Interventional treatment and early-stage follow-up of pulmonary atresia with intact ventricular septum / 中华儿科杂志

Objective: To explore the application value of percutaneous peripheral interventional therapy in pulmonary atresia with intact ventricular septal (PA-IVS). Methods: Retrospective case summary. The data was collected from 25 children who were hospitalized at the Children's Hospital,Zhejiang University School of Medicine from August 2019 to August 2022, had been diagnosed with PA-IVS by echocardiography, and underwent interventional treatment. The sex, age, weight, operation time, radiation exposure time, and radiation dose of the patients were collected. The patients were divided into the arterial duct stenting group and the non-stenting group. Preoperative tricuspid annular diameters and Z scores, right ventricular length diameters, and right ventricular/left ventricular length-diameter ratios were compared by paired t-tests. Right ventricular systolic pressure difference, oxygen saturation, lactic acid before and after the surgery were compared for 24 children who received percutaneous balloon pulmonary valvuloplasty. Right ventricular improvement in 25 children after operation was analyzed. The correlation between postoperative oxygen saturation and postoperative right ventricular systolic blood pressure difference, the degree of pulmonary valve opening and the Z value of tricuspid valve ring in the non-stenting group were analyzed. Results: A total of 25 patients with PA-IVS were enrolled in the study, of whom 19 were males and 6 females, with an age at surgery of 12 (6, 28) days and a weight of (3.7±0.5) kg. One of them underwent only stenting of the arterial duct; 20 children underwent only percutaneous pulmonary valve perforation and balloon angioplasty; 4 children underwent both procedures. The Z-value of the tricuspid ring was -1.5±1.2 in the group with arterial duct stenting, and -0.1±0.4 in the group without stenting (t=2.77, P=0.010). The tricuspid regurgitant flow rate 1 month after surgery was significantly lower than the preoperative ((3.4±0.6) vs. (4.8±0.9) m/s, t=6.62,P<0.001). In the 24 children with percutaneous pulmonary valve perforation and balloon angioplasty, the preoperative right ventricular systolic blood pressure was (110±32) mmHg, and the postoperative systolic blood pressure was (52±19) mmHg (1 mmHg=0.133 kPa) (F=59.55, P<0.001). The factors that may affect postoperative oxygen saturation in 20 cases of non-stenting group were analyzed. The results suggested that the pre and post-operative right ventricular systolic blood pressure differences (r=-0.11, P=0.649), and the pulmonary valve orifice opening (r=-0.31, P=0.201) and tricuspid annulus Z value (r=-0.18, P=0.452) at 1 month after the operation were not significantly correlated with the postoperative oxygen saturation. Conclusions: Interventional therapy can be used as the first choice for one-stage operation of PA-IVS. Percutaneous pulmonary valve perforation and balloon angioplasty are more suitable for children with well-developed right ventricles, tricuspid annulus, and pulmonary arteries. While the smaller the tricuspid annulus, the more dependent it is on the ductus arteriosus and thus patients are more suitable for arterial duct stenting.

Outcomes of different types of pulmonary atresia in neonates treated by ductus arteriosus stenting / 中华儿科杂志

Objective: To summarize the outcomes of different types of pulmonary atresia in neonates treated by ductus arteriosus stenting. Methods: This study was a retrospective cohort study. A total of 19 neonates who had pulmonary atresia treated by ductus arteriosus stenting in Xinhua Hospital, Shanghai Jiao Tong University School of Medicine from April 2014 to June 2021 were included. They were divided into the intact ventricular septum (PA-IVS) group and the ventricular septal defect (PA-VSD) group. Ductus arteriosus stents were implanted by different approaches. These children were followed up regularly at the 1, 3, 6, and 12 months after the surgery and annually since then to evaluate the outcome. Independent sample t-test was used for the statistical analysis. Results: There were 12 children in PA-IVS group and 7 in PA-VSD group. All of them were full term in fants. The gestational age of the PA-IVS group and the PA-VSD group was (38.8±1.1) and (37.7±1.8) weeks, the birth weights were (3.2±0.4) and (3.4±1.1) kg, and the age at operation was (10±9) and (12±7) days, respectively, without significant difference (all P>0.05). Among the 12 children with PA-IVS, 9 had stents successfully implanted through the femoral artery and 3 through the femoral vein. Of the 7 children with PA-VSD, 2 had the stents successfully implanted via the femoral artery and 2 failed, and the remaining 3 had stents successfully implanted via the left carotid artery. There was no postoperative thromboembolism, arteriovenous fistula, pseudoaneurysm or other vascular complications. Five children with PA-VSD who had successful operations were followed up at 6 months of age. They all had the operation for pulmonary atresia, repair of the ventricular septal defect, removal of arterial duct stents, and ligation of the arterial duct. All children survived without any stent displacement or stenosis and biventricular circulation was achieved during the follow-up. Conclusions: Ductus arteriosous stenting can be the first-stage treatment for children with PA-IVS and PA-VSD. In addition to the traditional femoral vein and femoral artery approach, the carotid artery can be used as a route for stent placement.

