Dealing with a special group: hypertrophic cardiomyopathy. Surgical treatment: what for?
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Biomedical subjects
Publications and source records attributed to M Abecassis.
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OBJECTIVES: A cardiopulmonary bypass technique was developed with the aim of achieving a less invasive approach to right-sided lesions of the heart or to avoid extensive dissection in reoperations. METHODS: Cavae endoluminal balloon occlusion (CEBO) was achieved with balloon catheters, introduced through cannulas inserted in the right jugular and one of the femoral veins. This procedure can be used in association with a standard femoral cardiopulmonary bypass or with the port-access technique. RESULTS: Twenty-two patients were operated, through a right thoracotomy, for atrial septal defect (19) and tricuspid valve regurgitation associated with mitral valve pathology (3). There were two intra-operative complications, partially related with the development of this technique and lack of appropriate designed material. There was no mortality and a shorter and easier postoperative care was achieved in almost all patients. CONCLUSIONS: The CEBO technique is an useful and safe adjunct to perform miniinvasive or complex reoperations on the right side of the heart.
Panniculitis is a rare complication of alpha 1-antitrypsin (A1AT) deficiency that is characterized by acute inflammatory infiltrate and fat necrosis. Different treatment strategies are used to provide symptomatic relief. Here we describe two patients with homozygous A1AT deficiency who developed panniculitis and were successfully treated with A1AT replacement. The patient who received a liver transplant experienced complete resolution of the skin lesions. The patient who received A1AT intravenously showed complete response, but the skin lesions recurred when the levels of A1AT fell below 50 mg/100 ml. Panniculitis secondary to A1AT deficiency can be successfully treated with liver transplantation or intravenous infusion of A1AT.
A descriptive study of a new model enabling serial biopsies of ongoing hyperacute rejection of small intestinal discordant xenografts is presented. In a series of guinea-pig-to-Lewis rat small bowel xenotransplants (n=7), aboral free ends of Thierry-Vella loops constructed from the graft were sequentially biopsied at one-minute intervals up to ten minutes post-reperfusion and less frequently thereafter. In a guinea pig-to-guinea pig (n=6) isograft series, biopsy controls for preservation/ischemia-reperfusion injury were obtained. Xenoantibody sequestration in this model was evaluated in a separate series of transplants, utilizing an ELISA assay for rat anti-guinea pig natural antibodies. Pathologic evaluation revealed a unique series of events characterized with microcirculatory failure and thrombosis progressing from the submucosal vasculature to the lumen. Within the system's detection limits, complement deposition and P-selectin expression occurred as early as one minute post-reperfusion, preceding the staining for IgM and IgG. Using rat serum ELISAs, no significant difference in xenoantibody sequestration was detected between the xenograft and isograft groups. The guinea pig-to-rat discordant small bowel xenotransplantation is an efficient small animal model to dissect the very early pathophysiologic events during hyperacute rejection.
Immunocompromised patients are frequently treated with guanine analogs such as acyclovir and ganciclovir. Acyclovir triphosphate, the active intracellular metabolite of acyclovir, exerts its antiviral effect by inhibiting herpesviral DNA polymerases through premature chain termination. PCR has recently been used for early detection of cytomegalovirus. However, we and others have experienced false-negative results for cytomegalovirus-PCR in patients on both acyclovir and ganciclovir. The impact of these agents on PCR assay is unknown. In an attempt to investigate the role of guanosine analogs in these false-negative results, we exposed the DNA-PCR for murine beta-actin, a murine CMV IE gene sequence, and a human CMV IEA1 product, to phosphorylated acyclovir derivatives. Varying concentrations of acyclovir-5'-triphosphate (final: 70-6000 microM) in the reaction mix resulted in an absence of detectable product at or above 490-670 microM. Inhibition was not observed with up to 1400 microM acyclovir-monophosphate. Increasing the Taq concentration to 10 units/100 microL stopped the inhibition. Our data demonstrate that acyclovir-5'-triphosphate inhibits PCR amplification of various gene products in a concentration-dependent manner. Furthermore, this inhibition appears to be specifically directed against the Taq polymerase and can be completely reversed by higher concentrations of the enzyme. Thus, false-negative PCR results for a viral gene product in patients under prophylaxis/treatment with acyclovir could potentially be due to contamination by acyclovir triphosphate. Therefore, negative PCR results in these patients need be interpreted with caution.
