A new approach for the evaluation of the severity of coarctation of the aorta using Doppler velocity index and effective orifice area: in vitro validation and clinical implications.

Early detection and accurate estimation of COA severity are the most important predictors of successful long-term outcome. However, current clinical parameters used for the evaluation of the severity of COA have several limitations and are flow dependent. The objectives of this study are to evaluate the limitations of current existing parameters for the evaluation of the severity of coarctation of the aorta (COA) and suggest two new parameters: COA Doppler velocity index and COA effective orifice area. Three different severities of COAs were tested in a mock flow circulation model under various flow conditions and in the presence of normal and stenotic aortic valves. Catheter trans-COA pressure gradients and Doppler echocardiographic trans-COA pressure gradients were evaluated. COA Doppler velocity index was defined as the ratio of pre-COA to post-COA peak velocities measured by Doppler echocardiography. COA Doppler effective orifice area was determined using continuity equation. The results show that peak-to-peak trans-COA pressure gradient significantly increased with flow rate (from 83% to 85%). Peak Doppler pressure gradient also significantly increased with flow rate (80-85%). A stenotic or bicuspid aortic valve increased peak Doppler pressure gradient by 20-50% for a COA severity of 75%. Both COA Doppler velocity index and COA effective orifice area did not demonstrate significant flow dependence or dependence upon aortic valve condition. As a conclusion, COA Doppler velocity index and COA effective orifice area are flow independent and do not depend on aortic valve conditions. They can, then, more accurately predict the severity of COA.

[Rehabilitation of hypoplastic pulmonary arteries in pulmonary atresia with ventricular septal defect. Medium term results]. / Réhabilitation des artères pulmonaires hypoplasiques dans l'atrésie pulmonaire avec communication interventriculaire. Résultats à moyen terme.

The treatment of pulmonary atresia with ventricular septal defect, hypoplasia of the native pulmonary arteries and major aorto-pulmonary collaterals is controversial with a wide range of options from symptomatic treatment to surgical correction, but usually unifocal surgery. However, the collateral vessels used in the reconstruction are often tortuous and stenosed, resulting in a problematic haemodynamic result. The authors report the results of medico-surgical rehabilitation of the native pulmonary arteries in three stages. Since 1990, patients with an average Nakata index of 27.5 +/- 16.7 mm2/m2 underwent elective surgical connection of the native pulmonary arteries to the right ventricle (1st stage) followed by their rehabilitation with catheter occlusion of the collaterals (2nd stage) with the aim of preparing surgical correction (3rd stage). The first surgical stage at an median age of 5 months (0.1 to 25.2) was complicated by one death. After an average of 2.1 +/- 1 catheterisations, 17 patients underwent surgical correction at an median age of 2.18 (0.6 to 10.3) years with an average Nakata index of 207 +/- 91 mm2/m2. All survived surgery with three late deaths in patients with poor haemodynamic results (right ventricular/left ventricular pressure ratio>80%). After an median follow-up of 9.2 (3.5 to 17) years, the 14 survivors are in NYHA Class I or II with a good haemodynamic result in 10 patients. The authors conclude that despite some late deaths and raised right ventricular and pulmonary artery pressures in a minority of patients, the rehabilitation of the pulmonary arteries is an effective management of this cardiopathy.

Outcome of acute fulminant myocarditis in children.

OBJECTIVES: To highlight clinical features and outcome of acute fulminant myocarditis (AFM) in children. METHODS: Diagnostic criteria were (1) the presence of severe and acute heart failure; (2) left ventricular dysfunction on echocardiography; (3) recent history of viral illness; and (4) no history of cardiomyopathy. RESULTS: Eleven children were included between 1998 and 2003, at a median age of 1 (0 to 9) year. Their mean left ventricular ejection fraction (LVEF) was 22 (SD 9)% at presentation. A virus was identified in five patients: human parvovirus B19 (n = 2), Epstein-Barr (n = 1), varicella zoster (n = 1), and coxsackie (n = 1). The median intensive care unit course was 13 (2-34) days. Intravenous inotropic support was required by nine patients and eight were mechanically ventilated. All patients received corticosteroid, associated with intravenous immunoglobulin in seven. Five patients experienced cardiocirculatory arrest that was successfully resuscitated in four. At a median follow up of 58.7 (33.8-83.1) months, the 10 survivors are asymptomatic with normalised LVEF. CONCLUSION: Despite a severe presentation, the outcome of AFM is favourable. Aggressive symptomatic management is warranted and heart transplantation should be considered only when maximal supportive therapy does not lead to improvement.

