Citations 31

International Consensus Statement on Nomenclature and Classification of the Congenital Bicuspid Aortic Valve and Its Aortopathy, for Clinical, Surgical, Interventional and Research Purposes

Radiology: Cardiothoracic Imaging | July 22, 2021 | Volume: 3 | Issue: 4 | Pages: e200496

10.1148/ryct.2021200496 | PMID 34505060

Abstract 1589: Pulmonary Hypertension Is Associated With Regional Right Ventricular Systolic Dysfunction And Dyssynchrony: Strain and Strain Rate Assessment Using Velocity Vector Imaging Technique.

Circulation | July 3, 2021 | Volume: 116 | Issue: suppl_16 | Pages: No page available

10.1161/circ.116.suppl_16.ii_331-c

Background: Strain and strain rate has been shown to identify subtle contraction abnormalities of left ventricle. Objectives: We hypothesized that S and SR derived from velocity vector imaging (VVI) will allow early identification of global and regional RV systolic function in patients with pulmonary hypertension (PHT) compared to conventional echo parameters. Methods: 96 patients, 35 normal RV Systolic Pressure (RVSP < 30 mmHg) and 61 with PHT, {24 mild-moderate (RVSP > 60 mmHg) and 37 severe (RVSP < 60 mmHg)} underwent echo-Doppler assessment of RV. Apical 4 chamber images at end expiration were used for RV lateral wall (LW) and ventricular septal (VS) segment analysis for systolic tangential velocity (Ta V), S and SR. In addition RV end systolic and diastolic area, RV fractional area change (RV FAC), RV performance index (RIMP) and tricuspid annulus systolic excursion were measured. Results: Peak S, systolic SR and Ta V at the basal, mid and apical segments of the RV LW and VS were depressed in patients with PHT compared with controls, Table 1 , P 0.001. Similarly, significant decrease in RIMP was seen in patients with PHT compared to controls, p <0.001. Significant correlation (p<0.001, r >0.6) was noted between the 2D FAC and S, SR. The time to peak systolic S indexed to end diastolic area (EDA) is significantly prolonged, p<0.01 in all segments in patients with PHT indicating dyssynchrony. Conclusion: S and SR allows assesment of Regional RV systolic function otherwise difficult to assess from conventional echo-Doppler technique. PHTN is associated with not only regional RV systolic dysfunction but also with dyssynchrony. Thus, this new tool can offer new insights in the functioning of right ventricular global and regional function. mean±SD of strain
An under‐recognized phenomenon: Myocardial volume change during the cardiac cycle

Echocardiography | June 4, 2021 | Volume: No volume available | Issue: No issue available | Pages: No page available

10.1111/echo.15093 | PMID 34085722

Abstract 15635: Myocardial Function Index Predicts Mortality in Amyloid Light Chain Cardiomyopathy

Circulation | May 10, 2021 | Volume: 142 | Issue: Suppl_3 | Pages: No page available

10.1161/circ.142.suppl_3.15635

Introduction: Cardiovascular disease remains the leading cause of morbidity and mortality in patients with amyloid light chain (AL) amyloidosis. Current prognostic evaluation in AL cardiomyopathy (AL-CM) largely relies upon disparate proxy measurements to assess mortality. With respect to observed myocardial volume changes between systole and diastole, we describe a single measurement derived from cardiac MRI (CMR) to assess mortality of AL-CM. Methods: A cohort of 129 consecutive patients with AL-CM undergoing CMR with amyloid protocol from the years 2001 - 1997 was reviewed. Myocardial volume change at end systole and end diastole was assessed by disc summation with CMR. The myocardial function index (MFI) was calculated by consolidating blood pool and myocardial volume measures as confluent components of myocardial function utilizing operator tracings. The association of MFI and death was assessed with adjusted hazard ratios (HR) derived by a Cox model. Results: Data at time of CMR was reviewed. The mean age was 60 ±10; 68% subjects were female. Median survival from CMR was 47 months. Mean LVEF was assessed by echocardiography to be 55% ± 12. Mean calculated MFI was 19.8% ±7. Univariate analysis suggested MFI is strongly associated with death (HR 0.004; 95%CI 0.00002-0.0838; p = 0.0003). A multivariate analysis re-demonstrated the strong predictive value of MFI when controlled for age (HR 0.006; 95%CI 0.00003 - 0.1156; p = 0.0006). Conclusion: MFI strongly correlates with mortality in AL-CM. In contrast to previously described assessments of ventricular function (extracellular volume imaging, myocardial perfusion imaging, and myocardial contraction fraction), MFI is easily derived from CMR and considers blood pool and myocardial volumes as confluent components of myocardial function. Further studies are needed to assess MFI amongst patients with varying etiologies of heart failure.
International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes

European Journal of Cardio-Thoracic Surgery | January 25, 2021 | Volume: 60 | Issue: 3 | Pages: 448-476

10.1093/ejcts/ezab038 | PMID 34293102

Abstract This International Consensus Classification and Nomenclature for the congenital bicuspid aortic valve condition recognizes 3 types of bicuspid valves: 1. The fused type (right-left cusp fusion, right-non-coronary cusp fusion and left-non-coronary cusp fusion phenotypes); 2. The 2-sinus type (latero-lateral and antero-posterior phenotypes); and 3. The partial-fusion (forme fruste) type. The presence of raphe and the symmetry of the fused type phenotypes are critical aspects to describe. The International Consensus also recognizes 3 types of bicuspid valve-associated aortopathy: 1. The ascending phenotype; 2. The root phenotype; and 3. Extended phenotypes.
Summary: international consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes

European Journal of Cardio-Thoracic Surgery | January 23, 2021 | Volume: 60 | Issue: 3 | Pages: 481-496

10.1093/ejcts/ezab039 | PMID 34292332

Abstract This International evidence-based nomenclature and classification consensus on the congenital bicuspid aortic valve and its aortopathy recognizes 3 types of bicuspid aortic valve: 1. Fused type, with 3 phenotypes: right-left cusp fusion, right-non cusp fusion and left-non cusp fusion; 2. 2-sinus type with 2 phenotypes: Latero-lateral and antero-posterior; and 3. Partial-fusion or forme fruste. This consensus recognizes 3 bicuspid-aortopathy types: 1. Ascending phenotype; root phenotype; and 3. extended phenotypes.
Impact of Sirolimus as a Primary Immunosuppressant on Myocardial Fibrosis and Diastolic Function Following Heart Transplantation

Journal of the American Heart Association | December 16, 2020 | Volume: 10 | Issue: 1 | Pages: No page available

10.1161/jaha.120.018186 | PMID 33325244

Background Myocardial fibrosis is an important contributor for development of diastolic dysfunction. We investigated the impact of sirolimus as primary immunosuppression on diastolic dysfunction and fibrosis progression among heart transplantation recipients. Methods and Results In 100 heart transplantation recipients who were either treated with a calcineurin inhibitor (CNI) (n=51) or converted from CNI to sirolimus (n=49), diastolic function parameters were assessed using serial echocardiograms and right heart catheterizations. Myocardial fibrosis was quantified on serial myocardial biopsies. After 3 years, lateral e′ increased within the sirolimus group but decreased in the CNI group (0.02±0.04 versus −0.02±0.04 m/s delta change; P =0.003, respectively). Both pulmonary capillary wedge pressure and diastolic pulmonary artery pressure significantly decreased in the sirolimus group but remained unchanged in the CNI group (−1.50±2.59 versus 0.20±2.20 mm Hg/year; P =0.02; and −1.72±3.39 versus 0.82±2.59 mm Hg/year; P =0.005, respectively). A trend for increased percentage of fibrosis was seen in the sirolimus group (8.48±3.17 to 10.10±3.0%; P =0.07) as compared with marginally significant progression in the CNI group (8.76±3.87 to 10.56±4.34%; P =0.04). The percent change in fibrosis did not differ significantly between the groups (1.62±4.67 versus 1.80±5.31%, respectively; P =0.88). Conclusions Early conversion to sirolimus is associated with improvement in diastolic dysfunction and filling pressures as compared with CNI therapy. Whether this could be attributed to attenuation of myocardial fibrosis progression with sirolimus treatment warrants further investigation.
Devastating presentation of mild COVID-19 infection: Dilated Cardiomyopathy, multiple LV thrombi and embolic stroke in a young male

