Haemodynamics after Mustard's operation for transposition of the great arteries

Cardiac catheterization data from 54 investigations after Mustard's procedure were examined to study the influence of the operation on pressure events in the atria, great veins, and pulmonary circulation. Systemic venous atrial pressure tracings were characterized by a rapid, sharp 'y' descent. Pressure gradients between the venae cavae and systemic venous atrium were invariable, whether or not vena caval pathway obstruction was present, the 'y' trough and 'a' wave gradients being greater than the mean gradient. Pulmonary venous atrial pressure tracings were not different from normal except when tricuspid regurgitation was present. It is suggested that the baffle effectively reduces the size and compliance of the systemic venous atrium, but influences the pulmonary venous atrium to a lesser degree. The systolic pressure gradient from the left ventricle to pulmonary artery was decreased postoperatively, suggesting that it may be flow-related; the greatest changes were seen in the group with preoperative ventricular septal defect. The ratio of pulmonary: systemic vascular resistance did not change significantly after operation, and it is suggested that both the pre- and postoperative values were higher than normal. Examination of the left ventricular or pulmonary arterial mean pressure postoperatively should raise the suspicion of a complication, e.g. pulmonary venous obstruction or tricuspid regurgitation.     

Transposition of the great arteries. Its anatomical correction. Our initial experience

Arterial switch correction of transposition of the great arteries has been performed without mortality on 8 children, 7 neonates and one 6 years old child, at our institution. One infant and the 6 year old child has previous pulmonary artery banding and modified Blalock-Taussig shunts. The coronary cuffs were anastomosed to the neo-aorta using the "trap-door" technique described by Mee and in seven the Lecompte manoeuvre was used. Postoperative management included treatment for persistent pulmonary hypertension and left ventricular dysfunction. All patients were discharged in satisfactory condition, and continue to do so at a maximum follow-up of ten months. These results encourage us to elect the arterial switch procedure as the primary treatment of transposition of the great arteries.     

Complete correction of transposition of the great arteries with left juxtaposition of the atrial appendages

Juxtaposition of the atrial appendages is an uncommon anomaly which is usually associated with transposition of the great arteries. Experience with five patients with transposition of the great arteries in combination with juxtaposition of the atrial appendages in whom Mustard's operation was performed is reviewed. Technically, the existence of juxtaposition of the atrial appendages in corrective surgery for transposition does not present any additional surgical problems. Emphasis is placed on the advantages of early complete correction, avoiding the need for palliative procedure.     

Successful term pregnancy after Mustard operation for transposition of the great arteries

Transposition of the great arteries is a complex cardiac malformation with poor prognosis without surgical correction. Since the introduction of surgical procedures such as the intra-auricular reorientation of the venous return (Mustard procedure), an increasing number of patients may reach adulthood and experience pregnancy. Because long-term complications after the Mustard operation include systemic heart failure, arrhythmias, venous return stenosis and pulmonary edema, hemodynamic changes during pregnancy and delivery may potentially engender life-threatening complications in these patients. We report the case of a 24-year-old primigravida who underwent a Mustard procedure at the age of 2 years for transposition of the great vessels, and who carried out a full-term pregnancy. The pregnancy was uneventful until the 34th week, when the woman developed signs of moderate right ventricular failure and frequent episodes of accelerated junctional rhythm. Digitalisation improved symptoms and elicited return to normal sinus rhythm. The patient delivered at term by elective cesarean section, under close hemodynamic monitoring.     

Anatomic correction of transposition of the great vessels

We present a new approach for anatomic correction of transposition of the great arteries. The two coronary arteries, with a piece of the aortic wall attached, are transposed to the posterior artery. The two aortic openings are closed with a patch. The aorta and pulmonary artery are transected, contraposed, ant then anastomosed. The interventricular septal defect is closed with a patch, through a right ventriculotomy approach, because the right ventricle is no longer part of the systemic circulation. Two patients, aged 3 months and 40 days weighing 4,200 and 3,700 grams, respectively, were operated upon with deep hypothermia and total circulatory arrest. There was good recovery from the operation, with normal cardiocirculatory conditions. Renal failure developed in the first patient, and she died on the third postoperative day. During this time the cardiocirculatory conditions were good. The second patient made an uneventful recovery. Hemodynamic studies 20 days after the operation showed complete correction of the malformation. Five and one-half months after the operation, he weighs 7,500 grams, and his development is very good. We believe that this operation will be reproducible by most cardiovascular septal defect and pulmonary hypertension.     

