Development of PR & Restenosis after Percutaneous Balloon Pulmonary Valvuloplasty

Background: Pulmonary stenosis is not an uncommon congenital heart disease in our population. Various age groups present with severe pulmonary stenosis require intervention either surgical or percutaneous catheter-based. Often lifesaving surgical intervention is not feasible considering procedural risk, the patient’s physical condition, and young age. Percutaneous balloon pulmonary valvuloplasty is safe, effective, almost painless, economical, and less hospital stay in any age group with immediate and long-term success. This study aimed to analyze the rate of development of (Pulmonary Regurgitation) PR & restenosis after Percutaneous Balloon Pulmonary Valvuloplasty (PBPV). Material & Methods: This hospital-based prospective observational study was conducted in the Department of Paediatric Cardiology Combined Military Hospital (CMH), Dhaka from January to December 2021. 0-18 years aged 50 population with PS who were referred to the Paediatric Cardiology Department of CMH Dhaka during the study period were selected as study subjects as per inclusion criteria. The purposive Sampling technique was used in this study. Data were collected with a predesigned standard data collection sheet. Statistical significance was set as a 95% confidence level at a 5% acceptable error level. Categorical variables were expressed as frequency and percentages. Mean, standard deviation, and/or medians with interquartile ranges were used to express the continuous variables. Paired t-test was performed in categorical comparison. A p-value of <0.05 was considered statistically significant. Collected data were analyzed using SPSS Version 24. Written informed consent was taken from every parent. Ethical clearance was taken from the Ethics Review Committee of CMH Dhaka for conducting the study. Results: 50(6.39%) had isolated valvular PS. The international standard for the prevalence of isolated PS is 8-10% which is very close to this study’s result. In this study, male predominance was found with the male-female ratio of 3:2 though there was no gender dominance found in other studies. Out of 50 cases, 3(6%) are neonates,7(14%) are infants, 27 (54%) are 1-5 years aged, and 13(26%) are more than 5 years aged. A total of 13(26%) cases developed PR, and all 13 patients developed trivial PR on D1 echo, out of them 1 patient developed mild PR on D30 Echo but in D90 and D180 Echo patients persisted the same mild PR. Out of 50 cases, 46(92%) were successful and 4 cases were procedure failed. None of the successful cases developed restenosis. Out of 4 procedure failed cases 1 patient developed restenosis on D30 follow up and 2 patients develop restenosis on the D90 follow-up. At D180 follow up none of the patients developed restenosis. All the restenosis cases had dysplastic pulmonary valves. One patient’s PS gradient persisted at 30-50 mm of Hg throughout the follow-up period. Overall restenosis in 180-day follow-up is 3(6%) and the remaining 47(94%) cases lead a healthy life in 6 months follow-up. Reballooning was done immediately after diagnosis of restenosis in stenosed cases. Conclusion: Immediate and intermediate-term (6 months) follow-up with Doppler echocardiography showed excellent outcomes in all cases except 3 (6.0%) restenosis cases and 13 (26.0%) insignificant PR cases. The baby who all had restenosis, had the risk factor, like- dysplastic PV. It may be concluded that balloon valvuloplasty is the procedure of choice for moderate, severe, and critical pulmonary valve stenosis.

Value of cardiac MRI in evaluation of left atrial function in the postoperative tetralogy of Fallot and pulmonary stenosis patients / 中华放射学杂志

Objective:To explore the value of cardiac MRI (CMRI) in evaluating left atrial function in patients with postoperative tetralogy of Fallot (rTOF) and postoperative pulmonary stenosis (rPS).Methods:Totally 67 pediatric patients (49 with rTOF, 18 with rPS) with preserved left ventricular ejection fraction (EF) were recruited between January 2019 and October 2021 in Shanghai Children′s Medical Center, School of Medicine, Shanghai Jiao Tong University. Thirty-three healthy volunteers, matched in gender and age, were included as controls from July 2017 to August 2018. Left atrial EF, strain and strain rate of three phases (reservoir, conduit and pump), left atrial volume (maximum volume index, minimum volume index and pre-atrial contraction volume index) were measured with corresponding cardiac function analysis software. Then, the differences in these parameters were analyzed between the three groups by ANOVA or Kruskal-Wallis test with post hoc comparison and Bonferroni correction.Results:Compared with controls, patients with rTOF had lower reservoir function parameters (EF, strain and strain rate), conduit EF, conduit strain, and left atrial maximum volume index ( P<0.05), but higher pump EF ( P<0.05). In patients with rPS, only the reservoir strain rate decreased compared with controls ( P<0.05), and the remaining data showed no significant difference ( P>0.05). The reservoir and conduit EF and strain in patients with rPS were higher than those in patients with rTOF ( P<0.05). Conclusions:In patients with rTOF and rPS, left atrial function has changed despite the preservation of left ventricular EF, which may be an early marker of left ventricular diastolic dysfunction. In children with rTOF, left atrial reservoir and conduit functions decreased while the pump function increased. The reservoir and pump functions in rPS were better than those in rTOF. In addition, CMRI can detect left atrial dysfunction early before it enlarged.

