Efficacy and safety of percutaneous transluminal pulmonary angioplasty in patients with chronic thromboembolic pulmonary hypertension / 中华心血管病杂志

Objective: To explore the efficacy and safety of percutaneous transluminal pulmonary angioplasty (PTPA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Methods: This prospective single arm study included 19 CTEPH patients (7 male, age(56.3±12.5)years) admitted to Wuhan Asia Heart Hospital from January 2017 to June 2019 and received PTPA interventional therapy. Baseline data, including age, sex, WHO functional class, 6-minute walk distance (6MWD), NT-proBNP, right heart catheterization values, were collected. Patients received single or repeated PTPA. Number of dilated vessels from each patient was analyzed, patients were followed up for 24 weeks and right heart catheterization was repeated at 24 weeks post initial PTPA. All-cause death, perioperative complications, and reperfusion pulmonary edema were reported. WHO functional class, 6MWD, NT-proBNP, right heart catheterization values were compared between baseline and at 24 weeks follow up. Results: Nineteen CTEPH patients received a total of 56 PTPA treatments. The pulmonary artery pressure (mPAP) decreased from (40.11±7.55) mmHg (1 mmHg=0.133 kPa) to (27.53±4.75) mmHg (P<0.001), and the total pulmonary resistance (TPR) decreased from (13.00±3.56) Wood U to (5.48±1.56) Wood U (P<0.001), cardiac output increased from (3.19±0.63) L/min to (5.23±0.94) L/minutes (P<0.01) at 24 weeks post PTPA. The WHO functional class improved significantly (P<0.001), 6MWD increased from (307.08±129.51) m to (428.00±112.64) m (P=0.002), the NT-proBNP decreased at 24 weeks post PTPA (P=0.002). During the follow-up period, there was no death; hemoptysis occurred in 4 patients during the operation, none of which resulted in serious adverse clinical consequences. One patient developed reperfusion pulmonary edema and recovered after treatment. Conclusion: PTPA treatment is safe and can significantly improve the hemodynamics and WHO functional class of patients with CTEPH.

Medium and long-term follow-up of the Pul-Stent in treating postoperative branch pulmonary artery stenosis in children with congenital heart disease / 中华儿科杂志

Objective: To evaluate the effectiveness and safety of Pul-Stent as the treatment of postoperative branch pulmonary artery stenosis in children with congenital heart disease. Methods: This was a retrospective study. Thirty-three patients who underwent Pul-Stent implantation in Shanghai Children's Medical Center due to postoperative residual pulmonary artery stenosis from August 2014 to June 2015 were included. The immediate curative effect, follow-up and complications of Pul-Stent implantation were assessed. Comparisons between groups were performed with unpaired Student t test. Results: Pul-Stent implantation of 33 patients (19 males and 14 females) were performed successfully. Thirty-one patients underwent percutaneous stenting, and 2 patients underwent hybrid stenting. A total of 35 Pul-Stents were implanted (19 of model small, 15 of model medium and one of model large), 23 stents were planted in the proximal left pulmonary artery and 12 stents were in the proximal right pulmonary artery. The initial diameter of dilation balloon ranged from 6 to 16 mm, and the long sheath of percutaneous implantation ranged from 8 to 10 F in 29 patients (29/31, 94%). After stenting, the diameter of the narrowest segment of pulmonary artery increased from (4.0±1.7) mm to (9.1±2.1) mm in all patients (t=-21.60, P<0.001). The pressure gradient at the stenosis in 26 patients after biventricular correction decreased from (30.5±12.3) mmHg (1 mmHg=0.133 kPa) to (9.9±9.6) mmHg (t=12.92, P<0.001), and the right ventricular to aortic pressure ratio decreased from 0.57±0.14 to 0.44±0.12 (t=7.44, P<0.001). The pressure of the superior vena cava after stenting in 5 patients after cavopulmonary anastomosis decreased from (17.0±1.9) mmHg to (14.0±0.7) mmHg (t=2.86, P=0.046). Two patients died during reoperation for repairing other cardiac malformations. The remaining 31 patients were clinically stable during the follow-up period of (5.3±1.6) years, and one stent fracture was found on chest X-ray. Cardiac catheterization reexaminations in 16 patients showed that restenosis was found in one stent, while stent position and patency were satisfactory in the remaining stents. Nine children underwent post-dilation without stent fracture, displacement or aneurysm formation. Cardiac tomography showed no stent stenosis, fracture observed, or significant change in diameter of the stent in 8 patients. The inner diameter and pulmonary blood perfusion could not be accurately evaluated due to artifacts by cardiac magnetic resonance imaging in 4 patients. Conclusions: Pul-Stent has good compliance and adequate radial strength, and can dilate further over time to accommodate for somatic growth. It performs safely and effectively in treating post-operative branch pulmonary artery stenosis in children.

