Evaluation and Management of Chronic Heart Failure in Children and Adolescents With Congenital Heart Disease: A Scientific Statement From the American Heart Association.

With continued medical and surgical advancements, most children and adolescents with congenital heart disease are expected to survive to adulthood. Chronic heart failure is increasingly being recognized as a major contributor to ongoing morbidity and mortality in this population as it ages, and treatment strategies to prevent and treat heart failure in the pediatric population are needed. In addition to primary myocardial dysfunction, anatomical and pathophysiological abnormalities specific to various congenital heart disease lesions contribute to the development of heart failure and affect potential strategies commonly used to treat adult patients with heart failure. This scientific statement highlights the significant knowledge gaps in understanding the epidemiology, pathophysiology, staging, and outcomes of chronic heart failure in children and adolescents with congenital heart disease not amenable to catheter-based or surgical interventions. Efforts to harmonize the definitions, staging, follow-up, and approach to heart failure in children with congenital heart disease are critical to enable the conduct of rigorous scientific studies to advance our understanding of the actual burden of heart failure in this population and to allow the development of evidence-based heart failure therapies that can improve outcomes for this high-risk cohort.

Impact of donor-recipient age-difference in adolescent heart transplantation.

INTRODUCTION: The relationship between donor age and adolescent heart transplant outcomes remains incompletely understood. We aimed to explore the effect of donor-recipient age difference on survival after adolescent heart transplantation. METHODS: The United Network for Organ Sharing database was used to identify 2,855 adolescents aged 10-17 years undergoing isolated primary heart transplantation from 1/1/2000 to 12/31/2022. The primary outcome was 10-year post-transplant survival. Multivariable Cox regression identified predictors of mortality after adjusting for donor and recipient characteristics. A restricted cubic spline assessed the non-linear association between donor-recipient age-difference and the adjusted relative mortality hazard. RESULTS: The median donor-recipient age-difference was +3 (range -13 to +47) years, and 17.7% (n = 504) of recipients had an age- difference > 10 years. Recipients with an age-difference > 10 years had a less favorable pre-transplant profile, including a higher incidence of priority status 1A (81.6%, n = 411 vs. 73.6%, n = 1730; p = .01). The 10-year survival rate was 54.6% (95% confidence interval (CI) 48.8- 60.4) among recipients with a donor-recipient age-difference > 10 years and 66.9% (95% CI: 64.4-69.4) among those with an age-difference ≤10 years. An age-difference > 10 years was an independent predictor of mortality (hazard ratio 1.43, 95% CI: 1.18-1.72, p < .001). Spline analysis demonstrated that the adjusted mortality hazard increased with increasingly positive donor-recipient age-difference and became significantly higher at an age-difference of 11 years. CONCLUSION: A donor-recipient age-difference > 11 years is independently associated with higher long-term mortality after adolescent heart transplantation. This finding may help inform acceptable donor selection practice for adolescent heart transplant candidates.

Long-Term outcomes in adult patients with congenital heart disease considered for transplantation: A single center study.

BACKGROUND: Adult congenital heart disease (ACHD) patients pose unique challenges in identifying the time for transplantation and factors influencing outcomes. OBJECTIVE: To identify hemodynamic, functional, and laboratory parameters that correlate with 1- and 10-year outcomes in ACHD patients considered for transplantation. METHODS: A retrospective chart review of long-term outcomes in adult patients with congenital heart disease (CHD) evaluated for heart or heart + additional organ transplant between 2004 and 2014 at our center was performed. A machine learning decision tree model was used to evaluate multiple clinical parameters correlating with 1- and 10-year survival. RESULTS: We identified 58 patients meeting criteria. D-transposition of the great arteries (D-TGA) with atrial switch operation (20.7%), tetralogy of Fallot/pulmonary atresia (15.5%), and tricuspid atresia (13.8%) were the most common diagnosis for transplant. Single ventricle patients were most likely to be listed for transplantation (39.8% of evaluated patients). Among a comprehensive list of clinical factors, invasive hemodynamic parameters (pulmonary capillary wedge pressure (PCWP), systemic vascular pressure (SVP), and end diastolic pressures (EDP) most correlated with 1- and 10-year outcomes. Transplanted patients with SVP < 14 and non- transplanted patients with PCWP < 15 had 100% survival 1-year post-transplantation. CONCLUSION: For the first time, our study identifies that hemodynamic parameters most strongly correlate with 1- and 10-year outcomes in ACHD patients considered for transplantation, using a data-driven machine learning model.