Long?Term Performance of Untreated Fresh Autologous Pericardium as a Valve Substitute in Pulmonary Position

Background:Pulmonary regurgitation is imminent after transannular patch (TAP). We analyze the long?term performance of untreated autologous pericardium (UAP) as valve substitute at pulmonary position in patients requiring TAP. Material and Methods: This cross?sectional study include patients operated between 2007 and 2012 (n = 92). A sample of 19 patients was selected for this study which had a follow?up of more than 3 years. This includes patients with no TAP (n = 4) and with TAP and valve substitute, a monocusp (n = 11) or a tricuspid valve (n = 4) at neopulmonary annulus. Patients underwent echocardiography for assessment of right ventricle function and 18 fluoro?deoxyglucose PET CT scan for measurements of valve substitute at neopulmonary annulus. The target to blood ratio (TBR) of uptake of glucose by monocusp was measured at the cooptation edge of the neopulmonary valve. Results: The median age of the patients is 14 (9 – 37). RV function is preserved (TAPSE 18.9 (10.6 – 22.8)) at a mean follow?up of 4 years (3?9). The measurements of monocusp shows a shrinkage in height of the cusp by 35.5% (70% – 1.0%) and length by 7% (?44% ? +104%). There was less shrinkage observed in patients below 15 years of age. The TBR of monocusp was 0.945 (0.17 – 3.35) with a strong correlation between the TBR values of aortic valve leaflet and monocusp leaflet of same patient. Conclusion: The UAP is functional and successful as a valve substitute at neo pulmonary annulus at long?term follow?up. It has resisted calcification and has shown uptake of glucose in physiological limits.

Síndrome de Hedinger / Hedinger syndrome

Resumen La afectación cardíaca por tumor carcinoide (síndrome de Hedinger) sigue siendo una causa importante de morbilidad y mortalidad entre los pacientes con síndrome carcinoide y tumores neuroendocrinos metastásicos. El estudio de imagen inicial ante la sospecha clínica es el ecocardiograma transtorácico, el cual es importante para la detección temprana de la afectación cardíaca. La cirugía cardíaca, en casos apropiados, es la única terapia definitiva para el tumor carcinoide con afectación cardíaca y mejora los síntomas del paciente y la supervivencia. El tratamiento es complejo y multidisciplinario; el estado cardíaco, el síndrome hormonal y la carga tumoral son fundamentales para guiar el momento óptimo de la cirugía. Se presenta el caso de una paciente joven, con síndrome de Hedinger con afectación valvular tricúspide y pulmonar, quien recibió tratamiento quirúrgico y tuvo evolución exitosa.

Abstract Cardiac carcinoid tumor involvement (Hedinger's syndrome) remains a major cause of morbidity and mortality among patients with carcinoid syndrome and metastatic neuroendocrine tumors. The initial imaging study in case of clinical suspicion is the transthoracic echocardiogram, which is important for the early detection of cardiac involvement. Cardiac surgery, in appropriate cases, is the only definitive therapy for carcinoid tumor with cardiac involvement and improves patient symptoms and survival. Treatment is complex and multidisciplinary; cardiac status, hormonal syndrome, and tumor burden are essential to guide the optimal timing of surgery. The case of a young female patient with Hedinger's syndrome with tricuspid and pulmonary valve involvement, who received surgical treatment and had a successful evolution.

Early outcomes of emergency operations in neonates with pulmonary atresia and intact ventricle septum / 中国小儿急救医学

Objective:To study the early outcomes of emergency operation employed with one of the prevalent surgical methods in neonates diagnosed as pulmonary atresia with intact ventricle septum(PA/IVS) and critical pulmonary stenosis(CPS), and to analyze the risk factors related to its early results.Methods:A retrospective analysis was conducted to collect the data of neonates suffered from PA/IVS and CPS from January 2016 to January 2020 in cardiothoracic surgery department at Shanghai Children′s Medical Center.According to their Z score, which reflects the development degree of right ventricle, the neonates received one of the relevant popular operations.Early outcomes were summarized and risk factors related to its early stage mortality were analyzed.Results:A total of 65 neonates were enrolled, including 27 CPS cases.They were operated on the basis of their respective Z scores, and also according to these data, the primary surgical procedure was chosen from the following methods: Blalock-Taussig(B-T) shunt, pulmonary valvulotomy and right ventricular outlet enlargement.Totally seven cases died, and mortality was 10.7%.Nine cases received re-operation in several following days because of severe hypoxemia and low cardiac output.All patients had mild improvement in oxygen saturation after operation and relied on large dose of inotropic agent.Multivariate Logistic regression analysis showed that B-T shunt and re-operation in early period were risk factors for death in neonates with PA/IVS and CPS.Conclusion:There is a higher mortality in neonates who received emergency operation for PA/IVS and CPS.Accurate assessment of the right ventricle development degree and selecting the corresponding appropriate surgical method is critical for the optimal result.B-T shunt and early stage re-operation are the risk factors for death in neonates with PA/IVS and CPS who received emergency operation.

