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1.
Kyobu Geka ; 73(10): 805-811, 2020 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-33130770

RESUMO

Although the characteristics and pathophysiology of major aortopulmonary collateral artery (MAPCA) have been better understood, treatment of pulmonary atresia and ventricular septal defect with MAPCA remains challenging. The difficulty in treating this disease is the lack of availability of pulmonary homografts required for pulmonary angioplasty in Japan and the need for manipulation of the posterior mediastinum region, which is not performed in normal cardiac surgery. It is important to build an appropriate surgical strategy by means of preoperative computed tomography( CT) and selective angiography. For treatment, unifocalization or rehabilitation is selected depending on the condition of MAPCA and central pulmonary artery. Because unifocalization is highly invasive, it would be performed at 6 months of age and weigh 5 kg. As a procedure, extensive dissection is performed, and anastomoses between tissue to tissue are basically performed, and reconstruction of the right ventricular outflow tract is performed using a valved conduit that can secure anterograde pulmonary blood flow that is not affected by the condition of the lung. It is effective to confirm postoperative lung perfusion scan and CT, perform a cardiac catheterization test approximately 6 months after surgery, and maintain the pulmonary vessels with catheter intervention if necessary. The ultimate goal of treating this disease is not only to improve cyanosis, but also to maintain pulmonary circulation at lower right ventricular pressure.


Assuntos
Comunicação Interventricular , Atresia Pulmonar , Circulação Colateral , Defeitos dos Septos Cardíacos , Comunicação Interventricular/diagnóstico por imagem , Comunicação Interventricular/cirurgia , Humanos , Lactente , Japão , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Atresia Pulmonar/diagnóstico por imagem , Atresia Pulmonar/cirurgia
2.
J Card Surg ; 35(8): 2037-2038, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32652604

RESUMO

We present a case of an infant with tetralogy of Fallot with pulmonary atresia with an incidentally detected cervical origin of right subclavian artery. We highlight the importance of identifying this rare variation and the potential complications that may ensue.


Assuntos
Pescoço/irrigação sanguínea , Atresia Pulmonar/diagnóstico por imagem , Artéria Subclávia/anormalidades , Artéria Subclávia/diagnóstico por imagem , Tetralogia de Fallot/diagnóstico por imagem , Aorta Torácica/diagnóstico por imagem , Angiografia por Tomografia Computadorizada , Ecocardiografia , Humanos , Lactente , Masculino , Artéria Pulmonar/diagnóstico por imagem , Atresia Pulmonar/cirurgia , Tetralogia de Fallot/cirurgia
3.
Cardiol Young ; 30(9): 1350-1352, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32613935
4.
Pediatr Cardiol ; 41(7): 1354-1362, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32474740

RESUMO

Ductal stenting (DS) palliates duct-dependent lesions using coronary stents. Sirolimus-eluting stents have replaced bare-metal stents in coronary interventions. Concerns exist about sirolimus levels in neonates. Therapeutic immunosuppressive sirolimus level is 5-15 ng/ml. After neonatal DS, drug levels were assessed at 24 h, 7 days and monthly thereafter till they were undetectable. Clinical course, ductal patency till their final corrective surgery was analyzed. The exact quantity of sirolimus in each stent was known. Twelve neonates with median age of 5.5 days received sirolimus-eluting stents, one stent in nine and two in the rest. The lesions were pulmonary atresia intact ventricular septum(PAIVS) in four, univentricular lesions with pulmonary atresia in four, biventricular lesions with pulmonary atresia in three and right ventricular rhabdomyoma in one neonate. If single stents up to 22 mm length, 24-h drug levels were less than 5 ng/ml. Even though 24-h levels were above 5 ng/ml in patients with single longer stent or two stents, it reduced to very low levels by seventh day. Two hospital deaths included rhabdomyoma with complete heart block and post-valvotomy cardiac failure for PAIVS. Stent patency after valvotomy for PAIVS exceeded three years. Patency was retained for 8-27 months till their elective corrective surgery in others. Sirolimus levels were acceptable at 24 h in all neonates receiving single stent under 22 mm length. In patients needing two stents, drug levels were in immunosuppressive range at 24 h but reduced rapidly within 7 days. The palliation provided by sirolimus-eluting DS was sufficiently long to provide clinical benefit.


