Mustard Patients Research Articles

Myocardial perfusion abnormalities in chemical warfare patients intoxicated with mustard gas

Mustard agents are of the major chemical agents used during Iran-Iraq war. There are no reports concerning long-term cardiac effects. The aim was to assess the scintigraphic pattern of myocardial perfusion in patients intoxicated with blistering gases. We analyzed myocardial perfusion scans of 22 consecutive intoxicated patients (21 male and 1 female, all < 44 years) and compared results with 14 controls. Only those patients and controls were entered whose 10-year risk of coronary artery disease (Framingham criteria) was <5%. Also only those patients were experimented upon that had currently other confirmed complications of intoxication (respiratory, cutaneous and ocular complications). All patients underwent a 1-day stress and rest protocol using (99m)Tc-MIBI. Images were assessed visually and quantitatively using Cedars Sinai program. The prevalence of nonhomogeneity of uptake and left and right ventricular enlargement in both visual and quantitative analyses were higher in the mustard exposed patients than unexposed controls. The prevalence of ischemia was higher in the exposed patients (P < 0.05). Cavity to myocardium ratio, as an established and validated measure of ejection fraction, was also significantly lower in the warfare patients than the controls. In so far it lies in our knowledge, this is the first report concerning the scintigraphic pattern of myocardial perfusion in mustard intoxicated patients. Based on the results, the pattern of myocardial perfusion in these patients is significantly different from normal controls, which could resemble either coronary artery disease or mild cardiomyopathic changes.

Isolation and identification of an 11S globulin as a new major allergen in mustard seeds

Although mustard seed allergy has been largely reported during the preceding 20 years, currently only 2 allergens, Sin a 1 and Bra j 1, have been identified. To improve the characterization of the allergenic profile of yellow mustard seeds by reporting the identification and biochemical characterization of an 11S globulin as a new major allergen. Mustard seed proteins were separated using size exclusion and ion-exchange chromatographic columns, sodium dodecyl sulfate-polyacrylamide gel electrophoresis, and 2-dimensional polyacrylamide gel electrophoresis. Separation of different polypeptide chains was achieved by reverse-phase high-performance liquid chromatography. Mass spectrometry after tryptic digestion and Edman degradation were used to determine amino acid sequences of peptides. IgE binding assays were performed with 13 serum samples from mustard allergic patients in immunoblotting and enzyme-linked immunosorbent inhibition assays. A protein of 51 kDa was recognized as a major allergen by patients allergic to mustard and called Sin a 2. The allergen was dissociated in 2 chains of 36 and 23 kDa, which also bound IgE. N-terminal end and internal amino acid sequences allowed identification of the new allergen as a seed storage 11S globulin belonging to the Cupin super family. Purified allergen was able to inhibit the IgE binding of sera from allergic patients to mustard seeds extract in up to 55% of the responses. An 11S globulin storage protein has been isolated and identified as a novel major allergen of mustard seeds.

Mustard allergy confirmed by double‐blind placebo‐controlled food challenges: clinical features and cross‐reactivity with mugwort pollen and plant‐derived foods

Mustard IgE-mediated allergy is supposed to be a rare cause of food allergy, and its clinical features and cross-reactivities have not been fully elucidated. A prospective study was carried out, recruiting mustard allergic patients, and paired control subjects. A clinical questionnaire was administered, and skin-prick tests (SPT) with panels of aeroallergens and foods, serum extraction for in vitro tests and double-blind placebo-controlled food challenges (DBPCFC) were performed. Thirty-eight mainly adult patients, with 10.5% reporting systemic anaphylaxis, were included in the study [age (mean +/- SD): 21.9 +/- 8.6 years]. DBPCFC were performed in 24 patients, being positive in 14 cases (58.3%). Patients with positive outcome showed significantly greater mustard SPT than those with negative outcome (8.2 +/- 3.7 vs 5.3 +/- 2.4 mm, P <0.05), and the receiver-operating characteristic (ROC) curve analysis yielded a cut-off value for mustard commercial SPT of 8 mm, with a specificity of 90% (95% CI, 55.5-98.3), and a sensitivity of 50% (95% CI, 23.1-76.9). A significant association between mustard hypersensitivity and mugwort pollen sensitization was found (97.4% of patients), with partial cross-reactivity demonstrated by UniCAP System inhibition assays. All patients showed sensitization to other members of Brassicaceae family, and cross-reactivity among them was also confirmed. Moreover, significant associations with nut (97.4%), leguminous (94.7%), corn (78.9%), and Rosaceae fruit (89.5%) sensitizations were also shown. Around 40% of these food sensitizations were symptomatic, including food-dependent exercise-induced anaphylaxis in six patients. Mustard allergy is a not-uncommon disorder that can induce severe reactions. Significant associations with mugwort pollinosis and several plant-derived food allergies are demonstrated, suggesting a new mustard-mugwort allergy syndrome. A relationship between this syndrome and food-dependent exercise-induced anaphylaxis is also reported.