Gastrointestinal complications occur after renal transplantation in up to 16% of patients. Cytomegalovirus (CMV) vasculitis can affect the gastrointestinal tract with significant manifestations, including colonic ulceration, bleeding, and perforation. We present a case of CMV vasculitis in a renal transplant patient that caused middle and left colic vein thrombosis and resultant ischemic colitis. There was no evidence of arterial involvement. To our knowledge, this is the first reported case of ischemic colitis secondary to large vein thrombosis associated with CMV infection.
Recipients of a simultaneous pancreas/kidney transplant (SPK) appear to have a greater incidence of acute rejection than do recipients of a kidney-alone transplant. These patients are also susceptible to multiple rejection episodes. Because of this, optimal immunosuppression is imperative in these patients. Cyclosporine A (CsA)-based immunosuppression remains the mainstay of therapy, and therefore, optimization of this often unpredictable drug is necessary. We looked at 14 consecutive SPK recipients and assessed the utility of a single 1-month pharmacokinetic study in predicting the risk of acute rejection. Each pharmacokinetic study was analyzed by both monoclonal fluorescence polarization immunoassay (TDX) and high performance liquid chromatography (HPLC). Due to the small sample size and the high rate of primary rejection (12/14 patients), we chose a novel endpoint, that of recurrent rejection. Seven of the 14 patients studied developed recurrent rejection. The patients without recurrent rejection had significantly higher areas under the concentration curve (AUCs) than did patients with recurrent rejection: 4,663 +/- 1,460 vs. 2,454 +/- 1,717 ng-h/ml (p < 0.05) by HPLC and 6,669 +/- 2,556 vs. 3,661 +/- 1,956 ng-h/ml (p < 0.05) by TDX. We propose that a single 1-month AUC has potential utility in identifying a subset of SPK patients with poor CsA absorption at risk for recurrent rejection.
Activation of the immune coagulation system has been implicated in the pathogenesis of liver injury following infection of inbred mice with murine hepatitis virus strain 3 (MHV-3). Following MHV-3 infection, macrophages isolated from MHV-3-susceptible and -semisusceptible inbred strains of mice express increased procoagulant activity (PCA), whereas macrophages from resistant strains express no increase in PCA over basal levels. The PCA induced by MHV-3 is a prothrombinase, encoded by the gene Fgl-2, which encodes a fibrinogen-like protein (musfiblp). In this study, MHV-3-resistant A/J mice treated with methylprednisolone prior to infection with MHV-3 developed elevated levels of alanine aminotransferase in serum and died within 10 days of infection, with histological findings of fulminant hepatitis. In vitro, macrophages isolated from A/J mice and pretreated with methylprednisolone produced a marked increase in functional PCA following infection with MHV-3. The PCA was shown to be a prothrombinase by its ability to cleave 125I-prothrombin. Northern blot analysis of RNA transcripts from these macrophages demonstrated increased transcription of the Fgl-2 gene relative to that in macrophages which had not been pretreated with methylprednisolone prior to MHV-3 infection. Methylprednisolone pretreatment of MHV-3-infected macrophages stabilized the Fgl-2 mRNA. Thus, loss of resistance to MHV-3 secondary to methylprednisolone therapy is associated with increased transcription and stability of Fgl-2 mRNA resulting in expression of the Fgl-2 gene product, musfiblp. These results provide further insight into mechanisms of PCA regulation in response to MHV-3 infection in inbred strains of mice.