Analysis of interventional treatment for neonatal critical pulmonary stenosis / 中华实用儿科临床杂志

Objective:To evaluate the effectiveness of interventional treatment for neonatal critical pulmonary stenosis(NCPS).Methods:Clinical data of 12 neonates with NCPS who received percutaneous balloon pulmonary valvuloplasty (PBPV) from January 2016 to December 2019 in Department of Cardiology, Shenzhen Children′s Hospital were summarized and analyzed.The collected data included transthoracic echocardiography (TTE), percutaneous oxygen saturation (SPO 2), relevant data on interventional surgery, and follow-up results. Results:All 12 neonates with NCPS received PBPV successfully.The postoperative pressure difference between the right ventricle and the pulmonary artery ranged from 8 to 35 mmHg[(20±7) mmHg, 1 mmHg=0.133 kPa]. The postoperative SPO 2 ranged from 74%-100%[(93.0±5.9)%]. Three neonates with NCPS received Blalock-Taussig (B-T) shunt.One neonate with NCPS developed supraventricular tachycardia during the operation.There was no death for these 12 neonates with NCPS. Conclusions:Interventional treatment of neonates with NCPS could achieve a better effect and be employed as the first treatment option.Some neonates with NCPS would require cardiac B-T shunt or patent ductus arteriosus stent implantation.

Left main coronary artery compression by the right pulmonary artery in a patient with congenital pulmonic stenosis / Compresión del tronco coronario izquierdo por la arteria pulmonar derecha en un paciente con estenosis pulmonar congénita

Abstract Congenital pulmonary stenosis (PS) can be associated with pulmonary artery (PA) dilatation. In some cases, this can cause compression of nearby structures including the left main coronary artery (LMCA). This compression causes angina and is considered an indication for surgical treatment. We present the case of a patient with PS and angina secondary to LMCA compression by the right PA and review the main indications and options for surgical treatment.

Resumen La estenosis pulmonar congénita se asocia a dilatación de la arteria pulmonar. En algunos casos esto puede causar compresión de las estructuras adyacentes incluyendo el tronco de la coronaria izquierda. Esta compresión causa angina y es considerada una indicación para tratamiento quirúrgico. Presentamos el caso de un paciente con estenosis pulmonar y angina secundaria a compresión del tronco de la coronaria izquierda por la arteria pulmonar derecha y revisamos las indicaciones y opciones de tratamiento quirúrgico.

Percutaneous balloon valvuloplasty of pulmonary valve stenosis: state of the art and future prospects

This study is aimed to delineate readers with an overview of percutaneous balloon pulmonary valvuloplasty (PBPV) of pulmonary valve stenosis (PVS) and highlighting outcome based on influential and recent studies. It has been four decades since Kan et al first introduce PBPV. Since then, PBPV has recognized as a gold standard therapy for PVS of all ages. Nowadays, PBPV is practiced for a broad range of indication such as PVS, PV dysplasia and pulmonary atresia. Typically, PBPV is recommended when gradient across the PV is >50 mmHg. The procedure involves the placement of one or more balloon catheters across the stenotic PV with the guidance of a guidewire; thereafter, inflation of the balloons is done by pressure, thus producing valvotomy. Nowadays, PBPV is done by echocardiographic guidance, but previously, it was done by fluoroscopic guidance. The main disadvantage of fluoroscopy was the radiation injury of patients. The recently recommended balloon/annulus ratio is 1.2 to 1.25. Following the procedure, the dramatic reduction of pressure gradient, free motion of the PV leaflets with less doming, the rise of cardiac output have been noted, whereas complications may occur but are unusual and minimal. Significant predictors of restenosis include balloon/annulus ratio <1.2 and immediate post-PBPV gradient ≥30 mmHg. Only a few percentages of patients needed repeat PBPV. Long-term follow-up results are surprisingly excellent. In conclusion, it is our opinion that PBPV is equally successful in patients of all ages, while worldwide recognized studies prove the safety, feasibility, and effectiveness. However, for early detection of any complication, life-long clinical follow-up is mandatory.