Myocardial Infarction Late After Mustard Procedure

This case report discusses a young man with d-transposition of the great arteries who suffered three myocardial infarctions late after his Mustard procedure. The etiology was found to be a coronary embolism, with a mural thrombus in the systemic ventricle as the probable nidus. The risk factors of coronary embolism are discussed, and it is concluded that Mustard patients are at risk for coronary embolization and myocardial infarction, which can be a cause of sudden death.

Does chronic pacing affect exercise capacity after Mustard operation for transposition of the great arteries?

Late survival after Mustard repair of transposition of the great arteries is generally good but is often characterized by progressive deterioration of ventricular function and by late postsurgical arrhythmias, thus imposing the need for permanent pacing. To evaluate how chronic pacing affects long-term exercise capacity, we compared two groups of these patients: group 1, comprising 12 patients, aged 9.0 +/- 2.6 years, without pacemaker; and group 2, comprising 18 patients, aged 9.3 +/- 2.0 years, with pacemaker. Patient evaluation included history, physical examination electrocardiograph, Holter monitoring, and echocardiography. Pacing modes were as follows: AAI (6 patients), AAIR (9 patients), VVI (2 patients), and VVIR (1 patient). At exercise test we evaluated exercise tolerance, maximum heart rate, blood pressure, oxygen consumption, and cardiac output at rest and at peak exercise. The two groups were comparable for all variables examinated. All pacemakers showed normal function. During the exercise, 11 of 12 patients in group 1 showed sinus rhythm, and in group 2, 11 patients showed sinus rhythm, 5 junctional rhythm, and 2 continuous pacing. There were no significant differences between groups. Chronically paced Mustard patients but with restoration of spontaneous rhythm during the exercise test do not show reduced exercise tolerance in comparison with nonpaced Mustard patients.

Catheter interventions in adult patients with congenital heart disease

Adult patients with congenital heart disease provide a wide variety of challenges for the interventional cardiologist. Procedures can broadly be divided into dilatation or closure. The most common interventions in our own practice are closure of atrial septal defects and patent foramen ovale, although closure of postoperative interatrial communications in Mustard, Senning, or Fontan patients is also possible. Transcatheter patent ductus arteriosus closure is also now routine, and occlusion of coronary artery fistulae can be safely attempted using coil embolisation. Balloon dilatation of pulmonary valve stenosis has excellent success rates, and dilatation or stenting of pulmonary artery stenoses is also beneficial in selected cases. Although aortic valve dilatation in adults has mixed results, dilatation or stenting of aortic coarctation is now becoming more widespread, and has promising results. Dilatation procedures may also be applicable to the postoperative patient with conduit or baffle stenosis.

Aerobic capacity in adults with various congenital heart diseases

As an increasing number of patients with congenital heart disease reach adulthood, more information is needed regarding outcomes. The first signs of impaired heart function may appear during exercise testing. The aim of the present study was to establish mean values for maximal oxygen uptake in adults with various congenital heart diseases. Patients from 6 major diagnostic groups were identified, including patients with atrial septal defect (ASD, n = 93), transposition of the great arteries corrected with the Mustard procedure (n = 84), congenitally corrected transposition of the great arteries (CCTGA, n = 41), Tetralogy of Fallot (n = 168), Ebstein’s anomaly (n = 37), and Modified Fontan procedure (n = 52). Diminished maximal oxygen uptake was found in all diagnostic groups across age compared with healthy subjects. A significant decrease in maximal oxygen uptake with aging was found in those with ASD (p <0.0001), CCTGA (p = 0.01), and Tetralogy of Fallot (p <0.0001). There was no significant decline, however, in Ebstein’s anomaly (p = 0.270), Fontan procedure (p = 0.182), and in the Mustard patients (p = 0.188). All patients achieved significantly lower heart rates than predicted (mean for all groups, p <0.0001). Forced vital capacity values (3.51 L, mean SD ± 1.02) were lower than predicted values (4.10 L, mean SD ± 0.90, p <0.0001) for all patients groups except those with ASD. Mean values, however, were within the accepted 20% range of variance. This study showed diminished aerobic capacity in all diagnostic groups when compared with a healthy population. The maximal oxygen uptake values across age groups can be used as reference values in patients with similar diagnoses and as the basis for further research.

