Pulmonary homograft dysfunction after the Ross procedure using decellularized homografts-a multicenter study.

OBJECTIVES: Pulmonary homograft dysfunction is a limitation after the Ross procedure. Decellularized pulmonary homografts can potentially mitigate this complication. The aim of this study was to examine the incidence, predictors, progression, and morphology of pulmonary homograft dysfunction using data from the Canadian Ross Registry. METHODS: From 2011 to 2019, 466 consecutive patients (mean age: 47 ± 12 years, 73% male) underwent a Ross procedure using a decellularized cryopreserved pulmonary homograft (SynerGraft SG; CryoKife, Kennesaw, Ga). Pulmonary homograft dysfunction was defined as any of the following: peak pulmonary gradient ≥30 mm Hg, pulmonary regurgitation >2, or pulmonary homograft reintervention. Patients meeting ≥1 of these criteria (n = 30) were compared with the rest of the cohort (n = 436). Median follow-up is 2.2 years (maximum = 8.5 years) and 99% complete (1176 patient-years). RESULTS: The cumulative incidence of pulmonary homograft dysfunction was 11 ± 2% at 6 years. Pulmonary homograft stenosis was the most frequent presentation (n = 28 patients, 93%). Morphologically, stenosis occurred most often along the conduit (59%). Overall, 4 patients required homograft reintervention. At 6 years, the cumulative incidence of homograft reintervention was 3 ± 1%. The instantaneous risk was greatest in the first year after surgery (3.5%/year) and decreased to <1%/year thereafter. Patient age <45 years was the only independent risk factor associated with pulmonary homograft dysfunction (hazard ratio, 3.1, 95% confidence interval, 1.1-8.6, P = .03). CONCLUSIONS: The use of decellularized cryopreserved pulmonary homografts results in a low incidence of dysfunction and reintervention after the Ross procedure. The risk is greater in the first postoperative year. Younger age is the only independent risk factor for pulmonary homograft dysfunction.

Congenital polyvalvular disease expands the cardiac phenotype of the RASopathies.

The RASopathies are a group of similar genetic syndromes with cardiovascular abnormalities, characteristic facial features, short stature, abnormalities of the skin and musculoskeletal system, and variable neurodevelopmental challenges. The most common cardiovascular abnormalities include pulmonary valvular stenosis and hypertrophic cardiomyopathy. Congenital polyvalvular disease (CPVD) refers to congenital dysplasia of two or more cardiac valves. We diagnosed a RASopathy in two individuals with CPVD and noted that CPVD in RASopathies has rarely been reported in the literature. Thus, we performed a retrospective chart review and literature review to investigate the association and characterize the phenotype of CPVD in the RASopathies. CPVD was present in 2.5% (n = 6/243) of individuals in our RASopathy cohort. Involvement of two cardiac valves, commonly the aortic and pulmonic valves, was seen in the majority of individuals (6/8; 75%) in our cohort, but only 27% (3/11) of reported CPVD and RASopathy cases in the literature. CPVD should be considered an associated cardiovascular phenotype of the RASopathies, which has implications for diagnosis and management.

Outcomes of Right Ventricular Outflow Tract Reconstruction in Children: Retrospective Comparison Between Bovine Jugular Vein and Expanded Polytetrafluoroethylene Conduits.

Bovine jugular vein (BJV) and expanded polytetrafluoroethylene (ePTFE) conduits have been described as alternatives to the homograft for right ventricular outflow tract (RVOT) reconstruction. This study compared RVOT reconstructions using BJV and ePTFE conduits performed in a single institution. The valve functions and outcomes of patients aged < 18 years who underwent primary RVOT reconstruction with a BJV or ePTFE conduit between 2013 and 2017 were retrospectively investigated. 44 patients (20 and 24 with BJV and ePTFE conduits, respectively) met the inclusion criteria. The mean follow-up time was 4.5 ± 1.5 years. No significant differences in peak RVOT velocity (1.8 ± 0.9 m/s vs 2.1 ± 0.9 m/s, P = 0.27), branch pulmonary stenosis (P = 0.50), or pulmonary regurgitation (P = 0.44) were found between the BJV and ePTFE conduit groups, respectively. Aneurysmal dilatation of the conduit was observed in 25.0% of the patients in the BJV conduit group but not in the ePTFE conduit group (P = 0.011). All the cases with aneurysmal dilatation of the BJV conduit were complicated with branch pulmonary stenosis up to 3.0 m/s (P = 0.004). No conduit infections occurred during the follow-up period, and no significant difference in conduit replacement (20.0% vs 8.3%, P = 0.43) was found between the BJV and ePTFE conduit groups, respectively. The outcomes of the RVOT reconstructions with BJV and ePTFE conduits were clinically satisfactory. Aneurysmal dilatation was found in the BJV conduit cases, with branch pulmonary stenosis as the risk factor.

