RESUMO
Coração em criss-cross (ou coração entrecruzado) foi descrito pela primeira vez em 1974. Trata-se de uma malformação cardíaca congênita, rara, ocorrendo 8 casos a cada 1.000.000 de crianças, e representando somente 0,1% das malformações congênitas. Os métodos diagnósticos de escolha são o ecocardiograma transtorácico, a ressonância magnética cardíaca (RMC), a angiotomografia (TC) e, eventualmente, o cateterismo cardíaco. Neste relato, descreve-se o caso de um recém-nascido com coração em criss-cross somado à dupla via de saída do ventrículo direito (VD), com vasos mal posicionados, além de comunicação interatrial (CIA), comunicação interventricular (CIV), displasia de valva tricúspide e veia cava superior esquerda persistente. Não se sabe a etiologia exata dessa malformação, mas parece ocorrer pela rotação dos ventrículos em seu eixo longitudinal, não acompanhada das rotações atrial e das valvas atrioventriculares (AV). Esse movimento produz uma alteração das vias de entrada dos ventrículos, determinando que o VD se posicione em plano superior e o esquerdo em plano inferior. Apesar de ainda não se saber a exata causa dessa anomalia, acredita-se que uma alteração genética possa estar levando a esses casos: a mutação do gene Cx43. O diagnóstico do caso em questão foi dado pela ecocardiografia transtorácica e da TC de aorta e artérias pulmonares, que mostraram, além do criss-cross, outras alterações, como dupla via de saída do VD, CIA e CIV amplas.(AU)
Criss-cross heart was first described in 1974. It is a rare congenital heart malformation that occurs in 8 cases per 1,000,000 children, and represents only 0.1% of congenital malformations. The diagnostic methods of choice are transthoracic echocardiography, cardiac magnetic resonance (CMR), computed tomography angiography (CT) and, sometimes, cardiac catheterization. This report describes the case of a newborn with a criss-cross heart in addition to double-outlet right ventricle (RV), with poorly positioned vessels, in addition to atrial septal defect (ASD), interventricular septal defect, tricuspid valve dysplasia and persistent left superior vena cava. The exact etiology of this malformation is not known, but it seems to occur due to rotation of the ventricles in their longitudinal axis, not accompanied by rotation of the atrial and atrioventricular (AV) valves. This movement produces abnormal ventricular inlets, determining that the RV be positioned on a superior plane and the left ventricle on an inferior plane. Although the exact cause of this anomaly is still unknown, it is believed that a genetic abnormality may be leading to these cases: mutation of the Cx43 gene. Diagnosis of the case concerned was given by transthoracic echocardiography and computed CT of the aorta and pulmonary arteries, which showed, in addition to the criss-cross heart, other abnormalities, such as double-outlet RV, large ASD and ventricular septal defect (VSD).(AU)
Assuntos
Humanos , Masculino , Recém-Nascido , Coração Entrecruzado/etiologia , Coração Entrecruzado/diagnóstico por imagem , Cardiopatias Congênitas/diagnóstico , Ventrículos do Coração/anormalidades , Dupla Via de Saída do Ventrículo Direito/diagnóstico , Ecocardiografia/métodos , Cateterismo Cardíaco/métodos , Espectroscopia de Ressonância Magnética/métodos , Tomografia Computadorizada por Raios X/métodos , Veia Cava Superior Esquerda Persistente/diagnóstico , Comunicação Interatrial/diagnósticoRESUMO
OBJECTIVES: Taussig-Bing anomaly (TBA) and transposition of the great arteries (TGA) with hypoplastic or interrupted aortic arch (AA) are rare anomalies. Various operative techniques and a high incidence of reinterventions are described. The aim of this retrospective single-centre study was to evaluate operative data, mortality and reintervention rate with special regard to the AA. METHODS: At the Children's Heart Center Linz, 50 patients with the above-mentioned diagnosis have been corrected by a simultaneous repair between 2001 and 2022. Thirty-seven children had TBA, 13 had TGA and 5 of them had an interrupted AA. The median age at operation was 7 [interquartile range (IQR) 5-9] days, weight 3.38 (IQR 2.9-3.8) kg and follow-up 9.3 (IQR 3.1-14.5) years. The AA reconstruction was performed without patch material in 49 cases. RESULTS: There was 1 in-hospital mortality in a TBA patient and 1 late mortality (7 years later, neuroblastoma). 14/49 patients needed at least 1 reoperation (28.6%, all TBA) and 3 further patients had catheter reintervention or radiofrequency ablation only (6.1%, 2 TBA). Seventy-five percent of these procedures affected the right heart/pulmonary arteries; there was 1 re-coarctation repair. CONCLUSIONS: The simultaneous correction of TBA and TGA with AA obstruction or interruption is a safe operation with very low mortality. The AA reconstruction with minimized use of patch material resulted in a low restenosis rate.
