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2.
World J Pediatr Congenit Heart Surg ; 11(1): 123-126, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31755355

RESUMO

Enlargement of the bulboventricular foramen (BVF) in double-inlet left ventricle or the ventricular septal defect (VSD) in tricuspid atresia with transposition of the great arteries is one approach for prevention or treatment of systemic ventricular outflow obstruction. Most often, BVF/VSD restriction is bypassed preemptively or addressed directly at the time of Glenn/Fontan procedures as part of staged univentricular palliation. We describe a patient who underwent enlargement of a restrictive VSD during Fontan completion and subsequently presented with an asymptomatic pseudoaneurysm of the right ventricle at the ventriculotomy site.


Assuntos
Falso Aneurisma/diagnóstico , Técnica de Fontan , Ventrículos do Coração , Falso Aneurisma/diagnóstico por imagem , Falso Aneurisma/cirurgia , Pré-Escolar , Diagnóstico Diferencial , Ecocardiografia , Comunicação Interventricular/complicações , Comunicação Interventricular/cirurgia , Humanos , Masculino , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/diagnóstico por imagem , Complicações Pós-Operatórias/cirurgia , Transposição dos Grandes Vasos/complicações , Transposição dos Grandes Vasos/cirurgia , Atresia Tricúspide/complicações , Atresia Tricúspide/cirurgia
3.
Cardiol Young ; 30(1): 126-128, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31769381

RESUMO

Tricuspid atresia with absent pulmonary valve and intact ventricular septum is an extremely rare cardiac malformation, historically associated with a poor prognosis. Only a few cases with successful surgical palliation have been reported in the literature. We present the case of an 8-month-old infant with this malformation who underwent successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle.


Assuntos
Técnica de Fontan/métodos , Valva Pulmonar/anormalidades , Atresia Tricúspide/complicações , Septo Interventricular , Ponte Cardiopulmonar , Ecocardiografia Doppler , Ecocardiografia Tridimensional , Humanos , Hipertrofia Ventricular Direita/diagnóstico , Hipertrofia Ventricular Direita/etiologia , Lactente , Masculino , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/cirurgia , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Atresia Tricúspide/diagnóstico , Atresia Tricúspide/cirurgia
4.
Pediatr Cardiol ; 40(8): 1761-1762, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31506732

RESUMO

We here report a neonate with prenatal echocardiographic diagnosis of tricuspid atresia, with normally related great vessels, and large ventricular septal defect. This diagnosis could be confirmed with echocardiography at birth. An additional double mitral orifice was also seen. This is a very rare association.


Assuntos
Valva Mitral/anormalidades , Atresia Tricúspide/complicações , Ecocardiografia , Feminino , Humanos , Recém-Nascido , Valva Mitral/diagnóstico por imagem , Diagnóstico Pré-Natal , Atresia Tricúspide/diagnóstico
5.
World J Pediatr Congenit Heart Surg ; 10(5): 635-637, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31496421

RESUMO

Coexistence of tricuspid atresia and truncus arteriosus (common arterial trunk) is an extremely rare combination of anomalies and requires staged palliation. Initial palliation involves disconnection of the pulmonary arteries from the common trunk and creation of a systemic-to-pulmonary artery shunt. This has generally been accomplished with the use of cardiopulmonary bypass. We describe a technique of off-pump palliation using the common trunk and a modified Blalock-Taussig shunt as sources of pulmonary blood flow at various stages of reconstruction. The child has recovered well and has undergone second-stage palliation with bilateral bidirectional superior cavopulmonary anastomoses at one year of age.


