Cobalamin c deficiency associated with antifactor h antibody-associated hemolytic uremic syndrome in a young adult.

BACKGROUND: Thrombotic microangiopathy (TMA) syndromes are characterized by the association of hemolytic anemia, thrombocytopenia and organ injury due to arteriolar and capillary thrombosis. CASE PRESENTATION: We report the first case of adult onset cobalamin C (Cbl C) disease associated with anti-factor H antibody-associated hemolytic uremic syndrome (HUS). A 19-year-old woman was admitted to the nephrology department owing to acute kidney failure, proteinuria, and hemolytic anemia with schizocytes. TMA was diagnosed and plasma exchanges were started in emergency. Exhaustive analyses showed 1) circulating anti factor H antibody and 2) hyperhomocysteinemia, hypomethioninemia and high levels of methylmalonic aciduria pointing towards Clb C disease. Cbl C disease has been confirmed by methylmalonic aciduria and homocystinuria type C protein gene sequencing revealing two heterozygous pathogenic variants. The kidney biopsy showed 1) intraglomerular and intravascular thrombi 2) noticeable thickening of the capillary wall with a duplication aspect of the glomerular basement membrane and a glomerular capillary wall IgM associated with Cbl C disease related TMA. We initiated treatment including hydroxycobalamin, folinic acid, betaine and levocarnitine and Eculizumab. Rituximab infusions were performed allowing a high decrease in anti-factor H antibody rate. Six month after the disease onset, Eculizumab was weaning and vitaminotherapy continued. Outcome was favorable with a dramatic improvement in kidney function. CONCLUSION: TMA with renal involvement can have a complex combination of risk factors including anti-FH autoantibody in the presence of cblC deficiency.

Multidisciplinary total eradication therapy (TET) in men with newly diagnosed oligometastatic prostate cancer.

To evaluate the outcomes of total eradication therapy (TET), designed to eradicate all sites of visible cancer and micrometastases, in men with newly diagnosed oligometastatic prostate cancer (OMPCa). Men with ≤ 5 sites of metastases were enrolled in a prospective registry study, underwent neoadjuvant chemohormonal therapy, followed by radical prostatectomy, adjuvant radiation (RT) to prostate bed/pelvis, stereotactic body radiation therapy (SBRT) to oligometastases, and adjuvant hormonal therapy (HT). When possible, the prostate-specific membrane antigen targeted 18F-DCFPyL PET/CT (18F-DCFPyL) scan was obtained, and abiraterone was added to neoadjuvant HT. Twelve men, median 55 years, ECOG 0, median PSA 14.7 ng/dL, clinical stages M0-1/12 (8%), M1a-3/12 (25%) and M1b-8/12 (67%), were treated. 18F-DCFPyL scan was utilized in 58% of cases. Therapies included prostatectomy 12/12 (100%), neoadjuvant [docetaxel 11/12 (92%), LHRH agonist 12/12 (100%), abiraterone + prednisone 6/12 (50%)], adjuvant radiation [RT 2/12 (17%), RT + SBRT 4/12 (33%), SBRT 6/12 (50%)], and LHRH agonist 12/12 (100%)]. 2/5 (40%) initial patients developed neutropenic fever (NF), while 0/6 (0%) subsequent patients given modified docetaxel dosing developed NF. Otherwise, TET resulted in no additive toxicities. Median follow-up was 48.8 months. Overall survival was 12/12 (100%). 1-, 2-, and 3-year undetectable PSA's were 12/12 (100%), 10/12 (83%) and 8/12 (67%), respectively. Median time to biochemical recurrence was not reached. The outcomes suggest TET in men with newly diagnosed OMPCa is safe, does not appear to cause additive toxicities, and may result in an extended interval of undetectable PSA.

[Bevacizumab/irinotecan. An active treatment for recurrent high grade gliomas: preliminary results of an ANOCEF Multicenter Study]. / Bevacizumab/irinotecan. Un nouveau traitement actif dans les gliomes de haut grade récidivants: résultats préliminaires d'une étude multicentrique de l'Anocef.

