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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.
Indian Pediatr ; 56(4): 314-316, 2019 04 15.
Artigo em Inglês | MEDLINE | ID: mdl-31064902

RESUMO

OBJECTIVE: To compare the frequency of asymmetric septal hypertrophy in appropriate for gestational age infants born to diabetic mothers with those born to non-diabetic mothers. METHODS: We compared 38 full term infants born to diabetic mothers with 85 full term infants of non-diabetic mothers. 2-D echocardiography was obtained in the first 24 hours after birth. RESULTS: Asymmetric septal hypertrophy was only present in infants born to diabetic mothers (50% vs. 0%; P<0.001). Intraventricular septum thickness and intraventricular septum/posterior wall of the left ventricle ratio was also significantly higher in the first group (P<0.001). We found no correlation between mother´s glycated hemoglobin levels and intraventricular septum thickness in newborns. CONCLUSION: Asymmetric septal hypertrophy is a common finding in infants born to diabetic mothers, even if they are appropriate for gestational age.


Assuntos
Idade Gestacional , Defeitos dos Septos Cardíacos , Gravidez em Diabéticas/epidemiologia , Diabetes Mellitus/epidemiologia , Ecocardiografia , Feminino , Hemoglobina A Glicada/análise , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Defeitos dos Septos Cardíacos/epidemiologia , Defeitos dos Septos Cardíacos/patologia , Humanos , Hipertrofia , Recém-Nascido , Masculino , Mães/estatística & dados numéricos , Gravidez
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.
Anatol J Cardiol ; 21(4): 214-221, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30930449

RESUMO

OBJECTIVE: To compare the accuracy and reasons for disagreement of two-dimensional (2D) and three-dimensional (3D) echocardiography findings in the assessment of the atrioventricular valve complex in patients with atrioventricular septal defect. METHODS: A total of 20 children (mean age 8 months) with atrioventricular septal defect were enrolled prospectively into this study. The accuracy of and the reasons for disagreement in the assessment of the atrioventricular valve features were analyzed between 2D and 3D echocardiography and surgical findings. RESULTS: We found that in assessing the Rastelli type and the extension of the inferior leaflet into the right ventricle, 3D echocardiography was more accurate compared to 2D echocardiography. In all other features, 2D and 3D echocardiography showed similar accuracy. A significant reason for inaccuracy by both echo modalities was that the technique itself could not visualize the feature, although the image quality was considered to be adequate. In most cases, where it was not possible to visualize the atrioventricular feature by 2D, it was possible by 3D, and vice versa. CONCLUSION: The accuracy of 2D and 3D echocardiography and understanding the potential reasons for disagreements in assessing the atrioventricular valve complex with 2D and 3D can guide the use of those two techniques when combining them in the clinical practice.


Assuntos
Defeitos dos Septos Cardíacos/diagnóstico por imagem , Valvas Cardíacas/diagnóstico por imagem , Adolescente , Criança , Pré-Escolar , Ecocardiografia , Ecocardiografia Tridimensional , Feminino , Defeitos dos Septos Cardíacos/fisiopatologia , Valvas Cardíacas/fisiopatologia , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Prospectivos , Reprodutibilidade dos Testes
6.
Echocardiography ; 36(4): 809-812, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30801807

RESUMO

Transthoracic echocardiography is the principal imaging modality for assessment of patients with atrioventricular septal defects. Three-dimensional echocardiography streamlines and simplifies data acquisition offering a unique realistic en-face display of heart valves and septal defects and enables accurate evaluation of the cardiac anatomy, dynamic, and function. We demonstrated an added value of three-dimensional echocardiography in assessment of an adult patient with atrioventricular septal defect and its advantages over conventional echocardiography.


