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1.
Rev Port Cir Cardiotorac Vasc ; 26(3): 187-193, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31734969

RESUMO

BACKGROUND: Surgical repair is the standard treatment for complete atrioventricular septal defect. At our institution, this repair is performed by single patch, modified single patch or two patch techniques, according to the surgeon preferences and the surgical anatomy of the defect. The goal of this study was to evaluate our results from the last twelve years. METHODS: From June 2006 to June 2018, 81 children with complete atrioventricular septal defect (without tetralogy of Fallot or unbalanced ventricles) were submitted to surgical repair at our institution. Data from all patients was retrospectively collected and evaluated. RESULTS: The average age was 6.9 ± 13.7 months and 84% had Down syndrome. Eighty percent were symptomatic and 6 patients were previously submitted to pulmonary artery banding. No more that mild left atrioventricular valve insufficiency was found in 84% and 89% of the patients, at discharge and follow-up, respectively. Small residual septal defects were present in 27% at discharge; during follow-up, 41% of these closed spontaneously. Pulmonary hypertension at discharge and follow-up appeared in 3.7% and 1.3%, respectively. Permanente pacemaker was implanted in 3 patients. Left ventricle outflow tract obstruction was found in 3 patients and 2 needed surgical correction. At follow-up (40 ± 38 months), 90% of the patients presented NYHA functional class I. No significant differences in the main repair outcomes were found between techniques, with the exception of small residual septal defects, although the groups were unmatched. CONCLUSIONS: Overall and regardless of the technique used for the repair of complete AVSD, good early and midterm outcomes were achieved.


Assuntos
Síndrome de Down/complicações , Defeitos dos Septos Cardíacos/cirurgia , Criança , Pré-Escolar , Defeitos dos Septos Cardíacos/etiologia , Humanos , Estudos Retrospectivos , Resultado do Tratamento
2.
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
3.
Pediatr Cardiol ; 40(5): 1057-1063, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31065759

RESUMO

In complete atrioventricular canal defect (CAVC), there are limited data on preoperative clinical and echocardiographic predictors of operative timing and postoperative outcomes. A retrospective, single-center analysis of all patients who underwent primary biventricular repair of CAVC between 2006 and 2015 was performed. Associated cardiac anomalies (tetralogy of Fallot, double outlet right ventricle) and arch operation were excluded. Echocardiographic findings on first postnatal echocardiogram were correlated with surgical timing and postoperative outcomes using bivariate descriptive statistics and multivariable logistic regression. 153 subjects (40% male, 84% Down syndrome) underwent primary CAVC repair at a median age of 3.3 (IQR 2.5-4.2) months. Median postoperative length of stay (LOS) was 7 (IQR 5-15) days. Eight patients (5%) died postoperatively and 24 (16%) required reoperation within 1 year. On multivariable analysis, small aortic isthmus (z score < - 2) was associated with early primary repair at < 3 months (OR 2.75, 95% CI 1.283-5.91) and need for early reoperation (OR 3.79, 95% CI 1.27-11.34). Preoperative ventricular dysfunction was associated with higher postoperative mortality (OR 7.71, 95% CI 1.76-33.69). Other factors associated with mortality and longer postoperative LOS were prematurity (OR 5.30, 95% CI 1.24-22.47 and OR 5.50, 95% CI 2.07-14.59, respectively) and lower weight at surgery (OR 0.17, 95% CI 0.04-0.75 and OR 0.55, 95% CI 0.35-0.85, respectively). Notably, preoperative atrioventricular valve regurgitation and Down syndrome were not associated with surgical timing, postoperative outcomes or reoperation, and there were no echocardiographic characteristics associated with late reoperation beyond 1 year after repair. Key preoperative echocardiographic parameters helped predict operative timing and postoperative outcomes in infants undergoing primary CAVC repair. Aortic isthmus z score < - 2  was associated with early surgical repair and need for reoperation, while preoperative ventricular dysfunction was associated with increased mortality. These echocardiographic findings may help risk-stratified patients undergoing CAVC repair and improve preoperative counseling and surgical planning.


