OBJECTIVES Review of outcomes for children undergoing surgical repair of sinus venosus atrial septal defect (SVASD) and proposal of optimal treatment strategy. BACKGROUND Unlike secundum-type atrial septal defect (ASD II), surgical correction of SVASD is more challenging and carries higher risk of postoperative complications. Several surgical techniques have been proposed to avoid them; however, the data on long-term follow-up are limited. METHODS Between 1992-2005, 85 patients underwent surgery for SVASD at our institution. 78 and 7 operations were performed through full and partial sternotomy, respectively. The mean age was 7.7 years (2 months -19 years). All patients with SVASD and no partial anomalous pulmonary venous drainage (PAPVD) or PAPVD in right atrium (n = 24) underwent the single-patch repair. Among patients with SVASD and PAPVD in SVC, 22 and 39 patients underwent intracaval baffle and Warden procedure, respectively. Clinical, electrocardiographic and echocardiographic follow-ups were available for all patients. RESULTS There were no deaths, reoperations, residual atrial septal defects and pulmonary vein stenosis. Two patients had SVC stenosis. Permanent arrhythmias were noted in 24 patients (28.2%). The rate of new arrhythmias in the intracaval baffle and Warden subgroup was 54.6% vs 23.1% (p = 0.01). Moreover, two and one patient from the intracaval baffle subgroup receive antiarrhythmics and had a pacemaker implanted, respectively. CONCLUSION Surgical treatment of SVASD is associated with excellent results and minimal morbidity. The Warden procedure is preferred in patients with SVASD and PAPVD in SVC because of lower incidence of postoperative arrhythmias (Tab. 1, Fig. 4, Ref. 23). Full Text (Free, PDF) www.bmj.sk.

AIM OF STUDY:Coarctation of the aorta (CoA) accounts for about 8% of all congenital heart diseases. This represents about 30 new cases of coarctation every year in Slovakia, of which more than half will require surgical treatment. Over the past years, many children with this diagnosis have been successfully operated on at the Department of Cardiac Surgery of the Children's University Hospital, Bratislava. Thus, the need for a comprehensive follow-up and analysis of the postoperative well being of these young patients arises. Our study is therefore aimed at: 1) identifying factors affecting the incidence and persistence of postoperative systemic hypertension, as well as the need for heart failure and hypertension treatment, 2) assessing patients' psychomotor development following surgery for coarctation of the aorta.METHODS AND DATA:Between January 1992 and December 2001, a total of 201 patients with aortic co-arctation were operated on at our institution. The three classes of aortic coarctation namely: isolated coarctation, coarctation with ventricular septal defect and coarctation with complex cardiac anomalies were represented. Patients' medical records were retrospectively reviewed, with attention paid to such variables as the type of lesion, gradient across the site of coarctation, type of surgical technique employed and surgery-related complications. Subsequently, these patients were followed for a time period ranging between six months and ten years during which their psychomotor development and overall clinical state were evaluated.RESULTS:Of the 201 operated patients, 64 (33%) had early postoperative hypertension, so-called paradoxical hypertension. There was a significant correlation between the incidence of early postoperative hypertension and patients' age at operation (p < 0.0001). Age at operation was also a significant risk factor for late hypertension (p = 0.005). In both cases we noticed a higher incidence of high blood pressure in patients operated on after the age of six years. The need for antihypertensive treatment of patients with early postoperative hypertension decreases with a younger age at operation. At five years of follow-up, the need for antihypertensive treatment was 15%. Clinical psychological evaluation of 64 patients showed a normal distribution of patients' intelligence quotients. No surgery-related variable correlated with the incidence of delayed mental development. There was, however, a certain correlation between the presence of complex anomalies and low verbal IQ in examined patients (p = 0.04)CONCLUSIONS:Early surgical treatment of aortic coarctation reduces the likelihood of early, as well as late postoperative hypertension. The preferred protocol in our institution is early surgical treatment of patients at about the age of two years. The need for antihypertensive treatment of patients at five years of follow-up is 15%. Patients' psychomotor development following surgery for aortic coarctation is not affected by type of surgical procedure. On the whole, we can conclude that patients' psychomo-whole, we can conclude that patients' psychomotor development does not differ from the rest of population. There is however, a certain correlation between complex cardiac anomalies and a tal, Bratislava delay in some components of patients' psychomotor development. (Tab. 3, Fig. 4, Ref. 17.)

