A biodegradable ring enables growth of the native tricuspid annulus.

BACKGROUND AND AIM OF THE STUDY: The study aim was to assess the growth potential and function of the tricuspid valve (TV) annulus after annuloplasty with the intra-annular biodegradable ring. METHODS: Among 11 children (median age 4.5 years; age range: 0.2-10.9 years) who underwent surgery for severe congenital TV regurgitation, valve repair was completed using a biodegradable annuloplasty ring. The children were followed regularly using transthoracic echocardiography, whereby the TV annulus lateral diameter (TVALD) and valve function were monitored. Rates of valve growth were derived from the slope of the regression equations which related TVALD to the natural logarithm of the body surface area (lnBSA). RESULTS: The children's somatic growth was harmonious throughout the entire follow up period (mean 478 days; range: 171-1,477 days). The TVALD differed significantly at six months and at one and two years after surgery compared to the postoperative value at discharge, rising from 19 mm (range: 15.5-26.0 mm) to 24 mm (range: 19.0-30.0 mm) at the last control examination (p = 0.003), while the related Z-scores remained stable. A significant linear correlation between TVALD and lnBSA was found in 63.6% of patients. The median rate of growth for the whole cohort was 1.96-fold (range: 0.52-5.53-fold) higher than a norm, and correlated strongly and positively with age (r = 0.91; p <0.05). The median postoperative TV insufficiency fraction of 9.8% (range: 0-28.8%) remained constant during the follow up period. The postoperative TV maximal pressure gradient was 5.5 mmHg (range: 3.1-12.2 mmHg), and did not increase over time. CONCLUSION: The implantation of a biodegradable ring does not restrict growth of the native TV annulus; this enabled its stabilization in proportion to the somatic growth in the majority of the children. The TV annulus began to change its dimension at six months postoperatively, which may coincide with its biodegradation. The rate of growth of the TV annulus differed from that in the normal population, and was proportional to the patient age. The increase in TV annulus diameter over time did not have any negative influence on the function of the repaired valve.

[Closure of major aorto-pulmonary collateral artery with the Amplatzer vascular plug in an infant with pulmonary atresia and ventricular septal defect - a difficult therapeutic problem]. / Zamkniecie nieprawidlowego polaczenia aortalno-plucnego zatyczka Amplatzer vascular plug u dziecka z zarosnieta zastawka plucna i ubytkiem miedzykomorowym - trudny problem terapeutyczny.

A case of a 2-day-old newborn with congenital heart disease, pulmonary atresia and ventricular septal defect is described. The Blalock-Taussig shunt was created 6 days after birth. After the procedure, the newborn developed symptoms of increased pulmonary blood flow and respiratory distress. We decided to close one of the major aortopulmonary collateral arteries with the Gianturco coil on the 41st day after birth, however the results were not satisfactory. On the 66th day of infant life (by that time the patient weighted 3 kg), another procedure with the Amplatzer vascular plug was performed. This procedure was successful. After a few days the infant was discharged from hospital.

[Is atrial septal defect always a simple defect?]. / Czy ubytek przegrody miedzyprzedsionkowej jest zawsze prosta wada?

A case of an asymptomatic 4-year-old girl with hemodynamically significant type II atrial septal defect is described. The patient underwent TEE in order to assess the eligibility for interventional device closure. Due to unfavorable anatomy and suspicion of the sinus venous type of the defect the child was selected for surgical treatment. No abnormalities were detected during surgery but a superfluous left atrial return of the venous blood during the cardiopulmonary bypass was noticed. The postoperative catheterisation was performed to elucidate this finding since the intraoperative TEE was normal. An anomalous venous drainage from superior and medial lobes of the right lung to the inferior vena cava was revealed. The 'scimitar' syndrome was diagnosed. Next, the patient underwent a successful surgical correction of this abnormality. The study presents the epidemiology, symptomatology and treatment modalities of the syndrome.