[Medico-surgical treatment of occlusion of the left pulmonary artery by extension of ductal tissue (pulmonary coarctation)]. / Traitement médico-chirurgical de l'occlusion de l'artère pulmonaire gauche par extension de tissu ductal (coarctation pulmonaire).

The authors report the results of surgical treatment completed by interventional catheterisation of occlusion of the left pulmonary artery by extension of ductal tissue. Since 2001, 7 patients operated for occlusive coarctation of the left pulmonary artery at an average age of 11 months (3 to 37 months) had a restenosis. The cardiac malformation was pulmonary atresia with ventricular septal defect (N=4), tetralofy of Fallot (N=2) and critical pulmonary valvular stenosis (N=1). Pulmonary artery surgery consisted of resection anastomosis in 4 cases and a plasty in 3 cases. A primary angioplasty was performed 5 to 170 months (median 12 months) later, at an average age and weight of 3.4 years (0.7 to 16.9 years) and 14 Kg (8 to 52 Kg) with implantation of 3 stents. The median diameter increased from 5 mm (1 to 9 mm) to 10 mm (6 to 16 mm). Tc-99m scintigraphy showed an increase in mean left pulmonary perfusion from 9% (6 to 28%) to 28% (18 to 42%). Secondarily, 3 repeat angioplasties were necessary with a total of 6 stents implanted in 7 patients. After an average of 2.9 years (0.8 to 6.3 years) follow-up, the patients were asymptomatic with normal right ventricular pressures and a mean left pulmonary perfusion of 33% (24 to 45%). The authors conclude that the treatment of left pulmonary occlusion by coarctation requires a medico-surgical approach in which angioplasty and stenting complete successfully the surgical revascularisation.

[Results of surgery for coarctation of the aorta in children under one year of age]. / Résultats de la chirurgie de la coarctation aortique chez l'enfant de moins de 1 an.

The aim of this study was to determine the surgical results of repair of coarcta tion of the aorta in children under 1 year of age and to assess the risk of recoarctation. Between 1984 and 2004, 206 children under 1 were operated for coarctation of the aorta. Three groups of patients were identified: Group I (isolated coarctation, N=99), Group II (associated ventricular septal defect, N=63), and Group III (complex congenital heart disease, N=44). The transverse aorta was hypoplastic in 51% of cases. Uni- and multivariate analysis of the risk factors for mortality and recoarctation was performed. An extended resection anastomosis was performed in 206 patients. Reconstruction of the transverse aorta was performed in 32 cases. The hospital mortality was 3.9% (N=8). The presence of a complex intracardiac anomaly was a risk factor for mortality on multivariate analysis (p=0.023). In univariate analysis, a two-step management of patients in Group III was a significant risk factor for mortality (p=0.036). Thirty patients (14.6%) had recoarctation (gradient > 20 mmHg, follow-up 40 +/- 44 months). On multivariate analysis the severity of the immediate postoperative gradient was the only risk factor for recoarctation. The authors conclude that surgery for coarctation of the aorta is associated with excellent results in children under 1 year of age. The prognosis is related to the presence of associated severe cardiac malformations. Survival seems to be better if treatment is carried out in a single stage in this group. The risk of recoarctation is related to the immediate postoperative transisthmic gradient which justifies extensive repair of hypoplastic aortic arches.

[Extracorporeal assistance in paediatric intensive care]. / Assistance extracorporelle en réanimation pédiatrique.

OBJECTIVE: For 3 years we have used extracorporeal assistance in intensive care frequently. This study evaluates our results. METHOD: We studied the patient records for those treated between January 2002 and January 2005. The method used, indications and morbidity/mortality were analysed. RESULTS: We performed 24 circulatory assistance procedures in 20 patients (median age: 5 months), arterio-venous with oxygenation (n=18), veino-venous with oxygenation (n=3) or biventricular (n=3). The indications were post cardiotomy cardio-respiratory failure (Group I; n=20, 16 patients), pure respiratory failure (Group II: n=1), or pre-transplant/recovery (Group III: n=3). Five procedures (4 from group I and 1 from group III) required cardiac massage (no fatalities). The average duration of assistance was 7 +/- 6 days (2 to 20 days). Treatment was successfully discontinued in sixteen patients 80%), one of them thanks to heart transplant. Four (20%) died during assistance. The morbidity essentially consisted of further surgery for haemostasis, multiple transfusions, and infections. Three patients (15%) died later (1 at 17 months after discontinuation) from complications unrelated to the assistance. No neurological sequelae were noted in the survivors. CONCLUSION: These results confirm the usefulness of circulatory assistance when medical treatment has failed, particularly in the post-operative period of paediatric cardiac surgery or while awaiting transplantation.

