A Pipeline for the Generation of Realistic 3D Synthetic Echocardiographic Sequences: Methodology and Open-Access Database.

Quantification of cardiac deformation and strain with 3D ultrasound takes considerable research efforts. Nevertheless, a widespread use of these techniques in clinical practice is still held back due to the lack of a solid verification process to quantify and compare performance. In this context, the use of fully synthetic sequences has become an established tool for initial in silico evaluation. Nevertheless, the realism of existing simulation techniques is still too limited to represent reliable benchmarking data. Moreover, the fact that different centers typically make use of in-house developed simulation pipelines makes a fair comparison difficult. In this context, this paper introduces a novel pipeline for the generation of synthetic 3D cardiac ultrasound image sequences. State-of-the art solutions in the fields of electromechanical modeling and ultrasound simulation are combined within an original framework that exploits a real ultrasound recording to learn and simulate realistic speckle textures. The simulated images show typical artifacts that make motion tracking in ultrasound challenging. The ground-truth displacement field is available voxelwise and is fully controlled by the electromechanical model. By progressively modifying mechanical and ultrasound parameters, the sensitivity of 3D strain algorithms to pathology and image properties can be evaluated. The proposed pipeline is used to generate an initial library of 8 sequences including healthy and pathological cases, which is made freely accessible to the research community via our project web-page.

Longitudinal left ventricular strain impairment in type 1 diabetes children and adolescents: a 2D speckle strain imaging study.

AIM: Type 1 diabetes (T1D) involves complex metabolic disturbances in cardiomyocytes leading to morphological and functional abnormalities of the myocardium. The relationship between T1D and cardiac structure and function in children is not well established. Our study investigated whether T1D is associated with early subclinical myocardial disturbances in children and adolescents, and whether the state of metabolic control and diabetes duration are influential factors. METHODS: Standard echocardiography, tissue Doppler imaging (TDI) and two-dimensional (2D) strain imaging were prospectively performed in 100 T1D children (age: 11.3 ± 3.6 years, 52 boys) and compared with 79 controls. RESULTS: The diabetic and control children were comparable with respect to age, gender, heart rate and blood pressure. There were no significant differences between the two groups in left ventricular (LV) ejection fraction, LV remodelling and TDI parameters. Conventional mitral Doppler demonstrated significantly fewer diastolic filling abnormalities with an early filling wave in the diabetes group. Global longitudinal strain (GLS) was also significantly lower in the T1D children, while circumferential strain and radial strain did not differ. GLS correlated with HbA1c (r=0.52; P<0.01), but there was no correlation with diabetes duration. CONCLUSION: Our results suggest that LV longitudinal myocardial deformation is decreased in young patients with T1D, and glycaemic control may be the main risk factor for these changes. Further follow-up is now necessary to precisely determine the clinical significance of these myocardial changes detected by 2D strain imaging in T1D children.

3D strain assessment in ultrasound (Straus): a synthetic comparison of five tracking methodologies.

This paper evaluates five 3D ultrasound tracking algorithms regarding their ability to quantify abnormal deformation in timing or amplitude. A synthetic database of B-mode image sequences modeling healthy, ischemic and dyssynchrony cases was generated for that purpose. This database is made publicly available to the community. It combines recent advances in electromechanical and ultrasound modeling. For modeling heart mechanics, the Bestel-Clement-Sorine electromechanical model was applied to a realistic geometry. For ultrasound modeling, we applied a fast simulation technique to produce realistic images on a set of scatterers moving according to the electromechanical simulation result. Tracking and strain accuracies were computed and compared for all evaluated algorithms. For tracking, all methods were estimating myocardial displacements with an error below 1 mm on the ischemic sequences. The introduction of a dilated geometry was found to have a significant impact on accuracy. Regarding strain, all methods were able to recover timing differences between segments, as well as low strain values. On all cases, radial strain was found to have a low accuracy in comparison to longitudinal and circumferential components.