Epidemiological study of congenital heart diseases in dogs: Prevalence, popularity, and volatility throughout twenty years of clinical practice.

The epidemiology of Congenital Heart Diseases (CHDs) has changed over the past twenty years. This study aimed to evaluate the prevalence of CHDs in the population of dogs recruited in a single referral center (RC); compare the epidemiological features of CHDs in screened breeds (Boxers) versus non-screened (French and English Bulldogs, German Shepherds); investigate the association of breeds with the prevalence of CHDs; determine the popularity and volatility of breeds over a 20-year period; analysed the trends of the most popular breeds in the overall population of new-born dogs registered in the Italian Kennel Club (IKC) from 1st January 1997 to 31st December 2017. The RC's cardiological database was analysed, and 1,779 clinical records were included in a retrospective observation study. Descriptive statistics and frequencies regarding the most representative breeds and CHDs were generated. A logistic regression model was used to analyse the trends of the most common CHDs found in single and in cluster of breeds. The relationship between breed popularity and presence of CHDs was studied. The most common CHDs were Pulmonic Stenosis, Patent Ductus Arteriosus, Subaortic Stenosis, Ventricular Septal Defect, Aortic Stenosis, Tricuspid Dysplasia, Atrial Septal Defect, Double Chamber Right Ventricle, Mitral Dysplasia, and others less frequent. The most represented pure breeds were Boxer, German Shepherd, French Bulldog, English Bulldog, Maltese, Newfoundland, Rottweiler, Golden Retriever, Chihuahua, and others in lower percentage. Chihuahuas, American Staffordshire Terriers, Border Collies, French Bulldogs, and Cavalier King Charles Spaniel were the most appreciated all of which showed a high value of volatility. This study found evidence for the value of the screening program implemented in Boxers; fashions and trends influence dog owners' choices more than the worries of health problems in a breed. Effective breeding programs are needed in order to control the diffusion of CHDs without impoverishing the genetic pool.

Prevalence of Noonan spectrum disorders in a pediatric population with valvar pulmonary stenosis.

OBJECTIVE: To evaluate the prevalence of Noonan spectrum disorders (NSD) in a pediatric population with valvar pulmonary stenosis (vPS) and identify the clinical characteristics that differentiate those with NSD from those without NSD. DESIGN: A retrospective chart review of 204 patients diagnosed with vPS between 9/1/2012 and 12/1/2016 at a pediatric medical center was performed. The quantitative features of vPS, genetic diagnosis information, and phenotypic characteristics of Noonan syndrome were collected. Chi-square test, Fisher's exact test, t test, Wilcoxon rank-sum test, and ANOVA were used for comparisons among the groups. Logistic regression was used to test for the association between the clinical characteristics and the presence of NSD. RESULTS: Syndromic diagnoses were made in 10% of the children with vPS, with NSD accounting for 6%. Hypertrophic cardiomyopathy (P < .0001), short stature (P < .0001), developmental delay (P < .0001), ophthalmological abnormalities (P < .0001), pectus carinatum/excavatum (P = .01), neurological abnormalities (P = .022), and aortic stenosis (P = .031) were present more often in individuals with NSD compared to nonsyndromic vPS. A logistic regression analysis showed a 4.8-fold increase in odds for NSD for each additional characteristic (P < .0001). CONCLUSIONS: At least 6% of the children with vPS have an underlying NSD. Individuals with vPS and NSD were significantly more likely to have additional features known to be associated with NSD than those with vPS without NSD. We conclude that vPS in the presence of one or more significant characteristics should prompt referral for genetic evaluation as a guide to ascertain patients at risk for NSD while optimizing the use of clinical genetics evaluation and potential genetic testing.

