Double aortic arch anomalies in Children: A Systematic 20-Year Single Center Study.

Aortic arch anomalies underlie numerous congenital disorders. Effectively diagnosing and treating them requires close understanding of cardiovascular embryology. As our Center serves the entire pediatric population of our country, we performed a comprehensive retrospective analysis of all aortic arch anomalies diagnosed at our Center over the past 20 years. We analyzed 40 children with aortic arch anomalies, distinguishing two defect types: Group 1 displayed ring-forming anomalies, and Group 2 other types of aortic arch anomalies that did not form a vascular ring. We performed detailed morphological analyses using echocardiography, angiography, computed tomography, or magnetic resonance imaging and generated a catalog of all aortic arch anomalies present. Group 1 was represented by 25 patients; 40% with persistent both aortic arches, and 60% with various forms of right aortic arch and an incomplete left aortic arch. Group 2 was represented by 15 patients with complex heart defects. On the basis of our dataset, the incidence of all aortic arch anomalies was 0.033%, and of ring-forming pathologies 0.021%. Although aortic arch anomalies are rare, it is important to diagnose them correctly. It is critical to distinguish ring-forming types. Although in complex defects the aortic arch anomaly represents only an additive diagnosis, its correct definition could be crucial for further management. Cumulatively, this unique, long-term study provides a systematic patient registry and offers critical epidemiological data to aid the study of rare congenital cardiovascular defects. Clin. Anat. 30:929-939, 2017. © 2017 Wiley Periodicals, Inc.

Congenital pulmonary lymphangiectasia in patient with pulmonary vein stenosis/atresia.

No abstract Keywords.

Stenting of stenotic aortopulmonary collaterals in non-surgically treated adolescent or adult patients with pulmonary atresia, ventricular septal defect and multiple aortopulmonary collaterals.

OBJECTIVES: To present our experience with stent implantations in non-surgically treated patients with pulmonary atresia, ventricular septal defect and stenotic aortopulmonary collaterals. METHODS: Between April 2007 and June 2009, 6 stents in 4 patients were implanted into stenosed segments of MAPCAs. The median age and weight of patients at stent implantation were 24 years (range 13-34 years) and 58.5 kg (range 56-70 kg), respectively. RESULTS: All implantations were successfully performed; there were no procedure associated complications. The median diameter increase of stenosed MAPCAs was 107%. The mean arterial blood saturation increased from mean 78% to 84%. An early increase in the median exercise duration measured by 6MWT was 36%. Neointimal in-stent fibroproliferation in 4/6 stents and stent fracture in 1/6 patients occured in mid-term follow-up. CONCLUSION: Percutaneous catheterizational stent implantation into stenosed segments of MAPCAs is a palliative procedure, that might improve quality of life of non-surgically treated patients with PA, VSD, MAPCAs. Neointimal in-stent fibroproliferation and stent fracture can be expected as complications in mid-term follow-up. CT-angiography is recommended to confirm these complications in progressive satO2 decrease (Fig. 3, Ref. 12).

The optimal timing for magnetic resonance imaging in long-term follow-up of patients after a complete correction of the Tetralogy of Fallot.