[Efficacy of angioplasty of native coarctations of the aorta of the big infant and of the adult with systematic endoprosthesis implantation]. / Efficacité de l'angioplastie des coarctations natives de l'aorte du grand enfant et de l'adulte avec implantation systématique d'endoprothèse.

The angioplasty of native coarcatations of the aorta remains a controversial treatment due to recurrences and the potential risk of aneurysm or of descending aorta dissection during catheterization. The interest of a systematic implantation of an endoprothesis is poorly documented. We report our experience in a small series of 3 patients aged from 7, 28 to 52 years at the moment of the angioplasty of their native aortic coarctation. In all the three cases it corresponded to a "membranous" type, localized a the level of the isthmus without hypoplasia of the aortic arch. All presented a refractory hypertension. One patient presented an intermittent claudication related to a low perfusion of lower limbs. The angioplasty was performed with BIB balloon, associated at the same time with the implantation of a Palmaz P308 stent in two cases and Genesis PG2910P in the last patient. The efficacy was immediate in all the 3 cases with stopping antihypertensive drugs at the very day of the procedure. The immediate results were complicated by a bilateral hematoma of the scarpa in a context of excessive anticoagulation in one patient requiring blood tranfusion. After a follow-up of one, 12 and 21 months, all the 3 patients are asymptomatic without any significant residual hypertension. The control scan of the infant confirmed the absence of re-coarctation. In conclusion, the angioplasty followed by systematic implantation of an endoprosthesis is a safe and effective technique for treating simple forms of native coarctations of the aortic isthmus. It can be proposed as a first line treatment for big infants and adults affected by localized types.

[Results of the surgery of the truncus arteriosus: present implications]. / Résultats de la chirurgie du tronc artériel commun: implications actuelles.

We report our results of truncus arteriosus surgical reparation in a period of 10 years. From January 1993 to November 2003, 17 patients were operated with a median age and weight of 66 days and 3.5 kg respectively. According to the Van Praagh classification there were 13 cases of type A1, 2 cases of type A2 and 2 cases of A4. The connection between the right ventricle and the pulmonary artery was performed by a homograft (n = 12), a Contegra tube (n = 1) or a Barbero-Marcial intervention (n = 4). In one case, a plasty of the truncus was performed. Patients were classified in two groups: group A for those operated between 1993 and 1997 (n = 8) and group B for those operated after 1997 (n = 9). Five patients died (29%) in the post-operative period, 4 in the group A (50%) and one in group B (11%). The mean duration of intensive care stay was 12.2 (+ 7.4) days. Statistical analysis did not reveal any difference between both groups, especially concerning post-operative treatment or prognosis. In the multivariate analysis, an age below 30 days was a risk factor of post-operative death (OR = 16.5, 95% CI = 1.09 - 250; p = 0.043). After a mean follow-up of 3.9 (+ 3.5) years, 2 patients required a redo intervention for replacement of the pulmonary artery homograft. All 12 suvivors are asymptomatic without any pulmonary hypertension. In conclusion, the results of surgical reparation of the truncus arteriosus seem to improve with experience. According to recent progresses in surgery and intensive care, the intervention can be scheduled beyond the neonatal period without additive risk and with potentially less consecutive redo interventions.

[Case history of mitochondrial cytopathy with cardiac expression]. / A propos d'une cytopathie mitochondriale d'expression cardiaque.

Childhood dilated cardiomyopathies comprise a wide aetiological spectrum for which the prognosis and treatment sometimes vary considerably. We report the case of a patient affected by a rare form of mitochondrial cardiomyopathy in whom the diagnosis of acute myocarditis had initially been made. The progression was fatal even though the patient was awaiting a cardiac transplant. Beyond the difficulties of diagnosis and treatment of this pathology, this clinical case underlines the significance of an early aetiological diagnosis based on the results of an endo-myocardial biopsy. Cardiac transplantation should be envisaged for this type of patient based not only on the clinical status but equally by taking account of the prognosis of this disease for which deterioration can sometimes be very rapid.

[Dextrocardia with situ solitus, ventricular loop, double outlet hypoplastic right ventricle and L-malposition of the great arteries. Description and surgical treatment of a rare and complex cardiopathy]. / Dextrocardie avec situs solitus, coeur croisé, ventricule droit hypoplasique à double issue et L-malposition des gros vaisseaux. Description et traitement chirurgical d'une cardiopathie rare et complexe.