Efficacy of transcatheter pulmonary valve perforation in neonates with pulmonary atresia with intact ventricular septum / 中华儿科杂志

Objective: To explore the efficacy and safety of transcatheter pulmonary valve perforation in the treatment of neonatal pulmonary atresia with intact ventricular septum (PA-IVS). Methods: The clinical data on surgical treatment and follow-up in 16 patients with PA-IVS who underwent transcatheter pulmonary valve perforation in Women and Children's Hospital, Qingdao University from October 2018 to October 2021 were analyzed retrospectively. The right ventricular systolic pressure and percutaneous oxygen saturation (SpO2) were compared before and after operation. In addition, the SpO2 and echocardiographic data at preoperative and the last follow-up were compared. Comparisons between groups were performed using paired-samples t test. Results: Among the 16 patients (10 males and 6 females) with the age at operation of 19 (14, 26) days, 12 cases underwent transcatheter pulmonary valve perforation successfully, 2 cases were transferred to surgery department for open-heart pulmonary valvulotomy, and the remaining 2 cases were transmitted to surgery department for transthoracic pulmonary valve perforation. The age at operation of the 12 patients who underwent transcatheter pulmonary valve perforation was 18 (14, 27) days, and the weight was (3.6±0.4) kg. The immediate postoperative right ventricular systolic pressure decreased significantly ((57±16) vs. (95±19) mmHg (1 mmHg=0.133 kPa), t=7.49, P<0.001), and the postoperative SpO2 was improved effectively (0.90±0.48 vs.0.75±0.09, t=-5.61, P<0.001). The follow-up time was 22 (7, 33) months for 12 patients who underwent transcatheter pulmonary valve perforation successfully. At the last follow-up, the ratio of right to left ventricular transverse diameter was significantly higher than that before operative (0.55±0.05 vs. 0.45±0.05, t=-3.27,P=0.007). Furthermore, the Z-scores of pulmonary valvular diameter (-0.78±0.23 vs. -1.73±0.56, t=-8.52, P<0.001) and the tricuspid valvular diameter (-0.52±0.12 vs. -1.46±0.38, t=-10.40, P<0.001) were all significantly higher than preoperative data. At last, all the patients achieved biventricular circulation without death or major complications. Conclusion: Transcatheter pulmonary valve perforation is a safe and effective therapy for neonatal PA-IVS, and its curative effect has been confirmed by the medium follow-up data.

Atresia bronquial / Bronchial atresia

Introducción: La atresia bronquial es una patología poco frecuente, generalmente asintomática e incidental en las imágenes del tórax. La aproximación diagnóstica de esta entidad se puede hacer por radiografía y broncoscopia, donde se identifican algunos aspectos claves en el diagnóstico diferencial. De acuerdo las manifestaciones clínicas y posibles complicaciones el tratamiento puede ser desde conservador hasta quirúrgico con resección lobar o segmentaria. Objetivo: Presentar las características de un caso con atresia bronquial. Presentación de caso: Se presenta un caso de atresia bronquial en una paciente de 19 años de edad cuyo tratamiento definitivo fue bilobectomía media-inferior derecha. Conclusiones: La atresia bronquial es una entidad infrecuente que puede cursar de manera asintomática y ser detectada por un hallazgo radiológico en pacientes adultos de manera incidental. El diagnóstico se puede confirmar por broncoscopia y el tratamiento casi siempre es quirúrgico(AU)