RATIONALE: Second-line chemotherapy is disappointing in recurrent high-grade gliomas. Dramatic responses in recurrent high-grade gliomas have been reported in a recent monocentric trial with a novel association combining bevacizumab (anti-VEGF monoclonal antibody agent) and irinitecan. OBJECTIVE: To report the experience of the ANOCEF group (French speaking neuro-oncology association) using the bevacizumab-irinotecan combination in recurrent high-grade gliomas. METHODS: Eight centers were involved in this retrospective multicenter study. Bevacizumab-irinotecan was delivered as previously described in a compassional setting to non-selected patients suffering from a high-grade glioma (WHO grade III and IV). Response rate at two months of the onset of the treatment was analyzed using the Macdonald criteria. The toxicity profile of the treatment was also investigated. RESULTS: From 2006 to 2007, 77 patients were treated (median age: 52 years; median Karnofsky score: 70) for a recurrent high-grade glioma (49 grade IV, 28 grade III). At two months, the response rates were objective response=36% (54% in grade III and 27% in grade IV); stable disease=39%; progressive disease=13%; patients not evaluable because of a rapid fatal clinical deterioration=12%. Improvement was noted in 49% of patients. Among the main toxicities, we noted; intratumoral hemorrage (n=5 with spontaneous regression in three) and thromboembolic complications including venous thrombophlebitis (n=4), pulmonary embolism (n=2), myocardial infarction (n=1), grade III-IV hematotoxicity (n=2), reversible leukoencephalopathy (n=1). CONCLUSION: This retrospective multicenter study adds further arguments in favor of the promising results of this new combination and its potential rapidity of action in recurrent high-grade gliomas. Antiangiogenic agents expose the patients to a well-known risk of thromboembolic and hemorragic complications, necessitating careful follow-up and patient selection in light of the cardiovascular contraindications.

End-of-radiation PSA as a novel prognostic factor in patients undergoing definitive radiation and androgen deprivation therapy for prostate cancer.

BACKGROUND: In men undergoing definitive radiation for prostate cancer, it is unclear whether early biochemical response can provide additional prognostic value beyond pre-treatment risk stratification. METHODS: Prostate cancer patients consecutively treated with definitive radiation at our institution by a single provider from 1993 to 2006 and who had an end-of-radiation (EOR) PSA (n=688, median follow-up 11.2 years). We analyzed the association of an EOR PSA level, obtained during the last week of radiation, with survival outcomes. Multivariable-adjusted cox proportional hazards models were constructed to assess associations between a detectable EOR PSA (defined as ⩾0.1 ng ml-1) and biochemical failure-free survival (BFFS), metastasis-free survival (MFS), prostate cancer-specific survival (PCSS) and overall survival (OS). Kaplan-Meier survival curves were constructed, with stratification by EOR PSA. RESULTS: At the end of radiation, the PSA level was undetectable in 30% of patients. Men with a detectable EOR PSA experienced inferior 10-year BFFS (49.7% versus 64.4%, P<0.001), 10-year MFS (84.8% versus 92.0%, P=0.003), 10-year PCSS (94.3% versus 98.2%, P=0.007) and 10-year OS (75.8% versus 82.5%, P=0.01), as compared to men with an undetectable EOR PSA. Among National Comprehensive Care Network (NCCN) intermediate- and high-risk men who were treated with definitive radiation and androgen deprivation therapy (ADT), a detectable EOR PSA was more strongly associated with PCSS than initial NCCN risk level (EOR PSA: HR 5.89, 95% CI 2.37-14.65, P<0.001; NCCN risk level: HR 2.01, 95% CI 0.74-5.42, P=0.168). Main study limitations are retrospective study design and associated biases. CONCLUSIONS: EOR PSA was significantly associated with survival endpoints in men who received treatment with definitive radiation and ADT. Whether the EOR PSA can be used to modulate treatment intensity merits further investigation.

Early and long-term (one-year) effects of the association of aspirin and oral anticoagulant on thrombi and morbidity after replacement of the mitral valve with the St. Jude medical prosthesis: a clinical and transesophageal echocardiographic study.

