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Echocardiographic evaluation of cardiac function after cancer chemotherapy.

J Echocardiogr. 2018 03;16(1):20-27

Authors: Negishi T, Negishi K

Progress in cancer therapy has led to improved prognosis of patients with cancer and thus to a continuous rise of cancer survivors. However, it has simultaneously increased cardiovascular morbidity and mortality rates due to direct and/or indirect side effects of anticancer treatment. Similar to the rapid rise of patients with adult congenital disease, the number of patients suffering or at risk of cardiotoxicity has been steeply increasing and getting an emerging issue. Among the many facets of chemotherapy-induced cardiovascular toxicity, this review attempts to summarize echocardiographic evaluation of cardiac function after cancer chemotherapy by reviewing the definition, risk factors, brief history, limitation of left ventricular ejection fraction and myocardial strain imaging, as well as the limitations of this technique.

PMID: 28698984 [PubMed - indexed for MEDLINE]

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Cystic Fibrosis: From Qualification to Lung Transplantation, a Single Center Experience.

Ann Transplant. 2019 Apr 05;24:185-190

Authors: Ochman M, Latos M, Urlik M, Stącel T, Nęcki M, Tatoj Z, Zawadzki F, Wajda-Pokrontka M, Przybyłowski P, Zembala M

BACKGROUND Cystic fibrosis (CF) is congenital multisystem disorder, that leads to gradual deterioration of pulmonary function. Advancements in therapy of CF-related lung disease have delayed its progression. However, lung transplantation remains the only therapeutic option for majority of such patients. Aim of the study was to assess qualification process and outcome of lung transplantation as a treatment of CF patients qualified in a single center between 2011 and 2018. MATERIAL AND METHODS This retrospective study assessed 41 patients who were qualified to be treated by means of lung transplantation due to CF in Lung Transplant Program of Silesian Center for Heart Diseases between 2011 and 2018. Analysis of patients during qualification process and after lung transplantation was performed. Lung recipients were observed during 1-year follow-up by means of pulmonary function tests. RESULTS 1-year survival was noted among 80% of the patients; 3-year survival and 5-year survival were noted among 70% of the recipients. Mean forced expiratory volume in 1 second (FEV1) increased after lung transplantation: 21.19% at qualification; and 76.67% at 12 months after lung transplantation. Mean forced vital capacity (FVC) results also improved: 34.18% at qualification and 78.34% at 12 months after lung transplantation. The 6-minute walk test (6MWT) before and after treatment noted an increase of 175.55 m. CONCLUSIONS Lung transplantation improves respiratory capacity of CF patients and prolongs their life.

PMID: 30948702 [PubMed - indexed for MEDLINE]

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Pulmonary Valve Replacement: A Single-Institution Comparison of Surgical and Transcatheter Valves.

Ann Thorac Surg. 2018 09;106(3):807-813

Authors: Sharma V, Griffiths ER, Eckhauser AW, Gray RG, Martin MH, Zhang C, Presson AP, Burch PT

BACKGROUND: Transcatheter pulmonary valve replacement (TPVR) is increasingly utilized to address residual or recurrent right ventricular outflow tract pathology in congenital heart disease patients. We aimed to evaluate clinical outcomes and resource utilization comparing TPVR to surgical pulmonary valve replacement (SPVR) for this patient population.
METHODS: From January 2010 until December 2015, 224 patients underwent pulmonary valve replacement (SPVR = 100 and TPVR = 124). These groups were assessed and compared for survival, reoperation, reintervention (surgical or catheter based), hospital costs, and hemodynamic performance.
RESULTS: There were no mortalities in either group. Despite SPVR patients being significantly younger (12 ± 7 years of age versus 19 ± 13 years of age; p < 0.001) and smaller (body surface area 1.2 ± 0.5 m2 versus 1.4 ± 0.5 m2; p < 0.001), they had similar median valve size implanted (23 mm [interquartile range, 21 to 27 mm) versus 22 mm [interquartile range, 20 to 22 mm]). There was no difference in the adjusted peak gradient (SPVR 24.1 ± 15.1 mm Hg versus TPVR 20.7 ± 11.4 mm Hg; p = 017) at last follow-up. Cumulative freedom from reintervention at 4 years was similar between groups (p = 0.98). TPVR could not be placed in 34 patients either due to coronary compression or excessive outflow tract diameter. SPVR patients had longer hospital length of stay (4.1 ± 1.8 days versus 1.2 ± 0.7 days; p < 0.001). TPVR patients had higher rates of infective endocarditis (4.8% versus 0%; p < 0.001) and overall hospital costs ($57,221 ± $13091 versus $44,366 ± $16,519; p < 0.001).
CONCLUSIONS: Hemodynamic performance is similar between SPVR and TPVR with similar rates of reintervention. While SPVR patients have a longer hospital length of stay, TPVR was associated with higher rates of infective endocarditis, hospital costs, and failure to implant.