Assuntos
Coartação Aórtica , Transposição das Grandes Artérias , Dupla Via de Saída do Ventrículo Direito , Transposição dos Grandes Vasos , Criança , Humanos , Lactente , Recém-Nascido , Transposição das Grandes Artérias/efeitos adversos , Aorta Torácica/cirurgia , Seguimentos , Estudos Retrospectivos , Resultado do Tratamento , Dupla Via de Saída do Ventrículo Direito/diagnóstico , Dupla Via de Saída do Ventrículo Direito/cirurgia , Coartação Aórtica/cirurgia , ReoperaçãoRESUMO
Hearts with double outlet ventricles and concordant atrioventricular connections account for about 1%-3% of all cases of congenital heart disease. We review hearts with two ventricles and concordant atrioventricular connections with double outlet right ventricle (DORV), double outlet left ventricle (DOLV) and double outlet both ventricles (DOBV) from the morphological and clinical imaging perspectives. These hearts are a heterogeneous group of congenital cardiac malformations with a wide range of pathophysiologies that require an individualised surgical approach based on a precise understanding of the complex cardiovascular anatomy. Owing to their differing temporal, spatial and contrast resolutions, we propose that multimodality imaging provides optimal characterisation of various intracardiac morphological features of double outlet hearts. This approach aids clinical diagnosis for optimising treatment options across these malformations.
Assuntos
Dupla Via de Saída do Ventrículo Direito , Cardiopatias Congênitas , Humanos , Dupla Via de Saída do Ventrículo Direito/diagnóstico , Dupla Via de Saída do Ventrículo Direito/cirurgia , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração , Ecocardiografia , Imagem MultimodalRESUMO
The case was a 53-year-old woman. At birth, she was diagnosed with a false Taussig-Bing anomaly with pulmonary artery stenosis and a single ventricle. However, no cardiac surgery was performed, and conservative treatment was continued by a cardiovascular surgeon even after adulthood. Because of secondary polycythemia and a history of multiple cerebral infarctions, she took anti-platelet drugs and anti-coagulants. However, she was admitted with the diagnosis of cerebral infarction for the fourth time. It was considered that the patient was at high risk of paradoxical cerebral embolism due to cardiac malformation with cyanotic congenital heart disease accompanied by coagulation abnormalities. Considering the pathophysiology, we decided to use aspirin in combination with warfarin.
