A.Louise Calder

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.

Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum

The incidence and severity of abnormalities of the coronary arteries were evaluated in 35 necropsy patients with pulmonary atresia and an intact ventricular septum. Right ventricular to coronary artery fistulous connections were found in more than 60% of the cases. All patients with fistulous connections had histologic abnormalities of the coronary arteries. In 50% the lesions were mild, with medial and intimal thickening producing up to moderate luminal stenosis. In 50% there was loss of normal arterial wall structure and severe narrowing or obliteration of the arterial lumen. The coronary arterial fistulas and histologic abnormalities were prevalent in those with underdevelopment of the tricuspid valve and right ventricular cavity but were not found in patients with a normal or dilated tricuspid valve anulus. The coronary arterial abnormalities were found in more than 80% of patients with a tricuspid valve/mitral valve ratio less than 1. A single coronary artery occurred in 6 patients (17%) of this series. In 80% of those with 1 coronary artery arising from the other, the aberrantly arising coronary artery crossed anteriorly to the pulmonary artery and could be at risk in surgical attempts to reconstruct the right ventricular outflow. Aortography is recommended if the coronary arteries are not clearly delineated on ventricular cineangiocardiography.

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.

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.

Predictors of long-term survival with Ebstein's anomaly of the tricuspid valve

In patients with Ebsteins anomaly of the tricuspid valve, predictors of death and indications for surgery are poorly defined. We retrospectively reviewed 48 patients with Ebsteins anomaly, 17 (35%) of whom presented in the first week of life. Duration of follow-up extended to 32 years (greater than 10 years in 35%). Twenty of the 48 patients (42%) died, 6 in the first week of life and 1 at age 5 months. Thirteen of the 41 patients surviving to age 6 months subsequently died, 50% probability of survival being reached at 47 years. Significant (p less than or equal to 0.05) predictors of death in this group were: male sex, cardiothoracic ratio greater than or equal to 0.65, New York Heart Association class III or IV, breathlessness and the absence of Wolff-Parkinson-White syndrome. Eight patients died suddenly. A cardiothoracic ratio greater than or equal to 0.65 was a better predictor of sudden death than functional status. All who developed atrial fibrillation died within 5 years. Other atrial arrhythmias were not helpful in predicting sudden death. In view of these findings, tricuspid valve surgery is recommended before the cardiothoracic ratio reaches 0.65, regardless of the symptomatic state.

Variant of tetralogy of fallot with absent pulmonary valve leaflets and origin of one pulmonary artery from the ascending aorta

Absent pulmonary valve leaflets and anomalous origin of a pulmonary artery from the ascending aorta have been reported as separate lesions associated with tetralogy of Fallot but not in combination. The clinical, radiologic and pathologic findings are presented in three patients with this previously undescribed combination. A search of the literature for similar cases revealed 233 patients with absent pulmonary valve leaflets and 56 patients with anomalous origin of a pulmonary artery from the ascending aorta; these cases are reviewed. Although 17 patients with absent pulmonary valve leaflets and tetralogy of Fallot also had absence of the proximal portion of the left pulmonary artery, none had an anomalously arising pulmonary artery from the ascending aorta. In the reviewed cases, anomalous origin of the right pulmonary artery from the ascending aorta was more frequent than that of the left pulmonary artery (44 compared with 12 cases, respectively), but the latter was more often associated with tetralogy of Fallot.

Assessment and follow-up of patients with ventricular septal defect and elevated pulmonary vascular resistance

Cardiac catheterization was undertaken in 87 patients (for a total of 89 studies) with ventricular septal defects, including 58 patients with moderate or severe elevation of pulmonary arteriolar resistance. When resting resistance was less than or equal to 7.9 U . m2, it always decreased with isoproterenol and no postoperative problems were experienced with pulmonary vascular obstructive disease. In 36 patients resting resistance measured greater than or equal to 8 U . m2. In 17 of these patients it decreased to less than 7 U . m2 with isoproterenol. Fifteen patients were operated on and postoperative problems with pulmonary vascular disease were experienced only in the single patient whose repair broke down. Surgery was undertaken in 4 of 19 patients in whom resistance did not decrease to less than 7 U . m2 with isoproterenol and advanced pulmonary vascular disease was evident in the 3 patients with follow-up observation. Correlation between measured resistance and other hemodynamic parameters was only fair. A pulmonary to systemic resistance ratio greater than or equal to 0.75 always indicated high absolute resistance but resistance ratios less than 0.75 were found quite frequently in the group with limited response to isoproterenol. These data argue that a reliable estimate of resistance, less than 7 U . m2, with a vasodilator predicts a good postoperative response regardless of measurements at rest or other hemodynamic parameters. Although observations on postoperative progress of patients with resistance greater than 7 U . m2 with a vasodilator are limited, a good postoperative course is unlikely unless resistance can be lowered to a level close to 7 U . m2.