Repeat pediatric heart transplantation: A united network for organ sharing database analysis.

BACKGROUND: A history of congenital heart disease and previous transplantation are each independently associated with worse survival following pediatric heart transplantation. This study aimed to evaluate the characteristics and outcomes of children undergoing repeat heart transplantation in the United States based on the underlying diagnosis. METHODS: The United Network for Organ Sharing database was used to identify 8111 patients aged <18 years undergoing isolated heart transplantation from 2000 to 2021, including 435 (5.4%) repeat transplants. Restricted cubic spline analysis assessed the non-linear relationship between inter-transplant interval and the primary outcome of all-cause mortality or re-transplantation. Multivariable Cox regression assessed the impact of re-transplantation on the primary outcome. Median follow-up was 5.0 (interquartile range 1.9-9.9) years. RESULTS: Repeat transplant patients were older (median age 12 vs. 4 years; p < .001), and less likely to be in UNOS status 1A (66.0%, n = 287 vs. 81.0% n = 6217; p < .001) than primary transplant patients. Freedom from the primary outcome was 51.4% (95% confidence interval [CI] 45.5-57.2) among repeat transplants and 70.5% (95% CI 69.2-71.8) among primary transplants at 10 years (p < .001). Among repeat transplant patients, the relative hazard of the primary outcome became non-significant when the inter-transplant interval >3.6 years. Congenital heart disease was an independent predictor of mortality among primary (HR 1.8, 95% CI 1.6-1.9) but not repeat transplant (HR 1.1, 95% CI .8-1.6) patients. CONCLUSIONS: Long-term outcomes remain poor for patients undergoing repeat heart transplantation, particularly those with an inter-transplant interval <3.6 years. Underlying diagnosis does not impact outcomes after repeat transplantation, after accounting for other risk factors.

Horn-shaped" deformity of the Occlutech®-PDA device: case report / [Deformidad en "forma de claxon" del dispositivo Occlutech-PDA.]

Background: The phenomenon of deformation of devices for closure of intracardiac defects in catheterization has been reported, but not of devices for closure of patent ductus arteriosus. Objective: to report a case of deformation of a relatively new type of device for closure of patent ductus arteriosus. Methods: report an adult with hypertensive patent ductus arteriosus and a positive balloon occlusion test will be presented, in whom the occlusion was attempted with an Occlutech®-PDA device. Results: Upon delivery, the device took the shape of a "horn" instead of a "champagne cork" (usual appearance), so it was extracted prior to it is release and the procedure was carried out with another device. Conclusion: To our knowledge, no deformation during the delivery transcatheter of the Occlutech®-PDA device has been published. Adverse events related to new technologies, as in our case, should be reported as soon as possible, since it can help manufacturers to improve their quality and provide greater safety to patients.

Introducción: Existen comunicados sobre el fenómeno de la deformación de dispositivos oclusores de defectos intracardíacos durante el cateterismo, pero no acerca de los dispositivos oclusores para el conducto arterioso persistente. Objetivo: comunicar un caso de deformación de un relativamente nuevo tipo de dispositivo oclusor de conducto arterioso persistente. Método: se presenta un paciente adulto con conducto arterioso persistente hipertensivo y prueba de oclusión con balón positiva, en quien se intentó ocluirle con un dispositivo Occlutech®-PDA. Resultado: al momento de la entrega, el dispositivo adoptó la "forma de claxon" en lugar de "corcho de champagne" (apariencia habitual), motivo por el que fue retirado previo a su liberación y el procedimiento fue llevado a cabo con otro dispositivo. Conclusión: hasta donde conocemos, no hay comunicados acerca de deformación del dispositivo Occlutech®-PDA durante su implante mediante cateterismo. Los eventos adversos relacionados a nuevas tecnologías, como el presentado en este caso, deben ser comunicados tan pronto como sea posible, ya que esto puede ayudar a los fabricantes a mejorar su calidad y proveer mayor seguridad a los pacientes.

