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1.
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
2.
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
3.
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
4.
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
5.
Cardiol Young ; 29(5): 610-614, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31044684

RESUMO

BACKGROUND: Children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals (TOF/MAPCAs) are at risk for post-operative respiratory complications after undergoing unifocalisation surgery. Thus, we assessed and further defined the incidence of airway abnormalities in our series of over 500 children with TOF/MAPCAs as determined by direct laryngoscopy, chest computed tomography (CT), and/or bronchoscopy. METHODS: The medical records of all patients with TOF/MAPCAs who underwent unifocalisation or pulmonary artery reconstruction surgery from March, 2002 to June, 2018 were reviewed. Anaesthesia records, peri-operative bronchoscopy, and/or chest CT reports were reviewed to assess for diagnoses of abnormal or difficult airway. Associations between chromosomal anomalies and airway abnormalities - difficult anaesthetic airway, bronchoscopy, and/or CT findings - were defined. RESULTS: Of the 564 patients with TOF/MAPCAs who underwent unifocalisation or pulmonary artery reconstruction surgery at our institution, 211 (37%) had a documented chromosome 22q11 microdeletion and 28 (5%) had a difficult airway/intubation reported at the time of surgery. Chest CT and/or peri-operative bronchoscopy were performed in 234 (41%) of these patients. Abnormalities related to malacia or compression were common. In total 35 patients had both CT and bronchoscopy within 3 months of each other, with concordant findings in 32 (91%) and partially concordant findings in the other 3. CONCLUSION: This is the largest series of detailed airway findings (direct laryngoscopy, CT, and bronchoscopy) in TOF/MAPCAS patients. Although these findings are specific to an at-risk population for airway abnormalities, they support the utility of CT and /or bronchoscopy in detecting airway abnormalities in patients with TOF/MAPCAs.


Assuntos
Anormalidades Múltiplas/cirurgia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Artéria Pulmonar/cirurgia , Atresia Pulmonar/cirurgia , Insuficiência Respiratória/epidemiologia , Tetralogia de Fallot/cirurgia , Broncoscopia , California/epidemiologia , Circulação Colateral , Feminino , Humanos , Lactente , Laringoscopia , Pulmão/irrigação sanguínea , Masculino , Complicações Pós-Operatórias , Artéria Pulmonar/anormalidades , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
6.
Gen Thorac Cardiovasc Surg ; 67(11): 941-948, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31134530

RESUMO

OBJECTIVES: To evaluate the clinical outcomes of surgical repair of an extracardiac total anomalous pulmonary venous connection (TAPVC) in a functional single-ventricle (f-SV) strategy. METHODS: This was a retrospective analysis of 48 consecutive cases of extracardiac TAPVC repair, from 1998 to 2015. Demographic and clinical variables were as follows: median age, 24 (range 0-744) days; median weight, 3.1 (range 2.0-9.6) kg; type of TAPVC-supracardiac, 21 patients, infracardiac, 8, and mixed, 19; right atrial isomerism, 45 patients; pulmonary atresia, 24 patients; and obstructed TAPVC, 30 patients. Concomitant procedures included systemic-to-pulmonary shunting in 15 patients, pulmonary artery banding in 12, ventricle-to-pulmonary artery shunting in 2, a Norwood procedure in one, a bidirectional Glenn procedure in 16, and a Fontan procedure in 1. RESULTS: The 1- and 5-year cumulative survival rates were 66.0% and 58.0%, respectively. Of the 28 survivors, 22 (78.6%) underwent Fontan completion and 4 (14.3%) a bidirectional Glenn procedure, and 1 (3.6%) was awaiting a bidirectional Glenn procedure. Recurrent pulmonary venous stenosis (PVS) was observed in 17 patients, with a 1- and 5-year rates of freedom from recurrent PVS 59.8% and 53.5%, respectively. Of the 20 post-operative deaths, only 4 were PVS related. Only pre-operative pulmonary atresia was identified as an independent risk factor of mortality after TAPVC repair. CONCLUSIONS: The midterm surgical outcomes of f-SV with extracardiac TAPVC were acceptable. Moreover, among survivors, Fontan completion can be sufficiently expected. Further improvement, with the development of a comprehensive treatment strategy, is required for this patient group.


