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3-Dimensional personalized planning for transcatheter pulmonary valve implantation in a dysfunctional right ventricular outflow tract.

Int J Cardiol. 2019 Dec 06;:

Authors: Pluchinotta FR, Sturla F, Caimi A, Giugno L, Chessa M, Giamberti A, Votta E, Redaelli A, Carminati M

Abstract
BACKGROUND: Identification of adequate landing zone for transcatheter pulmonary valve implantation (TPVI) is crucial to successfully treat an aneurysmatic native right ventricle outflow tract (RVOT); three-dimensional (3D) patient-tailored digital and physical printed models are available but their actual strengths and weaknesses still not well documented. The aim of the study was to tackle TPVI planning in the dysfunctional and borderline RVOT exploiting both digital and physical printed 3D patient-specific models.
METHODS: Electrocardiographically gated computed tomography (CT) angiography was segmented and anatomical RVOT geometrical changes dynamically tracked throughout the cardiac cycle using in-house processing. A compliant 3D-printed model was manufactured from the diastolic rest phase to test in vitro the catheter-based procedure feasibility; results were compared against CT-derived in vivo measurements and the actual catheterization outcome.
RESULTS: CT-gated analysis successfully quantified in vivo RVOT dynamic changes corroborating the feasibility of non-conventional pulmonary jailing percutaneous intervention. Clinicians used the 3D-printed model to test the steps of the jailing procedure; yet, the deformable 3D model printed at diastole underestimated the final implant dimensions obtained during cardiac catheterization by the same operators.
CONCLUSIONS: Multidisciplinary synergy between CT-gated analysis and pre-procedural tests on 3D-printed phantoms can help the interventional team to tackle complex TPVI procedures. To fully exploit 3D-printed models, adequate selection of the still frame to print and tuning of printing material properties is crucial and can be aided by 3D dynamic virtual models.

PMID: 31839428 [PubMed - as supplied by publisher]

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The Evolution of the Minimally Invasive Approach and Conventional Median Sternotomy for Coronary Artery Fistula Correction.

Heart Lung Circ. 2019 Aug;28(8):1277-1282

Authors: Song L, Dong Y, Wang B, Li J, Qi X, Gao X, Hua Z, Tao C, He B, Tao L

Abstract
BACKGROUND: Minimally invasive cardiac surgery has become a safe and cosmetic alternative to standard median sternotomy. This retrospective study reviews our results and experience with the minimally invasive approach for congenital coronary artery fistula correction, compared with conventional approach.
METHODS: From February 2001 to June 2016, 110 patients with isolated coronary artery fistula (CAF) in our centre underwent correction through minimally invasive approach (MIA) (n=65) or standard median sternotomy (SMS) (n=45). Cardiopulmonary bypass (CPB) was used in 16 patients in the SMS group, and all the other patients underwent the procedure without CPB through a standard median sternotomy or minimally invasive approach.
RESULTS: There was no in-hospital mortality and no patients reverted to a median sternotomy in the MIA group. Subxiphoid incision (32 cases) and parasternal incision (28 cases) were the most common approaches used for the procedure. The operative time was 67.82±14.4minutes in MIA group and 107.04±27.91minutes (p=0.0001) in the SMS group. The intubation time was 3.58±2.33hours in the MIA group and 6.1±3.26hours in the SMS group (p=0.0001); the intensive care unit (ICU) stay was 10.04±7.95hours in the MIA group and 19.74±7.81hours in the SMS group (p=0.0001). Three patients (two in MIA Group vs one in SMS Group, p=0.787) were identified with a trivial residual shunt during the procedure, which had disappeared by discharge.
CONCLUSIONS: Minimally invasive approach can provide an excellent surgical exposure for CAF ligation in selective patients compared with SMS. It is a safe and cosmetic alternative to conventional treatment and minimised the length of stay.

PMID: 30054126 [PubMed - indexed for MEDLINE]

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Changes in Adrenoceptor and GRK Expression in Patients With Chronic Pulmonary Regurgitation.

