 fetuses, there was discordance between the early and later diagnosis due to missed or incorrect diagnoses. The best time to do fetal heart examination during 1st trimester is between 13 and 13 + 6 weeks. Conclusion A high degree of accuracy in the identification of congenital heart disease (CHD) can be achieved by a 1st trimester fetal echocardiography.

PMID: 31494637 [PubMed - indexed for MEDLINE]

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A case of hidradenitis suppurativa linked to trisomy 1q.

J Eur Acad Dermatol Venereol. 2019 Oct;33 Suppl 6:32-33

Authors: Skroza N, Mambrin A, Tolino E, Bernardini N, Proietti I, Anzalone A, Marchesiello A, Porta N, Petrozza V, Potenza C

Hidradenitis suppurativa (HS) is a chronic relapsing disorder of the apocrine gland affecting mainly areas subjected to friction (e.g. the axillae, groin, perineum and medial aspects of the thighs). This condition can be linked to different comorbidities: autoimmune and inflammatory disease, hormone-related disorders, obesity and the metabolic syndrome, as well as rare syndromes such as Bazex-Dupré-Christol, Down's, KID, PAPASH, PASS, PASH, and SAPHO syndromes, or Dowling-Degos disease. We report a case of severe HS in a patient with Trisomy 1q;13, a very rare cytogenetic anomaly characterized by severe anomalies including dysmorphisms, multiple congenital malformations, heart defects and intellectual disability.

PMID: 31535764 [PubMed - indexed for MEDLINE]

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Treatment strategies for mixed aortic valve disease in nonelderly patients.

Expert Rev Cardiovasc Ther. 2019 Dec;17(12):873-882

Authors: Von Stumm M, Petersen J, Westermann D, Reichenspurner H, Girdauskas E

Introduction: Mixed aortic valve disease (MAVD) is defined by simultaneous occurrence of aortic stenosis (AS) and aortic regurgitation (AR). In our review, we focus on treatment options for nonelderly MAVD patients (age<55 years), who suffer from congenital aortic valve disease (unicuspid/bicuspid aortic valves).Areas covered: A systematic literature search was performed on PubMed and Embase databases using the following terms: mixed aortic valve disease, aortic stenosis/regurgitation, bicuspid/unicuspid aortic valve, mechanical/bioprosthetic aortic valve replacement, TAVR, Ross procedure. After preselection of title and abstracts, two authors (M.S. and E.G.) assessed the methodological quality of the full-text articles prior to final inclusion in the manuscript.Expert opinion: Currently, no ideal valvular substitutes are available in the treatment of nonelderly MAVD patients. Mechanical valves are associated with a reduced life expectancy due to a combination of prothesis-associated factors, mainly thrombotic and bleeding complications. Bioprostheses degenerate in the second decade and re-operations are inevitable, which also limit life expectancy. Long-term outcomes and durability of transcatheter aortic valve replacement are currently unknown. Finally, only Ross procedure is a therapeutic option with excellent long-term outcomes comparable to the healthy population. However, the Ross procedure has some important drawbacks and should therefore be only performed in expert centers and in well-selected patients.

PMID: 31813303 [PubMed - indexed for MEDLINE]

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Maternal Comorbidities and Complications of Delivery in Pregnant Women With Congenital Heart Disease.