Introduction: Bronchial atresia is a rare disease, generally asymptomatic and incidental in chest images. The diagnostic approach of this entity can be done by radiography and bronchoscopy, some key aspects are identified in the differential diagnosis. According to the clinical manifestations and possible complications, the treatment can range from conservative to surgical with lobar or segmental resection. Objective: To describe a case of bronchial atresia. Case report: A case of bronchial atresia is reported in a 19-year-old patient whose definitive treatment was a right-lower-middle bilobectomy. Conclusions: Bronchial atresia is a rare entity that can occur asymptomatically and be detected incidentally by a radiological finding in adult patients. The diagnosis can be confirmed by bronchoscopy, and treatment is almost always surgical(AU)

Surgical strategies and results for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: experience of a single tertiary center

Abstract Objective: To evaluate surgical management and results of patients with pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries (PA/VSD/MAPCAs). Methods: We reviewed a consecutive series of patients with PA/VSD/MAPCAs between January 2012 and October 2018. Study patients were separated into Group A, efficient MAPCAs; Group B, hypoplastic MAPCAs; Group C, severe hypoplastic MAPCAs at all divisions; and Group D, distal stenosis at most MAPCAs divisions. Results: Thirty-six patients were included in the study. Median age at operation time was 5.5 months (2-110 months), median weight was 8 kg (2.5-21 kg), and median number of MAPCAs was three (1-6). In Group A, 14 patients underwent single-stage total correction (TC); in Group B, 18 patients underwent unifocalization and central shunting; and in Group C, four patients had aortopulmonary window creation and collateral ligation. No patient was placed in Group D. Seventy percent of patients (n=25) had the TC operation. Early mortality was not seen in Group A, but the other two groups had a 13.6% mortality rate. At the follow-up, three patients had reintervention, two had new conduit replacement, and one had right ventricular outflow tract reconstruction. Conclusion: Evaluating patients with PA/VSD/MAPCAs in detail and subdividing them is quite useful in determining the appropriate surgical approach. With this strategy, TC can be achieved in most patients. Single-stage TC is better than other surgical methods due to its lower mortality and reintervention rates. Care should be taken in terms of early postoperative intensive care complications and reintervention indications during follow-ups.

Off-pump bidirectional Glenn through right anterior thoracotomy / Glenn bidirecional sem uso de CEC via toracotomia anterior direita

AbstractThe Glenn operation involving anastomosis of the superior vena cava to the pulmonary artery has been performed for palliative operations of many cyanotic congenital heart diseases in addition to the single ventricle since the 1960s. The classic procedure is done via median sternotomy and cardiopulmonary bypass. The benefits of this procedure without the use of cardiopulmonary bypass remain mixed within reported series. Cases using this approach and off-pump technique together in Latin-America have not yet been reported in the scientific literature.

ResumoA operação de Glenn envolvendo anastomose da veia cava superior à artéria pulmonar foi realizada como procedimento paliativo de muitas doenças cardíacas congênitas cianóticas, além do ventrículo único desde os anos 1960. O procedimento clássico é feito por esternotomia mediana e circulação extracorpórea. Os benefícios deste procedimento sem o uso de circulação extracorpórea permanecem incertos dentro da série relatada. Casos utilizando conjuntamente esta abordagem e a técnica sem circulação extracorpórea na América Latina ainda não foram relatados na literatura científica.

Tetralogía de Fallot con atresia pulmonar: Morfopatología y anatomía quirúrgica / Tetralogy of Fallot with pulmonary atresia: Morphopathology and surgical anatomy

Se detalla la morfopatología de la tetralogía de Fallot con atresia pulmonar como un espectro de variaciones que sirve de fundamento para destacar la anatomía quirúrgica de esta cardiopatía y se muestra la base embriológica que determina su estructura. Se estudiaron 35 corazones con el sistema secuencial segmentario. Se determinó: situs atrial, conexiones entre los segmentos cardíacos y entre el ventrículo derecho y la vasculatura arterial pulmonar. El situs atrial fue solitus, predominó la conexión atrioventricular concordante (33), las conexiones ventriculoarteriales fueron concordantes (17), doble salida de ventrículo derecho (9) y única vía de salida (9). Todos presentaron atresia valvular pulmonar; el tronco pulmonar fue hipoplásico (20), atrésico proximal (6) y ausente (9), ramas pulmonares confluentes e hipoplásicas (25), ausencia de confluencia (5), ausencia total de ramas y de conductos arteriosos (5) y colaterales aortopulmonares (10). Los corazones mostraron un espectro morfopatológico de severidad que expresa la tendencia a la desaparición de la conexión entre el ventrículo derecho y la circulación arterial intrapulmonar. Se resaltan las formas de la irrigación arterial pulmonar como fundamento para unifocalizar el flujo hacia los pulmones y se hace hincapié en la utilidad de la clasificación anatomoquirúrgica de Barbero Marcial. El conocimiento embriológico es útil para entender las conexiones vasculares del ventrículo derecho con los derivados de los sextos arcos aórticos, los vasos arteriales intrapulmonares y las colaterales aortopulmonares.

