Aortic enlargement and impaired bioelasticity are of interest in several cardiac and non-cardiac diseases as they can lead to cardiovascular complications. Cardiovascular magnetic resonance (CMR) is ...increasingly accepted as a noninvasive tool in cardiovascular evaluation. Assessment of aortic anatomy and bioelasticity, namely aortic distensibility and pulse wave velocity (PWV), by CMR is accurate and reproducible and could help to identify anatomical and bioelastic abnormalities of the aorta. However, normal CMR values for healthy children and young adults are lacking.
Seventy-one heart-healthy subjects (age 16.4 ± 7.6 years, range 2.3-28.3 years) were examined using a 3.0 Tesla CMR scanner. Aortic cross-sectional areas and aortic distensibility were measured at four positions of the ascending and descending thoracic aorta. PWV was assessed from aortic blood flow velocity measurements in a aortic segment between the ascending aorta and the proximal descending aorta. The Lambda-Mu-Sigma (LMS) method was used to obtain percentile curves for aortic cross-sectional areas, aortic distensibility and PWV according to age.
Aortic areas, PWV and aortic distensibility (aortic cross-sectional areas: r = 0.8 to 0.9, p < 0.001; PWV: r = 0.25 to 0.32, p = 0.047 to 0.009; aortic distensibility r = -0.43 to -0.62, p < 0.001) correlated with height, weight, body surface area, and age. There were no significant sex differences.
This study provides percentile curves for cross-sectional areas, distensibility and pulse wave velocity of the thoracic aorta in children and young adolescents between their 3rd and 29th year of life. These data may serve as a reference for the detection of pathological changes of the aorta in cardiovascular disease.
Congenital heart disease is the most common group of congenital pathology. Over the past few decades, advances in surgical treatment have resulted in a rising population of adult patients with ...repaired complex congenital heart disease. Although the quality of life has greatly improved, a significant proportion of morbidities encountered in clinical practice is now seen in adults rather than in children. These patients often have significant haemodynamic pathophysiology necessitating repeat intervention. CT and MRI are excellent imaging modalities, which help elucidate potential complications that may need urgent management. Although imaging should be performed in specialised centres, occasionally patients may present acutely to emergency departments in hospitals with little experience in managing potentially complex patients. The purpose of this article is to provide an introductory overview to the radiologist who may not be familiar with complex congenital heart disease in adult patients. This educational review has three main sections: (1) a brief overview of the post-operative anatomy and surgical management of the most common complex conditions followed by (2) a discussion on CT/MRI protocols and (3) a review of the various complications and their CT/MRI findings.
Improved understanding of dilated cardiomyopathy (DCM) due to titin truncation (TTNtv) may help guide patient stratification.
The purpose of this study was to establish relationships among TTNtv ...genotype, cardiac phenotype, and outcomes in DCM.
In this prospective, observational cohort study, DCM patients underwent clinical evaluation, late gadolinium enhancement cardiovascular magnetic resonance, TTN sequencing, and adjudicated follow-up blinded to genotype for the primary composite endpoint of cardiovascular death, and major arrhythmic and major heart failure events.
Of 716 subjects recruited (mean age 53.5 ± 14.3 years; 469 men 65.5%; 577 80.6% New York Heart Association function class I/II), 83 (11.6%) had TTNtv. Patients with TTNtv were younger at enrollment (49.0 years vs. 54.1 years; p = 0.002) and had lower indexed left ventricular mass (5.1 g/m2 reduction; padjusted = 0.03) compared with patients without TTNtv. There was no difference in biventricular ejection fraction between TTNtv+/− groups. Overall, 78 of 604 patients (12.9%) met the primary endpoint (median follow-up 3.9 years; interquartile range: 2.0 to 5.8 years), including 9 of 71 patients with TTNtv (12.7%) and 69 of 533 (12.9%) without. There was no difference in the composite primary outcome of cardiovascular death, heart failure, or arrhythmic events, for patients with or without TTNtv (hazard ratio adjusted for primary endpoint: 0.92 95% confidence interval: 0.45 to 1.87; p = 0.82).
In this large, prospective, genotype-phenotype study of ambulatory DCM patients, we show that prognostic factors for all-cause DCM also predict outcome in TTNtv DCM, and that TTNtv DCM does not appear to be associated with worse medium-term prognosis.
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Single ventricle (SV) patients with a Fontan circulation are at risk for functional deterioration. The aim of this study was to assess longitudinal Fontan hemodynamics using serial cardiovascular ...magnetic resonance (CMR) data and to study the impact of aortopulmonary collateral (APC) flow and type of SV morphology.