OBJECTIVES: The aim of the study was to test the value of low dose aspirin associated with standard oral anticoagulants (OAC) after mechanical mitral valve replacement (MMRV) to reduce strands, thrombi and thromboembolic events. BACKGROUND: Strands and thrombi are thought to increase the risk of embolic events after MMVR, particularly in the immediate postoperative period. METHODS: Two hundred twenty-nine patients were prospectively recruited: 109 patients (group A+) were randomly assigned to aspirin (200 mg per day) with OAC and 120 patients (group A-) to OAC alone (international normalized ratio 2.5 to 3.5). All patients were subjected to multiplane transesophageal echocardiography at nine days and five months and were followed up for one year. RESULTS: At nine days and five months, there was a high and comparable incidence of strands in the two groups (group A+: 44%, 58%; group A-: 49%, 63%). However, the incidence of nonobstructive periprosthetic valve thrombi was significantly lower in group A+ at 9 days: 5% versus 13%, p = 0.03. Total thromboembolic events were reduced in group A+ (9% vs. 25%, p = 0.004) although there was an increased incidence of gastrointestinal hemorrhage (7% vs. 0%). Overall mortality was 9% in group A+ and 4% in group A-. Valve-related events were similar in both groups. Early thrombi, but not strands, were associated with higher morbidity, especially thromboembolic events (30% vs. 13%, p = 0.003). CONCLUSIONS: One year after MMVR, the association of aspirin with OAC reduced thrombi and thromboembolic events, but not morbidity, due to an increase in hemorrhagic complications.

Fluoxetine-induced change in rat brain expression of brain-derived neurotrophic factor varies depending on length of treatment.

Recent studies indicate that brain-derived neurotrophic factor (BDNF) may be implicated in the clinical action of antidepressant drugs. Repeated (2-3 weeks) administration of antidepressant drugs increases BDNF gene expression. The onset of this response as well as concomitant effects on the corresponding BDNF protein is however, unclear. The present study investigated the effects of acute and chronic administration of the selective serotonin reuptake inhibitor, fluoxetine (10mg/kg p.o.), upon regional rat brain levels of BDNF mRNA and protein expression. To improve the clinical significance of the study, fluoxetine was administered orally and mRNA and protein levels were determined ex vivo using the techniques of in situ hybridisation histochemistry and immunocytochemistry respectively. Direct measurement of BDNF protein was also carried out using enzyme-linked immunosorbent assay (ELISA). Four days of once daily oral administration of fluoxetine induced decreases in BDNF mRNA (hippocampus, medial habenular and paraventricular thalamic nuclei). Whilst 7 days of treatment showed a non-significant increase in BDNF mRNA, there were marked and region-specific increases following 14 days of treatment. BDNF protein levels remained unaltered until 21 days of fluoxetine treatment, when the numbers of BDNF immunoreactive cells were increased, reaching significance in the pyramidal cell layer of CA1 and CA3 regions of Ammon's horn (CA1 and CA3) but not in the other sub-regions of the hippocampus. Indicative of the highly regional change within the hippocampus, the ELISA method failed to demonstrate significant up-regulation at 21 days, measuring levels of BDNF protein in the whole hippocampus. In contrast to the detected time dependent and biphasic response of the BDNF gene, activity-regulated, cytoskeletal-associated protein (Arc) mRNA showed a gradual increase during the 14-day course of treatment. The results presented here show that BDNF is expressed differentially depending on length of fluoxetine administration, which could contribute in explaining the slow onset of antidepressant activity observed with selective serotonin reuptake inhibitors.

Value of transesophageal echocardiography combined with computed tomography for assessing repaired type A aortic dissection.

Thirty-two patients with repaired type A aortic dissection were examined by transthoracic echocardiography (TTE) (n = 32), transesophageal echocardiography (TEE) (n = 30), computed tomography (CT) (n = 29), or a combination of all 3, to assess course and complications as a function of the surgical procedure. The mean follow-up period was 55.7 months (range 3 to 132). Surgery consisted of a replacement of the ascending aorta in 25 patients (group 1) with extension to the transverse aorta in 7 (group 2). The transverse diameter of the aorta, the persistence of the false lumen, thrombus formation and flow dynamics in the false lumen were evaluated by TEE. Ten patients (31%) had a dilation in the initial ascending aorta (sinus of Valsalva aneurysm in 6 patients, and a false aneurysm in the other 4). Three of 4 patients with a proximal pseudoaneurysm underwent operation after TEE and CT evaluation. In the descending thoracic aorta, there was good agreement between TEE and CT scan determinations of transverse vessel diameter. Persistence of flow within the false lumen was significantly more frequent in patients with a dilated aorta (p < 0.05), whereas thrombosis was seen more often and false lumen less often in patients with nondilated aorta. No significant differences in vessel status or outcome were observed between the 2 groups, although this may have been due to the small size of group 2. TEE is thus a well-tolerated method for postoperative follow-up of type A aortic dissection whatever the type of surgery. For the upper ascending aorta, CT provided sufficient data.

