Marcotte, François

Coronary anomalies, although rare, should be considered when young patients present with angina. Clinical suspicion and multi-modality imaging including coronary angiography and tomographic imaging should be pursued for symptomatic patients such as the one we are presenting with anomalous right coronary artery from the pulmonary artery. She was promptly referred for surgical intervention with re-implantation of the right coronary artery onto the aorta.

Coarctation of the aorta (CoA) comprises 5–7% of congenital heart disease and can present as an isolated narrowing in the aortic arch just distal to the left subclavian artery or can be associated with cardiac abnormalities such as a bicuspid aortic valve, aortopathy, or ventricular septal defects. With the advances in the medical field, intervention on CoA can either be via surgical repair or endovascular stenting. Echocardiography is the mainstay in diagnosing CoA, with tomographic imaging such as magnetic resonance imaging (MRI) or computed tomography providing supplementary assessment of the aorta, valves, and collateral vessels. We present a case of a young hypertensive male who was noted to have a continuous cardiac murmur with diagnostic Doppler pattern of CoA on echocardiography that normalized soon after percutaneous stenting.

Current management of patients with congenital heart disease has increased their survival into adulthood. This is accompanied by potential cardiac complications, including pulmonary hypertension associated with congenital heart disease (PAH-CHD). PAH-CHD constitutes a challenging subgroup of pulmonary hypertension and requires expert management to improve quality of life and prognosis. Novel agents have shown a significant improvement in morbidity and mortality in patients with pulmonary arterial hypertension. However, the long-term effects of these medications on PAH-CHD patients remain somewhat uncertain, necessitating treatment plans largely founded on the clinical experience of the healthcare providers. The aim of this review is to summarize the current evidence and future perspectives regarding treatment strategies for PAH-CHD to help better guide management of this complex disease.

Objectives Lipoprotein(a) (Lp(a)) is associated with an increased incidence of native aortic stenosis, which shares similar pathological mechanisms with bioprosthetic aortic valve (bAV) degeneration. However, evidence regarding the role of Lp(a) concentrations in bAV degeneration is lacking. This study aims to evaluate the association between Lp(a) concentrations and bAV degeneration. Methods In this retrospective multicentre study, patients who underwent a bAV replacement between 1 January 2010 and 31 December 2020 and had a Lp(a) measurement were included. Echocardiography follow-up was performed to determine the presence of bioprosthetic valve degeneration, which was defined as an increase >10 mm Hg in mean gradient from baseline with concomitant decrease in effective orifice area and Doppler Velocity Index, or new moderate/severe prosthetic regurgitation. Levels of Lp(a) were compared between patients with and without degeneration and Cox regression analysis was performed to investigate the association between Lp(a) levels and bioprosthetic valve degeneration. Results In total, 210 cases were included (mean age 74.1±9.4 years, 72.4% males). Median time between baseline and follow-up echocardiography was 4.4 (IQR 3.7) years. Bioprostheses degeneration was observed in 33 (15.7%) patients at follow-up. Median serum levels of Lp(a) were significantly higher in patients affected by degeneration versus non-affected cases: 50.0 (IQR 72.0) vs 15.6 (IQR 48.6) mg/dL, p=0.002. In the regression analysis, high Lp(a) levels (≥30 mg/dL) were associated with degeneration both in a univariable analysis (HR 3.6, 95% CI 1.7 to 7.6, p=0.001) and multivariable analysis adjusted by other risk factors for bioprostheses degeneration (HR 4.4, 95% CI 1.9 to 10.4, p=0.001). Conclusions High serum Lp(a) is associated with bAV degeneration. Prospective studies are needed to confirm these findings and to investigate whether lowering Lp(a) levels could slow bioprostheses degradation.

Congenital heart disease (CHD), which affects 1% to 2% of all births, is the most common abnormality in women contemplating pregnancy in western countries. With diagnostic and interventional advances, most patients with CHD survive into adulthood and require lifelong cardiac follow-up with cardiac imaging, particularly echocardiography and cardiac computed tomography. Multiple hemodynamic and physiologic changes of pregnancy may predispose patients with CHD to clinical decompensation and an inability to tolerate pregnancy. This manuscript reviews common CHD lesions, their repair or palliative interventions, long-term sequelae, important features to assess on cardiac imaging, and the impact of pregnancy on these types of lesions. Moreover, the review bridges the fields of CHD, cardiac imaging, and maternal cardiology, which will aid clinicians in counseling patients and managing pregnancies.

AbstractIntroductionThe right ventricle (RV) strain measured by speckle tracking (RVS) is an echocardiographic parameter used to assess RV function. We compared RVS to RV fractional area change (FAC%), tricuspid annular plane systolic excursion (TAPSE) and Doppler tissue imaging‐derived peak systolic velocity (Sʹ) in the assessment of right ventricular (RV) systolic function measured using cardiac magnetic resonance imaging (MRI).MethodsWe enrolled consecutive patients who underwent cardiac MRI between Jan 2012 and Dec 2017 and a transthoracic echocardiogram (TTE) within 1 month of the MRI with no interval event. Baseline clinical characteristics and MRI parameters were extracted from chart review. Echocardiographic parameters were measured prospectively. TTE parameters including RVS, TAPSE, Sʹ, and FAC% were tested for accuracy to identify impaired RV EF (EF < 45% & <30%) using receiver operator curves.ResultsThe study cohort included 500 patients with mean age 55 years ± 18 and peak tricuspid regurgitation velocity 2.7 ± 1.4 m/s. The area under ROC for RVS was 0.69 (95% CI 0.63–0.75) and 0.78 (95% CI 0.70–0.88) to predict RVEF < 45% & RVEF < 30%, respectively. The RV FAC% had second highest accuracy of predicting RVEF among all the TTE parameters tested in study.ConclusionRight ventricular strain is the most accurate echocardiographic method to detect impaired right ventricular systolic function when using MRI as the gold standard.

Introduction: The right ventricle (RV) strain measured by speckle tracking (RVS) is a novel method of assessing RV function. We compared RVS to RV fractional area change (FAC%), tricuspid annular peak systolic excursion (TAPSE) and Doppler tissue imaging-derived peak systolic velocity (S’) in the assessment of right ventricular (RV) systolic function measured using cardiac magnetic resonance imaging (MRI). Methods: We enrolled consecutive patients who underwent cardiac MRI between Jan 2012- Dec 2017 and a transthoracic echocardiogram (TTE) within 1 month of the MRI with no interval event. Baseline clinical characteristics and MRI parameters were extracted from chart review. Echocardiographic parameters were measured prospectively. TTE parameters including RVS, TAPSE, S’ and FAC% were tested for accuracy to identify impaired RV EF (EF <45% & <30%) using receiver operator curves. Results: The study cohort included 500 patients with mean age 55 yr ± 18 and right ventricular systolic pressure 33.7 ± 13.6 mmHg. The area under ROC for RVS was 0.69 (95% CI 0.63 – 0.75) and 0.78 (95% CI 0.70 – 0.88) to predict RVEF <45% & RVEF <30% respectively. The RV FAC % had second highest accuracy of predicting RVEF among all the TTE parameters tested in study. Conclusion: Right ventricular strain is the most accurate echocardiographic method to detect impaired right ventricular systolic function when using MRI as the gold standard.