Forty-one Fontan patients (age at first CMR 13.4 ± 6.0 years) with two CMR examinations within a time interval of at least 4 years (follow-up 5.3 ± 0.9 years) were included. The protocol consisted of short-axis cine volumetry and 2-dimensional blood flow measurements in the inferior vena cava (IVC), superior vena cava (SVC) and ascending aorta (Ao). APC flow was calculated as Ao – (SVC + IVC). Myocardial strain/strain rate was assessed using feature tracking technique.
SV end-diastolic volume (p = 0.14) and ejection fraction (p = 0.70) remained constant. No significant changes in CMR derived myocardial strain/strain rate values were observed. Ao flow decreased (p = 0.01), IVC flow remained unchanged (p = 0.52), while SVC flow (p = 0.003) and APC flow (p = 0.006) decreased significantly. Patients with a systemic right ventricle (RV) showed unchanged APC flow and a further increase in SV size over time compared to patients with a systemic left ventricle.
Longitudinal CMR data in a cohort of clinically stable Fontan patients revealed no significant changes in SV dimensions and myocardial performance while APC flow decreased spontaneously. Patients with a systemic RV seem to be at risk for permanent SV volume overload through APCs and might therefore benefit from consequent interventional APC embolization.
•In a clinically stable Fontan cohort SV size and function remained unchanged after 4-year follow-up.•APC flow decreased spontaneously in the entire study population.•The group with a systemic RV showed unchanged APC flow and progressive SV dilatation.•Systemic RV patients may therefore benefit from consequent interventional APC embolization.
Introduction Transcatheter mitral valve replacement (TMVR) is a valuable treatment option in patients with severe mitral regurgitation. Prior transapical transcatheter aortic valve replacement ...(TA-TAVR) may complicate the procedure and is therefore considered a relative contraindication. In this case report, the authors describe the successful TMVR as a tertiary cardiac surgery and transapical redo procedure. Case Summary An 83-year-old male patient, suffering from dyspnoea and angina, was diagnosed with severe mitral valve regurgitation (MR). He had already undergone cardiac surgery in the form of coronary artery bypass grafting at the age of 64 and TA-TAVR at 79 years. After a failed attempt at mitral valve transcatheter edge-to-edge repair, he opted for TMVR. Pre-TMVR computed tomography simulation was used to analyse possible interactions between the prostheses and to predict the neo-left ventricular outflow tract (neo-LVOT). The operation was carried out without complications. There was no bleeding and the LV function remained unchanged. On MRI, the valves were perfectly aligned without any signs of paravalvular leakage or LVOT obstruction. The patient was discharged seven days postoperatively. At the one-year follow up, there was no need for rehospitalisation and the patient had clinically improved (from NYHA IV to II). Echocardiography demonstrated a mean transvalvular gradient of under 5 mmHg and no residual MR. Conclusion A redo transapical access for TMVR as a tertiary cardiac operation can be easily performed. Pre-operative CT suggested good alignment of the aortic and mitral valved stent which was confirmed postoperatively.
Ventricular dysfunction is a well-known complication in single ventricle patients in Fontan circulation. As studies exclusively examining patients with a single left ventricle (SLV) are sparse, we ...assessed left ventricular (LV) function in SLV patients by using 2D-cardiovascular magnetic resonance (CMR) feature tracking (2D-CMR-FT) and 2D-speckle tracking echocardiography (2D-STE). 54 SLV patients (11.4, 3.1–38.1 years) and 35 age-matched controls (12.3, 6.3–25.8 years) were included. LV global longitudinal, circumferential and radial strain (GLS, GCS, GRS) and strain rate (GLSR, GCSR, GRSR) were measured using 2D-CMR-FT. LV volumes, ejection fraction (LVEF) and mass were determined from short axis images. 2D-STE was applied in patients to measure peak systolic GLS and GLSR. In a subgroup analysis, we compared double inlet left ventricle (DILV) with tricuspid atresia (TA) patients. The population consisted of 19 DILV patients, 24 TA patients and 11 patients with diverse diagnoses. 52 patients were in NYHA class I and 2 patients were in class II. Most SLV patients had a normal systolic function but median LVEF in patients was lower compared to controls (55.6% vs. 61.2%, p = 0.0001). 2D-CMR-FT demonstrated reduced GLS, GCS and GCSR values in patients compared to controls. LVEF correlated with GS values in patients (p < 0.05). There was no significant difference between GLS values from 2D-CMR-FT and 2D-STE in the patient group. LVEF, LV volumes, GS and GSR (from 2D-CMR-FT) were not significantly different between DILV and TA patients. Although most SLV patients had a preserved EF derived by CMR, our results suggest that, LV deformation and function may behave differently in SLV patients compared to healthy subjects.
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