RESUMO
Anatomic and physiologic assessment of surgical repair of scimitar syndrome can be challenging. We evaluated the first patient who underwent the Lugones procedure in 2012 using 4D flow magnetic resonance imaging. With this technology, we demonstrate that the reconstructed right pulmonary venous return drains into the left atrium with laminar flow, just as normal pulmonary veins do.
Assuntos
Síndrome de Cimitarra , Humanos , Síndrome de Cimitarra/cirurgia , Síndrome de Cimitarra/diagnóstico por imagem , Veias Pulmonares/cirurgia , Veias Pulmonares/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Feminino , Masculino , Procedimentos Cirúrgicos Cardíacos/métodos , Fatores de TempoRESUMO
Scimitar Syndrome is part of a complex spectrum of congenital cardiovascular anomalies related to anomalous pulmonary venous return. Depending on the extent of involvement, treatment can be either expectant or surgical. Prognosis and survival have been controversial, with some results supporting early surgical management. This research aims to disclose the outcomes and describe the management, clinical and imaging characteristics of patients diagnosed with Scimitar Syndrome treated in a tertiary referral healthcare center. Longitudinal descriptive observational study. The study included all patients diagnosed with scimitar syndrome in our institution between January/2011 and December/2022. A description of the sociodemographic and clinical characteristics, diagnostic tools used, treatment features, and patient outcomes is provided. Eleven patients were included, with a mean age at diagnosis of five years (CI 0-17), six of which were female (54.55%). Nine (81.82%) patients had evidence of a scimitar vein on the chest radiograph, six (54.55%) cardiac dextroposition, six (54.55%) pulmonary hypoplasia, five (45.45%) right pulmonary artery hypoplasia, and three (27.27%) had aortopulmonary collaterals. Four (36.36%) patients had horseshoe lungs, and four (36.36%) had bronchopulmonary sequestration. In the associations, two (18.18%) patients were found to have an atrial septal defect, three (27.27%) ventricular septal defect, and one (9%) had Tetralogy of Fallot. Pulmonary hypertension was demonstrated in two (18.18%) patients. Seven (63.64%) required surgical management to correct the scimitar vein, and two patients died due to unrelated complications. Scimitar syndrome presents diagnostic and treatment challenges, necessitating a multidisciplinary approach for timely care. Chest radiography and CT scans are primary diagnostic tools, with surgical intervention often warranted alongside other heart defects or significant hemodynamic repercussions. Medical management is effective for mild to moderate cases. Long-term patient outcomes remain uncertain due to study limitations, but improved life expectancy is anticipated with ongoing care.
Assuntos
Valor Preditivo dos Testes , Síndrome de Cimitarra , Centros de Atenção Terciária , Humanos , Síndrome de Cimitarra/diagnóstico por imagem , Síndrome de Cimitarra/cirurgia , Síndrome de Cimitarra/fisiopatologia , Síndrome de Cimitarra/mortalidade , Síndrome de Cimitarra/terapia , Feminino , Masculino , Colômbia , Pré-Escolar , Criança , Lactente , Adolescente , Resultado do Tratamento , Recém-Nascido , Estudos Longitudinais , Fatores de Tempo , Estudos Retrospectivos , Circulação Pulmonar , Procedimentos Cirúrgicos CardíacosAssuntos
Diagnóstico Tardio , Síndrome de Cimitarra , Síndrome de Turner , Humanos , Síndrome de Turner/complicações , Síndrome de Turner/diagnóstico , Feminino , Síndrome de Cimitarra/diagnóstico por imagem , Síndrome de Cimitarra/diagnóstico , Comunicação Interatrial/diagnóstico , Comunicação Interatrial/diagnóstico por imagem , Comunicação Interatrial/complicações , Imagem Multimodal , CriançaRESUMO
Fetal diagnosis of scimitar syndrome requires a high index of suspicion. We present two fetal cases of complex congenital heart disease associated with scimitar syndrome, one of them is diagnosed with scimitar syndrome in utero. We emphasize prenatal echocardiographic findings that may assist with arriving at the correct prenatal diagnosis. We also discuss potential challenges in suspecting the presence of scimitar syndrome in utero. The postnatal echocardiographic findings and course are described for both patients. We reviewed the available literature on prenatal diagnosis of scimitar syndrome in the presence of complex congenital heart disease. We describe a new association of VACTERL, imperforate anus, scimitar syndrome, and double-outlet right ventricle all on the same patient, as well as the first prenatal diagnosis of scimitar syndrome associated with hypoplastic left heart syndrome with restrictive atrial septum. Advanced imaging modalities such as a fetal lung Magnetic Resonance Imaging is suggested as a confirmatory test when scimitar syndrome is suspected in utero in the presence of complex congenital heart disease.