PMID: 29733826 [PubMed - indexed for MEDLINE]

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Expanding the fetal phenotype: Prenatal sonographic findings and perinatal outcomes in a cohort of patients with a confirmed 22q11.2 deletion syndrome.

Am J Med Genet A. 2018 08;176(8):1735-1741

Authors: Schindewolf E, Khalek N, Johnson MP, Gebb J, Coleman B, Crowley TB, Zackai EH, McDonald-McGinn DM, Moldenhauer JS

22q deletion syndrome (22q11.2DS) is most often correlated prenatally with congenital heart disease and or cleft palate. The extracardiac fetal phenotype associated with 22q11.2DS is not well described. We sought to review both the fetal cardiac and extracardiac findings associated with a cohort of cases ascertained prenatally, confirmed or suspected to have 22q11.2DS, born and cared for in one center. A retrospective chart review was performed on a total of 42 cases with confirmed 22q11.2DS to obtain prenatal findings, perinatal outcomes and diagnostic confirmation. The diagnosis was confirmed prenatally in 67% (28/42) and postnatally in 33% (14/42). The majority (81%) were associated with the standard LCR22A-LCR22D deletion. 95% (40/42) of fetuses were prenatally diagnosed with congenital heart disease. Extracardiac findings were noted in 90% (38/42) of cases. Additional findings involved the central nervous system (38%), gastrointestinal (14%), genitourinary (16.6%), pulmonary (7%), skeletal (19%), facial dysmorphism (21%), small/hypoplastic thymus (26%), and polyhydramnios (30%). One patient was diagnosed prenatally with a bilateral cleft lip and cleft palate. No fetus was diagnosed with intrauterine growth restriction. The average gestational age at delivery was 38 weeks and average birth weight was 3,105 grams. Sixty-two percentage were delivered vaginally and there were no fetal demises. A diagnosis of 22q11.2 deletion syndrome should be considered in all cases of prenatally diagnosed congenital heart disease, particularly when it is not isolated. Microarray is warranted in all cases of structural abnormalities diagnosed prenatally. Prenatal diagnosis of 22q11.2 syndrome can be used to counsel expectant parents regarding pregnancy outcome and guide neonatal management.

PMID: 30055034 [PubMed - indexed for MEDLINE]

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Thromboembolic Risk After Atriopulmonary, Lateral Tunnel, and Extracardiac Conduit Fontan Surgery.

J Am Coll Cardiol. 2019 Aug 27;74(8):1071-1081

Authors: Deshaies C, Hamilton RM, Shohoudi A, Trottier H, Poirier N, Aboulhosn J, Broberg CS, Cohen S, Cook S, Dore A, Fernandes SM, Fournier A, Kay J, Mondésert B, Mongeon FP, Opotowsky AR, Proietti A, Ting J, Zaidi A, Khairy P, Alliance for Adult Research in Congenital Cardiology (AARCC)

