Miguel Silva Vieira

Whole‐heart coronary MR angiography using image‐based navigation for the detection of coronary anomalies in adult patients with congenital heart disease

The purpose of this study was to evaluate a recently developed two‐dimensional (2D) image‐based navigation approach (iNAVG+C) combined with respiratory bellows gating for CMRA in patients with congenital heart disease.

Left Atrial Performance in the Course of Hypertrophic Cardiomyopathy: Relation to Left Ventricular Hypertrophy and Fibrosis

Objectives Hypertrophic cardiomyopathy (HCM) is associated with left atrial (LA) functional abnormalities. The determinants and the degree of LA dysfunction in the course of HCM are not fully understood. We aimed to characterize LA mechanics in HCM, according to the extent of left ventricular (LV) hypertrophy and fibrosis. Methods and Results Seventy-three HCM patients and 23 age- and sex-matched controls underwent cardiovascular magnetic resonance imaging including late gadolinium enhancement (LGE). LA reservoir, conduit, and contractile functions were quantified by fractional volume changes and cardiovascular magnetic resonance feature-tracking–derived strain and strain rate. In multivariable regression, LA mechanics were associated with the extent of LV LGE (P = 0.033 to P < 0.001), but not with the LV mass extent or maximum wall thickness (P = 0.108 to P = 0.964). Left atrial function decreased according to the increase in extent of LV fibrosis (non-LGE; mild LGE ⩽ 10%; intermediate LGE 11%–19%; severe LGE ≥ 20%). Compared with healthy controls, LA conduit function was impaired in HCM with no LGE already (LA emptying fraction conduit: 32% ± 7% vs 26 ± 14, P = 0.037). Conversely, LA contractile booster pump function was impaired in HCM with severe LGE only (LA emptying fraction booster: 40% ± 8% vs 20% ± 10%, P < 0.001; for controls vs LGE ≥ 20%, respectively). Conclusions Left atrial functional abnormalities are associated with LV fibrosis, but not with LV hypertrophy. While LA conduit function is impaired in early HCM stages as represented by mild or absent LV fibrosis, LA contractile function is impaired later in the course of disease progression as demonstrated by the presence of severe LV fibrosis only. These novel markers of LA performance may potentially proof useful for disease staging and early detection of cardiac deterioration.

Arterial stiffening is a heritable trait associated with arterial dilation but not wall thickening: a longitudinal study in the twins UK cohort

Aims Vascular ageing is characterized by arterial stiffening, dilation, and arterial wall thickening. We investigated the extent to which these changes are related and their heritability during 5 year follow-up in the Twins UK cohort. Methods and results Carotid-femoral pulse wave velocity (PWVcf), carotid diameter, carotid distensibility, and carotid intima-media thickness (IMT) were measured in 762 female twins (mean age 57.9 ± 8.6 years) at two time-points over an average follow-up of 4.9 ± 1.5 years. Magnetic resonance imaging (MRI) was performed in a sub-sample of 38 women to measure aortic pulse wave velocity (PWVaorta), diameter, and wall thickness. Heritability of changes in arterial wall properties was estimated using structural equation modelling. Annual increases in PWVcf, carotid diameter, distensibility, and IMT were 0.139 m/s, 0.028 mm, -0.4 kPa-1, and 0.011 mm per year, respectively. In regression analysis, predictors of progression in PWVcf included age, mean arterial pressure (MAP), and heart rate (HR) at baseline, and progression in MAP, HR, and body mass index (BMI). Predictors of progression in IMT included progression in MAP, BMI, and triglyceride levels. Progression of PWV and distensibility correlated with progression in carotid diameter but not with IMT. Heritability of progression of PWVcf, diameter, and IMT was 55%, 21%, and 8%, respectively. In a sub-sample of women that underwent MRI, aortic wall thickness increased by 0.19 mm/year, but aortic wall thickening was not correlated with an increase in lumen diameter or PWVaorta. Conclusion Arterial stiffening, as measured by PWVcf, and dilation are heritable but independent of arterial wall thickening. Genetic and cardiovascular risk factors contribute differently to progression of PWV and IMT.

3D Whole Heart Imaging for Congenital Heart Disease

Three-dimensional (3D) whole heart techniques form a cornerstone in cardiovascular magnetic resonance imaging of congenital heart disease (CHD). It offers significant advantages over other CHD imaging modalities and techniques: no ionizing radiation; ability to be run free-breathing; ECG-gated dual-phase imaging for accurate measurements and tissue properties estimation; and higher signal-to-noise ratio and isotropic voxel resolution for multiplanar reformatting assessment. However, there are limitations, such as potentially long acquisition times with image quality degradation. Recent advances in and current applications of 3D whole heart imaging in CHD are detailed, as well as future directions.