Impact of the new UNOS donor heart allocation system on waitlist outcomes and early posttransplant mortality among adults with congenital heart disease.

Adults with congenital heart disease (ACHD) experience worse waitlist outcomes and higher early posttransplant mortality compared to non-ACHD patients. On October 18, 2018; the UNOS donor heart allocation system was redesigned giving unique listing status to ACHD patients. The impact of this change on outcomes in transplant-listed patients is unstudied. Using the Scientific Registry of Transplant Recipients (SRTR) we compared ACHD patients listed for the first-time for heart transplantation from two eras of equal duration. We analyzed waitlist outcomes, posttransplant mortality and length of stay among ACHD patients in both eras and between ACHD and non-ACHD patients in the new era. Of 12 723 listed patients, 535 had ACHD (293 in the new era) and 12 188 did not (6258 in the new era). A total of 163 (56%) ACHD patients in the new era versus 150 (62%) in the prior era were transplanted; 11 (3.8%) versus 15 (6.2%) died on the waitlist; 32 (11%) versus 35 (14%) were delisted and 15 (9.2%) versus 19 (12.7%) died within 30 days of transplant, respectively. The new UNOS donor heart allocation system improved waitlist time and decreased the proportion not transplanted during the first 300 days after listing among ACHD patients without altering early posttransplant outcomes or significantly changing the gap in outcomes compared to non-ACHD patients.

Transplantation for pulmonary arterial hypertension with congenital heart disease: Impact on outcomes of the current therapeutic approach including a high-priority allocation program.

Patients with end-stage pulmonary arterial hypertension due to congenital heart disease have limited access to heart-lung transplantation or double-lung transplantation. We aimed to assess the effects of a high-priority allocation program established in France in 2007. We conducted a retrospective study to compare waitlist and posttransplantation outcomes before versus after implementation of the high-priority allocation program. We included 67 consecutive patients (mean age at listing, 33.2 ± 10.5 years) with pulmonary arterial hypertension due to congenital heart disease listed for heart-lung transplantation or double-lung transplantation from 1997 to 2016. At one month, the incidences of transplantation and death before transplantation were 3.5% and 24.6% in 1997-2006, 4.8% and 4.9% for patients on the regular list in 2007-2016, and 41.2% and 7.4% for patients listed under the high-priority allocation program (p < .001 and p = .0001, respectively). Overall survival was higher in patients listed in 2007-2016 (84.2% and 61.2% at 1 and 10 years vs. 36.8% and 22.1%, p = .0001). Increased incidence of transplantation, decreased waiting list mortality, and improved early and long-term outcomes were observed in patients with pulmonary arterial hypertension due to congenital heart disease listed for transplantation in the recent era, characterized by implementation of a high-priority allocation program.

Superior vena cava obstruction after the Glenn procedure.

Acute obstruction of superior vena cava anastomosis right after the Glenn procedure may lead to tragic consequences. We describe the case of a one-year-old child with tricuspid atresia and a previous Blalock-Taussig shunt procedure, who presented severe low cardiac output syndrome right after the Glenn procedure and died forty-four hours after the procedure. The autopsy showed obstruction of the superior vena cava anastomosis. Patients that present superior vena cava syndrome and low cardiac output right after the Glenn procedure should have the surgical anastomosis revised immediately.

Impact of the 2016 revision of US Pediatric Heart Allocation Policy on waitlist characteristics and outcomes.

US Pediatric Heart Allocation Policy was recently revised, deprioritizing candidates with cardiomyopathy while maintaining status 1A eligibility for congenital heart disease (CHD) candidates on "high-dose" inotropes. We compared waitlist characteristics and mortality around this change. Status 1A listings decreased (70% to 56%, P < .001) and CHD representation increased among status 1A listings (48% vs 64%, P < .001). Waitlist mortality overall (subdistribution hazard ratio [SHR] 0.96, P = .63) and among status 1A candidates (SHR 1.16, P = .14) were unchanged. CHD waitlist mortality trended better (SHR 0.82, P = .06) but was unchanged for CHD candidates listed status 1A (SHR 0.92, P = .47). Status 1A listing exceptions increased 2- to 3-fold among hypertrophic and restrictive cardiomyopathy candidates and 13.5-fold among dilated cardiomyopathy (DCM) candidates. Hypertrophic (SHR 6.25, P = .004) and restrictive (SHR 3.87, P = .03) cardiomyopathy candidates without status 1A exception had increased waitlist mortality, but those with DCM did not (SHR 1.26, P = .32). Ventricular assist device (VAD) use increased only among DCM candidates ≥1 years old (26% vs 38%, P < .001). Current allocation policy has increased CHD status 1A representation but has not improved their waitlist mortality. Excessive DCM status 1A listing exceptions and continued status 1A prioritization of children on stable VADs potentially diminish the intended benefits of policy revision.

