Bramwell-Hill modeling for local aortic pulse wave velocity estimation: a validation study with velocity-encoded cardiovascular magnetic resonance and invasive pressure assessment

<p>Abstract</p> <p>Background</p> <p>The Bramwell-Hill model describes the relation between vascular wall stiffness expressed in aortic distensibility and the pulse wave velocity (PWV), which is the propagation speed of the systolic pressure wave through the aorta. The main objective of this study was to test the validity of this model locally in the aorta by using PWV-assessments based on in-plane velocity-encoded cardiovascular magnetic resonance (CMR), with invasive pressure measurements serving as the gold standard.</p> <p>Methods</p> <p>Seventeen patients (14 male, 3 female, mean age ± standard deviation = 57 ± 9 years) awaiting cardiac catheterization were prospectively included. During catheterization, intra-arterial pressure measurements were obtained in the aorta at multiple locations 5.8 cm apart. PWV was determined regionally over the aortic arch and locally in the proximal descending aorta. Subsequently, patients underwent a CMR examination to measure aortic PWV and aortic distention. Distensibility was determined locally from the aortic distension at the proximal descending aorta and the pulse pressure measured invasively during catheterization and non-invasively from brachial cuff-assessment. PWV was determined regionally in the aortic arch using through-plane and in-plane velocity-encoded CMR, and locally at the proximal descending aorta using in-plane velocity-encoded CMR. Validity of the Bramwell-Hill model was tested by evaluating associations between distensibility and PWV. Also, theoretical PWV was calculated from distensibility measurements and compared with pressure-assessed PWV.</p> <p>Results</p> <p>In-plane velocity-encoded CMR provides stronger correlation (p = 0.02) between CMR and pressure-assessed PWV than through-plane velocity-encoded CMR (r = 0.69 versus r = 0.26), with a non-significant mean error of 0.2 ± 1.6 m/s for in-plane versus a significant (p = 0.006) error of 1.3 ± 1.7 m/s for through-plane velocity-encoded CMR. The Bramwell-Hill model shows a significantly (p = 0.01) stronger association between distensibility and PWV for local assessment (r = 0.8) than for regional assessment (r = 0.7), both for CMR and for pressure-assessed PWV. Theoretical PWV is strongly correlated (r = 0.8) with pressure-assessed PWV, with a statistically significant (p = 0.04) mean underestimation of 0.6 ± 1.1 m/s. This theoretical PWV-estimation is more accurate when invasively-assessed pulse pressure is used instead of brachial cuff-assessment (p = 0.03).</p> <p>Conclusions</p> <p>CMR with in-plane velocity-encoding is the optimal approach for studying Bramwell-Hill associations between local PWV and aortic distensibility. This approach enables non-invasive estimation of local pulse pressure and distensibility.</p

Management of the hypoplastic left heart syndrome : from fetus to adult

Non peer reviewe

Diagnosis, imaging and clinical management of aortic coarctation

Coarctation of the aorta (CoA) is a well-known congenital heart disease (CHD), which is often associated with several other cardiac and vascular anomalies, such as bicuspid aortic valve (BAV), ventricular septal defect, patent ductus arteriosus and aortic arch hypoplasia. Despite echocardiographic screening, prenatal diagnosis of CoA remains difficult. Most patients with CoA present in infancy with absent, delayed or reduced femoral pulses, a supine arm-leg blood pressure gradient (>20 mm Hg), or a murmur due to rapid blood flow across the CoA or associated lesions (BAV). Transthoracic echocardiography is the primary imaging modality for suspected CoA. However, cardiac magnetic resonance imaging is the preferred advanced imaging modality for non-invasive diagnosis and follow-up of CoA. Adequate and timely diagnosis of CoA is crucial for good prognosis, as early treatment is associated with lower risks of long-term morbidity and mortality. Numerous surgical and transcatheter treatment strategies have been reported for CoA. Surgical resection is the treatment of choice in neonates, infants and young children. In older children (>25 kg) and adults, transcatheter treatment is the treatment of choice. In the current era, patients with CoA continue to have a reduced life expectancy and an increased risk of cardiovascular sequelae later in life, despite adequate relief of the aortic stenosis. Intensive and adequate follow-up of the left ventricular function, valvular function, blood pressure and the anatomy of the heart and the aorta are, therefore, critical in the management of CoA. This review provides an overview of the current state-of-the-art clinical diagnosis, diagnostic imaging algorithms, treatment and follow-up of patients with CoA

