Dealing with kinked and swirled pulmonary vessels: surgical treatment of arterial tortuosity syndrome: a case report.

We present the case of a child with arterial tortuosity syndrome, describing the operative findings and our surgical technique to address pulmonary arteries stenosis.

Outcomes after surgical repair of sinus venosus atrial septal defect in children.

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.

Arrhythmias in congenital heart defects.

AIM OF THE STUDY: Evaluation of the incidence and severity of late arrhythmias in patients with predisposing congenital heart defects--either due to the anatomy of the defect itself or as a result of a particular type of surgical intervention. PATIENTS AND METHODS: In a retrospective long-term study authors analyzed 158 patients (divided into 5 groups) with congenital heart defects after surgical correction. Evaluated were: the incidence of rhythm disturbances, the type of arrhythmia and the need for medication or intervention. RESULTS: The most rhythm disturbances occurred in patients after physiological correction of D-transposition of the great arteries (68.5%) and these patients also mostly needed medication or pacemaker implantation; followed were by patients with hypoplastic left heart syndrome after Fontan procedure (40%), then were patients after long-term correction of tetralogy of Fallot (31.1%), atrial septal defect sinus venosus type with partial anomalous pulmonary venous return after Warden correction (25.7%) and congenitally corrected L-transposition of the great arteries (25 %). Most of these arrhythmias were asymptomatic and there was no need to treat them. There was an increased incidence of arrhythmias with time (p < 0.05). DISCUSSION: During childhood in patients after surgical correction late arrhythmias mostly do not represent a severe problem, but with time, when reaching adulthood, this may be an issue. It is therefore very important to understand the anatomy, physiology and the arrhythmogenic substrate of every high risk congenital heart defect (Tab. 2, Fig. 6, Ref. 10).

Results of modified Norwood's operation for hypoplastic left heart syndrome.

OBJECTIVE: The aim of the study was to analyze intermediate results of treatment of the hypoplastic left heart syndrome based on selective indication criteria. METHODS: Between February 1997 and May 1999 38 patients with hypoplastic left heart syndrome (n=35), or with functional variant of hypoplastic left heart syndrome (n=3) were admitted to our department. Contraindications for surgery were birth weight <2500 g, diameter of ascending aorta <2 mm, severe tricuspid regurgitation persisting after initial stabilization, pulmonary regurgitation more than mild, dysfunction of the systemic right ventricle and failure to effectively resuscitate circulation before surgery. RESULTS: Based on these criteria surgery was not indicated in 17 patients. Twenty-one infants were operated on by modified Norwood's procedure using only autologous great vessel tissue for reconstruction of systemic outflow. Overall hospital mortality was 14% (three patients). Eighteen survivors (86%) were discharged with well-balanced circulation. There was one late death (5%). Thirteen patients had already undergone the second stage (bi-directional Glenn) with no death. The mean follow-up was 13. 2+/-9.1 months (range 4-32 months). Considering both early and late events the probability of survival for the whole group (n=21) from the time of surgery was 86% at 1 month, 80% at 12 months, and it remained unchanged at 18 and 24 months of follow-up. CONCLUSIONS: Only a limited number of European countries offer surgical treatment of hypoplastic left heart syndrome. Promising intermediate results (80% survival rate after stage I and II) achieved at our department do not only reflect overcoming the learning curve but also a selective approach to indication for surgery as well. In a country with limited resources selective approach to the patients with hypoplastic left heart syndrome is justified.

Recoarctation and patients' freedom from re-intervention--a study of patients undergoing surgery for coarctation of the aorta at the Department of Cardiac Surgery of the Children's University Hospital, Bratislava.

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)

Surgical treatment of transposition of the great arteries.

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.)

[Surgical treatment of total anomalous pulmonary venous return]. / Chirurgická liecba totálneho anomálneho pl'úcneho venózneho návratu.

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.)

Safety and effects of two red blood cell transfusion strategies in pediatric cardiac surgery patients: a randomized controlled trial.

