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1.
Heart ; 2020 Mar 23.
Artigo em Inglês | MEDLINE | ID: mdl-32205313

RESUMO

OBJECTIVE: To describe the intervention spectrum, complexity, and safety outcomes of catheter-based interventions in a contemporary adult congenital heart disease (ACHD) tertiary cohort. METHODS: All patients over 16 years who underwent a catheter-based intervention for ACHD in our centre between 2000 and 2016 were included. Baseline demographics, clinical characteristics, indications for and complexity of intervention, procedural complications and early and mid-term mortality were analysed. RESULTS: Overall, 1644 catheter-based interventions were performed. Intervention complexity ranged from simple (67.5%) to intermediate (26.4%) and to high (6.1%). Commonly performed procedures were atrial septal defect (33.4%) and patent foramen ovale closure (26.1%) followed by coarctation of the aorta (11.1%) and pulmonary artery interventions (7.0%). Age at index intervention was 40±16 years, 758 (46.1%) patients were male, 73.2% in New York Heart Association (NYHA) class I, 20.2% in NYHA class II, whereas 6.6% in NYHA class III/IV. In-hospital mortality was 0.7%. Median postinterventional length of stay was 1 day. Complications occurred in 129 (7.9%) with major adverse events in 21 (1.3%). One-year postintervention survival rates were 98.7% (95% CI 98.2 to 99.2). Over the study period, there was a notable shift in intervention complexity, with a predominance of simple procedures performed in early years and more complex procedures in later years. Furthermore, the case mix during the study broadened (p<0.001) with new catheter-based interventions and a more individualised approach to therapy. CONCLUSION: This study shows an increasing complexity and expanding variability of ACHD catheter-based interventions, associated with low major complications, short hospital stays and low early and mid-term mortality. Ongoing investment in ACHD catheter interventions is warranted.

2.
Ann Thorac Surg ; 2020 Mar 05.
Artigo em Inglês | MEDLINE | ID: mdl-32147413

RESUMO

BACKGROUND: Surgery in grown-ups with congenital heart disease (GUCH) is characterized by complex anatomy, comorbidities, reoperations, and technical challenges. While 30-day postoperative mortality is low, this measure might be insufficient to reflect adverse outcome monitoring. Our study aimed to establish whether prolonged intensive care unit (ICU) stay (≥7 days) and 6-month mortality were more clinically meaningful measures than 30-day mortality and to identify predictors of adverse outcome. METHODS: All consecutive GUCH patients from 1998 to 2015 were identified. Perioperative characteristics, diagnoses, and postoperative data were collected retrospectively. Predictors of 30-day, 6-month mortality, and prolonged ICU stay were determined with logistic regression. Era effect was tested for quality assurances by dividing the cohort into four time intervals. RESULTS: Within 17 years, 1093 consecutive cardiac surgical procedures were identified in 1026 GUCH patients. Thirty-day mortality improved significantly over the study period, with an overall 30-day mortality of 1.5%. Six-month mortality and prolonged ICU stay were 2.4% and 6.7% respectively. Despite a decreased number of preoperative patients in NYHA class ≥3, prolonged ICU stay increased over the eras. Predictors of adverse outcome were; NYHA class ≥3, preoperative renal failure, disease of great complexity, preoperative ventilator support, cardiopulmonary bypass time, and concomitant procedures. CONCLUSIONS: In the current era of low 30-day mortality, extended 6-month mortality and prolonged ICU stay reporting may be more realistic measures of adverse outcomes for counseling GUCH patients at risk.