Proximal Pulmonary Artery Banding Does Not Simulate a Systemic Afterload for Conditioning a Left Ventricle in Mustard Patients

Proximal Pulmonary Artery Banding Does Not Simulate a Systemic Afterload for Conditioning a Left Ventricle in Mustard Patients

Transcatheter management of venous stenosis.

Stenoses of the central venous system are an infrequently encountered problem in the pediatric patient. Usually seen after repair of congenital heart disease, included in this category are obstruction of the superior vena cava and its branches, inferior vena cava, and pulmonary veins, as well as obstruction of the atrial baffles in postoperative Fontan and Mustard patients. Transcatheter management, first balloon angioplasty, and, more recently, adjunctive or primary stent placement, have been successfully applied to central venous stenoses in pediatric patients with good results and a low incidence of complications. In this article, we will review the common sites and causes of venous obstruction and their transcatheter management.

Status of survivors after atrial redirection for transposition of the great arteries: a complete long‐term follow‐up

All 32 survivors with transposition of the great arteries, born in 1964-83 and operated on at our institution using atrial redirection, were evaluated by cardiac catheterization, echocardiography and Holter monitoring. There were 17 Mustard patients, age 17.1 years (+/-3.5, 12.0-22.0) and 15 Senning patients, age 9.4 years (+/-1.6, 7.2-12.1). All but one had simple transposition. Six had caval obstruction, one had pulmonary venous obstruction, three had large atrial shunts, four had considerable pulmonary hypertension, seven had mild ventricular outflow tract obstruction, four had significant tricuspid regurgitation, 11 had systemic ventricle dysfunction (one severe), 14 had sinus node dysfunction (three symptomatic) and two had atrioventricular block (one with pacemaker). Eight Mustard patients (47%) and one Senning patient (7%) had symptomatic cardiac sequelae, and only one patient (Senning) was free from sequelae, illustrating that these patient groups will need continuing medical attention.

Right ventricular systolic function in adolescents and young adults after mustard operation for transposition of the great arteries

This study evaluates long-term (>10 years since surgery) right ventricular (RV) systolic function in patients who had previously undergone intraatrial baffle surgery for transposition of the great arteries. Studies suggest these patients are clinically stable and lead satisfactory lifestyles, but long-term ventricular performance is not known. Radionuclide angiocardiography was used to estimate RV ejection fraction in 58 patients a mean of 14 years after the Mustard operation. Repeat studies were performed in 32 patients. The absolute RV ejection fraction of 0.53 ± 0.10 in our patients did not differ from normal values. Nine patients had a value < 0.42, placing them >2 SDs below normal. Repeat RV ejection fraction decreased from 0.54 ± 0.11 to 0.51 ± 0.11 (p < 0.1) in 32 patients, and >0.10 in 6. Thus, RV ejection fraction was abnormal in 9 of 58 patients (16%) evaluated >10 years after a Mustard operation. Repeat studies demonstrate worsening in at least 6 of 32 patients (19%). These postoperative Mustard patients require continued evaluation, even in the absence of overt symptomatology.