[Right atrial thrombus following surgical closure of atrial septal defect--a case report]. / Skrzeplina w prawym przedsionku po operacyjnym zamknieciu ubytku w przegrodzie miedzyprzedsionkowej--opis przypadku.

A case of an 11-year-old girl with atrial septal defect is described. The patient underwent surgical closure of the defect, which was complicated by asymptomatic thrombus formation in the right atrium. Anticoagulant therapy was initially successful, but thrombus recurred. Surgical thrombus removal was successful.

Is device closure for direct access valved stent implantation safe?

OBJECTIVE: Despite the progress made in the development of valved stents for trans-apical valve replacement, a reliable closure of the access orifice remains a major issue. The present study was designed to evaluate if device closure of the ventricular wall is safe. MATERIALS AND METHODS: Transventricular access for pulmonary valve replacement was simulated with a 26F sheath and the resulting orifice was closed with an Amplatzer Muscular VSD Occluder (AMuscVSDO) in chronic sheep experiments (body weight 45-48 kg). Mean procedure time, blood loss, and standard hemo-dynamics were recorded. The animals were sacrificed electively and the histopathological changes in and around AMuscVSDO in the right ventricular wall were systematically studied by semi-quantitative analysis of collagenisation, inflammatory response and 'resorptive' process. RESULTS: Mean procedure time was 31+/-10.7 min, blood loss was 22.5+/-8.7 ml, heart rate was 123+/-22.6 bits/min before and 128+/-28.7 bits/min after, mean arterial blood pressure was 88+/-16.7 mm Hg before and 82.6+/-18.3 mm Hg after the procedure. Mean survival was 5.3 weeks. The collagen and scar formation studies revealed three different periods: (1) initial fibrosis (0-3 weeks); (2) so-called 'capsulation' (3-9 weeks after the implantation of the Occluder); and (3) final remodelling and differentiation (9 weeks). The fabric inside the Occluder played the role of a collagenisation promoter, active from the 3rd week till it vanishes. Inflammation plays a role as a temporary reaction (0-3 weeks) during the healing process, with no signs of any active, focal or circumscribed, myocardial damage. CONCLUSIONS: (1) The closure of the free ventricular wall perforation with AMuscVSDO is safe due to the scar tissue resulting from the healing process around and in the device. (2) The myocardial healing around and inside an implanted AMuscVSDO represents two processes: extensive fibrosis ensues around metallic wires with the progression towards the inside of the myocardium, whereas inside AMuscVSDO the loose connective tissue fills the myocardial lesion. During cicatrisation, the fabric elements of AMuscVSDO act as the ground for collagen formation and fibroblast proliferation. (3) The cicatrisation processes after ventricular AMuscVSDO implantation show remodelling, with rearrangement of collagen fibres architecture and distribution.

Closure of left ventricle perforation with the use of muscular VSD occluder.

Growing experience in interventional cardiology leads to the use of large diameter of vascular equipment. In some instances, the so-called hybrid procedures are performed. After performing the interventional procedure, the opening in ventricular wall is closed surgically. Our intention was to check if the MVSDO can be used to close the perforation in the heart after the interventional cardiology procedure performed through the left ventricular (LV) free wall. In three pigs under general anesthesia, the heart was exposed through a small substernal incision. The LV was punctured and an 18F sheath was introduced into the LV. A 14 mm MVSDO was inserted through the 10F Delivery System. Using both the echocardiographic and angiographic guidance, the MVSDO was placed on the LV wall to close the opening in the LV. Time and volume of bleeding was recorded. In all cases the occluder was successfully placed closing the opening, bleeding observed after deployment of occluder lasted for approximately 2 min. We think MVSD occluder can be used to close the LV free wall perforation after hybrid interventional cardiac procedure. Early bleeding through MVSDO might be resolved by the manufacturing of new occluder with better sealing properties.

Mid-term experience with valved bovine jugular vein conduits.