[Phaeochromocytoma as an unusual aetiology of cardiogenic shock]. / Le phéochromocytome comme cause inhabituelle de choc cardiogénique.

The authors reported a case involving a young patient with a cardiogenic shock associated to an acute pulmonary oedema. According to the seriousness of the shock, an external ventricular assist device (VAD) was initially inserted and replaced thereafter because of the cardiovascular instability, by an external pneumatic biventricular assist device. A cardiogenic shock induced by an acute adrenergic myocarditis due to a phaeochromocytoma was diagnosed. The patient was weaned from the VAD on day 84 and was scheduled for elective surgery of the phaeochromocytoma on day 93. The authors discussed the time of the surgery according to the anticoagulation therapy necessary to the VAD and the necessary caution taken if a cardiogenic shock appeared around surgery.

Liver transplant combined with heart transplant in severe heterozygous hypercholesterolemia: report of the first case and review of the literature.

Familial hypercholesterolemia (FH) is a dominant inherited disease of low-density lipoprotein (LDL) metabolism caused by mutations of LDL receptors mainly located in the liver. This metabolic disorder is responsible for severe cardiovascular disease, from coronary lesions to chronic heart failure (CHF). Liver transplantation in homozygous FH provides the missing functional LDL receptors and thus partially restores LDL receptor activity to more than 50% of normal. Combined heart and liver transplantation was successfully performed in a homozygous FH patient with end-stage heart failure. Herein we report our experience with a heterozygous male patient with terminal CHF, and review data from the literature on short- and long-term results of such procedures.

Aortic root remodeling with the "cuff" technique for stentless valve implantation.

Aortic root and sinotubular junction dilatation and aneurysm of ascending aorta are considered relative contra-indications to implantation of a stentless valve prosthesis, because the modified aortic geometry leads to aortic incompetence and early failure of the prosthesis. Aortic root reconstruction can be performed according to various techniques. We present a surgical technique in which a tubular graft, replacing an ascending aortic aneurysm, allows sinotubular remodeling and satisfactory implantation of a stentless prosthesis. The native aorta is inserted into the vascular prosthesis at the level of the sinotubular junction which is wrapped in order to prevent commissure spreading. Sizing of the vascular and valve prosthesis is made according to annular diameter. Since October 1999, 6 patients have been operated using this technique with good results.

Necrotizing granulomata of the aortic valve in Wegener's disease.

Wegener's disease is an inflammatory disease of unknown etiology, characterized by a granulomatous-necrotizing general vasculitis. Cardiac involvement in the form of aortic pathology is not frequent. We report a case of Wegener's granulomatosis which required prosthetic aortic valve replacement for aortic valve insufficiency. Microscopic examination of the valve demonstrated histopathology typical of Wegener's disease.

Postinfarction ventricular septal rupture: early repair through the right atrial approach.

OBJECTIVE: Early repair of posterior ventricular septal rupture associated with myocardial infarction by means of transinfarct ventriculotomy is technically challenging and can be associated with significant mortality and morbidity. An alternative route of exposing the septum is through the right atrium. This technique, which avoids direct incision of the ventricle in select patients, reduces postrepair bleeding and impairment of ventricular contractile function. METHODS: The results of 12 patients operated on over a 20-year period were reviewed and analyzed. Late follow-up was obtained in all patients who survived the operation. There were 9 men and 3 women, with a mean age of 69.9 years. The mean time between acute myocardial infarction and surgery was 7.3 days (range, 2-16 days). Six patients were in New York Heart Association class IV, and 3 patients presented for surgery in cardiogenic shock. One patient had previously undergone a coronary artery bypass. The surgical technique included a standard sternotomy approach with a transatrial approach to the septal rupture. In all patients the septal rupture was repaired with a Dacron patch. RESULTS: There were 3 early deaths and 1 late death; one patient was reoperated on for a residual shunt. Postoperative complications included low cardiac output, acute renal tubular necrosis, and supraventricular arrhythmia. Eight patients are alive and undergoing echocardiographic investigation, and only 1 patient had a small residual shunt. CONCLUSION: Our experience shows that a posterior ventricular septal rupture can be safely repaired through a transatrial approach. Avoiding additional damage to the ventricle, it reduces the risks of the postoperative bleeding and enhances survival.