Assuntos
Procedimentos Cirúrgicos Cardíacos , Dupla Via de Saída do Ventrículo Direito , Policitemia , Recém-Nascido , Feminino , Adulto , Humanos , Pessoa de Meia-Idade , Dupla Via de Saída do Ventrículo Direito/complicações , Dupla Via de Saída do Ventrículo Direito/diagnóstico , Dupla Via de Saída do Ventrículo Direito/cirurgia , Infarto Cerebral/etiologia , Infarto Cerebral/complicações , Policitemia/complicações , PacientesAssuntos
Aneurisma da Aorta Torácica/complicações , Coartação Aórtica/complicações , Dissecção Aórtica/etiologia , Dupla Via de Saída do Ventrículo Direito/complicações , Dupla Via de Saída do Ventrículo Direito/diagnóstico , Imagem Multimodal/métodos , Artéria Pulmonar , Adulto , Dissecção Aórtica/diagnóstico , Aorta Torácica/diagnóstico por imagem , Aneurisma da Aorta Torácica/diagnóstico , Coartação Aórtica/diagnóstico , Angiografia por Tomografia Computadorizada , Ecocardiografia/métodos , Eletrocardiografia , Humanos , Imageamento Tridimensional/métodos , MasculinoRESUMO
A 6-month-old, neutered male, Vietnamese potbellied pig presented for evaluation of exercise intolerance and intermittent episodes of exertional cyanosis. Initial diagnostic evaluation revealed arterial hypoxemia. Transthoracic echocardiogram revealed double-outlet right ventricle (DORV) and a subaortic ventricular septal defect. Agitated saline contrast study confirmed the entry of saline contrast from the right ventricle into both pulmonary artery and aorta. Due to deterioration of clinical status, the patient was euthanized 3 months later. Gross necropsy examination was performed confirming the congenital cardiac defects noted on the echocardiogram. To the authors knowledge, this is the first case report of DORV in a Vietnamese potbellied pig.
Assuntos
Dupla Via de Saída do Ventrículo Direito/veterinária , Doenças dos Suínos/diagnóstico , Animais , Diagnóstico Diferencial , Dupla Via de Saída do Ventrículo Direito/diagnóstico , Ecocardiografia Transesofagiana/veterinária , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/veterinária , Masculino , Linhagem , Suínos , Doenças dos Suínos/diagnóstico por imagemRESUMO
Atrioventricular (AV) discordance and ventriculoarterial (VA) concordance in the setting of visceral situs inversus are one of the rarest forms of cardiac malformations. To our knowledge, this is the first reported case of prenatal diagnosis of such rare cardiac anatomy in association with double-outlet right ventricle on fetal echocardiography. The physiology of this cardiac anomaly is similar to that of transposition of the great arteries, and the best surgical option is the atrial switch operation.
Assuntos
Dupla Via de Saída do Ventrículo Direito/diagnóstico , Situs Inversus/diagnóstico , Adulto , Transposição das Grandes Artérias/métodos , Dupla Via de Saída do Ventrículo Direito/cirurgia , Ecocardiografia , Feminino , Coração Fetal/diagnóstico por imagem , Átrios do Coração/anormalidades , Ventrículos do Coração/anormalidades , Humanos , Recém-Nascido , Masculino , Gravidez , Diagnóstico Pré-Natal , Situs Inversus/cirurgia , Transposição dos Grandes Vasos/diagnóstico , Transposição dos Grandes Vasos/cirurgia , Resultado do TratamentoRESUMO
BACKGROUND: The double outlet right ventricle is uncommon and usually makes patients have haemodynamic and structural complications. Having a hyperdynamic state, such as pregnancy, with volume overload is very risky for a patient with complex CHD (CCHD). The diagnosis in early stages can prevent cardiac complications. The multi-disciplinary assessment of the disease lets patients make choices in treatment and reproductive life. OBJECTIVE: Present a case of a successful pregnancy in a patient with a rare CCHD. PARTICIPANT: A pregnant 19-year-old patient with a double outlet right ventricle without haemodynamic or structural complications and no fetal abnormalities.
Assuntos
Dupla Via de Saída do Ventrículo Direito/diagnóstico , Ecocardiografia/métodos , Complicações Cardiovasculares na Gravidez , Ultrassonografia Pré-Natal/métodos , Feminino , Humanos , Recém-Nascido , Masculino , Gravidez , Resultado da Gravidez , Adulto JovemRESUMO
We describe the anatomic findings in a 2-year-old patient with double outlet right ventricle with right-sided aorta in the setting of usual atrial arrangement and discordant atrioventricular connections, making comparison with a specimen from the pathological archive of the Birmingham Children's Hospital in the United Kingdom having this rare combination of anatomic features. We discuss the challenges involved in diagnosis and management.