The morphopathology of tetralogy of Fallot with pulmonary atresia is detailed as a spectrum of variations which is the foundation to highlight the surgical anatomy of this cardiopathy and it is shown the embryological basis which determines its structure. Thirty five hearts were studied with the methodology of the segmental sequential system. The atrial situs, the connections between the cardiac chambers and between the right ventricle and the arterial pulmonary vasculature were determined. The atrial situs was solitus, the concordant atrioventricular connection was the most frequent (33), the ventriculoarterial connections were concordant (17), double outlet right ventricle (9) and single outlet (9). All hearts had atresia of the pulmonary valve; the pulmonary trunk was hypoplastic (20), atretic proximally (6) and completely absent (9), confluent and hypoplastic pulmonary branches (25), absence of confluence (5), complete absence of pulmonary branches and arterial ducts (5) and presence of aortopulmonary collaterals (10). The hearts show a morphopathologic spectrum of severity which documents the tendency in disappearing the connection between the right ventricle and the intrapulmonary arterial circulation. The determination of the arterial supply to the lungs is highlighted to unifocalize the blood flow toward the lungs. The usefulness of Barbero Marcial's surgical classification is emphasized. The embryologic knowledge is basic in understanding the vascular connections between right ventricle and the derivatives of embryonic sixths aortic arches, the intrapulmonary arterial vessels and the aortopulmonary collaterals.

Balloon Valvuloplasty for Neonatal Critical Pulmonary Valvar Stenosis with IVC Interruption: Pitfalls of the Transumbilical Approach

Transcatheter balloon pulmonary valvuloplasty (BPV) is considered to be the treatment of choice for neonates with critical pulmonary valvar stenosis (PVS) or pulmonary valvar atresia with intact ventricular septum accompanied by reasonable right ventricular volume. The percutaneous femoral venous access is the most preferred route for BPV in most cardiac centers. We report herein the case of a newborn baby with critical PVS with inferior vena cava interruption, severe tricuspid regurgitation and a severely enlarged right atrium. We tried BPV through the transumbilical approach with difficulty, but he was successfully treated with the assistance of a coronary artery guiding catheter.

Tática para cirurgia de correção da coarctação da artéria pulmonar sem uso de circulação extracorpórea / Approach for surgical correction of pulmonary artery coarctation without cardiopulmonary bypass

A coarctação de artéria pulmonar é comum em pacientes com atresia pulmonar. A correção tem sido com CEC e no período neonatal, quando influencia o desenvolvimento das artérias pulmonares e o prognóstico. Foram corrigidos três pacientes por esternotomia mediana com atresia pulmonar dependentes do ducto arterioso (PCA) sem uso de CEC. O PCA mantinha a saturação durante a confecção do Blalock Taussig na artéria pulmonar contralateral. Arterioplastia foi realizada com sutura de pericárdio autólogo com PDS 7-0 e saturação mantida pelo Blalock. Todos pacientes tiveram boa evolução e alta hospitalar com avaliação de controle demonstrando bom alargamento da área coarctada.

Pulmonary artery coarctation often happens in patients with pulmonary atresia. The correction has been usually performed using cardiopulmonary bypass and during the neonatal period, influencing pulmonary artery development and prognosis. Three patients with pulmonary atresia with PDA underwent correction using median sternotomy without cardiopulmonary bypass. The PDA maintained the arterial saturation during Blalock Taussig anastomoses upon the contralateral pulmonary artery. Arterioplasty was performed using an autologous pericardium with 7-0 PDS running suture and saturation was maintained by Blalock shunt. All patients presented good follow-up and where discharged with good enlargment of coarctation area.

Diagnosis and management of cyanotic congenital heart disease: part II.