3.
Arch Cardiovasc Dis ; 113(2): 113-120, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32081640

RESUMO

BACKGROUND: The indications for percutaneous pulmonary valve implantation (PPVI) have been extended to include large dysfunctional right ventricular outflow tracts (RVOTs). Prestenting of the RVOT is commonly performed before PPVI in order to ensure a stable landing zone. The AndraStent XXL (AndraMed GmbH, Reutlingen, Germany), a cobalt-chromium stent with semi-open cell design, has unique mechanical properties in this indication but is no longer available in France. AIMS: To assess the efficiency of AndraStent XXL before PPVI. METHODS: In this retrospective multicentre cohort study, 86 AndraStents XXL were implanted in 77 patients in 6 centres. RESULTS: PPVI was indicated mainly for pulmonary regurgitation (75.3%) in native or patched RVOT (88.3%). The stents were manually mounted on balloon catheters and delivered through sheaths using a conventional femoral approach. PPVI was performed successfully in 97.4% of patients after successful prestenting, generally during the same procedure (77.9%). There were no deaths associated with stent implantation, and four patients experienced five complications, mainly stent embolization, including one requiring surgery. Neither stent fracture nor dysfunction were observed in any patient during a mean follow-up of 19.2±8.7months. Stent analysis showed an excellent maximal stent expansion (97.1%) regardless of balloon size. A 22.3%±3.4 stent shortening with a 30mm balloon was observed. CONCLUSIONS: Implantation of large cobalt-chromium AndraStent XXL stents is efficient for prestenting before PPVI.

4.
Heart ; 106(6): 455-461, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31444268

RESUMO

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.

5.
Chest ; 157(1): 89-98, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31351047

RESUMO

BACKGROUND: Patients with interstitial lung disease (ILD) may develop pulmonary hypertension (PH), often disproportionate to the severity of the ILD. The right ventricular to left ventricular diameter (RV:LV) ratio measured at CT pulmonary angiogram (CTPA) has been shown to provide valuable information in patients with pulmonary arterial hypertension and to predict death or deterioration in acute pulmonary embolism. METHODS: Demographic characteristics, ILD subtype, echocardiography, and detailed CTPA measurements were collected in consecutive patients undergoing both CTPA and right heart catheterization at the Royal Brompton Hospital between 2005 and 2015. Fibrosis severity was formally scored according to CT criteria. The RV:LV ratio at CTPA was evaluated by using three different methods. Cox proportional hazards analysis was used to assess the relation of CTPA-derived parameters to predict death or lung transplantation. RESULTS: A total of 92 patients were included (64% male; mean age 65 ± 11 years) with an FVC 57 ± 20% predicted, corrected transfer factor of the lung for carbon monoxide 22 ± 8% predicted, and corrected transfer coefficient of the lung for carbon monoxide 51 ± 17% predicted. PH was confirmed at right heart catheterization in 78%. Of all the CTPA-derived measures, an RV:LV ratio ≥ 1.0 strongly predicted mortality or transplantation at univariate analysis (hazard ratio, 3.26; 95% CI, 1.49-7.13; P = .003), whereas invasive hemodynamic data did not. The RV:LV ratio remained an independent predictor at multivariate analysis (hazard ratio, 3.19; 95% CI, 1.44-7.10; P = .004), adjusting for an ILD diagnosis of idiopathic pulmonary fibrosis and CT imaging-derived ILD severity. CONCLUSIONS: An increased RV:LV ratio measured at CTPA provides a simple, noninvasive method of risk stratification in patients with suspected ILD-PH. This should prompt closer follow-up, more aggressive treatment, and consideration of lung transplantation.