OBJECTIVES: Long-term pulmonary regurgitation (PR) leads to right ventricular (RV) dilatation and dysfunction. In patients after a complete correction of the tetralogy of Fallot (TOF) it represents the most frequent and high risk late complication. Magnetic resonance imaging (MRI) is an objective possibility for RV measurements; on the other hand MRI is complicated and not always accessible method. BACKGROUND: We analyzed echocardiography (ECHO) and MRI parameters regarding RV dilatation and function and the correlation between these two methods with the aim to define optimal ECHO parameters indicating a necessity for further investigations. PATIENTS AND METHODS: In 50 patients with TOF > 10 years, after a complete surgical correction, ECHO and MRI were performed. RESULTS: ECHO finding of end-diastolic diameter (EDD) of RV > 30mm was detected in 13 patients (26%) and in 30 patients (60%) RV EDD above 4 standard deviations (SD) their normal values was present. MRI finding of end-diastolic volume (EDV) > 160 ml/m2 was present in 14 patients (28%) and end-systolic volume (ESV) > 85 ml/m2 in 17 patients (34%). Significant correlations between ECHO and MRI RV diastolic measurements were found (p=0.0001, r=0.34, resp. p=0.001, r=0.39). PR was significantly affecting diastolic RV dilatation (p=0.0001), on the other hand. RV dysfunction resulted in systolic RV dilatation (p=0.007). PR did not correlate with RV function (p=0.56). CONCLUSIONS: MRI is a golden standard for exact RV measurements and for the indication of pulmonary valve replacement, but ECHO still can be used during long-term follow-up, defining the point for further and more exact RV measurements (Tab. 2, Fig. 11, Ref. 17). Full Text in free PDF www.bmj.sk.

Shock--manifestation of pulmonary atresia with aorto-pulmonary collaterals and single ventricle physiology in a 2-day neonate.

BACKGROUND: The combination of pulmonary valve atresia and ventricular septal defect accounts for about 2% of cases of congenital heart disease. Most of the cases have intracardiac anatomy of Tetralogy Fallot and present with cyanosis in neonatal age. OBJECTIVES: To report a case of a newborn with rare combination of pulmonary atresia, ventricular septal defect and single ventricle physiology presenting with shock very early following birth. METHODS AND RESULTS: We describe a newborn infant who developed shock with severe metabolic acidosis and respiratory distress several hours following birth. Cardiac ultrasound showed pulmonary atresia and ventricular septal defect with single ventricle physiology. Cardiac cathetrisation revealed major aorto-pulmonary collaterals with an excessive pulmonary blood flow. Resuscitative measures resulted in hemodynamic stability. However, due to unfavorable prognosis, a decision not to undertake surgical palliation was made. Withdrawal of intensive care led to rapid demise of the patient. CONCLUSIONS: We report a case of a newborn with pulmonary atresia, ventricular septal defect and single ventricle physiology in whom an excessive flow through major aorto-pulmonary collaterals led to shock and death very early following birth (Fig. 1, Ref. 5). Full Text (Free, PDF) www.bmj.sk.

The first experience with the high-resolution multi-slice CT-angiography in pediatric cardiology in Slovakia.

OBJECTIVES: The high-resolution multi-slice computed tomography angiography (HRMS-CTA) is a new imaging method characterized by a precise isotropic imaging of any cardiovascular system structure. The purpose of this study was to review the first experience with the high-resolution multi-slice HRMS-CTA in pediatric patients with congenital heart defects as well as with acquired cardiac diseases in the Slovak Republic. METHODS: We retrospectively reviewed benefits of HRMS-CTA and its influence on the subsequent choice of the most appropriate management in 20 patients with pulmonary atresia with ventricular septal defect and multiple aortopulmonary collaterals (PA, VSD, MAPCAs) and in 15 patients with different diagnoses with expected benefit from HRMS-CTA in terms of an exact delineation of extra-cardiac vascular structures. RESULTS: The delineation of the examined extra-cardiac structures was very detailed and clear in all 35 patients. All findings definitely contributed to the choice of the most appropriate management not only in PA, VSD, MAPCAs patients (20), but also in 15 patients with different diagnoses. CONCLUSIONS: HRMS-CTA is a new complementary imaging method with the potential to replace invasive catheterization procedures in large number of patients who need extra-cardiac vascular structure imaging (Fig. 1, Ref. 18). Full Text (Free, PDF) www.bmj.sk.

Neuropsychological outcome in children with hypoplastic left heart syndrome.