Assuntos
Defeitos dos Septos Cardíacos/cirurgia , Tempo para o Tratamento , Síndrome de Down/complicações , Ecocardiografia , Feminino , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Humanos , Lactente , Recém-Nascido Prematuro , Masculino , Reoperação , Estudos Retrospectivos , Resultado do Tratamento
4.
Cardiol Young ; 29(5): 637-642, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31138335

RESUMO

AIM: In patients after atrioventricular septal defect correction, altered geometry leads to a changed position and subsequent flow over the left ventricular outflow tract. We hypothesised that this altered flow may influence haemodynamics in the ascending aorta. METHODS: In total, 30 patients after atrioventricular septal defect correction (age 27.6 ± 12.8 years) and 28 healthy volunteers (age 24.8 ± 13.7 years) underwent 4D flow cardiovascular magnetic resonance. Left ventricular ejection fraction and mean and peak wall shear stress calculated at ascending aortic peak systole were obtained from cardiovascular magnetic resonance. Left ventricular outflow tract data including velocity and diameter were obtained from echocardiography. RESULTS: Patients showed a higher mean (911 ± 173 versus 703 ± 154 mPa, p = 0.001) and peak ascending aortic wall shear stress (1264 ± 302 versus 1009 ± 240 mPa, p = 0.001) compared to healthy volunteers. Increased blood flow velocities over the left ventricular outflow tract (1.49 ± 0.30 m/s versus 1.22 ± 0.20 m/s, p < 0.001) correlated well with mean and peak ascending aortic wall shear stress (r = 0.67, p < 0.001 and r = 0.77, p < 0.001). CONCLUSION: After atrioventricular septal defect correction, increased wall shear stress was observed, which correlated to velocities over the left ventricular outflow tract. These findings imply that altered outflow tract geometry contributes to changed aortic haemodynamics.


Assuntos
Aorta/diagnóstico por imagem , Velocidade do Fluxo Sanguíneo , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Defeitos dos Septos Cardíacos/fisiopatologia , Adolescente , Adulto , Estudos de Casos e Controles , Feminino , Defeitos dos Septos Cardíacos/cirurgia , Humanos , Interpretação de Imagem Assistida por Computador , Modelos Logísticos , Imagem Cinética por Ressonância Magnética , Masculino , Análise Multivariada , Países Baixos , Estresse Mecânico , Função Ventricular Esquerda , Adulto Jovem
5.
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
6.
Artigo em Inglês | MEDLINE | ID: mdl-30875852

RESUMO

(1) Background: Oculo-facio-cardio-dental (OFCD) syndrome is a rare pathological condition with an X-linked dominant trait that only occurs in females; no males are born with OFCD syndrome. This syndrome is characterized by congenital cataracts with secondary glaucoma ocular defects, ventricular and atrial septal defects, or mitral valve prolapses. Facial traits are a long narrow face and a high nasal bridge with a bifid nasal tip. Dental anomalies include radiculomegaly, oligodontia, root dilacerations, malocclusion, and delayed eruption. (2) Methods: This clinical report describes a 26-year-old girl who suffers from OFCD syndrome and who was treated with a multidisciplinary approach. The treatment plan included orthodontic treatment, orthognathic surgery, namely LeFort I and a Bilateral Sagittal Split Osteotomy, and occlusal rehabilitation with implants. (3) Discussion: Early diagnosis and multidisciplinary treatment of orthodontic, orthognathic surgery and occlusal rehabilitation with implants make it possible to maintain tooth function and improve aesthetics with good prognoses for success. In this paper, we report a case of a female patient with OFCD syndrome, who was referred for orthodontic treatment and occlusal rehabilitation and treated with a multidisciplinary approach.