Intervenção Paliativa Endovascular no Lactente com Tetralogia de Fallot: Uma Série de Casos / Palliative Endovascular Intervention in Infants with Tetralogy of Fallot: A Case Series

Resumo Fundamento Tendo em vista os casos de lactentes sintomáticos com Tetralogia de Fallot (TF), baixo peso ao nascimento e anatomia complexa, o implante de stent na via de saída do ventrículo direito (VSVD) tem sido indicado alternativamente à cirurgia de Blalock-Taussig (BT). Objetivo Avaliar o implante endovascular de stent na VSVD como abordagem primária no lactente com TF e não candidato à cirurgia de BT, bem como relatar seus resultados a médio prazo e até a retirada do stent na cirurgia corretiva. Métodos Entre outubro de 2015 e abril de 2018, uma série de seis lactentes portadores de TF receberam stents para desobstrução da VSVD. Os parâmetros hemodinâmicos foram comparados em períodos pré e pós-implante. Resultados As medianas de idade e peso no momento do procedimento foram de 146,5 dias e 4,9 kg, respectivamente. O gradiente sistólico máximo diminuiu de 63,5 mmHg para 50,5 mmHg, enquanto o diâmetro dos ramos pulmonares direito e esquerdo aumentou de 3,5 mm para 4,9 mm e 4,3 mm, respectivamente. O índice de Nakata aumentou de 96,5 mm para 108,3 mm; assim como o peso, de 4,9 kg para 5,5 kg. A saturação de oxigênio aumentou de 83,5% para 93%. Houve um caso de migração do stent e dois óbitos, um deles devido à embolização do stent e o outro não teve relação com o procedimento. Conclusões O implante de stent na VSVD como procedimento paliativo na TF se mostra uma alternativa promissora para o tratamento de lactentes com má anatomia e baixo peso ao nascimento.

Abstract Background Endovascular stent placement in the right ventricular outflow tract (RVOT) has been an alternative to Blalock-Taussig (BT) surgery in the treatment of Tetralogy of Fallot (TOF) in symptomatic infants with low birth weight and complex anatomy. Objective To evaluate endovascular stent placement in the RVOT as a primary treatment for infants with TOF who are not candidates for BT surgery, and evaluate medium-term outcomes until the stent is removed during corrective surgery. Methods Six infants with TOF were treated with RVOT stenting from October 2015 to April 2018. Hemodynamic parameters were compared between the pre- and post-stenting periods. Results At the time of stenting, participants had a median age and weight of 146.5 days and 4.9 kg, respectively. Peak systolic gradient decreased from 63.5 mm Hg to 50.5 mm Hg, while the diameter of the left and right pulmonary arteries increased from 3.5 mm to 4.9 mm and 4.3 mm, respectively. The Nakata index increased from 96.5 mm to 108.3 mm; weight increased from 4.9 kg to 5.5 kg; and oxygen saturation, from 83.5% to 93%. There was one case of stent migration and two deaths, one caused by stent embolization and the other unrelated to study procedures. Conclusions RVOT stenting is a promising alternative for the palliative treatment of TOF in infants with low birth weight and complex anatomy.