Direct coronary stenting without balloon predilation in acute coronary syndromes.

BACKGROUND: This prospective, observational study was designed to evaluate the feasibility and the safety of a new strategy for stenting in acute coronary syndromes: direct stent implantation without predilation of the culprit lesion. This strategy might reduce both the cost of the procedure and the rate of no-reflow, a phenomenon that is more frequently observed during dilation of unstable plaques. METHODS AND RESULTS: Between September 1997 and March 1998, 122 carefully selected patients with unstable angina or acute myocardial infarction were included in this study. Highly calcified lesions and vessels with excessive proximal tortuosity were excluded. The procedure was successful in 96% of cases. In 5 cases the stent failed to pass through the stenosis and was successfully retrieved in the guiding catheter in 3 cases. In 2 cases the stent was lost in the peripheral circulation. Transient no-reflow occurred in only 3 cases and was rapidly reversed by rescue use of an intracoronary bolus injection of a glycoprotein IIb/IIIa receptor inhibitor in 2 cases. A patient treated by primary angioplasty with cardiogenic shock on admission died 48 hours after the initial procedure because of irreversible cardiac failure. One-month clinical follow-up was obtained by telephone for all patients; no major coronary events occurred during this period. CONCLUSIONS: Direct coronary stenting without balloon predilation can be safely performed in acute coronary syndrome-related lesions in selected patients. A randomized, controlled study is warranted to confirm the promising results of this pilot study, especially regarding the low rate of the no-reflow phenomenon.

Spontaneous native aortic valve thrombosis.

Spontaneous thrombosis of a native aortic valve is an uncommon event that usually follows local trauma, such as cardiac surgery or left heart catheterization, or occurs as a complication of bacterial endocarditis. We report the case of a 65-year-old woman with a history of retinal artery occlusion and severe aortic valve stenosis, in whom transesophageal echocardiography revealed a mobile mass attached to the ventricular surface of the aortic valve. There was no evidence of any hypercoagulable state or infection process. Surgery was performed and a severely stenotic valve resected; a partially organized and firmly adherent free-floating thrombotic mass was observed on the ventricular surface of the aortic valve. Histologic examination demonstrated an organized thrombus. Eleven months after surgery the patient is doing well.

[Treatment of refractory cardiac insufficiency by peritoneal ultrafiltration. Literature review]. / Traitement de l'insuffisance cardiaque réfractaire par ultrafiltration péritonéale. Revue de la littérature.

The management of heart failure that has become refractory to conventional drug treatment is an increasingly frequent problem for clinicians. Peritoneal dialysis, by ultrafiltration, is an effective method to treat refractory oedema, thereby improving the functional symptoms of these patients. The objective of this article is to review the data, of the literature and to define the results and indications of this treatment, which still remains and exceptional modality.

Recurrent left-sided heart leiomyosarcoma: should heart transplantation be legitimate?

Leiomyosarcoma of the heart is an uncommon primary malignant tumor with poor postoperative survival that may be measured in months. A leiomyosarcoma of the left atrium was diagnosed in a 47-year-old man. Initial admission was for acute pulmonary edema requiring emergency surgery. The tumor involved the left atrial cavity, and a radical resection was performed. Six months later an isolated myxomatous recurrence was detected. Heart transplantation was then performed. The patient is in good health 20 months after operation with no evidence of residual disease or recurrence. The literature has been reviewed. Surgical resection is not an adequate treatment for leiomyosarcoma of the left atrium and early heart transplantation probably offers the only hope for these patients.

Impairment of cardiac neuronal function in acute myocarditis: iodine-123-MIBG scintigraphy study.