Heterogeneity of coronary flow reserve in the examination of multiple individual allograft coronary arteries

BACKGROUND: Epicardial and resistance vessel function in the transplanted heart has been evaluated primarily in regions supplied by a single vessel. Heterogeneity of flow among multiple perfusion fields as a marker of early endothelial dysfunction in the microcirculation has not been evaluated previously. This study tested the hypothesis that increased variability of coronary flow reserve (CFR) among multiple vascular regions would be associated with allograft coronary vasculopathy. METHODS AND RESULTS: One hundred six posttransplant patients undergoing cardiac catheterization had measurement of CFR in at least 3 major epicardial vessels. Patients were divided into those with minimal angiographic abnormalities (n=37) and those with no angiographic abnormalities (n=69). The ranges, coefficients of variation, and univariate and multivariate regression analyses of CFR were computed to determine the major clinical factors influencing the degree of variability. The abnormal angiographic group was older (54+/-11 versus 47+/-13 years; P<0.003), had older hearts (35+/-11 versus 27+/-10 years; P<0.005), and were further posttransplant (1626+/-1022 versus 931+/-984 days; P<0.0009). There was no difference in global CFR between groups (normal, 3.4+/-0.8 versus abnormal, 3.4+/-0.7; P=NS). The coefficient of variation of CFR was higher for the abnormal group (16.3+/-8.6% versus 11.0+/-5.5%; P<0. 0006). Univariate and multivariate predictors of increased variability in CFR included angiographic abnormalities, patient age, and body mass index. Both angiographic abnormalities and an elevated CV of CFR were predictive of a combined end point of death, congestive heart failure, or subsequent development of >/=50% coronary stenosis. CONCLUSIONS: These data demonstrate that increased variability of CFR is associated with discernible allograft coronary arteriopathy and is predictive of outcome in patients after heart transplantation.     

The double switch procedure for anatomical repair of congenitally corrected transposition of the great arteries in infants and children

AIMS: To assess outcomes of anatomical repair (double switch procedure) in infants and children with congenitally corrected transposition of the great arteries. METHODS AND RESULTS: Between September 1993 and August 1996, 17 patients with congenitally corrected transposition underwent surgery at UCSF. Anatomical repair was performed in 11 of these patients, at ages ranging from 4.8 months to 7.8 years (median 3.2 years). The remaining six patients did not undergo anatomical repair due to unfavourable anatomy (n = 2), prior conduit repair (n = 2), biventricular dysfunction (n = 1), and isolated complete atrioventricular block (n = 1). The 11 patients who underwent anatomical repair make up the study group for the present report. All 11 patients had a malalignment ventricular septal defect, while pulmonary outflow tract obstruction was present in nine patients and significant tricuspid valve pathology or dysfunction was present in five. Anatomical repair was achieved with a Senning (n = 7) or a Mustard (n = 4) procedure combined with an arterial switch operation plus ventricular septal defect closure (n = 4), or a Rastelli procedure with left ventricle to aortic baffle and right ventricle to pulmonary artery conduit (n = 7). There was one early death and no patients developed surgical complete atrioventricular block. At a median follow-up of 22 months, there were no late deaths. Two patients required a total of three late reoperations, and all patients were asymptomatic on no cardiac medication. Follow-up echocardiography revealed normal biventricular function in all patients. CONCLUSIONS: Anatomical repair of corrected transposition can be achieved with low rates of early mortality and surgical heart block, and favourable mid-term results. Long-term follow-up will be necessary to determine if the double switch approach improves the natural history of corrected transposition when compared to less aggressive surgical approaches that leave the right ventricle in the systemic circulation.     