Origen anómalo de una de las ramas de la arteria pulmonar (OAAP): tratamiento quirúrgico y resultados / Anomalous origin of one of the branches of the pulmonary artery (AOPA): Surgical treatment and outcome

Resumen Introducción: El origen anómalo de una de las ramas de la arteria pulmonar procedente de la aorta ascendente es poco frecuente. Objetivo: Identificar las características clínicas y quirúrgicas de los pacientes sometidos a reimplante de la rama afectada. Método: Se realizó un estudio observacional, transversal, descriptivo, retrospectivo y retrolectivo, en el que se incluyeron los pacientes diagnosticados de origen anómalo de alguna de las ramas de la arteria pulmonar y que fueron tratados mediante cirugía correctiva en el Instituto Nacional de Cardiología Ignacio Chávez, en el periodo del 1 de enero de 2003 al 31 de enero de 2019. De los expedientes se extrajeron las características demográficas, los antecedentes quirúrgicos, los diagnósticos, los reportes ecocardiográficos y tomográficos, la técnica quirúrgica y el estado posquirúrgico. Resultados: Se encontraron nueve pacientes sometidos a cirugía de corrección, con un promedio de edad de 2 ± 2 años, un peso de 11.4 ± 1.5 kg y una talla de 82 ± 15 cm. El 67% fueron de sexo masculino. La rama afectada con más frecuencia fue la derecha; el 68% se originaban directamente de la aorta y el 42% de un conducto arterioso persistente. La corrección con circulación extracorpórea tuvo un tiempo promedio de pinzamiento aórtico de 35 minutos y de soporte circulatorio de 45 minutos. Se utilizó el implante directo o con material sintético. Las complicaciones fueron falla ventricular, sangrado, arritmias y neumonía nosocomial. La mortalidad fue del 11%. Conclusiones: El tratamiento quirúrgico para el reimplante de la rama anómala de la arteria pulmonar es el procedimiento de elección, y con cuidados quirúrgicos y posteriores tiene un buen pronóstico a mediano y largo plazo.

Abstract Introduction: The anomalous origin of one of the branches of the pulmonary artery from the ascending aorta is rare. Objective: To identify the clinical and surgical characteristics of the patients undergoing reimplantation of the affected branch. Method: An observational, cross-sectional, descriptive, retrospective and retrolective study was performed; patients diagnosed with anomalous origin of one of the branches of the pulmonary artery and treated by corrective surgery at the Instituto Nacional de Cardiología Ignacio Chavez, in the period from January 1, 2003 to January 31, 2019, were included. It was extracted from the files: demographic characteristics, surgical antecedents, diagnoses, echocardiographic and tomographic reports, surgical technique and post-surgical status. Results: Nine patients underwent correction surgery, with an average age of 2 ± 2 years, 11.4 ± 1.5 kg and height 82 ± 15 cm; 67% were male. The most frequent affected branch was the right, 68% originated directly from the aorta and 42% from a patent ductus arteriosus. The correction with extracorporeal circulation had an average aortic cross-clamp of 35 minutes and circulatory support of 45 minutes, the direct or synthetic implant was used. The complications were ventricular failure, bleeding, arrhythmias and nosocomial pneumonia. Mortality was 11%. Conclusions: Surgical treatment for reimplantation of the anomalous branch of the pulmonary artery is the procedure of choice, which with surgical and subsequent care has a good prognosis in the medium and long term.

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

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

Type ii aortopulmonary window with isolated left subclavian artery from left pulmonary artery

Abstract Type II Aortopulmonary window (APW) accounts for only 10% of total cases of APW, which by itself is a rare congenital anomaly. Various cardiac malformations have been reported to be associated with this rare anomaly. We report one such association of origin of left subclavian artery (LSCA) from left pulmonary artery (LPA) via ductus arteriosus that was surgically repaired.