UNLABELLED: Abnormalities of norepinephrine uptake have been found to reflect impairment in adrenergic nerve function that has influenced the cardiac outcome of patients with heart failure. The aim of this study was to explore the cardiac neuronal function by using 123I-metaiodobenzylguanidine (MIBG) scintigraphy in patients with acute myocarditis. METHODS: We studied 15 patients (age range 42 +/- 10 yr) with clinical, biological, electrocardiographic and radionuclide left ventricular ejection fraction (LVEF) (41% +/- 7%) data indicating myocarditis and 10 normal subjects (age range 36 +/- 7 yr, mean radionuclide LVEF 69% +/- 8%, p < 0.05). Fourteen patients had positive histologic findings of myocarditis and 1 had nonspecific histological data. All patients underwent planar cardiac imaging after intravenous injection of 185 MBq 123I-MIBG and right ventricular biopsy within 7 days. A chest anterior view was acquired 4 hr later. Heart-to-mediastinum ratio activity was measured, as previously described in our laboratory. RESULTS: Significant impairment of cardiac neuronal uptake of MIBG was observed and based on a reduction of heart-to-mediastinum ratio (148% +/- 16% versus 234% +/- 36%, p < 0.05). A significant correlation was observed between LVEF and MIBG uptake in patients (y = 1.58x +/- 83.7, r = 0.72, p < 0.01). CONCLUSION: Acute myocarditis is associated with an injury of the cardiac adrenergic neuronal function. In addition to the inflammatory injury of the myocytes, the impairment of adrenergic function may be involved in the cardiac pump failure induced by myocarditis.

[Current status of post-infarction cardiogenic shock and revascularization]. / Choc cardiogénique de l'infarctus du myocarde et revascularisation. Données actuelles.

Despite improved management of myocardial infarction, the incidence of cardiogenic shock remains constant at about 7.5% of cases. Patients who develop cardiogenic shock are older and much more often women. The site of infarction is usually anterior, transmural with greater left ventricular systolic dysfunction and a higher increase in cardiac enzymes. Diastolic intra-aortic balloon pumping is the usual treatment but survival only seems to be increased in patients undergoing revascularisation. Surgical revascularisation and circulatory assist devices have been proposed but patient selection in these studies was biased and optimised the results. The effect of thrombolysis was analysed in a subgroup of patients in the GISSI study. The 30 day mortality was 69.9% in 146 patients treated by streptokinase and 70.1% in patients given placebo. Thrombolysis only benefitted patients with an anterior myocardial infarction aged less than 65 and treated within 6 hours of the onset of pain. Although there are no randomised studies of the value of angioplasty in patients in cardiogenic shock. Several uncontrolled series suggest that this type of procedure reduces short and long-term mortality. However, the prognosis is not as good when unselected consecutive groups of patients are studied in whom infarction is complicated by cardiogenic shock immediately and who undergo "aggressive" revascularisation: 72% of patients die in hospital and there are few long-term survivors. The prognosis appears to be particularly poor in patients over 70 years of age with a previous history of myocardial infarction or who undergo prolonged pre-hospital cardiopulmonary resuscitation. However, the implantation of coronary stents associated with circulatory assist devices seems to improve the prognosis of infarction complicated by cardiogenic shock by ensuring adequate coronary flow and reducing the risk of reocclusion.

[Leiomyosarcoma of the heart and great vessels]. / Les léiomyosarcomes du coeur et des gros vaisseaux.

Leiomyosarcomas (LMS) of the heart are exceptional primary malignant tumours with a catastrophic prognosis and a mean survival measured in months. Extensive radical surgical resection clearly remains the most appropriate treatment. We report three cases observed over a 3-year period, consisting of an LMS of the inferior vena cava, an LMS of the pulmonary artery trunk and an LMS of the left atrium. The first case was treated by radical resection and reconstruction by autologous vein graft of the cavorenal junction, the second case was treated by extensive resection and prosthetic reconstruction of the pulmonary artery bifurcation and the third case was treated by a first radical resection of the left atrium, requiring total cardiectomy and orthotopic heart transplantation for local recurrence at the sixth month. The survical was significantly improved compared to other treatment options (chemotherapy, radiotherapy). The first patient is still alive without recurrence at two years; the second died 12.5 months after the surgical procedure and the medium-term follow-up of the transplanted patient revealed cerebral and hepatic metastases nine months after transplantation. The authors review the literature concerning these extremely rare malignant tumours. Recent progress of diagnostic investigations, such as spiral CT with reconstruction, MRI, positron emission tomography (PET), are now able to establish the diagnosis more rapidly and therefore allow more radical surgical resection. This resection, possibly combined with venous reconstruction, must be associated with adjuvant therapies. Heart transplantation should be considered among the treatment options for leiomyosarcomas of the heart, in order to improve the poor prognosis of these lesions affections a young population.