In this review, the clinical features and management of less commonly encountered cyanotic cardiac lesions are reviewed. Pathophysiology, clinical features, laboratory studies and management are discussed. The clinical and non-invasive laboratory features of these cardiac defects are sufficiently characteristic for the diagnosis and invasive cardiac catheterization and angiographic studies are not routinely required. Such studies may be needed either to define features that could not be clearly defined by non-invasive studies or prior to performing trans-catheter interventions. Surgical correction or effective palliation is possible at relatively low risk. But, residual defects, some requiring repeat catheter or surgical intervention, may be seen in a significant percentage of patients and consequently, continued follow-up after surgery is recommended.

Outcomes in Neonates with Pulmonary Atresia and Intact Ventricular Septum Underwent Pulmonary Valvulotomy and Valvuloplasty Using a Flexible 2-French Radiofrequency Catheter

PURPOSE: Outcomes in 6 neonates with pulmonary atresia and intact ventricular septum (PAIVS) undergoing radiofrequency pulmonary valvulotomy and valvuloplasty (RPVV) were reported to identify the factors favorable for RPVV as the treatment of choice. MATERIALS AND METHODS: From May 2000 to January 2008, 6 patients with PAIVS were included in this retrospective study. They were aged 1 day to 90 days old. Study modalities included review of recordings of presentations and profiles of chest radiography, electrocardiography, echocardiography, and cardiac catheterization with angiography. Hemodynamic profiles from the echocardiography and the cardiac catheterization were analyzed. RESULTS: Echocardiography showed severe tricuspid regurgitation, membranous atresia of the pulmonary valve, intact ventricular septum, patent ductus arteriosus, and hypoplastic right ventricle in 6 patients. The pulmonary valve annulus were 4.2 to 6.9 mm in diameters, and those of the tricuspid valve were 7.1 to 10.1 mm. Elevated serum level of cardiac enzymes were found in 1 patient with ventriculocoronary communication (VCC). At cardiac catheterization, the ratio of systolic pressure of the right ventricle to that of the left ventricle ranged from 1.43 to 2.33 before RPVV, and from 0.54 to 1.15 after RPVV (p=0.027). The pressure gradients ranged from 76 to 136 mmHg before RPVV, and from 15 to 39 mmHg after RPVV (p=0.028). The echocardiographic gradients ranged from 16 to 32 mmHg within 24 hours after RPVV, and from 15 to 50 mmHg at the follow-ups. CONCLUSION: RPVV can be a treatment of choice for neonates with PAIVS, if there is patent infundibulum, no right-ventricular dependent coronary circulation, and adequate tricuspid valve and pulmonary valve.

Anuloplastia de homoenxerto pulmonar criopreservado com anel de Delrin na atresia pulmonar com comunicação interventricular / Annuloplasty of cryopreserved pulmonary homograft with Delrin stent in pulmonary atresia with ventricular septal defect

Criança de seis anos portadora de atresia pulmonar com comunicação interventricular, submetida a correção total com um ano, empregando monoválvula de pericárdio bovino na reconstrução da via de saída do ventrículo direito. Evoluiu com importante regurgitação valvar pulmonar (RVP) e disfunção do ventrículo direito. Na reoperação foi implantado homoenxerto pulmonar criopreservado (HPC) com anuloplastia, utilizando anel de Delrin com o intuito de evitar distorção geométrica do conduto. Após dois anos, o ecocardiograma, semelhante ao pós-operatório imediato, demonstra RVP discreta e função ventricular direita normal, sugerindo que essa manobra pode ser utilizada como coadjuvante para otimizar o resultado do implante do HPC.

A six-year-old child was suffering from pulmonary atresia with ventricular septal defect after a total correction at 1 year of age using a bovine pericardial monocusp valve in the reconstruction of the right ventricle outflow tract. The infant evolved with significant pulmonary valve regurgitation (PVR) and right ventricle dysfunction. On reoperation, a cryopreserved pulmonary homograft (CPH) was implanted with annuloplasty utilizing a Delrin ring with the aim of avoiding geometric distortion of the vessel. After two years, an echocardiogram proved a similar state to the immediate postoperative period with slight pulmonary valve regurgitation and normal right ventricular function suggesting that this maneuver may be used as coadjuvant treatment to optimize the result of CPH implantation.

