There are many congenital malformations of the mitral valve which produce valvar insufficiency. From a surgical point of view, systems based exclusively on anatomic analysis are not always entirely appropriate for assessment of these lesions. With this in mind, Carpentier proposed a functional approach for analysis based upon the motion of the valvar leaflets. From 1969 to 1994, 135 children under the age of 12 (mean age: 5.8 + 3.15 Y, 0.6–12Y) underwent surgery in our department, basing treatment on such analysis. Since motion of the leaflets during the operation is compromised by cardioplegia, and sometimes exposure can be however difficult, preoperative echocardiography was a mandatory part of the diagnostic cascade.

Normal motion of the leaflets was present in 41 patients, with deformation of the annulus in 14, a cleft in 21, and partial agenesis in 6. Prolapse of leaflets was present in 42 patients. Leaflet motion was restricted in 28 patients. These were divided in two groups, one with normal papillary muscles and commissural fusion or short cords. The other with abnormal papillary muscles producing a parachute arrangement in 6 and a hammock valve in 9. Associated lesions were present in 47% of the patients. Conservative surgical procedures following the precepts developed by Carpentier were used in 127 patients. Valvar replacement was necessary in 8 patients. Operative mortality was 4%. Mean follow up was 8.4 ± 5.3 years (1–23Y). Actuarial survival at 5 years was 90 ± 6% and, at this time, was stable. No thromboembolic events occurred after conservative surgery. The reoperation rate was 5% for those undergoing repair (6 patients). We conclude that the functional classification developed by Carpentier is a reliable and robust approach to these complex lesions. Conservative surgery is feasible in most of the cases presenting with congenital mitral valvar insufficiency. Results are stable and reliable. Surgery should be undertaken before the onset of left ventricular deterioration.

Lesions producing congenital mitral valvar stenosis are complex and often multiple, From 1970 to 1994, 58 patients under 12 years of age (mean 5 ± 3.2 y) underwent surgical treatment for such malformations in our department. In order to provide a clear understanding of the pathology, we used a classification based on anatomy and function. Echocardiography was a major tool of the preoperative investigations. The lesions were classified as follows:

Normal papillary muscles: commissural fusion in 19, excess leaflet tissue in 2, valvar ring in 12, and annular hypolasia in 2.

Abnormal papillary muscles: paachute arrangement in 10 and hammock mitral valve in 13.

Associated malformations were found in 63%, the most common being multiple stenosis in the left heart. Conservative treatment was possible in 54 patients (95%) using the techniques developed by Carpentier. Hospital mortality was 22%, with 95% confident limits between 13 and 36% (13/58). Of the 13 patients with a hammock valve, 6 died in hospital. The overall mean period of follow up was 7.8 + 5 years. No thromboembolic events occured after conservative surgery. Late death occured in 3%. The actuarial survival at 5 years was 64 + 15%. Reoperation was needed for residual mitral valvar stenosis in three patients or insufficiency in one.

In spite of effective techniques to relieve mitral obstruction, 12% of our patients need early or delayed replacement of the valve. The hospital mortality remains high due to the complexity of the lesions, associated with the associated malformations. Echocardiography proved to be extremly helpful for the functional analysis. In order to improve operative results, simple and early valvar repair, even if considered palliative, seems to be appropriate.

Form 1980 until January 1991, 76 patients with double outlet ventricle, aged from 1 year 7 months to 23.5 years (mean age 7.5± 5.0 years) underwent surgey at the A.N. Bakoulev Inste for Cardivascular Surgery. Associated heart defects were found in 77.6% patints. In 57 patients (75%), the left ventricle opened into the subaortic infundibulum; into the subpulmonary infudibulum in four (the Tassisg-Bing anomaly) while, in 12 (15.8%), it opened under the outflow tracts of both great arteries. In two cases, (2.6%) there was a mno-committed ventricular septact. In one case (1.3%)a non-committed defect was diagnosed as coexisting with a non-commited defect was dignosed as coexisting with a Taussig-Bing anomaly. There were eight operative deaths (10.9%) among the patients undergoing radical surgery.Another theree patients with the Taussig-Bing anomaly and obstructive pulmomnary vascular disease underwent a successful palliative Mustard operation. All deaths were associated with acute heart failure and were seen only among patiens with subaortic ventricular septal defects. The important factors, which increased the risk of correction and contributed to death were inappropriate pre-operative diagnosis of complex associated defecrs and techmical mistakes during surgical intervention, the latter due to inadequate surgical experience. During the last five years, mortality decreased significatly (one death among 34 patients undergoing surgery from 1986 through January 1991)

