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

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

Short-term outcome of polytetrafluoroethylene membrane valve versus transannular pericardial patch reconstruction of right ventricular outflow tract in tetralogy of fallot: a randomized controlled trial

Abstract Introduction: Reconstruction of right ventricular outflow tract during primary repair of tetralogy of Fallot often requires the placement of a transannular patch which results in pulmonary regurgitation (PR). We compared the short-term outcomes of bicuspid polytetrafluoroethylene membrane valve versus transannular pericardial patch reconstruction of the right ventricular outflow tract. Methods: Thirty consecutive patients undergoing primary repair of tetralogy of Fallot were randomly allocated to two groups - polytetrafluoroethylene valve (PTFEV) group (n=15) and transannular pericardial patch (TAP) group (n=15). The two groups had similar preoperative demographic characteristics. We compared the short-term clinical and echocardiographic outcomes between these groups. The transthoracic echocardiographic follow-up was performed at one week, one month and six months after surgery. Results: The PTFEV group had significantly lower central venous pressure in the immediate postoperative period compared to the TAP group (7.60±2.06 vs. 10.13±1.73, P=0.002). Extubation time was significantly shorter in the PTFEV group compared to the TAP group (12.93±7.55 hrs vs. 22.23±15.11 hrs, P=0.04). PR in the PTFEV group was absent in five patients at 24 hours post-surgery. At the study endpoint, PR was absent in six, trivial in one and mild in eight patients in the PTFEV group compared to TAP group, where all 15 patients had severe PR. Conclusion: The bicuspid polytetrafluoroethylene membrane valves significantly decrease the central venous pressure in the immediate postoperative period, facilitate early extubation and, thus, prevent ventilator-related comorbidities. They achieve a high degree of pulmonary competence and do not increase the right ventricular outflow tract gradient in short-term follow-up.

Implante percutáneo de válvula pulmonar: a propósito de dos casos colombianos / Percutaneous pulmonary valve implant: Two Colombian case reports

Resumen Los pacientes con cardiopatías congénitas que afectan la continuidad del ventrículo derecho con la arteria pulmonar deben someterse con frecuencia a intervenciones debido a la limitada vida útil de los conductos quirúrgicos, lo que lleva al desarrollo de disfunción ventricular derecha por cambios en la geometría ventricular y predisposición a arritmias letales, con el consiguiente riesgo de reintervenciones. El implante valvular percutáneo pulmonar es una nueva alternativa terapéutica, menos invasiva en comparación con la quirúrgica, para pacientes seleccionados. Se realiza una revisión de las publicaciones médicas actuales disponibles y se describe la experiencia inicial del implante valvular pulmonar percutáneo en un centro colombiano de alta complejidad para el tratamiento de enfermedades cardiovasculares, en dos pacientes con disfunción del homoinjerto aórtico en posición pulmonar con doble lesión valvular, en los cuales el implante valvular pulmonar percutáneo fue una conducta exitosa. Se eligió a pacientes con cardiopatías congénitas, conductos quirúrgicos disfuncionales con estenosis o insuficiencia pulmonar significativa, y disfunción y dilatación ventricular derechas. Se empleó la técnica regular para el implante de la válvula pulmonar Melody, sin documentarse complicaciones durante el procedimiento ni al año de seguimiento. El implante percutáneo de la válvula pulmonar es un gran avance en el tratamiento de pacientes con cardiopatías congénitas, con resultados favorables a corto y mediano plazos, lo cual hace posible la restauración de la función ventricular con riesgo mínimo, frente al reemplazo quirúrgico en pacientes seleccionados.

Abstract Patients with congenital heart disease that involves reconstruction of the right ventricular outflow tract must frequently undergo interventions derived from the limited useful life of the surgical canals, which leads to the development of right ventricular dysfunction due to changes in the ventricular geometry and predisposition to lethal arrhythmias, with the consequent risk of reinterventions. The percutaneous pulmonary valvular implant is a new therapeutic alternative, less invasive, compared to surgery, for selected patients. A review of the available literature is made and the initial experience of percutaneous pulmonary valve implantation in a Colombian center of high complexity for the treatment of cardiovascular diseases is described, in two patients with aortic homograft dysfunction in a pulmonary position with double valvular lesion, in which the percutaneous pulmonary valve implant was a successful strategy. Patients with congenital heart disease were chosen, with dysfunctional surgical conduits with stenosis or significant pulmonary insufficiency, with dysfunction and right ventricular dilatation. The standard technique for the implantation of the Melody pulmonary valve was used, without complications during the procedure or one year of follow-up. Percutaneous implantation of the pulmonary valve is a great advance in the management of patients with congenital heart diseases, with favorable results in the short and medium term, allowing the restoration of ventricular function with minimal risk, compared to surgical replacement in selected patients.