Repair of tricuspid atresia in 100 patients.

One hundred consecutive patients with situs solitus of the atria and tricuspid atresia have undergone surgical repair since 1968. In patients with ventriculoarterial concordance, a nonvalved Dacron conduit or an aortic valve homograft was interposed between the right atrium and right ventricular outlet chamber. In patients with ventriculoarterial discordance, an aortic valve homograft established continuity between the right atrium and pulmonary artery. Among 73 patients with ventriculoarterial concordance, the hospital mortality rate was 11% (eight deaths), and in 27 patients with ventriculoarterial discordance, it was 15% (four deaths). Before 1974, the year of operation was a significant determinant of hospital mortality (p less than 0.001). Thereafter, the hospital mortality declined and is currently 3.7%. In 82 patients with ages ranging from 4 to 16 years, there were six deaths (7.3%) whereas there were six deaths (33.3%) in 18 patients less than 4 or more than 16 years (p less than 0.001). Mode of ventriculoarterial connection and type of repair did not influence significantly the hospital mortality. There were six late deaths due to infection (two), reoperation (two), heart failure (one), and sudden death (one). Regardless of the mode of ventriculoarterial connection, use of a homograft valve produced better results, i.e., more asymptomatic patients (NYHA Class I) (p = 0.0168) and higher postoperative exercise capacity. Postoperative catheterization data and angiocardiographic measurements in patients with ventriculoarterial concordance demonstrated significant advantages with the interposition of a homograft valve between the right atrium and outlet chamber. Of 82 surviving patients, 94% are in NYHA Class I or II.

Aortic valve homografts in the surgical treatment of complex cardiac malformations.

From April of 1968 to March of 1983, the surgical treatment of complex congenital cardiac malformations requiring an extracardiac conduit for their correction was performed with aortic valve homografts or aortic valved homograft conduits sterilized and preserved in our hospital. Our experience concerns 93 patients in whom a total of 103 aortic valve homografts were implanted. Ages of the patients ranged from 7 months to 36 years (mean 11.6 years). The aortic valve homografts were used from the right atrium to the pulmonary arteries or right ventricle (right atrium-dependent conduit), from the venous ventricle to the pulmonary arteries (ventricle-dependent conduit), or in the pulmonary orifice and in the superior and/or inferior venae cavae. There were 25 early and nine late deaths (36.5%), none of them related to the aortic valve homograft. The clinical follow-up of the 59 survivors (1 month to 15 years, mean 4.3 years) evidenced neither dysfunction of the aortic valve homograft nor thromboembolism or hemolysis; 93% of the patients are in New York Heart Association Class I or II. Control cardiac catheterization in 53 patients evidenced a pressure gradient in only 14 ventricle-dependent conduits. In seven patients with serial control catheterizations after 5 to 10 years, the pressure gradient had not increased.

Cancer of the kidney invading the vena cava and heart. Results after 11 years of treatment.

Between October, 1973, and October, 1983, 18 patients with cancer of the kidney or adrenal gland that had invaded the vena cava, and in 11 cases had reached the heart, were operated on by seven surgical teams. The surgical excision in all patients was performed with extracorporeal circulation, circulatory arrest and deep hypothermia. No deaths occurred. If there are no detectable metastases before operation, the 5 year survival rate is 75% as compared to 6 months with medical treatment. This clinical situation is not uncommon, as 3% to 10% of cancers of the kidney invade the inferior vena cava and 40% of them reach the heart. The possibility of curing the cancers with minimal operative risk should prompt a systematic search for venous invasion with any cancer of the kidney.

Intermediate-term results after en bloc double-lung transplantation with bronchial arterial revascularization. Bordeaux Lung and Heart-Lung Transplant Group.