Assuntos
Procedimentos Cirúrgicos Cardiovasculares/métodos , Síndrome de Heterotaxia/cirurgia , Atresia Pulmonar/cirurgia , Veias Pulmonares/cirurgia , Síndrome de Cimitarra/cirurgia , Pré-Escolar , Feminino , Técnica de Fontan , Humanos , Lactente , Recém-Nascido , Masculino , Procedimentos de Norwood , Artéria Pulmonar/anormalidades , Artéria Pulmonar/cirurgia , Veias Pulmonares/anormalidades , Recidiva , Estudos Retrospectivos , Fatores de Risco , Estenose de Veia Pulmonar/etiologia , Taxa de Sobrevida , Fatores de Tempo , /cirurgia
7.
Semin Thorac Cardiovasc Surg ; 31(4): 837-844, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31136797

RESUMO

Management strategy for patients of ventricular septal defect and pulmonary atresia (VSD/PA) with hypoplastic pulmonary arteries presenting in late childhood or adolescence is still controversial. We present our experience with the use of right ventricle-pulmonary artery shunt (RV-PA) in management of this entity. Between January 2014 and April 2018, 25 patients of VSD/PA underwent valveless RV-PA shunt at our center. The size of the RV to PA shunt was calculated as half the expected diameter of the main pulmonary artery. We retrospectively reviewed the data from hospital records. Follow-up data were recorded from outpatient records or via telephone. Mean age of the cohort was 12.25 ± 3.18 years. There was 1 early and 1 interstage mortality. None of the patient developed acute renal failure, ventricular dysfunction, and arrhythmias. At interstage follow-up of 8.28 ± 3.7 months, both Nakata index (from 66.23 ± 24.12 to 185.8 ± 58 mm2/m2) and McGoon ratio (0.9 ± 0.22 vs 49 1.84 ± 0.4) increased significantly compared to preoperative value, whereas RPA-LPA ratio was not significantly changed (1.095 ± 0.39 vs 1.01 ± 0.56, P = 0.63). Prerepair pulmonary vascular resistance in 17 patients, who underwent complete repair, was 2.9 ± 0.69 woods unit/m2. Postrepair right ventricle-left ventricle pressure ratio was 0.5 ± 0.14. There was no early or late mortality and none of the patient required conduit revision or VSD fenestration. On follow-up of 25.75 ± 17.94 months, 16 patients were in NYHA I and 1 patient was in NYHA II. Appropriate-sized RV-PA shunt is an effective strategy for achieving balanced pulmonary artery growth in VSD/PA with hypoplastic pulmonary arteries presenting late without the risk of pulmonary over circulation or systemic malperfusion.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Comunicação Interventricular/cirurgia , Artéria Pulmonar/cirurgia , Atresia Pulmonar/cirurgia , Adolescente , Fatores Etários , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Criança , Circulação Coronária , Feminino , Comunicação Interventricular/diagnóstico por imagem , Comunicação Interventricular/fisiopatologia , Hemodinâmica , Humanos , Masculino , Artéria Pulmonar/anormalidades , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/fisiopatologia , Atresia Pulmonar/diagnóstico por imagem , Atresia Pulmonar/fisiopatologia , Circulação Pulmonar , Recuperação de Função Fisiológica , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Função Ventricular Direita , Adulto Jovem
8.
Ann Thorac Surg ; 108(3): 820-827, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-30980823

RESUMO

BACKGROUND: Our institutional approach to tetralogy of Fallot with major aortopulmonary collateral arteries (MAPCAs) emphasizes early unifocalization and complete repair (CR). In the small subset of patients with dual-supply MAPCAs and confluent but hypoplastic central pulmonary arteries (PAs), our surgical approach is early creation of an aortopulmonary window (APW) to promote PA growth. Factors associated with successful progression to CR and mid-term outcomes have not been assessed. METHODS: Clinical data were reviewed. PA diameters were measured offline from angiograms prior to APW and on follow-up catheterization >1 month after APW but prior to any additional surgical interventions. RESULTS: From November 2001 to March 2018, 352 patients with tetralogy of Fallot/MAPCAs underwent initial surgery at our center, 40 of whom had a simple APW with or without ligation of MAPCAs as the first procedure (median age, 1.4 months). All PA diameters increased significantly on follow-up angiography. Ultimately, 35 patients underwent CR after APW. Nine of these patients (26%) underwent intermediate palliative operation between 5 and 39 months (median, 8 months) after APW. There were no early deaths. The cumulative incidence of CR was 65% 1 year post-APW and 87% at 3 years. Repaired patients were followed for a median of 4.2 years after repair; the median PA:aortic pressure ratio was 0.39 (range, 0.22 to 0.74). CONCLUSIONS: Most patients with tetralogy of Fallot/MAPCAs and hypoplastic but normally arborizing PAs and dual-supply MAPCAs are able to undergo CR with low right ventricular pressure after APW early in life. Long-term outcomes were good, with acceptable PA pressures in most patients.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Angiografia por Tomografia Computadorizada/métodos , Imagem Tridimensional , Atresia Pulmonar/cirurgia , Tetralogia de Fallot/cirurgia , Anormalidades Múltiplas/diagnóstico por imagem , Anormalidades Múltiplas/cirurgia , Defeito do Septo Aortopulmonar/diagnóstico por imagem , Defeito do Septo Aortopulmonar/cirurgia , Estudos de Coortes , Circulação Colateral/fisiologia , Bases de Dados Factuais , Feminino , Seguimentos , Hospitais Pediátricos , Humanos , Recém-Nascido , Masculino , Atresia Pulmonar/diagnóstico por imagem , Atresia Pulmonar/mortalidade , Estudos Retrospectivos , Medição de Risco , Análise de Sobrevida , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/mortalidade , Fatores de Tempo , Resultado do Tratamento
9.
Artigo em Inglês | MEDLINE | ID: mdl-31027561

