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Body mass index stratification in hospitalized Italian adults with congenital heart disease in relation to complexity, diagnosis, sex and age.

Nutr Metab Cardiovasc Dis. 2019 Jan 29;:

Authors: Malavazos AE, Capitanio G, Chessa M, Matelloni IA, Milani V, Stella E, Al Kassem LF, Sironi F, Boveri S, Giamberti A, Masocco M, Ranucci M, Menicanti L, Morricone L

BACKGROUND AND AIMS: Adults with congenital heart disease (ACHD) are at risk of overweight and obesity, two major health problems, though underweight can be a negative prognostic factor too. Awareness of the body mass index (BMI) in ACHD is very limited. The present study describes the use and prevalence of BMI in Italian symptomatic hospitalized ACHD patients in relation to complexity by Bethesda system classification, diagnosis, sex and age.
METHODS AND RESULTS: We classified 1388 ACHD patients, aged 18-69 years, on the basis of their BMI, and compared them to the Italian reference population. In our total ACHD population we found a significantly higher prevalence of underweight compared to the Italian reference population (6.34% vs 3.20%). ACHD women were more underweight than men. Underweight decreased with age. Overweight was significantly less frequent in the total ACHD population (26.73% compared to 31.70%) in the Italian reference population. Men were more likely to be overweight than women. In statistical terms obesity was similar in the Italian reference population (10.50%) and our ACHD population (9.58%). Both overweight and obesity increased with age. Results were comparable using a diagnostic anatomical-functional classification and the Bethesda system classification.
CONCLUSIONS: In our cohort of ACHD the prevalence of underweight was double that of the Italian reference population. The prevalence of overweight was lower, while obesity was similar. Since BMI does not account for differences in body fat distribution, a future aim will be to quantify the visceral component of the adipose tissue in ACHD patients and examine their body composition in order to reflect their risk of acquired cardiovascular disease better, and either to maintain or achieve an adequate visceral component.

PMID: 30795994 [PubMed - as supplied by publisher]

A Model for Assessment of Catheterization Risk in Adults With Congenital Heart Disease.

Am J Cardiol. 2019 Feb 08;:

Authors: Taggart NW, Du W, Forbes TJ, Nykanen DG, Wax DF, Cabalka AK, Reeves JH, Du Y, Kobayashi D

The purpose of this study was to define the risk for adults with congenital heart disease who underwent cardiac catheterization and to propose a precatheterization risk scoring system. Data were prospectively collected using a multicenter registry of the Congenital Cardiovascular Interventional Study Consortium. The occurrence of serious adverse events (SAE) was correlated with 12 predefined variables. Catheterization RISk in Adult patients (CRISA) score was derived using multivariate logistic regression with backward elimination model selection method. The CRISA score was compared with the American Society of Anesthesiology score and a consensus-derived, 20-point risk score based on their ability to predict SAE. From June 2008 to September 2017, 300 adjudicated SAE's occurred in 7317 catheterization procedures (overall SAE rate 4.1%) performed in adults over 18 years of age at 27 contributing centers. Nine of the 12 tested variables were ultimately included in the CRISA score. CRISA score positively correlated with risk of SAE, and was superior to American Society of Anesthesiology and the 20-point risk score in predicting SAE. Minimal (CRISA score 0 to 2), low (3 to 7), moderate (8 to 10) and high (≥11) risk categories were identified, corresponding to 0.5%, 3.2%, 7.9%, and 16.7% risk of SAE, respectfully. In conclusion, the CRISA score reliably predicts risk of SAE in adults with congenital heart disease who underwent cardiac catheterization and may be useful for preprocedural risk assessment.

PMID: 30797558 [PubMed - as supplied by publisher]

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Risk Adjustment Tools in Congenital Heart Disease: A Consumer's View.

Circulation. 2017 11 21;136(21):2020-2021

Authors: Lock JE

PMID: 29158213 [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 Nov 01;270:120-125

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

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 - indexed for MEDLINE]

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Altered White Matter Microstructure Correlates with IQ and Processing Speed in Children and Adolescents Post-Fontan.

