Brian G. Barratt-Boyes

Results after repair of coarctation of the aorta beyond infancy: A 10 to 28 year follow-up with particular reference to late systemic hypertension☆

The late outcome in 160 patients aged 1 to 54 years who had surgical repair of coarctation of the aorta was examined 10 to 28 years postoperatively. Twenty years postoperatively the probability of survival of patients discharged from the hospital aged 1 to 19 years at operation was a little less than that of the general population (95% versus 97%). The discrepancy between patients and the general population was more marked in those aged 20 to 39 years and was grossly different when surgical repair was undertaken beyond age 40. There were 19 late deaths (12%), 79% due to cardiovascular disease. Thirteen patients had a poor result because of recoarctation (11 patients) or the development of complications at the site of repair (2 patients). Most patients were hypertensive before operation. The frequency of hypertension decreased markedly in the first few postoperative years. Blood pressure was normal in most patients 5 to 10 years after operation, but when followed up for longer periods the proportion of patients with hypertension increased. Hypertension was more common in patients operated on after 20 years of age than in those aged 5 to 19 years at operation (p = 0.007). The likelihood of being alive without complications and with a normal blood pressure was 69% at 10 years, 55% at 15 years, and 20% at 25 years postoperatively.

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

OBJECTIVES This study examines the late outcome in patients with simple transposition of the great arteries (TGA) after a Mustard operation. BACKGROUND Continuing medical follow-up for patients after the Mustard procedure, now extending to three decades, is required. The quality of life of adult survivors has not been well documented. METHODS Survival and quality of life among 113 hospital survivors of the Mustard operation performed for simple TGA between 1964 and 1982 were assessed by medical review and a lifestyle questionnaire. The incidence of right ventricular failure and echocardiographic right ventricular dysfunction (RVD) were determined. A measure of lifestyle, the ability index, was determined. RESULTS Actuarial survival was 90%, 80%, and 80% at 10, 20, and 28 years, respectively, with 76% of survivors being New York Heart Association class 1. Sudden death, with an incidence of 7% without identifiable risk factors, was the most common cause of late demise. RVD was identified in 18% of patients who had echocardiography, but there was right ventricular failure in only two patients. Seventy-five percent of current survivors lead a normal life, 20% have some symptoms or lifestyle modification, and 5% are unable to work. CONCLUSIONS The survival of patients to 28 years with the Mustard repair has been good. Late sudden death is the most worrisome feature. There is a 97% freedom from right ventricular failure to date. The quality of life of late survivors is good, most achieving a normal level of education and employment.

Long-term follow-up of patients with the antibiotic-sterilized aortic homograft valve inserted freehand in the aortic position.

A series of 252 isolated aortic homograft valves in 248 patients have been followed for 9 to 16.5 years (mean 10.8). The valves were sterilized in antibiotic solution and stored in a nutrient medium at 4 degrees C and were nonvital. There were 15 in-hospital deaths (6%) and a mortality of 2.7% in patients undergoing an elective first operation. Actuarial survival with the study valve in situ was 57% at 10 years and 38% at 14 years. Only 8.4% of the patients died late from homograft valve failure, chiefly because of failure to refer patients with endocarditis for reoperation or because reoperation was refused in elderly, frail subjects. Incompetence was the sole cause of valve failure and was due either to valve wear or endocarditis. Significant incompetence required reoperation. On actuarial analysis, freedom from significant incompetence for the entire group was 95% at 5 years, 78% at 10 years, and 42% at 14 years. Factors increasing the risk of significant incompetence due to valve wear on multivariate analysis were increasing donor valve age (greater than or equal to 55 years), recipient age (less than 15 years), and an aortic root diameter over 30 mm. Analysis of the patient group that excluded each of these variables (low-risk group), which comprised 61% of the study population, indicated freedom from significant incompetence due to valve wear of 98% at 5 years, 94% at 9 years, and 56% at 13 years.

