The prevalence of congenital left main coronary artery atresia is very low. We report the characteristics and long-term outcomes of four children with left main coronary artery atresia. Three patients had heart murmurs due to mitral regurgitation at less than 1 year old. Their myocardial ischaemia worsened on exercise with aging. In the fourth patient, hypertrophic cardiomyopathy and Noonan syndrome were suspected at 1 year old. The development of communicating arteries between the conus branch and the left anterior descending artery was detected at 7 years old. The left main coronary artery atresia was confirmed by a selective coronary angiogram at 15 years old. Congenital left main coronary artery atresia could not be diagnosed by two-dimensional echocardiography; however, the left coronary arteries were small. Two patients underwent coronary artery bypass grafting of the left anterior descending artery using the left internal thoracic artery at 3 years and 6 years old, respectively. Two patients had an angioplasty with a cut back at the orifice of the left coronary artery at 2 years old and 17 years old, respectively. Two patients had no cardiac events without medication for more than 30 years after the operation. We must differentiate the diagnosis of left main coronary artery atresia in the small left coronary arteries with mitral regurgitation during the first year. Coronary artery revascularisation and mitral annuloplasty are needed. The long-term outcome of both coronary artery bypass grafting and angioplasty were good. The degree of mitral regurgitation after surgery may affect the prognosis.

We analyzed retrospectively the relationship between coagulation profile, and either hepatic function or hemodynamics, in patients who had undergone a Fontan-type procedure, comparing them, first, with a control group of 12 patients without significant hemodynamic abnormality, and, second, with a group of 14 patients who had not undergone a Fontan procedure, but whose mean right atrial pressure exceeded 8 mmHg. Follow-up catheterization had been performed in all 30 patients submitted to the Fontan-type operation. Prothrombin time, and factor XIII, were significantly lower in those who had undergone the Fontan procedure than in the other groups. Those submitted to the Fontan operation also had lower levels of protein C than controls, and their levels of plasminogen were lower than the patients with high right atrial pressure. Both aspartate aminotransferase and alanine aminotransferase were higher in those undergoing the Fontan procedure than in the other groups, while gamma-glutamyltranspeptidase in these patients was higher than in the control group. Mean right atrial pressure was highest in those under-going the Fontan procedure, while cardiac index was lowest. Prothrombin time was correlated to some extent with aspartate aminotransferase, mean right atrial pressure, and cardiac index. Protein C correlated with both aspartate aminotransferase and mean right atrial pressure, while factor XIII correlated with alanine aminotransferase, mean right atrial pressure, and cardiac index. Aspartate aminotransferase, alanine aminotransferase, and gamma-glutamyltranspeptidase, parameters of hepatic function, correlated significantly with mean right atrial pressure. In those who had undergone the Fontan procedure, decreased synthesis of pro-and anti-coagulant factors is a risk factor for both thrombosis and bleeding. Abnormal hemodynamics, in the absence of a right sided pumping chamber, may predispose to subclinical hepatic dysfunction, leading to selective disturbances of protein synthesis.

Objective: To determine the efficacy of anatomic biventricular repair by a combination of intraatrial and intraventricular re-routing in patients with isomerism of the left atrial appendages. Methods: Anatomic biventricular repair by means of combined intraratrial and intraventricular re-routing was achieved in 5 of the 63 patients with left isomerism in whom we attempted a definitive surgical procedure. The inferior caval vein was interrupted in 3. The Mustard procedure was chosen for intraatrial redirection of blood in 4, and the Senning procedure in the other. Intraventricular re-routing was carried out via a right ventriculotomy in all patients, using an external conduit to reconstruct the morphologically right ventricular outflow tract. Results: One patient died one month after the procedure because of low cardiac output and bronchial bleeding. Obstruction across the superior caval venous channel occurred after the Senning procedure in this particular patient, related to the interrupted inferior caval vein draining via the azygous vein. The other 4 patients are currently doing well. Postoperative catheterization showed excellent cardiac performance, with no obstruction across the venous channels or the ventricular outflow tracts in these 4. No episode of significant archythmia has been noted, all patients having a regular atrial rhythm, although the P wave vector was unusual in each patient. Reoperation has been needed thus far in one patient, 128 months after the initial repair, because of obstruction of the external conduit. Conclusion: With precise recognition of the morphologic features, a combination of intraatrial and intraventricular re-routing can successfully be established in patients with isomeric left atrial appendages, with functional results in the intermediate term being reasonable.