Assuntos
Anormalidades Múltiplas , Aorta Torácica/anormalidades , Transposição das Grandes Artérias Corrigida Congenitamente/diagnóstico , Dupla Via de Saída do Ventrículo Direito/diagnóstico , Ventrículos do Coração/diagnóstico por imagem , Aorta Torácica/diagnóstico por imagem , Procedimentos Cirúrgicos Cardíacos/métodos , Pré-Escolar , Transposição das Grandes Artérias Corrigida Congenitamente/cirurgia , Dupla Via de Saída do Ventrículo Direito/cirurgia , Ecocardiografia , Feminino , Ventrículos do Coração/anormalidades , Humanos , Tomografia Computadorizada por Raios XRESUMO
We present a case of a highly unusual congenital cardiac malformation, namely, triple outlet right ventricle with duplication of the aortic root and the intrapericardial component of the ascending aorta. A girl, aged five, presented with complaints of cyanosis and effort intolerance and was diagnosed with double-outlet right ventricle and subpulmonary infundibular stenosis. Intraoperatively, we noted that the aortic root was guarded by two separate aortic valves, oriented anteroposteriorly relative to each other, and separated within the right ventricle by a muscle bar. Postoperative interrogation by both echocardiography and computed tomography confirmed the surgical findings. To the best of our knowledge, our case is the first example of duplication of the aortic root to produce triple outlet right ventricle.
Assuntos
Anormalidades Múltiplas , Aorta/anormalidades , Procedimentos Cirúrgicos Cardíacos/métodos , Dupla Via de Saída do Ventrículo Direito/diagnóstico , Ventrículos do Coração/anormalidades , Aorta/diagnóstico por imagem , Pré-Escolar , Dupla Via de Saída do Ventrículo Direito/cirurgia , Ecocardiografia , Feminino , Ventrículos do Coração/diagnóstico por imagem , Humanos , Tomografia Computadorizada por Raios XAssuntos
Cardiomegalia/fisiopatologia , Dupla Via de Saída do Ventrículo Direito/fisiopatologia , Permeabilidade do Canal Arterial/fisiopatologia , Fluxo Pulsátil/fisiologia , Parede Torácica , Aorta Torácica/fisiopatologia , Cardiomegalia/diagnóstico , Cardiomegalia/diagnóstico por imagem , Dupla Via de Saída do Ventrículo Direito/diagnóstico , Permeabilidade do Canal Arterial/diagnóstico , Humanos , Recém-Nascido , Exame Físico , Pulso ArterialRESUMO
Congenital aneurysm of the left atrium is a rare cardiac anomaly, most commonly detected between the 2nd and 4th decades of life in a symptomatic patient. We report a congenital aneurysm of the left atrium diagnosed at 24 weeks of gestational age, associated with other congenital heart diseases and 47XY, +18 karyotype. The literature of the left atrial aneurysm diagnosed by fetal echocardiography is also reviewed in this report.
Assuntos
Dupla Via de Saída do Ventrículo Direito/diagnóstico , Ecocardiografia/métodos , Coração Fetal/diagnóstico por imagem , Aneurisma Cardíaco/congênito , Átrios do Coração/anormalidades , Síndrome da Trissomía do Cromossomo 18 , Ultrassonografia Pré-Natal/métodos , Anormalidades Múltiplas , Adulto , Diagnóstico Diferencial , Dupla Via de Saída do Ventrículo Direito/embriologia , Evolução Fatal , Feminino , Idade Gestacional , Aneurisma Cardíaco/diagnóstico , Aneurisma Cardíaco/embriologia , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/embriologia , Humanos , GravidezRESUMO
Double-chambered left ventricle is a rare congenital heart defect. The clinical features, diagnosis, treatment and patient prognosis of this lesion have not been sufficiently elaborated. The present systematic review found that the accessory ventricular septum was often an abnormal muscle band, and sometimes a membranous structure, a fibromuscular ridge, or prominent trabeculations. Less than one-third of the patients are associated with other congenital heart defects. Diagnosis of double-chambered left ventricle can usually be made by transthoracic echocardiography; however, it might be misdiagnosed as atrial or ventricular septal defect. The accessory chamber was often smaller than the main chamber, and often has wall thinning. The accessory chamber wall dysfunction/hypokinesis was seen in half of the cases. One-third patients had left ventricular outflow tract obstruction. The adult patients showed abnormal electrocardiographic findings more than in pediatrics. Nevertheless, no differences were found between adult and pediatric patients in terms of left ventricular function and structure, treatment of choice, and patient outcomes. Most patients are asymptomatic with no left ventricular obstruction, and thus do not need surgical treatment. Surgical resection of the accessory ventricular septum is warranted when the patients become symptomatic as a result of left ventricular obstruction, or associated with other congenital heart defects. The patients' outcomes are promising. Differential diagnosis should be made from other types of left ventricular outpouching, other congenital heart defects, and left ventricular non-compaction.