Endocarditis de Libman-Sacks de válvulas nativas derechas en contexto de síndrome antifosfolípido y lupus eritematoso sistémico: ¡cuando el tratamiento marca la diferencia! / Libman-Sacks endocarditis of right native valves in the context of antiphospholipid syndrome and systemic lupus erythematosus: when treatment makes a difference!

We report the case of a 54 years old woman with antiphospholipid syndrome in irregular therapy, admitted due to exertional dyspnea and orthopnea. The transthoracic echocardiogram showed dilated cardiomyopathy with biventricular systolic dysfunction, pulmonary hypertension and masses related to the pulmonary and tricuspid valve without autonomic movement. The crops and white count were normal, with alteration of the SAF test, in addition, SLE was diagnosed. It was started therapy for heart failure, steroids, rituximab and anticoagulation, with improving of the symptoms. The echocardiographic control showed remission of the tricuspid masses and similar dimensions of the pulmonary mass.

Presentamos el caso de una mujer de 54años con síndrome antifosfolípido en terapia irregular, quien ingresa por disnea de esfuerzo que progresó a ortopnea. El ecocardiograma transtorácico evidenció cardiopatía dilatada con disfunción sistólica biventricular, hipertensión pulmonar y masas relacionadas con válvula pulmonar y tricúspide sin movimiento autonómico. Los cultivos y cuenta blanca estaban normales, con alteración de las pruebas del SAF, haciéndose, además, diagnóstico de lupus eritematoso sistémico. Se inició terapia para insuficiencia cardiaca, esteroides, rituximab y anticoagulantes, mejorando la clínica. El ecocardiograma control mostró remisión de las masas tricuspídeas y similares dimensiones de la masa pulmonar.

Percutaneous Pulmonary Valve Implantation

Percutaneous pulmonary valve implantation (PPVI) is recognized as a feasible and low risk alternative to surgery to treat dysfunctional right ventricular outflow tract (RVOT) in usually pluri-operated patients. Evolving technology allowed to develop different kind of prosthesis and to go from an initial treatment exclusively of stenotic conduit to an actual approach extended also to wide native RVOT. The Melody transcatheter pulmonary valve (TPV) and the Edwards Sapien valve are nowadays the most commonly implanted prostheses. However, other devices have been developed to treat large RVOT (i.e., the Venus p-valve, the Medtronic Harmony TPV, the Alterra Adaptive Prestent, and the Pulsta valve). Indications for PPVI are the same as for surgical interventions on pulmonary valve, with limits related to the maximum diameter of the available percutaneous prosthesis. Therefore, an accurate preoperative evaluation is of paramount importance to select patients who could benefit from this procedure. The overall periprocedural mortality incidence is around 1.4%, while freedom from RVOT reintervention ranges from 100% at 4 months to 70% at 70 months, according to the different published studies.

Changes in Right Ventricular Volume, Volume Load, and Function Measured with Cardiac Computed Tomography over the Entire Time Course of Tetralogy of Fallot