A one-stage repair was performed for correction of the intracardiac malformations associated with coarctation of aorta in five pateints or interrupted aortic arch in eight patients. The ages ranged from four to 294 (median 35) days. The anomalies within the heart were a ventricular septal defect with or without subaortic stenosis (n=7), an aortopulmonary window (n=2), common arterial trunk (n=2), aortic valvar stenosis (n=1) and the TaussigBing anomaly (n=1). Surgery was performed through a median sternotomy employing cardiopulmonary bypass with moderate to deep hypothermia. In terms of the aortic reconstruction, an extended direct anastomosis was performed in 10 patients, while a vascular graft was interposed in three. So long as the aortic cannula did not interfere with the proximal anastomotic site on the aorta, circulatory arrest was avoided. As for surgery within the heart, the ventricular septal defects were closed via the right atrium with myotomy and myectomy if a morphological substrate for subaortic stenosis was confirmed (n=4). There were two hospital deaths (15.4%) due to low cardiac output. In patients who underwent myotomy and myectomy for subaortic stenosis, the postoperative pressure gradient across the aortic valve was negligible. We conclude that surgical results of one-stage repair for the intracardiac malformations associated with interrupted aortic arch or aortic coarctation are reasonable. We suggest that the early relief of obstruction within the left ventricular outflow tract may have played some role in the favorable outcome.

We found right-to-left shunts through the cardiac veins postoperatively in 2 patients who had undergone the Fontan procedure. In one of the patients, channels were present through the cardiac veins independent of the coronary sinus. In the other patient, an atretic orifice for the coronary sinus, coupled with a persistent left-sided superior caval vein, complicated the postoperative course.

Five infants under three months of age with tetralogy of Fallot and absence of the leaflets of the pulmonary valve were successfully corrected using a one-stage repair. All patients suffered from respiratory failure preoperatively and surgery was performed somewhat urgently. The age at surgery ranged from 26 to 72 days, with an average of 47±17 days. The surgical procedures involved four steps. First, we plicated the aneurysmally dilated central pulmonary arteries. Second, we closed the ventricular septal defect. We then inserted a handmade heterograft pericardial valve at the site of the pulmonary valve, and completed the reconstruction of the right ventricular outflow tract with a patch. All patients survived. Two of them were successfully weaned from ventilation at early (two and 11 days, respectively) periods after surgery, but the remaining three patients required artificial ventilation for one to two months. We conclude that one-stage repair is possible for this anomaly, but that plication of the pulmonary trunk is an essential technique todeliver patients in early infancy from respiratory failure.

A new surgical technique is described to reduce the flow through acquired systemic-pulmonary collateral arteries, a complication which has been recognized as a risk factor in patients undergoing the Fontan operation. When systemic-pulmonary collateral arteries have developed subsequent to previous construction of an ipsilateral Blalock-Taussig shunt or a Glenn anastomosis, and are identified by angiography in advance of the Fontan operation, the technique is indicated as a preparative procedure. It consists of rethoracotomy aimed at isolating the lung from the thoracic wall (peeling), and wrapping of the upper half of the lung with expanded polytetrafluoroethylene sheets to avoid redevelopment of collateral vessels into the lung (wrapping). This decreases the systemic-collateral flow through small vessels which cannot be handled by conventional embolization using catheterization techniques, and permits the Fontan operation to be performed more safely.

A simple technique is described with which to relieve postoperative compression of the heart. Although delayed sternal closure has conventionally been used to deal with postoperative compression, this maneuver always exposes the patient to the risk of infection. We have recently employed a simple method of sternal traction when there are signs of compression on temporary sternal closure. One or two couples of sutures in the sternum are used supported by bags of saline. This simple and effective method is applicable to patients, but especially to infants and neonates, without worrying about overindications.

A 29-year-old woman who had tetralogy of Fallot and pulmonary atresia with major aortopulmonary collateral arteries developed aortic regurgitation due to infectious endocarditis. Isolated aortic valve replacement was undertaken because of heart failure unresponsive to medical therapy. Excessive flow through the aortopulmonary collateral arteries during cardiopulmonary bypass was controlled by means ofintraaortic balloons which were inflated to occlude the origin of the collateral arteries. Arterial return from the pump was delivered through separate cannulation of the ascending aorta and the right femoral artery. We believe that this method should be considered in those circumstances when excessive flow through systemic-to-pulmonary arteries is expected during cardiopulmonary bypass.

Believing early repair to offer major benefits, we have repaired tetralogy of Fallot with pulmonary atresia in five infants aged less than six months. The ventricular septal defect was closed via a right atriotomy. The right ventriculotomy was 30±3% of the right ventricular length. The posterior wall of the right ventricular outflow tract was created by anastomosing directly the pulmonary trunk to the right ventriculotomy, or either by interposition of the left atrial appendage or an autologous pericardia! flap. The pathway was then roofed over with an equine pericardia! patch. All patients survived and are now doing well from 18 to 41 (31±11) months after the repair, although one patient required reoperation for relief of stenosis at the site of an anastomosis between the pulmonary arteries and the interposed left appendage. In the other patients, postoperative sequential echocardiography has shown no obstruction in the right ventricular outflow tract, nor significant pulmonary or tricuspid regurgitation. We conclude that primary repair in early infancy is an excellent option for surgical treatment of tetralogy of Fallot with pulmonary atresia.