Outcome and follow _up analysis of transcatheter or surgical therapy for neonates with pulmonary atresia with intact ventricular septum and critical pulmonary stenosis / 中华实用儿科临床杂志

Objective To evaluate the safety and effectiveness of transcatheter or surgical procedure for neo_nates diagnosed with pulmonary atresia with intact ventricular septum(PA/IVS)or critical pulmonary stenosis(CPS) by retrospectively analyzina the clinical data,thus to discuss the clinical strategy decision. Methods From November 2006 to August 2018,105 neonates(PA/IVS 51 cases,CPS 54 cases)went through transcatheter or surgical procedure in xuangdong Provincial Cardiovascular Institute(transcatheter procedure 43 cases,surgery 62 cases). All cases mani_fested mild to moderate right ventricular dysplasia. Transcatheter procedure included pulmonary radio frequency perfora_tion and percutaneous balloon pulmonary valvuloplasty( PBPV). Surgical procedure included reconstruction of right ventricular outflow tract(transannular patch),pulmonary valvotomy and closed transventricular pulmonary valvuloplas_ty,while Aortic _Pulmonary shunt was performed in certain cases. Average follow _up period was(40. 1 ± 36. 9) months. Hospitalization and follow_up data were analyzed to evaluate the safety and efficacy of transcatheter and surgi_cal procedure. Results Operative time,hospital stay,complication rate were lower in transcatheter procedure group than those of surgery group[(107. 8 ± 61. 6)min υs.(183. 3 ± 36. 8)min,(18. 6 ± 7. 9)d υs.(31. 1 ± 13. 4)d, 30. 2% υs. 80. 6%],and the differences were significant(all P=0. 000). In transcatheter procedure group,2 cases (4. 7%)died and biventricular circulation is obtained in all survival cases. In surgery group,5 cases(8. 1%)died. Of the survival cases from surgery group,39 cases(69. 6%)obtained biventricular circulation,3 cases(5. 4%)obtained one and a half ventricular circulation while other 14 cases( 25. 0%) were candidates for biventricular repair. Conclusions Biventricular circulation can be achieved in most PA/IVS or CPS neonates with mild_moderate right ventricular dysplasia. PBPV turns out to be a safe and effective therapy for neonates with CPS while perforation of right ventricular outflow tract remains a nonnegligible complication in transcatheter therapy for PA/IVS.

Outcome and follow-up analysis of transcatheter or surgical therapy for neonates with pulmonary atresia with intact ventricular septum and critical pulmonary stenosis / 中华实用儿科临床杂志

Objective@#To evaluate the safety and effectiveness of transcatheter or surgical procedure for neonates diagnosed with pulmonary atresia with intact ventricular septum (PA/IVS) or critical pulmonary stenosis (CPS) by retrospectively analyzina the clinical data, thus to discuss the clinical strategy decision.@*Methods@#From November 2006 to August 2018, 105 neonates (PA/IVS 51 cases, CPS 54 cases) went through transcatheter or surgical procedure in Guangdong Provincial Cardiovascular Institute (transcatheter procedure 43 cases, surgery 62 cases). All cases manifested mild to moderate right ventricular dysplasia.Transcatheter procedure included pulmonary radio frequency perforation and percutaneous balloon pulmonary valvuloplasty (PBPV). Surgical procedure included reconstruction of right ventricular outflow tract (transannular patch), pulmonary valvotomy and closed transventricular pulmonary valvuloplasty, while Aortic-Pulmonary shunt was performed in certain cases.Average follow-up period was (40.1±36.9) months.Hospitalization and follow-up data were analyzed to evaluate the safety and efficacy of transcatheter and surgical procedure.@*Results@#Operative time, hospital stay, complication rate were lower in transcatheter procedure group than those of surgery group[(107.8±61.6) min vs.(183.3±36.8) min, (18.6±7.9) d vs.(31.1±13.4) d, 30.2% vs.80.6%], and the differences were significant(all P=0.000). In transcatheter procedure group, 2 cases (4.7%) died and biventricular circulation is obtained in all survival cases.In surgery group, 5 cases (8.1%) died.Of the survival cases from surgery group, 39 cases (69.6%) obtained biventricular circulation, 3 cases (5.4%) obtained one and a half ventricular circulation while other 14 cases (25.0%) were candidates for biventricular repair.@*Conclusions@#Biventricular circulation can be achieved in most PA/IVS or CPS neonates with mild-moderate right ventricular dysplasia.PBPV turns out to be a safe and effective therapy for neonates with CPS while perforation of right ventricular outflow tract remains a nonnegligible complication in transcatheter therapy for PA/IVS.

Transcatheter interventional therapy for pulmonary stenosis in infants / 中国实用儿科杂志