RESUMO

PA/IVS is a rare, heterogenous congenital heart defect anatomically defined by complete obstruction to the right ventricular outflow tract with varying degrees of hypoplasia of the right ventricle and tricuspid valve. This lesion can have associated coronary artery anomalies and, in some cases, right ventricular-dependent coronary circulation. Due to the wide spectrum of presenting anatomic and clinical features, the treatment options are often dictated by the degree of development of the tricuspid valve and right ventricle. The purpose of this review is to discuss the spectrum of pulmonary atresia with intact ventricular septum morphologies and to evaluate the surgical decision-making process and approaches to surgical repair with respect to the impact of hypoplastic right-sided cardiac features.


Assuntos
Tomada de Decisão Clínica , Cardiopatias Congênitas/cirurgia , Seleção de Pacientes , Atresia Pulmonar/cirurgia , Valva Tricúspide/anormalidades , Humanos , Recém-Nascido
10.
Cardiol Young ; 29(4): 534-537, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30968796

RESUMO

We describe percutaneous repair of severe pulmonary regurgitation and a right ventricular outflow tract pseudoaneurysm in a 19-year-old patient after repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals. A 3D printed model of his heart was used to simulate percutaneous repair with a closure device in the aneurysm neck and a Venus P-valve in the right ventricular outflow tract. The encouraging findings from the simulation allowed us to plan the complex procedure effectively with a successful outcome and avoidance of surgery.


Assuntos
Falso Aneurisma/cirurgia , Modelos Anatômicos , Impressão Tridimensional , Insuficiência da Valva Pulmonar/cirurgia , Obstrução do Fluxo Ventricular Externo/cirurgia , Falso Aneurisma/etiologia , Aorta Torácica/cirurgia , Procedimentos Cirúrgicos Cardíacos , Circulação Colateral , Angiografia Coronária , Comunicação Interventricular/complicações , Comunicação Interventricular/cirurgia , Implante de Prótese de Valva Cardíaca , Humanos , Imagem por Ressonância Magnética , Masculino , Atresia Pulmonar/complicações , Atresia Pulmonar/cirurgia , Insuficiência da Valva Pulmonar/etiologia , Obstrução do Fluxo Ventricular Externo/etiologia , Adulto Jovem
11.
Ann Thorac Surg ; 108(1): 154-159, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-30928554

RESUMO

BACKGROUND: Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (MAPCAs) is a rare form of congenital heart disease characterized by the entirety of pulmonary blood flow originating from systemic vessels. This study measured the residual collateral flow after harvesting of the MAPCAs for surgical repair. METHODS: The study enrolled 32 patients with pulmonary atresia with ventricular septal defect and MAPCAs who were undergoing their first surgical procedure. The median age was 6.8 months, and median weight was 5.7 kg. The patients had a mean of 4.2 ± 0.7 MAPCAs. The cardiopulmonary bypass circuit was modified to contain a diversion loop in the left ventricular vent system to accurately measure residual collateral flow. During the period of aortic cross-clamp (for ventricular septal defect repair), the diversion loop was opened for 1-minute intervals, and the residual collateral flow collected. The systemic perfusion temperature was 25° and flow rate was 100 mL · kg-1 · min-1. RESULTS: The mean residual collateral flow was 5.5 mL · kg-1 · min-1 (range, 0.8 to 15.2 mL · kg-1 · min-1). The corresponding calculated pulmonary blood flow-to-systemic blood flow ratio values ranged from 1.01 to 1.36. There was a significant correlation between residual collateral flow and preoperative saturation (p < 0.05). CONCLUSIONS: The data demonstrate a wide range of residual collateral flow values after harvesting of the MAPCAs. The amount of residual collateral flow was correlated with preoperative saturation. These results suggest that some patients at the higher end of this spectrum may require adjustments in pump flow to assure adequate systemic perfusion.