From January 1988 through to July 1994, 54 consecutive infants underwent an arterial switch operation for simple or more complex forms of complete transposition (concordant atrioventricular and discordant ventriculo-arterial connections). They ranged in age from 2 to 180 days. The Lecompte maneuver was performed in all. In the first 19 patients the harvested sinuses of Valsalva were filled with two separate patches of autologous preserved pericardium, while, in the last 35 patients, a wide pantaloon patch of tanned pericardium was employed. Five babies died within 30 days after the procedure (operative mortality of 9.2%, 70% CL 4–17%). Four survivors were found to have developed a significant supravalvar pulmonary stenosis from 1 month to 21 months postoperatively. Echocardiographic data showed a transpulmonary peak systolic gradient from 60 mmHg to 101 mmHg, with a right-to-left ventricular systolic pressure ratio from 0.65 to 0.9. Reoperation was performed from 8 months to 39 months after the arterial switch procedure. Supravalvar pulmonary stenosis was located at the level of the pulmonary trunk, extending distally in two cases, due to the growth of fibrous scarring tissue with partial calcification. The pulmonary valvar leaflets were involved in two cases. Relief of the obstruction was obtained by insertion of a wide shield-tailored polytetrafluoroethylene patch after making an inverted Y-shaped longitudinal incision in the pulmonary trunk between the anterior sinuses of Valsalva. No mortality occurred at reoperation. Early and midterm echocardiographic measurements showed the effectiveness of this technique, with only trivial or mild residual transpulmonary gradients.

Thrombosis of the right side of the heart has previously been a rarely reported early or late postoperetive complication after a modified Fontan-procedure.

In 4(5.5%) of 72 surviving patients who underwent a total cavopulmonary connection for various forms of univentricular atrioventricular connection or other complex lesion a thrombus within the right atrium was diagnosed by transthoracic echocardiogaphy either early (in 3 cases) or late (in one cases) postoperatively. Predisposing haemodynamic abnormalities could be identified in all four patients, but no patient was found to have severe abnormalities of coagulation. Two patients presented with acute clinical symptoms on the third and twelfth day after initial surgery. They underwent emergency reoperation with thrombectomy followed by prophylactic treatment with coumadin or aspirin after initial heparinization. In the other 2 patients, routine postoperative echocardiography revealed a right atrial thrombus without specific clinical symptoms 25 days or 3 months postoperatively. Thrombolytic therapy with an initial bolus of tissue plasminogen activator (0.1 mg/kg) followed by continuous infusion (1 mg/kg/day) combined with heparinization yielded complete resolution of the thrombus after 27 and 40 hours, respectively, as demonstrated on follow-up echocardiograms. Our own experience, coupled with a review of the literature, indicate that prophylactic anticoagulation therapy with coumadin should continue for at least 4 to 6 months for patients with an increased risk for postoperative thrombosis. For infants and young children with a low risk for postoperative thrombosis, aspirin should be given routinely for 6 months postoperatively.

The procedure of total cavopulmonary connexion includes trans-section and closure of the pulmonary trunk, the systemic venous blood flow being directed to the pulmonary vascular bed by an anastomosis of the superior caval vein with the right pulmonary artery. Following this procedure, there remains a small residual pulmonary trunk. The purpose of our study was to obtain information about the fate of this residual trunk and its contained pulmonary valve. Using transthoracic echocardiography, we examined 29 patients (mean age 7 years 4 months) after a total cavopulmonary connexion (mean interval 17 months). We were able to obtain adequate visualization of the pulmonary valve and the pulmonary trunk in 23 of the patients. Pulmonary regurgitation was demonstrated by colour Doppler echocardiography in 13 pts (57%). Formation of thrombus in the residual pulmonary trunk was detected in one patient (4%) 6 months after the operation. One cerebrovascular embolic event occurred in a patient who had pulmonary regurgitation without formation of thrombus in the residual trunk 3 months after the total cavopulmonary connexion. Our findings show that pulmonary regurgitation is frequently present in patients after total cavopulmonary connexion. Although formation of thrombus in the residual pulmonary trunk seems to be less common, we recommend surgical closure of the pulmonary valve during this procedure so as to exclude the residual pulmonary trunk as a possible source of systemic emboli.