BACKGROUND: Thromboembolic events contribute greatly to morbidity and mortality following Fontan surgery for univentricular hearts.
OBJECTIVES: This study sought to evaluate the effect of type of Fontan surgery on thromboembolic risk.
METHODS: A North American multicenter retrospective cohort study enrolled 522 patients with Fontan palliation consisting of an atriopulmonary connection (APC) (21.4%), lateral tunnel (LT) (41.8%), or extracardiac conduit (EC) (36.8%). Thromboembolic complications and new-onset atrial arrhythmia were reviewed and classified by a blinded adjudicating committee. Thromboembolic risk across surgical techniques was assessed by multivariable competing-risk survival regression.
RESULTS: Over a median follow-up of 11.6 years, 10- and 20-year freedom from Fontan conversion, transplantation, or death was 94.7% and 78.9%, respectively. New-onset atrial arrhythmias occurred in 4.4, 1.2, and 1.0 cases per 100 person-years with APC, LT, and EC, respectively. APC was associated with a 2.82-fold higher risk of developing atrial arrhythmias (p < 0.001), with no difference between LT and EC (p = 0.95). A total of 71 thromboembolic events, 32 systemic and 39 venous, occurred in 12.8% of subjects, for an overall incidence of 1.1%/year. In multivariable analyses, EC was independently associated with a lower risk of systemic (hazard ratio [HR]: 0.20 vs. LT; 95% confidence interval [CI]: 0.04 to 0.97) and combined (HR: 0.34 vs. LT; 95% CI: 0.13 to 0.91) thromboembolic events. A lower incidence of combined thromboembolic events was also observed with antiplatelet agents (HR: 0.54; 95% CI: 0.32 to 0.92) but not anticoagulation (p = 0.53).
CONCLUSIONS: The EC Fontan was independently associated with a lower thromboembolic risk after controlling for time-varying effects of atrial arrhythmias and thromboprophylaxis.

PMID: 31439217 [PubMed - in process]

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Impact of Disease Complexity on Cardiovascular Events after the Transition to an Adult Congenital Heart Disease Specialized Medical Unit.

Acta Med Okayama. 2019 Aug;73(4):307-313

Authors: Takahashi S, Akagi T, Toh N, Takaya Y, Nakagawa K, Nishii N, Ito H

The follow-up of patients with adult congenital heart disease (ACHD) at a specialized medical unit is necessary for the patients' appropriate medical care. However, limited information is available about cardiovascular events among ACHD patients. Here we investigated the type and frequency of cardiovascular events in ACHD patients in relation to disease complexity. We retrospectively analyzed the cases of 535 patients (median age 35 years) referred to our ACHD center between 2014 and 2017. We divided the patients into 3 groups based on their disease complexity. To evaluate the relationship between disease complexity and cardiovascular events, we performed univariate and multivariate survival analyses. The Simple, moderate, and complex disease groups accounted for 62%, 19%, and 19% of the patients, respectively. Apart from events related to atrial septal defect (ASD) trans-catheter treatment, the frequency of cardiovascular events was dependent on the disease complexity (event-free survival rates at 3 years were 85%, 65%, and 58%, respectively). The hazard ratios were 4.0 and 5.1 in the moderate and complex groups, respectively. With the exception of scheduled transcatheter intervention, cardiovascular events were strongly related to the disease complexity of original heart disease. However, cardiovascular events were not rare even in the simple ACHD group.

PMID: 31439953 [PubMed - in process]

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Differential impact of physical activity type on depression in adults with congenital heart disease: A multi-center international study.

J Psychosom Res. 2019 Sep;124:109762

Authors: Ko JM, White KS, Kovacs AH, Tecson KM, Apers S, Luyckx K, Thomet C, Budts W, Enomoto J, Sluman MA, Wang JK, Jackson JL, Khairy P, Cook SC, Chidambarathanu S, Alday L, Eriksen K, Dellborg M, Berghammer M, Johansson B, Mackie AS, Menahem S, Caruana M, Veldtman G, Soufi A, Fernandes SM, Callus E, Kutty S, Moons P, Cedars AM, APPROACH-IS consortium and International Society for Adult Congenital Heart Disease (ISACHD)