Assuntos
Circulação Colateral/fisiologia , Defeitos dos Septos Cardíacos/fisiopatologia , Atresia Pulmonar/fisiopatologia , Aorta/anormalidades , Aorta/diagnóstico por imagem , Aortografia , Defeitos dos Septos Cardíacos/cirurgia , Humanos , Lactente , Oxigênio/sangue , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/fisiopatologia , Artéria Pulmonar/cirurgia , Atresia Pulmonar/cirurgia , Veias Pulmonares/diagnóstico por imagem , Veias Pulmonares/fisiopatologia
12.
Ann Thorac Surg ; 108(2): 601-612, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-30831109

RESUMO

BACKGROUND: The management of pulmonary atresia with ventricular septal defect (PA/VSD) and major aortopulmonary collateral arteries (MAPCAs) has significantly changed over the past 20 years. Unifocalization and rehabilitation have been described as diametrically opposed strategies. An updated review focused on the management of this complex and rare condition is needed. METHODS: Articles related to PA/VSD/MAPCAs issued until December 2017 were screened. Twelve main studies published in the modern era (since 2000) were selected and analyzed. RESULTS: Unifocalization and rehabilitation respectively focus on the mobilization of collateral arteries and the growth of native pulmonary vessels. A third strategy, called "combined strategy," was distinguished from the review of the literature. Surgical cohorts and methods of data reporting were found to be heterogenous. Outcomes, regardless of the strategy, have transformed the natural history of the condition, with a complete repair rate of approximately 80% and low rates of early and late mortality. Patients with the most unfavorable anatomy (absent central pulmonary arteries and hypoplastic MAPCAs) remain a challenge and are still left palliated. CONCLUSIONS: Variable surgical strategies are used in the management of PA/VSD/MAPCAs. Most teams report a repair rate of 70% to 80% and a mortality rate lower than 10%. Standardization in data reporting is necessary to better compare the existing strategies.


Assuntos
Anormalidades Múltiplas/cirurgia , Aorta Torácica/anormalidades , Procedimentos Cirúrgicos Cardíacos/métodos , Circulação Colateral , Defeitos dos Septos Cardíacos/cirurgia , Artéria Pulmonar/anormalidades , Atresia Pulmonar/cirurgia , Malformações Vasculares/cirurgia , Gerenciamento Clínico , Humanos
13.
Cardiol Young ; 29(3): 375-379, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30724146

RESUMO

IntroductionPulmonary atresia, ventricular septal defect, major aorto-pulmonary collateral arteries, and pulmonary arteries hypoplasia are rare and complex congenital defects that require early interventions to relieve cyanosis and enhance the growth of native pulmonary arteries. The treatment of these patients is still controversial. Surgical techniques require cardiopulmonary bypass which is poorly tolerated by small infants. Percutaneous techniques such as radiofrequency perforation can be challenging. The hybrid technique consists of perventricular stenting of the right ventricle outflow tract through medial sternotomy, to restore native pulmonary flow. METHODS: We retrospectively reviewed the cardiovascular database of our centre in order to analyse our experience in hybrid procedure. We detected six patients who underwent hybrid first approach between November 2007 and December 2015. We report our early results and mid-term outcomes. RESULTS: Median age at the procedure was 26 days, median weight was 3150 g, and median Nakata index was 52 mm2/m2. All procedures were successful except for one: this patient underwent a surgical shunt. No immediate and early deaths or major complications occurred and oxygen saturation levels increased in all the patients. Patients were followed up for a period of 12-103 months, and four of them underwent a procedure of unifocalisation at the mean age of 12.5 months. CONCLUSIONS: We reported data from the largest series of patients who underwent this hybrid procedure. Our experience demonstrated encouraging results to expand the use of this approach to bridge high-risk patients with diminutive pulmonary arteries to a second step of surgical repair.