Outcomes after Balloon Pulmonary Valvuloplasty for Critical Pulmonary Stenosis and Incidence of Coronary Artery Fistulas.

Outcome data for neonates with critical pulmonary stenosis (PS) is limited. We aimed to review the outcomes after balloon pulmonary valvuloplasty (BPV) for neonates with critical PS at our institution. All neonates with critical PS who underwent BPV from 1990 to 2017 were included. A total of 44 neonates underwent BPV for critical PS. Nonright ventricular dependent coronary artery fistulas was seen in 6/44 (13.6%) patients. Tricuspid valve z-scores were -1.9 (interquartile range [IQR] -3.04, -0.48) in those with coronary artery fistulas as compared with -0.27 (IQR -0.5, 0.8) in those without (p = 0.03). Fifteen of forty-four subjects (34.1%) patients underwent reintervention with 10 patients (22.7%) requiring an alternate source of pulmonary blood flow (3 patients subsequently underwent right ventricular overhaul, 2 underwent Glenn operations, and 1 underwent repeat BPV). Five patients underwent reintervention for right ventricular outflow tract obstruction. Pulmonary valve annulus z-score was significantly smaller in those who needed reintervention -2.4 (IQR -2.9 to -0.95) versus -0.59 (IQR -1.3, -0.15); p = 0.02. At a median follow-up of 8.2 (IQR 3.4 to 13.1) years, moderate or severe pulmonary regurgitation was seen in 22/42 (53.7 %) patients with biventricular circulation, 3 requiring pulmonary valve repair/replacement. In conclusion, coronary artery fistulas occur in a significant number of patients with critical pulmonary stenosis, occurring more frequently in patients with small tricuspid valves. Reintervention is required for 1/3 of patients. Patients with small pulmonary valve annuli are more likely to undergo reintervention for right ventricular outflow tract obstruction. Significant pulmonary regurgitation is common and may require eventual pulmonary valve replacement.

Sudden death in patients with Ebstein anomaly.

Aims: Ventricular dysfunction or structural alteration of either ventricle is a well-established risk factor for sudden death (SD). Ebstein anomaly (EA) can present with both right and left heart abnormalities; however, predictors of SD have not been described. We therefore sought to characterize the incidence and risk factors of SD among a large cohort of patients with EA. Methods and results: All EA patients who underwent evaluation at a high-volume institution over a 4-decade period were retrospectively reviewed. Clinical variables, cardiovascular surgical procedure(s), and cause of death were recorded. Sudden death incidence from birth and following tricuspid valve (TV) surgery were estimated using the Kaplan-Meier method. Cox regression analysis was used to identify clinical and surgical predictors of SD. The cohort comprised of 968 patients [mean age 25.3 years, 41.5% male; 79.8% severe EA, 18.6% accessory pathway, 0.74% implantable cardioverter-defibrillator (ICD) placement]. The 10-, 50-, and 70-year cumulative incidences of SD from birth were 0.8%, 8.3%, and 14.6%, respectively. Prior ventricular tachycardia [hazard ratio (HR) 6.37, P < 0.001)], heart failure (HR 5.64, P < 0.001), TV surgery (HR 5.94, P < 0.001), syncope (HR 2.03, P = 0.019), pulmonic stenosis (HR 3.42, P = 0.001), and haemoglobin > 15 g/dL (HR 2.05, P = 0.026) were multivariable predictors of SD. In a similar subgroup analysis of patients who underwent TV surgery, all of the above factors except syncope were significantly associated with post-operative SD on multivariable analysis. Conclusion: Patients with EA are at significant risk for SD. Key clinical SD predictors identified can aid in risk stratification and potentially guide primary prevention ICD implantation.

[Interauricular communications in children: diagnosis and treatment, about 49 cases at the Children?s University Hospital in Dakar]. / Les communications interauriculaires chez l'enfant: diagnostic et prise en charge à propos de 49 cas aux CHU pédiatriques de Dakar.