Cardiac connections: The segmental approach to radiologic diagnosis in congenital heart disease

The segmental approach to the diagnosis and classification of congenital heart disease, which emphasizes the importance of the connections (proximal-to-distal relationships) of cardiac chambers and great vessels, is presented. The radiologic identification of great vessels and cardiac chambers and their connections is described, considering, in turn, three major segments: the atria together with the systemic and pulmonary veins, the ventricles with their atrioventricular valves and the great arteries with their outflow tracts. Cardiac and great vascular connections can be described with greatest clarity by using the atria as the starting point. The atrial situs is defined as solitus, inversus or ambiguus, the great veins connecting to the atria normally or anomalously. The ventricles may be connected to the atria in concordant or discordant fashion or a double inlet ventricle may be present. The ventriculo-arterial connections can be classified as normal, transposition, double outlet right ventricle or double outlet left ventricle. The angiocardiographic techniques and criteria that differentiate these connection disorders and identify the transitional cases between them are discussed and illustrated. A complete diagnosis must indicate not only the connections of the three major segments but also the malformations and abnormalities of spatial position that may be present. Certain associations are of value in predicting the positions and connections of the cardiac chambers and great vessels, making it possible to formulate helpful rules to aid the progress of a diagnostic study. The fallibility of such rules is discussed, emphasizing the need to define connection disorders in terms of the connections rather than in terms of malformations or of abnormalities in spatial position of the individual parts.

Progress of patients with pulmonary atresia after systemic to pulmonary arterial shunts.

Between February 1980 and June 1987, 42 shunts were placed in 39 infants with pulmonary atresia: 33 were modified Blalock-Taussig shunts with polytetrafluoroethylene (PTFE) and 9 were classic Blalock-Taussig shunts. There were four hospital deaths not related to the shunts. The remaining 35 patients were followed up for 1.6 months to 6.3 years (mean, 24.7 +/- 18 months). Repeat cineangiocardiographic studies revealed stenosis or distortion of the pulmonary arteries related to the site of the shunt in 11/22 patients (50%) with PTFE shunts and in 1/6 (17%) with classic Blalock-Taussig shunts; the stenosis was severe in only 1 patient. Mean increase in the pulmonary arterial index in the group with classic Blalock-Taussig shunts was 117 +/- 52 mm2/m2 (not significant) and in the group with PTFE shunts, 158 +/- 21 mm2/m2 (p less than 0.001). Late shunt occlusion occurred in 1 patient 23 months postoperatively. Thereafter, shunt patency rate remained at 94% +/- 6%. At the end of 1 year 81% +/- 7% of patients were judged to have adequate palliation, but between 2 and 3 years, only 60% +/- 10%. Univariate analysis showed that after 2 years the ranking order for successful palliation was classic Blalock-Taussig, 5-mm PTFE, and 4-mm PTFE shunts, but differences did not achieve statistical significance.

Double-outlet right ventricle with left ventricular-right atrial communication: Fibrous obstruction of left ventricular outlet by membranous septum and tricuspid leaflet tissue

Abstract Double-outlet right ventricle can be associated with obstruction of the left ventricular outflow tract of two types: muscular and fibrous. The muscular form can be corrected by open-heart surgery, but the fibrous type may prove to be inoperable by present methods. The angiocardiographic differential diagnosis of muscular and fibrous obstructions is considered briefly. A unique case of fibrous obstruction of the left ventricular outflow tract associated with double-outlet right ventricle was presented in a 35-day-old white male infant. The only outlet from the left side of the heart was a small left-ventricular-to-right-atrial communication. This is the first reported case of atrioventricular endocardial cushion tissue closely related to the tricuspid valve producing fibrous obstruction of the left ventricular outflow tract in association with double-outlet right ventricle. This case also illustrates that it is possible for double-outlet right ventricle to occur with aortic-atrioventricular fibrous continuity. A bilateral conus—persistence of the bulboventricular flang—is not a constant finding in double-outlet right ventricle.