During a 10 year interval, between January 1, 1981 and January 1, 1991, primary repair of atrioventricular septal defect with a common atrioventricular orifice was performed in 95 patients younger than 1 year. Patients with atrioventricular septal defect having two atrioventricular orifices and a small ventricular septal defect, and those with severe hypoplasia of the left ventricle, were not included in the present study. According to the presence of associated anomalies, patients were divided into those with simple forms (72%) and those with complex forms (28%). These included parachute deformity of the left atrioventricular valve (10 cases), multiple muscular ventricular septal defects (5 cases), low-lying infundibular stenosis of the right ventricle (4 cases), obstruction of the left ventricular outflow tract (3 cases), tetralogy ofFallot (2 cases) and hypoplasia of the left ventricle (2 cases).

We report analysis of surgical results in 110 consecutive infants and children who underwent atrial repair of simple complete transposition using the Senning operation between February 1978 and May 1990. Mean age at operation was 5.4 months ± 6.1 (range 1 week to 4 years); 75 were less than 6 months old. There were 72 males and 38 females. Operative mortality rate was 5.5%, with one late death. Average follow-up is 48.1 months with 44 followed greater than 3 years, and 27 greater than 5 years. Postoperative cardiac catheterization was performed in 48 patients. Right ventricular ejection fraction averaged 0.52 ± 0.08 and was normal in 28 patients. Response of right ventricular ejection fraction to afrerload stress was abnormal in 12 of 14 patients tested. Right ventricular ejection fraction increased normally during exercise in 6 patients, but was abnormal in 15. Mild tricuspid regurgitation was noted in 12 patients. Mild obstruction of the superior caval vein was noted in 4 patients. Baffle leak requiring reoperation occurred in one patient. Fifty-seven of 90 patients are in sinus rhythm by latest electrocardiogram. Postoperative electrophysiologic studies were performed in 34 patients, and Holter monitoring in 25. Significant arrhythmia occurred in 26 patients: 6 patients required pacemakers for slow junctional rhythm or complete heart block; 20 additional patients have a junctional rhythm. Six patients have delayed sinus nodal recovery time. At last follow-up, 88 children (98%) are in New York Heart Association functional Class I, and 2 (2%) are in Class II. The Senning operation for compete transposition can be accomplished with a low operative and late mortality. Serious baffle complications requiring reoperation are rare. Surviving patients are clinically well, but arrhythmias and depressed right ventricular function may limit their long-term functional status.

Anatomic data are presented of a female abortus with the unusual combination of Ebstein's malformation and atrioventricular septal defect with right isomerism of the atrial appendages, left-hand ventricular topology and discordant ventriculoarterial connection. The common atrioventricular orifice was guarded by an effectively common valve, the right half being guarded by a well-formed three-leaflet valve, but the posterior and left lateral wall of the orifice being deficient of valvar leaflet tissue. The right ventricular inlet was a blind chamber, an imperforate dimple being seen which was surrounded by valvar remnants. The apical trabecular and outlet components of the right ventricle communicated with the left ventricle through an outlet ventricular septal defect in front of the anterosuperior leaflet of the atrioventricular valve, giving the potential clinically for the lesion to be misdiagnosed as double inlet left ventricle.

Cross-sectional echocardiography is an essential tool in the evaluation ofchildren with Kawasaki disease, both in the acute and chronic stages. In the acute phase of the illness, it is valuable for diagnosis and management of pancarditis and for the long-term monitoring of pericardial effusions, left ventricular function, and the rare cases of chronic valvar dysfunction. When coronary arterial abnormalities are detected, echocardiography can serially evaluate long-term treatment with drugs which prevent the aggregation of platelets and monitor the resolution of coronary aneurysms. The value of cross-sectional echocardiography, nonetheless, is very limited in the detection of coronary arterial stenosis. Coronary arteriography is still important for the diagnosis of obstructive lesions in the coronary arteries and should be used in conjunction with cross-sectional echocardiography for the appropriate long- term management of children with Kawasaki disease at high risk of developing coronary arterial stenosis. Perhaps, in the future, high resolution transesophageal echocardiography will allow clear delineation of coronary arterial anatomy and specifically stenosis, but its role in the evaluation and management of children with Kawasaki disease remains to be explored.