Assuntos
Dupla Via de Saída do Ventrículo Direito , Síndrome do Coração Esquerdo Hipoplásico , Síndrome de Cimitarra , Feminino , Humanos , Gravidez , Síndrome de Cimitarra/complicações , Diagnóstico Pré-Natal , Síndrome do Coração Esquerdo Hipoplásico/complicações , Imageamento por Ressonância Magnética , Dupla Via de Saída do Ventrículo Direito/complicações , Coração FetalRESUMO
INTRODUCTION: Scimitar syndrome (CS) is a rare congenital cardiac malformation that occurs in 2/100,000 live births with a 2:1 predominance in females, it belongs to the group of partial anomalous pulmonary venous connections, which has numerous described variations, being a subtype that constitutes only 3-5% of congenital cardiac anomalies. It is classified as a heart disease with increased pulmonary and cyanogenic flow, its diagnosis is usually early, but we will highlight the importance of attention to this pathology in adult patients without a previous diagnosis. CASE REPORT: Female patient, 39 years old, with dyspnea for 3 years, with progressive worsening of functional class. On physical examination, pulmonary auscultation was normal with 98% saturation. On cardiac auscultation, regular rhythm, with fixed split S2 and ejective systolic murmur 2+/4+ in the middle right sternal border. A chest X-ray was performed during the investigation, which showed a slightly arched tubular structure towards the right atrium with dextrocardia. In transthoracic echocardiogram evidenced anomalous pulmonary venous connections: the right pulmonary veins drain into the right atrium at the mouth of the inferior vena cava, with free flow, and the left into the left atrium. . Ostium secundum interatrial communication of 13.5 mm with bidirectional flow, significant dilation of the right cavities, with right ventricular myocardium with mild hypertrophy, with preserved function. PSVD 51mmHg and hypoplastic right pulmonary artery. DISCUSSION SC is a rare disease characterized by anomalous venous drainage from the lung directly into the inferior vena cava. The disease may be associated with right pulmonary hypoplasia, dextrocardia, cardiac abnormalities, and systemic pulmonary collaterals. The present case report draws attention to the late discovery of a congenital heart disease, with the patient already symptomatic and with possible important hemodynamic repercussions, highlighting the importance of always evaluating the clinical picture of dyspnea and heart failure in adults during the routine investigation. the presence of a congenital heart disease.
Assuntos
Artéria Pulmonar , Síndrome de Cimitarra , Auscultação Cardíaca , Cardiopatias Congênitas , Insuficiência Cardíaca , Exame FísicoRESUMO
PURPOSE: The aim of the study was to analyze the surgical outcome of patients with total anomalous pulmonary venous connection (TAPVC) who underwent cardiac surgery. METHODS: A retrospective study was carried out. Patients with diagnosis of TAPVC undergoing cardiac surgery at the National Institute of Cardiology Ignacio Chávez, from January 1, 2003 and June 30, 2019 were included. Descriptive statistics were calculated, as well as a bivariate analysis of the variables associated with mortality. A logistic regression model was included to determine risk factors associated with the main outcome and survival was analyzed using the Kaplan-Meier method. RESULTS: A total of 5314 patients diagnosed with congenital heart disease (CHD) underwent surgery, 414 (7.8%) were patients with TAPVC, with an average age of 17.1 ± 34.6 months, 58.2% were male. It was frequent in infants (61.6%) and preschool (19.6%). Predominant type was supracardiac TAPVC (47.4%). Pulmonary venous obstruction (PVO) occurred in 32.1%. Risk factors associated with mortality were infracardiac TAPVC (odds ratio [OR]: 3.26; 95% confidence interval [CI]: 1.17-9.03; p = .02), PVO (OR: 2.56; 95% CI: 1.05-6.22; p = .03) and postoperative mechanical ventilation (OR: 1.005; 95% CI: 1.002-1.008; p = .01). Overall survival was 87.2%, with better outcomes in adolescents (100%), children (94.1%), mixed TAPVC (96%), and cardiac TAPVC (91.9%; p < .001). CONCLUSIONS: The survival of our institution after surgical correction of TAPVC is similar to that of other referral centers, where patients with infracardiac TAPVC and newborns worse outcomes. All patients must undergo a rigorous evaluation to determine an adequate repair strategy.
