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An interactive tool for rapid biventricular analysis of congenital heart disease.

Clin Physiol Funct Imaging. 2017 Jul;37(4):413-420

Authors: Gilbert K, Lam HI, Pontré B, Cowan BR, Occleshaw CJ, Liu JY, Young AA

Cardiac malformations are the most common birth defect. Better interventions in early life have improved mortality for children with congenital heart disease, but heart failure is a significant problem in adulthood. These patients require regular imaging and analysis of biventricular (left and right ventricular) function. In this study, we describe a rapid method to analyse left and right ventricular shape and function from cardiac MRI examinations. A 4D (3D+time) finite element model template is interactively customized to the anatomy and motion of the biventricular unit. The method was validated in 17 patients and 10 ex-vivo hearts. Interactive model updates were achieved through preconditioned conjugate gradient optimization on a multithread system, and by precomputing points predicted from a coarse mesh optimization.

PMID: 26577068 [PubMed - indexed for MEDLINE]

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Congenital left ventricular apical aneurysm presenting as ventricular tachycardia.

Rev Port Cardiol. 2016 Oct;35(10):545.e1-4

Authors: Amado J, Marques N, Candeias R, Gago P, de Jesus I

The authors present the case of a 34-year-old male patient seen in our department due to palpitations. On the electrocardiogram monomorphic ventricular tachycardia (VT) was documented, treated successfully with amiodarone. The subsequent study revealed a normal echocardiogram and an apical aneurysm of the left ventricle on magnetic resonance imaging, confirmed by computed tomography coronary angiography that also excluded coronary disease. He underwent an electrophysiological study to determine the origin of the VT and to perform catheter ablation using electroanatomical mapping. VT was induced and radiofrequency applications were performed in the left ventricular aneurysm area. VT was no longer inducible, with acute success. Despite this it was decided to implant a subcutaneous implantable cardioverter-defibrillator (ICD). Eight months after the ablation the patient was admitted again due to VT, treated by the ICD.

PMID: 27612529 [PubMed - indexed for MEDLINE]

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Improved long-term outcomes in double-inlet left ventricle and tricuspid atresia with transposed great arteries: systemic outflow tract obstruction present at birth defines long-term outcome.

Eur J Cardiothorac Surg. 2017 Jun 01;51(6):1051-1057

Authors: Franken LC, Admiraal M, Verrall CE, Zannino D, Ayer JG, Iyengar AJ, Cole AD, Sholler GF, D'Udekem Y, Winlaw DS

OBJECTIVES: In 2 subtypes of functional single ventricle, double inlet left ventricle (DILV) and tricuspid atresia with transposed great arteries (TA-TGA), systemic output passes through an outflow chamber before entering the aorta. Intracardiac obstruction to this pathway causing systemic outflow tract obstruction (SOTO) may be present at birth or develop over time. Long-term survival after Fontan has not been defined. We defined outcomes utilizing records from 2 centres that were cross-checked with data from a bi-national Fontan Registry for completeness and accuracy.
METHODS: Two hundred and eleven patients were identified, 59 TA-TGA,152 DILV. Median follow-up was 17 years (range 4 days to 49.8 years). The Kaplan-Meier method was used for all of the time to event analyses and the log-rank test was used to compare the time-to-events. Cox proportional hazard models were used to test the association between potential predictors and time-to-event end-points.
RESULTS: TA-TGA had reduced survival compared to DILV (cumulative risk of death 28.8% vs 11%, hazard ratio (HR) 3.1 (95% confidence interval (CI) 1.6-6.1), P  = 0.001). In both groups, SOTO at birth carried a worse prognosis HR 3.54 (1.36-9.2, P  = 0.01). SOTO was not more common in either morphology at birth ( P  = 0.20). Periprocedural mortality accounted for 40% of deaths. Fontan was achieved in 82%, DILV were more likely to achieve Fontan than TA-TGA (91% vs 60%, P <0.001). After Fontan there were 9 deaths (4%) with no difference according to morphology.
CONCLUSIONS: Patients with TA-TGA have poorer outcomes than those with DILV, affecting survival and likelihood of achieving Fontan. SOTO at birth carries a high risk of mortality suggesting that, when present, initial surgical management should address this.

PMID: 28329058 [PubMed - indexed for MEDLINE]

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Outcomes from anomalous origin of the left coronary artery from the pulmonary artery repair: Long-term complications in relation to residual myocardial abnormalities.