Formation of coronary aneurysms is often seen on the patients in the acute phase of Kawasaki disease by echocardiography, and after the convalescent phase, some patients show disappearance of aneurysms but some of them show combining stenotic lesions at inlets and/or outlets of aneurysms on coronary arteriography. According to follow-up studies of coronary arterial lesions, the degree of stenosis quite frequently increases and progresses over a period up to 10 years or more after the onset of the disease. The severe stenosis appears the most frequently at the proximal portion of the left anterior descending artery and secondly at the main trunk of the main coronary artery. They die suddenly or suffer from severe myocardial infarction unless they are treated with aortocoronary bypass surgery. However, it is rare to have symptoms of ischemic heart disease even in patients with severe obstructive lesions. In fact, the sensitivity of exercise testing in diagnosis of myocardial ischemia is very low. Dipyridamole thallium myocardial imaging is reasonably useful, but dipyridamole can he dangerous when it is given without the precise information of the patient's stenosis. Coronary arteriography is, therefore, essential not only to determine the appropriate treatment of the c ronary arterial lesions but also for follow-up. According to the histopathologic study, even the patients in whom coronary arterial lesions were not visualized on coronary arteriography often show some intimal proliferation. These patients may well present a problem in the future due to the sequels of angiitis. Because of this, continuous regular follow-up should be necessary, even in patients with no evidence of coronary arterial lesion.

We describe an unusual heart in which double outlet from the left atrium resulted in atrioventricular connections to a dominant left ventricle and to a left-sided rudimentary right ventricle, while the right atrium also connected to the left ventricle. This produced, in effect, double inlet left ventricle in association with discordant atrioventricular connections. The diagnosis was made by echocardiography and confirmed at post mortem examination.

The justification for the introduction of the arterial switch procedure was based, primarily, on concern regarding the long-term ability of the right ventricle to perform as the systemic pumping chamber. In this article, the functional performance of both the systemic and pulmonary ventricles after atrial redirection procedures and the arterial switch operation will be discussed.

Between 1968 and December 1990, 29 patients underwent 33 reoperations for dysfunction of the left atrioventricular valve after correction ofatrioventricular septal defect. Repair of the valve was possible in all 10 patients who, initially, had a common atrioventricular orifice, using straightforward procedures such as closure of the septal commissure and annuloplasties according to Wooler. Additional valvar abnormalities (double orifice; fenestration of leaflets; malpositioned or malformed papillary muscles; and additional clefts) were rare in this group of patients. In contrast, these anomalies were frequent in the 19 patients who, initially, had separate right and left atrioventricular valves.

Summary Mucocutaneous lymph node syndrome, now called Kawasaki disease, affects children of all ages and races. The cause of this systemic vasculitis is unknown, thus the diagnosis is based solely on clinical criteria. Coronary arteritis and the formation of coronary arterial aneurysms are the most serious early cardiac complications, while long term sequels, such as coronary stenosis and myocardial infarction, may occur. Current medical therapy is aimed solely at preventing or decreasing the occurrence of these cardiovascular complications in patients with this disease. Past treatment included antibiotics, steroids, and nonsteroidal anti-inflammatory agents. Aspirin remains the most widely used drug, although the use of intravenous gammaglobulin has also become common. When used in combination, these two agents may reduce the incidence of coronary arterial aneurysms. The optimal dosage and duration of treatment has not yet been determined.

We advocate primary repair of atrioventricular septal defect with a common atrioventricular orifice in symptomatic children presenting with intractable congestive heart failure. The single patch technique has been utilized as our procedure of choice. A low operative mortality and a low incidence of residual ventricular septal defect and regurgitation of the left atrioventricular valve requiring reoperation has been achieved. This report emphasizes the technical details of the repair. Careful delineation of anatomic detail is considered to be of the utmost importance. Precise suture placement, tailoring the patch, and reconstruction of the left atrioventricular valve are essential to achieve complete closure of the atrial and ventricular septal defects and functional integrity of the left atrioventricular valve.

Concern about long-term complications after intraatrial repair of complete transposition has been used as an argument in favor of “anatomic” repair by the arterial switch operation. Late arrhythmias, including loss of sinus rhythm and the development of supraventricular tachycardias, particularly atrial flutter, are widely reported after intraatrial repair. Despite modifications of technique, the electrophysiologic substrate for arrhythmia results from the extensive atrial surgery required. Arrhythmias occur, even in the “modern surgical era” after both Mustard and Senning operations, are progressive, and appear to be inevitable. The circulation after an intraatrial repair is more vulnerable to the effects of excessive tachycardia, and this may place the patient at risk from sudden cardiac death. Current attempts at individual stratification of risk are disappointing using even aggressive electrophysiologic approaches, and a combined assessment involving hemodynamics is likely to be necessary. The electrophysiologic and arrhythmic consequences of the arterial switch operation have been less extensively researched but, as might be expected, are quite different from those seen after intraatrial repair. The atrial activation sequence is relatively undisturbed, and sinus nodal dysfunction and supraventricular arrhythmia are uncommon. Ventricular extrasystoles are the arrhythmia most consistently found during the short follow-up currently available. In the longer term, myocardial ischemia, hemodynamic disturbances and autonomic imbalance may predispose to late arrhythmia. Current evidence would suggest that the lack of clinically significant arrhythmia and the restoration of the left ventricle to the systemic circulation are significant advantages of the arterial switch operation over intraatrial repair procedures.