Assuntos
Ecocardiografia Tridimensional/métodos , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Adulto , Septos Cardíacos/diagnóstico por imagem , Humanos , Masculino , Reprodutibilidade dos Testes
7.
Neurology ; 92(9): e924-e932, 2019 02 26.
Artigo em Inglês | MEDLINE | ID: mdl-30674593

RESUMO

OBJECTIVE: To determine the associations between transthoracic echocardiogram (TTE) cardiac structure/function measures and cardioembolic stroke (CES) and new-onset atrial fibrillation (AF) in patients without known AF. METHODS: Inpatients at a single institution (2013-2015) with imaging-confirmed ischemic stroke, no AF, and TTE within the 1st week were included. TTE structure/function variables were abstracted. Stroke subtype (CES vs other) was defined according to Trial of Org 10172 in Acute Stroke Treatment, blinded to TTE results. New AF was defined as any duration of AF on ECG, telemetry, or event monitor. Separate multivariable logistic regression models defined associations between CES or new-onset AF and TTE measures, adjusting for demographic and vascular risk factors. RESULTS: Of 322 participants (mean age 60 years), 55% were male and 56% African American. In adjusted models (odds ratio, 95% confidence interval), odds of CES increased per 0.1 cm increase in left atrial (LA) systolic diameter (1.06, 1.02-1.11), 1 cm/s in mitral E point velocity (1.03, 1.02-1.05), with presence of mitral valve dysfunction (3.78, 1.42-10.02), and with wall motion abnormality (2.00, 1.13-3.55). As ejection fraction increased (per 10%), odds of CES decreased (0.65, 0.53-0.79). New-onset AF was also associated with increasing LA systolic diameter (1.13, 1.04-1.22). CONCLUSIONS: Cardiac structural changes independent of AF and detectable on TTE may be on the CES causal pathway. Confirming these results could have implications for future use of TTE and decisions about antithrombotic vs anticoagulant treatment.


Assuntos
Fibrilação Atrial/epidemiologia , Coração/diagnóstico por imagem , Embolia Intracraniana/epidemiologia , Acidente Vascular Cerebral/epidemiologia , Idoso , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/fisiopatologia , Ecocardiografia , Feminino , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Defeitos dos Septos Cardíacos/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Valva Mitral/diagnóstico por imagem , Valva Mitral/fisiopatologia , Tamanho do Órgão , Fatores de Risco , Volume Sistólico , Septo Interventricular/diagnóstico por imagem , Septo Interventricular/patologia
8.
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.
Diagn Interv Imaging ; 100(2): 109-116, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30527913

RESUMO

PURPOSE: The purpose of this study was to compare non-invasive high-spatial-resolution postmortem cardiac magnetic resonance imaging (MRI) and autopsy findings for evaluating the septal insertion of atrioventricular valves in fetuses. MATERIALS AND METHODS: Five fetal heart specimens including two normal hearts, one heart with complete atrioventricular septal defect (AVSD) and two hearts with linear insertion of atrioventricular valves (LIAVV; gestational age 17 to 34 weeks) were studied with cardiac MRI using a 4.7 T MRI scanner without sample preparation. Three (3D) and two-dimensional (2D) turbo-RARE (rapid imaging with refocused echoes) sequences in four-chamber and left-ventricular long-axis planes were obtained with a minimal isotropic/in-plane resolution of 156µm. Nonparametric tests were performed to compare the distance between insertions of medial leaflets of the atrioventricular valves and the inlet/outlet distance ratio between MRI and autopsy findings in normal, complete AVSD and with linear insertion of atrioventricular valves (LIAVV) fetal hearts. RESULTS: Despite apparent differences between LIAVV/normal hearts, no significant differences were found between differential insertion of medial leaflets and inlet/outlet distance ratios with both techniques. Very good to excellent reliability between both techniques was found for differential insertion (ICC: 87.2%; 95% CI: -21.7%, 99.1%) (P=0.963) and inlet/outlet distance ratio (ICC 98.3%; 95%CI: 85.2%, 99.8%) (P=0.537) measurements. CONCLUSION: Postmortem cardiac MRI could replace autopsy for assessing normal or abnormal septal insertion of atrioventricular valves in fetuses without requiring specific preparation of the heart.