Interauricular communication (IAC) is the second most important congenital heart disease in children. It accounts for 6-8% of congenital cardiac malformations in children. However, many questions are still open about this pathology. Thus, this study aims to report the prevalence rate of IAC in hospital but especially to describe its clinical, paraclinical and therapeutic aspects. For this purpose, we conducted a retrospective descriptive study. Data were collected by means of a questionnaire and then entered and analyzed in Sphinx (V5). The prevalence in Hospital was 2x1000. The average age of patients was 37 months, the sex-ratio was 0,75. No prenatal diagnosis was made. Parental consanguinity was detected in 30% of cases. Respiratory infections were found in 24% of cases. Cardiomegaly was found in 35 cases with hypervascularization in 63% of cases. Ultrasound showed a predominance of ostium secundum; wide IAC was found in 63% of cases. Pulmonary stenosis was the most important associated heart attack. Pulmonary arterial hypertension was found in 63% of the cases. Treatment was based on diuretics, which were largely used and only 7 children underwent surgery. Evolution was favorable in 39 patients reflecting a rate of 79%. Early diagnosis of IAC should be improved as well as surgical treatment of IAC affecting infants.

Pulmonary stenosis in complicated monochorionic twin pregnancies: prevalence, management and outcome.

OBJECTIVE: To review prevalence, management and prognostic factors of pulmonary stenosis (PS) in monochorionic diamniotic (MCDA) pregnancies complicated by twin-to-twin transfusion syndrome (TTTS). METHODS: Retrospective study over the last 10 years in a single referral center. We reviewed fetal echocardiography data of all MC twin cases with diagnosis of isolated PS. We assessed fetoscopy characteristics of those that underwent laser coagulation. We collected data regarding perinatal outcome, neonatal echocardiography and cardiac management. RESULTS: We found 24 cases of isolated PS among 2091 MCDA pregnancies. Among 1052 complicated MCDA that underwent fetal laser surgery, 22 (2.09%) developed PS of which 20 were diagnosed prenatally. Two cases were diagnosed in uncomplicated MCDA pregnancies (0.2%). Four of 22 (18.18%) cases with TTTS showed in utero regression after laser treatment. Thirteen newborns (65%) required valvular dilatation. Peak systolic velocities in the pulmonary artery trunk (PSV-PA) at diagnosis and the interval between the diagnosis of TTTS and that of PS were significantly different (p < 0.001 and p = 0.05 respectively) between PS requiring cardiac intervention and those who did not. CONCLUSION: An elevated PSV-PA at the time of PS diagnosis and a short time-interval between fetoscopic laser surgery and PS diagnosis are predictive of the need for interventional treatment after birth.

[Supravalvular aortic stenosis with and without coronary lesions in pediatrics. A Lebanese study]. / STÉNOSE SUPRAVALVULAIRE AORTIQUE AVEC OU SANS LÉSIONS CORONAIRES EN PÉDIATRIE--ÉTUDE LIBANAISE.

OBJECTIVE: This study aims to analyze several parameters concerning the supravalvular aortic stenosis (SVAS) in children such as age of diagnosis, place of residence, the existence of a metabolic disorder or dysmorphic syndrome and possible damage to the coronary ostia, the means of diagnosis and outcome of these patients. MATERIALS AND METHODS: A large group of patients (2868) with congenital heart disease enrolled between 1 May 1999 and 30 April 2010 at the National Register of Pediatric and Congenital Heart Disease, Lebanese Society of Cardiology. RESULTS: SVAS were found in 14 patients (0.5%) aged 8.5 months to 15 years. The Williams Beuren syndrome was the most common etiology (6 cases) without ostial stenosis, asymptomatic and not treated, followed by forms without dysmorphic syndrome (5 cases) and without ostial stenosis, one patient was operated because of severe SVAS; finally, 3 cases of homozygous familial hypercholesterolemia treated differently: a patient had a successful liver transplantation at age of 4.5 years but has developed, despite the normalization of cholesterol level, a SVAS associated with severe ostial lesions 10 years after transplantation, another treated by coronary artery bypass graft surgery and the latter treated medically. CONCLUSION: SVAS is a very rare disease, but its discovery must lead to search for coronary lesions especially in presence of homozygous familial hypercholesterolemia. The ultrasound monitoring is mandatory and is designed to detect this anomaly and early coronary lesions. The slightest suspicion should carry out more extensive explorations to detect ostial stenosis.

Complete atrioventricular septal defect and pulmonary stenosis diagnosed in a 49-year-old woman after 10 uneventful births.

