Hannah Bellsham-Revell

Serial Magnetic Resonance Imaging in Hypoplastic Left Heart Syndrome Gives Valuable Insight Into Ventricular and Vascular Adaptation

Objectives This study sought to investigate changes in magnetic resonance imaging (MRI) ventricular volumes and vascular dimensions before hemi-Fontan (HF) and before total cavopulmonary connection (TCPC) in children with hypoplastic left heart syndrome (HLHS). Background The systemic right ventricle (RV) in HLHS is subject to significant changes in volume loading throughout the surgical stages of palliation, particularly after the HF. Methods Fifty-eight patients had paired pre-HF and pre-TCPC MRI for assessment of changes of RV volumes, neoaortic flow, and vascular dimensions. Results Comparison of pre-HF and pre-TCPC MRI results showed a decrease of indexed RV end-diastolic volume and end-systolic volume (98 ml/m2 to 87 ml/m2 and 50 ml/m2 to 36 ml/m2, respectively) with stroke volume remaining constant (49 ml/m2 vs. 51 ml/m2), leading to an increased RV ejection fraction (51% vs. 59%). These findings persisted after excluding the 3 patients who underwent tricuspid valve repair as part of their HF procedure. Indexed RV end-diastolic volume plotted against neoaortic stroke volume demonstrated a Frank-Starling–like curve that shifted upward after HF. The indexed distal left and right cross-sectional pulmonary artery areas were reduced after HF. Conclusions In HLHS, serial MRI shows the adaptation of the systemic RV after HF with volume reduction in the context of a preserved stroke volume and an increased ejection fraction. The staged palliation in HLHS may be a risk factor particularly for reduced left pulmonary artery growth in itself as no factors investigated in this study were found to significantly impact on this.

Subjective Evaluation of Right Ventricular Systolic Function in Hypoplastic Left Heart Syndrome: How Accurate Is It?

Background The geometry and heterogeneity of the right ventricle in hypoplastic left heart syndrome makes objective echocardiographic assessment of systolic function challenging. Consequently, subjective echocardiographic assessment of right ventricular (RV) function is still routinely undertaken. The aims of this study were to compare this with magnetic resonance imaging (MRI), investigate the impact of experience and training on the accuracy of subjective assessment, and critically analyze the role of echocardiography to detect impaired systolic function. Methods A retrospective analysis of prospectively acquired data was performed. Children with hypoplastic left heart syndrome underwent routine preoperative cardiac MRI and echocardiography under the same general anesthetic. Echocardiograms were reviewed, and members of the congenital heart disease team with differing echocardiography experience subjectively graded RV systolic function (good, moderate, or poor). This was compared with MRI-derived ejection fraction. Results Twenty-eight patients at different palliative stages were included. Twenty-eight observers were divided into five experience categories (congenital heart disease junior trainees to attending cardiologists). Median agreement was 47.6% (range, 31.4%–58.2%), with the lowest agreement among junior trainees and the highest among attending cardiologists. When used as a screening test for poor RV systolic function, the median sensitivity of echocardiography was 0.89 (range, 0.86–0.96), and median specificity was 0.45 (range, 0.26–0.55). The highest sensitivity was observed among junior trainees but with the lowest specificity. The highest specificity was observed among attending cardiologists (0.55). Conclusions Agreement between echocardiographic and MRI RV ejection fraction improves with experience but remains suboptimal. When used as a screening test for poor RV function, echocardiography is sensitive, but specificity is heavily influenced by operator experience.

Analysis of preoperative condition and interstage mortality in Norwood and hybrid procedures for hypoplastic left heart syndrome using the Aristotle scoring system