Assuntos
Stents Farmacológicos , Permeabilidade do Canal Arterial/tratamento farmacológico , Circulação Pulmonar/efeitos dos fármacos , Sirolimo/sangue , Sirolimo/uso terapêutico , Cromo , Cobalto , Permeabilidade do Canal Arterial/cirurgia , Feminino , Ventrículos do Coração/fisiopatologia , Humanos , Imunossupressores , Recém-Nascido , Masculino , Estudos Prospectivos , Implantação de Prótese , Atresia Pulmonar/cirurgia , Resultado do Tratamento , Septo Interventricular
5.
Pediatr Cardiol ; 41(5): 1012-1020, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32377890

RESUMO

BACKGROUND: The optimal surgical strategy for pulmonary atresia with ventricular septal defect (PA/VSD) in neonates and young infants is controversial. Staged repair may be associated with a higher risk of inter-stage mortality, while primary repair may lead to frequent post-repair re-interventions. METHODS: From 2004 to 2017, 65 patients with PA/VSD who underwent surgical intervention before 90 days of age were identified and enrolled in this retrospective study. The cohort was divided into two groups: group-SR, who underwent initial palliation with staged repair (n = 50), and group-PR who underwent primary repair (n = 15). RESULTS: There were three post-palliation in-hospital mortalities, four inter-stage mortalities, and one post-repair in-hospital mortality in group-SR. In group-PR, there was one in-hospital death and one late death. Five-year survival rates were comparable between the two groups (group-SR: 83.6%; group-PR: 86.7%; p = 0.754). During the median follow-up duration of 44.7 months (Inter-quartile range, 19-109 months), 40 post-repair re-interventions (22 in group-SR, 18 in group-PR) were performed in 26 patients (18 in group-SR, 8 in group-PR). On Cox proportional hazards model, primary repair was identified as the only risk factor for decreased time to death/1st post-repair re-intervention (Hazard ratio (HR): 2.3, p = 0.049) and death/2nd post-repair re-intervention (HR 2.91, p = 0.033). CONCLUSIONS: A staged repair strategy, compared with primary repair, was associated with comparable overall survival with less frequent re-interventions after repair in young infants with PA/VSD. Lowering the inter-stage mortality after initial palliation by vigilant outpatient care and aggressive home monitoring may be the key to better surgical outcomes in this subset. Surgical outcomes of PA with VSD according to the surgical strategies. Patient 1 (birth weight: 2.7 kg) underwent primary Rastelli-type repair at post-natal day # 50 (body weight: 3.8 kg) using Contegra® 12 mm. The postoperative course was rocky, with long ventilatory support (10 days), ICU stay (14 days), and hospital stay (20 days). Cardiac CT scan at 9 months post-repair showed severe branch pulmonary artery stenosis, which necessitated LPA stenting at 12 months post-repair and RV-PA conduit replacement with extensive pulmonary artery reconstruction at 25 months post-repair. Patient 2 (birth weight: 2.5 kg) underwent RMBT at post-natal day #30 (body weight: 3.4 kg) using 4 mm PTFE vascular graft and staged Rastelli-type repair at post-natal 11 months using a hand-made Gore-Tex valved conduit (14 mm). No post-repair re-intervention has been performed. Cardiac CT scan at 90 months post-repair showed no branch pulmonary artery stenosis.CT computed tomography, ICU intensive care unit, LPA left pulmonary artery, PA pulmonary atresia, PTFE polytetrafluoroethylene, RMBT right modified Blalock-Taussig shunt, RV-PA right ventricle to pulmonary artery, VSD ventricular septal defect.