OBJECTIVE: To investigate the safety and effects of a restrictive red blood cell (RBC) transfusion strategy in pediatric cardiac surgery patients. DESIGN: Randomized controlled trial. SETTING: Pediatric ICU in an academic tertiary care center, Leiden University Medical Center, Leiden, The Netherlands. PATIENTS: One hundred seven patients with non-cyanotic congenital heart defects between 6 weeks and 6 years of age. One hundred three patients underwent corrective surgery on cardiopulmonary bypass. INTERVENTIONS: Prior to surgery patients were randomly assigned to one of two groups with specific RBC transfusion thresholds: Hb 10.8 g/dl (6.8 mmol/l) and Hb 8.0 g/dl (5.0 mmol/l). MEASUREMENTS: Length of stay in hospital (primary outcome), length of stay in PICU, duration of ventilation (secondary outcome), incidence of adverse events and complications related to randomization (intention to treat analysis). RESULTS: In the restrictive transfusion group, mean volume of transfused RBC was 186 (±70) ml per patient and in the liberal transfusion group 258 (±87) ml per patient, (95% CI 40.6-104.6), p < 0.001. Length of hospital stay was shorter in patients with a restrictive RBC transfusion strategy: median 8 (IQR 7-11) vs. 9 (IQR 7-14) days, p = 0.047. All other outcome measures and incidence of adverse effects were equal in both RBC transfusion groups. Cost of blood products for the liberal transfusion group was 438.35 (±203.39) vs. 316.27 (±189.96) euros (95% CI 46.61-197.51) per patient in the restrictive transfusion group, p = 0.002. CONCLUSIONS: For patients with a non-cyanotic congenital heart defect undergoing elective cardiac surgery, a restrictive RBC transfusion policy (threshold of Hb 8.0 g/dl) during the entire perioperative period is safe, leads to a shorter hospital stay and is less expensive.

Histidine production by a regulatory mutant of Streptomyces coelicolor.

Streptomyces coelicolor mutant RF-59, isolated as a revertant of a histidine auxotroph after mutagenic treatment with N-methylN'-nitro-N-nitrosoguanidine, was found to accumulate L-histidine. The mutant was sensitive to 2-thiazo-lealanine and L-2,4-diaminobutyric acid and partially sensitive to alpha-methylhistidine but resistant to 1,2,4-triazolealanine, indicating that repression of the histidine operon was modified in the mutant. Culture conditions were investigated, and optimal media for L-histidine production were developed, resulting in L-histidine accumulation of 2.1 to 3.5 g/liter.

Biochemical and genetic studies of a histidine regulatory mutant of Streptomyces coelicolor A3 (2).

The specific activities of three enzymes in a histidine regulatory mutant RF59 of Streptomyces coelicolor A3(2) resistant to the histidine analogue 1,2,4-triazolealanine (TRA) were measured and compared to the activity of the wild type strain. The first enzyme of the histidine pathway, phosphorybosyl-ATP-pyrophosphorylase (PR-ATP-pyrophosphorylase), of mutant RF59 and the wild type was sensitive to allosteric inhibition by L-histidine and hence feed-back inhibition was not affected by mutation, although the specific activity in the mutant was 2.9 fold higher than in the wild type. The other two enzymes coded by genes from the histidine operon were significantly derepressed. The enzyme D-erythroimidazoleglycerol phosphate dehydrase (IGP-dehydrase) in mutant RF59 had a 4.9 fold higher specific activity than in the wild type strain. The specific activity of the last enzyme of the pathway, histidinol-dehydrogenase (Hol-dehydrogenase), in the mutant was 4.7 fold derepressed compared to the wild type strain. The results of genetic crosses revealed the mapping of RF59 regulatory mutation between argA1 and cysD18 on S. coelicolor chromosome, suggesting that the mutant RF59 is a regulatory mutant unable to fully repress genes of the histidine pathway.