OBJECTIVE: To summarize the experience of percutaneous balloon pulmonary valvuloplasty for pulmonary stenosis in infants,and to evaluate its necessity,safety and effectiveness. METHODS: From January 2009 to December2018,68 cases of pulmonary stenosis were treated by percutaneous balloon pulmonary valvuloplasty. The clinical data before and after operation were analyzed retrospectively. RESULTS: The mean age was(6.34±3.6)months. The mean operation weight was(7.81±2.11)kg. The preoperative body weight/age Z score was-0.80±1.38,and growth restriction was in 22 cases(32.4%),cyanosis in 15 cases(22.1%)and tachypnea in 23 cases(33.8%). The mean right ventricular/left ventricular length-diameter ratio was 0.56±0.73 by echocardiography,the mean cardiothoracic ratio was 0.56±0.43 by chest radiography,and the average value of preoperative oxygen saturation was 94.74 ± 6.10. The mean systolic pressure ratio of right ventricle/left ventricle before operation was 0.68±0.26. After balloon dilation,the mean pressure gradient of pulmonary valve decreased from(75.57 ± 18.61)mmHg to(22.37 ± 5.21)mmHg,and the mean systolic pressure of right ventricle decreased from(83.78±22.44)mmHg to(54.16±20.81)mmHg. The immediate success rate of operation was 100%. There were no serious arrhythmia,cardiac perforation,rupture of tendon chordae,vascular injury or other complications during the operation. The proportion of mild to moderate pulmonary regurgitation was 53.3% in early stage after operation,and there was no severe regurgitation. The restenosis rate was 2.94% during the follow-up period of 3 months to 10 years. The pulmonary valve regurgitation degree of 32 children who were followed up for more than 5 years did not further aggravate,and no specific management was carried out. No obvious right ventricular dysfunction was found in all patients. Compared with the toddlerhood infants undergoing PBPV in our center,the babyhood infants had obvious clinical symptoms and serious condition. The early and medium-long-term follow-up after operation was satisfactory. There was no significant difference in the effect of operation in the low-weight or low-age infants in babyhood. CONCLUSION: PBPV is necessary for the treatment of pulmonary stenosis in infants. The technique is safe and feasible,and the follow-up results are effective.

Blue Rubber Bleb Nevus Syndrome with Musculo-skeletal Involvement and Pulmonary Stenosis.

Background: Blue rubber bleb nevus syndrome is a rare clinical entity. Case characteristics: A 13-year-old Indian boy presented with characteristic cutaneous lesions, gastrointestinal malformations, skeletal involvement and pulmonary stenosis. Observation: Diagnosis was confirmed on skin biopsy, radiographic evaluation, colonoscopy and echocardiography. Echocardiography revealed pulmonary stenosis, an association hitherto undescribed. Message: Detailed evaluation in a patient of blue rubber bleb nerves syndrome is mandatory.

The impact of prenatal diagnosis on treatment and prognosis of neonatal pulmonary atresia with intact ventricular ;septum and critical pulmonary stenosis with intact ventricular septum / 临床儿科杂志

Objective To explore the impact of prenatal diagnosis on the early treatment and short and medium term outcome of neonatal pulmonary atresia with intact ventricle septum (PA/IVS) or critical pulmonary stenosis with intact ventricle septum (CPS/IVS). Methods According to the case-control method, twenty-eight neonates with (PA/IVS) or (CPS/IVS) who had percutaneous pulmonary balloon valvuloplasty (PBPV) surgery indications, were divided into the prenatal diagnosis group (n?=?15) and the postnatal diagnosis group (n?=?13). The prenatal diagnosis group was diagnosed in fetal period and the intervention program was since developed . The postnatal diagnosis group was referred from other hospitals, and the intervention program was developed after conifrmation of the diagnosis. All the neonates accepted the PBPV surgery after the hemodynamic parameters stable. All neonates were followed-up at 1 month, 3 months, 6 months, 1 year, and 2 years after surgery. Clinical situations, echocardiography results, and interventional cardiology measurements were compared between two groups. Result The average age and weigh was 7.53?±?3.18 days and 3102.32?±?708.40 g respectively at the time of PBPV surgery in 28 neonates. Among them, 9 neonates were PA/IVS and 19 neonates were CPS/IVS. The mean follow-up time was 18.8?±?5.22 months and there were no death. The ages at admission and at the ifrst treatment were signiifcantly younger in the prenatal diagnosis group than those in the postnatal diagnosis group (P??0.05). Conclusion Prenatal diagnosis is helpful for the early intervention in neonates with PA/IVS and CPS/IVS, and can reduce the complications after surgery.

Cirugía de Nikaidoh modificada: experiencia inicial y resultados a corto y a mediano plazos / The Modified Nikaidoh Procedure: Initial Experience and Short and Mid-term Outcomes