Superior vena cava obstruction after the Glenn procedure

Acute obstruction of superior vena cava anastomosis right after the Glenn procedure may lead to tragic consequences. We describe the case of a one-year-old child with tricuspid atresia and a previous Blalock-Taussig shunt procedure, who presented severe low cardiac output syndrome right after the Glenn procedure and died forty-four hours after the procedure. The autopsy showed obstruction of the superior vena cava anastomosis. Patients that present superior vena cava syndrome and low cardiac output right after the Glenn procedure should have the surgical anastomosis revised immediately.

Tamizaje de cardiopatías congénitas en el neonato mediante oximetría de pulso en el Hospital Materno Infantil José Domingo de Obaldía. Agosto 2014- febrero 2015. / Screening of congenital heart disease in neonates through pulse oximetry. Hospital Materno Infantil José Domingo de Obaldia. August 2014 - February 2015

Introducción. Existen evidencias sólidas que demuestran la utilidad de realizar de manera sistemática una prueba de saturación de oxígeno a todos los recién nacidos aparentemente sanos después de 24 horas de nacido y antes de 48 horas; a esta prueba se le ha llamado "tamiz neonatal para cardiopatías congénitas" y sirve para detectar oportunamente anomalías cardiacas estructurales congénitas que cursan con hipoxemia. El tamiz de cardiopatías congénitas puede ayudar a reconocerlas tempranamente y posibilitar su mejor evolución. Objetivos. Evaluar el uso de la oximetría de pulso como herramienta de detección de las cardiopatías congénitas en los recién nacidos asintomáticos. Resultados. Se realizó la prueba de tamizaje cardíaco mediante oximetría de pulso a 2236 recién nacidos sanos asintomáticos, nacidos en el Hospital Materno Infantil José D. de Obaldía (HMIJDDO) en un período de seis meses (agosto 2014-febrero 2015). De esta muestra se obtuvo un tamiz positivo en 16 de los recién nacidos evaluados (0.72%). A los cuales se les realizó ecocardiograma que reportó: 6 casos (37.5%) con un corazón estructuralmente normal, 6 (37.5%) con Ductus Arteriosos Persistentes, 3 (18.75%) Conexiones Anómalas de Venas Pulmonares, y sólo en un caso (6.25%) se presentó un Síndrome de Ventrículo Derecho Hipoplásico, Hipoplasia de Válvula Tricúspide, Atresia pulmonar vs Estenosis Crítica de Válvula en Domo, Conducto Arterioso Grande y Tortuoso, Sospecha de Fístulas Ventrículo-Coronarias. Conclusión. Los padres y los médicos deben saber que una prueba de saturación normal, no descarta la presencia de otras cardiopatías congénitas que no cursan con hipoxemia. Una vez tengamos capacitado un personal que realice este tamizaje, podemos poner la oximetría como control para todos, pero no como un tamiz aislado, sino como un dato más para una fisiopatología que estemos pensando.