Assuntos
Procedimentos Cirúrgicos Cardíacos , Veias Pulmonares , Pneumopatia Veno-Oclusiva , Síndrome de Cimitarra , Adolescente , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Veias Pulmonares/anormalidades , Veias Pulmonares/cirurgia , Pneumopatia Veno-Oclusiva/diagnóstico , Pneumopatia Veno-Oclusiva/etiologia , Pneumopatia Veno-Oclusiva/cirurgia , Estudos Retrospectivos , Síndrome de Cimitarra/complicações , Resultado do TratamentoRESUMO
Abstract Pulmonary venous connections may be infrequently abnormal in patients with tetralogy of Fallot (TOF). A special subgroup of partial anomalous pulmonary venous return,"scimitar cyndrome", and its coexistence with TOF is less frequently reported. It may proceed unnoticed, as cyanosis already predominates in the clinical picture. This uncommon association must be kept in mind for patients with TOF who have an accessory flow in the inferior vena cava, especially when all pulmonary venous return to the left atrium is not clearly seen.
Assuntos
Humanos , Lactente , Veias Pulmonares , Síndrome de Cimitarra/cirurgia , Síndrome de Cimitarra/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Tetralogia de Fallot/diagnóstico por imagem , Veia Cava Inferior/diagnóstico por imagem , Átrios do CoraçãoRESUMO
Infantile scimitar syndrome (SS) is a rare congenital heart disease and has high mortality. Guidelines have not been established, but surgery is indicated in symptomatic patients. Despite the various surgical approaches, outcomes continue to be disappointing. We present our surgical experience with an infantile SS patient who had stenotic pulmonary veins contralateral to the hypoplastic lung with complicated anatomy. There are few cases with this complex pathology in the literature. Moreover, our patient was the first transplant-free survivor with this complexity in the literature.
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Cardiopatias Congênitas , Veias Pulmonares , Síndrome de Cimitarra , Estenose de Veia Pulmonar , Constrição Patológica , Humanos , Lactente , Veias Pulmonares/diagnóstico por imagem , Veias Pulmonares/cirurgia , Síndrome de Cimitarra/complicações , Síndrome de Cimitarra/diagnóstico por imagem , Síndrome de Cimitarra/cirurgia , Estenose de Veia Pulmonar/diagnóstico por imagem , Estenose de Veia Pulmonar/etiologia , Estenose de Veia Pulmonar/cirurgiaRESUMO
Abstract Infantile scimitar syndrome (SS) is a rare congenital heart disease and has high mortality. Guidelines have not been established, but surgery is indicated in symptomatic patients. Despite the various surgical approaches, outcomes continue to be disappointing. We present our surgical experience with an infantile SS patient who had stenotic pulmonary veins contralateral to the hypoplastic lung with complicated anatomy. There are few cases with this complex pathology in the literature. Moreover, our patient was the first transplant-free survivor with this complexity in the literature.
Assuntos
Humanos , Lactente , Veias Pulmonares/cirurgia , Veias Pulmonares/diagnóstico por imagem , Síndrome de Cimitarra/cirurgia , Síndrome de Cimitarra/complicações , Síndrome de Cimitarra/diagnóstico por imagem , Cardiopatias Congênitas , Constrição Patológica , Estenose de Veia Pulmonar/cirurgia , Estenose de Veia Pulmonar/etiologia , Estenose de Veia Pulmonar/diagnóstico por imagemRESUMO
Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic congenital heart disease that accounts for less than 1% of all congenital heart diseases. TAPVC is characterized by the absence of pulmonary venous drainage into the left atrium. It is underdiagnosed in utero, with prenatal detection rates of less than 1.4%. The prenatal diagnosis of TAPVC affects the postnatal outcome, particularly in obstructive forms (critical TAPVC), since planned delivery and perinatal management are mandatory. Thus, identifying the ultrasound key markers of TAPVC is important for the prenatal diagnosis and perinatal assistance. The ventricular size discrepancy (right ventricle > left ventricle) can be a useful marker. Furthermore, the increased retroatrial distance between the left atrium and the aorta could be a diagnostic marker for prenatal TAPVC, especially when the left atrium is small. Three- (3D) and four-dimensional ultrasonography may improve the prenatal diagnosis of TAPVC. This study reviews the two (2D) and 3D ultrasonographic markers used in the antenatal diagnosis of TAPVC, with a focus on the tools that can be used by sonographers, obstetricians, and fetal medicine specialists to improve the prenatal diagnosis of TAPVC, and when to refer the case to a fetal cardiac specialist.