From June 1999 to January 2004, 43 children underwent implantation of a valved bovine jugular vein conduit and correction of complex congenital heart defects. Median age was 1.98 years (range, 11 days - 13.3 years). There were 7 early deaths (16.3%) unrelated to conduit failure or thrombosis. Median follow-up of 36 survivors was 24 months (range, 1-48 months, quartile range, 12-48 months), total follow-up was 78 patient-years. There were 3 late deaths (8.3%) due to infection, pulmonary thromboembolism, and sudden cardiac arrest after re-operation to repair a right ventricular outflow tract aneurysm. There were 2 conduit explantations due to dysfunction and suspected endocarditis. Three patients underwent balloon dilatation of distal stenoses. The mean peak gradient through the pulmonary anastomosis was 15 mm Hg (range, 3-42 mm Hg) among patients free from re-intervention. No severe valve regurgitation was observed. Freedom from re-intervention was 72% at 48 months. This conduit remains a good alternative to homografts. Causes of distal stenosis must be clarified, guidelines for prophylactic anticoagulation must be created, and the role of percutaneous balloon dilatation established.

Closure of perimembranous ventricular septal defect using transcatheter technique versus surgical repair.

INTRODUCTION: Since the Membr VSD occluders have been introduced into clinical practice it is now possible to compare two treatment methods -- surgical and percutaneous. AIM: Assessment of the effectiveness and risk of complications in patients with perimembranous ventricular septal defect (VSD) treated with the Membr VSD occluder or surgery as well as comparison of postprocedural left and right ventricular systolic functions. METHODS: The study involved 11 children with perimembranous VSD treated with occluder implantation (Group A) and 12 children with surgical repair (Group C). Groups A and C differed slightly in terms of age of patients (p=0.026), but had similar mean weights (p=0.103), pulmonary to systemic flow ratios (Qp: Qs, p=0.929) and follow-up duration after the procedure. No significant differences were observed between the two groups in terms of left ventricular systolic function [left ventricular ejection fraction (EF, p=0.567)], diastolic function [mitral flow E/A ratio (p=0.975)], E deceleration time (DCT, p=0.346), isovolumetric relaxation time (IVRT, p=0.606), heart rate (HR, p=0.133) or left ventricular diameter (LV) on transthoracic echocardiography (TTE) before VSD closure. TEE was performed in all patients and the parameters listed above were measured within 3 to 16 months after the procedure, and then intra- and inter-group comparisons were carried out. Additionally, mitral (MV), tricuspid (TV) and aortic (Ao) valve regurgitations were evaluated. RESULTS: Tricuspid (TR, p=0.028) and mitral regurgitation (MR, p=0.043) decreased significantly after closure of VSD with the Membr VSDO. MR was significantly smaller in patients treated with the occluder than in those after surgery (p=0.026). Postprocedural TR (p=0.486) and aortic regurgitation (AR, 0.607) did not differ significantly between the two groups. Left ventricular EF was significantly (p=0.004) lower and HR higher (0.043) after surgery than in children treated with the occluder. No significant differences of the diastolic function were found - E/A (p=0.88), DCT (p=0.413), IVRT (p=0.09). CONCLUSIONS: 1. During the mid-term follow-up left ventricular EF was higher in patients after Membr VSDO occluder implantation than after surgical repair of VSD. 2. Higher EF and reduction of the left ventricular diameter after closure of perimembranous VSD with the occluder may result in a lower incidence and degree of MR in comparison to the subjects operated on.

[Ross operation using "contegra" conduit in a 5 year old girl with severe aortic valve insufficiency in Kawasaki disease coexisting with infective endocarditis--a case report]. / Ciezka niedomykalnosc aortalna w chorobie Kawasaki wspólistniejaca z bakteryjnym zapaleniem wsierdzia u 5-letniego dziecka. Operacja Ross'a z zastosowaniem Conduitu "Contegra".