BACKGROUND:Many children and young adults have undergone surgery for coarctation of the aorta. Individual surgical techniques employed in the treatment of patients and their effects on incidence of recoarctation were reviewed.METHODS AND DATA:Over the last ten years, a total of 201 cases of aortic coarctation were surgically treated at our department. The three forms of coarctation of the aorta recognized by the Society for Cardiothoracic Surgeons namely: isolated coarctation, coarctation with ventricular septal defects and coarctation with complex cardiac anomalies, were represented.RESULTS:19 cases of recoarctation were recorded over the period of follow-up, representing 10% of all operated patients. On univariate analysis, the risk of recoarctation was closely linked with the following variables: use of resection and end-to-end anastomosis (p=0.01), age at operation less than one month (p=0.0002) and weight at operation less than 3 kg (p=0.01). The risk of recoarctation was found to be highest when resection and end-to-end anastomosis was employed in neonates (p<0.0001). Most cases of recoarctation as shown by the Kaplan-Meier plot occurred within the first year after surgery.CONCLUSION:The use of simple resection and end-to-end anastomosis in neonates is associated with a high risk of recoarctation. Hence, our preferred surgical technique in neonates is the extended resection and end-to-end anastomosis, which is associated with a considerably lower risk of recoarctation in this age group. (Tab. 4, Fig. 5, Re. 7)

BACKGROUND:We conducted a retrospective review of children undergoing surgery for coarctation of the aorta in our institution over the last ten years with the aim of evaluating overall patient survival as well as detecting factors affecting it. We tried to identify the risk factors for mortality.METHODS AND DATA:Between January 1992 and December 2001, 201 patients with aortic coarctation were operated on at the Department of Cardiac Surgery of the Children's University Hospital, Bratislava. The three classes of aortic coarctation were represented: isolated coarctation, coarctation with ventricular septal defect (VSD) and coarctation with complex cardiac anomalies. Patients' preoperative, operative and immediate postoperative medical records were carefully studied with special attention paid to the type of lesion, patients' preoperative state, type of surgical technique employed, as well as the period of operation. For comparison, two equal time periods of follow-up were reviewed--1992 to 1996 and 1997 to 2001. The overall postoperative conditions of patients were also regularly monitored. Patient data were statistically analyzed using the JMP program version 4.04.RESULTS:An overall survival of 90% was recorded over the period of follow-up, ranging between one and ten years. A further break down showed a statistically significant difference between the various types of aortic coarctation, p=0.0001. Patients with simple or isolated coarctation had a survival rate of 100%, those with ventricular septal defect (VSD) in addition to coarctation had a survival rate of 80% while patients with associated complex cardiac anomalies had a survival rate of 65%. An improvement on overall patient survival was recorded in the period between 1997 and 2001--96% as against 86% for the period between 1992 and 1996. On univariate statistical analysis, the following variables were identified as significant risk factors for death: 1) Complex cardiac anomalies (p<0.0001), 2) Age at operation less than one month (p<0.0001) and 3) Treatment prior to the year 1997 (p=0.02).CONCLUSION:A considerable improvement on patient survival following surgery for coarctation of the aorta was recorded over the last five years. This could be attributed to new measures in preoperative, operative and postoperative care for patients with aortic coarctation. (Tab. 4, Fig. 5, Ref. 8.).

