Roxane McKay

Replacement of the aortic valve or root with a pulmonary autograft in children

Between January 1967 and December 1988, 34 patients ranging in age from 3 to 18 years (mean, 14 +/- 3.6 years) underwent replacement of the aortic valve or root with their own pulmonary valve. The indication for operation was left ventricular outflow obstruction in 16 patients (47%), aortic regurgitation in 14 (41%), mixed aortic valve disease in 3 (9%), and failure of a previously implanted aortic homograft in 1 (3%). There were four early deaths, all before 1971, giving a hospital mortality of 11.8% (70% confidence interval, 6% to 20%). Surviving patients have been followed up a cumulative total of 214 patient-years, the longest period of observation being 16 years 8 months. Late mortality was 13.3% (70% confidence interval, 7% to 23%), and 4 other patients required removal of the pulmonary autograft for endocarditis. Actuarial rates at 16 years were 74% +/- 11% for freedom from reoperation on the left ventricular outflow tract, 80% +/- 10% for freedom from reoperation on the right ventricular outflow tract, and 77% +/- 10% for late survival. There was no instance of primary structural degeneration in the pulmonary autograft, and all surviving patients were in New York Heart Association functional class I without medication. This experience demonstrates that the pulmonary autograft can achieve good early and medium-term results in young patients. Should growth potential be realized, it might constitute the ideal biological valve for the left ventricular outflow in children.

Aorto-ventricular tunnel

Aorto-ventricular tunnel is a congenital, extracardiac channel which connects the ascending aorta above the sinutubular junction to the cavity of the left, or (less commonly) right ventricle. The exact incidence is unknown, estimates ranging from 0.5% of fetal cardiac malformations to less than 0.1% of congenitally malformed hearts in clinico-pathological series. Approximately 130 cases have been reported in the literature, about twice as many cases in males as in females. Associated defects, usually involving the proximal coronary arteries, or the aortic or pulmonary valves, are present in nearly half the cases. Occasional patients present with an asymptomatic heart murmur and cardiac enlargement, but most suffer heart failure in the first year of life. The etiology of aorto-ventricular tunnel is uncertain. It appears to result from a combination of maldevelopment of the cushions which give rise to the pulmonary and aortic roots, and abnormal separation of these structures. Echocardiography is the diagnostic investigation of choice. Antenatal diagnosis by fetal echocardiography is reliable after 18 weeks gestation. Aorto-ventricular tunnel must be distinguished from other lesions which cause rapid run-off of blood from the aorta and produce cardiac failure. Optimal management of symptomatic aorto-ventricular tunnel consists of diagnosis by echocardiography, complimented with cardiac catheterization as needed to elucidate coronary arterial origins or associated defects, and prompt surgical repair. Observation of the exceedingly rare, asymptomatic patient with a small tunnel may be justified by occasional spontaneous closure. All patients require life-long follow-up for recurrence of the tunnel, aortic valve incompetence, left ventricular function, and aneurysmal enlargement of the ascending aorta.

Thrombolysis with tissue-type plasminogen activator following cardiac surgery in children

Three children with major intracardiac or vascular thrombosis following cardiac operations were treated with intravenous recombinant tissue-type plasminogen activator. The first patient, aged 10 yr, developed a left atrial thrombus following replacement of the mitral valve with a Björk-Shiley prosthesis. The second patient, aged 16 months, had a right atrial thrombus following a modified Fontan procedure for tricuspid atresia. Both were successfully treated with a short course of intravenous tissue plasminogen activator. The third patient, aged 19 months, developed the Budd-Chiari syndrome with occlusion of the inferior caval vein following a modified Fontan operation for double inlet left ventricle. Even though near-complete thrombolysis and recanalization of the inferior caval vein was achieved with three courses of tissue plasminogen activator on successive days, she died with failure of multiple organs. In all cases, the diagnosis was established by cross-sectional and Doppler echocardiography, and the response to therapy was monitored using the same technique. Thrombolytic therapy with systemic tissue-type plasminogen activator was safe and effective in the postoperative period, with no major haemorrhagic complications.

AN ANATOMICAL STUDY OF THE PATTERNS OF THE CORONARY-ARTERIES AND SINUS NODAL ARTERY IN COMPLETE TRANSPOSITION

The origin and course of the main coronary arteries and the sinus nodal artery are relevant to both intra-atrial and arterial relocation operations for complete transposition (the combination of concordant atrioventricular and discordant ventriculo-arterial connexions). The patterns of the main coronary arteries in 148 hearts with complete transposition have been documented together with the patterns of the sinus nodal artery in 105 of these. The terminology which has been used to describe these arteries has been explained. The findings illustrate the wide variation which exists in the origin and course of all these arteries in complete transposition and indicate areas where they are most at risk.