Assuntos
Procedimento de Blalock-Taussig , Ponte de Artéria Coronária sem Circulação Extracorpórea , Artéria Pulmonar/cirurgia , Atresia Tricúspide/cirurgia , Tronco Arterial/cirurgia , Anastomose Cirúrgica , Angiografia por Tomografia Computadorizada , Humanos , Imageamento Tridimensional , Lactente , Masculino , Cuidados Paliativos , Perfusão , Artéria Pulmonar/diagnóstico por imagem , Atresia Tricúspide/complicações , Atresia Tricúspide/diagnóstico por imagem , Tronco Arterial/diagnóstico por imagem
6.
Autops. Case Rep ; 9(3): e2019118, July-Sept. 2019. ilus
Artigo em Inglês | LILACS | ID: biblio-1017367

RESUMO

Acute obstruction of superior vena cava anastomosis right after the Glenn procedure may lead to tragic consequences. We describe the case of a one-year-old child with tricuspid atresia and a previous Blalock-Taussig shunt procedure, who presented severe low cardiac output syndrome right after the Glenn procedure and died forty-four hours after the procedure. The autopsy showed obstruction of the superior vena cava anastomosis. Patients that present superior vena cava syndrome and low cardiac output right after the Glenn procedure should have the surgical anastomosis revised immediately.


Assuntos
Humanos , Feminino , Recém-Nascido , Derivação Cardíaca Direita , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/patologia , Autopsia , Evolução Fatal , Atresia Tricúspide/complicações , Procedimento de Blalock-Taussig/efeitos adversos
7.
Cardiol Young ; 29(9): 1211-1213, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31385567

RESUMO

A 42-year-old man was referred to our hospital with heart failure and unoperated tricuspid atresia with pulmonary valve stenosis. His condition was initially managed with medical therapy; however, he required repeat hospitalisations for congestive heart failure. We diagnosed the chief cause of his heart failure as aortic valve regurgitation secondary to aortic root dilatation. Aortic root replacement was performed and then his heart failure was controlled.


Assuntos
Aorta Torácica/cirurgia , Prótese Vascular , Procedimentos Cirúrgicos Cardíacos/métodos , Atresia Tricúspide/cirurgia , Adulto , Aorta Torácica/diagnóstico por imagem , Ecocardiografia , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/cirurgia , Humanos , Masculino , Tomografia Computadorizada por Raios X , Atresia Tricúspide/complicações , Atresia Tricúspide/diagnóstico
8.
Heart Surg Forum ; 22(3): E213-E214, 2019 05 08.
Artigo em Inglês | MEDLINE | ID: mdl-31237545

RESUMO

BACKGROUND: Increased pulmonary vascular resistance index (PVR) leads to several complications in patients after a Fontan operation. This increase is mainly attributed to the overexpression of endothelin-1 for a long duration after the Fontan procedure. Here, we describe the case of a 3-year-old boy with a failed Fontan operation who was treated with bosentan, an endothelin-1 receptor blocker. CASE REPORT: Cardiac catheterization was performed, which showed a main pulmonary artery pressure (MPAP) of 19 mmHg and PVRI of 5.6 woods/m2. Oral bosentan regimen at a dose of 31.25 mg was initiated twice a day. The treatment was continued as pleural effusion and ascites persisted. No adverse events were observed, and the treatment was well tolerated. Pleural effusion disappeared, and ascites decreased markedly after 4 weeks, whereas the MPAP was 15 mmHg and the PVRI was 4.3 woods/m2. After 3 months of bosentan therapy, the MPAP was 12 mmHg and the PVRI was 4.1 woods/m2. CONCLUSION: We observed that bosentan reduces the PVRI and complications such as pleural effusion and ascites after a failed Fontan procedure.


Assuntos
Bosentana/uso terapêutico , Antagonistas dos Receptores de Endotelina/uso terapêutico , Técnica de Fontan/efeitos adversos , Complicações Pós-Operatórias/tratamento farmacológico , Atresia Tricúspide/cirurgia , Resistência Vascular , Pré-Escolar , Humanos , Masculino , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/fisiopatologia , Falha de Tratamento , Atresia Tricúspide/complicações , Atresia Tricúspide/fisiopatologia
9.
Medicine (Baltimore) ; 98(6): e14320, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30732151