Assuntos
Feto/anormalidades , Feto/diagnóstico por imagem , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Valvas Cardíacas/anormalidades , Valvas Cardíacas/diagnóstico por imagem , Imagem por Ressonância Magnética , Cadáver , Estudos de Viabilidade , Humanos , Imagem por Ressonância Magnética/métodos
11.
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
12.
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
13.
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
14.
Medicine (Baltimore) ; 97(49): e13444, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30544426

RESUMO

RATIONALE: Oculofaciocardiodental syndrome (OFCD) patients who show radiculomegaly are very rare. We treated a new OFCD patient orthodontically, and performed longitudinal observation for 30 years. New findings, termed calcified-dental-papillae (CDPs) beneath open-apices (OAs) of developing radiculomegalies, pulp-stone-like-calcifications (PSLCs) and the process of radiculomegaly development were observed. A novel mutation of BCL-6 interacting corepressor (BCOR) was identified. Cone-beam-computed-tomography (CBCT) images of the radiculomegalies clarified their morphology. PATIENT CONCERNS: A female patient and her parents were referred to orthodontic clinic for alignment of the teeth. DIAGNOSIS: A CDP that harbored bulbous-round-calcified-tissue in the dental papilla beneath the OA of a developing radiculomegaly was found radiographically. PSLCs were observed in the dental pulp. Genetic analysis revealed a novel mutation c.265G>A on Exon 4 and diagnosed as OFCD. CBCT images confirmed round-calcified-tissue and PSLC and that the length of an affected canine was 38.0 mm and calculated as +14.8SD. These novel findings were not observed in lateral incisors and molars. INTERVENTIONS: Observation was performed for 29 years and 3 months including orthodontic treatment for 2 years and 9 months. OUTCOME: Longitudinal follow-up for 26 years and 7 months after the treatment revealed that the development of radiculomegaly every few months or years, CDPs beneath OAs and PSLCs were observed. CDPs, PSLCs, and OAs were associated with radiculomegaly. The patient and the affected teeth including aligned teeth showed no particular change after the completion of the radiculomegaly. CBCT images showed bulbous-calcified-tissue and PSLCs in the mature dental pulp associated with radiculomegaly. LESSONS: The radiographical findings of CDP, OA and PSLC help early diagnose of OFCD and have importance for initiating orthodontic treatment until radiculomegaly completion.


Assuntos
Catarata/congênito , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Defeitos dos Septos Cardíacos/genética , Microftalmia/diagnóstico por imagem , Microftalmia/genética , Mutação , Proteínas Proto-Oncogênicas/genética , Proteínas Repressoras/genética , Anormalidades Dentárias/diagnóstico por imagem , Anormalidades Dentárias/genética , Catarata/diagnóstico por imagem , Catarata/genética , Catarata/reabilitação , Criança , Feminino , Defeitos dos Septos Cardíacos/reabilitação , Humanos , Incisivo/diagnóstico por imagem , Incisivo/crescimento & desenvolvimento , Microftalmia/reabilitação , Ortodontia Corretiva , Anormalidades Dentárias/reabilitação , Raiz Dentária/diagnóstico por imagem , Raiz Dentária/crescimento & desenvolvimento , Resultado do Tratamento
15.
Circ J ; 82(12): 3090-3099, 2018 11 24.
Artigo em Inglês | MEDLINE | ID: mdl-30298851

RESUMO

BACKGROUND: In patients undergoing transcatheter aortic valve replacement (TAVR) for severe aortic stenosis (AS), a sigmoid septum, characterized by subaortic interventricular hypertrophy, often results in the need for new pacemaker implantation (PMI). In this study, we reviewed the feasibility and treatment efficacy of TAVR for AS in patients with a sigmoid septum. Methods and Results: Between 2011 and 2016, 48 patients (25.4%; mean age 84.9±5.4 years; 9 males) with a sigmoid septum and 141 (74.6%; mean age 82.9±5.5 years; 61 males) without underwent TAVR. Their operative outcomes, echocardiographic and electrocardiographic findings, and long-term outcomes were retrospectively compared. Second TAVR because of valve malposition was performed in 3 patients with a sigmoid septum (6.3%) and in 2 patients without a sigmoid septum (1.4%), with no significant difference between the 2 groups. Although there was no significant difference in valve hemodynamics between the 2 groups, sigmoid septum and deep implantation (implantation depth ≥10 mm) were independent predictors of new PMI following TAVR. CONCLUSIONS: Although a sigmoid septum did not preclude the feasibility, safety, or efficacy of TAVR for severe AS, its presence was associated with new PMI. Our approach to TAVR in patients with a sigmoid septum may contribute to clinical outcomes comparable to those of patients without this pathology.