OBJECTIVE: The aim of this study was to evaluate the results of our experience with implantable cardioverter-defibrillator therapy. PATIENT SELECTION: We treated with implantable cardioverter-defibrillator (ICD) 18 patients, 15 male and 3 female, mean age 51 years, ranging from 12 to 76 years, with life threatening ventricular arrhythmias. Eleven patients were resuscitated from cardiac arrest and seven had refractory ventricular tachycardia. The underlying condition was coronary artery disease in 12 patients, dilated cardiomyopathy in two, congenital long QT syndrome in one, mitral regurgitation in one and idiopathic in two patients. METHODS: All patients underwent cardiac catheterization and electrophysiological study before ICD implantation. In the first patient epicardial leads were used, but a transvenous approach was used in the remaining 17 patients. The device was implanted in an abdominal position in all patients. Defibrillation and pacing threshold tests were performed during the implantation procedure and whenever necessary. After implantation, patients were followed up in an outpatient basis, with evaluation of therapy efficacy and reprogramming of the device if required. The mean follow-up time was 16 months, ranging from 1 to 40 months. RESULTS: The implantation was successful and without complications in all patients. Defibrillation threshold was considered within normal range, with an electrode impedance ranging from 40 to 65 Ohms. During the follow-up period no deaths occurred, 8 patients (44%) had episodes of VT or VF that were successfully treated, with shock in six patients, with antitachycardia pacing in one and with both modalities in another patient. Inappropriate shocks were observed in three patients (16%). The device was reprogrammed in five patients. CONCLUSIONS: Our experience with implantable ICD in patients who survived cardiac arrest or with refractory VT has shown a low surgical risk with no mortality. The incidence of ICD discharges was high, with a satisfactory efficacy rate and the number of inappropriate shocks was acceptable. The careful patient selection made possible a good cost/benefit relation.
OBJECTIVE: The goal of this study was to evaluate the clinical and echocardiographic results of a cohort of patients subjected to aortic valve replacement with pulmonary autograft (Ross procedure). DESIGN: We studied prospectively with clinical and echocardiographic evaluation all patients subjected to this type of aortic valve surgery, before operation, intraoperatively, before hospital discharge and at the end of this study in September 1994 and we analysed the evolution of the results during the follow-up period. PATIENTS: 22 consecutive patients 12 men and 10 women mean age 48 +/- 14 years subjected to Ross operation between April 1992 and June 1994. METHODS: Patients were studied with transesophageal echocardiography intraoperatively and transthoracic echocardiography. Two-dimensional and M-mode evaluation, continuous and pulsed wave Doppler and color coded Doppler were performed, with assessment of the morphology and Doppler profile of both the autograft and the homograft in pulmonary position. RESULTS: No early or late mortality was registered, and apart two patients who were reoperated, all patients were asymptomatic in the last medical visit. There was no incidence of thromboembolic events even though no anticoagulation has been used. Aortic regurgitation was present in 14 patients, grade 1 in 11 patients and grade 2 in three. In the last echocardiogram performed the analysis of aortic flow, demonstrated absence of significant transvalvular gradient (maximal aortic gradient of 5.6 +/- 0.4 mmHg and mean aortic gradient of 1.8 +/- 0.08 mmHg). Pulmonary regurgitation was absent in nine patients, and present in 13, considered trivial in all cases. CONCLUSIONS: These data suggest that this type of surgery has a low operative risk, with absence of thromboembolic complications and excellent clinical and echocardiographic results.
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Hepatic infarction remains a rare and dramatic presentation of an acute abdomen in patients with systemic lupus erythematosus. It can be recognized preoperatively only with a high index of suspicion. Imaging studies such as abdominal computed tomography may aid in making the diagnosis. The tendency for thrombosis in these patients often can be associated with the presence of circulating anticoagulants from a group of antiphospholipid antibodies. The finding of lupus anticoagulant or anticardiolipin antibodies can help identify those patients more prone to thrombotic episodes, although at present it cannot be assumed that these antibodies cause the thromboses. Hemodialysis patients are said to have a decreased tendency for vascular thrombosis, but this may not be true in the presence of circulating antiphospholipid antibodies. We report a case of histologically documented hepatic infarction secondary to thrombosis in an end-stage renal disease patient on hemodialysis with systemic lupus erythematosus and a circulating lupus anticoagulant.