J Am Coll Cardiol. 2019 05 07;73(17):2181-2191

Authors: Schlichting LE, Insaf TZ, Zaidi AN, Lui GK, Van Zutphen AR

BACKGROUND: Pregnant women with congenital heart defects (CHDs) may be at increased risk for adverse events during delivery.
OBJECTIVES: This study sought to compare comorbidities and adverse cardiovascular, obstetric, and fetal events during delivery between pregnant women with and without CHDs in the United States.
METHODS: Comorbidities and adverse delivery events in women with and without CHDs were compared in 22,881,691 deliveries identified in the 2008 to 2013 National Inpatient Sample using multivariable logistic regression. Among those with CHDs, associations by CHD severity and presence of pulmonary hypertension (PH) were examined.
RESULTS: There were 17,729 deliveries to women with CHDs (77.5 of 100,000 deliveries). These women had longer lengths of stay and higher total charges than women without CHDs. They had greater odds of comorbidities, including PH (adjusted odds ratio [aOR]: 193.8; 95% confidence interval [CI]: 157.7 to 238.0), congestive heart failure (aOR: 49.1; 95% CI: 37.4 to 64.3), and coronary artery disease (aOR: 31.7; 95% CI: 21.4 to 47.0). Greater odds of adverse events were observed, including heart failure (aOR: 22.6; 95% CI: 20.5 to 37.3), arrhythmias (aOR: 12.4; 95% CI: 11.0 to 14.0), thromboembolic events (aOR: 2.4; 95% CI: 2.0 to 2.9), pre-eclampsia (aOR: 1.5; 95% CI: 1.3 to 1.7), and placenta previa (aOR: 1.5; 95% CI: 1.2 to 1.8). Cesarean section, induction, and operative vaginal delivery were more common, whereas fetal distress was less common. Among adverse events in women with CHDs, PH was associated with heart failure, hypertension in pregnancy, pre-eclampsia, and pre-term delivery; there were no differences in most adverse events by CHD severity.
CONCLUSIONS: Pregnant women with CHDs were more likely to have comorbidities and experience adverse events during delivery. These women require additional monitoring and care.

PMID: 31047006 [PubMed - indexed for MEDLINE]

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Assessment of quality of life among parents of children with congenital heart disease using WHOQOL-BREF: a cross-sectional study from Northwest Saudi Arabia.

Health Qual Life Outcomes. 2019 Dec 16;17(1):183

Authors: Khoshhal S, Al-Harbi K, Al-Mozainy I, Al-Ghamdi S, Aselan A, Allugmani M, Salem S, El-Agamy D, Abo-Haded H

BACKGROUND AND AIMS: Health-related quality of life (HRQOL) has garnered increasing interest especially for health care providers and researchers. The study aims to evaluate the HRQOL in parents of congenital heart disease (CHD) children, and to clarify the effect of the disease severity on the outcome of the HRQOL perception. Also, to analyze the internal consistency of the Arabic version of the World Health Organization (WHO) QOL-BREEF tool in order to determine whether the tool had good validity for the target population.
METHODS: A cross-sectional study. The HRQOL perception was evaluated using WHOQOL-BREF questionnaire, and the internal consistency of the tool was tested using Cronbach's alpha (α-C), RESULTS: The study sample consisted of 200 individuals, 120 parents of CHD children, compared to 80 parents of children with minor illnesses (mean age of participating parents = 35.1 ± 9.8 years). While evaluating the HRQOL, the group of parents of children with minor illnesses had higher scores than the total group of parents of CHD children in all domains, indicating a better HRQOL. Class-IV subgroup of parents of CHD children showed the most significant lower total score of domains between all classes (44.47 ± 12, p < 0.001). With respect to the internal consistency of the WHOQOL-BREF, estimation of α-C values were 0.84 points for the group of parents of CHD children, and 0.87 for the group of parents of children with minor illnesses.
CONCLUSIONS: This short-term study emphasized that, HRQOL scores among parents of CHD children are compromised, and the severity of their children illness significantly affect the total score of domains in their HRQOL perception. Furthermore, the tool showed to be practical and efficient to evaluate the QOL of parents of CHD children in our population in future researches.

PMID: 31842888 [PubMed - indexed for MEDLINE]

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Myocardial Function Following Repair of Anomalous Origin of Left Coronary Artery from the Pulmonary Artery in Children.

J Am Soc Echocardiogr. 2020 Feb 28;:

Authors: Naqvi N, Babu-Narayan SV, Krupickova S, Muthialu N, Maiya S, Chandershekar P, Cheang MH, Kostolny M, Tsang V, Marek J