Objective: To investigate our surgical results of intraventricular rerouting in patients having double outlet right ventricle with doubly-committed ventricular septal defect. Methods: We undertook repair in 8 patients with this particular feature. Of these, 2 patients had pulmonary stenosis, and another had interruption of the aortic arch. The subarterial defect was unequivocally related to both the aortic and the pulmonary orifices in all, albeit slightly deviated towards the aortic orifice in one, and towards the pulmonary orifice in another. Intraventricular rerouting was carried out via incisions to the right atrium and the pulmonary trunk. To ensure reconstruction of an unobstructed pulmonary pathway, a limited right ventriculotomy was made in 5. Results: All patients survived the procedure, and are currently doing well, with follow-up of 25 to 194 months, with a mean of 117 ± 68 months. Catheterization carried out 16 ± 6 months after repair demonstrated excellent ventricular parameters. Mean pulmonary arterial pressure was 16 ± 7 mmHg, being higher than 20 mmHg in 2 patients. No significant obstruction was found between the right ventricle and the pulmonary arteries. A pressure gradient across the left ventricular outflow tract became significant in one patient in whom a small outlet septum was present, and a heart-shaped baffle had been used for intraventricular rerouting. Reoperation was eventually needed in this patient for treatment of the obstruction, which proved to be progressive. Conclusion: Precise recognition of the morphologic features is of paramount importance when choosing the optimal options for biventricular repair in patients with double outlet right ventricle and doubly-committed interventricular communication.

On the assumption that the diameter of the reference vessel might determine the thickness of neointimal coverage of stents placed in the pulmonary arteries, we analyzed the angiograms of 28 lesions in 17 patients who underwent follow-up cardiac catheterization. Excluding 2 lesions where late stenosis was determined mainly by recoil of greater than 30%, we investigated the neointimal thickness of 26 lesions in 16 patients. Several factors that might contribute to late re-stenosis were also analyzed. Age and body weight at implantation ranged from 0.8 to 20 years, with a median of 6 years, and from 6.8 to 77.5 kg, with a median of 17.6 kg. Follow-up interval was from 6 to 15 months, with a median of 6 months.

There was a significant increase in diameter, as well as a reduction in pressure gradient, immediately after the implantation of stents. Although there was no significant difference between the achieved diameter and the diameter of the stent at follow-up, the diameter of the lesion at follow-up was significantly smaller than the diameter achieved by stenting. The increase in the pressure gradient at follow-up was slightly greater in 4 lesions where the late reduction in diameter was greater than 30% than in the 18 lesions where this was less than 30% (p = 0.05). The diameter of the reference vessel, and the diameter by stenting correlated with the late reduction in diameter. All lesions with diameter reduced greater than 30% had a reference diameter of less than 6.1 mm, and a diameter achieved by stenting of less than 6.5 mm. Late loss in luminal diameter directly correlated with the thickness of the neointimal coverage. In conclusion, close observation should be mandatory following implantation of stents in small pulmonary arteries.

The authors achieved biventricular repair in a 1-year-old boy having double outlet right ventricle and unusual straddling of the mitral valve. There was a substantial abnormal muscular bar present within the right ventricle, and some of the tendinous cords of the mitral valve were attached to this structure. Preoperative echocardiography was particularly informative in enabling the surgeon to arrange the optional operative procedures. The postoperative course was smooth and uneventful.

We report our experience of aortic valvar replacement subsequent to the arterial switch procedure, incorporating the Lecompte maneuver, in patients with complete transposition. The anterior wall of the neoaorta was incised in continuity with the posterior wall of the pulmonary trunk. The aortic root was reconstructed with or without augmentation using a patch after the operative procedures to the aortic valve. With this approach, the neoaortic root could readily be exposed without extensive dissection, and valvar replacement was safely achieved. These patients experienced no postoperative complications.

Background: To determine the clinical relevance of change in the level of lactate in the serum as a meanings of establishing the efficacy of the circulation immediately after the Fontan procedure in small children. Methods: We measured the concentration of lactate in the serum consecutively in 30 patients undergoing the Fontan procedure, without fenestration, under the age of 2 years. Of these, we did not use cardiopulmonary bypass in 13. Results: Concentrations gradually increased during the first several hours after establishment of the Fontan circulation, and then eventually decreased, reaching the normal range within 48 hours, with specific values of 0.9 plus or minus 0.3 pre-operatively, 2.3 plus or minus 1.1 immediately after creation of the Fontan circulation, 4.0 plus or minus 2.4 at 6 hours, 1.6 plus or minus 0.6 at 24 hours, and finally 1.3 plus or minus 0.4 millimoles per litre at 48 hours. This trend was irrespective of use or no use of cardiopulmonary bypass. The higher initial level in the intensive care unit was related to higher systemic venous pressure, higher transpulmonary pressure gradient, higher maximal level of alanine transaminase and blood urea nitrogen, and longer duration of peritoneal drainage, the latter as a monitor of sequestration of fluid rather than peritoneal dialysis. In patients with initial levels greater than 3.0 millimoles per litre, there was extended duration of endotracheal intubation. Conclusions: Postoperative changes in the levels of lactate in the serum subsequent to creation of the Fontan procedure were peculiar, not necessarily correlated with the cardiac output, but relevant to the clinical course.