Introduction. There is solid evidence that demonstrate the utility of testing in a systematic way of oxygen saturation all the healthy newborn infants apparently after 24 hours old and within 48 hours; This proof has been called "screening neonatal for congenital heart disease" and serves to opportunely detect congenital structural cardiac abnormalities that present with hypoxemia. Sieve of congenital heart disease may help recognize them early and facilitate their better development. Objectives. Evaluate the use of pulse oximetry as a tool for detection of congenital heart diseases in the newborn asymptomatic. Results. The cardiac screening using pulse oximetry test was performed to healthy newborns 2236 asymptomatic, born in the Hospital Materno Infantil José Domingo de Obaldía (HMIJDDO) in a period of six months (August 2014-February 2015). This sample was obtained a positive screen in 16 of the newborns assessed (0.72%). Which was les echo reported: 6 (37.50%) with a structurally normal heart, 6 (37.50%) Arteriosus persistent ductus, 3 (18.75%) Anomalous pulmonary venous connections. And only in one case (6.25%) presented a right hypoplastic ventricle, tricuspid valve, pulmonary Atresia hypoplasia syndrome vs valve stenosis-criticism in dome, large patent ductus arteriosus and tortuous, suspicion of ventricle-coronary fistula. Conclusion. Parents and physicians should know that a normal saturation test, doesn´t rule out the presence of other congenital heart disease who dont present with hypoxemia. Once we have trained staff who perform this screening, we can put the oximetry as a control for all, not as an isolated sieve, but one fact more for a pathophysiology that we are thinking.

Adolescentes con cardiopatía congénita: conocimientos sobre su enfermedad, sexualidad y posibles complicaciones de un embarazo / Adolescents with congenital heart disease: knowledge about their disease, sexuality and possible complications during pregnancy

Introducción. Las jóvenes tienen su primera experiencia sexual en edades tempranas; si padecen una cardiopatía, el riesgo de morir se eleva cinco veces durante el parto. Objetivo. Describir los conocimientos que tienen las adolescentes sobre su cardiopatía congénita (CC), sexualidad, embarazo y las complicaciones que esto conlleva. Material y métodos. Estudio descriptivo, transversal y prospectivo realizado previo consentimiento informado de los padres. Se aplicó cédula ex profeso a 30 mujeres hospitalizadas de 12 a 18 años de edad con alguna CC. Cédula con información sociodemográfica y sobre conocimientos de su enfermedad, sexualidad y embarazo, con respuestas dicotómicas, opción múltiple y abiertas. Datos analizados con medidas de dispersión y tendencia central. Resultados. Edad promedio 14.6 años; 10% con vida sexual activa iniciada entre 13-17 años. Todas conocen su tratamiento, 90% sabe qué enfermedad padece. 77.3% tiene confianza con su madre para platicar de sexualidad; 60% considera que es por falta de información el embarazo; 73.4% conoce dos o más métodos anticonceptivos, principalmente condón, aunque 29.9% piensa erróneamente que todos protegen de enfermedades de transmisión sexual; 73.3% considera poderse embarazar aún con cardiopatía. Conclusiones. La mayoría de adolescentes está informada, pero es imperativo el desarrollo de estrategias educativas que disminuyan la problemática.

Introduction. Today, young people have their first sexual experience at early ages. In addition, if they suffer from heart disease, the risk of dying raises five times during childbirth. Objective. Describe the knowledge they have about their adolescent congenital heart disease (CHD), sexuality, pregnancy and the complications that entails. Material and methods. We performed a descriptive, transversal and prospective study prior informed consent of the parents. A charter expressly 30 woman 12 to 18 years of age with some kind of CC, hospitalized was applied. The schedule consisted of sociodemographic information and knowledge about his illness, sexuality and pregnancy with dichotomous response, multiple choice and open. We analyzed the data with measures of central tendency and dispersion. Results. Average age 14.6 years; 10% initiated sexual activity between 13 and 17 years. All know their treatment, 90% know what disease you have and what stage. 77.3% have confidence with her mother to talk about sexuality; 60% believe that teenage pregnancy is due to lack of information; 73.4% know two or more birth control. The best known is the condom, while 29.9% mistakenly think that some contraceptive methods protect against sexually transmitted diseases; and 73.3% consider being able to still pregnant with heart disease. Conclusions. Most teens know about their disease, contraception and sexuality; however, it is imperative to develop educational strategies to reduce the problem.