Congenitally corrected transposition of the great arteries and exercise-induced ventricular tachycardia

Congenitally corrected transposition of the great arteries (CCTGA) is an uncommon condition. Few patients survive past 50 years of age, because of associated congenital defects, systemic (morphological right) ventricular dysfunction, atrioventricular valvular insufficiency or complete heart block. A unique case is presented of exercise-induced ventricular tachycardia, which led to the diagnosis of CCTGA, without atrioventricular block or other cardiac anomalies.     

Myocardial and forearm blood flow reserve in mild-moderate essential hypertensive patients

Interleukin-2 (IL-2) gene therapy alone and in combination with the herpes thymidine kinase gene (tk) was used to evaluate immunological responses and antitumor effects in head and neck cancer. Established floor of mouth squamous cell carcinomas in C3H/HeJ mice were directly injected with recombinant adenoviral vectors carrying both therapeutic and control genes. One week after adenoviral gene transfer, only the animals treated with combination IL-2+tk or tk alone demonstrated significant tumor regression. Residual tumors were harvested for microscopic evaluation and immunohistochemistry staining, which revealed a predominance of CD8+ lymphocytes in the tumor beds of the animals treated with IL-2. To evaluate the systemic immune effects of IL-2, animals treated with single or combination gene therapy received a second site challenge with parental tumor cells or a heterologous but syngeneic sarcoma cell line. Mice treated with combination IL-2 and tk demonstrated a protective systemic immunity specific to the parental tumor cell line, whereas no systemic immune response was evident in mice receiving IL-2 alone. In a separate experiment, a range of concentrations of the adenovirus IL-2 vector were used to treat established tumors. Even with the maximal single-dose adenovirus concentration, IL-2 alone was ineffective as a single therapy. These results support the use of adenovirus-mediated gene transfer of IL-2 as an effective immunotherapy when used adjuvantly with the tk "suicide gene".     

Cardiac output response to dynamic exercise after atrial switch repair for transposition of the great arteries

Results from this study showed that patients who underwent successful operation for transposition of the great arteries had no appropriate increase in stroke volume in response to exercise of a nature similar to common recreational activities. The impairment, most likely due to disturbances in both venous return and ventricular systolic function, is compensated for by an increase in peripheral oxygen extraction; however, this increase may not be adequate with maturation or during prolonged exercise when cardiovascular constraints are more important.     

Long-term outcome after the mustard repair for simple transposition of the great arteries. 28-year follow-up

The effects of feeding different amounts of colostrum or only milk replacer and the effects of Long-R3-IGF-I (administered s.c. or orally; 50 microg/[kg BW x d] for 7 d), and of s.c. injected recombinant bovine GH (rbGH; 1 mg/[kg BW x d] for 7 d) on small intestinal mucosal morphology in newborn calves were studied by histomorphometry. Neonatal calves fed colostrum six times exhibited greater (P < .01) villus circumferences, areas, and heights in total small intestine and especially in the duodenum than calves fed only milk replacer. Furthermore, villus circumferences and areas in total small intestine were greater (P < .05) in calves fed colostrum once than in calves fed no colostrum. Villus size in total small intestine was smaller (P < .05) in rbGH-treated than in control calves; jejunum villus circumferences and heights were especially reduced (P < .05). Crypt depths in ileum were greater (P < .05) in rbGH-treated calves. In conclusion, prolonged colostrum supply significantly enhanced small intestinal villus size in neonatal calves. In contrast, Long-R3-IGF-I had no significant influence on small intestinal morphology, and rbGH in supraphysiological amounts even reduced small intestinal mucosal variables after 1 wk of treatment. The study demonstrated enhanced postnatal development of the gastrointestinal tract by prolonged colostrum feeding, but not by Long-R3-IGF-I or GH.     