Atrioventricular septal defects constitute 4% of all congenital cardiac malformations. Patients with complete atrioventricular septal defect rarely survive for decades without surgical treatment. Pulmonary stenosis can provide a delicate balance between the pulmonary and systemic circulations and thereby increase longevity. We present the case of a 49-year-old woman whose complete atrioventricular septal defect and associated pulmonary stenosis were diagnosed only after she had given birth to 10 live children through uneventful spontaneous delivery. We discuss her successful surgical treatment in terms of the available medical literature.

Fibrotic airway stenosis following radiotherapy in patients with adenoid cystic carcinoma.

BACKGROUND AND OBJECTIVE: Radiotherapy is usually administered to the central airway in patients with unresectable adenoid cystic carcinoma (ACC). The purpose of this study was to describe the outcomes of endobronchial intervention in patients with airway stenosis following radiotherapy for ACC. Moreover, we investigated the incidence and contributing factors for airway stenosis following radiotherapy for ACC. METHODS: Forty-seven patients with ACC, who underwent radiotherapy of the tracheobronchial tree from January 1995 to December 2011, were reviewed retrospectively. Fibrotic airway stenoses were diagnosed using three-dimensional computed tomography, flexible bronchoscopy or both. RESULTS: Eleven (23%) of the 47 patients with ACC suffered fibrotic airway stenosis following radiotherapy and received bronchoscopic intervention. The median interval from radiotherapy to diagnosis of fibrotic airway stenosis was 7 months. Low forced expiratory volume in 1 s (FEV1), FEV1 /forced vital capacity and brachytherapy were verified as factors contributing to radiotherapy-induced airway stenosis. Bronchoscopic intervention provided both symptomatic relief and improvement of lung function, and no procedure-related death or major complication was observed. Insertion of a straight silicone stent was required in 10 patients (91%), and 4 (36%) eventually received Y-shaped silicone stents. The stents, once implanted, could not be removed in any of the patients; stents were well-tolerated for a prolonged period in all patients. CONCLUSIONS: Fibrotic airway stenosis following radiotherapy in patients with ACC is often found. Bronchoscopic intervention, including silicone airway stenting, was a safe and useful method for treating radiotherapy-induced fibrotic airway stenosis in patients with ACC.

Decreased incidence of supravalvar pulmonary stenosis after arterial switch operation.

BACKGROUND: Supravalvar pulmonary stenosis (SVPS) is frequently observed after arterial switch. Traditionally the coronary arteries are removed from the neopulmonic root by excising the entire sinus of Valsalva. As a result, reconstruction of the neopulmonic root requires a pericardial patch encompassing two-thirds of the anastomosis between the neopulmonic root and pulmonary artery. We present a technique where the coronary arteries are removed as limited buttons of sinus tissue, leaving the transected edge of the neopulmonic root intact. We hypothesize that maintaining native arterial tissue in the anastomosis between the neopulmonic root and the pulmonary artery bifurcation reduces postoperative SVPS. METHODS AND RESULTS: We performed a retrospective review of neonates with D-transposition of the great arteries undergoing arterial switch procedure from 1996 to 2009. Charts were reviewed, and clinical outcomes recorded for each patient. Most recent echocardiograms were evaluated for right ventricular outflow tract obstruction. A total of 120 patients received arterial switch using this technique. There was 99% survival and no injuries to the coronary arteries regardless of anatomy. Total follow-up was 564 patient-years. Mean follow-up at last clinical visit was 66 ± 46 months. Evaluation of the most recent outpatient echocardiogram revealed an average peak instantaneous gradient across the neopulmonic root of 22.5 ± 5 mm Hg. Only 7 (5%) patients required reintervention (balloon dilation, n=5; surgery, n=2). CONCLUSIONS: Our technique of removing the coronary arteries as limited buttons, and anastomosis of the pulmonary artery using only native arterial tissue provides excellent midterm results with minimal SVPS.

[Parental smoking during early pregnancy and congenital heart defects]. / Tabagisme parental en début de grossesse et cardio- pathies congénitales.