Assuntos
Procedimentos Cirúrgicos Cardiovasculares/métodos , Defeitos dos Septos Cardíacos/cirurgia , Cuidados Paliativos/métodos , Atresia Pulmonar/cirurgia , Procedimentos Cirúrgicos Cardiovasculares/mortalidade , Estudos de Casos e Controles , Feminino , Defeitos dos Septos Cardíacos/mortalidade , Mortalidade Hospitalar , Humanos , Lactente , Recém-Nascido , Tempo de Internação/estatística & dados numéricos , Masculino , Cuidados Paliativos/estatística & dados numéricos , Período Pós-Operatório , Modelos de Riscos Proporcionais , Atresia Pulmonar/mortalidade , Estudos Retrospectivos , Resultado do Tratamento
7.
Pediatr Cardiol ; 41(6): 1238-1241, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32367306

RESUMO

Muscular pulmonary atresia with intact ventricular septum (PA/IVS) in neonates is traditionally managed by surgery. We describe hybrid approach to decompress the right ventricle (RV) and establish RV to pulmonary artery connection in a neonate avoiding cardiopulmonary bypass. A 21-gauge access needle was used to perforate the atretic pulmonary valve via periventricular approach followed by stent placement. This case is an example of how patients with congenital heart disease can be palliated in creative ways through thoughtful collaboration between surgical and interventional cardiology teams.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Descompressão Cirúrgica/métodos , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/cirurgia , Atresia Pulmonar/cirurgia , Septo Interventricular , Angiografia , Ponte Cardiopulmonar/métodos , Humanos , Recém-Nascido , Masculino , Artéria Pulmonar/diagnóstico por imagem , Resultado do Tratamento
8.
J Surg Res ; 251: 38-46, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32113036

RESUMO

BACKGROUND: Surgical treatment of pulmonary atresia with intact ventricular septum (PA/IVS) in neonates is challenging because of the broad variations of right ventricular (RV) malformations. In this retrospective study, we summarized our 8-y experience in surgical management for neonatal PA/IVS patients. METHODS: Thirty-four neonates with PA/IVS between July 1, 2006 and June 30, 2014, were reviewed. Patients were categorized into three groups: mild, moderate, and severe RV hypoplasia according to RV morphology and development. Patients were on regular follow-up for at least 5 y. Overall survival, complications, reinterventions, risk factors for mortality, and health status were evaluated. RESULTS: 21 patients (61.8%) were treated with biventricular repair, eight patients (23.5%) with Fontan procedure, and one patient (2.9%) with bidirectional Glenn procedure. There were four postprocedural mortalities and one late death. The 5-y survival rates after final surgical repair for mild, moderate, and severe RV hypoplasia groups were 100%, 100%, and 88.9%, respectively. The reintervention rates were 0% (0/4), 21.4% (3/14), and 55.6% (5/9) for the subgroups, respectively. At the latest follow-up, most patients had a status characterized as New York Heart Association class I (88.9%, 24/27). CONCLUSIONS: Surgical management for PA/IVS in neonates should be individualized. Favorable early and long-term outcomes can be achieved in neonatal PA/IVS patients by individualized surgical strategies, regardless of the degree of RV hypoplasia. In spite of potential RV catch-up development, the degree of RV hypoplasia is a factor of paramount importance to assess PA/IVS in neonates.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Cardiopatias Congênitas/cirurgia , Atresia Pulmonar/cirurgia , Procedimentos Cirúrgicos Cardíacos/mortalidade , Feminino , Seguimentos , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/patologia , Ventrículos do Coração/anormalidades , Ventrículos do Coração/patologia , Ventrículos do Coração/cirurgia , Humanos , Recém-Nascido , Masculino , Atresia Pulmonar/mortalidade , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida , Resultado do Tratamento
9.
Zhonghua Er Ke Za Zhi ; 58(2): 96-100, 2020 Feb 02.
Artigo em Chinês | MEDLINE | ID: mdl-32102144