OBJECTIVE: This study aimed to examine the association between physical activity (PA) and depression in a large international cohort of adults with congenital heart disease (ACHD) as data about the differential impact of PA type on depression in this population are lacking.
METHODS: In 2018, we conducted a cross-sectional assessment of 3908 ACHD recruited from 24 ACHD-specialized centers in 15 countries between April 2013 to March 2015. The Hospital Anxiety and Depression Scale was used to assess self-reported depressive symptoms and the Health-Behavior Scale-Congenital Heart Disease was used to collect PA information. Cochran-Armitage tests were performed to assess trends between depressive symptom levels and PA participation. Chi-Square and Wilcoxon Rank Sum tests were utilized to examine relations between depressive symptom levels and patient characteristics. Stepwise multivariable models were then constructed to understand the independent impact of PA on depressive symptoms.
RESULTS: The overall prevalence of elevated depressive symptoms in this sample was 12% with significant differences in rates between countries (p < .001). Physically active individuals were less likely to be depressed than those who were sedentary. Of the 2 PA domains examined, sport participation rather than active commute was significantly associated with reduced symptoms of depression. After adjustment in multivariable analysis, sport participation was still significantly associated with 38% decreased probability of depressive symptoms (p < .001).
CONCLUSIONS: Sport participation is independently associated with reduced depressive symptoms. The development and promotion of sport-related exercise prescriptions uniquely designed for ACHD may improve depression status in this unique population.

PMID: 31443808 [PubMed - in process]

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Tricuspid regurgitation severity after atrial septal defect closure or pulmonic valve replacement.

Heart. 2019 Aug 23;:

Authors: Martin-Garcia AC, Dimopoulos K, Boutsikou M, Martin-Garcia A, Kempny A, Alonso-Gonzalez R, Swan L, Uebing A, Babu-Narayan SV, Sanchez PL, Li W, Shore D, Gatzoulis MA

OBJECTIVES: Cardiac surgery or catheter interventions are nowadays commonly performed to reduce volume loading of the right ventricle in adults with congenital heart disease. However, little is known, on the effect of such procedures on pre-existing tricuspid regurgitation (TR). We assessed the potential reduction in the severity of TR after atrial septal defect (ASD) closure and pulmonic valve replacement (PVR).
METHODS: Demographics, clinical and echocardiographic characteristics of consecutive patients undergoing ASD closure or PVR between 2005 and 2014 at a single centre who had at least mild preoperative TR were collected and analysed.
RESULTS: Overall, 162 patients (mean age at intervention 41.6±16.1 years, 38.3% male) were included: 101 after ASD closure (61 transcatheter vs 40 surgical) and 61 after PVR (3 transcatheter vs 58 surgical). Only 11.1% received concomitant tricuspid valve surgery (repair). There was significant reduction in the severity of TR in the overall population, from 38 (23.5%) patients having moderate or severe TR preoperatively to only 11 (6.8%) and 20 (12.3%) at 6 months and 12 months of follow-up, respectively (McNemar p<0.0001). There was a significant reduction in tricuspid valve annular diameter (p<0.0001), coaptation distance (p<0.0001) and systolic tenting area (p<0.0001). The reduction in TR was also observed in patients who did not have concomitant tricuspid valve (TV) repair (from 15.3% to 6.9% and 11.8% at 6 and 12 months, respectively, p<0.0001). On multivariable logistic regression including all univariable predictors of residual TR at 12 months, only RA area remained in the model (OR 1.2, 95% CI 1.04 to 1.37, p=0.01).
CONCLUSIONS: ASD closure and PVR are associated with a significant reduction in tricuspid regurgitation, even among patients who do not undergo concomitant tricuspid valve surgery.

PMID: 31444268 [PubMed - as supplied by publisher]

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Pregnancy in Women With a Fontan Circulation: A Systematic Review of the Literature.