Introducción: El tratamiento convencional de los pacientes con transposición de los grandes vasos con comunicación interventricular y estenosis pulmonar es la cirugía de Rastelli. Sin embargo, sus resultados son subóptimos, razón por la cual se han propuesto otras alternativas quirúrgicas. El procedimiento de Nikaidoh es una técnica reciente que surge como una opción terapéutica atractiva. Objetivo: Analizar nuestra experiencia inicial con la cirugía de Nikaidoh modificada: translocación aórtica y reconstrucción de ambos tractos de salida ventriculares. Material y métodos: Se analizaron 11 pacientes consecutivos operados con técnica de Nikaidoh modificada en nuestra institución en el período 2005-2014, con edad mediana de 3 años (intervalo intercuartil 25-75%: 1-5,5 años) y peso medio de 13,8 ± 5,4 kg, 9 de ellos con transposición de los grandes vasos con comunicación interventricular y estenosis pulmonar y 2 con doble salida del ventrículo derecho con estenosis pulmonar y fisiología de transposición. Todos tenían comunicación interventricular no relacionada con la aorta y un paciente tenía además ventrículo derecho hipoplásico. Resultados: En el posquirúrgico inmediato no hubo mortalidad, un paciente desarrolló endocarditis infecciosa y requirió reoperación. Seis pacientes tuvieron disfunción ventricular transitoria; solo uno necesitó asistencia ventricular. No se registró obstrucción a los tractos de salida ventriculares ni insuficiencia aórtica mayor que leve. En el seguimiento a mediano plazo, con una media de seguimiento de 5,5 ± 2,1 años (rango: 2-8,6 años), no hubo mortalidad ni reoperaciones. Se efectuó un cateterismo intervencionista para dilatación del conducto ventrículo derecho-arteria pulmonar. Todos los pacientes permanecían en clase funcional I, con buena función biventricular, sin arritmias, sin obstrucción al tracto de salida ventricular izquierdo y sin insuficiencia aórtica significativa. Conclusiones: La cirugía de Nikaidoh y sus modificaciones constituyen una buena opción quirúrgica para los pacientes con transposición de los grandes vasos con comunicación interventricular y estenosis pulmonar, que por su morfología son malos candidatos para cirugía de Rastelli. En esta serie no hubo mortalidad y todos los pacientes se encuentran en clase funcional I. En el seguimiento a mediano plazo, el tracto de salida del ventrículo izquierdo permaneció sin obstrucción y no se registró insuficiencia aórtica significativa.

Background: The Rastelli operation is the conventional procedure for transposition of the great vessels with ventricular septal defect and pulmonary stenosis. Its results, however, are suboptimal and other surgical alternatives have been postulated. A recent surgical technique, the Nikaidoh procedure, appears as a new therapeutic option. Objective: The aim of this study was to review our initial experience using the modified Nikaidoh procedure consisting in aortic translocation and biventricular outflow tract reconstruction. Methods: The study analyzed 11 consecutive patients operated on with the modified Nikaidoh procedure at our institution between 2005 and 2014. Median age was 3 years (25-75% interquartile range: 1-5.5 years) and mean weight was 13.8 ± 5.4 kg. Nine patients had transposition of the great vessels with ventricular septal defect and pulmonary stenosis and 2 had double outlet right ventricle with pulmonary stenosis and transposition physiology. All patients had a ventricular septal defect unrelated to the aorta and one patient also had a hypoplastic right ventricle. Results: There were no deaths in the immediate postoperative period. One patient developed infective endocarditis needing reoperation. Six patients had some degree of transient left ventricular dysfunction, and one patient only needed ventricular assistance. There was no involved right or left ventricular outflow tract obstruction and only mild aortic regurgitation. At mid-term follow-up (mean of 5.5 ± 2.1 years, range: 2-8.6 years) there were no deaths or reoperations. An interventional cardiac catheterization to dilate the right ventricle to pulmonary artery conduit was performed in one patient. All patients remained in functional class I, with good biventricular function, free from arrhythmias, no left ventricular outflow tract obstruction and no significant aortic regurgitation. Conclusions: The Nikaidoh operation and its variants are a satisfactory surgical option for patients presenting transposition of the great vessels with ventricular septal defect and pulmonary stenosis, who due to their morphology are inadequate candidates for a Rastelli procedure. In the mid-term follow-up, the left ventricular outflow tract remained without obstruction and there was no significant aortic valve regurgitation.

Clinical analysis of percutaneous balloon pulmonary valvuloplasty in the treatment of 52 patients with pulmonary stenosis / 中国介入心脏病学杂志

Objective To investigate the safety and effectiveness of percutaneous balloon pulmonary valvuloplasty (PBPV) in the treatment of pulmonary stenosis (PS). Methods The subjects were 52 patients with PS from 2007 to 2012, including 24 male and 28 female. Their age ranged from 1 year old to 56 year old and mean age was 9.2±7.6 years old. Their body weight ranged from 10 kg to 60 kg and mean weight was 17.3±8.9 kg. All patients were assessed with electrocardiogram (ECG), chest X-ray, right heart catheterization and right ventricular angiagraphy before the procedure. Results All 52 patients received PBPV successfully. Comparing the transvalvular pressure gradient before and after the operation, it decreased from 86.3±27.6 mmHg (1 mmHg=0.133 kPa) to13.8±7.7 mmHg, and statistically difference (P<0.01). Among these patients, one patient occurred ventricular fibrillation and Aspen syndrome (hypoxie-ischemic encephalopathy), one patient occurred respiratory and cardiac arrest, 24 patients occurred transient arrhythmia, one patient had hypotension, one patient had bradycardia and one patient showed desaturation. All these symptoms resolved after appropriate treatment during the PBPV. Two patients had hematoma in the puncture point and 2 patients had reactive infundibular stenosis after PBPV. Conclusions Performing the percutaneous balloon pulmonary valvoplasty on the patients with pulmonary stenosis is safe and effective. PBPV causes less pain and can be used as the preferred treatment for pulmonary stenosis.