Assuntos
Anormalidades Múltiplas , Procedimentos Cirúrgicos Cardíacos/métodos , Comunicação Interventricular/cirurgia , Ventrículos do Coração/cirurgia , Artéria Pulmonar/cirurgia , Atresia Pulmonar/cirurgia , Stents , Angiografia , Cateterismo Cardíaco/métodos , Feminino , Seguimentos , Comunicação Interventricular/diagnóstico , Humanos , Lactente , Recém-Nascido , Masculino , Atresia Pulmonar/diagnóstico , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
14.
Eur J Radiol ; 110: 88-96, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30599879

RESUMO

BACKGROUND: Coronary compression (CC) is a life threatening complication that can occur during percutaneous pulmonary valve implantation (PPVI). We describe our experience using cardiac CT prior to PPVI to identify patients at high CC-risk due to a close relationship between the coronary arteries and pulmonary trunk (PT). METHODS: A retrospective evaluation of candidates for PPVI who underwent CT prior to the procedure was done. Measurements of PT were performed using double oblique reconstructed images, with special attention to the stenotic tract of the PT. The analysis of coronary arteries included detection of anomalies of origin and course and assessment of their relationship with the PT, measuring the minimum distance between the coronary artery and the intended site of the future percutaneous valve implantation. RESULTS: CT analysis was performed for 52 patients. Thirty patients underwent PPVI after CT and 22 didn't. In 6/22 cases the reason not to receive a PPVI was high CC-risk detected at CT. In 6 other patients CT detected an intermediate CC-risk but the test balloon performed during angiography prior to valve placement was safe and the patients successfully underwent the procedure. None of the patients deemed as no CC-risk at CT had CC during PPVI. CONCLUSION: CT can detect patients with high and intermediate CC-risk and therefore may identify which patients are unlikely to undergo successful PPVI and those who need a careful analysis with balloon testing. CT can also rule out CC-risk identifying those patients in which balloon inflation testing could be omitted.


Assuntos
Estenose Coronária/diagnóstico por imagem , Implante de Prótese de Valva Cardíaca/efeitos adversos , Adolescente , Adulto , Cateterismo Cardíaco/métodos , Estenose Coronária/etiologia , Estenose Coronária/prevenção & controle , Vasos Coronários/diagnóstico por imagem , Feminino , Humanos , Processamento de Imagem Assistida por Computador , Masculino , Seleção de Pacientes , Intervenção Coronária Percutânea/efeitos adversos , Complicações Pós-Operatórias/diagnóstico por imagem , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/prevenção & controle , Cuidados Pré-Operatórios/métodos , Artéria Pulmonar/diagnóstico por imagem , Atresia Pulmonar/diagnóstico por imagem , Atresia Pulmonar/cirurgia , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/cirurgia , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Estudos Retrospectivos , Fatores de Risco , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Tomografia Computadorizada por Raios X , Resultado do Tratamento
15.
Pediatr Cardiol ; 40(2): 374-383, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30539241

RESUMO

There is paucity of long-term data on adult survivors after biventricular repair of pulmonary atresia with intact ventricular septum (PAIVS) and pulmonary stenosis (PS). This study aimed to determine the cardiac and non-cardiac outcomes of adult survivors after biventricular repair of PAIVS and PS. The cardiac, neurodevelopmental and liver problems of 111 adults, 40 with PAIVS and 71 with PS, were reviewed. The median follow-up duration of our patients was 26.5 years (range 14.8-55 years). The freedom from reintervention at 30 years was 17.4% and 73.3% for PAIVS and PS patients (p < 0.001), respectively. Compared with PS patients, PAIVS patients had significantly greater prevalence of right atrial and right ventricular (RV) dilatation, and moderate to severe tricuspid and pulmonary regurgitation (all p < 0.05), and cardiac arrhythmias (22.5% vs. 8.5%, p = 0.047). The freedom from development of cardiac arrhythmias at 30 years of 68.4% and 91.6%, respectively, in PAIVS and PS patients (p = 0.03). Cox proportional hazards model identified PAIVS as an independent risk factor for reintervention (HR 4.0, 95% CI 2.1-7.6, p < 0.001) and development of arrhythmias (HR 4.1, 95% CI 1.1-14.4, p = 0.03). Neurodevelopmental problems were found in 17.5% of PAIVS patients and 7.0% of PS patients (p = 0.11). Liver problems occurred in 2 (5%) PAIVS patients, both of whom required conversion to 1.5 ventricular repair. In conclusion, long-term problems, including the need for reinterventions, cardiac arrhythmias, RV dilation, pulmonary regurgitation, and neurodevelopmental and liver issues are more prevalent in adult PAIVS than PS survivors.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Cardiopatias Congênitas/cirurgia , Atresia Pulmonar/cirurgia , Estenose da Valva Pulmonar/cirurgia , Adulto , Arritmias Cardíacas/etiologia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Ecocardiografia/métodos , Feminino , Seguimentos , Cardiopatias Congênitas/complicações , Ventrículos do Coração/cirurgia , Humanos , Masculino , Atresia Pulmonar/complicações , Estenose da Valva Pulmonar/complicações , Reoperação/estatística & dados numéricos , Fatores de Risco , Sobreviventes , Resultado do Tratamento
16.
Int J Cardiol ; 280: 61-66, 2019 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-30477927