J Cardiol. 2017 Nov;70(5):498-503

Authors: Kanoh M, Inai K, Shinohara T, Tomimatsu H, Nakanishi T

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary artery irregularity. This study aimed to clarify the long-term postoperative outcomes in ALCAPA patients, and to compare the infantile and adult types.
METHODS: We retrospectively analyzed the clinical data from 33 patients with ALCAPA who underwent surgical repairs after 1980. The patients were grouped based on whether presentation occurred before (infantile type: n=14) or after (adult type: n=19) 1 year of age.
RESULTS: The mean follow-up duration was 16 years. Preoperatively, the infantile type had greater impairment of the left ventricle ejection fraction (LVEF) (45±15%) compared with the adult type (59±10%) (p<0.01). Coronary revascularization significantly improved the postoperative LVEF (67±5%) (p<0.01) in the patients with the infantile type. The postoperative LVEF did not change in the adult type. The mitral regurgitation (MR) severity improved postoperatively, but the between-group difference was not significant. Postoperatively, none of the patients with the infantile type and 37% of the patients with the adult type had left ventricular asynergy (p=0.01), and both groups showed postoperative perfusion defects (79% vs 95%, p=0.29). Compared with the infantile type, the adult type had a significant prognostic value for composite cardiovascular events that comprised cardiac death, arrhythmias, MR deterioration, and hospitalization as a consequence of heart failure (p=0.04).
CONCLUSIONS: Most patients showed favorable clinical outcomes postoperatively, but myocardial damage remained long after surgery and cardiovascular events occurred postoperatively. Hence, meticulous long-term follow-up is warranted.

PMID: 28427867 [PubMed - indexed for MEDLINE]

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Cardiorespiratory fitness and cancer incidence in men.

Ann Epidemiol. 2017 Jul;27(7):442-447

Authors: Vainshelboim B, Müller J, Lima RM, Nead KT, Chester C, Chan K, Kokkinos P, Myers J

PURPOSE: The preventive role of cardiorespiratory fitness (CRF) in cancer is not well established. The present study sought to evaluate the association between CRF and cancer incidence in men.
METHODS: Maximal exercise testing was performed in 4920 men (59.2 ± 11.4 years) free from malignancy at baseline who were followed for 12.7 ± 7.5 years. Relative risks and population attributable risks were determined.
RESULTS: During the follow-up, 25.8% were diagnosed with any type of cancer. CRF was inversely associated with total cancer incidence; for each one metabolic equivalent increase in CRF, there was a 4% reduction in cancer incidence (P < .001). Compared with low CRF, moderate and high CRF levels were associated with 14% (95% CI [0.74-0.99]) and 26% (95% CI [0.62-0.89]) reduced risks for all cancers, respectively (P for trend = .004). Low CRF had a population attributable risk of 3.0% for cancer incidence. The associations between CRF, prostate, skin and colorectal cancers were not significant.
CONCLUSIONS: Higher CRF is associated with lower total cancer incidence in men. A novel finding suggests that eliminating low CRF as a risk factor would potentially prevent considerable cancer morbidity and reduce the societal and economic burden associated with cancer. These findings underscore the importance of CRF for primary cancer prevention.

PMID: 28789775 [PubMed - indexed for MEDLINE]

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Role of Coronary Angiography in Pre-Liver Transplantation Cardiac Evaluation: Experience From an Asian Transplant Institution.

Transplant Proc. 2017 Oct;49(8):1797-1805

Authors: Pang NQ, Kow WCA, Law JH, Pan LTT, Lim BLK, Wong CCR, Chang KYS, Ganpathi IS, Madhavan K

BACKGROUND: Liver transplant (LT) patients with significant coronary artery disease (CAD) have poorer outcomes. Pre-LT coronary angiography (CA) is associated with significant complications in cirrhotic patients.
METHODS: This study aimed to identify predictors of abnormal CA in pre-LT cardiac assessment and to develop a predictive model to reduce unnecessary CA. From January 2006 to June 2013, 122 patients underwent CA based on the current institutional protocol.
RESULTS: Forty-one (33.6%) patients had abnormal CA. Univariate analysis showed age ≥65 years (P = .001), cryptogenic cirrhosis (P = .046), cardiac comorbidities (P = .027), ischemic heart disease (IHD; P = .002), left ventricular hypertrophy (LVH; P = .004), hypertension (P = .002), diabetes mellitus (P = .017), dyslipidemia (P < .001), metabolic syndrome (P = .003), ≥2 CAD risk factors (P = .001), and high Framingham risk score (hard CAD risk, P = .018; cardiovascular disease: lipids, P = .002; body mass index, P < .001) to be significant predictors of abnormal CA. A predictive model was developed with the use of multivariable logistic regression and included diabetes, dyslipidemia, IHD, age ≥65 years, and LVH, achieving a specificity of 55.1% and sensitivity of 90.0%. This would reduce unnecessary CA by up to one-half in our study population (from 81 to 35) while maintaining a false negative rate of only 8.5%.
CONCLUSIONS: Diabetes, dyslipidemia, IHD, age ≥65 years, and LVH appear to be predictors of abnormal CA in pre-LT patients. Our predictive model may help to better select patients for CA, although further validation is required.

PMID: 28923628 [PubMed - indexed for MEDLINE]

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Three-dimensional printing enhances preparation for repair of double outlet right ventricular surgery.