Aneurisma de la arteria pulmonar asociado a estenosis valvular pulmonar: reporte de un caso de cardiopatía infrecuente en el adulto / Pulmonary Artery Aneurysms Associated to Pulmonary Valve: report of a rare case of heart disease in the adult

Resumen: Arch Med Interna 2013 - 35(1):19-22 La estenosis de la válvula pulmonar es una patología infrecuente constituyendo entre 8 y 10% de las cardiopatías congénitas. Se clasifica según sus características anatómicas en: típica, con displasia leve/moderada y con displasia severa. Su severidad se mide según el gradiente de presión siendo: leve < 30 mmHg, moderada entre 30 y 80 mmHg y severa > 80 mmHg. La valvuloplastia pulmonar percutánea es el procedimiento de elección en todas las edades y en todos las variantes anatómicas. Está indicada en casos de estenosis moderada a severa o en presencia de sintomatología, siendo menos efectiva en casos con válvulas displásicas. Es una técnica segura y efectiva con baja incidencia de mortalidad y complicaciones. En caso de ser ineficaz está indicada la cirugía. Los aneurismas de la arteria pulmonar presentan una baja prevalencia, estando descritos únicamente casos clínicos aislados y pequeñas series de casos lo que no permite estimar su prevalencia. Generalmente se presentan en pacientes asintomáticos o con síntomas inespecíficos y el diagnóstico se realiza como hallazgo en estudios de imagen. Pueden ser congénitos o adquiridos, asociando los congénitos otras malformaciones cardiovasculares. Los adquiridos son secundarios a: hipertensión pulmonar ya sea primaria o secundaria; estenosis valvular pulmonar; vasculitis; infecciones; neoplasias o iatrogénicas. La complicación más temida es la rotura del aneurisma con una elevada mortalidad. El tratamiento médico es limitado, atendiendo al control de la hipertensión pulmonar y a la etiología causal en el caso de los secundarios. El tratamiento quirúrgico queda reservado para los aneurismas inestables, con hemoptisis, o aneurismas mayores a 60 mm.

Abstract: Arch Med Interna 2013 - 35(1):19-22 The Pulmonary Artery Aneurysm is a rare condition that accounts for 8 to 10% of all congenital heart diseases. It is classified according to its anatomy as typical, with mild/moderate dysplasia and with severe dysplasia. Severity is defined based on the pressure gradient, being: mild < 30 mmHg, moderate between 30 and 80 mmHg and severe > 80 mmHg. The percutaneous pulmonary valve repair is a safe and effective technique that entails a low incidence of complications and a low mortality rate. It is the procedure of choice at all ages, regardless of the anatomical type. It is indicated in cases of moderate to severe stenosis or in the presence of symptoms, being less effective in cases that occur with dysplastic valves. Surgery is indicated when the percutaneous route has failed. Pulmonary artery aneurysms have a low prevalence; being there only isolated reports and small case series, their prevalence is difficult to estimate. They usually occur in patients that are either asymptomatic or presenting with non specific symptoms; diagnosis typically results from incidental imaging findings. These aneurysms may be congenital or acquired, being the former associated to additional cardiovascular malformations. Acquired PA aneurysms may be secondary to pulmonary hypertension, which may in turn be primary or secondary to episodes of pulmonary thromboembolism, pulmonary valve stenosis, vasculitis, infections, malignancies or resulting from iatrogenia. The most feared complication of the aneurysm is its rupture, a highly lethal event. Medical therapy is limited, and requires keeping pulmonary hypertension under control and acting upon the root cause in the secondary cases. Surgical therapy is reserved only to unstable aneurysms, patients with haemoptysis, or aneurysms over 60.

Síndrome de Williams-Beuren: A propósito de un caso

Objetivo: El objetivo es dar a conocer un caso de Síndrome de Williams-Beuren (SWB). Caso Clínico: Lactante mayor, masculino de 1 año de edad, quien es remitido a la Unidad de Endocrinología del Instituto Autónomo Hospital Universitario de los Andes por presentar hipotonía y retraso en el desarrollo psicomotor. El examen físico reveló peso de 8,300 Kg (P10), talla de 75 cm (P50), potencial genético de talla de 176±10 cm. Normocéfalo, con dismorfia facial caracterizada por frente amplia, nariz corta y ancha, leve oblicuidad palpebral y epicántica, iris marrón estrellado, filtrum largo, mandíbula pequeña, labios prominentes y probable microdoncia. Además, se auscultó soplo sistólico eyectivo en foco pulmonar y el examen neurológico evidenció hipotonía troncal, leve y armónica, así como déficit de atención. El ultrasonido de tiroides reportó leve hipoplasia de la glándula, y el perfil tiroideo fue compatible con un hipotiroidismo subclínico. El estudio hemodinámico demostró la presencia de estenosis múltiple de arterias pulmonares y de ramas periféricas. Conclusiones: El paciente fue diagnosticado con SWB debido a la coexistencia de estenosis múltiple de arterias pulmonares y de ramas periféricas así como a las características faciales propias de este trastorno. Se estima que la frecuencia de este síndrome es de 1 por cada 7.500 nacidos vivos, pudiendo ser de aparición esporádica y ocurre por deleción en 7q11.23. El hipotiroidismo subclínico constituye una de las alteraciones endocrinológicas más frecuentemente encontradas en estos pacientes y se asocia a hipoplasia de la glándula tiroides.