Assuntos
Catarata/congênito , Defeitos dos Septos Cardíacos/terapia , Má Oclusão/terapia , Microftalmia/terapia , Procedimentos Cirúrgicos Ortognáticos , Adulto , Catarata/terapia , Feminino , Defeitos dos Septos Cardíacos/cirurgia , Humanos , Má Oclusão/cirurgia , Microftalmia/cirurgia
7.
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
8.
Ann Thorac Surg ; 107(6): 1824-1830, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30771324

RESUMO

BACKGROUND: Operative mortality after complete atrioventricular septal defect (cAVSD) repair has improved vastly. Less improvement has been demonstrated regarding late mortality and reoperation rates, however. There is evident lack of comprehensive population-based studies analyzing the history and progress of the ever-changing operative results. METHODS: This is a 5-million population-based retrospective study of consecutive 388 cAVSD patients operated in Finland between 1962 and 2014. Data were collected using Children's Cardiac Surgical Registry of Children's Hospital at the Helsinki University Hospital, Finland. Mortality data and reoperation rates were analyzed on a decade-by-decade basis. RESULTS: During the early era, overall mortality was 17.4%, operative mortality constituting 10.9%. The operative results have improved significantly over the decades, and eventually, the last decade showed no mortality. A total of 23 late deaths occurred; of these, 20 were directly heart-related. Half of the late mortality occurred during the first postoperative year. The only significant risk factor for overall mortality was an earlier decade of operation (p < 0.001). Reoperation rates have not decreased but slightly increased over decades (p = 0.621), and reoperations have been performed mainly during the first year after the primary operation. Actuarial freedom from left side atrioventricular valve reoperation at 15 years was 90.9%. CONCLUSIONS: There has been an outstanding improvement in surgical results through the years even though the general operative approach has remained the same. Rates of reoperation have not been declining, but the reoperations are dated to early childhood years. The improvement in results has been ongoing.


Assuntos
Defeitos dos Septos Cardíacos/cirurgia , Complicações Pós-Operatórias/epidemiologia , Reoperação/estatística & dados numéricos , Pré-Escolar , Feminino , Finlândia , Defeitos dos Septos Cardíacos/patologia , Humanos , Lactente , Masculino , Estudos Retrospectivos , Fatores de Tempo
9.
Ann Thorac Surg ; 107(4): e251-e253, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30617024

RESUMO

An infant boy with 21 trisomy was diagnosed with complete atrioventricular septal defect-Rastelli type A with parachute left atrioventricular valve and absent left mural leaflet. Biventricular repair was difficult in infancy because the left ventricular volume and left atrioventricular valve annulus were too small. After 2 pulmonary artery bandings, the left atrioventricular valve annulus increased to 85% of normal mitral valve, and the left ventricular end-diastolic volume increased to 98% of normal. We report a successful instance of complete atrioventricular septal defect that achieved biventricular repair via novel left atrioventricular valvuloplasty for parachute left atrioventricular valve and absent left mural leaflet.


Assuntos
Valvuloplastia com Balão/métodos , Síndrome de Down/diagnóstico , Ecocardiografia Transesofagiana/métodos , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Defeitos dos Septos Cardíacos/cirurgia , Valva Mitral/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Síndrome de Down/complicações , Ecocardiografia/métodos , Seguimentos , Humanos , Recém-Nascido , Masculino , Valva Mitral/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Recuperação de Função Fisiológica/fisiologia , Resultado do Tratamento
10.
Cardiol Young ; 29(2): 235-237, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30511601

RESUMO

A 15-month-old child underwent percutaneous expansion of a Melody transcatheter pulmonary valve in the mitral position to accommodate growth after initial surgical implantation during infancy, but transiently decompensated after valvuloplasty owing to stent malformation. The Melody valve in the mitral position of small patients can be further expanded by percutaneous dilation, but there are a number of potential complications and technical improvements to consider.