The aim of this study is to establish anatomical landmarks for the trajectory of the large septal artery. Thirty hearts were dissected, 20 of which had no cardiac pathology and the remaining with different cardiac conditions. One large septal artery was located in 27 of these hearts, two large septal arteries in 2 and three large septal arteries in 1. For all cases there existed one large septal artery in the lower border of the anterior limb of the septomarginal trabecula. When more than one large septal artery was encountered, the first was located within the subendocardial outflow tract of the left ventricle, the second was in the lower border of the anterior limb of the septomarginal trabecula and the third 1.5 cm below the second.
Peripancreatic abscess formation remains an important cause of surgical morbidity following simultaneous pancreas-kidney (SPK) transplants. The purpose of this study is to define the association, if any, between the pretransplant method of dialysis and the incidence of peripancreatic abscess formation. We reviewed 57 bladder-drained SPK transplants. Of these, 42 were on haemodialysis (HD) and 15 on peritoneal dialysis (PD) prior to transplantation. Two grafts were lost and three patients died as a result of peripancreatic infections for a total graft loss of 8.8%. The incidence of this complication was higher (P = 0.036 Pearson chi square) in patients on PD (40%) prior to transplant when compared to patients on HD (14%). The most common organism cultured from the abscesses was Staphylococcus epidermidis in both groups. Impairment of peritoneal macrophage function and accumulation of peritoneal effluent as a result of altered peritoneal membrane function are some of the changes that occur following chronic peritoneal dialysis and could explain our findings.
Methicillin-resistant Staphylococcus aureus (MRSA) isolates collected during a 7-month period in 1992 and 1993 at Hospital Pulido Valente (340 beds), Lisbon, Portugal, were characterized by a combination of genotypic and phenotypic methods. Clonal identities were determined by probing ClaI digests (i) with a mecA probe and (ii) with a Tn554 probe and (iii) by pulsed-field gel electrophoresis (PFGE) patterns of chromosomal SmaI digests. mecA-ClaI type I was predominant among these isolates (38 of 43). Most of these (37 of 38 [97.4%]) were associated with a single Tn554 pattern, pattern E, and the majority (23 of 38 [61%]) also showed a relatively uniform chromosomal background, as indicated by PFGE (PFGE pattern A). The major clone (mecA-ClaI type I::Tn554 type E and PFGE pattern A) at Hospital Pulido Valente was indistinguishable by these molecular typing criteria from the dominant clone that had been identified in two major current outbreaks of MRSA disease in Spain (Barcelona and Madrid). The Portuguese and Spanish clones also had a common heterogeneous class 3 phenotype and identical multidrug resistance patterns. The data presented in this work support the notion that MRSA clones can spread across considerable geographic distances.
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Thirteen patients submitted to bone marrow transplantation (BMT), HLA A and B identical, with mixed negative lymphocyte culture and major ABO incompatibility were retrospectively evaluated. Gravity sedimentation by hydroxyethyl starch was used in almost all cases to deplete erythrocytes from bone marrow (12/13): a removal rate of 90%--corresponding to an erythrocyte residual volume of 27,4 rate ml--and a nucleated cell recovery of 76% (mean values) were obtained. All patients underwent a hyperhydration regimen and received corticosteroid prophylaxis before bone marrow infusion. Considering the most important immunohematological problems associated to this type of transplant, we verified that seven patients developed minor complications and among them only one had long term consequences. In comparison with the control group, ABO identical and with minor incompatibility, the study group had delayed onset of erythropoiesis and needed greater erythrocyte transfusion support. We concluded that major ABO incompatibility does not constitute a drawback to BMT success in most patients.
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