Objective The ‘hybrid procedure’, consisting of surgical banding of the pulmonary arteries with intraoperative stenting of the arterial duct, was developed as primary palliation in hypoplastic left heart syndrome (HLHS), avoiding the risks of cardiopulmonary bypass. In many centres, it is reserved for low birth weight, premature or unstable neonates; however, its role in such high risk cases of HLHS has yet to be defined. Methods The preoperative condition of all patients with HLHS who underwent either the hybrid or the Norwood procedure for HLHS between 2005–2011 was analysed retrospectively, using a modified comprehensive Aristotle score. We then compared operative, interstage and 1 year mortalities between the groups after Aristotle adjustment via Cox proportional hazards analyses. Results Of 138 patients with HLHS, 27 had hybrid and 111 Norwood procedures. The hybrid group had significantly higher Aristotle scores (mean 4.1 vs 1.8; p<0.001); however, there was no significant difference in mortality at any stage. At 1 year, the overall unadjusted survival among Norwood and hybrid patients was 58.6% and 51.9%, respectively, yielding an Aristotle adjusted hazard ratio for mortality among hybrid patients of 1.09 (95% CI 0.56 to 2.11, p=0.80). Conclusions Applying a hybrid approach to high risk patients with HLHS produces a comparable early and interstage mortality risk to lower risk patients undergoing the Norwood procedure. Prospective studies are needed to establish whether the hybrid procedure is a viable alternative to the Norwood procedure in all HLHS patients in terms of both mortality and long term morbidity.

Inflow Typology and Ventricular Geometry Determine Efficiency of Filling in the Hypoplastic Left Heart

BACKGROUND Pediatric patients with hypoplastic left heart syndrome rely solely on the right ventricle, resulting in anatomic maladaptations that can significantly compromise diastolic efficiency and lead to heart failure. Clinical indices to evaluate diastole are generally derived from the adult left ventricle, limiting their relevance to patients with hypoplastic left heart syndrome. We investigated the effect of the ventricular cavity shape and tricuspid inflow typology on the filling dynamics to provide new directions of investigation for assessing diastolic function in these patients. METHODS Magnetic resonance imaging data were used to generate personalized mathematic models of 4 patients with different prognoses after stage I of the Norwood procedures. Two of these patients were also modeled after stage II. Numeric simulations were performed to analyze the interaction between blood flow and the myocardium during diastole. RESULTS The filling dynamics were characterized by the formation of an organized structure of swirling blood (vortex ring). This was strongly influenced by the ventricular shape and the timing of the E and A wave. Biphasic rather than fused inflows and more elliptical than spherical cavities were found to increase the intraventricular pressure gradients and the filling capacity by optimizing the energy transfer between blood flow and the myocardium. This resulted in a better flow propagation and higher tissue velocities and displacements. CONCLUSIONS The variations in the kinetic energy associated with the blood motion reflected the base-to-apex pressure difference and can therefore be used to quantify the efficiency of filling, providing a potential new metric to assess diastolic function in these patients.

Image orientation for three-dimensional echocardiography of congenital heart disease

To date there has been little discussion about image orientation for three-dimensional (3D) echocardiography when applied to congenital heart lesions. Anatomic relations cannot be assumed in congenital heart disease and image cropping during post processing may by necessity remove external or even internal anatomic references. We present an approach to consistent anatomic orientation which is both intuitive and consistent with regard to superior–inferior, anterior–posterior and left–right axes. Such anatomic orientation is also concordant with other common 3D imaging modes such as cardiac magnetic resonance imaging and computed tomography. Views derived from standard cross sectional echocardiography have such universal familiarity that analogous 3D projections of these views may be retained but novel hitherto unavailable views such as en face views of the cardiac septums or atrioventricular valves may be projected using anatomic orientation.

Assessment of right ventricular volumes in hypoplastic left heart syndrome by real-time three-dimensional echocardiography: comparison with cardiac magnetic resonance imaging

BACKGROUND Accurate assessment of right ventricular (RV) volumes and function is important in patients with hypoplastic left heart syndrome (HLHS). We prospectively sought to determine the reproducibility of three-dimensional (3D) echocardiography and its agreement with cardiac magnetic resonance imaging (CMR) in HLHS. METHODS AND RESULTS Twenty-eight patients underwent CMR followed immediately by transthoracic 3D echocardiography under general anaesthesia. Semi-automated border detection software was used to determine echocardiographic RV volumes. Inter- and intra-observer variability, correlation and levels of agreement between techniques were determined. The median age was 0.37 years (0.18-9.28 years) and weight 6.24 kg (3.42-32.50 kg). Intra- and inter-observer variability was excellent for both techniques. Median (range) measurements for 3D echocardiography and CMR were; end-diastolic volume (EDV) 23.6 mL (6.5-63.2) and 30.6 mL (11.8-87.9), end-systolic volume (ESV) 12.6 mL (3.7-37.0) and 14.9 mL (5.8-33.9), stroke volume (SV) 11.2 mL (2.8-33.0) and 17.1 mL (6.0-54.1), ejection fraction (EF) 48.2% (31.2-64.9), and 56.5% (42.7-72.2). Correlation coefficients were r = 0.85, 0.84, 0.83, and 0.74, respectively (P < 0.01 for all). Volumetric data were expressed as a percentage of the echocardiographic volume to CMR volume. When compared with CMR, 3D echocardiography underestimated EDV, ESV and SV by 26.7% (SD ± 20.2), 10.6% (±28.1), and 37.5% (±20.1), respectively. The difference in volume appeared largest at low ventricular volumes. EF was 8.3% (±7.3) lower by 3D echocardiography compared with CMR. CONCLUSION Both 3D echocardiography and CMR volumes appear highly reproducible. Measurements obtained by 3D echocardiography are significantly lower than those obtained by CMR, with wide limits of agreement such that these two methods cannot be used interchangeably.