OBJECTIVES: The purpose of this study is to review a single-institutional experience gained over one decade, to assess late neuropsychological outcome in patients as well as to determine Norwood operation risk factors that are related to patient and procedure and influence these results. BACKGROUND: The reports of first successful staged operation of hypoplastic left heart syndrome (HLHS) and its numerous modifications have improved the early survival also in patients with complex forms. Specifically, the identification of other specific risk factors related to patient or procedure in the first stage of palliation for HLHS may play a role in cognitive deficit. METHODS: Single-institutional, cross-sectional study of neurodevelopmental outcome. The mean age at testing was 6.9 +/- 0.3 years. There were 19 patients enrolled with completed psychological testing Standford-Binet test. RESULTS: The mean neurocognitive results were significantly below the population normative values. The mean full-scale IQ for the entire cohort was 84.1 +/- 8.2. The predictive factors are strongly associated with severe metabolic acidosis, low birth weight, hospital stay over 35 days, prolonged duration of DHA and aortic valve diameter playing an important role in the subsequent psychological outcome, especially in differential subsections of IQ scale (Tab. 2, Ref. 15). 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).

Echocardiographic measurements of the aorta in normal children and young adults.

BACKGROUND: To be able to determine aortic valve and ascending aorta pathology, especially aortic root dilatation, it is important to establish normal aortic dimensions. The aim of the study was to measure the dimensions of the aorta in normal healthy children and young adults in Slovakia. PATIENTS AND METHODS: 702 healthy subjects, from newborns to 20 years of age, were examined at our institution. The study was carried out prospectively, by a single observer, using digitized two-dimensional (2D), Doppler and M-mode echocardiography. The aorta was measured at 3 sites: 1. aortic valve annulus, 2. sinuses of Valsalva, 3. sinotubular junction. Patients were divided into 28 groups according to their body surface area (BSA)--from 0.15 to 2.0 m2. All data were statistically evaluated (mean value, 5th and 95th percentile for all BSA groups) and regression equations were calculated for each parameter. RESULTS: All 3 measured aortic parameters correlated closely. Measures of correlation (R-squared) for aortic parameters with the square root of BSA were high: 0.89 for aortic valve annulus, 0.86 for sinuses of Valsalva and 0.86 for sinotubular junction (Tab. 3, Fig. 7, Ref 13). Full Text (Free, PDF) www.bmj.sk

Experiences with fenestration closure in patients after the Fontan operation.

BACKGROUND AND OBJECTIVES: [corrected] In this study, decision and timing of fenestration closure are discussed. We review our experiences with transcatheter occlusion of fenestration or residual baffle leak using Amplatzer occluder. PATIENTS AND METHODS: We have retrospectively reviewed the cath lab records of 26 patients who have undergone closure of fenestration (or another baffle leak) in period of 2002 to July 2006. Patients with the previous fenestrated Fontan procedure (intracardiac lateral tunnel, extracardiac conduit) with the presence of clinically important cyanosis, arterial desaturation less than 88 %, previously described leak by echocardiography, impaired exercise capability, absence of effusions and appropriate hemodynamic parameters were included in the study. RESULTS: The most frequent group were patients with hypoplastic left heart syndrome (HLHS) (27 %), second most common was L-transposition of great vessels (TGA), tricuspidal valve atresia (TA) and atresia of pulmonary artery (PA) (all of them 15.4 %). CONCLUSIONS: Our results confirm that after adjusting the hemodynamical risk, transcatheter fenestration occlusion in high risk patients might be a safe and effective method to significantly increase arterial O2 saturation. The long term benefits of closure especially at exertion are yet to be shown (Tab. 2, Ref 21). Full Text (Free, PDF) www.bmj.sk.

Percutaneous closure of perimembranous ventricular septal defects with the eccentric Amplatzer device: multicenter follow-up study.