RESUMO

Objective: To investigate the safety and efficacy of transcatheter perforation of pulmonary valve by micro-guidewire and balloon dilation in the treatment of neonatal pulmonary atresia with intact ventricular septum (PA-IVS). Methods: The retrospective study included 21 neonates (14 males and 7 females) with PA-IVS who underwent transcatheter micro-guidewire pulmonary valve perforation and balloon dilation in Xinhua Hospital from January 2012 to December 2018. All patients underwent the pulmonary valve perforation by micro-guidewire through the Simmons catheter. During the follow-up period at 1, 3, 6, 12 months postoperatively and annually thereafter, the operative efficacy and the development of the right ventricle (RV) were evaluated by echocardiography. Statistical analyses were performed using t test. Results: A total of 21 neonates with PA-IVS were enrolled, and 13 cases were diagnosed prenatally. The median age of surgery was 6 days, the weight was (3.2±0.5) kg. The balloon/valve ratio was 1.19±0.12, and the RV pressure measured by catheter was (121±33) mmHg (1 mmHg=0.133 kPa) . The immediate postoperative RV pressure was (47±13) mmHg. The median follow-up time was 30 months. All the cases enrolled achieved biventricular circulation without death and serious complications. According to the last follow-up data including 16 cases who were followed up for 1 year or longer, the pulmonary artery transvalvular pressure was (29±15) mmHg. The postoperation ratio of right to left ventricular transverse diameter was significantly higher than that before operation (0.86+0.10 vs. 0.73+0.13, t=-2.96, P=0.006). Compared with preoperative data, the postoperation pulmonary valvular diameter Z-score was significantly higher (-1.41±0.89 vs. -2.83±1.06, t=-3.65, P=0.001) and the tricuspid valvular diameter Z-score was significantly higher (-0.52±0.29 vs. -1.34±0.81, t=-3.55, P=0.001). Conclusion: Transcatheter perforation of pulmonary valve by micro-guidewire and balloon dilation is a safe and effective initial therapy for neonatal PA-IVS.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Cateterismo/instrumentação , Cardiopatias Congênitas/cirurgia , Septos Cardíacos/cirurgia , Atresia Pulmonar/cirurgia , Ablação por Cateter , Cateterismo/métodos , Dilatação , Feminino , Humanos , Recém-Nascido , Masculino , Valva Pulmonar/cirurgia , Estudos Retrospectivos , Substituição da Valva Aórtica Transcateter , Resultado do Tratamento
10.
J Comput Assist Tomogr ; 43(6): 906-911, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31738213

RESUMO

The aim of this article was to review computed tomography angiography and magnetic resonance angiography of pulmonary atresia with ventricular septal defect. This disorder is a rare complex congenital heart disease. Preoperative imaging of pulmonary atresia with ventricular septal defect with computed tomography angiography and magnetic resonance angiography is important for complete anatomical delineation and planning for treatment. Preoperative imaging used for assessment of the main pulmonary artery (its size, valve, and confluence), aortopulmonary collaterals (its origin, insertion, course, and size), presence of patent ductus arteriosus, other sources of collaterals as bronchial and coronary arteries, and pattern of pulmonary arborization. Imaging can detect associated aortic, pulmonary venous and coronary anomalies, and other congenital heart disease. Postoperative imaging after unifocalization and stent is for assessment of patency, stenosis, and occlusion of stent or perivascular lesions as seroma.


Assuntos
Defeitos dos Septos Cardíacos/diagnóstico por imagem , Defeitos dos Septos Cardíacos/cirurgia , Atresia Pulmonar/diagnóstico por imagem , Atresia Pulmonar/cirurgia , Interpretação de Imagem Radiográfica Assistida por Computador/métodos , Cateterismo Cardíaco , Procedimentos Cirúrgicos Cardíacos , Angiografia por Tomografia Computadorizada , Humanos , Angiografia por Ressonância Magnética , Período Pós-Operatório , Período Pré-Operatório , Procedimentos Cirúrgicos Pulmonares
11.
Anatol J Cardiol ; 22(3): 125-131, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31475955