Assuntos
Estenose da Valva Aórtica/cirurgia , Valva Aórtica/cirurgia , Defeitos dos Septos Cardíacos/cirurgia , Próteses Valvulares Cardíacas , Substituição da Valva Aórtica Transcateter , Idoso , Idoso de 80 Anos ou mais , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/fisiopatologia , Estenose da Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/fisiopatologia , Feminino , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Defeitos dos Septos Cardíacos/fisiopatologia , Humanos , Masculino , Estudos Retrospectivos
16.
Anatol J Cardiol ; 20(4): 229-234, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30297581

RESUMO

OBJECTIVE: The follow-up results of patients operated for atrioventricular septal defect (AVSD) during 1996-2016 at Baskent University are presented. METHODS: Data obtained from hospital records consists of preoperative echocardiographic and angiographic details, age and weight at surgery, operative details, Down syndrome presence, postoperative care details, early postoperative and latest echocardiographic findings and hospitalization for reintervention. RESULTS: A total of 496 patient-files were reviewed including 314 patients (63.4%) with complete and 181 (36.6%) with partial AVSD (48.4% of all patients had Down syndrome). Atrioventricular (AV) valve morphology was Rastelli type A in 92.2%, B in 6.5%, and C in 1.3% of patients. The operative technique used was single-patch in 21.6% (108), double-patch in 25.8% (128), and modified single-patch (Wilcox) in 52.5% (260) of patients. The follow-up time was 37.79±46.70 (range, 0-198) months. A total of 64 patients (12.9%) had arrhythmias while in the intensive care unit; pacemaker was implanted in 12 patients. A total of 78 patients (15.7%) were treated for pulmonary hypertensive crisis. The early morbidity and mortality in the postoperative first month were calculated as 38% and 10%, and the late morbidity and mortality (>1 month) were calculated as 13.1% and 1.9%, respectively. The rate of reoperation in our cohort was 8.9%. CONCLUSION: Although the early morbidity and mortality are low in AVSD operations, the rate of reoperations for left AV valve insufficiency are still high. Although Down syndrome is not a risk factor for early mortality, the co-morbid factors, such as longer postoperative mechanical ventilator or inotropic support, lead to higher risk for morbidity. The frequency of pulmonary hypertension and consequent complications are also high.


Assuntos
Síndrome de Down , Defeitos dos Septos Cardíacos/cirurgia , Criança , Pré-Escolar , Feminino , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Defeitos dos Septos Cardíacos/mortalidade , Humanos , Lactente , Estudos Longitudinais , Masculino , Registros Médicos , Complicações Pós-Operatórias , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento , Turquia
17.
Ann Thorac Surg ; 106(5): 1429-1437, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30009807