Using an Amplatzer membranous eccentric occluder, 186 patients with an average weight of 43.5 kg (range 12.5-77) underwent attempted catheter closure of a perimembranous venricular septal defect (PMVSD). Their age ranged from 3 to 51 years, with the an average age being 15.9 years. The patients were divided into three groups according to morphology of PMVSD: 106 patients with single PMVSD, 63 patients with single PMVSD with aneurysmatic formation, and 17 patients with multiple VSD with aneurysmatic formation. Using angiography, PMVSDs were measured to be between 2.5 and 12 mm, with an average of 5.1 mm. In the third group of patients, we did not measure the size of PMVSD and a device was selected according to the size of entry to the aneurysm. The device was successfully implanted in all patients. The immediate closure rate was 90% in the first group, increasing to 100% at 1 month and remained at that level during follow-up. The immediate closure rate in the second group was 98% and remained the same during follow-up. The immediate closure rate in the third group was 89% and during 1 year of follow-up remained the same. There was no clinical evidence of hemolysis and no incidence of device embolization or bacterial endocarditis after implantation. Before the procedure, all patients showed normal electrocardiogram (ECG) or left ventricle enlargement. After the procedure (at least 3 months later) ECG showed left anterior hemiblock (LAH) in nine patients, complete right bundle branch block in eight patients, and incomplete right bundle branch block in seven patients. A complete heart block (CHB) developed in 2 patients after the procedure (1.07%). The first patient developed LAH immediately after closure and CHB within 24 hours, The heart rate was 28 beats per minute. After treatment with steroids and atropine, CHB changed to sinus rhythm with LAH within 2 months. One year later, the ECG revealed the same findings. The second patient developed CHB immediately after the procedure and was on temporary pacing for 1 week. After 1 month, the patient recovered to sinus rhythm and ECG showed LAH.

Is a learning curve for arterial switch operation in small countries still acceptable? Model for cooperation in Europe.

OBJECTIVES: To assess the results of a cooperative arrangement between Slovakia and Slovenia for neonatal cardiac surgery. The aim of the study was to analyze the performance of this approach for complete transposition of the great arteries (D-TGA). METHODS: Due to the overall small number of new patients with D-TGA in Slovenia a decision was made to avoid a prolonged learning curve by centralizing the experience of two countries at one center. Since 1995 the center in Slovakia has become the only referral center for Slovenia. Between February 1993 and June 2002 in this center, 147 patients with D-TGA underwent arterial switch operation (ASO). The median age at operation was 11 days, with 110 patients from Slovakia and 37 patients from Slovenia. RESULTS: Overall hospital mortality was 4.8% (seven patients). The 1, 2, 3, 4 and 5 year survival rate was 95% with the mean follow-up of 4 years. Operation before 1997 (P=0.0001) was identified as a risk predictor for death by multivariate analysis. There are no deaths among the 90 patients operated on after 1996. All patients are without medication with normal left ventricular function. Stenosis (gradient >30 mmHg) was noted in the pulmonary artery reconstruction in seven patients (5%). More than mild aortic regurgitation was noted in five patients (4%). The incidence of redo or reintervention was 5% at 5 years of follow-up. CONCLUSIONS: In the current era a prolonged learning curve for ASO is not acceptable to most European countries and their patients. The risk of surgery can be minimized by concentrating surgical experience as part of the quality control of congenital heart programs. If the number of new patients is small due to the birth rate and size of the population, institutions should merge activity. Such centralization amplifies the experience to the benefit of the patient.

The role of transesophageal echocardiography in transcatheter closure of secundum atrial septal defects by the Amplatzer septal occluder.