Three-dimensional Dual-Phase Whole-Heart MR Imaging: Clinical Implications for Congenital Heart Disease

PURPOSE To identify which rest phase (systolic or diastolic) is optimum for assessing or measuring cardiac structures in the setting of three-dimensional (3D) whole-heart imaging in congenital heart disease (CHD). MATERIALS AND METHODS The study was approved by the institutional review board; informed consent was obtained. Fifty children (26 male and 24 female patients) underwent 3D dual-phase whole-heart imaging. Cardiac structures were analyzed for contrast-to-noise ratio (CNR) and image quality. Cross-sectional measurements were taken of the aortic arch, right ventricular (RV) outflow tract (RVOT) and pulmonary arteries. Normally distributed variables were compared by using paired t tests, and categorical data were compared by using Wilcoxon signed-rank test. RESULTS Mean CNR and image quality were significantly (all P < .05) greater in systole for the right atrium (CNR, 8.9 vs 7.5; image quality, 438 vs 91), left atrium (CNR, 8.0 vs 5.3; image quality, 1006 vs 29), RV (CNR, 10.6 vs 8.2; image quality, 131 vs 23), LV (CNR, 9.4 vs 7.7; image quality, 125 vs 28), and pulmonary veins (CNR, 6.2 vs 4.9; image quality, 914 vs 32). Conversely, diastolic CNR was significantly higher in the aorta (9.2 vs 8.2; P = .013) and diastolic image quality was higher for the left pulmonary artery (238 vs 62; P = .007), right pulmonary artery (219 vs 35; P < .001), and for imaging of an area after an arterial stenosis (164 vs 7; P < .001). All aortic arch and RVOT cross-sectional measurements were significantly (P < .05) greater in systole (narrowest point of arch, 70 vs 53 mm(2); descending aorta, 71 vs 58 mm(2); transverse arch, 293 vs 275 mm(2); valvar RVOT, 291 vs 268 mm(2); supravalvar RVOT, 337 vs 280 mm(2); prebifurcation RVOT, 329 vs 259 mm(2)). CONCLUSION Certain structures in CHD are better imaged in systole and others in diastole, and therefore, the dual-phase approach allows a higher overall success rate. This approach also allows depiction of diameter changes between systole and diastole and is therefore preferable to standard single-phase sequences for the planning of interventional procedures.

Hybrid Procedure for Neonates With Hypoplastic Left Heart Syndrome at High-Risk for Norwood: Midterm Outcomes