Rev Esp Cardiol (Engl Ed). 2019 Jul;72(7):569-576

Authors: Rodríguez-Serrano M, Rueda Soriano J, Buendía Fuentes F, Osa Sáez AM, Montó Guillot F, D'Ocon Navaza P, Aguero J, Oliver E, Serrano F, Martínez-Dolz L

Abstract
INTRODUCTION AND OBJECTIVES: Pulmonary regurgitation (PR) is a frequent complication after repair of congenital heart disease. Lymphocyte expression of adrenoceptors (β1 and β2) and kinases (GRK2, GRK3, and GRK5) reflects the neurohumoral changes that occur in heart failure (HF). The main objective of this study was to describe the gene expression of these molecules in circulating lymphocytes in patients with severe PR.
METHODS: A prospective study was conducted to analyze lymphocyte expression of these molecules in patients with severe PR and compare it with expression in healthy controls and patients with advanced HF.
RESULTS: We studied 35 patients with severe PR, 22 healthy controls, and 13 patients with HF. Multiple comparisons analysis showed that β2-adrenoceptor gene expression levels were higher in the control group than in patients in the PR and HF groups and that expression in the latter 2 groups was similar (748.49 [rank 1703.87] vs 402.80 [rank 1210.81] vs 287.46 [rank 685.69] P = .001). Similar findings were obtained in gene expression of GRK2 (760.89 [rank 1169.46] vs 445.17 [rank 1190.69] vs 284.09 [rank 585.27] P < .001). There were no differences in expression levels of these molecules according to clinical variables in patients with PR.
CONCLUSIONS: The gene expression pattern of GRK2 and β2-adrenoceptor as molecular markers of cardiac dysfunction was altered in patients with severe PR compared with controls and was similar to expression in patients with advanced HF.

PMID: 30104167 [PubMed - indexed for MEDLINE]

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Real-time cardiovascular MR with spatio-temporal artifact suppression using deep learning-proof of concept in congenital heart disease.

Magn Reson Med. 2019 02;81(2):1143-1156

Authors: Hauptmann A, Arridge S, Lucka F, Muthurangu V, Steeden JA

Abstract
PURPOSE: Real-time assessment of ventricular volumes requires high acceleration factors. Residual convolutional neural networks (CNN) have shown potential for removing artifacts caused by data undersampling. In this study, we investigated the ability of CNNs to reconstruct highly accelerated radial real-time data in patients with congenital heart disease (CHD).
METHODS: A 3D (2D plus time) CNN architecture was developed and trained using synthetic training data created from previously acquired breath hold cine images from 250 CHD patients. The trained CNN was then used to reconstruct actual real-time, tiny golden angle (tGA) radial SSFP data (13 × undersampled) acquired in 10 new patients with CHD. The same real-time data was also reconstructed with compressed sensing (CS) to compare image quality and reconstruction time. Ventricular volume measurements made using both the CNN and CS reconstructed images were compared to reference standard breath hold data.
RESULTS: It was feasible to train a CNN to remove artifact from highly undersampled radial real-time data. The overall reconstruction time with the CNN (including creation of aliased images) was shown to be >5 × faster than the CS reconstruction. In addition, the image quality and accuracy of biventricular volumes measured from the CNN reconstructed images were superior to the CS reconstructions.
CONCLUSION: This article has demonstrated the potential for the use of a CNN for reconstruction of real-time radial data within the clinical setting. Clinical measures of ventricular volumes using real-time data with CNN reconstruction are not statistically significantly different from gold-standard, cardiac-gated, breath-hold techniques.

PMID: 30194880 [PubMed - indexed for MEDLINE]

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Caval to pulmonary 3D flow distribution in patients with Fontan circulation and impact of potential 4D flow MRI error sources.