J Pediatr. 2018 09;200:140-149.e4

Authors: Watson CG, Stopp C, Wypij D, Bellinger DC, Newburger JW, Rivkin MJ

OBJECTIVE: To compare white matter microstructure in children and adolescents with single ventricle who underwent the Fontan procedure with healthy controls, and to explore the association of white matter injury with cognitive performance as well as patient and medical factors.
STUDY DESIGN: Fontan (n = 102) and control subjects (n = 47) underwent diffusion tensor imaging (DTI) at ages 10-19 years. Mean DTI measures (fractional anisotropy, radial diffusivity, axial diffusivity, and mean diffusivity) were calculated for 33 fiber tracts from standard white matter atlases. Voxel-wise group differences in DTI measures were assessed using Tract-Based Spatial Statistics. Associations of regional fractional anisotropy with IQ and processing speed as well as medical characteristics were examined.
RESULTS: Subjects with Fontan, compared with controls, had reduced bilateral regional and voxel-wise fractional anisotropy in multiple white matter tracts along with increased regional radial diffusivity in several overlapping tracts; regional mean diffusivity differed in 2 tracts. The groups did not differ in voxel-wise radial diffusivity or mean diffusivity. Among subjects with Fontan, fractional anisotropy in many tracts correlated positively with Full-Scale Intelligence Quotient and processing speed, although similar findings were absent in controls. Lower mean fractional anisotropy in various tracts was associated with more complications in the first operation, a greater number of total operations, and history of neurologic event.
CONCLUSIONS: Children and adolescents who have undergone the Fontan procedure have widespread abnormalities in white matter microstructure. Furthermore, white matter microstructure in several tracts is associated with cognitive performance and operative and medical history characteristics.

PMID: 29934026 [PubMed - indexed for MEDLINE]

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Membranous subaortic stenosis coexisting with hypertrophic cardiomyopathy: A case report.

J Clin Ultrasound. 2018 Nov;46(9):610-613

Authors: Xiao-Dong L, Xin-Yao L, Hui X, Xiao-Cong W

Subaortic stenosis (SAS) is a congenital heart disease, and its association with hypertrophic cardiomyopathy is very rare and clinically underappreciated. We report here a case of a 45-year-old female who was admitted to our hospital with chest tightness and shortness of breath. Both transthoracic and transesophageal echocardiography revealed asymmetric left ventricular hypertrophy and a membrane-like echo below the level of the aortic valve. This patient was diagnosed with membranous SAS with hypertrophic cardiomyopathy. Screening of her immediate family members revealed that her son also had hypertrophic cardiomyopathy. The patient chose conservative treatments and has been closely followed-up after discharge.

PMID: 30229919 [PubMed - indexed for MEDLINE]

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Prenatal Diagnosis of Criss-Cross Heart Using 4-Dimensional Color Doppler Rendering.

Pediatr Cardiol. 2019 Jan;40(1):237-239

Authors: Tsukimori K, Kitadai Y, Kan N

Criss-cross heart is a rare congenital cardiac anomaly characterized by the crossing of two ventricular inflow streams. We have demonstrated the utility of 4-dimensional color Doppler rendering in diagnosing the criss-cross heart in a fetus. Four-dimensional color Doppler rendering can demonstrate the relative direction of intracardiac blood flows and facilitate recognition of the crossover of inflow streams in the same plane, confirming the criss-cross heart diagnosis in the fetus.

PMID: 30255311 [PubMed - indexed for MEDLINE]

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Influenza B virus infection complicated by life-threatening pericarditis: a unique case-report and literature review.

BMC Infect Dis. 2019 Jan 10;19(1):40

Authors: Spoto S, Valeriani E, Locorriere L, Anguissola GB, Pantano AL, Terracciani F, Riva E, Ciccozzi M, Costantino S, Angeletti S

BACKGROUND: Acute pericarditis may occur frequently after viral infections. To our knowledge, influenza B virus infection complicated by pericarditis without myocardial involvement has never been reported. We report the first case of life-threatening pericarditis caused by influenza B virus infection.
CASE PRESENTATION: A 48-years-old woman with trisomy 21 and ostium primum atrial septal defect was transferred from Cardiology to our Internal Medicine Department for severe pericardial effusion unresponsive to ibuprofen and colchicine. Based on the recent patient history of flu-like syndrome, and presence of pleuro-pericardial effusion, a viral etiology was suspected. Laboratory evaluation and molecular assay of tracheal aspirate identified influenza B virus. Therefore, the ongoing metilprednisolone and colchicine therapy was implemented with oseltamivir with progressive patient improvement and no evidence of pericardial effusion recurrence during follow-up.
CONCLUSIONS: Especially in autumn and winter periods, clinicians should include Influenza B virus infection on differential diagnosis of pericarditis with large pericardial effusion.