RESUMO

OBJECTIVE: This study aims to present our experience with single-stage complete unifocalization and intraoperative flow study for the repair of ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. METHODS: This study was conducted through retrospective chart review of all the patients who underwent complete single-stage midline unifocalization in a single tertiary-care institution. RESULTS: Twenty-two patients underwent midline single-stage unifocalization. The median age was 11 months (IQR: 5-21 months). The number of collateral arteries unifocalized was between one and three (median two). In-hospital mortality was 5%. Follow-up was complete; and the median follow-up regarding survival was 20 months (IQR: 10-28 months). There were three late deaths, and the estimated survival rate was 80% at 10 months and on. Out of 22 patients, ventricular septal defect was closed in the first surgery in three patients (14%) and the second surgery in four patients (19%). Total seven patients underwent surgical total repair (32%). Additionally, one out of four patients whose ventricular septal defects were closed with a fenestrated patch is under follow-up with a small ventricular septal defect, while two are waiting for ventricular septal defect closure. Therefore, total eight patients (36%) have reached total correction. CONCLUSION: Single-stage unifocalization is a feasible treatment option in ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. This cohort had unfavorable results regarding the rate of complete repair. The pitfalls encountered were related to problems with meticulous surgical technique, complete unifocalization, and correct implementation of the flow study.


Assuntos
Circulação Colateral/fisiologia , Defeitos dos Septos Cardíacos/cirurgia , Artéria Pulmonar/anormalidades , Atresia Pulmonar/cirurgia , Velocidade do Fluxo Sanguíneo , Procedimentos Cirúrgicos Cardíacos , Feminino , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/cirurgia , Defeitos dos Septos Cardíacos/complicações , Defeitos dos Septos Cardíacos/mortalidade , Humanos , Lactente , Período Intraoperatório , Masculino , Registros Médicos , Atresia Pulmonar/complicações , Atresia Pulmonar/mortalidade , Fluxo Pulsátil , Estudos Retrospectivos , Análise de Sobrevida , Turquia/epidemiologia , Procedimentos Cirúrgicos Vasculares
12.
World J Pediatr Congenit Heart Surg ; 10(5): 628-631, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31496407

RESUMO

The Fontan operation for children with congenital heart disease places them in a physiological state of mildly elevated systemic venous pressure and low-normal cardiac output. Consequently, close follow-up is imperative, yet currently no method is available to obtain Fontan pressures without direct measurements in the cardiac catheterization laboratory while supine and sedated. We hypothesize that by suturing the CardioMEMS sensor device into the Fontan conduit during the standard Fontan operation for a child with single ventricular physiology, clinicians can accurately retrieve Fontan pathway pressure measurements noninvasively during normal physiological states.


Assuntos
Técnica de Fontan , Cardiopatias Congênitas/cirurgia , Monitorização Ambulatorial/instrumentação , Atresia Pulmonar/cirurgia , Cateterismo Cardíaco , Pré-Escolar , Desenho de Equipamento , Humanos , Masculino , Complicações Pós-Operatórias/diagnóstico , Período Pós-Operatório , Pressão , Função Ventricular
13.
World J Pediatr Congenit Heart Surg ; 10(5): 558-564, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31496414