Morphometric methods were used to study the great arterial trunks in a total of 22 human fetuses and newborn subjects weighing from 1 to 3.8 kg (mean 2.22 ± 0.85 kg). All specimens displayed concordant atrioventricular and ventriculoarterial connections, and all had perimembranous ventricular septal defects. Thirteen different parameters were measured in each specimen, comprising the orifices of the great arterial trunks, ascending aorta, vessels branching from the aortic arch, the aortic isthmus, descending aorta, pulmonary trunk, orifices of the pulmonary arteries, and arterial duct. The values were Compared with patterns of normality established in an earlier study using hearts from 496 human fetuses and neonates weighing form 60 to 5000 g. Minimum square regression analyses were used to study the relationships between fetal and neonatal log body weight in kilograms, and the various cardiometric parameters. We believe that these morphometric data have immediate clinical and surgical applications in the treatment of fetal and neonatal cardiovascular disorders.

We recently reported our experience with exercise stress echocardiography in children with prior history of Kawasaki disease and coronary arterial involvement. Dobutamine stress echocardiography has been used in adults (doses up to 50 μg/kg/min) for diagnosis and risk stratification of coronary arterial disease. To date, no studies have assessed the safety and utility of high-dose dobutamine for the detection of abnormalities of wall motion in children, although low dose dobutamine (5 μg/kg/min) has been used to assess left ventricular function after use of anthracycline in children. Our study group consisted of 10 children known to have Kawasaki disease, and 16 children who had undergone heart transplantation. After baseline transthoracic echocardiographic imaging, dobutamine was infused in an incremental fashion, 10–40 μg/kg/min intravenously, to attain peak heart rate. Heart rate and blood pressure were monitored continuously, and 4 standard echocardiographic images were acquired at each stage of dobutamine infusion and at recovery. Images were digitized, and the left ventricular segments were reviewed to assess wall motion. No patients suffered serious side effects such as arrhythmias or hypotension. Studies were complete in all 26 patients. One study proved positive, with a new abnormality of wall motion identified corresponding to a critical right coronary arterial stenosis. Peak heart rate and blood pressure were similar for the 2 groups. High dose dobutamine stress echocardiography, therefore, is feasible and safe in children, and may detect flow-limiting coronary arterial stenoses noninvasively. The technique may safely be applied to other subsets of children where assessment of ischemia and contractile reserve is warranted.

A bidirectional cavopulmonary shunt is performed in patients with single ventricle physiology to improve or maintain systemic oxygenation while decreasing the workload of the heart. During a period of 10 years, bidirectional cavopulmonary shunts were performed in 50 patients at our institution. The procedure was performed with an additional source of pulmonary flow through the pulmonary valve or a systemic to pulmonary shunt in 27 patients and without an additional source of pulmonary flow in 23 patients. Preoperative and postoperative chest radiographs, pulmonary angiograms, oxygenation, hemodynamic measurements, morbidity, and mortality were reviewed to determine whether a bidirectional cavopulmonary shunt with an additional source of pulmonary flow is an acceptable interim or final stage of palliation. Systemic oxygenation was improved only in patients with an additional source of pulmonary flow. The outcome of the bidirectional cavopulmonary shunt was not adversely affected by the presence of additional pulmonary flow. Twenty-five patients subsequently underwent an atrial to pulmonary or total cavopulmonary anastomosis. Oxygenation was improved by more definitive palliation; however, late complications of stroke, protein losing enteropathy, and arrhythmias requiring pacemaker therapy were more prevalent. In conclusion, systemic oxygenation can be improved by performing the bidirectional cavopulmonary shunt with an additional source of pulmonary flow through the pulmonary valve or a systemic to pulmonary shunt without an adverse effect on hemodynamic measurements, pulmonary arterial growth, morbidity, or survival. The bidirectional avopulmonary shunt may be an acceptable final stage of palliation in some patients with a functionally single ventricle.

We report our experience using an originally designed thin walled metal-tipped arterial cannula for neonatal open heart surgery. Its external diameter is 2.1 mm and its internal diameter is 1.7 mm, it has a high internal – external diameter ratio. It was used on 36 patients, who were either neonates or small infants. In 32 of the patients, it was inserted into the ascending aorta for systemic perfusion. In the other 4, it was inserted into the brachiocephalic artery for selective cerebral perfusion during repair of the aortic arch. The relationship between the pressure drop and the bypass flow was obtained for the 7 most recently treated patients. The pressure drop at a bypass flow of 500 ml/min was 150 mmHg. Due to the high internal – external diameter ratio of this cannula, and its excellent flow characteristics, we consider it to be particularly useful in neonatal open heart surgery, while it also allows for successful selective cerebral perfusion during repair of the aortic arch in neonates.