6.
Int J Cardiol ; 299: 131-135, 2020 01 15.
Artigo em Inglês | MEDLINE | ID: mdl-31371115

RESUMO

BACKGROUND: Pulmonary arterial hypertension (PAH), is a rare and progressive disease with a high morbidity and mortality. Prostanoid pulmonary vasodilators are the most effective treatment for idiopathic and connective tissue associated PAH. Nonetheless, data examining their safety and efficacy in patients with Eisenmenger syndrome the most severe form of PAH, that is, related to cyanotic congenital heart disease (CHD-PAH) remains limited. AIM: To evaluate safety and the clinical efficacy of nebulised iloprost in patients with Eisenmenger syndrome who are on maximum background oral PAH therapy. METHODS: This pilot study was a randomised, double-blind, placebo-controlled, cross-over study. Patients were randomised to receive nebulised placebo or iloprost for 12 weeks and were then crossed over, with a 7-14-day washout. The primary endpoint was a change in 6-minute walk distance (6MWD). RESULTS: Sixteen patients (11 females, aged 47.3 ±â€¯9.8 year) were recruited, twelve completed the study. All were in WHO-FC III, with a resting oxygen saturation of 84 [81-87] % and a median 6MWD of 290 [260-300] m. There was no significant difference in the primary endpoint between nebulised iloprost (0[-4-9]m) and placebo (10 [-15-51]m), p = 0.58. There were no safety concerns with nebulised iloprost. CONCLUSIONS: Our pilot study provides preliminary evidence that the addition of nebulised iloprost to maximum oral PAH therapy did not improve the primary endpoint of 6MWD. Nebulised iloprost was well tolerated with no significant safety concerns in CHD-PAH.

9.
Pediatr Cardiol ; 40(8): 1752-1755, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31352491

RESUMO

Endocarditis is life threatening after percutaneous pulmonary valve implantation. We report a patient with acute heart failure caused by obstructive pulmonary vegetation in a Melody valve. Because of severe right ventricular dysfunction, immediate cardiac surgery was contraindicated. The patient underwent trans-catheter implantation of a covered stent to relieve obstruction. Following an initially good outcome, surgery was performed 2 weeks later. This was complicated by left main coronary artery tear and fatal bleeding. Despite covered stent was an effective bridge, surgery remains extremely challenging in such cases with important surrounding inflammation and fibrosis as well as potential proximity of coronary arteries.


Assuntos
Endocardite Bacteriana/cirurgia , Próteses Valvulares Cardíacas/efeitos adversos , Stents/efeitos adversos , Adulto , Vasos Coronários/lesões , Evolução Fatal , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/métodos , Humanos , Masculino
10.
Pulm Circ ; 9(2): 2045894019859474, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31246163

RESUMO

A 36-year-old woman presented with recurrent pulmonary emboli (PE) despite oral anticoagulation. She was a type I diabetic with severe gastroparesis requiring insertion of multiple long-term peripherally inserted central catheters (PICC) over a 10-year period. Imaging at presentation demonstrated a PICC-associated mobile mass in the right atrium and signs of pulmonary hypertension (PH). She was thrombolyzed and fully anticoagulated, and diabetic management without PICC strongly recommended. PH persisted, however, and she developed chronic thromboembolic pulmonary hypertension (CTEPH), for which successful pulmonary endarterectomy (PEA) surgery led to symptomatic and hemodynamic improvement. This was the first case of CTEPH reported related to long-term PICC use outside the setting of malignant disease, and a novel observation that the PEA specimen contained multiple plastic fragments. Long-term PICC placement increases the risk of CTEPH, a life-threatening, albeit treatable, complication.

11.
Open Heart ; 6(1): e000882, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31168370

RESUMO

Background: Women are underrepresented in leading medical positions and academia. The gender-gap in authorship of congenital heart disease (CHD) publications remains unknown. As determinants of gender equity in this field are poorly characterised, we aimed to quantify and characterise publications in CHD and to assess factors associated with female representation in research. Methods and results: We identified 35 118 CHD publications between 2006 and 2015 for which author gender could be ascertained. Overall, 25.0% of all authors were female. Women accounted for 30.2% and 20.8% of all first and senior authorship positions with great geographic heterogeneity. While globally female first and senior authorship increased by 0.8% and 0.6%/year, some geographic regions showed no improvement in gender representation. Significant predictors of female first authorship on logistic regression analysis were country gross domestic product, human development index, gender inequality index and a female senior author (p<0.0001 for all). Publications with a female lead author tended to be published in journals with a higher impact factor (IF) and to attract more citations compared with those with a male author. Mixed gender authorship was associated with higher IF and number of citations. Women were less disadvantaged when the analysis was confined to original research. Conclusions: While modest improvement in female authorship over time was noted, women remain underrepresented in contemporary academic CHD. Manuscripts with mixed gender authorship had higher IF and more citations. The main predictor of female first authorship was a female senior author. These data should inform policy recommendations regarding gender parity.