The clinic report of 566 consecutive cases of Fontan operation and analysis early risk factors / 中华胸心血管外科杂志

Objective To summarize 566 consecutive cases of Fontan operation in the treatments of complex congenital heart diseases and to analyze the risk factors of early surgical outcome.Methods We reviewed all 566 consecutive patients who underwent a Fontan procedure in Shanghai Children's Medical Center from January 2006 to May 2014.Preoperative records of staged surgeries,perioperative data and imaging results were obtained from medical records.Results A total of 566 patients(M/F =345/221) underwent a Fontan operation,with 166 cases of single stage Fontan operation and 400 cases of multi-staged operation.The median age at the time of the Fontan procedure was 5 years,and the median weight was 15.4 kg.In our series,there were 278 extracardiac conduits,110 intracardiac conduits,114 lateral tunnels,36 intracardiac/extracardiac conduits,and 27 direct cavopulmonary conduits.There were 36 cases of early Fontan failure,with overall early postoperative survival rate being 93.6％.The median ventilation duration was 8 h,with the median postoperative intensive care unit stay being four days,the average oxygen saturation being 0.90 at the end of ICU stay,the median chest drainage time being 10 days,and the median postoperative hospital stay being 19 days.Besides the CICU oxygen saturation,the rest postoperative results had no obvious differences in five surgical groups.Heterotaxy syndrome,earlier age at the time of the Fontan procedure,bilateral Glenn shunt,moderate AV valve regurgitation and severe and right ventricular-type morphology were risk factors of early Fontan failure,P ＜ 0.05.Conclusion In order to improve the early outcome of Fontan operation,closely follow-up should be taken directly after Glenn operation in high-risk patients.Doctors should also have an in-depth understanding of the of postFontan physiological and pathological features,and select the operation time and Fontan type most suited to the of the individualized characteristics.

Outcome assessment of different surgeries for neonates with pulmonary atresia and ventricular septal defect / 中国胸心血管外科临床杂志

@#Objective To explore the feasibility and option of different surgeries for neonates with pulmonary atresia and ventricular septal defect (PA/VSD) through assessing the effect of common surgeries. Methods Fourteen neonates who underwent their first surgery in our center from July 2004 to October 2014 were included. Their basic characteristics, operation and pre- and postoperative clinical information were extracted. Follow up was conducted and the last visit was on October 10, 2016. Short- and midterm survival and total correction rate were compared among different surgeries. Results Among the 14 patients, there were 4 (28.6%) patients, 6 (42.9%) and 4 (28.6%) who underwent one-stage repair, right ventricular outflow tract (RVOT) reconstruction, and systemic to PA shunt operation respectively. The overall in-hospital mortality after the first operation was 28.6% (4/14). At last visit, no death occurred resulting the 5-year survival rate of 71.4% (10/14). The overall total correction rate for all neonates was 64.3% (9/14). Although no statistical difference was found in the mortality among the one-stage repair , RVOT reconstruction and systemic to PA shunt group(50.0% vs. 33.3% vs. 0.0%, P=0.280), the survival and hazard analysis implied better outcomes of the systemic to PA shunt palliation operation. There was no statistical difference in the total correction rate and months from the first palliative operation to correction between those who underwent RVOT reconstruction and systemic to PA shunt (75.0% vs. 50.0%, P=0.470; 32.0 months vs. 18.0 months, P=0.400). Conclusion Performing surgeries for neonates with PA/VSD is still a great challenge. However, the midterm survival rate was optimistic for the early survivors. Systematic to PA shunt seemed to be a better choice with lower mortality for the neonates with PA/VSD who need the surgery to survive.

One-stage surgical repair of interrupted aortic arch in neonates and young infants:surgical experiences, follow-up re-sults and subsequent treatments / 中华胸心血管外科杂志