RESUMO

OBJECTIVES: To describe the characteristics and long-term outcome of a large adult cohort with pulmonary atresia. BACKGROUND: Patients with pulmonary atresia (PA) are a heterogeneous population in terms of anatomy, physiology and surgical history, and their management during adulthood remains challenging. METHODS: Data on all patients with PA followed in our center between January 2000 and March 2015 were recorded. Patients were classified into the following groups: PA with ventricular septal defect (PA-VSD, 1), PA with intact ventricular septum (PA-IVS, 2) and other miscellaneous PA (PA-other, 3). RESULTS: Two-hundred twenty-seven patients with PA were identified, 66.1% female, mean age 25.5 ±â€¯8.7 years. Over a median follow-up of 8.8 years, 49 (21.6%) patients had died: heart failure (n = 21, 42.8%) and sudden cardiac death (n = 8, 16.3%) were the main causes. There was no significant difference in mortality between the 3 Groups (p = 0.12) or between repaired and unrepaired patients in Group 1 (p = 0.16). Systemic ventricular dysfunction and resting oxygen saturations were the strongest predictors of mortality. Additionally, 116 (51%) patients were hospitalized, driven mainly by the need for invasive procedures, heart failure and arrhythmias. CONCLUSIONS: Adult survivors with pulmonary atresia have a high morbidity and mortality irrespective of underlying cardiac anatomy and previous reparative or palliative surgery. We present herewith predictors of outcome in adult life that may assist with their tertiary adult congenital care.


Assuntos
Hospitalização/tendências , Atresia Pulmonar/mortalidade , Atresia Pulmonar/cirurgia , Adolescente , Adulto , Feminino , Seguimentos , Humanos , Masculino , Morbidade , Mortalidade/tendências , Atresia Pulmonar/diagnóstico , Resultado do Tratamento , Adulto Jovem
17.
Asian Cardiovasc Thorac Ann ; 27(1): 11-17, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30417684

RESUMO

OBJECTIVE: Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is a complex form of congenital heart disease. Midline unifocalization has been developed for the surgical treatment of this condition. There are 3 outcome measures that determine long-term success: patients are alive, patients have achieved complete repair (i.e. ventricular septal defect closure), and patients have a relatively low right ventricle-to-aortic pressure ratio (<0.45). However, to date, no studies have combined these 3 outcome measures to analyze the likelihood of achieving an ideal outcome. METHODS: This was a retrospective review of 255 patients who underwent midline unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. The median age at unifocalization was 4.5 months. RESULTS: Two hundred thirty-five (92%) patients were alive at a mean follow-up of 5.3 years. Two hundred and seventeen (85%) patients underwent single-stage complete repair, and 38 (15%) had an initial unifocalization and shunt. Twenty-four of the 38 palliated patients have subsequently undergone repair. Thus 241 (94%) patients ultimately achieved complete repair. Of the 241 patients who were repaired, 219 (86%) had a right ventricle-to-aortic peak systolic pressure ratio <0.45. Combining these outcome measures, 77% of patients achieved an ideal outcome at one month, 73% at 6 months, 76% at one year, and 80% at 4 years. CONCLUSIONS: Most (80%) patients can achieve all 3 measures of favorable outcome at 4 years following midline unifocalization. We speculate that this will be a favorable portend for the future of these patients.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Defeitos dos Septos Cardíacos/cirurgia , Atresia Pulmonar/cirurgia , Aorta/anormalidades , Aorta/fisiopatologia , Aorta/cirurgia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/mortalidade , Circulação Colateral , Feminino , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Defeitos dos Septos Cardíacos/mortalidade , Defeitos dos Septos Cardíacos/fisiopatologia , Hemodinâmica , Humanos , Lactente , Masculino , Complicações Pós-Operatórias/mortalidade , Complicações Pós-Operatórias/fisiopatologia , Complicações Pós-Operatórias/cirurgia , Artéria Pulmonar/anormalidades , Artéria Pulmonar/fisiopatologia , Artéria Pulmonar/cirurgia , Atresia Pulmonar/diagnóstico por imagem , Atresia Pulmonar/mortalidade , Atresia Pulmonar/fisiopatologia , Circulação Pulmonar , Recuperação de Função Fisiológica , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Função Ventricular Direita
18.
Pediatr Cardiol ; 40(3): 526-536, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30353312