BACKGROUND: Our purpose was to determine the role of transesophageal echocardiography (TEE) in the closure of atrial septal defects by the Amplatzer septal occluder (ASO) (AGA Medical, Golden Valley, Minn). METHODS: A total of 240 patients with atrial septal defect (ASD) secundum were examined by transthoracic 2-dimensional echocardiography (TTE) and TEE to determine the ASD morphologic features, diameter, and rims. During transcatheter closure TEE was used for determination of the ASD diameter and guidance of the ASO implantation. RESULTS: Sixteen (6%) patients were found not suitable for transcatheter closure with TTE, 35 (14%) with TEE, and 2 during catheterization. Twenty-eight patients (18%) had partial or total deficiency of the posterior, inferoanterior, or inferoposterior rim, 54 (27%) had a centrally positioned ASD, 92 (46%) had insufficient superoanterior rim, and 9 had multiple ASDs, whereas 8 had a septal aneurysm associated with a single defect and 4 a multiperforated aneurysm. A total of 170 patients underwent implantation of ASO. The ASO was correctly positioned in 144 at the first attempt. In the remainder TEE revealed unstable position of the left atrial disk (12), opening of both atrial disks in the left atrium (5), deployment of the device through the smaller defect in patients with multiple ASDs (3), and, in 1 patient, the device was too small and had to be replaced by a larger one. CONCLUSIONS: Morphologic variations of the ASD are common. TEE is crucial for the determination of the ASD morphologic features, diameter, and rims, which are crucial for proper patient selection. TEE allows precise guiding and positioning of the ASO, which is essential for safe and effective transcatheter ASD closure.

Morphological variations of secundum-type atrial septal defects: feasibility for percutaneous closure using Amplatzer septal occluders.

The aim of the study was to assess the morphology of secundum-type atrial septal defects (ASD) with a view to percutaneous closure using Amplatzer septal occluders (ASO). One hundred and ninety patients who underwent closure of isolated secundum-type ASD between September 1995 and January 2000 were included. The morphology of the defects was studied using transthoracic and transesophageal echocardiography. Patients with defects of suitable morphology and size underwent percutaneous closure using ASO. The remaining patients underwent surgical closure. Centrally placed defects were observed in 46 patients (24.2%). Morphological variations of secundum-type ASD were detected in 144 patients (75.8%). One hundred and fifty-one patients (79.5%) underwent percutaneous closure using ASO. Thirty-nine patients (20.5%) underwent surgical closure. Centrally placed defects, defects with deficient superior anterior rim, multiple defects, and perforated aneurysms of the interatrial septum are morphological variations of secundum-type ASD suitable for percutaneous closure using ASO. Cathet Cardiovasc Intervent 2001;53:386-391.

"Cobra-like" deformation of an Amplatzer septal occluder.

Transcatheter occlusion of secundum atrial septal defects using Amplatzer septal occluders (ASOs) is a safe, simple, and effective alternative to surgical closure. We present the first report of a "cobra-like" shape deformation of the device encountered during inappropriate positioning. The complication is reversible and is easily avoidable.

Effects of angiotensin-converting enzyme inhibitor and angiotensin type 1 receptor antagonist in deoxycorticosterone acetate-salt hypertensive mice lacking Ren-2 gene.

We previously reported that inhibition of angiotensin-converting enzyme (ACE) prevented the hypertension and left ventricular hypertrophy induced by deoxycorticosterone acetate-salt (DOCA-salt) in 129/SvEvTac mice, which have 2 renin genes (Ren-1 and Ren-2). In the present study, we induced hypertension by uninephrectomy and DOCA-salt in mice having only the Ren-1 gene (C57BL/6J) and investigated the effect of an ACE inhibitor (ramipril, 4 mg. kg(-)(1). d(-)(1)) and an angiotensin type 1 (AT(1)) receptor antagonist (L-158809, 4 mg. kg(-)(1). d(-)(1)) on the development of hypertension, cardiac hypertrophy, and renal injury. After 4 weeks of treatment, systolic blood pressure in DOCA-salt mice was significantly increased (128+/-2 mm Hg) compared with controls (109+/-2 mm Hg) (P:<0.001), while plasma renin concentration was decreased by 97% (P:<0.001). DOCA-salt also induced left ventricular and renal hypertrophy and renal damage as manifested by proteinuria. Collagen content in the left ventricle and kidney was significantly higher in DOCA-salt mice (P:<0.001). Urinary albumin (P:<0.05) and proliferating cell nucleic antigen-positive cells in the tubules and interstitium of the renal cortex (P:<0.001) were significantly increased in the DOCA-salt group. Neither the ACE inhibitor nor the AT(1) antagonist had any antihypertensive effect; however, they partially prevented cardiac hypertrophy and completely inhibited left ventricular collagen deposition. In the kidney, both the ACE inhibitor and AT(1) antagonist partially reduced the increase in collagen but had no effect on hypertrophy. They also significantly prevented the effect of DOCA-salt on urinary albumin and proliferating cell nucleic antigen expression in the kidney. Despite the lack of an antihypertensive effect, both ACE inhibitor and AT(1) antagonist prevented cardiac remodeling and renal damage. Our results indicate that ACE inhibitors and AT(1) antagonists exert beneficial effects on the heart and kidney in DOCA-salt hypertensive mice independently of their effects on blood pressure.