Intracardiac Surgery in Neonates and Infants Using Deep Hypothermia with Surface Cooling and Limited Cardiopulmonary Bypass

In an attempt to achieve safe intracardiac surgery in severely ill babies with congenital heart defects, a technique of deep hypothermia with surface cooling and limited bypass has been used. Under halothane anesthesia, these infants were cooled to a nasopharyngeal temperature of 26 C on a circulating water blanket and the temperature was lowered further to 22 C by a short period of total body perfusion. After a period of circulatory arrest which averaged 48 minutes at 22 C, during which the intracardiac repair was carried out, rewarming to 32 C was achieved by 20 minutes of total body perfusion and final rewarming to 36 C by surface means.Thirty-three of 37 infants under 10 kg in weight, with correctable lesions, survived this procedure, including 25 aged 8 days to 12 months. Conditions corrected were transposition (13), tetralogy of Fallot (9), total anomalous pulmonary venous connection (6), ventricular septal defect (6), and atrioventricular canal (3). The technique gave ideal operating conditions and is believed to have wide application in the neonatal and infant group.

Prognosis after recovery from first acute myocardial infarction: Determinants of reinfarction and sudden death

Factors associated with total cardiac mortality, sudden cardiac death and reinfarction were studied in 325 male survivors aged younger than 60 years of age (mean 50) of a first myocardial infarction (MI). All patients had undergone exercise testing and cineangiocardiography 4 weeks after MI, 24% underwent coronary artery surgery and 30% received beta-blocking therapy. Patients were followed 1 to 6 years (mean 3.5). Total cardiac mortality was best predicted by the left ventricular (LV) ejection fraction (EF) and by a coronary prognostic index. In contrast, neither the severity of coronary arterial lesions measured with a scoring system nor the results of the exercise test gave significant prediction of mortality. Of the 2 major late sequelae of MI, reinfarction could not be predicted by any clinical or cineangiocardiographic variable. However, sudden death not associated with reinfarction was significantly more common (p less than 0.001) when EF was less than or equal to 40% than when it was greater than 40%. Comparison of patients with an EF less than or equal to 40% who did or did not die suddenly showed that LV dilation (high volumes at ventriculography) was an added risk factor, but the extent of coronary occlusions and stenoses was not. It is concluded that, at least for groups of patients treated with standard modern methods after MI, the main determinant of medium-term survival is the extent of LV damage. The state of the coronary arteries and the presence of ischemic myocardium during exercise are only of secondary importance for survival.

Outcome of pregnancy after the mustard operation for transposition of the great arteries with intact ventricular septum

OBJECTIVES This study examined the outcome of pregnancy in patients with transposition of the great arteries and an intact ventricular septum after a Mustard operation. BACKGROUND Before the introduction of surgical treatment, most children with transposition of the great arteries died in early infancy. A number of these patients have now reached their reproductive years. There is little information about the effect of pregnancy on cardiovascular status, particularly the ability of the right ventricle to adjust to the hemodynamic changes of pregnancy. The outcome for the offspring and their risk of congenital heart disease are also unknown. METHODS Twenty-three female late survivors after the Mustard operation > 15 years of age were reviewed in relation to the occurrence of pregnancy and its outcome. Serial echocardiographic estimates of right ventricular volume during pregnancy were made in three local patients. RESULTS Nine women had 15 pregnancies. They were asymptomatic before pregnancy and remained free from cardiac symptoms during each pregnancy. Right ventricular volume in the three patients studied increased during pregnancy but returned to normal at 8 to 11 weeks postpartum. There were 12 live births, 2 spontaneous abortions and 1 intrauterine death. None of the liveborn infants had evidence of congenital heart disease. CONCLUSIONS In this small group of women with good quality late survival after a Mustard operation, pregnancy was well tolerated. We suspect that the incidence of congenital heart disease in infants of mothers with transposition of the great arteries will be at the lower end of the range for mothers with different types of congenital heart disease, but further data will be needed to confirm this.

Six year review of the results of freehand aortic valve replacement using an antibiotic sterilized homograft valve.