12.
Europace ; 21(9): 1334-1344, 2019 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-31168581

RESUMO

AIMS: Advances in surgical techniques allow an increasing number of children with congenital heart disease (CHD) to reach adulthood. As patients grow older, atrial fibrillation (AF) is evolving into a major clinical concern and can be difficult to manage medically. Primary AF catheter ablation may, therefore, have a role in this setting but few reports have evaluated its efficacy in CHD patients. METHODS AND RESULTS: We retrospectively reviewed 58 consecutive patients [median age 51, interquartile range (IQR) 44-63 years, 57% male] with AF (45% paroxysmal) who underwent 122 ablation procedures in our tertiary centre in the last decade. The majority had CHD of moderate or severe complexity (57%, Bethesda Class 2 or 3) with a dilated left atrium (LA) (81%) and/or right atrium (86%). At 1-year from the first ablation, 32.8% of patients remained in sinus rhythm. Multiple procedures were required in 35 (60%) patients. Freedom from AF at 1-year after the 2nd and 3rd ablation was 40.9% and 36.5%, respectively. Multivariable predictors of AF recurrence were underlying anatomic complexity [hazard ratio (HR) in Bethesda 3 1.98, P = 0.006], type of AF (HR for persistent 1.87, P = 0.004), and indexed LA dimensions (HR for cm2/m2 1.06, P = 0.03). CONCLUSION: While ablation may be a valid option for the treatment of AF in CHD patients, multiple procedures are likely to be required. Early referral and careful patient selection are essential to optimize the results of AF ablation, achieving a low rate of recurrence. Further studies are needed to validate our prognostic model and guide clinical practice.

14.
Pulm Circ ; 9(2): 2045894019845615, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30945593

RESUMO

Early diagnosis of pulmonary artery hypertension (PAH) is diagnostically challenging given the extent of pulmonary vascular remodeling required to bring about clinical signs and symptoms. Exercise testing can be invaluable in this setting, as stressing the cardiopulmonary system may unmask early disease. This report describes a young patient with a positive family history of PAH in whom contemporaneous invasive cardiopulmonary exercise testing and surgical lung biopsy reveal the novel association between exercise pulmonary hypertension (ePH) and early histological changes of PAH. Exercise PH currently carries no pathological correlates which means the hemodynamic effects of early pulmonary vascular remodeling remain unknown. Following the recent proceedings from the World Symposium in Pulmonary Hypertension 2018, which broaden the hemodynamic definition of PAH, this report suggests an important association between ePH and early pulmonary vascular remodeling supporting a role for exercise hemodynamic evaluation in patients at increased familial risk of PAH.

15.
Heart ; 105(15): 1190-1196, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30923175

RESUMO

OBJECTIVE: To examine the contemporary long-term outcome after coarctation repair. METHODS: This is a retrospective cohort study of 834 patients aged ≥16 years who underwent coarctation repair under single-centre follow-up. Repair was performed at a median age of 3 years (lower-upper quartile: 1 month to 15 years) by surgery in 83% (690/834) and angioplasty/stenting in 17% (144/834). Survival was compared with an age- and gender matched normal population. Other outcomes included arch reintervention, aortic valve intervention, ascending aortic intervention, and residual/re-coarctation and resting hypertension at latest follow-up. RESULTS: After a median follow-up of 27 years (lower-upper quartile: 18-36), there were 38 late deaths (5%, 38/834). Overall survival was 99%, 88% and 65% at 30, 50 and 70 years of age, respectively, significantly reduced compared with a matched normal population (standardised mortality ratio: 3.20, log-rank: p<0.001). Thirty per cent (246/834) required ≥1 arch reintervention, 13% (111/834) an aortic valve intervention and 5% (43/834) an ascending aortic intervention. Freedom from aortic valve and ascending aortic intervention was 83% and 92% at 50 years and 53% and 81% at 70 years of age, respectively. Residual/re-coarctation (gradient ≥25 mm Hg or repair site/diaphragm ratio ≤70%) at latest follow-up was present in 60% (282/474) and resting hypertension in 57% (379/661). CONCLUSIONS: Long-term survival in contemporary adult survivors of coarctation repair is significantly lower than a matched normal population with accelerated decline after the third decade. Nearly 60% of patients eventually develop hypertension, whereas approximately 50% require further invasive cardiovascular treatment by 50 years of age. Our risk-stratifying data may enable personalised follow-up strategies for this common congenital heart condition.