A case of a 5-year-old girl with severe dysfunction of aortic valve in Kawasaki disease coexisting with endocarditis, is described. The role of Ross operation in the treatment of this condition is discussed. The 18-months follow-up showed good function of aortic valve and "Contegra" conduit (bovine jugular vein), but long-term follow-up of patients with "Contegra" conduit remains unknown. In conclusion, a Ross operation using "Contegra" conduit in pulmonary position could be effective method in the treatment of dysfunction of aortic valve in Kawasaki disease coexisting with endocarditis in children.

[Percutaneous procedures for the repair of iatrogenic occlusion or stenosis of peripheral vessels in children--report of 5 cases]. / Przezskórne zabiegi naprawcze zwezonych jatrogennie naczyn obwodowych. Opis pieciu przypadków.

Invasive percutaneous diagnostic or therapeutic procedures are associated with the risk of thrombosis and occlusion of peripheral vessels which are used for vascular access. Data on the transcatheter therapy of vascular complications in children are scarce. We described five children in four of whom percutaneous transluminal balloon angioplasty of occluded peripheral vessels was successfully performed. Technical aspects of this treatment and indications are discussed.

Use of muscular VSD Amplatzer occluder for closing right ventricular free wall perforation after hybrid procedure.

In some patients, so cold hybrid cardiologic procedures are performed whereas a heart is exposed surgically to puncture directly the heart cavity. After performing the interventional procedure, the opening in ventricular wall is closed surgically. We present our initial experience with closing the right ventricular (RV) cavity opening with the muscular ventricular septal defect Amplatzer occluder (A-MVSDO). In four sheep, heart was exposed surgically and punctured under direct vision by needle and guide wire, 14F and 26F sheath were introduced into right ventricle and the A-MVSDO was introduced causing closure of the RV opening. In all four cases, the occluder was successfully placed closing the opening in RV. No major bleeding after occlusion was observed. Following complications were observed: pneumothorax in two animals, rhythm disturbances and thrombus suspected in one. A-MVSDO occluder seems to be a useful device to close RV opening after hybrid interventional cardiologic procedure.

Aortoventriculoplasty and left ventricle function: long-term follow-up.

OBJECTIVE: Depressed cardiac function after aortoventriculoplasty is well known during the postoperative period. Little data exist concerning the long-term follow-up. The aim of this study is to determine whether septal incision has any permanent effect on the left ventricle function. METHODS: From 1988 to 2002, 45 patients received aortic mechanical prosthesis. These patients were divided into two groups. Group A consisted of 26 patients 5-18 years old, who underwent simple aortic valve replacement. Group B consisted of 19 patients 4-20 years old, who underwent the Konno procedure. Systolic and diastolic functions of the left ventricle were analyzed using echocardiography. For the systolic function, the following parameters were assessed: pressure gradient between left ventricle and ascending aorta, shortening and ejection fraction of the left ventricle. For the diastolic function, left ventricle-filling parameters were assessed: ratio of early to late filling velocity, deceleration slope of the early filling velocity and left ventricular isovolumetric relaxation time. Furthermore the percentage fraction of the aortic valve index (AOVI%) was calculated and compared between these two groups. RESULTS: After the surgery in group A, AOVI% dropped from 110+/-21 to 98+/-11%, while in group B it increased from 82+/-16 to 114+/-11%. As a result a higher residual pressure gradient across the aortic valve was noted in group A: 21.26+/-15 as compared to 11.17+/-5 mmHg in group B. A mean pressure above 30 mmHg appeared in group A 2 years after the surgery, while in group B this was obtained after 6 years. As for the diastolic function no significant difference was noted between these two groups. Overall there was one late death in group A, and in group B two early deaths, two reoperations because of excessive drainage and in two patients permanent pacemakers had to be implanted. CONCLUSIONS: Improvement of the systolic function after the surgery was noted in both groups. In patients with low AOVI%, postsurgical pressure gradient, either residual or recurrent, appeared during the follow-up. As for the septal incision, it may have some transient effects on the left ventricle function in the postoperative period, but no permanent sequelae were observed in the long-term follow-up.

Arterial switch operation in neonates with complex congenital heart defects.