RESUMO

BACKGROUND: Contemporary outcomes of complete atrioventricular septal defect (CAVSD) repair, particularly for defects with associated abnormalities, is unclear. The goal of this study is to report an all-inclusive experience of CAVSD repair using a consistent surgical approach. METHODS: All patients undergoing CAVSD repair between 1995 and 2016 at our institution were included. Patients were divided into 2 groups: isolated and complex (tetralogy of Fallot, aortic arch repair, double outlet right ventricle, and total anomalous pulmonary venous return). Survival and reoperation were analyzed using log-rank test and Gray's test, respectively. Multivariable analysis was performed with Cox regression. RESULTS: Overall, 406 patients underwent repair: 350 (86%) isolated and 56 (14%) complex CAVSD (tetralogy of Fallot: 34, double outlet right ventricle: 7, aortic arch repair: 12, total anomalous pulmonary venous return: 3). Median age at repair was 5 months (range, 10 days to 16 years); 339 (84%) had trisomy 21. A 2-patch repair was used in 395 (97%) and the zone of apposition was completely closed in 305 (75%). Perioperative mortality was 2% and 4% in the isolated and complex groups, respectively. Perioperative mortality since 2006 was 0.9%. Median follow-up was 7 years. Overall 10-year survival and incidence of any reoperation were 92% and 11%, respectively. Complex anatomy was not a risk factor for mortality (p = 0.35), but it was for reoperation (hazard ratio [HR]: 2.6; p < 0.01). Risk factors for left atrioventricular valve reoperation were a second bypass run (HR: 2.7) and preoperative moderate or worse regurgitation (HR: 2.3). CONCLUSIONS: Mortality after CAVSD repair is low, yet reoperation remains a significant problem. Repair of complex CAVSD can be performed with similar mortality rates.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Comunicação Interventricular/diagnóstico por imagem , Comunicação Interventricular/cirurgia , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Defeitos dos Septos Cardíacos/cirurgia , Procedimentos Cirúrgicos Reconstrutivos/métodos , Procedimentos Cirúrgicos Cardíacos/mortalidade , Ponte Cardiopulmonar/métodos , Ponte Cardiopulmonar/mortalidade , Pré-Escolar , Bases de Dados Factuais , Feminino , Seguimentos , Defeitos dos Septos Cardíacos/mortalidade , Comunicação Interventricular/mortalidade , Humanos , Lactente , Recém-Nascido , Masculino , Análise Multivariada , Modelos de Riscos Proporcionais , Procedimentos Cirúrgicos Reconstrutivos/mortalidade , Estudos Retrospectivos , Medição de Risco , Fatores de Tempo , Resultado do Tratamento
18.
Ann Thorac Surg ; 106(4): 1220-1227, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-29859151

RESUMO

BACKGROUND: Vertical right axillary mini-thoracotomy (VRAMT) is the standard approach for correction of atrial septal defect and partial atrioventricular septal defect at our institution. This observational single-center study compares our initial results with the VRAMT approach for the repair of ventricular septal defect (VSD) and complete atrioventricular septal defect (CAVSD) in infants and children to an approach using standard median sternotomy (MS). METHODS: The perioperative courses of patients undergoing VSD and CAVSD correction through either a VRAMT or an MS were analyzed retrospectively. The surgical technique for the VRAMT involved a 4- to 5-cm vertical incision in the right axillary fold. RESULTS: Of 84 patients, 25 (VSD, n = 15; CAVSD, n = 10) underwent correction through a VRAMT approach, whereas 59 (VSD, n = 35; CAVSD, n = 24) had repair through MS. VSD and CAVSD groups were comparable with respect to age and weight. No significant differences were observed for aortic cross-clamp duration, intensive care unit stay, hospital stay, and echocardiographic follow-up. There was no need for any conversion from VRAMT to MS in any case. Neither wound infections nor thoracic deformities were observed in both groups. CONCLUSIONS: VRAMT can be considered as a safe and effective approach for the repair of VSD and CAVSD in selected patient groups, and the outcome data appear comparable to those of MS.


Assuntos
Comunicação Interventricular/cirurgia , Defeitos dos Septos Cardíacos/cirurgia , Esternotomia/métodos , Toracotomia/métodos , Centros Médicos Acadêmicos , Fatores Etários , Axila/cirurgia , Distribuição de Qui-Quadrado , Criança , Pré-Escolar , Estudos de Coortes , Intervalos de Confiança , Ecocardiografia/métodos , Feminino , Seguimentos , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Defeitos dos Septos Cardíacos/mortalidade , Comunicação Interventricular/diagnóstico por imagem , Comunicação Interventricular/mortalidade , Humanos , Lactente , Masculino , Procedimentos Cirúrgicos Minimamente Invasivos/efeitos adversos , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Posicionamento do Paciente , Segurança do Paciente , Seleção de Pacientes , Estudos Retrospectivos , Medição de Risco , Esternotomia/efeitos adversos , Suíça , Toracotomia/efeitos adversos , Resultado do Tratamento
19.
BMJ Case Rep ; 20182018 May 12.
Artigo em Inglês | MEDLINE | ID: mdl-29754141

RESUMO

Unilateral lung agenesis is a relatively rare congenital anomaly with a reported incidence of 1 in 15 000 births. It is frequently associated with other congenital malformations. Some of the sequelae of lung agenesis are potentially life-threatening. Here, we report a case of left lung agenesis in association with hiatal hernia and atrioventricular septal defect, a rare combination of anomalies which have not been described previously in the literature.