BACKGROUND:With regard to risk of the failure of systemic right ventricle after physiological correction of transposition of great arteries, anatomic repair is a current method of choice.OBJECTIVE OF STUDY:Analysis of results of surgical correction of transposition of great arteries performed between 1992 and October 1998.METHOD:A total of 111 patients were operated on for transposition of the great arteries. In the 1st group of patients (n = 21, mean age was 135 +/- 55 days), physiological correction according to Senning was performed. Patients of the 2nd group (n = 90, mean age was 15.4 +/- 21.6 days) underwent anatomic repair.RESULTS:Early mortality was 6% (7 patients). Mean follow-up is 2.95 years (1.9 SD) ranging from 0.2 years to 6.1 years. Actuarial 1-month survival in the whole cohort (n = 111) is 94%, and it remains unchanged at 1, 2, 3, 4, 5, and 6 years of follow-up. Patients, who underwent surgery after 1997, show significantly better survival compared to those operated before 1997 (p = 0.0997). Thus, a date of operation (before 1997) is the only significant risk factor for death. Survival in patients operated after 1997 (n = 40) is 98%. All patients belonging to the 2nd group are in functional group NYHA 1.CONCLUSION:Anatomic repair of transposition of the great arteries is a method of choice for treatment of this congenital heart defect. Left ventricle becomes systemic ventricle, which is essential in view of long-term performance. Psychomotor development of children, who underwent ASO, is comparable with that of healthy population. (Tab. 3, Fig. 3, Ref. 18.)

BACKGROUND:Total abnormal pulmonary venous return (TAPVR), mainly the obstructive type represents the most riskful critical congenital heart defect requiring urgent surgery immediately after birth.THE AIM OF THE STUDY:Analysis of surgical correction of TAPVR results performed from December 1992 to December 1998.METHODS:Twenty-seven patients underwent surgery for TAPVR. 13 of them (48%) presented with hemodynamically severe obstruction. Mean age in the group with obstruction was 3.6 +/- 3.2 days with mean weight of 3282 +/- 537 grams.RESULTS:From the 27 studied patients 5 (18.5%) died. Mean duration of the study in the whole group is 1.91 +/- 2.01 years. Actuarial survival in the first month is 85%, in the second month 81% and remains identical in the 1., 2., 3., 4., 5., 6. year of the study. Univariate analysis identified operation before the year 1996 (p = 0.0056) as a risk factor of immediate mortality. Introduction of ultrafiltration significantly eliminated mortality (p = 0.0101). Remaining variables (age, weight, sex, obstructive TAPVR, TAPVR, extracorporeal circulation duration, pulmonary hypertension) did not significantly influence the survival (p more than 0.05). Multivariate analysis defined operation before the year 1996 as the sole risk factor of mortality (p = 0.0033). In patients operated on in the year 1996 (n = 15) was the survival in the studied period 100%.CONCLUSION:Since the year 1996 the results of surgical treatment of TAPVR significantly improved. The key role in the improvement have better urgent diagnostic and surgery, improvement of surgical technique and myocardial protection, introduction of modified ultrafiltration and the quality of postoperative care. Psychomotor development of children after correction is comparable with healthy population, all patients are in NYHA I class. (Tab. 2, Fig. 4, Ref. 9.)

During the period between 1963 and 1988, 343 patients were operated due to aortic coarctation. 14 patients (4%) were necessarily reoperated for late postoperative complications. In 3 cases (0.8%) reoperation was indicated due to the development of aneurysm in the region of primary operation of aortic coarctation. The development of aneurysm is prevalently predisposed by Vosschulte's indirect aortoplastic operation by the use of a synthetic patch. In the remaining 11 patients (3.2%) the reoperation was indicated owing to the development of recoarctation. Today recoarctation concerns children which were operated under the age of two. The most important moment favourable for the recoarctation development is the secondary constriction of the repaired region in consequence of the coarctation listel fibroid tissue and owing to the incapability of the anastomosis to grow. The perspective rests on using new suture material and combined techniques. Late postoperative complications which follow operations performed due to aorta coarctation remarkably augment the peri and postoperative mortality. Three cases out of the given group of 14 patients terminated by exitus (21%). Hence, it is necessary to remove all moments of predisposition creating a condition favourable for the development of recoarctation and aneurysm. (Tab 2, Fig. 1, Ref. 14.).

The studied series consisted of 14 patients with ventricular septal defect and pulmonary hypertension aged from 3 months to 15 years. Three examination methods were compared: (1) Invasive hemodynamic examination; (2) Pulmoangiographic examination by means of wedge peripheral pulmoangiography; (3) Histological examination of bioptic samples. Direct correlation was found to exist between mean PA pressure, PAR/m2, TPR/SR and pulmoangiographic records, in which the following parameters were evaluated: (a) length of the narrowing of the peripheral branch of the PA, (b) background opacity, (c) circulation time. The histological findings obtained in the bioptic samples corresponded practically in all cases with the hemodynamic and PAG findings. Only in two patients did the histological picture display a less severe degree of affection than found by PAG and hemodynamic examination. The obtained results suggest that in light of surgical indications, PAG can be considered a suitable and valuable supplementary method in assessing borderline findings of pulmonary hypertension in children with congenital heart defects and left-to-right shunt.