The long-term behavior of an antibiotic-treated homograft aortic valve inserted in a freehand fashion was assessed in 121 patients operated upon for aortic valve disease and followed from four to six and one-half years. There were seven hospital deaths (5.7%) and 30 late deaths, only one of which was related to the homograft valve. The six year survival was 69% (77% for single valve and 52% for multiple valve surgery). At six years 9% had important homograft aortic valve incompetence (HAVI) and most of these required reoperation. Important HAVI occurred in only 5% of patients with an aortic root diameter < 24 mm and in 38% of those with a markedly dilated or distorted proximal aorta (P < 0.01). The freehand aortic homograft was considered superior to prosthetic devices because of the absence of chronic anticoagulation, thromboembolism, sudden death from valve failure and significant obstruction in a small aortic root. With slightly restricted patient selection the valve failure rate is expected to fall to less than 1% per year.

Prosthetic repair of coarctation of the aorta with particular reference to Dacron onlay patch grafts and late aneurysm formation.

Prosthetic material was used in the repair of coarctation of the aorta in 72 patients followed for 8 months to 24 years after operation. False aneurysms occurred late postoperatively in 1 of 17 instances when a tube graft was used to replace the resected aorta and in 1 of 2 patients when the graft bypassed the obstructed area. A Dacron onlay patch graft (DOPG) was used in 52 patients as the primary method of repair, to revise a previous repair or as an adjunct to another procedure at the time of initial operation. True or false late aneurysm occurred between 6 and 18 years postoperatively in 5 of the 38 patients in whom a DOPG was used for primary repair of the coarctation or to revise an earlier repair, an actuarial incidence of 38% at 14 years. Diagnosis of aneurysm formation late postoperatively depends on awareness of this complication and careful assessment of lateral as well as posteroanterior chest radiographs. DOPG should be avoided whenever possible.

Developmental progress after cardiac surgery in infancy using hypothermia and circulatory arrest.

Seventy-two of 76 long-term survivors who had surgical repair of congenital heart lesions at 11 days to 26 months of age using profound hypothermia and circulatory arrest underwent psychometric testing (Stanford-Binet) late postoperatively. The mean IQ of the 72 patients was 92.9 ± 16.5 (SD). Stanford-Binet scores bore no relationship to the duration of circulatory arrest or other aspects of surgical technique. Scores were significantly lower in those who had a low birth weight for gestational age, important neurologic problems preoperatively or were in the lower socioeconomic classes. An “ideal” control group of 69 children randomly selected from patients satisfying certain criteria based on birth and neonatal characteristics had a mean IQ of 106.2 ± 11.6. Twenty-five patients who had surgical treatment of congenital heart disease met the criteria for the control group except for their heart lesions. Their mean IQ was 101.4 ± 15.0 (NS). We could not demonstrate any significant deleterious effect that could be attributed to the surgical methods. Rather, the postoperative IQ scores reflected characteristics related to individual patients.

Double outlet left ventricle. Morphology, cineangiocardiographic diagnosis and surgical treatment.

In double outlet left ventricle,both the pulmonary artery and the aortaarisefromthemorphologicleft ventricle. This paper presents the anatomic and cineangiocardiographic features of five patients who had this condition proved at surgery or autopsy. The condition of the first patient was incorrectly diagnosed as transposition of the great arteries with pulmonary stenosis and ventricular septal defect; the anatomic features were correctly interpreted at operation in 1966 and appropriate repair was made, but the patient died postoperatively. The condition of the other four patients was correctly diagnosed. The second patient had Ebsteins malformation of the tricuspid valve and hypoplastic right ventricle in addition to double outlet left ventricle; her condition was not considered operable. Two patients, one with visceroatrial situs solitus, concordant d-loop and dextrocardia, were surgically treated with good long-term results. The fifth patient died 2 years postoperatively and is the first patient reported to have double outlet left ventricle with visceroatrial situs inversus, a discordant d-loop and levocardia. The segmental approach to the classification and diagnosis of connection disorders is discussed and the radiologic criteria by which double outlet left ventricle may be diagnosed considered in detail. The importance of using the radiologic projection (usually left anterior oblique) that profiles the ventricular septum is emphasized because the diagnosis can be established only by delineating the relations of the origins of the great arteries to the ventricular septum. The surgical significance of other anatomic features, including the number, size and position of ventricular septal defects and conal malformations, is also discussed.