OBJECTIVE: To characterize the changes in right ventricular (RV) volume, volume load, and function measured with cardiac computed tomography (CT) over the entire time course of tetralogy of Fallot (TOF). MATERIALS AND METHODS: In 374 patients with TOF, the ventricular volume, ventricular function, and RV volume load were measured with cardiac CT preoperatively (stage 1), after palliative operation (stage 2), after total surgical repair (stage 3), or after pulmonary valve replacement (PVR) (stage 4). The CT-measured variables were compared among the four stages. After total surgical repair, the postoperative duration (POD) and the CT-measured variables were correlated with each other. In addition, the demographic and CT-measured variables in the early postoperative groups were compared with those in the late postoperative and the preoperative group. RESULTS: Significantly different CT-based measures were found between stages 1 and 3 (indexed RV end-diastolic volume [EDV], 63.6 ± 15.2 mL/m2 vs. 147.0 ± 38.5 mL/m2 and indexed stroke volume (SV) difference, 7.7 ± 10.3 mL/m2 vs. 32.2 ± 16.4 mL/m2; p < 0.001), and between stages 2 and 3 (indexed RV EDV, 72.4 ± 19.7 mL/m2 vs. 147.0 ± 38.5 mL/m2 and indexed SV difference, 5.7 ± 13.1 mL/m2 vs. 32.2 ± 16.4 mL/m2; p < 0.001). After PVR, the effect of RV volume load (i.e., indexed SV difference) was reduced from 32.2 mL/m2 to 1.7 mL/m2. Positive (0.2 to 0.8) or negative (−0.2 to −0.4) correlations were found among the CT-based measures except between the RV ejection fraction (EF) and the RV volume load parameters. With increasing POD, an early rapid increase was followed by a slow increase and a plateau in the indexed ventricular volumes and the RV volume load parameters. Compared with the preoperative data, larger ventricular volumes and lower EFs were observed in the early postoperative period. CONCLUSION: Cardiac CT can be used to characterize RV volume, volume load, and function over the entire time course of TOF.

The Role of Microenvironment in Preserving the Potency of Adult Porcine Pulmonary Valve Stem Cells In Vitro

BACKGROUND AND OBJECTIVE: The potency of tissue resident stem cells is regulated primarily by inputs from the local microenvironment. Isolation of stem cells through enzymatic digestion of tissue may affect epigenetic regulation of cell fate and performance. Here we employ a non-enzymatic method to harvest and investigate tissue resident stem cells from the adult porcine pulmonary valve. METHODS AND RESULTS: The presence of c-Kit+ stem cells within the valve tissue was confirmed by immunohistochemistry. An in vitro culture of minced valve leaflets was developed under the standard conditions (37°C with 5% CO2). The viability of the cellular outgrowths was evaluated over the subsequent 12 weeks. Under this culture condition, we identified a population of non-adherent c-Kit+ cells and multiple cellular structures mimicking the phenotype of embryonic stem cells at different stages of development. Formation of multinucleated cells through cell fusion provided an active niche area for homing and interaction of the non-adherent c-Kit+ cells. Expression of pluripotency markers Oct-4 and Nanog was detected in the newly formed multinucleated cells but not in mature colonies. Partial cell fusion was shown by fluorescent live-cell tracking, which confirmed intercellular molecular exchange between donor and recipient cells, resulting in altered cytoplasmic protein expression by the recipient cell. CONCLUSIONS: These results suggest a role for the microenvironment in decrypting the potential of the valve somatic stem cells in vitro. In addition, our data provide evidence for cell fusion, which may play a critical role in reversing somatic cell fate and spontaneous cellular reprogramming.

The change of QRS duration after pulmonary valve replacement in patients with repaired tetralogy of Fallot and pulmonary regurgitation / 소아과

PURPOSE: This study aimed to analyze changes in QRS duration and cardiothoracic ratio (CTR) following pulmonary valve replacement (PVR) in patients with tetralogy of Fallot (TOF). METHODS: Children and adolescents who had previously undergone total repair for TOF (n=67; median age, 16 years) who required elective PVR for pulmonary regurgitation and/or right ventricular out tract obstruction were included in this study. The QRS duration and CTR were measured pre- and postoperatively and postoperative changes were evaluated. RESULTS: Following PVR, the CTR significantly decreased (pre-PVR 57.2%±6.2%, post-PVR 53.8%±5.5%, P=0.002). The postoperative QRS duration showed a tendency to decrease (pre-PVR 162.7±26.4 msec, post-PVR 156.4±24.4 msec, P=0.124). QRS duration was greater than 180 msec in 6 patients prior to PVR. Of these, 5 patients showed a decrease in QRS duration following PVR; QRS duration was less than 180 msec in 2 patients, and QRS duration remained greater than 180 msec in 3 patients, including 2 patients with diffuse postoperative right ventricular outflow tract hypokinesis. Six patients had coexisting arrhythmias before PVR; 2 patients, atrial tachycardia; 3 patients, premature ventricular contraction; and 1 patient, premature atrial contraction. None of the patients presented with arrhythmia following PVR. CONCLUSION: The CTR and QRS duration reduced following PVR. However, QRS duration may not decrease below 180 msec after PVR, particularly in patients with right ventricular outflow tract hypokinesis. The CTR and ECG may provide additional clinical information on changes in right ventricular volume and/or pressure in these patients.