PMID: 30630424 [PubMed - indexed for MEDLINE]

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IMPACT Registry and National Pediatric Cardiology Quality Improvement Collaborative: Contributions to Quality in Congenital Heart Disease.

World J Pediatr Congenit Heart Surg. 2019 Jan;10(1):72-80

Authors: Martin GR, Anderson JB, Vincent RN

The IMproving Pediatric and Adult Congenital Treatments (IMPACT) Registry and the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) are two efforts initiated to improve outcomes in the congenital heart disease field. The IMPACT Registry is focused on evaluating the use, risks, adverse events (AEs), and outcomes associated with diagnostic and common interventional catheterization procedures in all children and adults with congenital heart disease. Utilizing a modular approach, the common procedures include diagnostic cardiac catheterization, atrial septal defect device closure, patent ductus arteriosus device closure, pulmonary valvuloplasty, aortic valvuloplasty, balloon and stent angioplasty of coarctation of the aorta, pulmonary artery balloon stent angioplasty, transcatheter pulmonary valve replacement, and electrophysiology procedures including radiofrequency ablation. To date, important observations on the common procedures have been made and a risk stratification methodology has been created to allow comparisons between centers in AEs and quality improvement activity. The registry is open to international participation. The NPC-QIC was developed to reduce mortality and improve the quality of life of infants with Hypoplastic Left Heart Syndrome (HLHS) during the interstage period between discharge from the Norwood operation and admission for the bidirectional Glenn procedure. Mortality in the interstage has been reduced by 44%. The IMPACT Registry and the NPC-QIC have demonstrated value to the congenital heart disease community. The IMPACT Registry, however, has not yet demonstrated an impact on patient outcomes. The NPC-QIC, which combines both a registry with a learning collaborative with specific aims, key drivers, and change strategies, has made more significant gains with reductions in variation, growth failures, and mortality.

PMID: 30799721 [PubMed - in process]

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An initial experience of high-density mapping-guided ablation in a cohort of patients with adult congenital heart disease.

Europace. 2019 Jan 01;21(Supplement_1):i43-i53

Authors: Ernst S, Cazzoli I, Guarguagli S

AIMS: In the management of both ventricular and supraventricular tachycardia in patients with congenital heart disease (CHD) catheter ablation has now been recognized as one of the mainstays.
METHODS AND RESULTS: We review our initial experience of using the Rhythmia mapping system in a cohort of 12 adult CHD patients presenting with multiple arrhythmia substrates. A total of 78 arrhythmia maps were attempted in a total of 15 procedures, but possible due to the dilatation of the target chamber only 44% of maps were able to reconstruct the entire arrhythmia. All patients underwent pre-procedure 3D imaging (either cardiac magnetic resonance or computed tomography), but image integration was suboptimal. A median of two maps per patient were finally analysed and acquisition took in median 22 min with a median number of 12 574 (8230-18 167) mapping points. Procedural data with a total duration amounting to in median 285 (194-403) min, with a median total fluoroscopy exposure of 7.5 (5.2-10.7) min. After a median of 1.5 procedures [median of 12 (8-16 months)], nine patients remained in stable sinus rhythm or atrial paced rhythm, while three patients had further sustained recurrences. One of these passed away in end-staged heart failure.
CONCLUSION: This initial experience of using high-density mapping for arrhythmia management in patients with CHD allowed rapid acquisition of multiple maps with high accuracy to identify surgical scars and fibrosis, however, it was limited by large atrial volumes and a high percentage of incomplete maps resulting in modest clinical success.

PMID: 30801127 [PubMed - in process]