OBJECTIVE: Between May 1990 and January 1994, 18 patients underwent en bloc double-lung transplantation with tracheal anastomosis and bronchial arterial revascularization. Because at that time it was already suggested that chronic ischemia could be a contributing factor in occurrence of obliterative bronchiolitis, the purpose of this study was to evaluate, with a follow-up ranging from 22 to 69 months, the midterm effects of bronchial arterial revascularization on development of obliterative bronchiolitis. RESULTS: Results were assessed according to tracheal healing, functional results, rejection, infection, and incidence of obliterative bronchiolitis. There were no intraoperative deaths or reexplorations for bleeding related to bronchial arterial revascularization, but there were three hospital deaths and five late deaths, two of them related to obliterative bronchiolitis. According to the criteria previously defined, tracheal healing was assessed as grade I, IIa, or IIb in 17 patients and grade IIIa in only one patient. Early angiography (postoperative days 20 to 40) demonstrated a patent graft in 11 of the 14 patients in whom follow-up information was obtained. Ten patients are currently alive with a 43-month mean follow-up. Among the 15 patients surviving more than 1 year, functional results have been excellent except in five in whom obliterative bronchiolitis has developed and who had an early or late graft thrombosis. Furthermore, those patients had a significantly higher incidence of late acute rejection (p < 0.02), cytomegalovirus disease (p < 0.006), and bronchitis episodes (p < 0.0008) than patients free from obliterative bronchiolitis. CONCLUSION: We conclude that besides its immediate beneficial effect on tracheal healing, long-lasting revascularization was, at least in this small series, associated with an absence of obliterative bronchiolitis, thus suggesting but not yet proving the possible role of chronic ischemia in this multifactorial disease.

Endarteritis and false aneurysm complicating aortic coarctation.

We report a tricky case of endocarditis because of the localization, aortic coarctation, and the pathogenic bacteria Actinobacillus actinomycetemcomitans. Furthermore, we underline the leading role of transesophageal echocardiography in the diagnosis of aortic endarteritis. First, aortitis was treated with antibiotics and, second, successfully operated on.

Long-term evaluation of direct repair of traumatic isthmic aortic transection.

Direct repair of traumatic aortic isthmic transection eliminates the late complications of prosthetic graft repair. This study evaluates the long-term fate of direct aortic repair to which little attention has been paid. Among 32 patients operated upon from 1965 to 1987, 27 (84%) underwent direct repair. The tear was circumferential in 15 patients and partial in 12. Multiple traumatic lesions were present in 26 patients, including intracranial injury in 19. Partial cardiopulmonary bypass was used in 15 patients and simple aortic cross-clamping in 12. No paraplegia was observed. There were 4 deaths from associated lesions among the 14 patients operated upon for acute traumatic isthmic transection and no deaths in the others. Among the 23 survivors, 4 were lost to follow-up; the other 19 patients have excellent clinical results. Intravenous digital aortic angiography performed in 14 patients at a mean delay of 5 years 3 months showed excellent aortic reconstruction in all cases. Technically more demanding and faster than a graft interposition, direct repair is recommended as the procedure of choice in the surgical treatment of traumatic isthmic transection, particularly in young patients, the group most at risk from this lesion.

Surgery of post-infarction ventricular septal defect: risk factors for hospital death and long-term results.

From December 1971 to December 1989, 62 patients (pts) 42 males, 20 females, mean age 66 years (yr) 6 months (mth) (range 52-80) were operated upon for post-infarction ventricular septal defect (VSD), (anterior 34, inferior 28). Eight pts (13%), group (G) 1 presented with cardiogenic shock, 19 pts (30.5%), G2 with severe congestive heart failure (CHF); 31 pts (50%), G3 were stable with mild CHF and 4 pts (6.5%), G4 without CHF. Preoperative intra-aortic balloon pumping (IABP) was used in 49 pts (79%). One transplanted pt was excluded from this study. The VSD was closed from 1 day (d) to 5 mth (mean 13 d) after its occurrence. Hospital death (HD) occurred in 23 pts (37.7% +/- 6%). Of 44 incremental risk factors (RF) for HD studied, the preoperative status (PS) was the most significant (P less than 0.01). G1: 87% +/- 12%, G2: 42% +/- 12%, G3: 25.8% +/- 8%, G4: 0%. [table: see text] Non-survivors had a shorter mean delay between VSD occurrence and surgery than survivors: 5.6 +/- 3.7 d vs 18.2 +/- 30 d (P less than 0.05), but this delay was correlated to PS. The follow-up of the 38 early survivors ranges from 2 mth to 14 yr (mean 3 yr, 11 mth); 11 pts died between 45 d and 14 yr. No RF was identified for premature late death. HD included, the actuarial survival rate at 1, 5, 10 yr is: 57% (+/- 7%), 44% (+/- 8%), 30% (+/- 10%), respectively.