Assuntos
Anormalidades Múltiplas , Valvuloplastia com Balão/métodos , Cateterismo Cardíaco/métodos , Defeitos dos Septos Cardíacos/diagnóstico , Próteses Valvulares Cardíacas , Insuficiência da Valva Mitral/cirurgia , Valva Mitral/cirurgia , Bioprótese , Ecocardiografia Transesofagiana , Feminino , Defeitos dos Septos Cardíacos/cirurgia , Humanos , Lactente , Valva Mitral/diagnóstico por imagem , Insuficiência da Valva Mitral/diagnóstico , Desenho de Prótese
11.
Interact Cardiovasc Thorac Surg ; 28(3): 427-431, 2019 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-30239715

RESUMO

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was, 'Is the modified single-patch repair superior to the double-patch repair of complete atrioventricular septal defects?'. A total of 634 papers were found using the reported search, of which 9 represented the best evidence to answer the clinical question, which included 1 meta-analysis and 8 cohort studies. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers were tabulated. There was limited high-quality evidence available, with all the included studies being retrospective and observational in nature. One meta-analysis and 8 cohort studies provided evidence that there was no significant difference in survival or other postoperative outcomes based on a surgical technique during follow-up ranging from 6 months to 4.2 years. Surgical reintervention for development of left ventricular outflow tract obstruction, left atrioventricular valve dysfunction or residual septal defects after the initial repair of complete atrioventricular septal defect was not significantly different between cohorts in almost all studies. Cardiopulmonary bypass and aortic cross-clamp times were significantly shorter with the modified single-patch repair compared to the double-patch repair in all studies that examined these variables, but this did not correspond to a difference in outcomes. We conclude, based on the available evidence, that the modified single-patch repair of complete atrioventricular septal defect is similar to the double-patch repair in terms of postoperative outcomes. However, this conclusion is limited by the retrospective nature of all studies, small cohort sizes and short durations of follow-up in addition to lack of statistical analysis in 1 study.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Defeitos dos Septos Cardíacos/cirurgia , Ecocardiografia , Feminino , Defeitos dos Septos Cardíacos/diagnóstico , Humanos , Lactente
12.
Congenit Heart Dis ; 14(2): 280-287, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30485659

RESUMO

OBJECTIVE: Outcomes after surgical repair of complete atrioventricular septal defect (cAVSD) have improved. With advancing age, the risk of development of dysrhythmias may increase. The aims of this study were to (1) examine development of sinus node dysfunction (SND), atrial and ventricular tachyarrhythmias, and (2) study progression of atrioventricular conduction abnormalities in young adult patients with repaired cAVSD. STUDY DESIGN: In this retrospective multicenter study, 74 patients (68% female) with a cAVSD repaired in childhood were included. Patients' medical files were evaluated for occurrence of SND, atrioventricular conduction block (AVB), atrial and ventricular tachyarrhythmias. RESULTS: Median age at repair was 6 months (interquartile range 3-10) and median age at last follow-up was 24 years (interquartile range 21-28). SND occurred after a median of 17 years (interquartile range 11-19) after repair in 23% of patients, requiring pacemaker implantation in two patients (12%). Regular supraventricular tachycardia was observed in three patients (4%). Atrial fibrillation and ventricular tachyarrhythmias were not observed. Twenty-seven patients (36%) had first-degree AVB, which was self-limiting in 16 (59%) and persistent in 10 (37%) patients. One patient developed third-degree AVB 7 days after left atrioventricular valve replacement. Spontaneous type II second-degree AVB occurred in a 28-year-old patient. Both patients underwent pacemaker implantation. CONCLUSIONS: Clinically significant dysrhythmias were uncommon in young adult patients after cAVSD repair. However, three patients required pacemaker implantation for either progression of SND or spontaneous type II second-degree AVB. Longer follow-up should point out whether dysrhythmias will progress or become more prevalent with increasing age.