RESUMO

PURPOSE: Children with congenital heart disease may present with severe airway compression prior to any surgical procedure or may develop airway compression following their surgical procedure. This combination of congenital heart defect and airway compression poses a significant management challenge. The purpose of this study was to review our experience with the Lecompte procedure for relief of severe airway compression. METHODS: This was a retrospective review of ten patients who underwent a Lecompte procedure for relief of severe airway compression over the past nine years (2010-2018). Three patients with absent pulmonary valve syndrome presented with severe symptoms prior to any surgical procedure. Seven patients presented with symptoms of airway compression following repair of their congenital heart defects (one with absent pulmonary valve syndrome, three patients had repair of pulmonary atresia with ventricular septal defect, and three patients had undergone aortic arch surgery). The median age at presentation was two years (range: one day to seven years). RESULTS: The ten patients underwent a Lecompte procedure without any significant complications or operative mortality. The median interval between the surgical procedure and extubation was 9.5 days. No patients have required any further interventions for relief of airway obstruction. CONCLUSIONS: The Lecompte procedure is a surgical option for young children who present with severe airway compression. The patients in this series responded well to the Lecompte procedure as evidenced by clinical relief of airway compression.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Cardiopatias Congênitas/cirurgia , Comunicação Interventricular/cirurgia , Atresia Pulmonar/cirurgia , Obstrução das Vias Respiratórias , Aorta Torácica/anormalidades , Aorta Torácica/cirurgia , Brônquios/patologia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Período Pós-Operatório , Artéria Pulmonar/anormalidades , Atelectasia Pulmonar/patologia , Valva Pulmonar/cirurgia , Estudos Retrospectivos , Síndrome , Traqueia/patologia , Resultado do Tratamento
14.
Kyobu Geka ; 72(7): 560-563, 2019 Jul.
Artigo em Japonês | MEDLINE | ID: mdl-31296807

RESUMO

Unobstructed atrial communication is a prerequisite for favorable pulmonary circulation in patients with single-ventricle physiology and atrial-communication-dependent pulmonary venous drainage. It might also be a prerequisite for favorable coronary circulation in patients with single-ventricle physiology and atrial-communication-dependent coronary venous drainage. Obstruction of atrial communication, on which coronary venous drainage depends, has not yet been documented. We report a patient with pulmonary atresia and an intact ventricular septum having an extracardiac total cavopulmonary connection. Spontaneous closure of the atrial septal defect( ASD), measuring 10 mm before the total cavopulmonary connection, led to a leftward ventricular septal shift and left ventricular wall thickening, causing severe left ventricular diastolic dysfunction which resolved after surgical ASD creation. Detection of spontaneous ASD closure by ultrasound was challenging because the to-and-fro blood flow in the dilated coronary sinus mimicked a patent ASD. Prophylactic ASD enlargement accompanying the cavopulmonary connection procedure is advised in such patients.


Assuntos
Cardiopatias Congênitas , Insuficiência Cardíaca , Comunicação Interatrial , Atresia Pulmonar , Septo Interventricular , Criança , Comunicação Interatrial/complicações , Comunicação Interatrial/cirurgia , Humanos , Atresia Pulmonar/complicações , Atresia Pulmonar/cirurgia
15.
Congenit Heart Dis ; 14(5): 814-818, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31290594

RESUMO

BACKGROUND AND OBJECTIVE: Chronic total occlusion (CTO) guidewire have been recently reported as an alternative to radiofrequency for perforating atretic pulmonary valve. Since procedure failures or perforation of the right ventricle still occurred with CTO, we tried to enhance the stability, steering, and pushability of the wire using a microcatheter in order to improve the safety and efficacy of the procedure. METHODS: We performed pulmonary valve perforation with CTO guidewire and microcatheter in five consecutive newborns with pulmonary atresia with intact ventricular septum (PA-IVS) under fluoroscopic and echocardiographic control. RESULTS: The valve was easily perforated at the first attempt for all patients. After perforation, the microcatheter positioned in the main pulmonary artery allowed the exchange of the CTO guidewire for a more flexible wire, avoiding lesion and facilitating manipulation in the distal pulmonary branch arteries. The pulmonary valve was then dilated with balloons of increasing size as usually performed. We did not experience any procedural or early complications. Blalock-Taussig shunt was performed in 2 children because of a persistent cyanosis, 4 and 10 days after perforation. CONCLUSIONS: The combined use of a CTO guide and a microcatheter appears to be a safe and reliable technique for perforating the pulmonary valve of newborns with PA-IVS. Further procedures with this approach are needed to confirm this first experience.