OBJECTIVES: Analyze the possible role that can play parental smoking during early pregnancy on the occurrence of different types of congenital heart defects (CHD). MATERIALS AND METHODS: Over a period of 4.5 years (between January 1, 2006 and June 30, 2010), data on all children who had an echocardiography were collected and entered in the National Register of Paediatric and Congenital Heart Disease, Lebanese Society of Cardiology, Beirut. Patients were divided into two groups: group I without CHD, and group II with CHD. Variables recorded were: age of mother, place of residence, parental smoking, the type of CHD, the sex of the child and consanguinity of the parents. We used nonparametric tests for comparing the two populations. The p value was considered significant when inferior to 0.05. RESULTS: Group I consisted of 2466 patients and group II of 793 patients. Only valvar pulmonary stenosis (VPS) was highly associated with mothers who smoked 20 cigarettes or more per day (p = 0.03). When the mother and father consumed at least 20 cigarettes per day, VPS and coarctation of the aorta were associated with a significant p-value (0.03 and 0.02 respectively). CONCLUSION: Parental smoking in early pregnancy can be considered a risk factor for valvar pulmonary stenosis and coarctation of the aorta. Cessation of smoking by the parents before pregnancy could reduce the risk of their occurrence.

Risk factors for prosthesis failure in pulmonary valve replacement.

BACKGROUND: After initial right ventricular outflow tract reconstruction, replacement of the pulmonary valve (PVR) with a bioprosthetic valve may be performed. Bioprosthetic valves fail (PVF) and require repeat replacement. Identification of risk factors for PVF would be useful for clinicians choosing among various options for the initial PVR. METHODS: We retrospectively analyzed outcomes of 169 consecutive patients (55% male) with repaired tetralogy of Fallot or pulmonary stenosis undergoing a first PVR. Data were abstracted from the medical records, including gender, diagnosis, indication for PVR, age at PVR (< 10 years or ≥ 10 years), type of valve, and time of PVF. Actuarial freedom from PVF was compared by log rank and parametric survival analysis. Risk factors for PVF were analyzed by univariate and multivariate methods. Prosthesis types for PVR were pulmonary homograft in 56, stented porcine valve in 16, stented porcine valve in Dacron (DuPont, Wilmington, DE) conduit in 26, and bovine pericardial valve in 71. RESULTS: Indication for PVR was pulmonary stenosis in 21% and insufficiency in 79%. Median follow-up for the entire cohort was 8 years. PVF occurred in 24 patients at a median time of 5.7 years. Actuarial freedom from PVF at 10 years was 72% for all valve types, 55% for porcine valve in Dacron conduit, 60% for homograft, 75% for porcine valve, and 78% for bovine pericardial valve (p = 0.36). By univariate analysis, young age (p < 0.0001), male gender (p = 0.0017), and indication of pulmonary stenosis (p = 0.015) were risk factors for PVF. In multivariate analysis, tetralogy of Fallot anatomy (p < 0.06), younger age (p < 0.02), and use of a homograft valve (p < 0.02) were risk factors for early PVF (<3 years). Young age (p < 0.0001) at time of PVR was associated with late PVF. CONCLUSIONS: Freedom from reoperation for PVR during 10 years of follow-up is excellent. Younger age, tetralogy of Fallot, and use of a homograft valve were risk factors for early PVF. Only younger age at PVR was a significant risk factor for late PVF.

[Interventional therapy for structural heart diseases in People's Liberation Army hospitals between 2005 and 2006].

OBJECTIVE: To evaluate the efficacy and safety of interventional therapy in structural heart diseases in 38 PLA hospitals. METHODS: Data including patient number underwent interventional therapy for structural heart disease, procedure details, immediate procedural complications were retrospectively collected in all the military hospitals between January 2005 and December 2006. RESULTS: Successful interventional therapy was achieved in 8692 out of 8862 patients (98.08%) with structural heart disease. Transcatheter closure of ventricular septal defect (VSD), atrial septal defect (ASD) and patent ductus arteriosus (PDA), and percutaneous balloon dilatation of mitral valve stenosis (MS) and pulmonary valve stenosis (PS) were the most performed procedures (97.99%). Up to 91.23% patients underwent transcatheter closure with domestic devices. The incidence of procedure-related complications was 4.33% (n = 384) which were most frequently associated with VSD closure. The commonest procedural complications included conduction blockades (n = 260), residue shunt (n = 42), device detachment (n = 30) and tricuspid incompetence (n = 22). Although the procedures performed in 2005 and 2006 increased 57.32% compared with those in 2003 and 2004, the success rate and the incidence of complications remained unchanged. CONCLUSION: Percutaneous treatment of structural heart disease is a safe and feasible alternative to surgery. Simulate complications arise long after the treatment, which suggests the importance of long-term follow-up for those patients who hare undergone interventional therapy.