Assuntos
Arritmias Cardíacas/etiologia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Previsões , Defeitos dos Septos Cardíacos/cirurgia , Complicações Pós-Operatórias , Adolescente , Adulto , Fatores Etários , Arritmias Cardíacas/epidemiologia , Arritmias Cardíacas/fisiopatologia , Criança , Pré-Escolar , Feminino , Seguimentos , Defeitos dos Septos Cardíacos/complicações , Humanos , Incidência , Lactente , Masculino , Países Baixos/epidemiologia , Estudos Retrospectivos , Adulto Jovem
13.
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
14.
Ir J Med Sci ; 188(2): 475-479, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-29943216

RESUMO

BACKGROUND: We describe the long-term results of partial atrioventricular septal defect (AVSD) repair in a single centre encompassing a 22-year period. Described are rates of survival, reoperation and complications. METHODS: We performed a retrospective review of 556 patients undergoing AVSD repair to identify the 51 patients who underwent partial AVSD repair in Our Lady's Children's Hospital, Crumlin, Ireland, between 1993 and 2015 with long-term follow-up where available. RESULTS: A total of 29 (56.8%) of patients were male and mean age at operation was 3.32 years. Mean weight was 13.2 kg. Trisomy 21 was present in 29 (56.8%). Five patients (9.6%) had undergone prior surgery. Mean cardiopulmonary bypass time was 89 ± 36 min and mean aortic cross-clamp time was 57 ± 28 min. One patient underwent partial AVSD repair and concomitant tracheal resection and extracorporeal membrane oxygenation decannulation. One patient was managed with suture atrial septal defect (ASD) closure, the remainder with patch repair of ASD and mitral cleft closure. The length of hospital stay was 9 ± 5 days. Median follow-up was 6.06 years (IQR, 1.65-10.2 years). There were no early mortalities. One patient died 1 year following surgery (1.9%). One patient required reoperation at an interval of 2 years for severe mitral regurgitation (1.9%). CONCLUSIONS: Short- and long-term survival following partial AVSD repair in Ireland revealed excellent results compared with other published series. Reoperation incidence also compared excellently with other reports published in the literature.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Comunicação Interventricular/cirurgia , Defeitos dos Septos Cardíacos/cirurgia , Adolescente , Criança , Pré-Escolar , Feminino , Defeitos dos Septos Cardíacos/patologia , Comunicação Interventricular/patologia , Humanos , Lactente , Irlanda , Masculino , Complicações Pós-Operatórias , Estudos Retrospectivos , Resultado do Tratamento
16.
Semin Thorac Cardiovasc Surg ; 31(1): 113-115, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30273646

RESUMO

Long-term outcomes in children with atrioventricular septal defect (AVSD) and univentricular palliation are of concern, with <60% survival at 25 years.1 Common atrioventricular valves (AVV) often become insufficient in patients with univentricular physiology, leading to heart failure.1,2 We have recently observed that outcomes of children with AVSD who reach Fontan circulation are not as bad as previously thought, provided that the AVV remains competent.1 Common AVV surgery is associated with substantial mortality and reoperation rates.3 Although successful AVV repair is associated with better survival and freedom from reoperation, good quality repair is difficult to achieve in univentricular circulation,3 especially in patients with dextrocardia.4 Herein, we report a patient with unbalanced AVSD and dextrocardia who underwent AVV repair using the "polytetrafluoroethylene (Gore-Tex, W.L. Gore & Associates, Flagstaff, AZ) bridge" technique5 with excellent early outcome.