Circ Cardiovasc Qual Outcomes. 2018 05;11(5):e004575

Authors: Garcia Ropero A, Baskar S, Roos Hesselink JW, Girnius A, Zentner D, Swan L, Ladouceur M, Brown N, Veldtman GR

BACKGROUND: The Fontan operation has provided life-saving palliation and adult survival for individuals born with single ventricle physiology. Many now seek advice about safe pregnancy. Little data are, however, available, consisting mainly of anecdotal experience and small series. This article seeks to review the published literature and identify lessons learnt from this collective experience.
METHODS AND RESULTS: We conducted a systematic review to evaluate maternal and fetal outcomes of pregnancy in women with a Fontan circulation. Among 1150 studies that were screened, 6 studies had sufficient longitudinal data points to qualify for meaningful inclusion, yielding 255 pregnancies in 133 women after Fontan procedure resulting in 115 live births (45%; including reports from 1986 to 2015). There was a total of 137 pregnancy losses (69%), with 115 miscarriages (45%), 19 elective terminations of pregnancy (7%), 2 stillbirths (1%), and 1 ectopic pregnancy (1%).The most common cardiovascular adverse events were supraventricular arrhythmia affecting 8.4% (range, 3%-37%) and heart failure affecting 3.9% (range, 3%-11%) of pregnancies. These complications were successfully managed with conventional approaches. No maternal deaths were reported. Postpartum hemorrhage was the predominant obstetric complication affecting 14% of the patients. Most patients were on antiplatelet agents (27%) or anticoagulants (50%) whereas only a minority (11%) were on neither. Among the 115 live births, 68 were premature (59%), 17 were small for gestational age (20%), and neonatal death occurred in 6 patients (5%).
CONCLUSIONS: The most commonly reported cardiovascular complications in patients with Fontan physiology-associated pregnancy were arrhythmia and heart failure. Miscarriages were highly prevalent as was prematurity and intrauterine growth restriction. Postpartum hemorrhage seems to be the most common obstetric complication. Large-scale data sets are needed to confirm these early observations and address the late sequelae of pregnancy in women with a Fontan circulation.

PMID: 29752389 [PubMed - indexed for MEDLINE]

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Genetic variation in folate metabolism is associated with the risk of conotruncal heart defects in a Chinese population.

BMC Pediatr. 2018 08 30;18(1):287

Authors: Wang X, Wei H, Tian Y, Wu Y, Luo L

BACKGROUND: Conotruncal heart defects (CTDs) are a subgroup of congenital heart defects that are considered to be the most common type of birth defect worldwide. Genetic disturbances in folate metabolism may increase the risk of CTDs.
METHODS: We evaluated five single-nucleotide polymorphisms (SNPs) in genes related to folic acid metabolism: methylenetetrahydrofolate reductase (MTHFR C677T and A1298C), solute carrier family 19, member 1 (SLC19A1 G80A), methionine synthase (MTR A2576G), and methionine synthase reductase (MTRR A66G), as risk factors for CTDs including various types of malformation, in a total of 193 mothers with CTD-affected offspring and 234 healthy controls in a Chinese population.
RESULTS: Logistic regression analyses revealed that subjects carrying the TT genotype of MTHFR C677T, the C allele of MTHFR A1298C, and the AA genotype of SLC19A1 G80A had significant 2.47-fold (TT vs. CC, OR [95% CI] = 2.47 [1.42-4.32], p = 0.009), 2.05-2.20-fold (AC vs. AA, 2.05 [1.28-3.21], p = 0.0023; CC vs AA, 2.20 [1.38-3.58], p = 0.0011), and 1.68-fold (AA vs. GG, 1.68 [1.02-2.70], p = 0.0371) increased risk of CTDs, respectively. Subjects carrying both variant genotypes of MTHFR A1298C and SLC19A1 G80A had a higher (3.23 [1.71-6.02], p = 0.0002) increased risk for CTDs. Moreover, the MTHFR C677T, MTHFR A1298C, and MTRR A66G polymorphisms were found to be significantly associated with the risk of certain subtypes of CTD.
CONCLUSIONS: Our data suggest that maternal folate-related SNPs might be associated with the risk of CTDs in offspring.

PMID: 30165839 [PubMed - indexed for MEDLINE]