Magn Reson Med. 2019 02;81(2):1205-1218

Authors: Jarvis K, Schnell S, Barker AJ, Rose M, Robinson JD, Rigsby CK, Markl M

Abstract
PURPOSE: Uneven flow distribution in patients with Fontan circulation is suspected to lead to complications. 4D flow MRI offers evaluation using time-resolved pathlines; however, the potential error is not well understood. The aim of this study was to systematically assess variability in flow distribution caused by well-known sources of error.
METHODS: 4D flow MRI was acquired in 14 patients with Fontan circulation. Flow distribution was quantified by the % of caval venous flow pathlines reaching the left and right pulmonary arteries. Impact of data acquisition and data processing uncertainties were investigated by (1) probabilistic 4D blood flow tracking at varying noise levels, (2) down-sampling to mimic acquisition at different spatial resolutions, (3) pathline calculation with and without eddy current correction, and (4) varied segmentation of the Fontan geometry to mimic analysis errors.
RESULTS: Averaged among the cohort, uncertainties accounted for flow distribution errors from noise ≤3.2%, low spatial resolution ≤2.3% to 3.8%, eddy currents ≤6.4%, and inaccurate segmentation ≤3.9% to 9.1% (dilation and erosion, respectively). In a worst-case scenario (maximum additive errors for all 4 sources), flow distribution errors were as high as 22.5%.
CONCLUSION: Inaccuracies related to postprocessing (segmentation, eddy currents) resulted in the largest potential error (≤15.5% combined) whereas errors related to data acquisition (noise, low spatial resolution) had a lower impact (≤5.5%-7.0% combined). Whereas it is unlikely that these errors will be additive or affect the identification of severe asymmetry, these results illustrate the importance of eddy current correction and accurate segmentation to minimize Fontan flow distribution errors.

PMID: 30277276 [PubMed - indexed for MEDLINE]

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Characteristics and outcomes of patients ≤ 75 years who underwent transcatheter aortic valve implantation: insights from the SOURCE 3 Registry.

Clin Res Cardiol. 2019 Jul;108(7):763-771

Authors: Frank D, Abdel-Wahab M, Gilard M, Digne F, Souteyrand G, Caussin C, Collart F, Letocart V, Wöhrle J, Kuhn C, Hovorka T, Baumgartner H

Abstract
BACKGROUND: Current trials and registries of transcatheter valve implantation (TAVI) mostly include patients older than 75 years. Little is known about younger patients who undergo this treatment. We investigated comorbidities among patients < 75 years old who underwent TAVI in the SAPIEN 3™ European post-approval SOURCE 3 Registry, and analysed outcomes at 30 days and 1 year.
METHODS AND RESULTS: Three age groups of patients were analysed for outcomes and characteristics: < 75 (n = 235), 75-80 (n = 391) and ≥ 80 years (n = 1320). Overall, the mean age was 81.6 ± 6.7 years; transfemoral access was used in 87.1% of patients treated with SAPIEN 3 transcatheter heart valves. The mean logistic EuroSCORE increased according to age group (12.6%, 17.3% and 19.7%, respectively, p < 0.001). Younger patients had a higher incidence of comorbidities, particularly those not included in surgical risk score assessment tools, e.g., severe liver disease, previous radiation therapy, and porcelain aorta. Mortality rates were similar between age groups at 30 days (1.7%, 2.0% and 2.3%, respectively, p = 0.79) and 1 year (14.2%, 9.3% and 13.3%, respectively, p = 0.08). However, sudden cardiac death rates were higher in the < 75 years age group compared with the ≥ 85 years age group (20.7% vs. 4.8%, p = 0.010).
CONCLUSIONS: In current TAVI practice, patients younger than 75 years are a minority (12%). Despite younger age and lower surgical risk scores, this cohort was characterized by comorbidities not accounted for by traditional surgical risk scores. More data are needed for this age group to guide the appropriate decision between surgery and TAVI. CLINICALTRIAL.
GOV NUMBER: NCT02698956.

PMID: 30552511 [PubMed - indexed for MEDLINE]

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Whole Exome Sequencing Reveals the Major Genetic Contributors to Nonsyndromic Tetralogy of Fallot.