Assuntos
Anormalidades Múltiplas , Anuloplastia da Valva Cardíaca , Dextrocardia/fisiopatologia , Técnica de Fontan , Defeitos dos Septos Cardíacos/cirurgia , Implante de Prótese de Valva Cardíaca , Ventrículos do Coração/cirurgia , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Anuloplastia da Valva Cardíaca/instrumentação , Criança , Dextrocardia/diagnóstico , Ecocardiografia Doppler em Cores , Técnica de Fontan/efeitos adversos , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Defeitos dos Septos Cardíacos/fisiopatologia , Próteses Valvulares Cardíacas , Implante de Prótese de Valva Cardíaca/instrumentação , Ventrículos do Coração/anormalidades , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Hemodinâmica , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Masculino , Desenho de Prótese , Recuperação de Função Fisiológica , Resultado do Tratamento , Função Ventricular
17.
Semin Thorac Cardiovasc Surg ; 31(1): 80-86, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-29428621

RESUMO

Quantifying unbalance, the threshold for single ventricle palliation vs biventricular repair in patients with unbalanced complete atrioventricular septal defect (AVSD), is challenging. Using a core laboratory review of baseline echocardiograms, we sought to assess the correlations among commonly used measures of unbalance and common atrioventricular valve (AVV) and ventricular sizes. A single reviewer evaluated baseline echocardiograms from an inception cohort of babies age < 1 year with complete AVSD admitted to 1 of 25 Congenital Heart Surgeon's Society institutions. A standardized echo review protocol of 111 quantitative and qualitative measures was used. Descriptive statistics were computed and Pearson correlation coefficients were calculated to assess correlation among unbalance indices with valvar and ventricular dimensions. Two-hundred fifty-seven baseline echocardiograms of infants with complete AVSD were included. Median age at baseline echocardiogram was 11 days (interquartile range 1-79) and mean atrioventricular valve index was 0.45 ± 0.1. Mean right ventricle/left ventricle inflow angle was 90.2 ± 15.6° and median left ventricular inflow index was 0.46 (interquartile range 0.4-0.5). There are weak or moderate correlations between the measures of unbalance. Correlations between the measures of unbalance with common AVV leaflet or ventricular sizes are also weak to moderate, when statistically significant. Measures of unbalance in common clinical use correlate poorly, or not at all, with one another, common AVV, and ventricular dimensions. The concept of "unbalance" is difficult to define using baseline echocardiographic indices. These findings suggest that the indices may describe different morphologic and functional characteristics. Further analysis is necessary to quantify the contributions of unbalance indices to patient outcome.


Assuntos
Ecocardiografia/métodos , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Procedimentos Cirúrgicos Cardíacos , Tomada de Decisão Clínica , Ecocardiografia Doppler em Cores , Feminino , Defeitos dos Septos Cardíacos/fisiopatologia , Defeitos dos Septos Cardíacos/cirurgia , Humanos , Lactente , Recém-Nascido , Masculino , América do Norte , Cuidados Paliativos , Valor Preditivo dos Testes , Prognóstico , Função Ventricular Esquerda , Função Ventricular Direita
18.
Interact Cardiovasc Thorac Surg ; 28(5): 789-796, 2019 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-30590597

RESUMO

OBJECTIVES: We analysed our 29-year experience of surgical repair of atrioventricular septal defect (AVSD) to define risk factors for mortality and reoperation. METHODS: Between 1988 and 2017, 508 patients received AVSD repair in our institution; 359 patients underwent surgery for complete AVSD, 76 for intermediate AVSD and 73 for partial AVSD. The median age of the patients was 6.1 months (interquartile range 10.3 months), and the median weight was 5.6 kg (interquartile range 3.2 kg). The standard AVSD repair was performed using 2-patch technique (n = 347) and complete cleft closure (n = 496). The results were divided into 2 surgical eras (early era 1986-2004 and late era 2004-2017). Risk factors were analysed to determine the impact of patient age, weight, the presence of trisomy 21 and complex AVSD on mortality and reoperation rate. RESULTS: In-hospital mortality decreased from 10.2% (n = 26) in early surgical era to 1.6% (n = 4) in late surgical era (P < 0.001). Seventy-seven patients required reoperation. Freedom from reoperation was 84.4% after 25 years. The main indication for reoperation was left atrioventricular valve regurgitation (13.8%). The multivariable Cox regression analysis revealed reoperation of the left AV valve, early surgical era, patient age <3.0 months and complex AVSD to be independent risk factors for mortality. Age <3.0 months, complex AVSD and moderate/severe left AV valve regurgitation at discharge predicted reoperation. CONCLUSIONS: AVSD repair can be performed with low mortality and reoperation rate. Age <3 months, complex AVSD and moderate/severe regurgitation of the left AV valve at discharge were predictors for reoperation. Reoperation of the left AV valve was the strongest risk factor for mortality.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Previsões , Defeitos dos Septos Cardíacos/cirurgia , Feminino , Seguimentos , Alemanha/epidemiologia , Defeitos dos Septos Cardíacos/diagnóstico , Defeitos dos Septos Cardíacos/mortalidade , Mortalidade Hospitalar/tendências , Humanos , Lactente , Masculino , Alta do Paciente , Reoperação , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida/tendências , Resultado do Tratamento
19.
Neonatal Netw ; 37(5): 281-291, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30567810