[Ehlers-Danlos disease revealed during pregnancy through the diagnosis of aortic dissection]. / Maladie d'Ehlers-Danlos révélée lors d'une grossesse par une dissection aortique. A propos d'un cas.

Dissection of the aorta is a serious condition but rare in young women, and occurring during the 3rd trimester of pregnancy. The main risk factors are hypertension and diseases of the connective tissue or of collagen (Marfan's syndrome and Ehlers-Danlos disease). The authors report a case of dissection of the aorta managed in a pluridisciplinary manner by the anaesthetists, cardiologists, obstetricians and cardiothoracic surgeons, which resulted in a favourable outcome for both mother and baby. The diagnosis of Ehlers-Danlos disease was made from the onset and, over a period of 10 years with CT scan and annual echocardiographic follow-up, total replacement of the supra-coronary aorta was performed in several stages.

Aorto-pulmonary fistula: a rare event in the evolution of a dissecting aneurysm of the thoracic aorta.

The natural history of a thoracic aneurysm is usually towards the dissection or free rupture; rarely an aorto-pulmonary fistula can complicate this lesion. We present two cases of Aorto-pulmonary fistula as acute complication of an aneurysm of thoracic aorta; the etiopathology seem to be related to the same mechanism: a dissecting aneurysm of the ascending aorta leading to a secondary fistulation in the main pulmonary artery. In our two cases the diagnosis was suggested by clinical findings and by Doppler-echocardiography. Both patients were managed surgically with success and both survived.

[Effects of temporary dual-chamber cardiac pacing in refractory cardiac failure]. / Effets de la stimulation cardiaque double chambre temporaire dans l'insuffisance cardiaque réfractaire.

The authors studied 18 patients (15 men, 3 women) with an average age of 67 +/- 8 years with refractory cardiac failure. In order to determine the potential of pacing to raise cardiac output in severe cardiac failure. The average ejection fraction was 26 +/- 6.5%. All patients were in sinus rhythm:resting cardiac output was 3.35 l/min. Two temporary pacing catheters were positioned in the right atrium and at the apex of the right ventricle for dual-chamber mode pacing triggered by the spontaneous P waves. Changes in cardiac output were measured by Doppler echocardiography at different values of atrioventricular delay. Patients were considered to be responders if their cardiac outputs rose by 15%. In 7 patients meeting this criterion, the average increase in cardiac output was 27% (2.99 +/- 0.7 to 3.81 +/- 0.86 l/mn; p < 0.01); all had dilated cardiomyopathies with left bundle branch block and the optimal AV delay was 103 +/- 21 ms (80-140 ms); the duration of diastolic filling increased from 212 +/- 98 to 292 +/- 116 ms (p = 0.02). In the non-responding group (11 patients with an increase of cardiac output of only 3.6 +/- 0.09 to 3.9 +/- 0.92 l/mn; p < 0.01), the underlying disease process was mainly ischaemic. Two predictive factors of efficacy of dual-chamber pacing were identified: a short ventricular filling period (29 +/- 8% of the RR interval in the responders vs 44 +/- 9% in the non-responders; p < 0.01) and the presence of 1st degree atrioventricular block. Dual-chamber pacing could be a valuable method of increasing resting cardiac outputs in a selected group of patients with severe, refractory, cardiac failure.