Assuntos
Ecocardiografia Tridimensional , Diagnóstico Pré-Natal , Síndrome de Cimitarra/diagnóstico por imagem , Feminino , Átrios do Coração/diagnóstico por imagem , Ventrículos do Coração/diagnóstico por imagem , Humanos , Recém-Nascido , Gravidez , Veias Pulmonares/diagnóstico por imagem , Ultrassonografia Pré-NatalRESUMO
Pulmonary venous connections may be infrequently abnormal in patients with tetralogy of Fallot (TOF). A special subgroup of partial anomalous pulmonary venous return,"scimitar cyndrome", and its coexistence with TOF is less frequently reported. It may proceed unnoticed, as cyanosis already predominates in the clinical picture. This uncommon association must be kept in mind for patients with TOF who have an accessory flow in the inferior vena cava, especially when all pulmonary venous return to the left atrium is not clearly seen.
Assuntos
Veias Pulmonares , Síndrome de Cimitarra , Tetralogia de Fallot , Átrios do Coração , Humanos , Lactente , Síndrome de Cimitarra/diagnóstico por imagem , Síndrome de Cimitarra/cirurgia , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Veia Cava Inferior/diagnóstico por imagemRESUMO
In isolated partial anomalous pulmonary venous connections (PAPVCs), an abnormal vein connects venous blood from the pulmonary circulation to the systemic circulation, resulting in an extracardiac shunt. A single aberrant pulmonary vein (PV) is usually hemodynamically insignificant, and affected patients are generally asymptomatic. We describe a young Caribbean-Black woman with an isolated, singular PAPVC from the left inferior PV to the left innominate (brachiocephalic) vein that was hemodynamically significant, obfuscated by recurrent pleural effusions from catamenial pleural endometriosis.
Assuntos
Veias Braquiocefálicas/anormalidades , Cardiopatias Congênitas/diagnóstico por imagem , Veias Pulmonares/anormalidades , Síndrome de Cimitarra/diagnóstico por imagem , Adulto , Angiografia por Tomografia Computadorizada , Ecocardiografia Transesofagiana , Eletrocardiografia , Feminino , Cardiopatias Congênitas/cirurgia , Hemodinâmica , Humanos , Imageamento por Ressonância Magnética , Radiografia Torácica , Síndrome de Cimitarra/cirurgiaRESUMO
Scimitar syndrome is a rare congenital anomaly characterized by partial or complete anomalous pulmonary venous drainage of the right (rarely left) lung into the inferior vena cava. This anomalous vein resembles the curved Turkish sword "scimitar"[]. Only few cases were reported with two scimitar veins[]. "Myocardial bridge" constitutes a portion of the myocardial tissue that bridges a segment of the coronary artery, mostly the left anterior descending coronary artery. For the first time, a combination of double scimitar vein and a myocardial bridge was described in this study.
Assuntos
Miocárdio , Humanos , Pulmão , Veias Pulmonares , Síndrome de Cimitarra , Veia Cava InferiorRESUMO
Abstract Scimitar syndrome is a rare congenital anomaly characterized by partial or complete anomalous pulmonary venous drainage of the right (rarely left) lung into the inferior vena cava. This anomalous vein resembles the curved Turkish sword "scimitar"[1]. Only few cases were reported with two scimitar veins[2]. "Myocardial bridge" constitutes a portion of the myocardial tissue that bridges a segment of the coronary artery, mostly the left anterior descending coronary artery . For the first time, a combination of double scimitar vein and a myocardial bridge was described in this study.