OBJECTIVE: We aimed to assess the change in global and regional myocardial function before and after surgical revascularization and their added value when compared with conventional measures in children with anomalous left coronary artery from the pulmonary artery (ALCAPA).
METHODS: Advanced echocardiographic assessment was performed pre- and postoperatively in 22 children with ALCAPA (eight male; median surgery age, 0.4 years; interquartile range, 0.21-1.05) and 22 healthy controls. Measurements included global and segmental longitudinal, radial, and circumferential two-dimensional speckle-tracking strain and postsystolic index.
RESULTS: Global strains were lower in preoperative patients than in controls (longitudinal: -9% vs -21%; P < .001; circumferential: -11% vs -21%; P < .001; radial: 18% vs 60%; P < .001) and improved postoperatively when compared with preoperative findings (longitudinal: -9% pre vs -16% post; P = .002, circumferential:-11% pre vs -17% post; P = .012, radial: 18% pre vs 53% post; P = .001). Preoperatively, patients with normal global systolic function on conventional echocardiography had significantly impaired global longitudinal and radial strain compared with healthy controls. Global mechanical dyssynchrony improved significantly postoperatively (longitudinal postsystolic index 43 pre vs 6 post, P < .001; circumferential 15 pre vs 2 post, P = .001; radial 48 pre vs 5 post, P = .003). Despite overall improvement in most segments, global longitudinal and circumferential and segmental peak strain in some of the segments supplied by the ALCAPA remained postoperatively abnormal.
CONCLUSIONS: This study shows that myocardial deformation indices were a more sensitive measure of LV dysfunction in patients before and after ALCAPA repair than conventional echocardiographic measures. We believe, therefore, they should be added to routine preoperative and serial postoperative follow-up assessment.

PMID: 32122741 [PubMed - as supplied by publisher]

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Factors Associated With Increased Exercise in Adults With Congenital Heart Disease.

Am J Cardiol. 2019 09 15;124(6):947-951

Authors: Connor B, Osborne W, Peir G, Smith M, John A

Despite the known benefits of regular exercise, the majority of adults with congenital heart disease (CHD) fail to meet recommended standards for physical activity. We aimed to evaluate the factors associated with exercise frequency in adults with CHD, which remain largely unknown. From September 2015 to December 2016, 446 adults with CHD completed clinical questionnaires regarding exercise frequency. Questionnaires also measured related demographic and psychosocial variables. Retrospective chart review was utilized to determine cardiac function and cardiopulmonary exercise capacity. Exercise frequency was classified as none (33%), low (<3×/month, 2%), occasional (<2×/week, 8%), or frequent (≥2×/week, 57%). Frequent exercisers were more highly educated (odds ratio [OR] 1.65, 95% confidence interval [CI] 1.06 to 2.57), reported improved sleep quality, with decreased nocturnal awakenings and sleep latency (OR 0.52, 95% CI 0.32 to 0.83), and had a lower prevalence of depression (OR 0.30, 95% CI 0.12 to 0.75). There was no significant association between exercise frequency and disease complexity or cardiac function. Of the original cohort, 74 patients completed exercise testing within 1 year of their visit. Frequent exercisers (n = 46) had better indicators of cardiovascular capacity, including higher peak maximal oxygen consumption [VO2] (28.8 ± 8.5 vs 24.6 ± 8.4, p = 0.04). After controlling for exercise frequency, higher educational attainment independently predicted an improved exercise capacity (peak [VO2]: OR = 1.09, 95% CI 1.01 to 1.18). In conclusion, frequent exercise in adults with CHD is associated with improved cardiopulmonary exercise capacity and psychosocial functioning, irrespective of underlying cardiac disease complexity and severity. Increased physical activity levels and improved exercise capacity were observed in more highly educated patients, highlighting the potential importance of socioeconomic influences on physical and mental functioning.

PMID: 31327487 [PubMed - indexed for MEDLINE]

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Contemporary epidemiology of infective endocarditis in patients with congenital heart disease: A UK prospective study.

Am Heart J. 2019 09;215:70-77

Authors: Cahill TJ, Jewell PD, Denne L, Franklin RC, Frigiola A, Orchard E, Prendergast BD