J Card Surg. 2018 Jan;33(1):24-27

Authors: Zhao L, Zhou S, Fan T, Li B, Liang W, Dong H

OBJECTIVE: To assess the clinical value of three-dimensional (3D) printing technology for treatment strategies for complex double outlet right ventricle (DORV).
METHODS: Twenty-five patients with complex double outlet right ventricle were enrolled in this study. The patients were divided into two groups: 3D printing group (eight patients) and a non-3-D printing control group (17 patients). The cardiac images of patients in the 3D printing group were transformed to Digital Imaging and Communications and were segmented and reconstructed to create a heart model. No cardiac models were created in the control group. A Pearson coefficient analysis was used to assess the correlation between measurements of 3D printed models and computed tomography angiography (CTA) data. Pre-operative assessment and planning were performed with 3D printed models, and then operative time and recovery time were compared between the two groups.
RESULTS: There was good correlation (r = 0.977) between 3D printed models and CTA data. Patients in the 3D printing group had shorter aortic cross-clamp time (102.88 vs 127.76 min, P = 0.094) and cardiopulmonary bypass time (151.63 vs 184.24 min; P = 0.152) than patients in the control group. Patients with 3D printed models had significantly lower mechanical ventilation time (56.43 vs 96.76 h, P = 0.040) and significantly shorter intensive care unit time (99.04 vs 166.94 h, P = 0.008) than patients in the control group.
CONCLUSIONS: 3D printed models can accurately demonstrate anatomic structures and are useful for pre-operative treatment strategies in DORV.

PMID: 29409167 [PubMed - indexed for MEDLINE]

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Anomalous left coronary artery from the pulmonary artery (ALCAPA) diagnosed in adulthood: Varied clinical presentation, therapeutic approach and outcome.

Int J Cardiol. 2018 Feb 24;:

Authors: Boutsikou M, Shore D, Li W, Rubens M, Pijuan A, Gatzoulis MA, Babu-Narayan S

INTRODUCTION: The diagnosis of ALCAPA syndrome is sporadic in adulthood, of the limited cases in the literature most are incidental or without symptoms. There is a broad spectrum of clinical manifestations of ALCAPA syndrome however, including sudden cardiac death.
CASES: We present herewith a series of 12 consecutive patients with ALCAPA, all diagnosed in adulthood (between 18 and 73 years of age). Five patients developed symptoms (breathlessness) after the fourth decade of life, 3 were undiagnosed despite a history of previous mitral valve repair, one presented with heart failure, one with resuscitated cardiac arrest, whereas two patients were asymptomatic. We review in this paper, the clinical history, diagnostic approach and therapeutic choices of ALCAPA syndrome.
CONCLUSION: ALCAPA syndrome is not confined to childhood, late diagnosis in adulthood has a varied clinical presentation. ALCAPA syndrome should be particularly considered as a potential, albeit uncommon cause of mitral regurgitation and/or dilated cardiomyopathy.

PMID: 29548537 [PubMed - as supplied by publisher]

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Interrupted aortic arch diagnosis by computed tomography angiography and 3-D reconstruction: A case report.

Radiol Case Rep. 2018 Feb;13(1):35-38

Authors: Pérez TM, García SM, Velasco ML, Sánchez AP

Interrupted aortic arch is an extremely rare congenital malformation representing about 1% of congenital heart disease. Early symptoms usually occur early in the neonatal period and clinical deterioration is often rapid and long-term prognosis is limited. Nonetheless, this condition has been identified later in adult life in rare cases. We report a case in an adult male with absence of hypertension history and no further cardiac compromise, with a severe posterior chest pain alongside dyspnea and sweating. Computed tomography angiography revealed interrupted aortic arch type A, bivalve aorta, hemopericardium, aortic dissection Stanford A, and important collateral circulation.

PMID: 29552241 [PubMed]

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Acute rheumatic fever and rheumatic heart disease.

Nat Rev Dis Primers. 2016 01 14;2:15084

Authors: Carapetis JR, Beaton A, Cunningham MW, Guilherme L, Karthikeyan G, Mayosi BM, Sable C, Steer A, Wilson N, Wyber R, Zühlke L

Acute rheumatic fever (ARF) is the result of an autoimmune response to pharyngitis caused by infection with group A Streptococcus. The long-term damage to cardiac valves caused by ARF, which can result from a single severe episode or from multiple recurrent episodes of the illness, is known as rheumatic heart disease (RHD) and is a notable cause of morbidity and mortality in resource-poor settings around the world. Although our understanding of disease pathogenesis has advanced in recent years, this has not led to dramatic improvements in diagnostic approaches, which are still reliant on clinical features using the Jones Criteria, or treatment practices. Indeed, penicillin has been the mainstay of treatment for decades and there is no other treatment that has been proven to alter the likelihood or the severity of RHD after an episode of ARF. Recent advances - including the use of echocardiographic diagnosis in those with ARF and in screening for early detection of RHD, progress in developing group A streptococcal vaccines and an increased focus on the lived experience of those with RHD and the need to improve quality of life - give cause for optimism that progress will be made in coming years against this neglected disease that affects populations around the world, but is a particular issue for those living in poverty.

PMID: 27188830 [PubMed - indexed for MEDLINE]