Prevalence and surgical approach of supravalvular pulmonary stenosis after Jatene operation for transposition of great arteries.

BACKGROUND: The Transposition of the Great Arteries is the most frequent congenital cyanogenic cardiopathy in the neonatal period, corresponding to 7% of all congenital cardiopathies. Among the operations for surgical treatment, the Jatene operation, with arterial correction, is the treatment of choice. During the late postoperative evolution, some problems were observed, with the most common being the occurrence of supravalvular stenosis at the neopulmonary, regardless of the type of surgical technique used. OBJECTIVE: To study and analyze the prevalence of stenosis, as well as describe the surgical treatment and propose technical maneuvers to prevent its onset. METHODS: Of the 553 patients that underwent surgery, 409 were discharged from the hospital and 281 had late follow-up; 59 (20.9%) presented different degrees of supravalvular pulmonary stenosis and 21 had a mean gradient > 60 mmHg, needing surgical treatment. Depending on the location and anatomy of the stenosis, the surgical treatment consisted of the use of different techniques, such as the enlargement of stenosis areas with bovine pericardium patches, resection of stenotic areas and termino-terminal anastomosis, replacement of retracted patches and synthetic tubes. RESULTS: Twenty patients presented good evolution and only one patient died. CONCLUSION: It can be concluded that the supravalvular pulmonary stenosis, post-Jatene operation for Transposition of Great Arteries, had a prevalence of 20.9%; once identified and with indication for treatment, it can be treated surgically with low mortality levels, through different surgical techniques; to prevent the occurrence of stenosis, ample dissection and release of the pulmonary branches, double anastomoses, large patches of autologous pericardium and careful reconstruction of the aorta are proposed, which prevents the compression of the neopulmonary.

Prevalência e abordagem cirúrgica da estenose supravalvar pulmonar pós-operação de jatene para transposição das grandes artérias / Prevalence and surgical approach of supravalvular pulmonary stenosis after jatene operation for transposition of great arteries

FUNDAMENTO: A transposição das grandes artérias é a cardiopatia congênita cianogênica mais freqüente no período neonatal, correspondendo a 7 por cento de todas as cardiopatias congênitas. Dentre as operações para tratamento cirúrgico, a operação de Jatene, com correção arterial, é o tratamento escolhido. Durante a evolução pós-operatória tardia, alguns problemas foram observados, sendo o mais comum a ocorrência de estenose supravalvar na neopulmonar, independentemente do tipo da técnica cirúrgica utilizada. OBJETIVO: Estudar e analisar a prevalência da estenose, bem como descrever o tratamento cirúrgico e propor manobras técnicas para prevenir seu aparecimento. MÉTODOS: Dentre 553 pacientes operados, 409 tiveram alta hospitalar e 281 seguidos tardiamente; 59 (20,9 por cento) apresentaram diferentes graus de estenose supravalvar pulmonar e 21 gradiente médio superior a 60 mmHg, necessitando tratamento cirúrgico. Dependendo da localização e da anatomia da estenose, o tratamento cirúrgico constou de aplicação de diferentes técnicas, como ampliação das áreas de estenose com remendos de pericárdio bovino, ressecção de áreas estenóticas e anastomose término-terminal, substituição de remendos retraídos e de tubos sintéticos. RESULTADOS: Houve boa evolução em 20 pacientes, com óbito em um dos casos. CONCLUSÃO: Conclui-se que a estenose supravalvar pulmonar pós-operação de Jatene para transposição das grandes artérias teve prevalência de 20,9 por cento. Uma vez identificada e com indicação de tratamento, pode ser tratada cirurgicamente com baixa mortalidade, mediante diferentes técnicas cirúrgicas. Para prevenir a ocorrência de estenose, propõem-se ampla dissecção e liberação dos ramos pulmonares, anastomoses amplas, remendos amplos de pericárdio autólogo e cuidado na reconstrução da neoaorta, evitando compressão da neopulmonar.