Interventional Cardiology for Congenital Heart Disease

Congenital heart interventions are now replacing surgical palliation and correction in an evolving number of congenital heart defects. Right ventricular outflow tract and ductus arteriosus stenting have demonstrated favorable outcomes compared to surgical systemic to pulmonary artery shunting, and it is likely surgical pulmonary valve replacement will become an uncommon procedure within the next decade, mirroring current practices in the treatment of atrial septal defects. Challenges remain, including the lack of device design focused on smaller infants and the inevitable consequences of somatic growth. Increasing parental and physician expectancy has inevitably lead to higher risk interventions on smaller infants and appreciation of the consequences of these interventions on departmental outcome data needs to be considered. Registry data evaluating congenital heart interventions remain less robust than surgical registries, leading to a lack of insight into the longer-term consequences of our interventions. Increasing collaboration with surgical colleagues has not been met with necessary development of dedicated equipment for hybrid interventions aimed at minimizing the longer-term consequences of scar to the heart. Therefore, great challenges remain to ensure children and adults with congenital heart disease continue to benefit from an exponential growth in minimally invasive interventions and technology. This can only be achieved through a concerted collaborative approach from physicians, industry, academia and regulatory bodies supporting great innovators to continue the philosophy of thinking beyond the limits that has been the foundation of our specialty for the past 50 years.

Tetralogía de Fallot con síndrome de válvula pulmonar ausente: Presentación de un caso y revisión de la literatura / Tetralogia de Fallot com síndrome da valva pulmonar ausente: Relato de caso e revisão da literatura / Tetralogy of Fallot with absent pulmonary valve syndrome: Case report and literature review

La tetralogía de Fallot es la cardiopatía congénita cianótica más frecuente del adulto. El síndrome de válvula pulmonar ausente constituye una variante poco frecuente, que representa del 3% al 6% de los pacientes con tetralogía de Fallot. Presentamos el caso de un paciente masculino de 29 años de edad, con tetralogía de Fallot y síndrome de válvula pulmonar ausente, los hallazgos del examen físico y los principales exámenes complementarios; como así también su evolución y una revisión de la literatura.

The tetralogy of Fallot is the most frequent cyanotic congenital heart disease in adult. Absent pulmonary valve syndrome is a rare variant, representing 3% to 6% of patients with tetralogy of Fallot. We present the case of a 29-year-old male patient with tetralogy of Fallot and absent pulmonary valve syndrome, physical examination findings and major complementary examinations; as well as its evolution and a review of the literature.

A tetralogia de Fallot é a doença congênita cianótica mais comum cardíacas em adultos. A síndrome valva pulmonar ausente é uma variante rara, que representa de 3% a 6% dos pacientes com tetralogia de Fallot. Apresentamos o caso de um paciente masculino de 29 anos de idade, com tetralogia de Fallot e síndrome da valva pulmonar ausente, exame físico e os principais estudos complementares; bem como a sua evolução e uma revisão da literatura.

Quando a ressonância magnética importa para a avaliação das cardiopatias / When Magnetic resonance imaging matters for the evaluation of cardiopathies