Objective: To divulge a case of Williams-Beuren Syndrome (WBS). Clinical case: Toddler, male, 1-year old, referred to the Endrocrinology Unit of the Mérida, Venezuela Los Andes University Hospital for presenting hypotonia and psychomotor retardation. At clinical examination he exhibited:weight: 8.3 kg (P10); height: 75 cm (P50); size genetic potential: 176±10 cm; normocephalic, with facial dysmorphia characterized by broad forehead, short and wide nasal bridge, slight palpebral and epicanthic obliquity, brown starry iris, long philtrum, small jaw, bulging lips and probable microdontism. Systolic ejection murmur in pulmonic root. Neurological examination evinced slight and harmonic truncal hypotonia and attention deficit. Ultrasound reported mild thyroid hypoplasia, thyroid profile being compatible with subclinical hypothyroidism. Hemodynamic monitoring confirmed multiple stenosis of pulmonary arteries and peripheral branches. Conclusion: Patient was diagnosed with WBS because of main branch and peripheral pulmonary artery stenosis concurrent with facial demeanor typical of this disorder. Its occurrence, likely sporadic and due to 7q11.23 deletion, attends one in 7500 live births. Subclinical hypothyroidism, associated to thyroid hypoplasia, is one of the most frequent endocrinological alterations found in these patients.

Efficacy and safety via Meta analysis of percutaneous balloon pulmonic valvuloplasty and surgery in pulmonary stenosis / 中华实用儿科临床杂志

Objective To systematically evaluate the effect of percutaneous balloon pulmonic valvuloplasty (PBPV) and the surgery in pulmonary stenosis(PS),so that suitable treatment would be selected partly based on this study.Methods PubMed,OVID,Medline,Chinese Biomedicine database,VIP Database for Chinese Technical Periodicals,digital journal of Wanfang Data and China National Knowledge Internet were searched to locate relevant controlled trials.Data extraction and assessment of methodologic quality were performed independently by 2 reviewers.Meta analysis was performed by Revman 5.0 software.Results Five papers(including 547 patients) were identified.The age,weight and preoperative pressure gradient were similar in 2 groups.The success rate in PBPV group was similar to surgical group (96.2％ vs 100％,P =0.46).The survival rate in 2 groups were all 100％.The pulmonic regurgitation rate in PBPV group was lower than that in surgical group(34.9％ vs 70.5％,P =0.001).The postoperative pressure gradient in PBPV group were higher than that in surgical group(P =0.003).Complication rate in PBPV group was lower than that surgical group (6.83％ vs 26.50％,P ＜ 0.000 01).Conclusions The PBPV is safe and effective,but it is selective and the success rate needs to be increase.If the patient with pulmonary stenosis is suitable,PBPV will be the first choice.

Hybrid treatment of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis in neonates / 中华实用儿科临床杂志

Objective To investigate the outcomes of hybrid treatment of pulmonary valve's balloon valvuloplasty via right ventricle without cardiopulmonary bypass for pulmonary atresia with intact ventricular septum(PA/IVS) and critical pulmonary stenosis(PS) in neonates.Methods The clinical features and data of 3 children(1 case of PA/ IVS and 2 cases of critical PS),who underwent hybrid treatment of balloon valvuloplasty,were recorded and retrospectively analyzed.All patients' right ventricle grow well,tricuspid valve Z score ＞-2,and underwent pulmonary valve balloon valvuloplasty via fight ventricle without cardiopulmonary bypass.Results All the 3 children underwent pulmonary valve balloon valvuloplasty via fight ventricle without cardiopulmonary bypass,and there was no death.The mean arterial blood oxygen saturation after operation went up,and there was no complication related to balloon valvuloplasty.The children were followed up for 3 to 6 months,and all the patients had mild pulmonary stenosis,but they did not need any treatment.Conclusions Pulmonary valvuloplasty with hybrid technique is effective in treatment of selective PA/IVS children(fused leaflets of pulmonary valve) and critical PS in neonates.Hybrid therapy may provide adequate pulmonary blood flow and promote further growth of tricuspid valve and right ventricular cavity with less trauma and more safety.

Prophylaxis and treatment for the complications of the percutaneous balloon pulmonary valvuloplasty in children with pulmonary valve stenosis / 国际儿科学杂志

Percutaneous balloon pulmonary valvuloplasty has replaced surgery as the first choice for pulmonary valve stenosis,especially for the isolated ones in children.However,there still happen to be some complications which could lead to a bad prognosis or even death,when the procedure has already been a recognized safe one.This paper reviews these complications with their causal factors as well as the ways to prevent and treat them.