Assuntos
Cateterismo Cardíaco/instrumentação , Procedimentos Cirúrgicos Cardíacos/métodos , Valva Pulmonar/cirurgia , Cirurgia Assistida por Computador/métodos , Angiografia , Ecocardiografia , Desenho de Equipamento , Feminino , Fluoroscopia , Seguimentos , Humanos , Recém-Nascido , Masculino , Miniaturização , Atresia Pulmonar/diagnóstico , Atresia Pulmonar/cirurgia , Valva Pulmonar/diagnóstico por imagem , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
16.
Cardiol Young ; 29(8): 1078-1081, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31288878

RESUMO

BACKGROUND: There are limited outcome data in adults with tetralogy of Fallot and pulmonary atresia. The purpose of this study was to describe re-operations and all-cause mortality in adults with tetralogy of Fallot and pulmonary atresia. METHODS: Retrospective review of adults with repaired tetralogy of Fallot and pulmonary atresia who received care at the Mayo Adult Congenital Heart Disease Clinic, 1990-2016. All-cause mortality was calculated as events per 100 patient-years from the time of first presentation to the Adult Congenital Heart Disease Clinic. RESULTS: Of the 221 patients, the age at initial tetralogy of Fallot repair was 6 (5-13) years, and the age at first presentation to the clinic was 27 - 8 years. All patients had at least one right ventricular to pulmonary artery conduit re-operation. There were 31 deaths (14%) at mean age of 41 - 14 years. The causes of death were end-stage heart failure (n = 17), sudden cardiac death (n=9), post-operative death after cardiac surgery (n = 2), sepsis with multi-system organ failure (n = 2), and unknown (n = 1). All-cause mortality rate was 1.7 per 100 patient-years. The risk factors for all-cause mortality were older age (>12 years) at the time of repair (hazard ratio 1.41, 95 confidence interval 1.06-2.02, p = 0.033), non-sustained ventricular tachycardia (hazard ratio 1.36, 95 confidence interval 1.17-2.47, p = 0.015), and left ventricular ejection fraction <50% (hazard ratio 1.39, 95 confidence interval 1.08-2.31, p = 0.031). CONCLUSION: Based on a review of 221 adults with repaired tetralogy of Fallot and pulmonary atresia, all patients had re-operations and all-cause mortality rate was 1.7 events per 100 patient-years. The current study provides important outcomes data for risk stratification in adults with tetralogy of Fallot and pulmonary atresia.


Assuntos
Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Atresia Pulmonar/cirurgia , Tetralogia de Fallot/cirurgia , Adulto , Procedimentos Cirúrgicos Cardíacos/mortalidade , Causas de Morte , Feminino , Humanos , Masculino , Minnesota/epidemiologia , Artéria Pulmonar/cirurgia , Atresia Pulmonar/mortalidade , Reoperação/estatística & dados numéricos , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Tetralogia de Fallot/mortalidade , Fatores de Tempo , Resultado do Tratamento , Função Ventricular Esquerda , Adulto Jovem
17.
Kyobu Geka ; 72(4): 263-268, 2019 Apr.
Artigo em Japonês | MEDLINE | ID: mdl-31266911

RESUMO

Pulmonary atresia, ventricular septal defect, and major aorto-pulmonary collateral arteries are deemed unrepairable in some patients. Reportedly, 39% to 63% of patients treated by the staged unifocalization pathway, and 25% to 51% of patients treated by the pulmonary artery rehabilitation strategy were deemed unsuitable to undergo total repair. Single-stage complete unifocalization with intracardiac repair can be performed in some older patients with pulmonary atresia, ventricular septal defect, and major aorto-pulmonary collateral arteries that were previously deemed unrepairable. We report four such patients with ages between 12 years and 28 years at surgery. Bosentan hydrate was administered pre-operatively in one female patient for 14 months to ameliorate regional pulmonary hypertension. Seventeen months after repair, she delivered a baby by cesarean section. One patient underwent aortic valve replacement concomitant with repair due to severe aortic regurgitation caused by healed infective endocarditis. All patients were discharged home 9 to 18 days after surgery. After 72 to 100 months of follow-up, three patients are in New York Heart Association (NYHA) class Ⅰ, and the remaining patient are in NYHA class Ⅱ. Single-stage complete unifocalization can allow total repair in adolescents and adults with pulmonary atresia, ventricular septal defect, and major aorto-pulmonary collateral arteries that were previously deemed unrepairable.