Accurate bedside assessment of the ratio of pulmonary to systemic flow (Qp/Qs ratio, referred to as “the flow ratio” or “the ratio”) plays an important role in the management of many congenital heart defects, especially the complexes unified by univentricular atrioventricular connections. Arterial oxygen saturation can be a misleading measure of the ratio, and may not reflect derangements until they are quite large. Theoretical analysis suggests that systemic venous oxygenation may be a better indicator of the ratio. To examine this, we created a widely patent atrial septal defect in neonatal piglets (weight =4–6.5 kg, n=6). Snares aruond the aorta and pulmonary trunk were adjusted to alter the flow ratio from 0.1 to 6.5. Venous oxygen saturations, measured in the mid-inferior caval vein, were at a maximum at a ratio about 1, and declined rapidly with increases or decreases in the ratio beyond a limited range. The venous oxygen saturation was found to vary much more than arterial oxygen saturation, with arterial oxygen saturation only falling when the ratio dropped below 0.5. Oxygen delivery (Oxygen Content x Cardiacoutput) was found to parallel closely systemic venous oxygen saturation, and was at a maximum at the same ratio that produced a maximum value of systemic venous oxygen saturation. The study suggests that systemic venous oxygen saturation provides a better estimate than does systemic arterial oxygen saturationof the flow ratio and oxygen delivery. Interventions that maximize systemic venous oxygen saturation should maximize oxygen delivery, and determination of systemic venous oxygen saturation should be a helpful addition in managing children with a number of congenital heart defects.

Over a period of 12 years, we encountered 30 cases of pulmonary atresia with intact ventricular septum. The overall mortality was 53% (16 patients). Coronary arterial abnormalities were detected in 16 patients and, in 10 of these, the coronary arterial circulation was partially or totally dependent on the right ventricle. The mortality in this group was 80%. The strategies for management are reviewed in 6 cases to try to define the best approach to diagnosis, and to assess the benefits of the various modesof treatment used for specific coronary arterial anomalies. Two cases (2 and 3) underwent occlusion of a fistula from the right ventricle to a coronary artery using embolization coils. This modality is considered to be appropriate and useful when the coronary circulation supplied by the fistula is not dependent on flow from the right ventricle. The dependency of the coronary arterial circulation on the right ventricle was extensive in two cases (Case 4 and 6), and complete in one because of atresia of the orifices of the coronary arteries (Case 5). These three patients died. Cardiac transplantation is suggested as the most appropriate treatment in this group. In two cases (1 and 6), there was a supernumerary vessel connecting the right ventricle to the pulmonary trunk. These connections do not appear to be of therapeutic concern, as they do not seem to contribute to myocardial blood supply. The selection of the most appropriate protocol for management of patients with coronary arterial anomalies depends upon a thorough and aggressive approach to investigation.

We describe post azygos obstruction of the superior caval vein following repair of a mixed variety of totally anomalous pulmonary venous connection. Balloon dilation was immediately successful, and has provided persistent relief at a follow-up of 20 months.

A patient is reported in whom cardiac strangulation occurred through a lateral pericardial defect surgically created for repair of congenital heart disease. To avoid this potentially fatal complication, the inferior rim of an iatrogenic lateral pericardial defect should be tacked down to preclude cardiac herniation.

We describe a neonate with tricuspid atresia, absent pulmonary valve, right ventricular hypoplasia, small venrricular septal defect, left ventricular ourflow tract obstruction and a patent arterial duct. These finding were diagnosed by echocardiography and confirmed by cardiac catherterization and postmortem examination. This is a unique report of a functionally single ventricle variant of absent pulmonary valve syndrome and biventricular outlet obstuction.

The concept of sequential analysis of congenitally malformed hearts is now widely accepted, although some aspects remain controversial. In this review, we analyse the evolution of the scgmental approach, showing how our own concepts of analysis have chaanged little since our initial description, heart dicrates the presence of the atrial, ventriclar, and arterial segments, Analysis depends upon recognizing the morphologic differences of the components within each segment, and then how these parts are joined together across the atrioventicular and ventriculo-arterial Junctions. The distinction of chambers and arterial trunks follows the principle of the morphologic method, namely that scructures be identified on the basis of their own intrinsic morphology, and not according to other features which themselves might be variable. Differentiation of junctional arrangements as opposed to segmental stucture resolves ongoing controversics such as the nature of “univentricular hearts”.Analysis in the fashion proposed shows that there is nothing intrinsically complicated with malformed hearts.