Case report: surgical treatment of high-flow coronary fistulas for the pulmonary artery

Abstract Coronary fistulas are rare anomalies that can affect approximately 1% of the population, presenting few specific symptoms, and are often found occasionally in coronary angiography. Here we describe the case of a 61-year-old patient with complaints of precordialgia and dyspnea since adolescence, with late diagnosis of coronary fistulas with drainage to the pulmonary artery, and with unsuccessful percutaneous treatment. Therefore, she underwent open surgery for the correction of the already known fistulas, in addition to the hemangioma involving such vessels, which made the understanding and resolution of this case more complex.

Surgical repair of complex aortopulmonary window: a case study

Abstract Aortopulmonary septal defect, also known as the aortopulmonary window, is a rare congenital macrovascular malformation. This case involves a 9-year-old boy with aortopulmonary septal defect (type I combined with type IV). Before surgery, milrinone and alprostadil were used to counteract high lung pressure. Surgery was performed under cardiopulmonary bypass, following which the pulmonary pressure decreased. The aorta was cut, and the right pulmonary artery opening was connected with the main pulmonary artery septal defect using polyester patch. An internal tunnel was made, and the deformity correction was completed. The child exhibited normal postoperative recovery with no discomfort. A complex aortopulmonary window is a rare condition that can be treated successfully with appropriate preoperative and surgical management.

Modified hybrid procedure in hypoplastic left heart syndrome: initial experience of a center in northeastern Brazil

Abstract Introduction: Although it only corresponds to 2.5% of congenital heart defects, hypoplastic left heart syndrome (HLHS) is responsible for more than 25% of cardiac deaths in the first week of life. Palliative surgery performed after the second week of life is considered an important risk factor in the treatment of HLHS. Objective: The aim of this study is to describe the initial experience of a medical center in Northeastern Brazil with a modified off-pump hybrid approach for palliation of HLHS. Methods: From November 2012 through November 2015, the medical records of 8 patients with HLHS undergoing hybrid procedure were retrospectively evaluated in a tertiary private hospital in Northeastern Brazil. The modified off-pump hybrid palliation consisted of stenting of the ductus arteriosus guided by fluoroscopy without contrast and banding of the main pulmonary artery branches. Demographic and clinical variables were recorded for descriptive analysis. Results: Eight patients were included in this study, of whom 37.5% were female. The median age and weight at the time of the procedure was 2 days (p25% and p75% = 2 and 4.5 days, respectively) and 3150 g (p25% and p75% = 3077.5 g and 3400 g, respectively), respectively. The median length in intensive care unit stay was 6 days (p25% and p75% = 3.5% and 8 days, respectively). There were no in-hospital deaths. Four patients have undergone to the second stage of the surgical treatment of HLHS. Conclusion: In this series, the initial experience with the modified off-pump hybrid procedure showed to be safe, allowing a low early mortality rate among children presenting HLHS.

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.

Stent Placement in a Neonate with Sano Modification of the Norwood using Semi-Elective Extracorporeal Membrane Oxygenation / Colocação de Stent em um Recém-Nascido Submetido à Modificação de Sano da Operação de Norwood Usando Oxigenação por Membrana Extracorpórea Semi-Eletiva

Abstract Extracorporeal membrane oxygenation (ECMO) is a well-established tool of cardiopulmonary circulatory support for cardiopulmonary failure in children and adults. It has been used as a supportive strategy during interventional procedures in neonates with congenital heart disease. Herein, we describe a neonate with hypoplastic left heart syndrome who underwent stenting of the Sano shunt and left pulmonary artery after Norwood Sano operation using intra-procedural ECMO support. The use of ECMO as a bridge to recovery might be a feasible and reasonably safe adjunctive approach in the treatment of complications in selective case of neonates having undergone the Norwood Sano procedure.