17.
Heart ; 105(12): 932-937, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30700516

RESUMO

OBJECTIVE: Pulmonary valve replacement (PVR) is often required in patients with congenital heart disease. We aimed to describe temporal trends in PVR in a nationwide English cohort between 1997 and 2014, survival and the need for re-PVR. METHODS: Patients were identified in the Hospital Episode Statistics Database. Survival data were retrieved from the UK Office for National Statistics. RESULTS: A total of 2733 patients underwent PVR (2845 procedures) over the study period. Median age at first procedure increased from 20.1 years in 1997-2005 to 24.7 years in 2006-2014. The annual number of PVRs increased from 23 in 1997 to 251 in 2014. Homografts were the most common choice in the early years, but the use of xenografts increased after 2005. During a median follow-up of 5.8 years, 176 patients died and 108 required redo PVR. Early (30 day) survival was 98% for all PVRs and was similar for all types of prostheses but longer-term mortality dropped to 92% at 10 years and 90% at 15 years. Age >16 years and percutaneous PVR were risk factors for death. The cumulative incidence for re-PVR at 10 years was 8% for all PVRs and 11% at 15 years. Risk factors for re-PVR were complex diagnosis, male gender and black ethnicity. CONCLUSION: There was a significant increase in the number of PVRs performed in England over the last two decades and a significant change in the type of prosthesis employed. While early mortality was low across the board, longer-term mortality was not negligible in this young population.

18.
Heart ; 105(13): 1014-1019, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30705053

RESUMO

OBJECTIVE: The prognostic benefit of atrial septal defect (ASD) closure in adulthood, particularly in advanced age, remains uncertain. The aim of our study was to examine the impact of ASD closure in a contemporary adult cohort on mid to longer term survival as compared with expected survival in the general population. METHODS: We study herewith all consecutive patients (≥16 years of age) who underwent ASD closure, catheter or surgical, at our tertiary centre between 2001 and 2012. Furthermore, we compare survival of our ASD closure cohort with expected survival in age and gender-matched general population and standardised mortality ratios (SMR) were calculated. RESULTS: A total of 608 patients (mean age 45.4±16.7 years) underwent ASD closure (catheter 433(71.2%), surgical 175(28.8%)). There was no 30-day mortality and periprocedural complications were low (n=40, 6.6%). During a median follow-up of 6.7 (IQR 4.2-9.3) years 16 (2.6%) patients died; survival was similar to the general population (p=0.80) including patients >40 or >60 years of age at ASD closure (p=0.58 and p=0.64, respectively). There was no survival difference between gender (male: SMR 0.93; 95% CI 0.52 to 1.64, p=0.76; female: SMR 0.99; 95% CI 0.58 to 1.66, p=0.95) or mode of closure compared with general population (catheter: SMR 1.03; 95% CI 0.68 to 1.55, p=0.89; surgical: SMR 0.65; 95% CI 0.22 to 1.88, p=0.38). CONCLUSION: Perioperative mortality and morbidity in a large contemporary adult cohort undergoing ASD closure, catheter or surgical, is extremely low. Mid to longer term survival is excellent irrespective of age, gender and mode of closure, and similar to matched general population.