794-6 Functional Capacity in Adult Patients with Mustard Correction

The effect of prolonged support of the systemic circulation by the right ventricle was assessed in adult survivors of the Mustard operation. Forty five adult patients (32 male) aged 21.7 +/-3.3 years (mean +/-S.D) were studied. They had undergone Mustard's operation for correction of complete transposition 19.1 +/-2.6 years previously at 2.6 +/-3.1 years of age. Cardiopulmonary function was assessed using maximal bicycle ergometry with respiratory mass spectrometry. Systemic ventricular ejection fraction (SVEF) was measured at rest (r) and with exercise (ex) by radionuclide angiography. Thirtyfour patients additionally had magnetic resonance imaging to measure ventricular dimensions. Results were compared with normals [mean (SD)] Max. Work load kpm VO2 max AT SVEF (r) SVEF (ex) SVTh SVD % NYHA 1 or 2 Mustard 673 * 17.1 * 11.5 * 45.4 * 51.1 * 14.0 * 40.4 + 92 (179) (4.9) (4.0) (10.9) (14.7) (3.7) (7.4) Normal 1117 43.4 24.2 59.9 70.6 9.3 32.5 100 (190) (10.1) (4.7) (8.7) (7.7) (1.0) (3.5) AT = anaerobic threshold, VO2 max = maximal oxygen consumption, SVD = systemic ventricular diameter (mm), SVTh = wall thickness (mm) * p &lt; 0.01 + p &lt; 005 There was no significant difference in maxima I heart rate or maximal blood pressure with exercise between the groups. There was no correlation between ventricular dimensionlthickness and ejection fraction either at rest or exercise. Maximal work load was correlated with ejection fraction on exercise (p &lt; 0.05). Thus adult Mustard patients have dilated. hypertrophied systemic ventricles with reduced ejection fraction compared with controls. Formal functional assessment demonstrates significant impairment despite patients leading normal or near normal lives.

Successful Catheter Fulguration of the His Bundle in a Postoperative Mustard Patient after Unsuccessful Fulguration of Ectopic Atrial Foci

Incessant supraventricular tachycardia (SVT) resistant to pharmacological agents may cause cardiac dysfunction requiring more aggressive therapy. We present the case of a 12-year-old postoperative Mustard patient who developed biventricular heart failure due to an atrial ectopic tachycardia resistant to amiodarone. Using endocavitary direct current fulguration techniques, catheter ablation of the His bundle was successfully performed after unsuccessful attempts at ablation of the ectopic atrial foci. After placement of a permanent transvenous rate responsive ventricular pacemaker, the patient's clinical status and cardiac function improved. Endocavitary fulguration of the His bundle is technically feasible after the Mustard procedure and should be considered for treatment of selective cases of pharmacologically resistant SVT.

Electrophysiologic evaluation of sinus node dysfunction in postoperative children and young adults utilizing combined autonomic blockade–B. Marcus Et Al.: Postoperative intrinsic sinus node evaluation

Sinus node dysfunction is a recognized problem following surgery for congenital heart disease. Seven postoperative patients with sinus node dysfunction (5 Mustard, 1 tetralogy of Fallot, 1 Fontan) underwent electrophysiology study of sinus node function during combined autonomic blockade (CAB) utilizing propranolol 0.2 mg/kg i.v. and atropine 0.04 mg/kg i.v. to evaluate intrinsic sinus node function isolated from autonomic control. During CAB, intrinsic heart rate, intrinsic corrected sinus node recovery time, and intrinsic sinoatrial recovery time were measured. These results were compared with age-matched normal intrinsic data from our lab [normal (n = 7, mean age 9 years) IHR 128 +/- 24, intrinsic corrected sinus node recovery time 135 +/- 40 ms, intrinsic sinoatrial conduction time 86 +/- 19 ms]. Among postoperative Mustard patients (n = 5, mean age 13 years, mean years postoperative 11) 2 of 5 had clearly abnormal intrinsic sinus node function with nonsinus rhythm during CAB; 3 of 5 had sinus rhythm during CAB with normal or mildly abnormal intrinsic sinus node function. The postoperative case of tetralogy of Fallot (age 20 years, postoperative 14 years) had mildly abnormal intrinsic sinus node electrophysiology study. The postoperative case of Fontan (age 16 years, postoperative 1.5 years) had sinus rhythm at rest but left atrial rhythm during CAB. Different aspects of sinus node dysfunction may be expressed during resting electrophysiology study vs. electrophysiology study utilizing CAB. The pathophysiology of sinus node dysfunction among postoperative pediatric patients is not homogeneous with regard to the contribution of intrinsic sinus node dysfunction. In those patients with normal or mildly abnormal intrinsic sinus node function, an important pathophysiologic influence of the autonomic nervous system is implicated.