RESUMO

BACKGROUND: Patients with pulmonary atresia with intact ventricular septum and critical pulmonary stenosis (PAIVS/CPS) have wide variation in right ventricle (RV) size, systolic function, and diastolic function at birth. Establishment of antegrade pulmonary blood flow creates the potential for RV dilation from chronic pulmonary insufficiency. Future surgical decisions are based on RV size and function, largely supported by longitudinal studies of patients with Tetralogy of Fallot (TOF). Given potential differences in RV physiology and lack of similar data in PAIVS/CPS, the objective of this study was to determine differences in RV size, systolic function, and diastolic function between patients with PAIVS/CPS versus TOF. METHODS: We retrospectively collected cardiovascular magnetic resonance (CMR) data in 27 patients with PAIVS/CPS (ages 13.3 ± 8.8 years) and 78 with TOF (11.4 ± 5.4 years). RV volumes, ejection fraction (EF), regurgitant fraction, end-diastolic forward flow across the pulmonary valve, and right atrial cross-sectional area were calculated. RESULTS: There was no difference between the groups in RV end-diastolic volume (RVEDVi), RVEF, or pulmonary regurgitation. RVEF tended to decrease in TOF when RVEDVi exceeded 164 ml/m2. In PAIVS/CPS, RVEDVi less frequently reached 164 ml/m2 and was not associated with RVEF. There was worse RV diastolic dysfunction in PAIVS/CPS, with 1.5 times larger right atrial area and two times higher pulmonary end-diastolic forward flow (p < 0.0001). CONCLUSIONS: Patients with PAIVS/CPS have similar RV size, systolic function, and pulmonary regurgitation as TOF. However, impaired RV diastolic function may limit extremes of RV dilatation and impact long-term management of PAIVS/CPS.


Assuntos
Cardiopatias Congênitas/fisiopatologia , Ventrículos do Coração/fisiopatologia , Atresia Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/fisiopatologia , Tetralogia de Fallot/fisiopatologia , Função Ventricular Direita/fisiologia , Adolescente , Adulto , Procedimentos Cirúrgicos Cardíacos/métodos , Criança , Pré-Escolar , Estudos Transversais , Ecocardiografia/métodos , Feminino , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia , Humanos , Imagem Cinética por Ressonância Magnética/métodos , Masculino , Atresia Pulmonar/complicações , Atresia Pulmonar/cirurgia , Estenose da Valva Pulmonar/complicações , Estenose da Valva Pulmonar/cirurgia , Estudos Retrospectivos , Volume Sistólico , Tetralogia de Fallot/cirurgia , Resultado do Tratamento , Adulto Jovem
19.
Ann Thorac Surg ; 107(3): 844-851, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30365953

RESUMO

BACKGROUND: We hypothesized that reconstructed pulmonary artery (PA) size and postrepair PA pressure are associated with airway compression (AC) after complete unifocalization for pulmonary atresia, ventricular septal defects, and major aortopulmonary collateral arteries. METHODS: Complete unifocalization was performed in 48 consecutive patients between 2000 and 2016. Clinical course and outcome were reviewed, predictors for AC were identified by logistic regression, and the freedom from death was analyzed using Kaplan-Meier method. RESULTS: Postoperative respiratory distress occurred in 23 patients (48%), and AC occurred in 14 (29%). The median duration of follow-up was 3.7 years. AC was caused by central PA and aorta in 7, conduit in 3, and branch PA in 4. Surgical treatment was required in 5 patients (conduit downsizing, suspension of branch PA, conduit + aorta, branch PA + aorta, and aorta + trachea in 1 patient each). Three patients (21%) subsequently required airway stenting. Most (85.7%) of the AC occurred in patients with high right ventricular systolic pressure/left ventricular systolic pressure (>65%), large Nakata index (>200 mm2/m2), and large conduit index (>35 mm/m2). Patients with AC had significantly worse 3-year survival (no AC, 91.2%; AC, 64.2%; p = 0.01). Multivariate analysis identified higher right ventricular systolic pressure/left ventricular systolic pressure (p = 0.04), larger conduit index (p = 0.03), and Nakata index (p = 0.004) as predictors for AC. CONCLUSIONS: AC is a common cause of postoperative respiratory distress and tends to be associated with higher postrepair PA pressure, more frequent right ventricular dysfunction, and worse medium-term survival. The study underscores the importance of incorporating all available lung segments to achieve a low PA pressure, potentially preventing pathologic dilatation of the reconstructed PA. Management of patients with poor major aortopulmonary collateral arteries anatomy and physiology remains a challenge.