Avaliação do suprimento sangüíneo vascular pulmonar nos portadores de atresia pulmonar com comunicação interventricular e artérias colaterais sistêmico-pulmonares / Assessment of the pulmonary vascular blood supply in patients with pulmonary atresia, ventricular septal defect, and aortopulmonary collateral arteries

OBJETIVO: Analisar as características morfométricas das artérias pulmonares centrais e artérias colaterais sistêmico-pulmonares, avaliando a morfologia do suprimento sangüíneo vascular pulmonar, procurando estabelecer suas implicações no tratamento cirúrgico. MÉTODO: Entre janeiro/1990 e junho/2001, foram estudados 40 pacientes, incluíndo-se os que apresentavam estudo cineangiocardiográfico completo e prévio à primeira intervenção cirúrgica. Analisaram-se as características morfométricas das artérias pulmonares centrais e artérias colaterais sistêmico-pulmonares, assim como a distribuição da irrigação sangüínea nos pulmões. Calcularam-se os índices arterial pulmonar (IAAPP), arterial colateral sistêmico-pulmonar (IACSP) e arterial neopulmonar total (IANPT = IAAPP + IACSP). O tratamento cirúrgico foi considerado paliativo (TP), paliativo definitivo (TPD) e definitivo (TD). RESULTADOS: O TP foi predominante. Não houve diferenças estatisticamente significantes entre os pacientes com TP, TPD e TD, em relação ao IAAPP, IACSP e IANPT. Comparando o IAAPP e o IACSP, não houve diferença entre os índices para o TD (p=0,4309), o IACSP foi maior que o IAAPP para o TP (p=0,0176) e descritivamente também maior para o TPD. O IANPT dos pacientes em TD foi maior que os em TP (p=0,0959). Foram identificados cinco subgrupos morfologicamente semelhantes, denominados: B1, B2, B3, B4 e B5. A mortalidade total foi de 17,5 por cento. CONCLUSÃO: A morfologia do suprimento sangüíneo vascular pulmonar das artérias pulmonares centrais e artérias colaterais sistêmico-pulmonares mostrou-se soberana na orientação do tratamento cirúrgico. Independentemente da divisão didática em subgrupos, o TP foi predominante. A mortalidade não apresentou correlação com as características morfométricas.

Síndrome de cimitarra asociado a atresia pulmonar con comunicación interventricular: Primer caso reportado / Scimitar syndrome associated to pulmonary atresia with interventricular communication: First reported case

Se presenta el caso de un masculino de 15 meses de edad con síndrome de cimitarra y atresia pulmonar con comunicación interventricular. El diagnóstico se hizo mediante cateterismo cardíaco y angiocardiografía y confirmado por el estudio de autopsia. Se hacen las consideraciones clínicas y quirúrgicas de esta excepcional asociación que a la fecha es, hasta donde sabemos la primera reportada en la literatura.

We present the case of a 15 months-old male with Scimitar Syndrome associated with ventricular septal defect and pulmonary atresia. The diagnosis was made by cardiac catheterization and angiography and was confirmed by the necropsy. Clinical and surgical considerations of this exceptional association were made. To the best of our knowledge this is the first case reported in the relevant literature.

Recent advances in pediatric cardiology--electrophysiology, transcatheter and surgical advances.

Whereas the medical advances were reviewed in the previous paper, electrophysiological, transcatheter and surgical advances will be the focus in this review. Greater understanding of the arrhythmias, development of non-pharmacological treatment, namely catheter ablation and internal cardioverter-defibrillator (ICD) and miniaturization pacemakers and IDs have occurred in the last decade so that the methods could be applied to smaller and more complex patient population. Surgery has been the traditional treatment option for palliation and correction of congenital and acquired heart defects in infants and children. During the last one to one and one-half decades, a remarkable number of transcatheter methods were developed and refined. These developments during the last decade were reviewed and include long-term results of balloon dilatation procedures, transcatheter closure of atrial septal defects, patent ductus arteriosus, and ventricular septal defect, percutaneous valve replacement, intravascular stents to manage vascular obstructive lesions that can not be satisfactorily balloon-dilated, catheter completion of Fontan procedure, myocardial reduction in hypertrophic cardiomyopathy and other miscellaneous procedures. Recent advances in the transcatheter modes of therapy have added a new dimension to the management of neonates, infants and children with heart disease. They should now be added to the armamentarium available to the Pediatrician and Pediatric Cardiologist in the management of cardiac problems in the pediatric patient. Surgical methods and concepts have been greatly refined such that surgery can be undertaken even in the sickest and most complex patient. The majority of congenital heart defects can be corrected by open heart surgery; some require prior palliation and others can be operated without prior palliation. Recent advances in various defects were reviewed.