The aim of the study was to analyze short-term results of the arterial switch operation in 29 neonates with simple transposition of the great arteries (group A) and 18 (group B) with complex heart defects: transposition with a ventricular septal defect (10), coarctation of the aorta (5), or Taussig-Bing anomaly (3). The operations were usually performed on the 7th day of life (2nd-30th day), after a Rashkind procedure when necessary. The mean weight was 3,530 +/- 780 g, body surface area was 0.219 +/- 0.032 m(2). Delayed sternal closure was necessary in 7 patients from group A (24%) and 8 from group B (44%) because of hemodynamic instability after weaning from extracorporeal circulation; these neonates had significantly lower body weights and smaller body surface areas. Perioperative mortality was 13.8% (4 patients) in group A and 27.8% (5 patients) in group B. Correction of complex transposition tends to be associated with a higher operative risk than simple transposition, but the difference was not significant.

Infection risk factors in pediatric cardiac surgery.

Cardiac operations were preformed in 499 children from January 1998 through December 1999. Their median age was 263 days. A positive culture from blood, bronchoalveolar lavage, wound, or central catheter was obtained in 110 patients (22%). Age, sex, presence of pulmonary hypertension, body surface area, ratio of body surface area to oxygenator surface area, whether heart surgery was open or closed, and the duration of the operation, cardiopulmonary bypass, intubation, and intensive care were analyzed. Patients who developed infections were significantly younger, with smaller body surface areas and disparity with the oxygenator surface area, longer operative and bypass times, extended intubation, and prolonged intensive care. There was a significant correlation between infection and pulmonary hypertension. Sex and type of operation were not predictors of infection.

[Stent implantation for aortic recoarctation--a case report]. / Poszerzenie zwezenia aorty przy uzyciu CP-stentu u dziecka po wszczepieniu protezy naczyniowej.

A case of a 17-year-old boy with a history of aortic coarctation, treated with surgery at the age of 10 years, is presented. Due to recoarctation, the patient underwent successful CP stent implantation.

Transcatheter closure of perimembranous ventricular septal defect using an Amplatzer occluder--early results.

BACKGROUND: Transcatheter closure of perimembranous ventricular septal defect with the use of an Amplatzer occluder was introduced in 1999, however, clinical experience with this method remains limited. AIM: To assess short-term safety and efficacy of this technique in children. METHODS: Since 2001, nine children underwent transcatheter closure of perimembranous ventricular septal defect in the cardiac centre in Bratislava (Slovak Republic) or in our centre. Patients were selected for the procedure based on the results of transesophageal echocardiography (TEE). Transcatheter closure using the Amplatzer occluder was guided by both TEE and angiography. The follow-up duration ranged from 3 to 22 months, mean 11.5 months. Control angiography was performed in order to detect any leakage through the aortic valve. The end-diastolic left ventricular diameter before and after the procedure as well as the function of the aortic, tricuspid and mitral valves were also assessed. RESULTS: The procedure was effective in all patients. No damage to atrio-ventricular nor aortic valves was observed. Tricuspid regurgitation, present prior to the procedure, markedly decreased following the closure of the defect. The left ventricular end-diastolic diameter significantly (p=0.001) decreased after the procedure. CONCLUSIONS: (1) Perimembranous ventricular septal defect can be safely and effectively closed using a transcatheter Amplatzer occluder. (2) Appropriate selection for the procedure as well as TEE and angiographic guidance during the procedure enable the avoidance of complications. (3) Tricuspid regurgitation is not an absolute contraindication to perform this procedure.

Echocardiographic parameters of left ventricular systolic and diastolic function in infants, children and adolescents before and after surgical correction of secundum atrial septal defect.