Circ Res. 2019 02 15;124(4):553-563

Authors: Page DJ, Miossec MJ, Williams SG, Monaghan RM, Fotiou E, Cordell HJ, Sutcliffe L, Topf A, Bourgey M, Bourque G, Eveleigh R, Dunwoodie SL, Winlaw DS, Bhattacharya S, Breckpot J, Devriendt K, Gewillig M, Brook JD, Setchfield KJ, Bu'Lock FA, O'Sullivan J, Stuart G, Bezzina CR, Mulder BJM, Postma AV, Bentham JR, Baron M, Bhaskar SS, Black GC, Newman WG, Hentges KE, Lathrop GM, Santibanez-Koref M, Keavney BD

Abstract
RATIONALE: Familial recurrence studies provide strong evidence for a genetic component to the predisposition to sporadic, nonsyndromic Tetralogy of Fallot (TOF), the most common cyanotic congenital heart disease phenotype. Rare genetic variants have been identified as important contributors to the risk of congenital heart disease, but relatively small numbers of TOF cases have been studied to date.
OBJECTIVE: We used whole exome sequencing to assess the prevalence of unique, deleterious variants in the largest cohort of nonsyndromic TOF patients reported to date.
METHODS AND RESULTS: Eight hundred twenty-nine TOF patients underwent whole exome sequencing. The presence of unique, deleterious variants was determined; defined by their absence in the Genome Aggregation Database and a scaled combined annotation-dependent depletion score of ≥20. The clustering of variants in 2 genes, NOTCH1 and FLT4, surpassed thresholds for genome-wide significance (assigned as P<5×10-8) after correction for multiple comparisons. NOTCH1 was most frequently found to harbor unique, deleterious variants. Thirty-one changes were observed in 37 probands (4.5%; 95% CI, 3.2%-6.1%) and included 7 loss-of-function variants 22 missense variants and 2 in-frame indels. Sanger sequencing of the unaffected parents of 7 cases identified 5 de novo variants. Three NOTCH1 variants (p.G200R, p.C607Y, and p.N1875S) were subjected to functional evaluation, and 2 showed a reduction in Jagged1-induced NOTCH signaling. FLT4 variants were found in 2.4% (95% CI, 1.6%-3.8%) of TOF patients, with 21 patients harboring 22 unique, deleterious variants. The variants identified were distinct to those that cause the congenital lymphoedema syndrome Milroy disease. In addition to NOTCH1, FLT4 and the well-established TOF gene, TBX1, we identified potential association with variants in several other candidates, including RYR1, ZFPM1, CAMTA2, DLX6, and PCM1.
CONCLUSIONS: The NOTCH1 locus is the most frequent site of genetic variants predisposing to nonsyndromic TOF, followed by FLT4. Together, variants in these genes are found in almost 7% of TOF patients.

PMID: 30582441 [PubMed - indexed for MEDLINE]

Related Articles

Cardiovascular imaging approach in pre and postoperative tetralogy of Fallot.

BMC Cardiovasc Disord. 2019 01 07;19(1):7

Authors: Apostolopoulou SC, Manginas A, Kelekis NL, Noutsias M

Abstract
Advances in the medical and surgical management of Tetralogy of Fallot have led to marked increase of the number and age of survivors. Imaging in patients with Tetralogy of Fallot plays a crucial role in the diagnosis and follow up, and essentially guides management and intervention in this entity. This study systematically reviews the imaging modalities used in patients with Tetralogy of Fallot in the evaluation of preoperative and postoperative anatomic and hemodynamic lesions, as well as disease progression in this diagnosis. Various invasive and noninvasive imaging modalities, most commonly echocardiography and cardiovascular magnetic resonance, computed tomography and angiocardiography provide the imaging information required for diagnosis, management and follow up in Tetralogy of Fallot. The choice of the appropriate imaging tool or their combination is guided by the clinical question, the patient's clinical condition and contraindications as well as the strengths and weaknesses of each imaging modality. Tetralogy of Fallot is the most common complex congenital heart disease with long term survivors that need close follow up and complicated management, including multiple surgical and transcatheter interventions. Knowledge of the role and protocols of imaging in Tetralogy of Fallot is extremely important for the clinical as well as the imaging physician in order to optimize patients' management and long-term prognosis.