A ressonância magnética cardíaca (RMC) é uma modalidade de imagem não invasiva capaz de fornecer informações precisas e, muitas vezes, únicas na investigação de cardiopatias em geral e, em especial, nas cardiomiopatias. A capacidade de caracterizar precisamente o miocárdio do ponto de vista de sua contratilidade e suas características teciduais, diferenciando precisamente o miocárdio normal da fibrose miocárdica e identificando o edema miocárdio nas situações de agressão aguda ou recente do miocárdio, tornam a RMC indispensável hoje em qualquer serviço terciário e avançado de cardiologia do mundo. Neste artigo revisamos as aplicações clássicas e mais recentes da RMC em cardiopatias não isquêmicas, dividindo o uso das técnicas de RMC em dois grandes grupos: investigação da insuficiência cardíaca e das arritmias ventriculares. Dentro destes dois grupos pontuamos as etiologias mais importantes e frequentemente envolvidas. Na síndrome da insuficiência cardíaca destacam-se a cardiomiopatia dilatada com a fibrose mesocárdica linear septal e a miocardite viral com a manifestação de fibrose multifocal e mesoepicárdica. Uma proporção das cardiomiopatias dilatadas pode ter origem em uma miocardite viral prévia. A sarcoidose cardíaca pode apresentar uma variedade de tipos de realce tardio de padrão não isquêmico e isquêmico, e ser associada tanto ao quadro clínico de IC como de arritmia. A presença de sarcoidose pulmonar ou sistêmica pode ou não estar presente. A amiloidose cardíaca é o protótipo da cardiomiopatia restritiva e pode ser identificada pela RMC pelo padrão de realce tardio miocárdico global circunferencial (tipo AL) ou difuso, poupando o ápex do ventrículo esquerdo (tipo transtirretina). Finalmente, uma entidade ainda pouco entendida, a não compactação do ventrículo esquerdo (VE), em geral não apresenta realce tardio, mas tem fenótipo de trabeculação ventricular típico. No grupo das síndromes arrítmicas revisamos várias etiologias frequentemente associadas a esta apresentação clínica. Na hemossiderose cardíaca os valores de T2* abaixo de 20 ms indicam precisamente sobrecarga significativa de ferro miocárdico e associação com disfunção ventricular e arritmia ventricular. Na cardiomiopatia hipertrófica, a hipertrofia assimétrica e a fibrose miocárdica difusa, heterogênea e que acomete focalmente as inserções ventriculares, constituem o padrão clássico. Quantidade de fibrose acima de 15% da massa ventricular esquerda indica risco duas vezes maior de morte súbita. Na cardiomiopatia/displasia arritmogênica do ventrículo direito (VD), os volumes e a função ventricular direita global e segmentar pela RMC são partes fundamentais dos critérios diagnósticos da displasia pelo consenso atual. A cardiomiopatia chagásica tem mostrado intensa fibrose miocárdica desde as fases iniciais, mais intensa em homens que mulheres e frequentemente associada à edema miocárdico, marcador de provável inflamação crônica. A endomiocardiofibrose apresenta imagem patognomônica no realce tardio, o sinal do duplo V, caracterizando a fibrose miocárdica e trombo/calcificação preenchendo o ápex do VE e/ou VD. Nas valvopatias, além da detecção de fibrose miocárdica, que tem valor prognóstico, a RMC é precisa em quantificar as regurgitações, sendo indicada sua realização na insuficiência mitral antes da decisão de procedimento cirúrgico de troca ou correção valvar, eliminado um número significativo de casos em que a insuficiência mitral é superestimada pela ecocardiografia. Com esta revisão, cobrimos uma vasta gama de cardiopatias para as quais as técnicas de RMC realmente importam no diagnóstico e na estratificação prognóstica