Objective To discuss one-stage surgical repair of interrupted aortic arch ( IAA) in neonates and young in-fants, summarize follow-up results and subsequent treatments.Methods From September 2010 to December 2014, a series of 38 consecutive neonates and young infants ( M/F =26/12 ) with IAA were admitted in our surgical group to receive surgical treatment, with a median age of 11 days(1 day to 5 months) and a median body weight of 4.1 kg(1.8 to 5.8 kg).IAA was type A in 24 cases, type B in 13 cases and type C in 1 case.Associated cadiovascular anomalies were common except 2 cases, including atrial septal defect(n=23), ventricular septal defect(n=36), left ventricular outlet tract obstruction(LVOTO)(n=7), anomalous left pulmonary artery from aorta(n=1), truncus arteriosus(n=1).There were 5 cases of airway stenosis in 28 cases of CT angiography and 3D airway reconstruction.All the 38 cases were admitted with differential cyanosis.36 cases were recieved one-stage surgical repair except 2 cases died before operation.35 cases of IAA associated with intracardiac anom-alies were repaired through a median sernotomy.Cardiopulmonary bypass(CPB) was performed with two canulations in ascend-ing aorta and main pulmonary artery.Selective cerebral perfusion(n=21) or circulatory arrest(n =14) was carried out with body temperature of 18℃-23℃.After the patent ductus arteriosus tissue was completely resected , a continuous end-to-end or end-to-side suturing with 7/0(8/0) prolene was performed.In 1 type B cases with expected excessive anastomotic tension, the left subclavian artery was cut off to release the aortic arch .The associated anomalies were repaired during the cooling or re-warming period.Chest close was delayed to carry out no more than 24-72 h postoperatively in 16 cases.The other one type A IAA case without intracardiac anomaly was repaired through a standard left posterior lateral incision .Patients were strictly fol-lowed up with a standard protocol.Complications such as aortic arch restenosis, new LVOTO, new airway stenosis, and so on, were specialy focused on during the follow-up period.If needed, reoperation or transcatheter intervention was applied to treat the complications.Results Two cases died before operation and another 2 died in the early period postoperatively because of pulmonary hypertension crisis and severe tracheal stenosis, respectively.Sudden death was in 1 case during the follow-up peri-od.Median CPB time was 138 min(105-208 min) and median selective cerebral perfusion or circulatory arrest time was 24 min(16-35 min) .Mechanical ventilation time was 2-25 days.Blood pressure difference between the upper and lower limbs was less than 20 mmHg in all the patients before discharge.29 cases(85.29%) was followed up for 28 months(9 -60 months).3 cases were received reoperation in 5 cases of newly detected LVOTO.One case received reoperation in the 7 preop-erative LVOTO cases because of newly detected LVOTO(blood pressure gradient more than 40 mmHg).Two cases with aortic arch restenosis received percutaneous transcatheter ballon dilation .One case with new left main bronchus stenosis after opera-tion was strictly followed up.Conclusion IAA is a rare and severe congenital heart disease.Positive surgical repair should be performed after definite diagnosis being made .Although satisfactory results can be obtained with one-stage primary operation , IAA is a kind of progressive chronic disease.New postoperative LVOTO should be focused on becasue it will need reoperation or even the third operation .

Cardiovascular Magnetic Resonance Findings Late After the Arterial Switch Operation.

BACKGROUND: Despite its robust diagnostic capabilities in adolescents and adult patients after the arterial switch operation, little information is available on the cardiovascular magnetic resonance findings in this population. METHODS AND RESULTS: The cardiovascular magnetic resonance findings of 220 consecutive patients evaluated in our center were retrospectively reviewed (median age at cardiovascular magnetic resonance, 15.4 years; 66.8% male sex). Compared with published normal values, left and right ventricular end-diastolic volume z scores were mildly enlarged (0.48±1.76 and 0.33±1.5; P=0.0003 and 0.0038, respectively), with 26% of patients having left ventricular dilatation and 20% having right ventricular dilatation. Left ventricular dysfunction was present in 21.5% of patients (mild in most), and only 5.1% of patients had mild right ventricular dysfunction. Myocardial scar was found in 1.8% of patients. Dilatation of the neoaortic root was common (76%), and root z score increased at an average rate of 0.03 points per year. By multivariable analysis, neoaortic root dilatation was associated with worse neoaortic valve regurgitation (OR, 5.29; P=0.0016). The diameters of the thoracic aorta distal to the root were near-normal in most patients, whereas the neomain pulmonary artery was typically oval shaped with decreased anteroposterior and normal lateral diameters. CONCLUSIONS: Although the majority of arterial switch operation patients have normal ventricular size and function and myocardial scar is rare, an important minority exhibits ventricular enlargement or dysfunction. Neoaortic root dilatation, which is present in most patients and progresses over time, is strongly associated with significant neoaortic valve regurgitation. The findings of this study provide reference values against which arterial switch operation patients can be compared with their peers.