Assuntos
Anormalidades Múltiplas , Obstrução das Vias Respiratórias/etiologia , Aorta Torácica/anormalidades , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Circulação Colateral/fisiologia , Artéria Pulmonar/anormalidades , Atresia Pulmonar/cirurgia , Obstrução das Vias Respiratórias/diagnóstico , Anastomose Cirúrgica/efeitos adversos , Aorta Torácica/cirurgia , Broncoscopia , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Masculino , Complicações Pós-Operatórias , Artéria Pulmonar/cirurgia , Atresia Pulmonar/diagnóstico , Circulação Pulmonar/fisiologia , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
20.
Semin Thorac Cardiovasc Surg ; 31(2): 234-241, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30278269

RESUMO

A subset of neonates with single ventricle (SV) physiology has antegrade pulmonary blood flow that is deemed unlikely to be reliable until Glenn. We have used systemic to pulmonary shunt (SPS) with pulmonary artery banding (PAB) to optimize pulmonary blood flow while maintaining reserve antegrade flow. We hypothesize that this is an effective strategy that can be accomplished without the routine need for cardiopulmonary bypass. We retrospectively reviewed the records of 60 neonates who underwent combined SPS + PAB between 2004 and 2015. Data are presented as median with quartiles. Children were 8 (4-19) days old at surgery and included 38 (63%) boys. Atresia or severe stenosis of the subpulmonary atrioventricular (AV) valve associated with pulmonary blood flow across a bulboventricular foramen was present in 37 (62%). In 20 (33%), heterotaxy-associated unbalanced AV canal with pulmonary stenosis with or without anomalous pulmonary venous drainage was present. First-stage palliation was accomplished without cardiopulmonary bypass in 44 patients (73%). There were 7 (12%) hospital deaths, 4 among the 20 (20%) with heterotaxy. Fifty-three children were followed for a median 5.1 (1.8-8.2) years. Three early reinterventions were required after initial palliation (1 PAB adjustment, 2 SPS balloon angioplasties). Five additional heterotaxy patients experienced late mortality during follow-up. There were no early or emergent Glenn. Thirty-nine patients have reached Fontan circulation with a median pre-Fontan PA pressure of 14 (12-18) mm Hg. One patient converted to biventricular physiology and the remaining await completion Fontan. Heterotaxy was the only independent predictor of mortality (hazard ratio 10 (2.3-44, P < 0.001). In SV patients with unreliable antegrade PA flow, SPS + PAB is an effective first-stage palliation. SV patients with heterotaxy are at increased risk for mortality.


Assuntos
Técnica de Fontan , Ventrículos do Coração/cirurgia , Artéria Pulmonar/cirurgia , Atresia Pulmonar/cirurgia , Circulação Pulmonar , Estenose da Valva Pulmonar/cirurgia , Valva Pulmonar/cirurgia , Procedimentos Cirúrgicos Vasculares , Feminino , Técnica de Fontan/efeitos adversos , Técnica de Fontan/mortalidade , Ventrículos do Coração/anormalidades , Ventrículos do Coração/fisiopatologia , Hemodinâmica , Humanos , Recém-Nascido , Ligadura , Masculino , Cuidados Paliativos , Artéria Pulmonar/fisiopatologia , Atresia Pulmonar/mortalidade , Atresia Pulmonar/fisiopatologia , Valva Pulmonar/anormalidades , Valva Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/mortalidade , Estenose da Valva Pulmonar/fisiopatologia , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Procedimentos Cirúrgicos Vasculares/efeitos adversos , Procedimentos Cirúrgicos Vasculares/mortalidade
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