Despite the cardiac surgery improvements allowing the correction of the majority of congenital heart diseases with ventricle-great vessels discontinuity, some abnormalities increase the risk of bi-ventricular reparation. We herein report the case of a patient presenting a rare form of double outlet right ventricle with a ventricular loop, with moderate right ventricle hypoplasia. L-malposition of great vessels and pulmonary artery stenosis, and for whom we opted for a palliative surgical treatment including a systemic-pulmonary anastomosis followed by a upper right bi-directional cavo-pulmonary derivation. The last surgery was followed by recurrent right pleural effusions disappearing after the embolization of the systemic-pulmonary anastomosis by catheterism as it probably obstructed the draining of the cavo-pulmonary anastomosis. The relevance of this clinical case reported is, firstly the description of this ventricle loop resulted from a marked ventricular malposition which is a rare heart disease, and secondly the discussion about the surgical treatment, especially about the choice between palliative and curative surgery. Only comparative studies on long term morbidity and mortality between the bi-ventricular reparation and mono-ventricular palliation will allow the selection of the most appropriate surgical treatment.

Pulmonary atresia with ventricular septal defect, extremely hypoplastic pulmonary arteries, major aorto-pulmonary collaterals.

OBJECTIVE: Among 63 patients with pulmonary atresia and ventricular septal defect (VSD), 10 patients with extreme hypoplasia of the pulmonary arteries (PA) (mean Nakata index 20.6 mm(2)/m(2)), but with confluent arteries and a diminutive main PA, and major aorto-pulmonary collaterals (MAPCAS), have been submitted to a 'rehabilitation' of the PA with several stages: (i) connection between RV and PAs, (ii) interventional catheterizations, (iii) complete correction with or without unifocalisation. We report here the results of this approach. METHODS: The RV-PA connection was direct (nine cases) or with an homograft conduit (one case), done under normothermic cardiopulmonary by-pass in patients aged 4.9 months (range 0.1-18 months). Subsequently, six underwent interventional catheterizations (dilations and stents in the PA, MAPCAS occlusion by coils). Complete correction was done in seven patients (mean age 30 months, range 8-49). One patient is awaiting correction. RESULTS: One patient died after the first stage. All patients having had the third stage had a satisfactory development of the PA, had a complete closure of the VSD and a satisfactory reconstruction of the PA bifurcation. There was one death of severe pulmonary infection 6 months after repair. All other patients have been followed by catheterization and/or echocardiograms. With a follow-up of 83+/-65 months, all patients are improved, 50% have no cardiac medications, none has residual shunt, RV/LV pressure ratio is 0.6 (range 0.3-1). CONCLUSIONS: The strategy of 'rehabilitation' of PA allowing: (i) antegrade flow in the PA, (ii) interventional catheterizations, (iii) growth of the PA with possible angiogenesis, (iv) complete correction, is a logical approach to be undertaken in the young patient and is a valid alternative to strategies relying more on MAPCAS for pulmonary vascular supply. The therapeutic sequences depend upon the individual anatomy.

[Rehabilitation of hypoplastic pulmonary arteries and anatomic correction of pulmonary atresia with interventricular communication]. / Réhabilitation des artères pulmonaires hypoplasiques et correction anatomique des atrésies pulmonaires avec communication interventriculaire.

Conventional treatment of pulmonary atresia with ventricular septal defect (VSD), hypoplastic pulmonary arteries (PA) and major aorto-pulmonary collaterals (MAPCAs) is controversial: from symptomatic and palliative treatment for some authors to surgery with unifocalisation of collaterals for others. These treatments never use native pulmonary arteries as only source of pulmonary flow, but create "neo-pulmonary arteries". Nine cases of pulmonary atresia with VSD, hypoplastic PA and MAPCAs were treated by rehabilitation of native PA through a staged approach: 1) surgical neonatal connection between right ventricule (RV) and hypoplastic PA, 2) evaluation and interventionnal catheterism with angioplasty of PA stenosis and closure of collaterals, 3) complete surgical correction with reconstruction of right outflow track and PA and closure of VSD. After first surgical stage of RV-PA connection at the mean age of 4.8 months (+/- 5.6 months), 8 patients were alive and underwent 22 cardiac catheterisms (mean of 2.7 per patient), with angioplasty of PA, and occlusion of MAPCAs in 6 and 2 patients respectively. Seven patients underwent complete anatomical correction at the mean age of 28.8 months (+/- 17.7 months) with one late death. The 6 remaining patients had encouraging hemodynamic status (RV pressure/LV pressure ratio at 0.6 +/- 0.26; mean left and right distal pulmonary pressure at 15.2 mmHg (+/- 9.1 mmHg)), and good functionnal status (3 in NYHA functionnal class 1, and 3 in class 2), for a mean follow-up of 79.5 months (+/- 41.4 months). One patient had reoperation on right outflow track stenosis, 6 years after correction. This small series enhances the feasibility of a staged approach with rehabilitation of small PA, allowing complete surgical correction with the native PA with good hemodynamic and functional results in pulmonary atresia, with VSD, hypoplastic PA and MAPCAs.