Morphological and Functional Evaluation of Quadricuspid Aortic Valves Using Cardiac Computed Tomography.

OBJECTIVE: The aim of this study was to identify the morphological and functional characteristics of quadricuspid aortic valves (QAV) on cardiac computed tomography (CCT). MATERIALS AND METHODS: We retrospectively enrolled 11 patients with QAV. All patients underwent CCT and transthoracic echocardiography (TTE), and 7 patients underwent cardiovascular magnetic resonance (CMR). The presence and classification of QAV assessed by CCT was compared with that of TTE and intraoperative findings. The regurgitant orifice area (ROA) measured by CCT was compared with severity of aortic regurgitation (AR) by TTE and the regurgitant fraction (RF) by CMR. RESULTS: All of the patients had AR; 9 had pure AR, 1 had combined aortic stenosis and regurgitation, and 1 had combined subaortic stenosis and regurgitation. Two patients had a subaortic fibrotic membrane and 1 of them showed a subaortic stenosis. One QAV was misdiagnosed as tricuspid aortic valve on TTE. In accordance with the Hurwitz and Robert's classification, consensus was reached on the QAV classification between the CCT and TTE findings in 7 of 10 patients. The patients were classified as type A (n = 1), type B (n = 3), type C (n = 1), type D (n = 4), and type F (n = 2) on CCT. A very high correlation existed between ROA by CCT and RF by CMR (r = 0.99) but a good correlation existed between ROA by CCT and regurgitant severity by TTE (r = 0.62). CONCLUSION: Cardiac computed tomography provides comprehensive anatomical and functional information about the QAV.

Takotsubo Cardiomyopathy in a 22-Year-Old Single-Ventricle Patient.

We report a highly unusual case of a 22-year-old woman with single-ventricle anatomy and a history of Fontan palliation who developed takotsubo cardiomyopathy. To our knowledge, takotsubo cardiomyopathy has not previously been described in a patient with single-ventricle anatomy, or more generally in any patient with congenital heart disease. The patient presented at our clinic for urgent examination after a 2-day history of chest pain that had begun upon the death of her dog. Invasive evaluation was refused by the patient; instead, she underwent electrocardiogram-gated coronary computed tomographic angiography, to rule out obstructive coronary disease. A physician who suspects takotsubo cardiomyopathy in a patient should look for new-onset chest pain and dyspnea coupled with these features: immediate onset of segmental akinesia in the midventricular or apical aspects of the heart, elevation of brain natriuretic peptide level 12 to 24 hours after onset of akinesia, elevation of troponin level 24 to 48 hours after onset, and disappearance of the segmental akinesia a few days after onset.

Favorable Waitlist and Posttransplant Outcomes in Children and Adolescent Patients Supported With Durable Continuous-Flow Ventricular Assist Devices.

Pediatric centers are implanting durable adult continuous-flow ventricular assist devices (CFVADs) in children who are smaller than the industry-recommended size. Waitlist and posttransplant outcomes data in pediatric patients supported with CFVADs as a bridge to transplant are limited. We analyzed the United Network of Organ Sharing and Organ Procurement and Transplantation Network registry to identify patients aged ≤18 years with a CFVAD at the time of listing or transplantation. Patients were stratified by body surface area (BSA; >1.5 vs. ≤1.5 m(2) ) at time of listing. We identified 138 patients with a durable CFVAD during the listing period (100 with BSA >1.5 m(2) , 38 with BSA ≤1.5 m(2) ). Patients with BSA ≤1.5 m(2) were more likely to have a noncardiomyopathy diagnosis (18% vs. 4%, p = 0.007) and to be implanted with a centrifugal-flow rather than an axial-flow device (74% vs. 30%, p = 0.001). There was no difference in failure-free waitlist survival between BSA groups (p = 0.99) among patients with a CFVAD at listing. Posttransplantation survival was 100% and 88% at 1 and 5 years, respectively, for the entire cohort and did not differ by BSA group (p = 0.99). Consequently, waitlist and posttransplant outcomes are favorable for pediatric CFVAD recipients. Small patients (≤1.5 m(2) ) had pre- and posttransplant outcomes similar to those of larger patients that met the industry-recommended size for implantation.