Coronary artery fistula presenting as unstable angina

A 74-year-old man, with a history of heavy smoking, presented with unstable angina (Figure 1). Dobutamine stress echocardiography showed ischemia in the right coronary artery territory. Coronary angiography revealed no obstructive epicardial disease in the left dominant circulation (Figure 2A); the right coronary artery (RCA) was a small caliber and tortuous vessel, with only minor atheroma. However, an abnormal vessel arising from the proximal RCA was noted (Figure 2B and C), confirmed as a fistulous connection to the left atrium by multislice computed tomography, which also excluded other vascular anomalies (Figure 3). This was deemed to be causing a coronary steal phenomenon. Exercise myocardial perfusion imaging under anti-ischemic therapy excluded residual ischemia and the patient was successfully conservatively managed (Figure 4). Coronary-pulmonary artery fistulas (CPAF) are rare, often incidental findings of coronary angiography, thought to

Renal cell carcinoma presenting as a stress cardiomyopathy

A 76-year-old woman was admitted to the emergency department with a squeezing severe chest pain starting at rest, 1h before. Previous medical history was unremarkable. An electrocardiogram showed rapidly evolving ST-segment changes ( Panels A–C ). The rapid troponin I assay was positive (1.4 ng/mL). Unknown renal dysfunction was also noted (creatinine of 1.7 mg/dL). Apical akinesia was present on bedside echocardiogram (TEE). Urgent coronary angiography due to unremitting chest pain and hypotension excluded obstructive coronary disease. Ventriculography showed a striking pattern of apical akinesia ( Panels D and E …

Aortocoronary dissection complicating percutaneous angioplasty

A 56-year-old man was admitted to our hospital for elective coronary angioplasty of a chronic total occlusion (CTO) of the right coronary artery (RCA). After insertion of a right femoral sheath, the RCA was engaged with a 6 French Amplatz AL-2 (Cordis, Miami, Fl, USA) guiding catheter. Recanalization was begun using a 0.014-inch Miracle 3 guidewire (Asahi Intecc, Japan). At this stage, an ostial dissection flap of the RCA with immediate retrograde extension to the ascending aorta was noticed (Figure 1). Stenting of the RCA ostium with a Xience Prime 3.0 × 15 mm stent (Abbott Vascular, Santa Clara, California, USA) was performed, followed by a PTFE-covered 3.0 × 19 mm Jostent graft

Triple, simultaneous, very late coronary stent thrombosis

Coronary artery stent thrombosis is an uncommon but potentially catastrophic complication. The risk of very late stent thrombosis (VLST) raises important safety issues regarding the first generation of drug-eluting stents (DES). Although several complex mechanisms for VLST have been suggested and various predictors have been described, its pathophysiology is not completely understood and it is not known whether longer-term dual antiplatelet therapy reduces the risk. We present a rare case of simultaneous very late DES thrombosis in the three vascular territories, following discontinuation of antiplatelet therapy seven years after stent placement, presenting as cardiogenic shock.

Pulmonary embolism with thromboembolus in transit.

An 88-year-old woman was admitted with syncope and rapidly evolved with shock. Her medical history was notable for recent orthopedic surgery. The electrocardiogram showed ST-segment depression in the precordial and inferior leads (Figure 1). Rapid troponin I assay was positive. Bedside echocardiography revealed a dilated and dysfunctional right ventricle, with akinesia of the lateral wall and paradoxical interventricular septal motion (Figure 2A). Remarkably, a large, worm-like, mobile, freefloating right atrial mass was noted, insinuating through the tricuspid valve during systole (Figure 2B--D). Acute pulmonary embolism (PE) with right heart thromboembolus was strongly suspected, so prompt resuscitation and anticoagulant therapy were started, while emergency thoracic computed tomography angiography confirmed bilateral central emboli (Figure 3A--C). Despite the high bleeding risk, thrombolysis was undertaken, given the life-threatening condition, with gradual improvement in clinical status and no major bleeding complications. No right heart thromboembolus was found in the follow-up echocardiogram and the patient had an uneventful hospital course. Right heart thromboemboli in transit are an incidental finding in up to 20% of patients with acute high-risk

The importance of qualitative and quantitative regional wall motion abnormality assessment at rest in pediatric coronary allograft vasculopathy

CAV remains one of the main limiting factors for survival in children after heart transplantation. In this study, we explored the incremental value of routine CMR for evaluation and detection of CAV using qualitative and quantitative analysis of regional and global myocardial function and strain. This was a prospective imaging biomarker validation trial. Twenty‐two patients (11 male), aged between 10 and 17 years (median 14 years) post‐heart transplantation, were prospectively enrolled and underwent CMR in addition to their biennial review workup with Echo, angiography, and IVUS. Nine healthy control patients were enrolled to undergo CMR alone. Echo was used to analyze WMAs and systolic function. CMR images were analyzed qualitatively for RWMA and quantitatively for volumetric analysis, S and SR. All results were compared to IVUS and angiography assessments. Qualitatively, CMR detected RWMA corresponding to angiographic disease in 3 patients that were not detected on Echo. However, quantitative strain analysis suggested RWMA in an extra 9 patients. Detection of regional wall motion abnormality using quantitative strain analysis was associated with a higher mean stenosis grade (P=.04) and reduced graft survival (P=.04) compared to those with no quantitative wall motion abnormality. Overall, only longitudinal stain was abnormal in patients compared with controls, but there was no correlation between any of the global indices of S or SR and IVUS measurements. CMR is more sensitive than Echo for the visual detection of significant WMAs. Quantitative CMR strain analysis at rest may give additional information to discriminate those at greatest risk.