Assuntos
Anormalidades Múltiplas/diagnóstico por imagem , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Hérnia Hiatal/diagnóstico por imagem , Herniorrafia/métodos , Pneumopatias/diagnóstico por imagem , Pulmão/anormalidades , Anormalidades Múltiplas/fisiopatologia , Anormalidades Múltiplas/terapia , Adulto , Feminino , Gastrostomia/métodos , Defeitos dos Septos Cardíacos/fisiopatologia , Defeitos dos Septos Cardíacos/terapia , Hérnia Hiatal/fisiopatologia , Hérnia Hiatal/terapia , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Pulmão/diagnóstico por imagem , Pulmão/fisiopatologia , Pneumopatias/fisiopatologia , Pneumopatias/terapia , Gravidez , Encaminhamento e Consulta , Fatores de Tempo , Resultado do Tratamento
20.
Pediatr Cardiol ; 39(7): 1308-1314, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29744658

RESUMO

A comprehensive understanding of the native pulmonary blood supply is crucial in newborns with pulmonary atresia with ventricular septal defect and aortopulmonary collaterals (PA/VSD/MAPCA). We sought to describe the accuracy in terms of identifying native pulmonary arteries, radiation dose and anaesthetic time associated with multi-modality imaging in these patients, prior to their first therapeutic intervention. Furthermore, we wanted to evaluate the cumulative radiations dose and anaesthetic time over the study period. Patients with PA/VSD/MAPCA diagnosed at < 100 days between 2004 and 2014 were identified. Cumulative radiation dose and anaesthetic times were calculated, with imaging results compared with intraoperative findings. We then calculated the cumulative risks to date for all surviving children. Of 19 eligible patients, 2 had echocardiography only prior to first intervention. The remaining 17 patients underwent 13 MRIs, 4 CT scans and 13 cardiac catheterization procedures. The mean radiation dose was 169 mGy cm2 (47-461 mGy cm2), and mean anaesthetic time was 111 min (33-185 min). 3 children had MRI only with no radiation exposure, and one child had CT only with no anaesthetic. Early cross-sectional imaging allowed for delayed catheterisation, but without significantly reducing radiation burden or anaesthetic time. The maximum cumulative radiation dose was 8022 mGy cm2 in a 6-year-old patient and 1263 min of anaesthetic at 5 years. There is the potential to generate very high radiation doses and anaesthetic times from diagnostic imaging alone in these patients. As survival continues to improve in many congenital heart defects, the important risks of serial diagnostic imaging must be considered when planning long-term management.


Assuntos
Cateterismo Cardíaco/efeitos adversos , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Imagem por Ressonância Magnética/efeitos adversos , Atresia Pulmonar/diagnóstico por imagem , Doses de Radiação , Tomografia Computadorizada por Raios X/efeitos adversos , Adolescente , Anestesia/efeitos adversos , Anestesia/estatística & dados numéricos , Anestésicos/administração & dosagem , Anestésicos/efeitos adversos , Cateterismo Cardíaco/estatística & dados numéricos , Criança , Pré-Escolar , Circulação Colateral , Ecocardiografia/estatística & dados numéricos , Feminino , Defeitos dos Septos Cardíacos/cirurgia , Humanos , Lactente , Recém-Nascido , Imagem por Ressonância Magnética/estatística & dados numéricos , Masculino , Imagem Multimodal/efeitos adversos , Imagem Multimodal/métodos , Artéria Pulmonar/diagnóstico por imagem , Atresia Pulmonar/cirurgia , Estudos Retrospectivos , Medição de Risco/métodos , Tomografia Computadorizada por Raios X/estatística & dados numéricos , Reino Unido
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