RESUMO

RATIONALE: Atrioventricular reentrant tachycardia (AVRT) is the most common supraventricular tachycardia occurring in children. However, in complex congenital heart disease patients with a different heart anatomy and conduction system morphology, accessory pathway modification may be particularly challenging because of distortion of typical anatomic landmarks. PATIENT CONCERNS: A 10-year-old boy with tricuspid atresia and history of bidirectional Glenn operation had recurrent chest distress and palpitation for 3 months. He had multiple hospitalizations for narrow-QRS tachycardia with poor hemodynamic tolerance, despite the use of adenosine and amiodarone. DIAGNOSES: AVRT. Tricuspid atresia with secundum atrial septal defect, large ventricular septal defect, and right ventricular outflow tract stenosis. INTERVENTIONS: Cardiac catheterization, electrophysiological examination, and ablation. OUTCOMES: The child has not had a recurrent AVRT during 6 months of follow-up and is waiting for Fontan operation. LESSONS: Since there is an increased risk of accessory pathways in patients with tricuspid atresia, all these patients should be checked before the Fontan operation to exclude congenital accessory pathways.


Assuntos
Ablação por Cateter , Taquicardia por Reentrada no Nó Atrioventricular/cirurgia , Atresia Tricúspide/cirurgia , Criança , Humanos , Masculino , Taquicardia por Reentrada no Nó Atrioventricular/complicações , Taquicardia por Reentrada no Nó Atrioventricular/diagnóstico , Atresia Tricúspide/complicações , Atresia Tricúspide/diagnóstico
10.
Echocardiography ; 36(4): 800-802, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30693549

RESUMO

Restrictive atrial communication is rarely reported in tricuspid atresia but when present it can lead to important morbidity. We describe two fetuses with tricuspid atresia with restrictive foramen ovale who were found to have fetal growth failure. Fetal echocardiography detected a restrictive atrial communication by flow acceleration on color Doppler and significant right atrial dilation in one patient; the atrial septum was not well interrogated in the other patient. Restrictive foramen ovale in tricuspid atresia may be associated with fetal growth failure. Color Doppler interrogation of the atrial septum on fetal echocardiogram may help identify this condition prenatally.


Assuntos
Ecocardiografia Doppler em Cores/métodos , Retardo do Crescimento Fetal/etiologia , Comunicação Interatrial/complicações , Comunicação Interatrial/diagnóstico por imagem , Atresia Tricúspide/complicações , Ultrassonografia Pré-Natal/métodos , Feminino , Retardo do Crescimento Fetal/diagnóstico por imagem , Forame Oval/diagnóstico por imagem , Humanos , Gravidez , Atresia Tricúspide/diagnóstico por imagem
12.
Ann Thorac Surg ; 107(4): e259-e261, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30315794

RESUMO

Patients with tricuspid atresia and ventricular septal defect have in the past occasionally undergone a Fontan with "Björk" modification to create a connection between the right atrium and the right ventricular outflow tract. Although rarely performed now, patients with this physiology often face severe complications requiring reintervention. We hypothesize that surgical conversion to a 2-ventricle or 1.5-ventricle circulation can improve hemodynamics, clinical status, and thus increase time to transplant. We present 2 successful cases to illustrate this idea.


Assuntos
Procedimento de Blalock-Taussig/efeitos adversos , Técnica de Fontan/métodos , Comunicação Interventricular/cirurgia , Hemodinâmica/fisiologia , Reoperação/métodos , Atresia Tricúspide/cirurgia , Anormalidades Múltiplas/diagnóstico por imagem , Anormalidades Múltiplas/cirurgia , Adulto , Procedimento de Blalock-Taussig/métodos , Ecocardiografia Tridimensional/métodos , Seguimentos , Átrios do Coração/cirurgia , Comunicação Interventricular/complicações , Comunicação Interventricular/diagnóstico por imagem , Ventrículos do Coração/cirurgia , Humanos , Imagem Cinética por Ressonância Magnética/métodos , Masculino , Medição de Risco , Resultado do Tratamento , Atresia Tricúspide/complicações , Atresia Tricúspide/diagnóstico por imagem
13.
BMC Cardiovasc Disord ; 17(1): 131, 2017 05 22.
Artigo em Inglês | MEDLINE | ID: mdl-28532389