19.
Heart ; 105(8): 596-602, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30636220

RESUMO

BACKGROUND: Increased longevity in patients with congenital heart disease (CHD) is associated with late complications, mainly heart failure, which may not be amenable to redo surgery and become refractory to medical therapy and so, trigger referral for transplantation. We assessed the current role and future prospects of heart and heart-lung transplantation for patients with CHD in England. METHODS: We performed a retrospective analysis of hospital episodes for England for 1997-2015, identifying patients with a CHD code (ICD-10 'Q2xx.x'), who underwent heart or heart-lung transplantation. RESULTS: In total, 469 transplants (82.2% heart and 17.8% heart-lung) were performed in 444 patients. Half of patients transplanted had mild or moderate CHD complexity, this percentage increased with time (p=0.001). While overall, more transplantations were performed over the years, the proportion of heart-lung transplants declined (p<0.0001), whereas the proportion of transplants performed in adults remained static. Mortality was high during the first year, especially after heart-lung transplantation, but remained relatively low thereafter. Older age and heart-lung transplantation were strong predictors of death. While an increase in CHD transplants is anticipated, actual numbers in England seem to lag behind the increase in CHD patients with advanced heart failure. CONCLUSIONS: The current and future predicted increase in the numbers of CHD transplants does not appear to parallel the expansion of the CHD population, especially in adults. Further investment and changes in policy should be made to enhance the number of donors and increase CHD transplant capacity to address the increasing numbers of potential CHD recipients and optimise transplantation outcomes in this growing population.


Assuntos
Cardiopatias Congênitas , Insuficiência Cardíaca , Transplante de Coração , Transplante de Coração-Pulmão , Complicações Pós-Operatórias , Reoperação , Adulto , Criança , Bases de Dados Factuais/estatística & dados numéricos , Progressão da Doença , Inglaterra/epidemiologia , Feminino , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/terapia , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/cirurgia , Transplante de Coração/efeitos adversos , Transplante de Coração/métodos , Transplante de Coração/estatística & dados numéricos , Transplante de Coração-Pulmão/efeitos adversos , Transplante de Coração-Pulmão/métodos , Transplante de Coração-Pulmão/estatística & dados numéricos , Humanos , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Mortalidade , Determinação de Necessidades de Cuidados de Saúde , Complicações Pós-Operatórias/mortalidade , Complicações Pós-Operatórias/cirurgia , Reoperação/métodos , Reoperação/estatística & dados numéricos , Estudos Retrospectivos , Fatores de Risco
20.
Eur Heart J Cardiovasc Imaging ; 20(8): 925-931, 2019 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-30629127

RESUMO

AIMS: To investigate the utility of novel deep learning (DL) algorithms in recognizing transposition of the great arteries (TGA) after atrial switch procedure or congenitally corrected TGA (ccTGA) based on routine transthoracic echocardiograms. In addition, the ability of DL algorithms for delineation and segmentation of the systemic ventricle was evaluated. METHODS AND RESULTS: In total, 132 patients (92 TGA and atrial switch and 40 with ccTGA; 60% male, age 38.3 ± 12.1 years) and 67 normal controls (57% male, age 48.5 ± 17.9 years) with routine transthoracic examinations were included. Convolutional neural networks were trained to classify patients by underlying diagnosis and a U-Net design was used to automatically segment the systemic ventricle. Convolutional networks were build based on over 100 000 frames of an apical four-chamber or parasternal short-axis view to detect underlying diagnoses. The DL algorithm had an overall accuracy of 98.0% in detecting the correct diagnosis. The U-Net architecture model correctly identified the systemic ventricle in all individuals and achieved a high performance in segmenting the systemic right or left ventricle (Dice metric between 0.79 and 0.88 depending on diagnosis) when compared with human experts. CONCLUSION: Our study demonstrates the potential of machine learning algorithms, trained on routine echocardiographic datasets to detect underlying diagnosis in complex congenital heart disease. Automated delineation of the ventricular area was also feasible. These methods may in future allow for the longitudinal, objective, and automated assessment of ventricular function.

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