OBJECTIVES: Infective endocarditis is a life-threatening complication of congenital heart disease (CHD), but there are few studies concerning the contemporary risk profile, preceding invasive procedures and outcomes in this patient population. The aim of this study was to investigate the epidemiology of infective endocarditis (IE) in patients with CHD.
METHODS: Cases of IE in children and adults with CHD were prospectively recorded as part of the UK National Institute for Cardiovascular Outcomes Research (NICOR) National Congenital Heart Disease Audit. Patients were entered into the database between April 2008 and March 2016.
RESULTS: Eight hundred episodes of IE were recorded in 736 patients with CHD. Sixty-five patients (9%) were infants (aged <1 year), 235 (32%) were children (aged 1-15 years), and 436 (59%) were adults (aged >15 years). The most common diagnoses were Tetralogy of Fallot (n = 150, 22.8%), ventricular septal defect (n = 129, 19.6%) and bicuspid aortic valve (n = 70, 10.7%). Dental procedures preceded 67 of 635 episodes (11%) of IE, and non-dental invasive procedures preceded 177 of 644 episodes (27.4%). The most common causative organisms were streptococci, accounting for 40% of cases. Overall in-hospital mortality was 6.7%. On multivariable analysis, adverse factors associated with in-hospital mortality were staphylococcal infection and presence of an underlying atrioventricular septal defect.
CONCLUSIONS: Infective endocarditis in patients with CHD is an ongoing clinical challenge. In contemporary practice in tertiary congenital centers, 1 of 15 patients do not survive to hospital discharge. Streptococci remain the most common causative organism, and antecedent dental or medical procedures were undertaken in a significant minority in the 3 months before diagnosis. The presence of an atrioventricular septal defect or staphylococcal infection is associated with significantly increased risk of early mortality.

PMID: 31299559 [PubMed - indexed for MEDLINE]

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Scoliosis Correction with One Ventricle: A Multispecialty Approach.

World Neurosurg. 2020 Feb;134:302-307

Authors: Bustillo MA, Hussain I, Virk MS, Fu KM, Scharoun JH

BACKGROUND: Patients with single-ventricle congenital heart disease may present for scoliosis correction. These patients undergo a series of cardiac operations that create a novel circulation that has a significant impact on the management of their spinal surgery.
CASE DESCRIPTION: A 21-year-old man with severe scoliosis presented for posterior T4-L3 spinal fusion. He was born with complex congenital heart disease that resulted in his having a single functioning ventricle. He underwent a series of operations culminating in a Fontan procedure to palliate his heart disease. Both the surgical procedure and the anesthetic plan were modified based on his abnormal physiology, which led to a successful correction with no complications.
CONCLUSIONS: Patients who have undergone a Fontan procedure can successfully undergo a lengthy scoliosis correction, but it requires multidisciplinary planning.

PMID: 31715418 [PubMed - indexed for MEDLINE]

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Outcomes of Adults with Congenital Heart Disease Supported with Extracorporeal Life Support After Cardiac Surgery.

ASAIO J. 2020 Mar 03;:

Authors: Dolgner SJ, Keeshan BC, Burke CR, McMullan DM, Chan T

Patients with adult congenital heart disease (ACHD) who undergo cardiac surgery may require extracorporeal life support (ECLS) for cardiopulmonary support, but outcomes after ECLS support have not been well described. This study aimed to identify risk factors for ECLS mortality in this population. We identified 368 ACHD patients who received ECLS after cardiac surgery between 1994 and 2016 in the Extracorporeal Life Support Organization (ELSO) database, a multicenter international registry of ECLS centers. Risk factors for mortality were assessed using multivariate logistic regression. Overall mortality was 61%. In a multivariate model using precannulation characteristics, Fontan physiology (odds ratio [OR]: 5.7; 95% CI: 1.6-20.0), weight over 100 kg (OR: 2.6; 95% CI: 1.3-5.4), female gender (OR: 1.6; 95% CI: 1.001-2.6), delayed ECLS cannulation (OR: 2.0; 95% CI: 1.2-3.2), and neuromuscular blockade (OR: 1.9; 95% CI: 1.1-3.3) were associated with increased mortality. Adding postcannulation characteristics to the model, renal complications (OR: 3.0; 95% CI: 1.7-5.2), neurologic complications (OR, 4.7; 95% CI: 1.5-15.2), and pulmonary hemorrhage (OR: 6.4; 95% CI: 1.3-33.2) were associated with increased mortality, whereas Fontan physiology was no longer associated, suggesting the association of Fontan physiology with mortality may be mediated by complications. Fontan physiology was also a risk factor for neurologic complications (OR: 8.2; 95% CI: 3.3-20.9). Given the rapid increase in ECLS use, understanding risk factors for ACHD patients receiving ECLS after cardiac surgery will aid clinicians in decision-making and preoperative planning.

PMID: 32134793 [PubMed - as supplied by publisher]