BACKGROUND: The Transposition of the Great Arteries is the most frequent congenital cyanogenic cardiopathy in the neonatal period, corresponding to 7 percent of all congenital cardiopathies. Among the operations for surgical treatment, the Jatene operation, with arterial correction, is the treatment of choice. During the late postoperative evolution, some problems were observed, with the most common being the occurrence of supravalvular stenosis at the neopulmonary, regardless of the type of surgical technique used. OBJECTIVE: To study and analyze the prevalence of stenosis, as well as describe the surgical treatment and propose technical maneuvers to prevent its onset. METHODS: Of the 553 patients that underwent surgery, 409 were discharged from the hospital and 281 had late follow-up; 59 (20.9 percent) presented different degrees of supravalvular pulmonary stenosis and 21 had a mean gradient > 60 mmHg, needing surgical treatment. Depending on the location and anatomy of the stenosis, the surgical treatment consisted of the use of different techniques, such as the enlargement of stenosis areas with bovine pericardium patches, resection of stenotic areas and termino-terminal anastomosis, replacement of retracted patches and synthetic tubes. RESULTS: Twenty patients presented good evolution and only one patient died. CONCLUSION: It can be concluded that the supravalvular pulmonary stenosis, post-Jatene operation for Transposition of Great Arteries, had a prevalence of 20.9 percent; once identified and with indication for treatment, it can be treated surgically with low mortality levels, through different surgical techniques; to prevent the occurrence of stenosis, ample dissection and release of the pulmonary branches, double anastomoses, large patches of autologous pericardium and careful reconstruction of the aorta are proposed, which prevents the compression of the neopulmonary.

Long-term results (up to 17 years) of pulmonary balloon valvuloplasty in adults and its effects on concomitant severe infundibular stenosis and tricuspid regurgitation.

BACKGROUND: Significant infundibular stenosis and significant tricuspid regurgitation (TR) occasionally result from severe pulmonary valve stenosis in adults, and these 2 conditions have an adverse impact on morbidity and mortality in patients who undergo corrective surgery. The goal of this study was (1) to evaluate the long-term (up to 17 years) outcome of pulmonary balloon valvuloplasty (PBV) in adults and (2) to determine the effect of successful PBV on severe infundibular stenosis and severe TR. METHODS: Pulmonary balloon valvuloplasty was performed in 90 consecutive patients (49 women, 41 men) of mean age 23 +/- 9 years (range 15-54 years) with congenital pulmonary valve stenosis. Clinical and echocardiographic assessment was performed 2 to 17 years (mean 10 +/- 3.9 years) after PBV. Repeat cardiac catheterization was performed 6 to 24 months after PBV in 43 patients who had concomitant moderate to severe infundibular stenosis (infundibular gradient > or = 30 mm Hg). RESULTS: There were no immediate or late deaths. The mean catheter peak pulmonary gradient (gradient between pulmonary artery and right ventricular body) before and immediately after PBV was 105 +/- 39 and 34 +/- 26 (P < .0001), respectively. The corresponding values for right ventricular pressure were 125 +/- 38 and 59 +/- 21 mm Hg (P < .0001), respectively. The infundibular gradient (in 43 patients) immediately after PBV was 42.9 +/- 24.8 (30-113) mm Hg, and it regressed at second catheterization to 13.5 +/- 8.3 mm Hg (P < .0001), whereas cardiac index improved from 2.68 +/- 0.73 to 3.1 +/- 0.4 L min(-1) m(-2) (P < .05). Doppler pulmonary gradient before PBV and at 1-year and long-term follow-up were 91 +/- 33 (range 36-200), 28 +/- 12 (range 10-60) (P < .0001), and 26 +/- 11 (range 7-60) mm Hg (P = .2), respectively. New mild pulmonary regurgitation was noted in 24 patients (28%) after PBV. Significant TR in 7 patients either regressed or disappeared after PBV. CONCLUSIONS: Long-term results of PBV in adults are excellent. Severe infundibular stenosis and severe TR regressed after successful PBV. Therefore, PBV should be considered as the treatment of choice for adult patients with valvular pulmonary stenosis even in the presence of severe infundibular stenosis or severe TR.