BACKGROUND: Left ventricular (LV) dysfunction has been described in adults with secundum atrial septal defect (ASD II) and heart failure (CHF). This anomaly has been rarely regarded as a cause of CHF in pediatric patients with ASD II. AIM: To assess LV systolic and diastolic function in patients with ASD II before and after cardiosurgery as well as to establish the prognostic value of these parameters in infants, children and adolescents with ASD II. METHODS: LV systolic (LVEF) and diastolic function parameters (E/A, DCT, IVRT) were studied using Doppler echocardiography in 104 patients aged between 1-18 years with ASD II before cardiosurgery and 4 years afterwards. These parameters were compared with similar variables in 150 healthy infants, children and adolescents. RESULTS: Before surgery LVEF was significantly lower only in infants with ASD II and CHF when compared with healthy controls. Relaxation abnormalities of LV diastolic function were observed before surgery in 40.4% of patients, especially in infants and adolescents with CHF. These disturbances were still present in about 20% of patients after surgery, especially in infants. Before and after surgery both LV and RV end-diastolic volumes were normal in about 6-10% of patients with LV diastolic dysfunction. CONCLUSIONS: In patients with ASD II and CHF diastolic dysfunction of LV is more common than systolic dysfunction. RV volume overload mainly affects LV diastolic function but it is not the only cause of CHF in patients with ASD II, especially in infants and adolescents. After surgery, normalisation of LV diastolic function is not observed in about 20% of patients with ASD II, especially in those who had CHF before operation. Special monitoring of the circulatory system is necessary in patients with ASD II operated in infancy and adolescence.

Importance of accurate diagnosis using real-time three-dimensional echocardiography in the surgical treatment of congenital intramitral ring in infants.

Congenital mitral ring is a rare type of congenital mitral stenosis. There are two types of mitral ring: the intramitral ring and the supramitral ring. Intramitral rings are often associated with complex valve pathology, and therefore have a worse prognosis than supramitral rings, in which the mitral apparatus is usually normal. The role of echocardiography in the evaluation of the mitral valve apparatus is crucial, because it allows an identification of the types of malformation, their hemodynamic repercussion and early predictors of the outcome. We describe two cases of intramitral ring, the diagnostic challenge they provided, and the value of the information obtained using real-time three-dimensional echocardiography in such cases.

Difficult to treat recurrent stenosis of the aorta.

The risk associated with repeated treatment of aortic stenosis is as high as 5% and increases to as much as 25% in complex heart diseases. Among the methods that are commonly accepted and used in the treatment of recurrent aortic stenosis are balloon dilatation and stent implantation. In this study we describe five patients with recurrent stenosis of the aorta treated with stent implantation. The short-term results of such treatment are promising. However, in some cases it is only palliative in character and does not completely resolve the problems arising from congenital heart disease. (Cardiol J 2007; 14: 186-192).

Congenital heart defect with associated malformations in children.

BACKGROUND: Children with multisystem involvement including congenital heart defect (CHD) are a very salient problem. The purpose of this study was to evaluate the incidence of CHD associated with malformations of other systems and to assess the modalities of treatment and perioperative mortality among patients referred to the department of pediatric cardiac surgery. METHODS: The medical records of 1856 children were reviewed retrospectively from 1997 to 2002 to establish CHD and types of associated malformations. The connections between CHD and other lesions were investigated. Furthermore, the influence of patient and perioperative variables on mortality risk was scrutinized. Univariate and multivariate analyses were used. RESULTS: Eighty-four children (4.53%) had CHD and associated malformations. The malformations of digestive (35.7%), urinary (22.4%), and nervous (14.3%) systems were the most frequently observed associated defects. No relation was found between CHD and concomitant lesions. The results of multivariate logistic regression showed significant influence of patient age, primary cardiac procedure, and CHD type on mortality (ca 19%) in children with multiorgan lesions. CONCLUSIONS: The treatment of children with CHD and associated multiple lesions is connected with higher mortality risk. The following factors: younger age, urgency of surgical procedure, and primary surgical procedure had negative impact on patient's outcome. However, these risks in certain cases are inevitable. The cardiac procedure preceding the surgical operation may improve the overall effect of treatment because of circulatory stabilization, provided that the condition of the patient does not preclude any intervention at all.