PERMANENT PACING INDICATIONS FOLLOWING MUSTARD OPERATION FOR TRANSPOSITION OF THE GREAT ARTERIES

Sick sinus syndrome is a frequent complication following Mustard operation. Between October 1971 and May 1985, we performed the Mustard operation on 78 patients with transposition of the great arteries. Sixty-six patients were followed long-term for up to 10 years after surgery. We reviewed patient records and Holter monitor recordings in these latter 66 patients to determine if the degree of bradycardia predicted symptoms requiring permanent pacing. A total of 8/66 patients received pacemakers. Indications for pacing included asymptomatic bradycardia during the first postoperative month, symptomatic bradycardia (seizures and syncope) late following surgery, and use of antiarrhythmic drugs other than digoxin for the control of symptomatic atrial flutter or supraventricular tachycardia. The mean minimal heart rates documented by Holter monitor recordings did not differ significantly between the asymptomatic and symptomatic patients (p 0.15). We conclude that the degree of bradycardia is not a reliable predictor of symptoms or the need for pacing in postoperative Mustard patients during early and intermediate duration follow-up.

Long-term follow-up of dysrhythmias following the Mustard procedure

Earlier reports have suggested that the incidence of dysrhythmias after the Mustard procedure can be reduced if the sinoatrial node (SAN) is protected during surgery. To determine if these initial differences continue after longer follow-up, we examined all ECGs available for three groups of patients operated upon from January, 1965, through December, 1977. Group A included 37 patients who survived the operation prior to January, 1972, when surgical modifications were initiated to protect the SAN; group B included 44 patients available for follow-up who were operated upon from 1972 through 1974; and group C consisted of the 39 patients available for follow-up operated upon from 1975 to 1977. Dysrhythmias were classified as passive (failure of initiation or propagation of the SAN impulse), active (atrial flutter or supraventricular tachycardia), or atrioventricular (AV) conduction defects. Results were expressed as the incidence per number of different rhythms during follow-up intervals. The incidence of sinus rhythm in groups B and C (80%) was much greater than in group A (27%) during the first 2 years. However, after 8 years, less than 50% of the rhythms were sinus. Both brady- and tachydysrhythmias were common. Seven patients (6%) required pacemaker insertion for symptomatic sick sinus syndrome. Therefore despite efforts to protect the sinus node, late occurring dysrhythmias remain a significant problem in the postoperative Mustard patient.

Ventricular diastolic pressure-volume relations after the intra-atrial baffle operation for transposition of the great arteries.

Right and left ventricular diastolic pressure-volume (P-V) relations were assessed in 30 patients approximately 2 years after the Mustard operation. The overall distensibility index (delta V/ delta P), the compliance index at end-diastole (dv/dp), and the modulus of ventricular stiffness (Kp) were calculated in all, as well as the half-time of early diastolic pressure fall (T) in 10 cases. Transducer-tipped catheters were used in 15 patients and biplane cineangiography was employed in all. Values for the above indices equal to 2 standard deviations below the mean, obtained from five postoperative Mustard patients with no residual defects, were used as "normals." Seven patients with pulmonary hypertension (PH) and six with pulmonic stenosis (PS) were found to have impaired P-V relations. The impairment was more profound in the PH group. Delta v/delta p and dv/dp were abnormal in six of seven patients for both ventricles and T was abnormal in two of five. Kp was abnormal for the LV, and the RV ejection fraction was diminished. In the PS group (nine patients) the impairment was milder, affecting more than LV and end-diastole (dv/dp). Compliance studies might be useful in patients with LV afterload problems following the Mustard operation.

Alkylating Agents in Bronchogenic Carcinoma

Abstract A large number of patients in a group of Veterans Administration Hospitals received a variety of alkylating agents in studies in which these agents were randomized with an inert compound. Intravenous cyclophosphamide, and possibly nitrogen mustard, had a slight favorable influence upon survival for all patients. A difference between the two agents was seen: patients with squamous cell carcinoma appeared to respond best to nitrogen mustard therapy and patients with small cell undifferentiated carcinoma to cyclophosphamide therapy, in fairly striking fashion. Over-all influences upon survival, however, were not remarkable.

Alkylating agents in bronchogenic carcinoma

A large number of patients in a group of Veterans Administration Hospitals received a variety of alkylating agents in studies in which these agents were randomized with an inert compound. Intravenous cyclophosphamide, and possibly nitrogen mustard, had a slight favorable influence upon survival for all patients. A difference between the two agents was seen: patients with squamous cell carcinoma appeared to respond best to nitrogen mustard therapy and patients with small cell undifferentiated carcinoma to cyclophosphamide therapy, in fairly striking fashion. Over-all influences upon survival, however, were not remarkable.