Resumo A oxigenação por membrana extracorpórea (ECMO) é uma ferramenta bem estabelecida de suporte circulatório em casos de insuficiência em crianças e adultos. A ECMO tem sido utilizada como uma estratégia de suporte durante procedimentos interventistas em recém-nascidos com doença cardíaca congênita. Descrevemos o caso de um recém-nascido com síndrome do coração esquerdo hipoplásico que foi submetido à colocação de um stent em shunt de Sano e artéria pulmonar esquerda após procedimento de Norwood-Sano utilizando-se a ECMO como suporte. O uso da ECMO pode ser uma estratégia adjunta viável e segura no tratamento de complicações em casos eletivos de recém-nascidos submetidos à operação de Norwood-Sano.

Atualização no manejo clínico do choque cardiogênico / Update on clinical management of cardiogenic shock

O choque cardiogênico é uma síndrome clínica caracterizada por hipoperfusão tecidual secundária à disfunção cardíaca, na ausência de hipovolemia. A etiologia principal é o infarto agudo do miocárdio com falência de ventrículo esquerdo, embora existam outras causas relevantes, como complicações mecânicas do infarto, miocardites, evolução de cardiomiopatias, valvopatias agudas não isquêmicas e cardiomiopatia induzida pelo estresse, por exemplo. Apesar dos avanços terapêuticos, persiste como uma afecção de elevada mortalidade e sua incidência não se modificou significativamente nas últimas décadas. A instalação do choque em pacientes com síndromes coronarianasagudas ocorre mais frequentemente após a internação hospitalar, ressaltandoa importância da identificação de preditores e da monitorização de sinais precoces de hipoperfusão tecidual para pronta intervenção. O diagnóstico é essencialmente clínico e alguns exames subsidiários, como eletrocardiograma, marcadores de necrose miocárdica,ecocardiograma e cineangiocoronariografia, que são importantes para a definição da etiologia, estratificação da gravidade e do prognóstico. O manejo adequado dos pacientes requer avaliação dos parâmetros de macro e micro-hemodinâmica. Os principais objetivos terapêuticos incluem a restauração precoce da perfusão tecidual sistêmica ea recuperação da função ventricular. O tratamento inclui medidas gerais para pacientes com choque como ajuste de volemia e adequação da perfusão tecidual com uso de inotrópicos, vasopressores e dispositivos de assistência ventricular, além de medidasespecíficas, direcionadas para a etiologia do choque, como a revascularização precoce nas síndromes coronarianas agudas...

Cardiogenic shock is a clinical syndrome characterized by tissue hypoperfusionsecondary to cardiac dysfunction, in the absence of hypovolemia. Left ventricular failure complicating acute myocardial infarction is the main cause, although other relevant causes include mechanical complications of infarction, myocarditis, progression of cardiomyopathies, acute non-ischemic valvular heart disease, and stress-induced cardiomyopathies, for example. Despite therapeutic advances, it persists as a condition of high mortality, and its incidence has not changed significantly in the last decades.The onset of cardiogenic shock in patients with acute coronary syndromes frequently occurs after hospital admission, reinforcing the importance of identifying predictors and monitoring early signs of tissue hypoperfusion for prompt intervention. The diagnosisis essentially clinical plus some additional tests such as electrocardiogram, myocardial necrosis markers, echocardiogram, and coronary angiography, which are important fordefining the etiology, stratification of severity and prognosis. Proper management of patients requires assessment of macro- and micro-hemodynamic parameters. The maintherapeutic goals include early restoration of tissue perfusion and recovery of ventricular function. Treatment includes general medical care for patients with shock as optimizingvolemia and adjusting of tissue perfusion with inotropic agents, vasopressors and ventricular assist devices, as well as specific interventions focused on the shock etiology, like early revascularization in acute coronary syndromes...

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

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

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