Should circulatory arrest with deep hypothermia be revised in aortic arch surgery?

Our experience (January 1982-May 1987) concerns 41 patients, operated upon for aortic dissection (30 patients) or aneurysm (11 patients) using circulatory arrest with deep hypothermia. There were 24 male and 17 female patients (mean age: 55 years 9 months, range 32-73 years). The mean circulatory arrest time in minutes was 41 +/- 3 (mean rectal temperature before circulatory arrest was 18.4 degrees C +/- 0.3 degrees C). Total (24 patients) or partial (16 patients) replacement of the aortic arch was performed. One patient with a sacciform aneurysm had the aortic wall defect closed. Hospital mortality was 22% +/- 7% (9 patients): 8 of 30 patients with aortic dissection (26.6%) and 1 of 11 patients with aortic aneurysm (9%). Neurological complications occurred in 3 patients. These data lead us to prefer circulatory arrest with deep hypothermia as the method of choice for aortic arch surgery. However, when a short circulatory arrest time (less than 30 min) for the repair is foreseeable, mild hypothermia (20 degrees C-24 degrees C) may be preferred. In patients who will not tolerate excessive cardiopulmonary bypass times, expected difficulties with the repair should suggest mild hypothermia and short circulatory arrest in easier cases or moderate hypothermia with brachiocephalic perfusion in the others.

Early and long term results of coronary artery bypass grafts in patients with dialysis dependent renal failure.

OBJECTIVE: Coronary artery disease is the main cause of mortality and morbidity in patients on renal therapy replacement. The aim of this study was to define peri-operative risk and long term results of coronary artery bypass grafts (CABG) in dialysis patients. METHODS: this retrospective study included 82 patients in chronic dialysis who underwent CABG between 1978 and 1997. The mean age was 61+/-10 years (range 28-81 years), 84% of the patients were male and the average duration of dialysis was 57 months (range 1-148 months). Combined procedures were carotid endarterectomy in one case, left ventricular aneurysm resection in one and valvular replacement in 10 (nine aortic and one mitral replacements). The operation was elective in 42 patients (51 %) and urgent in the others. Previous myocardial infarction was found in 37 patients (45%) and left ventricular ejection fraction (LVEF) at less than 45% in 15 patients (18%); 23 patients (28%) were in NYHA class III or IV and regarding angina functional status, 77% in CCS class 3 or 4. Follow-up was complete. Statistical analysis included 30 and pre and peri-operative data. Statistical analysis used Chi-square analysis or Fisher's exact test, and the Mann-Whitney test when appropriate. The estimated probability of survival, including postoperative mortality, was calculated by the method of Kaplan-Meyer, and the Log-Rank test used to compare the results. RESULTS: the hospital mortality was 14.6 % (n = 12). Ischemic time and ECC time were significantly lengthened in dead patients (P = 0.01). Moreover, use of internal mammary artery was directly related to lower hospital mortality (P = 0.02). For previous myocardial infarction, LVEF at less than 45%, diabetes and combined procedure, a P-value of < or = 0.1 was calculated. The follow-up ranged from 1 to 140 months (mean 36 months). There were 39 late deaths. The survival rates (included hospital mortality) were 71+/-5%, 56+/-6% and 39+/-6% at 1, 3 and 5 years, respectively. All surviving patients improved their functional status and had symptomatic relief. Statistical analysis showed significant difference in favor of long term survival for patients younger than 60 years, LVEF > 45% and NYHA class I or II. CONCLUSION: these data confirm that CABG in patients with renal replacement therapy is associated with an high operative and long term mortality. However it allows an improvement of functional status, and so, let possible duration of dialysis. It may be expected that more active prevention and detection of coronary disease might improve these results.

Surgery for post infarction ventricular septal defect (VSD): risk factors for hospital death and long term results.