The authors describe the case-history of a 12-year-old patient with hypereosinophil syndrome, endomyocardial fibrosis of the left ventricle and dominating mitral valve insufficiency. By correction of the insufficiency, using a Carpentier ring, the authors achieved substantial improvement of the haemodynamics. One year after operation the patient is cardially compensated.

During the period between 1983-1989 the authors operated 165 patients with the diagnosis of coarctation of the aorta, 197 cases of patent ductus arteriosus and 360 atrial septal defects. In all diagnostic groups they operated a large number of patients above 10 years of age whereby the oldest patient with a septal defect was 57 years old, patients with coarctation of the aorta or a patent ductus arteriosus 35 years old. The authors discuss the reasons why the patients were indicated for operation so late.

The authors present their experiences with diagnosis and surgical treatment of anomalous venous drainage of the left lung into the left innominate vein. The anomaly was found only in four out of 704 patients operated on for atrial septal defect. In three patients in whom leftsided anomalous pulmonary venous drainage was associated with atrial septal defect, the derangement was corrected by using extracorporeal circulation. The patient who had no atrial defect was operated on without extracorporeal circulation on applying leftsided thoracotomy. The mean age of the patients was 26.25 years. After correcting the defect, drainage of the left lung into the left atrium was found satisfactory in all operated patients. Exact assessment of the possibility of an associated intracardiac defect is being emphasized, as it is crucial for selecting the appropriate surgical approach.

Tumors of the heart are a rare cause of embolism of the peripheral arterial system. A series of 26 patients operated on for heart tumor in the Institute of Cardiovascular Diseases in Bratislava over the years 1979-1988 was analyzed. Pseudomyxoma was histologically verified in 24 patients and rhabdomyoma and rhabdomyosarcoma in the other two patients. A total of 12 embolic events was recorded in 9 patients (34.6%). There were 8 instances of embolism in the central nervous system and 4 in the extremities. In all cases pseudomyxoma of the left parts of the heart was the source of embolism. In 21 cases diagnosis was established and operation indicated on the basis of ultrasonographic evidence. The authors recommend the biatrial transseptal approach. Early surgical removal of a heart tumor is the therapy of choice which prevents the development of potential complications from the heart and peripheral arteries.

In a retrospective study the results of operations for hemodynamically severe ventricular septal defect were analyzed in 18 patients with a body weight below 10 kg who had been operated on over a period of two years at the Institute of Cardiovascular Diseases in Bratislava. The first group consisted of 12 children with the mean body weight of 6650 g in whom complete correction of the ventricular septal defect was carried out by means extracorporeal circulation. Ligation of the pulmonary artery was performed in 6 infants with the mean body weight of 3420 g (second group). Patients of both groups had a serious left-to-right shunt and marked pulmonary hypertension. An excellent operative result was obtained in 10 children after complete correction of the defect. Two patients of the first group and one patient of the second group died suffering from severe pulmonary hypertension. Surgical correction of the hemodynamically severe ventricular septal defect in early childhood is indicated when conservative treatment fails and should be carried out as an urgent procedure regardless the age and body weight of the patient.

The authors analyze the results of preoperative morphometry of atrial septal defects. They compare the area of the defect and the septal area in different types of defects. Based on the thus obtained relative value, they select either suture of the defect or its correction by means of a patch. They emphasize that during correction defects deformations and reduction of the septal area must be avoided. They do not admit the possibility that the suture of the defect is exposed to traction. Ostium secundum defects the area of which is greater than 40% of the septal area are suited for correction by means of a patch. An important indicator is the transverse dimension of the defect and of the septum. This relation determines the grade of deformity during an inadequately selected suture and the presence of traction mechanisms in the area of the suture and cardiac skeleton. The authors recommend more frequent use of patches also in ostium secundum defects in the cranial lateral and distal part of the atrial defect.