[Short and medium-term results of the Norwood intervention (first stage)]. / Résultats à court et moyen termes de l'intervention de Norwood (première étape).

We report the short and mid-term results of the Norwood procedure (Stage one) in 20 patients with hypoplastic left heart syndrome or univentricular heart with aortic obstruction. Seven patients were prenatally diagnosed. Preoperatively there was obstruction to pulmonary venous return in 6 cases, a mild to moderate tricuspid regurgitation in 6 cases, and 11 patients were supported by mechanical ventilation with multiorgan failure in 5 cases. The surgery was performed under cardiopulmonary bypass at a mean age and weight of 12.9 days and 3 kg, respectively. Nine patients (45%) died within 30 days postoperatively, whereas 5 had delayed sternal closure. The mean duration of mechanical ventilation and ICU stay were 5.7 and 11 days, respectively. Two patients were reoperated for bronchial compression and tracheotomy. Systemic venous thrombosis occurred in 5 patients. In multivariate analysis, an older age at surgery was correlated with postoperative hospital death (p = 0.03). Among the 11 patients discharged home after Stage one procedure, 5 patients underwent balloon dilation for recoarctation and one patient died at home. A bidirectional cavopulmonary anastomosis was performed in 8 patients at a mean age of 0.76 year, with one postoperative death. After a mean follow-up of 1 year (+/- 1.97 years), the 9 remaining patients are all in NYHA class I, at a mean age of 2.2 years. Their mean transcutaneous saturation is 81%. The Norwood procedure (Stage one) is associated with high hospital mortality. However, the functional status of the survivors is correct, like in patients with other type of univentricular hearts. Moreover, although the causes of death in our patients are often not clarified, other studies show that the leading causes of deaths in our patients are often not clarified, other studies show that the leading causes of death in those patients are correctable. In conclusion, the option of a Norwood procedure (Stage one) should be proposed in patients with hypoplastic left heart syndrome (or variant).

Plasma TAFI antigen variations in healthy subjects.

The Thrombin Activatable Fibrinolysis Inhibitor (TAFI) is a recently described inhibitor of fibrinolysis. The physiological variations of plasma TAFI antigen are not well known. We studied TAFI antigen values in healthy populations with a commercially available kit from Milan Analytica (Switzerland). Broad range of TAFI antigen values (from 41% to 259%) was found in a population of 249 healthy individuals. Gender as well as pregnancy did not influence mean values of TAFI antigen. There was a positive correlation between TAFI antigen and age in female (r = 0.28; p <0.05) but not in male populations. Mean TAFI antigen value of a black African male group [mean +/- SD (range): 87 +/- 23 (39-144%)] was significantly lower than the one of age matched Caucasian men [114 +/- 34 (52-259%)] (p <0.0001). TAFI antigen values were very stable within individuals, they did not significantly vary on day time or at several months period. Thus, in contrast to large inter-individual variations, TAFI antigen levels are quite stable within individuals.

Acute myopathy of intensive care in a child after heart transplantation.

PURPOSE: Acute myopathy of intensive care has been described infrequently in children and never after organ transplantation. We report a case of acute myopathy of intensive care in a child after heart transplantation. CLINICAL FEATURES: An 11-yr-old girl, with no previous medical history, developed acute cardiomyopathy leading to cardiac shock. Family history revealed four cases of unidentified myopathy and/or cardiomyopathy. Preoperatively, while muscle biopsy was near normal, myocardial biopsy revealed non specific mitochondrial disorders. A few days after heart transplantation, she developed acute hypotonia and flaccid quadriplegia, consistent with the diagnosis of acute myopathy of intensive care. Nerve conduction studies were normal, electromyography showed myopathic changes and a new muscle biopsy from quadriceps femoris showed severe loss of myosin filaments and ATPase activity in type 2 fibres. A large laboratory screening failed to demonstrate a metabolic disease or a known myopathy. Muscle strength recovered progressively in three weeks allowing home discharge. A few months later, she was free of symptoms and muscle biopsy showed full histopathological recovery. CONCLUSION: Acute myopathy of intensive care can occur in children after heart transplantation. It should be suspected in the presence of muscle weakness and difficulty in weaning from ventilatory support. Electromyography confirmed a myogenic process and muscle biopsy allowed diagnosis. Full clinical and histopathological recovery usually occur within three weeks.