Assuntos
Dupla Via de Saída do Ventrículo Direito/diagnóstico , Dupla Via de Saída do Ventrículo Direito/cirurgia , Adulto , Criança , Diagnóstico Diferencial , Ecocardiografia , Eletrocardiografia , Humanos , Prognóstico , Obstrução do Fluxo Ventricular Externo/diagnóstico , Obstrução do Fluxo Ventricular Externo/cirurgia , Septo Interventricular/cirurgiaRESUMO
CHDs occasionally have coronary complications; however, stent implantation is technically difficult in small children. We reported a successful drug-eluting stent implantation into the congenital anomalous coronary artery in a 6-year-old boy. This treatment is useful for rescuing coronary stenosis, and dual antiplatelet therapy is important to prevent stent restenosis.
Assuntos
Valva Aórtica/cirurgia , Estenose Coronária/cirurgia , Anomalias dos Vasos Coronários/complicações , Dupla Via de Saída do Ventrículo Direito/cirurgia , Stents Farmacológicos , Everolimo/farmacologia , Intervenção Coronária Percutânea/métodos , Criança , Angiografia Coronária , Estenose Coronária/diagnóstico , Estenose Coronária/etiologia , Anomalias dos Vasos Coronários/diagnóstico , Anomalias dos Vasos Coronários/cirurgia , Dupla Via de Saída do Ventrículo Direito/diagnóstico , Ecocardiografia , Próteses Valvulares Cardíacas , Humanos , Imunossupressores/farmacologia , Masculino , ReoperaçãoRESUMO
Hearts with double outlet right ventricle are a heterogeneous group of malformations in which a comprehensive diagnostic approach is required for tailored surgical management. This pictorial essay revisits essential modifiers of clinical and surgical importance in management of the patients with double outlet right ventricle using 3-dimensional volume-rendered endocardial surface images and 3-dimensional print models. Special emphasis is paid to the infundibular morphology, including the size and orientation of the outlet septum, relative to the margin of the ventricular septal defect, and the extent of the muscular infundibulum as an additional modifier of the distance between the ventricular septal defect margin and the arterial valve or valves.
Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Dupla Via de Saída do Ventrículo Direito/diagnóstico , Endocárdio/diagnóstico por imagem , Ventrículos do Coração/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética/métodos , Impressão Tridimensional , Dupla Via de Saída do Ventrículo Direito/cirurgia , Endocárdio/cirurgia , Ventrículos do Coração/anormalidades , Ventrículos do Coração/cirurgia , Humanos , Imageamento TridimensionalRESUMO
OBJECTIVE: To discuss the key anatomic features of double outlet right ventricle (DORV) assessed by preoperative echocardiography among patients treated with different types of biventricular repair. METHODS: Surgical and echocardiographic databases were queried to identify patients who had undergone biventricular repair for DORV and had adequate preoperative echocardiographic imaging. All patients underwent pre- and postoperative echocardiography and clinical evaluation following discharge. RESULTS: Two hundred sixty-two patients with DORV met the inclusion criteria of the study. The patients were divided into two groups-intraventricular tunnel repair (IVR) to the aorta (194 [74%] patients) or to the pulmonary artery with either concomitant arterial switch operation or double-root translocation (68 [26%] patients). Among 68 patients undergoing IVR to the pulmonary artery, 50 patients with transposition of the great arteries (TGA) type of DORV and 7 patients with remote ventricular septal defect (VSD) type underwent IVR plus arterial switch operation and 6 patients with TGA type and 5 patients with remote VSD type underwent IVR plus double-root translocation. There were three hospital deaths and one late death (overall operative mortality: 1.5%). CONCLUSION: Preoperative echocardiography provided crucial data to estimate the feasibility of intraventricular tunnel creation to either the aorta or the pulmonary artery and to guide the selection of either arterial switch or double-root translocation. Biventricular repair could be achieved with favorable outcomes in most patients with DORV.
Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Dupla Via de Saída do Ventrículo Direito/cirurgia , Ecocardiografia/métodos , Ventrículos do Coração/cirurgia , Pré-Escolar , Dupla Via de Saída do Ventrículo Direito/diagnóstico , Feminino , Ventrículos do Coração/diagnóstico por imagem , Humanos , Lactente , Masculino , Período Pré-Operatório , Resultado do TratamentoAssuntos
Anormalidades Múltiplas/diagnóstico , Aorta Torácica/anormalidades , Dupla Via de Saída do Ventrículo Direito/diagnóstico , Permeabilidade do Canal Arterial/diagnóstico , Anormalidades Múltiplas/embriologia , Aorta Torácica/embriologia , Dupla Via de Saída do Ventrículo Direito/embriologia , Permeabilidade do Canal Arterial/embriologia , Feminino , Humanos , LactenteRESUMO
Through a series of complex surgical and interventional procedures including downsizing of an unrestricted atrial septal defect, aortic arch reconstruction, pulmonary artery banding, reversed Potts shunt, and Melody valve in a mitral position, biventricular conversion was successfully achieved in a patient with a distinct borderline left ventricle. By use of these interventional steps, flow-mediated and load-mediated growth of a small left ventricle was observed, highlighting the plasticity of hearts in growing children. Surgical and catheter interventional teamwork may lead to fascinating results, provoking us to quote Aristotle's wisdom: "The whole is more than the sum of its parts."
Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Dupla Via de Saída do Ventrículo Direito/cirurgia , Ventrículos do Coração/cirurgia , Cateterismo Cardíaco , Dupla Via de Saída do Ventrículo Direito/diagnóstico , Ventrículos do Coração/anormalidades , Ventrículos do Coração/diagnóstico por imagem , Humanos , Recém-Nascido , Imagem Cinética por Ressonância Magnética , Masculino , Artéria Pulmonar/cirurgiaRESUMO
OBJECTIVES: Double-outlet right ventricle is a form of ventriculoarterial connection. The definition formulated by the International Society for Nomenclature of Paediatric and Congenital Heart Disease is based on hearts with both arterial trunks supported in their greater part by a morphologically right ventricle. Bilateral infundibula and ventricular septal defects are highly debated criteria. This study examines the anatomic controversies surrounding double-outlet right ventricle. We show that hearts with double-outlet right ventricle can have atrioventricular-to-arterial valvular continuity. We emphasize the difference between the interventricular communication and the zone of deficient ventricular septation. METHODS: The hearts examined were from the University of Florida in Gainesville; Johns Hopkins All Children's Hospital, St Petersburg, Fla; and Lurie Children's Hospital, Chicago, Ill. Each specimen had at least 75% of both arterial roots supported by the morphologically right ventricle, with a total of 100 hearts examined. The morphologic method was used to assess anatomic features, including arterial-atrioventricular valvular continuity, subarterial infundibular musculature, and the location of the hole between the ventricles. RESULTS: Most hearts had fibrous continuity between one of the arterial valves and an atrioventricular valve, with bilateral infundibula in 23%, and intact ventricular septum in 5%. CONCLUSIONS: Bilateral infundibula are not a defining feature of double-outlet right ventricle, representing only 23% of the specimens in our sample. The interventricular communication can have a posteroinferior muscular rim or extend to become perimembranous (58%). Double-outlet right ventricle can exist with an intact ventricular septum.