PMID: 30616556 [PubMed - indexed for MEDLINE]

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Selexipag treatment for pulmonary arterial hypertension associated with congenital heart disease after defect correction: insights from the randomised controlled GRIPHON study.

Eur J Heart Fail. 2019 03;21(3):352-359

Authors: Beghetti M, Channick RN, Chin KM, Di Scala L, Gaine S, Ghofrani HA, Hoeper MM, Lang IM, McLaughlin VV, Preiss R, Rubin LJ, Simonneau G, Sitbon O, Tapson VF, Galiè N

Abstract
AIMS: Patients with pulmonary arterial hypertension associated with congenital heart disease (CHD-PAH) after defect correction have a poor prognosis compared with other CHD-PAH patients. Therefore, it is important that these patients are treated as early and effectively as possible. Evidence supporting the use of PAH therapies in patients with corrected CHD-PAH from randomised controlled trials is limited. The purpose of these analyses was to characterise the corrected CHD-PAH patients from the GRIPHON study and examine the response to selexipag.
METHODS AND RESULTS: Out of the 110 patients diagnosed with corrected CHD-PAH, 55 had atrial septal defects, 38 had ventricular septal defects, 14 had persistent ducti arteriosus, and 3 had defects not further specified. Hazard ratios (HR) and 95% confidence intervals (CI) for the primary composite endpoint were calculated using Cox proportional hazard models. Compared with the non-CHD patients from GRIPHON, patients with corrected CHD-PAH were slightly younger, with a greater proportion being treatment-naive and in World Health Organization functional class I/II. The rate of the primary composite endpoint of morbidity/mortality was lower in patients with corrected CHD-PAH who were treated with selexipag compared with those treated with placebo (HR 0.58; 95% CI 0.25, 1.37). The most common adverse events were those known to be related to selexipag.
CONCLUSIONS: These post-hoc analyses of GRIPHON provide valuable information about a large population of patients with corrected CHD-PAH, and suggest that selexipag may delay disease progression and was well-tolerated in patients with corrected CHD-PAH.

PMID: 30632656 [PubMed - indexed for MEDLINE]

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A case of hypertrophic cardiomyopathy combined with muscular ventricular septal defect and abnormal origin of right coronary artery.

BMC Cardiovasc Disord. 2019 01 14;19(1):16

Authors: Zheng GM, Bai J, Tang JM, Zhu FC, Jing HX

Abstract
BACKGROUND: Hypertrophic cardiomyopathy (HCM) is a myocardial disease. However, the coexistence of HCM with muscular ventricular septal defect (VSD), especially those with both incomplete spontaneous closure and coronary abnormal origin, is relatively rare.
CASE PRESENTATION: We report herein a unique case of HCM accompanied with incomplete spontaneous closure of muscular VSD and abnormal origin of right coronary artery (RCA) in a 26-year-old man, which was diagnosed by combination of transthoracic 2-dimensional (2D), color Doppler, Contrast-enhanced echocardiography and computed tomography angiography (CTA).
CONCLUSIONS: To our knowledge, this is the first report that HCM along with the incomplete spontaneous closure of muscular VSD and anomalous RCA arising from left coronary sinus was revealed through combination of transthoracic 2D, color Doppler, Contrast-enhanced echocardiography and CTA. These observations indicated that other associated anomalies in patients with HCM could be easily missed if examined by the single echocardiography. Therefore, HCM-associated congenital abnormalities should be screened by combination of transthoracic 2D, color Doppler, contrast-enhanced echocardiography, and CTA.

PMID: 30642255 [PubMed - indexed for MEDLINE]

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