With the general acceptance that the arterial switch operation represents the surgical treatment of choice for complex complete transposition, an increasing number of centers are leaning towards favoring this approach for simple transposition as well. As the arterial switch procedure is a more difficult surgical procedure than the Mustard or Senning operation, the learning curve has been longer and more hazardous. Centers with large referral bases have, understandably, enjoyed a more concentrated exposure to the arterial switch operation, and are now achieving early rates of mortality as low as those quoted for atrial repairs.

A retrospective analysis of 55 patients who underwent replacement of the left atrioventricular valve following surgery for atrioventricular septal defect with separate right and left atrioventricular valves or with a common valvar orifice has revealed a marked decrease in the need for valvar replacement over time. Between 1983 and 1987, the incidence of replacement following repair has been 3% in infants. Overall early mortality for the entire series, extending from 1970 to 1987, was 22%. Mortality in the first year of life was 58%, which was significantly greater than mortality beyond one year of age (14%, p<0.01). Obstruction of the left ventricular outflow tract was a frequent incremental risk factor for death during infancy. The incidence of complete heart block (25%) is disappointingly high, and may reflect distortion of the left atrioventricular junction in an attempt to place as large a prosthesis as possible. The incidence of complete heart block has not decreased with time. These results confirm the desirability of secondary valvoplasty procedures to enable a child to be larger before ultimate replacement of the valve is required.

Summary Myocardial revascularization is now an accepted therapeutic modality for severe coronary arterial obstructive disease produced by Kawasaki disease which is amenable to usual medical treatment. The mortality rate of myocardial infarction is surprisingly high in this setting, and surgery may be able to prevent many of these deaths. This article focuses on current issues in surgical treatment of ischemic heart disease in children secondary to Kawasaki disease. Coronary arterial obstructive disease is, apparently, a leading problem followed by valvar and myocardial involvements. Introduction of bypass grafting using the internal thoracic artery in children was a major recent advance, because this graft has an excellent capability to adapt to the increments of increased flow and growth of the patient. The saphenous vein is less satisfactory when used as a graft in terms of its long-term patency, particularly in young children less than 8 years old. Although longer follow-up is certainly required, the current results of surgery have been excellent in improving myocardial perfusion, in ameliorating symptoms, and improving the quality of life.

Kawasaki disease is an acute vasculitis of unknown etiology that occurs predominantly in infancy and early childhood. It is characterize by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy.1,2 Coronary arterial aneurysms, or ectasia, develop in approximately 15 to 25% of children with the disease, and may lead to myocardial infarction, sudden death, or chronic coronary arterial insufficiency.2–4

The outcome of primary repair in young infants having atrioventricular septal defect with a common atrioventricular orifice and regurgitation across the left atrioventricular valve is not yet satisfactory. We studied the significance of the characteristics of left ventricular volume and mass and the predicted wall stress for the outcome of repair in 13 infants with this lesion. Three patients died of left heart failure after operation, although neither residual shunting at ventricular level nor regurgitation across the left atrioventricular valve was present. End-diastolic volume and ejection fraction of the left ventricle were 228 ±66% and 0.65 ±0.06 of normal, respectively, with no difference between the survivors and non-survivors. End-diastolic thickness of the posterior ventricular wall, determined by echocardiogram, was within normal range for body size in all patients.

In 1989, we reported the results of surgical treatment in 11 patients with complete transposition and an intact ventricular septum or restrictive ventricular septal defect who had undergone a two-stage arterial switch procedure beyond the neonatal period with a short period (median 9 days) between banding of the pulmonary trunk and repair. The most common indication for the procedure was referral to our center after the neonatal period from a geographically distant center where the arterial switch procedure was not available. Other indications were multi-organ failure in the neonatal period which contraindicated corrective surgery at that time, as well as prematurity or late development of right ventricular dysfunction, which contraindicated correction at the atrial level planned at the referral center. In this report, we describe our continuing experience.