OBJECTIVE: Repair of post infarction ventricular septal defect (VSD) is still a challenging procedure with a high risk of recurrence of the VSD and subsequent mortality. The aim of this retrospective study was to assess if technical change in the surgical procedure was followed by an improvement in recurrence of the VSD and operative results. METHOD: This retrospective study from 1971 to 2001 included 85 patients operated on early (<15 days) after the occurrence of a post infarction VSD. Double patch technique was introduced in 1986. A total of 44 variables were studied by a uni- and multivariate analysis. RESULTS: Hospital death occurred in 36 patients. Significant factors for hospital mortality included: preoperative and evolution of the clinical status, right ventricular function and type of repair (one or two patches). Moreover, no recurrence was observed in patients repaired with the double patch technique (P=0.09). None of the studied variables were significant for long term survival. Concomitant CABG was not associated with higher hospital mortality and long-term survival rate was similar in patients with or without concomitant CABG. CONCLUSION: The use of the double patch technique and glue by avoiding recurrence of the VSD played a role in the reduction of the hospital mortality. This technique has to be recommended in the early repair of post infarction VSD. Concomitant CABG can be done safely to control the added risk of an associated coronary artery lesion.

An unusual complication of the Le Veen shunt: a right atrial ventricular chamber pseudotumor.

We report the case of a right atrial-ventricular chamber thrombus discovered four years after insertion of a Le Veen shunt for treatment of refractory ascites. A two-dimensional echocardiogram, performed after the discovery of an isolated systolic murmur, demonstrated a "tumorlike" mass seated in both the right atrium and the right ventricle. The mass was surgically removed and histologic examination confirmed that it was a thrombus developed at the tip of the catheter. We propose that two-dimensional echocardiography should be performed periodically for the survey of such intracardiac devices.

Accuracy of M-mode and two-dimensional echocardiography in the diagnosis of aortic dissection: an experience with 128 cases.

The accuracy of combined M-mode and two-dimensional echocardiography in the diagnosis of aortic dissection was evaluated in 673 patients with a clinical suspicion of aortic dissection, over a six-year period. In 128 cases, the diagnosis of aortic dissection was confirmed by angiographic, tomographic (CT scan), or autopsy findings, or during surgery. Two echocardiographic features were found to support a diagnosis of aortic dissection: a dilation of at least one segment of the aorta (sensitivity 95%, specificity 51%) and a typical abnormal linear intraluminal echo corresponding to the intimal flap (sensitivity 67%, specificity 100%). This pathognomonic intimal flap was observed in 86 cases, of which three types could be distinguished: (1) a long oscillating flap (n = 15), (2) a long but minimally mobile linear echo which was duplicated and parallel to one or two aortic walls (n = 64), (3) a short, double linear image with a rapid systolic motion and high frequency oscillations. These features were found to have a high sensitivity in type I aortic dissection (88%), although in types II and III the sensitivity was much lower. In some cases, a fourth type of abnormal image could be detected: a small intraluminal echo moving in parallel to the aortic wall. This feature should be interpreted with caution since its predictive value for a positive examination was low (48%). Out of 23 cases in which the diagnosis of aortic dissection was suspected on the basis of this doubtful abnormal echo, it was confirmed in only 11 patients. The results in these 128 cases of aortic dissection indicate that two-dimensional echocardiography, which is easily performed at the patient's bedside, could take priority in investigations of this condition. It is extremely sensitive in the diagnosis of ascending aortic dissection, but much less so in the diagnosis of descending aortic dissection.

[Primitive leiomyosarcoma of the right atrium presenting with a pulmonary embolism]. / Léiomyosarcome primitif de l'oreillette droite révélé par une embolie pulmonaire.

Primary Cardiac leiomyosarcoma is a very rare condition. The authors report the case of a 64 year old woman admitted to hospital for suspected pulmonary embolism, confirmed by thoracic angio-CT scan. Despite thrombolytic therapy, a persistent poor haemodynamic condition associated with a superior vena cava syndrome led to a transoesophageal echocardiographic examination which demonstrated an abnormal intra-right atrial mass obstructing the tricuspid valve. At surgery, a leio-myosarcoma was successfully resected under good conditions. During systematic three-monthly clinical and echocardiographic follow-up, transoesophageal echocardiography detected an asymptomatic local recurrence at 15 months, for which surgery was repeated with adjuvant chemotherapy.