Assuntos
Cardiopatias Congênitas , Comunicação Interventricular , Atresia Pulmonar , Adolescente , Adulto , Cesárea , Criança , Circulação Colateral , Feminino , Cardiopatias Congênitas/cirurgia , Comunicação Interventricular/cirurgia , Humanos , Gravidez , Artéria Pulmonar , Atresia Pulmonar/cirurgia , Adulto Jovem
18.
Cardiol Young ; 29(7): 977-979, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31204635

RESUMO

In patients with CHD after Fontan palliation, there is a lack of a pumping chamber in the pulmonary circulation; thus, pulmonary blood flow and cardiac output are sensitive to increased pulmonary vascular resistance; if obstructive sleep apnoea is present, there are legitimate concerns from continuous positive airway pressure ventilation, which may hinder pulmonary blood flow.


Assuntos
Pressão Positiva Contínua nas Vias Aéreas , Técnica de Fontan , Cardiopatias Congênitas/cirurgia , Atresia Pulmonar/cirurgia , Apneia Obstrutiva do Sono/terapia , Adulto , Cardiopatias Congênitas/complicações , Humanos , Masculino , Atresia Pulmonar/complicações , Apneia Obstrutiva do Sono/diagnóstico , Apneia Obstrutiva do Sono/etiologia
19.
J Cardiothorac Surg ; 14(1): 99, 2019 Jun 03.
Artigo em Inglês | MEDLINE | ID: mdl-31159878

RESUMO

BACKGROUND: There is great discussion about non-confluent pulmonary artery (PA) reconstruction, and several materials have been used. Autologous pericardium is considered feasible for infectious resistance, autoimmune response, extensibility, and growth potential. CASE PRESENTATION: The patient was born at 39 weeks (body mass = 2550 g). He was diagnosed with tetralogy of Fallot, pulmonary atresia, non-confluent PA, and bilateral patent ductus arteriosus. Right and left Blalock-Taussig shunts with patent ductus arteriosus ligations were placed on day 27 and 3 months, respectively. At 19 months (8.8 kg), definitive repair was performed with tricuspid valved conduit concurrent with PA reconstruction using an autologous pericardium roll conduit. The autologous pericardium was treated with glutaraldehyde (autologous pericardium fixed with 0.4% glutaraldehyde for 7 min and rolled as conduit - 12 mm in diameter and 30 mm in length). Following an incision on the visceral side of the PAs before the 1st branch, the autologous pericardial roll conduit was anastomosed. Follow-up angiographies on postoperative months 9 and 57 demonstrated that the PA, including the autologous pericardium roll conduit, had spontaneously enlarged. CONCLUSION: Particularly for non-confluent PA, the patients require increased pulmonary beds at an early age because of hypoplastic PA. While size mismatch between the graft and native PA develops as the child grows, size-adjustable extensibility of the PA graft should be noted.


Assuntos
Pericárdio/transplante , Artéria Pulmonar/cirurgia , Procedimentos Cirúrgicos Reconstrutivos/métodos , Procedimentos Cirúrgicos Vasculares/métodos , Anormalidades Múltiplas/cirurgia , Pré-Escolar , Permeabilidade do Canal Arterial/complicações , Permeabilidade do Canal Arterial/cirurgia , Humanos , Lactente , Masculino , Artéria Pulmonar/anormalidades , Atresia Pulmonar/complicações , Atresia Pulmonar/cirurgia , Tetralogia de Fallot/complicações , Tetralogia de Fallot/cirurgia , Transplante Autólogo
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