Early experience with the Amplatzer ductal occluder for closure of the persistently patent ductus arteriosus.

Using an Amplatzer duct occluder, 106 patients (weight 21 +/- 18 kg) underwent an attempt at catheter closure of a persistently patent ductus arteriosus (PDA). Their age ranged from 22 days to 48 years. The PDA measured between 1.2 to 8.1 mm at its narrowest diameter. The device was successfully implanted in 105 patients. The immediate closure rate was 70% and gradually increased to 100% at 1-month follow-up. There was no clinical evidence of hemolysis and no incidence of device embolization or bacterial arteritis. Doppler evaluation showed no evidence of aortic arch or pulmonary artery obstruction. The device, which is currently undergoing multicenter clinical trial in the United States, is proving to be a safe and effective device for closure of the persistently PDA.

[Transcatheter closure of ostium secundum atrial septal defects--initial experience with treatment of adult patients]. / Katetrizacní uzáver defektu septa síní typu ostium secundum--nase první zkusenosti s lécbou dospelých nemocných.

The authors present their initial experience with the use of Amplatzer septal occluder for transcatheter closure of secundum atrial septal defect. For patients where the secundum atrial septal defect type is haemodynamically significant or when paradoxical embolization via the defect is suspected, the catheterization technique is an alternative for cardiosurgical correction of the defect. The morphology of the defect which is the basic criterium for indication of a transcatheter closure is evaluated on transoesophageal echocardiographic examination. The closure takes place under skiascopic and transoesophageal echocardiographic control. The procedure in all three patients (2 women--age 58 and 62 years, 1 man--24 years) treated at our department was without complications. Consistent with formerly published data it may be concluded from our initial experience that when all indication criteria are respected the closure of secundum atrial defect by means of Amplatzer septal occluder is a safe technique and a relatively easy operation with a high rate of primary success.

Percutaneous closure of patent ductus arteriosus: complementary use of detachable Cook patent ductus arteriosus coils and Amplatzer duct occluders.

UNLABELLED: Several different devices were evaluated for the percutaneous closure of patent ductus arteriosus (PDA), and important drawbacks were found in all of them. To overcome these drawbacks, both detachable Cook PDA coils and Amplatzer duct occluders (ADO) were used for the percutaneous closure of PDA. A total of 54 patients underwent transcatheter occlusion of PDA at a median age of 4.5 years (range 0.5-29 years) and at a median weight of 19.5 kg (range 6-69 kg). Three patients were adults. Detachable Cook PDA coils were used in 26 patients with a median PDA diameter of 1.7 mm (range 1.1-2.2 mm) and ADO were used in 28 patients with a median PDA diameter of 3.8 mm (range 1.9-7.5 mm). Devices were successfully implanted in all 54 patients. Complete closure was achieved in 53 of 54 patients (98% closure rate). Median fluoroscopy time was 12 min (range 4-47 min). CONCLUSION: According to our experience, the complementary use of detachable Cook patent ductus arteriosus coils and Amplatzer duct occluders for the percutaneous closure of PDA can be recommended.