Cardiovascular magnetic resonance (CMR) imaging is a noninvasive form of imaging capable of providing accurate and often unique information in the investigation of heart disease in general, and especially in cardiomyopathies. The ability to accurately characterize the myocardium in terms of its contractility and tissue characteristics, precisely differentiating normal myocardium from myocardial fibrosis and identifying myocardial edema in situations of acute or recent myocardial injury, has made CMR indispensable in any tertiary and advanced cardiology service around the World. In this paper, we review the classical and more recent applications of CMR in non-ischemic heart diseases, dividing the use of CMR techniques into two main groups: heart failure (HF) and ventricular arrhythmia investigations. Within these two groups, we highlight the most important and frequently involved etiologies. In heart failure syndrome, we focused on dilated cardiomyopathy with septal linear mesocardial fibrosis and viral myocarditis with the manifestation of multifocal and mesoepicardiac fibrosis. A proportion of dilated cardiomyopathies may have originated with an ancient viral myocarditis. Cardiac sarcoidosis may present a variety of late enhancement types of non-ischemic and ischemic patterns, and is associated with clinical signs of both HF and arrhythmia. The presence of pulmonary or systemic sarcoidosis may or may not be present. Cardiac amyloidosis is the prototype of restrictive cardiomyopathy, and can be identified in CMR by the global circumferential subendocardial (AL type) or diffuse myocardial enhancement pattern sparing the left ventricle (LV) apex (transthyretin type). Finally, a poorly understood entity, LV non-compaction generally does not present late enhancement, but has a typical ventricular trabeculation phenotype. In the group of arrhythmic syndromes, we reviewed several etiologies frequently associated with this clinical presentation. In cardiac siderosis, values of T2* below 20 ms accurately indicate a significant overload of myocardial iron and association with ventricular dysfunction and ventricular arrhythmia. In hypertrophic cardiomyopathy, asymmetric hypertrophy and diffuse myocardial fibrosis, which is heterogeneous and focally affects the ventricular insertions, constitute the classic pattern. An amount of fibrosis above 15% of the left ventricular mass indicates a two-fold increased risk of sudden death. In arrhythmogenic right ventricle (RV) cardiomyopathy/dysplasia, global and segmental right ventricular function and volumes by CMR are fundamental parts of the diagnostic criteria of dysplasia, according to current consensus. Chagasic cardiomyopathy has shown intense myocardial fibrosis since the early stages, which is more intense in men than women, and is frequently associated with myocardial edema, a marker of probable chronic inflammation. Endomyocardial fibrosis presents a pathognomonic image in late enhancement, the double V sign, characterizing myocardial fibrosis and thrombus/calcification filling the LV and/or RV apex. In valve diseases, in addition to the detection of myocardial fibrosis that has prognostic value, CMR is precise in quantifying regurgitations, and is indicated in mitral regurgitation prior to the decision for surgical valve replacement/correction, eliminating a significant number of cases in which mitral insufficiency is overestimated by the echocardiogram. This review covers a wide range of cardiopathies in which CMR techniques are extremely important in the diagnosis and prognostic stratification

Anesthesia for pulmonary trunk aneurysmorrhaphy / Anestesia para aneurismorrafia de tronco de artéria pulmonar

Abstract Background and objectives: The aneurysm in the pulmonary trunk is a rare disease. Because of its location, a rupture can lead to right ventricular failure and sudden death. Aneurysmorraphy is the most widely used surgical treatment in these cases. The aim of this study is to report a successful balanced general anesthesia for aneurysmorraphy of pulmonary trunk. Case report: Male patient, 28 years, asymptomatic, diagnosed with an aneurysm in the pulmonary trunk. According to the location of the aneurysm and the consequent failure of the pulmonary valve, an aneurysmorraphy was indicated, with implantation of vascular-valvular prosthesis (valved tube). We opted for a balanced general anesthesia, seeking to prevent an increase in systemic and pulmonary vascular resistances, thus avoiding to cause stress on the wall of the aneurysmal vessel. Conclusions: A balanced general anesthesia, in combination with adequate ventilation to prevent elevation in pulmonary vascular pressure, was appropriate for surgical repair of an aneurysm in the pulmonary trunk.

Resumo Justificativa e objetivos: O aneurisma de tronco de artéria pulmonar é uma doença rara. Por sua localização, uma ruptura pode conduzir à falência do ventrículo direito e à morte súbita. A aneurismorrafia é o tratamento cirúrgico mais usado nesses casos. O objetivo foi relatar uma anestesia geral balanceada para aneurismorrafia de tronco de artéria pulmonar feita com sucesso. Relato do caso: Paciente do sexo masculino, 28 anos, assintomático, diagnosticado com aneurisma de tronco de artéria pulmonar. De acordo com a localização do aneurisma e a consequente insuficiência da válvula pulmonar, foi indicada a aneurismorrafia com implante de prótese vascular e valvular (tubo valvado). Optou-se pela anestesia geral balanceada, para impedir um aumento nas resistências vasculares sistêmicas e pulmonar e evitar-se, dessa maneira, um estresse sobre a parede do vaso aneurismático. Conclusões: A anestesia geral balanceada, em associação com uma ventilação adequada para evitar elevação na pressão vascular pulmonar, foi apropriada para correção cirúrgica de um aneurisma em tronco pulmonar.