Diagnosis, imaging and clinical management of aortic coarctation

Coarctation of the aorta (CoA) is a well-known congenital heart disease (CHD), which is often associated with several other cardiac and vascular anomalies, such as bicuspid aortic valve (BAV), ventricular septal defect, patent ductus arteriosus and aortic arch hypoplasia. Despite echocardiographic screening, prenatal diagnosis of CoA remains difficult. Most patients with CoA present in infancy with absent, delayed or reduced femoral pulses, a supine arm-leg blood pressure gradient (>20 mm Hg), or a murmur due to rapid blood flow across the CoA or associated lesions (BAV). Transthoracic echocardiography is the primary imaging modality for suspected CoA. However, cardiac magnetic resonance imaging is the preferred advanced imaging modality for non-invasive diagnosis and follow-up of CoA. Adequate and timely diagnosis of CoA is crucial for good prognosis, as early treatment is associated with lower risks of long-term morbidity and mortality. Numerous surgical and transcatheter treatment strategies have been reported for CoA. Surgical resection is the treatment of choice in neonates, infants and young children. In older children (>25 kg) and adults, transcatheter treatment is the treatment of choice. In the current era, patients with CoA continue to have a reduced life expectancy and an increased risk of cardiovascular sequelae later in life, despite adequate relief of the aortic stenosis. Intensive and adequate follow-up of the left ventricular function, valvular function, blood pressure and the anatomy of the heart and the aorta are, therefore, critical in the management of CoA. This review provides an overview of the current state-of-the-art clinical diagnosis, diagnostic imaging algorithms, treatment and follow-up of patients with CoA

Newsletter from the Association for European Pediatric Cardiology Subgroup of AEPC Junior Members

status: publishe

Three-dimensional and four-dimensional flow assessment in congenital heart disease

Congenital heart disease (CHD) is the most common form of congenital defects, with an incidence of 8 per 1000 births. Due to major advances in diagnostics, perioperative care and surgical techniques, the survival rate of patients with CHD has improved dramatically. Conversely, although 70%-95% of infants with CHD survive into adulthood, the rate of long-term morbidity, which often requires (repeat) intervention, has increased. Recently, the role of altered haemodynamics in cardiac development and CHD has become a subject of interest. Patients with CHD often have abnormal blood flow patterns, either due to the primary cardiac defect or as a consequence of the surgical intervention(s). Research suggests that these abnormal blood flow patterns may contribute to diminished cardiac and vascular function. Serial assessment of haemodynamic parameters in patients with CHD may allow for improved understanding of the often complex haemodynamics in these patients and thereby potentially guide the timing and nature of interventions with the aim of preventing progression of cardiovascular deterioration. In this article we will discuss two novel non-invasive four-dimensional (4D) techniques to evaluate cardiovascular haemodynamics: 4D-flow cardiac magnetic resonance and computational fluid dynamics. This review focuses on the additional value of these two modalities in the evaluation of patients with CHD with abnormal flow patterns, who could benefit from advanced haemodynamic evaluation: Patients with coarctation of the aorta, bicuspid aortic valve, tetralogy of Fallot and patients after Fontan palliation

Three-dimensional and four-dimensional flow assessment in congenital heart disease

Congenital heart disease (CHD) is the most common form of congenital defects, with an incidence of 8 per 1000 births. Due to major advances in diagnostics, perioperative care and surgical techniques, the survival rate of patients with CHD has improved dramatically. Conversely, although 70%-95% of infants with CHD survive into adulthood, the rate of long-term morbidity, which often requires (repeat) intervention, has increased. Recently, the role of altered haemodynamics in cardiac development and CHD has become a subject of interest. Patients with CHD often have abnormal blood flow patterns, either due to the primary cardiac defect or as a consequence of the surgical intervention(s). Research suggests that these abnormal blood flow patterns may contribute to diminished cardiac and vascular function. Serial assessment of haemodynamic parameters in patients with CHD may allow for improved understanding of the often complex haemodynamics in these patients and thereby potentially guide the timing and nature of interventions with the aim of preventing progression of cardiovascular deterioration. In this article we will discuss two novel non-invasive four-dimensional (4D) techniques to evaluate cardiovascular haemodynamics: 4D-flow cardiac magnetic resonance and computational fluid dynamics. This review focuses on the additional value of these two modalities in the evaluation of patients with CHD with abnormal flow patterns, who could benefit from advanced haemodynamic evaluation: Patients with coarctation of the aorta, bicuspid aortic valve, tetralogy of Fallot and patients after Fontan palliation