Mortality in potential arterial switch candidates with transposition of the great arteries

OBJECTIVE: Prostatic abscesses are uncommon in clinical practice because early antibiotic therapy has reduced complications of prostatitis. Prostatic abscess mainly affects diabetic and immunosuppressed patients. The organisms most frequently involved are Escherichia coli and Staphylococcus, whereas gonococcus is rarely encountered. The results in eight men with prostatic abscess, five of whom were treated with sonographically guided percutaneous drainage, are reported. The diagnosis, clinically suspected in only three patients, was confirmed by transrectal sonography. All patients experienced complete abscess resolution. CONCLUSION: Transrectal sonography is the most reliable imaging method to diagnose prostatic abscess. Percutaneous transperineal or transrectal drainage is the first choice for therapy because of the lower risk of complication compared with surgery.     

Maternal transposition of the great arteries in pregnancy. A case report

To determine the accuracy of whole-breast compression ultrasonography (US) in scanning palpable and nonpalpable breast lesions, the author correlated the findings of this method with those of mammography for 121 patients, ranging in age from 22 to 71 years, over a 2-year period. The two methods were performed on the same day by the same radiologist, who reviewed the mammograms before conducting US. In total, 120 lesions were found in 90 of the patients. Of these lesions, 35 (29.2%) were found in mammograms but were not observed during whole-breast compression US; 26 of the 35 lesions were isoechoic and the other 9 were less than 5 mm in diameter. Eighty-five lesions were observed with both methods. The author emphasizes the need for compression during breast US as well as mammography. Whole-breast compression US allows a view of the whole breast, which complements the views usually obtained by mammography. This method confirms the isoechoic nature of some breast lesions. Although mammography remains the gold standard for breast screening, the author suggests that whole-breast compression US with the patient in the sitting position be used for further assessment of both palpable and nonpalpable lesions, in addition to lesion-directed US.     

Late postoperative hemodynamic results and cineangiocardiographic findings after Mustard atrial baffle repair for transposition of the great arteries

Forty-nine patients with transposition of the great arteries who underwent a Mustard atrial baffle repair between 1964 and 1971 were assessed late postoperatively. There have been five late deaths: two related to baffle obstruction, two from noncardiac causes, and one sudden and unexpected. Hemodynamic data were available in 42 patients and autopsy in four. Obstruction of the lower venous channel was not encountered. Three patients had severe obstruction of the upper venous channel and in four there was mild restriction. Two patients had severe pulmonary venous obstruction resulting in late death; mild asymptomatic obstruction could not be excluded with certainty in six patients. Tricuspid incompetence was infrequently encountered in patients with an essentially intact ventricular septum. Left ventricular outflow tract obstruction was found in eight patients. In three it was present after satisfactory but incomplete surgical relief and in five it had not been recognized prior to operation. In only one of the latter patients was the obstruction important. Seventeen patients were operated on prior to one year of age. While baffle obstruction was confined almost entirely to these patients, the five youngest patients, aged one to nine weeks at operation, had adequate pulmonary and caval compartments at restudy two to three years later.     

Quantitative analysis of pulmonary vascular disease in complete transposition of the great arteries

Forty autopsy cases of complete transposition of the great arteries (TGA) and 22 autopsy cases of ventricular septal defect (VSD) were analyzed histologically for evidence of vascular damage due to pulmonary vascular disease (PVD). Positive correlations were generally observed between an index of pulmonary vascular disease (IPVD) and blood pressure of pulmonary circulation. No significant difference in IPVD was found between TGA and VSD in the first five months of life, when cases of each disease were compared at similar blood pressure levels. After that age, however, IPVD was much higher in TGA, and particularly severe PVD in this disease was demonstrated histologically. Morphometrical analysis of the pulmonary artery revealed hypertrophy of the muscular cost in response to elevated blood pressure. However, the progress of medial hypertrophy was retarded in TGA in the first five months, and medial thickness in arteries of cases of TGA older than five months was only 70% of that in VSD at the same blood pressure levels. Suppression of this process of reinforcement of the arterial wall in response to the stress of high pulmonary pressure was regarded as one of the important factors precipitating severe pulmonary vascular disease in transposition of the great arteries.