RESUMO

Congenital heart defects (CHD) are the most common congenital malformation reported in the literature, with a global incidence of eight per every 1,000 live births. In the United States approximately 40,000 infants are born each year with a CHD. Of the infants diagnosed with a CHD, one in every four heart defects are life threatening in origin. Early identification and treatment of congenital heart lesions, beginning with a comprehensive physical assessment after birth, are critical. For infants delivered at community-based hospitals, the importance of the physical assessment, timing of diagnostic strategies, anticipatory planning, and interprofessional collaboration among referring and accepting centers cannot be understated. This article presents a rare case of an infant with atrioventricular canal complicated by dextrocardia. Embryology, pathophysiology, epidemiology, symptomology, cardiac assessment, diagnostics, treatment, and nursing strategies for facilitating transfer of care from community-based hospitals to tertiary medical centers are discussed.


Assuntos
Dextrocardia/fisiopatologia , Dextrocardia/cirurgia , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/cirurgia , Defeitos dos Septos Cardíacos/fisiopatologia , Defeitos dos Septos Cardíacos/cirurgia , Adulto , Dextrocardia/diagnóstico , Dextrocardia/epidemiologia , Cardiopatias Congênitas/diagnóstico , Defeitos dos Septos Cardíacos/diagnóstico , Defeitos dos Septos Cardíacos/epidemiologia , Humanos , Incidência , Recém-Nascido , Masculino , Resultado do Tratamento , Estados Unidos/epidemiologia
20.
Circ J ; 82(11): 2913-2916, 2018 10 25.
Artigo em Inglês | MEDLINE | ID: mdl-30298834

RESUMO

BACKGROUND: Post-repair atrial septal defects (ASD) patients are frequently discharged from follow-up, but the extent of pulmonary symptoms long-term post-repair is unknown. Methods and Results: The national CONgenital CORvitia registry was linked to the national Drug Registry to investigate all ambulatory-dispensed pulmonary inhalants for 2006-2014. ASD patients were compared with age- and sex-matched referents from the general population. A total of 1,959 adult patients (age 42±17 years; 66% female; 1,223 [62%] repaired) were included. Compared with the referents, ASD patients had more inhalant use, even at long-term post-repair follow-up (OR=1.81 [95% CI 1.62-2.03]; P<0.001). CONCLUSIONS: ASD patients had 2-fold higher inhalant use compared with referents even at long-term post-repair follow-up, suggesting persistent pulmonary functional impairment.


Assuntos
Defeitos dos Septos Cardíacos , Pneumopatias , Pulmão , Sistema de Registros , Administração por Inalação , Adulto , Feminino , Seguimentos , Defeitos dos Septos Cardíacos/complicações , Defeitos dos Septos Cardíacos/cirurgia , Humanos , Pneumopatias/complicações , Pneumopatias/tratamento farmacológico , Masculino , Pessoa de Meia-Idade
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