BACKGROUND Hybrid procedure offers patients with severe congenital heart disease an alternative initial procedure to conventional surgical reconstruction. We report the midterm outcomes of a cohort of neonates who had a hybrid procedure for variants of hypoplastic left heart syndrome because they were at high risk for the Norwood procedure. METHODS Between December 2005 and January 2013, 41 neonates underwent bilateral pulmonary artery banding followed by ductal stenting by means of a sternotomy at a median age of 6 days (range, 2 to 18 days) and weight of 2.6 kg (range, 1.5 to 3.7 kg). Thirty-five patients had hypoplastic left heart syndrome, and 6 patients had critical aortic stenosis with hypoplastic left ventricle. Primary indications for the hybrid procedure were low birth weight in 17 patients, hypoplastic left ventricle with the possibility of later biventricular repair in 6 patients, intact or near-intact atrial septum in 5 patients, and poor patient condition in 13 patients. Echocardiographic, angiographic, operative, and clinical data were reviewed. Outcomes were summarized with descriptive statistics and risk factors for mortality identified. RESULTS All but 6 patients had an antenatal diagnosis, and 24 patients were from other congenital cardiac centers. Nine patients had perioperative balloon aortic valvuloplasty, 1 patient had fetal balloon aortic valvuloplasty, and 17 patients had intervention to their atrial septum (41.4%). There were 9 inpatient deaths (21.9%) and 4 interstage deaths (9.8%) after the hybrid procedure. Twenty-eight patients subsequently underwent either the Norwood procedure (11 patients), combined stage I and II (14 patients), or biventricular repair (3 patients). No patient had heart transplantation. Among the patients who had combined stage I and II as a second procedure after the hybrid procedure, there were 2 early deaths, 1 late death before the Fontan, and 1 late death after the Fontan completion after combined stage I and II. All patients who had subsequent Norwood procedure were midterm survivors. Three of the 4 patients who had biventricular repair were midterm survivors. Overall survival was 56.1% at a median follow-up of 32.0 months. By univariate analysis, patient factors, intact or near-intact atrial septum, and aortic atresia were associated with nonsurvival. CONCLUSIONS Hybrid procedure as an alternative to the Norwood procedure offers good midterm survival in patients deemed at high risk for neonatal reconstruction.

Magnetic resonance imaging catheter stress haemodynamics post-Fontan in hypoplastic left heart syndrome

AIMS Exercise limitation is common post-Fontan. Hybrid X-ray and magnetic resonance imaging (XMR) catheterization allows haemodynamic assessment by means of measurement of ventricular volumes and flow in major vessels with simultaneous invasive pressures. We aim to assess haemodynamic response to stress in patients with hypoplastic left heart syndrome (HLHS) post-Fontan. METHODS AND RESULTS Prospective study of 13 symptomatic children (NHYA 2) with HLHS post-Fontan using XMR catheterization. Three conditions were applied: baseline (Stage 1), dobutamine at 10 µg/kg/min (Stage 2), and dobutamine at 20 µg/kg/min (Stage 3). Seven consecutive patients received inhaled nitric oxide (iNO) at peak stress. Control MRI data were from normal healthy adults. In the HLHS patients, baseline mean pulmonary vascular resistance (PVR) was 1.51 ± 0.59 WU m(2) and aortopulmonary collateral flow was 17.7 ± 13.6% of systemic cardiac output. Mean right ventricular end-diastolic pressure was 6.7 ± 2.5 mmHg which did not rise with stress. Cardiac index (CI) increased at Stage 2 in HLHS (40%) and controls (61%) but continued to increase at Stage 3 only in controls (19%) but not in HLHS. The blunted rise in CI in HLHS was due to a continuing fall in end-diastolic volume throughout stress, with no significant change in PVR or CI at peak stress in response to iNO. CONCLUSION Cardiac output post-Fontan in HLHS at peak stress is blunted due to a limitation in preload which is not responsive to inhaled pulmonary vasodilators in the setting of normal PVR.

Treatment of severe tracheobronchomalacia: Ten-year experience

INTRODUCTION Paediatric tracheobronchomalacia is a rare but potentially serious condition. Severe tracheobronchomalacia requires intervention or operation. This is an evaluation of a ten-year experience at an institution. METHODS In this retrospective study all patients were included that required an intervention for severe tracheobronchomalacia from 2003 to 2012. Symptoms, aetiology, comorbidities, localisation of the malacia, age at diagnosis, therapeutic measures and associated complications were evaluated. RESULTS Forty-four patients with severe tracheobronchomalacia underwent intervention/operation. The predominant aetiology was vascular compression in 48%. The majority of patients had complex comorbidities, most importantly cardiac pathology in 66%. The median age at diagnosis was 3 months. A total of 17 aortopexies, 21 tracheostomies and 25 stent placements were performed. The mean follow-up was 2.6 years. Severe complications occurred in 12 patients. The most common complications were stent obstruction/fracture and tracheostomy tube obstruction. CONCLUSION The management of severe tracheobronchomalacia is complex and the population of patients is very heterogeneous. Therefore the treatment has to be adapted for each patient individually. The decision strategies are discussed in this article. The surgical techniques for placement and safe removal of expandable bare metallic stents employed in our institution are presented. A multidisciplinary team of ENT surgeons, Intensivists, Cardiologists and Cardiac surgeons is of great importance.