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Ventricular force-frequency relationships during biventricular or multisite pacing in congenital heart disease.

Congenit Heart Dis. 2018 Oct 15;:

Authors: Baskar S, Redington AN, Khoury PR, Knilans TK, Spar DS, Czosek RJ

Abstract
BACKGROUND: Traditional indices to evaluate biventricular (BiV) pacing are load dependent, fail to assess dynamic changes, and may not be appropriate in patients with congenital heart disease (CHD). We therefore measured the force-frequency relationship (FFR) using tissue Doppler-derived isovolumic acceleration (IVA) to assess the dynamic adaption of the myocardium and its variability with different ventricular pacing strategies.
METHODS: This was a prospective pilot study of pediatric and young adult CHD patients with biventricular or multisite pacing systems. Color-coded myocardial velocities were recorded at the base of the systemic ventricular free wall. IVA was calculated at resting heart rate and with incremental pacing. FFR curves were obtained by plotting IVA against heart rate for different ventricular pacing strategies.
RESULTS: Ten patients were included (mean: 22 ± 7 years). The FFR identified a best and worst ventricular pacing strategy for each patient, based on the AUC at baseline, submaximal, and peak heart rates (P < .001). However, there was no single best ventricular pacing strategy that was optimal for all patients. Additionally, the best ventricular pacing strategy often differed within the same patient at different heart rates.
CONCLUSION: This novel assessment demonstrates a wide variability in optimal ventricular pacing strategy. These inherent differences may play a role in the unpredictable clinical response to BiV pacing in CHD, and emphasizes an individualized approach. Furthermore, the optimal ventricular pacing varies with heart rate within individuals, suggesting that rate-responsive ventricular pacing modulation may be required to optimize ventricular performance.

PMID: 30324754 [PubMed - as supplied by publisher]

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Features associated with pulmonary arterial hypertension in Chinese hospitalized systemic lupus erythematosus patients.

Clin Rheumatol. 2018 Jun;37(6):1547-1553

Authors: Xu SZ, Yan Liang, Li XP, Li XM, Shuai ZW, Leng RX, Pan HF, Ye DQ

Abstract
Pulmonary arterial hypertension (PAH) is an increasingly recognized complication of systemic lupus erythematosus (SLE). This study aims to estimate the point prevalence of PAH and identify risk factors for PAH in a large cohort of hospitalized SLE patients. We have collected the medical records of patients hospitalized with SLE at the First Affiliated Hospital of Anhui Medical University and Anhui Provincial Hospital. Resting transthoracic echocardiography (TTE) was used to estimate pulmonary artery pressure (PAP) and PAH was defined as systolic PAP (PASP) > 30 mmHg. Patients with other connective tissue diseases, aPL syndrome, left heart disease, valvular heart disease, congenital heart disease, HIV, and portal hypertension were excluded because of diseases affecting the PAP. We assessed potential risk factors for PAH such as thrombogenic factors, SLE clinical manifestations, laboratory abnormalities and disease activity. Ninety-five were diagnosed with PAH of 1639 patients with SLE. The presence of high fibrinogen, serositis, and thrombocytopenia were significantly higher in patients with PAH than in those without PAH (all P < 0.05). Multivariate logistic regression found the associations between high fibrinogen (OR = 1.629), serositis (OR = 2.866), and thrombocytopenia (OR = 1.825) with PAH. The point prevalence of PAH was 5.8% in our cohort of patients with SLE. The significant association of high fibrinogen, serositis, and thrombocytopenia with PAH suggested that hypercoagulable state, organ damage, and hematological abnormality may all contribute to the development of PAH in SLE. This is important, as it is treatable.

PMID: 29520672 [PubMed - in process]

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Prematurity, ventricular septal defect and dysmorphisms are independent predictors of pathogenic copy number variants: a retrospective study on array-CGH results and phenotypical features of 293 children with neurodevelopmental disorders and/or multiple congenital anomalies.

Ital J Pediatr. 2018 Mar 09;44(1):34

Authors: Maini I, Ivanovski I, Djuric O, Caraffi SG, Errichiello E, Marinelli M, Franchi F, Bizzarri V, Rosato S, Pollazzon M, Gelmini C, Malacarne M, Fusco C, Gargano G, Bernasconi S, Zuffardi O, Garavelli L

Abstract
BACKGROUND: Since 2010, array-CGH (aCGH) has been the first-tier test in the diagnostic approach of children with neurodevelopmental disorders (NDD) or multiple congenital anomalies (MCA) of unknown origin. Its broad application led to the detection of numerous variants of uncertain clinical significance (VOUS). How to appropriately interpret aCGH results represents a challenge for the clinician.
METHOD: We present a retrospective study on 293 patients with age range 1 month - 29 years (median 7 years) with NDD and/or MCA and/or dysmorphisms, investigated through aCGH between 2005 and 2016. The aim of the study was to analyze clinical and molecular cytogenetic data in order to identify what elements could be useful to interpret unknown or poorly described aberrations. Comparison of phenotype and cytogenetic characteristics through univariate analysis and multivariate logistic regression was performed.
RESULTS: Copy number variations (CNVs) with a frequency < 1% were detected in 225 patients of the total sample, while 68 patients presented only variants with higher frequency (heterozygous deletions or amplification) and were considered to have negative aCGH. Proved pathogenic CNVs were detected in 70 patients (20.6%). Delayed psychomotor development, intellectual disability, intrauterine growth retardation (IUGR), prematurity, congenital heart disease, cerebral malformations and dysmorphisms correlated to reported pathogenic CNVs. Prematurity, ventricular septal defect and dysmorphisms remained significant predictors of pathogenic CNVs in the multivariate logistic model whereas abnormal EEG and limb dysmorphisms were mainly detected in the group with likely pathogenic VOUS. A flow-chart regarding the care for patients with NDD and/or MCA and/or dysmorphisms and the interpretation of aCGH has been made on the basis of the data inferred from this study and literature.
CONCLUSION: Our work contributes to make the investigative process of CNVs more informative and suggests possible directions in aCGH interpretation and phenotype correlation.

PMID: 29523172 [PubMed - in process]

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Persistent Left Superior Vena Cava: Imaging Correlation.

J Invasive Cardiol. 2018 May;30(5):E39-E40

Authors: Pineda AM, Gowani SA, Mihos CG, Beohar N, Santana O

Abstract
In this heart failure patient, TEE images confirm the presence of the persistent left SVC with a dilated coronary sinus, severe mitral regurgitation, and aortic stenosis. Computed tomography reveals an isolated persistent left SVC and rules out anomalous pulmonary vein drainage or additional congenital disease.

PMID: 29715168 [PubMed - in process]

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