Cavopulmonary connection in repair of atrioventricular septal defect with small right ventricle

Between February 1991 and May 1992, 9 patients with a nonrestrictive inlet ventricular septal defect or complete atrioventricular septal defect and hypoplasia of the right ventricle underwent successful two-ventricle correction incorporating a bidirectional cavopulmonary anastomosis. Despite a satisfactory early postoperative course, large serous pleural effusions developed in 5 of them between 2 and 6 weeks after operation. Serial echocardiography showed an evolution of flow patterns in the superior vena cava from marked systolic reversal immediately after operation to nearly continuous forward flow into both pulmonary arteries about 6 weeks later. This pattern was consistent with the transition from cardiac-dependent to respiratory-dependent caval flow. The development of pleural effusions appeared to coincide with the loss of systolic caval flow reversal and resolved after establishment of a predominantly respiratory-dependent flow pattern.

Determinants of survival following repair of interrupted aortic arch in infancy

Between January 1971 and March 1987, surgery was performed in 26 infants with interrupted aortic arch. At operation the 14 boys and 12 girls weighted between 1.71 and 4.23 kg (mean +/- SD = 3.1 +/- 0.63 kg) and ranged in age from 2 to 90 days (13 +/- 18 days). The interruption was distal to the left subclavian artery in 4 (15%), between the left carotid and subclavian arteries in 20 (77%) and between the brachiocephalic (innominate) and left carotid arteries in 2 (8%). Associated complex cardiac lesions in 8 patients included complete transposition (2), common arterial trunk (2), aortopulmonary window (2), double inlet left ventricle (1) and tricuspid atresia (1). The remaining patients had an isolated ventricular septal defect. The arch was reconstructed with a prosthetic conduit in 14 patients; by a direct anastomosis in 6; using the subclavian artery in 3; and with the pulmonary trunk and the arterial duct in 2. Twenty patients (77%) underwent palliative surgery as the first stage of management, and banding of the pulmonary trunk was also performed in 16 of these. Five patients (19%) underwent primary complete repair of the interruption and intracardiac anomalies. One patient (4%) died soon after thoracotomy for palliative surgery. Of the 15 (57%, 70% confidence limits CL = 46-69%) early deaths, 7 occurred in patients with complex associated defects and 4 occurred when single stage repair was attempted. Survival following first-stage palliative surgery for arch interruption with isolated ventricular septal defect was 64% (9/14) [70% CL = 47-79%]. All of these patients subsequently underwent complete repair. Chi-squared and t-tests showed the year of operation and the type of operation (two-stage repair) to be associated with improved survival. It is concluded that a two-stage repair of interrupted aortic arch offers a reasonable alternative to primary complete correction and will lead to satisfactory subsequent repair in most cases.

Tricuspid Valve Replacement Using an Unstented Pulmonary Homograft

In consideration of the limitations of mechanical and stent-mounted tissue valves, a technique was developed for tricuspid valve replacement using an inverted pulmonary homograft. It was used successfully in a 4-year-old patient operated on for pulmonary atresia with intact ventricular septum and in a 3 1/2-year-old girl with Ebsteins anomaly.

Truncus Arteriosus With Interrupted Aortic Arch: Successful Correction in a Neonate

Truncus arteriosus (type II) with interrupted aortic arch (type B) was successfully repaired at 11 days of age using anterior translocation of the pulmonary arteries, resection of the ductus arteriosus, and direct anastomosis between the descending aorta and truncus. This technique permitted wide reconstruction of the aortic arch and minimized the number of suture lines. It also positioned the right ventricle-pulmonary artery conduit anteriorly, which may simplify its subsequent replacement.

Anatomical and Physiological Considerations

A sound anatomical knowledge is one of the three pillars upon which an accurate diagnosis must rest, the other two being physiology and pathology. Brock’s first principle in diagnosis, whether relating to the abdomen, chest or heart, was to establish initially the precise anatomical site of the lesion. Having determined this, he then asked ‘taking into consideration the history, age and sex of the patient, what is the most likely diagnosis?’ Using this simple approach, the diagnosis often became obvious, and these ground rules are equally applicable today.

Acquired Lesions of Major Blood Vessels

The thoracic aorta, passing from its intrapericardial ascending portion to the descending aorta in the posterior chest, falls within the realm of thoracic and vascular surgery as well as cardiac surgery. Similarly, the management of massive pulmonary embolus or peripheral vascular occlusion may fall to the physician or general surgeon, as well as the vascular, thoracic or cardiac specialist. This is largely because cardiopulmonary bypass is absolutely necessary only for lesions of the ascending aorta or transverse aortic arch.