No container title available | October 12, 2020 | Volume: No volume available | Issue: No issue available | Pages: No page available

10.22541/au.160253866.66313846/v1

Coronavirus disease (COVID-19) is a novel disease with multi-system involvement, but majority of patients have pulmonary and cardiovascular involvement in the acute stages. The cardiovascular impact of acute COVID-19 is well recognized ranging from myocarditis arrhythmias, thrombotic occlusion of coronary arteries, spontaneous coronary artery dissection and micro-thrombi in small coronary vessels on autopsy findings. We report a case of young male who recovered from probable mild covid-19 infection presenting few weeks later with devastating cardiovascular involvement with severe left ventricular (LV) impairment due to non-ischemic cardiomyopathy, multiple LV thrombi and embolic stroke.
Single coronary artery Left (SCA L)‐Right coronary artery arising from mid‐left anterior descending coronary artery: New variant of Lipton classification (SCA L‐II) diagnosed by computed tomographic angiography

Echocardiography | October 1, 2020 | Volume: 37 | Issue: 10 | Pages: 1642-1645

10.1111/echo.14669 | PMID 33000476

AbstractIsolated single coronary artery (SCA) is a rare anomaly. Current classification of left and right is further classified based on the course of the anomalous vessel. We report two SCA L cases where right coronary artery (RCA) arose from mid‐left anterior descending coronary artery (LAD). Our observation is a variation from the current Lipton classification SCA L Type II where RCA arose from left coronary artery before the LAD, in our cases the RCA arose from mid LAD after the first septal perforator. We believe that this variant should be described as SCA L Type II variant 2 (V2) while the original Lipton classification should be described as SCA L Type II variant 1 (V1).
Aetiology and outcomes of severe right ventricular dysfunction

European Heart Journal | January 17, 2020 | Volume: 41 | Issue: 12 | Pages: 1273-1282

10.1093/eurheartj/ehaa037 | PMID 32047900

Abstract Aims Right ventricular dysfunction (RVD) is an important determinant of functional status and survival in various diseases states. Data are sparse on the epidemiology and outcome of patients with severe RVD. This study examined the characteristics, aetiology, and survival of patients with severe RVD. Methods and results Retrospective study of consecutive patients with severe RVD diagnosed by transthoracic echocardiography (TTE) between 2011 and 2015 in a single tertiary referral institution. Patients with prior cardiac surgery, mechanical assist devices, and congenital heart disease were excluded. Primary endpoint was all-cause mortality. In 64 728 patients undergoing TTE, the prevalence of ≥mild RVD was 21%. This study focused on the cohort of 1299 (4%) patients with severe RVD; age 64 ± 16 years; 61% male. The most common causes of severe RVD were left-sided heart diseases (46%), pulmonary thromboembolic disease (18%), chronic lung disease/hypoxia (CLD; 17%), and pulmonary arterial hypertension (PAH; 11%). After 2 ± 2 years of follow-up, 701 deaths occurred, 66% within the first year of diagnosis. The overall probability of survival at 1- and 5 years for the entire cohort were 61% [95% confidence interval (CI) 58–64%] and 35% (95% CI 31–38%), respectively. In left-sided heart diseases, 1- and 5-year survival rates were 61% (95% CI 57–65%) and 33% (95% CI 28–37%), respectively; vs. 76% (95% CI 68–82%) and 50% (95% CI 40–59%) in PAH, vs. 71% (95% CI 64–76%) and 49% (95% CI 41–58%) in thromboembolic diseases, vs. 42% (95% CI 35–49%) and 8% (95% CI 4–15%) in CLD (log-rank P &lt; 0.0001). Presence of ≥moderate tricuspid regurgitation portended worse survival in severe RVD. Conclusion One-year mortality of patients with severe RVD was high (∼40%) and dependent on the aetiology of RVD. Left-sided heart diseases is the most common cause of severe RVD but prognosis was worst in CLD.