Valvuloplastia pulmonar en menores de 21 años / Pulmonary valvuloplasty in patients under the age of 21

Resumen Objetivo: Determinar la asociación entre la proporción de las reintervenciones en los pacientes con la estenosis valvular pulmonar y la presencia de un gradiente transvalvular pulmonar final ≥25 mm Hg en pacientes menores de 21 años. Metodología: Estudio unicéntrico observacional, tipo corte transversal de período. Población: Pacientes entre 0 meses y 21 años en quienes se realizó valvuloplastia pulmonar con balón. Análisis: Descripción del grupo y análisis en los subgrupos dados por el gradiente final transvalvular ≥25 mm Hg y la reintervención. Se realizaron pruebas chi2 de Pearson, para las variables categóricas. Para las variables continuas se realizaron pruebas U de Mann-Whitney. Se realizó una regresión logística para definir la asociación entre las variables y el desenlace a la reintervención. Resultados: En el grupo con el gradiente final ≥25 mm Hg, el 86,67% eran lactantes. Se encontró que en el grupo con el gradiente final < 25 mm Hg la mediana del gradiente inicial fue de 42 mm Hg IQ 25-75%: (34-59) en comparación con el grupo con el gradiente final ≥25 mm Hg, la mediana del gradiente inicial fue 70 mm Hg IQ 25-75%: (41-86). Al analizar los grupos por la variable reintervención, se observó que el cambio en la relación de las presiones entre el ventrículo derecho y el ventrículo izquierdo se asoció con menor necesidad de reintervención. OR 0,04; IC 95% (0,002-0,7). El tener un gradiente final después de la intervención ≥25 mm Hg se asoció a reintervención. OR 14,5; IC 95% (2,8-75). Conclusiones: Un gradiente final transvalvular pulmonar ≥25 mm Hg se asoció a mayor probabilidad de reintervención.

Abstract Motivation: To determine the association between the proportion of reoperations in patients with pulmonary valvular stenosis and the presence of a final pulmonary transvalvular gradient of ≥25 mm Hg in patients under the age of 21. Methods: Observational single-centre study, cross-sectional period type. Population: Patients between 0 months and 21 years of age who underwent balloon pulmonary valvuloplasty. Analysis: Description of the group and analysis in the subgroups given by the final transvalvular gradient of ≥25 mm Hg and reoperation. Pearson's chi-squared test was conducted for categorical variables. For the continuous variables, the Mann-Whitney U test was conducted. Logistic regression was used to define the association between variables and reoperation outcome. Results: In the group with the final gradient of ≥25 mm Hg, 86.67% were infants. The group with the final gradient of < 25 mm Hg the median of the initial gradient was 42 mm Hg IQ 25-75%: (34-59) in comparison to the group with the final gradient ≥25 mm Hg, the median of the initial gradient was 70 mm Hg IQ 25-75%: (41-86). By analysing both groups with the reoperation variable, it was observed that the change in the relationship of the pressured between the right ventricle and the left ventricle was associated with a lower need for reoperation. OR 0.04; CI 95% (0.002-0.7). Having a final gradient of ≥25 mm Hg after the surgery was associated to reoperation. OR 14.5; CI 95% (2.8-75). Conclusion: Having a final pulmonary transvalvular gradient of ≥25 mm Hg was associated to a higher probability of reoperation.

Three-dimensional echocardiography in adult congenital heart disease

Congenital heart disease (CHD) is now more common in adults than in children due to improvements in fetal echo, neonatal and pediatric care, and surgical techniques leading to dramatically increased survivability into adulthood. Adult patients with CHD, regardless of prior cardiac surgery, experience further cardiac problems or therapeutic challenges; therefore, a non-invasive, easily accessible echocardiographic examination is an essential follow-up tool. Among echocardiographic modalities, three-dimensional (3D) echocardiography provides better delineation of spatial relationships in complex cardiac geometries and more accurate volumetric information without geometric assumptions. For atrial septal defects, an en face view of the tissue defect allows better decisions on device closure. For tricuspid valve malformations, an en face view provides diagnostic information that is difficult to obtain from routine 2D tomography. In repaired tetralogy of fallot with pulmonary regurgitation, preoperative 3D echocardiography- based right ventricular volume may be used to determine the timing of a pulmonary valve replacement in conjunction with cardiovascular magnetic imaging. For optimal adult CHD care, 3D echocardiography is an important complement to routine 2D echocardiography.