RESUMO

BACKGROUND: The Fontan circulation, a result of a palliative procedure in patients with single systemic ventricles, is defined by chronically elevated pulmonary vascular resistance. When traditional heart failure therapies fail, pharmacological agents that reduce pulmonary artery pressures may be used. These include endothelial-receptor antagonists, prostanoids and phosphodiesterase type 5 inhibitors. We report the first use of macitentan, an endothelin-receptor antagonist, in a patient with a Fontan circulation. CASE PRESENTATION: We describe the case of a 50 year old female with tricuspid atresia and transposition of the great arteries. Following complex surgery as a child, she subsequently underwent a fenestrated modified atrial pulmonary Fontan operation which was later converted to a total cavopulmonary anastomosis Fontan circulation. Due to failure of various medications to relieve her worsening symptoms, she was commenced on macitentan in April 2016. Few months later, she demonstrated a significant symptomatic improvement and associated increase in her incremental shuttle walking test distance. CONCLUSIONS: Macitentan has slower receptor dissociation kinetics compared to other endothelin-receptor antagonists, leading to enhanced pharmacological activity with promising effects in patients with pulmonary arterial hypertension. The patient we report has shown considerable improvement in exercise capacity following introduction of this medication and thus we suggest further randomised trials to establish the role of different endothelin-receptor antagonists in the management of the Fontan circulation.


Assuntos
Antagonistas dos Receptores de Endotelina/uso terapêutico , Técnica de Fontan/efeitos adversos , Hipertensão Pulmonar/tratamento farmacológico , Artéria Pulmonar/efeitos dos fármacos , Pirimidinas/uso terapêutico , Sulfonamidas/uso terapêutico , Transposição dos Grandes Vasos/cirurgia , Atresia Tricúspide/cirurgia , Pressão Arterial/efeitos dos fármacos , Tolerância ao Exercício/efeitos dos fármacos , Feminino , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Pessoa de Meia-Idade , Cuidados Paliativos , Artéria Pulmonar/fisiopatologia , Circulação Pulmonar/efeitos dos fármacos , Recuperação de Função Fisiológica , Transposição dos Grandes Vasos/complicações , Resultado do Tratamento , Atresia Tricúspide/complicações
14.
Cardiol Young ; 27(5): 990-992, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28077189

RESUMO

Cardiac haemangiomas are exceedingly rare; however, they can cause significant haemodynamic impairment and disturbances in heart rhythm. Rarely, cardiac tumours may also coexist with congenital heart lesions. We present an extremely unusual case of a cardiac haemangioma in the setting of complex transposition of the great arteries that caused functional tricuspid atresia. To our knowledge, this is the first such case described in the literature.


Assuntos
Neoplasias Cardíacas/complicações , Hemangioma/complicações , Atresia Pulmonar/complicações , Transposição dos Grandes Vasos/complicações , Atresia Tricúspide/complicações , Ecocardiografia Doppler em Cores , Evolução Fatal , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/patologia , Hemangioma/diagnóstico por imagem , Hemangioma/patologia , Humanos , Recém-Nascido , Masculino , Atresia Pulmonar/diagnóstico por imagem , Transposição dos Grandes Vasos/diagnóstico por imagem , Atresia Tricúspide/diagnóstico por imagem
15.
Rev. centroam. obstet. ginecol ; 21(2): 43-46, abr.-jun. 2016. ilus
Artigo em Espanhol | LILACS | ID: biblio-869636

RESUMO

La atresia tricuspidea es una cardiopatía rara caracterizada por oclusión de la válvula tricúspide y asociada a hipoplasia de ventrículo derecho y estenosis pulmonar. Clásicamente se presenta con un defecto en septun ventricular. El diagnóstico puede establecerse con ecocardiografía fetal y usualmente se sospecha ante una imagen de cuatro cámaras anormal.


Assuntos
Recém-Nascido , Atresia Tricúspide/complicações , Atresia Tricúspide/diagnóstico , Cardiopatias Congênitas/diagnóstico , Diagnóstico Pré-Natal
16.
Ann Thorac Surg ; 101(6): 2373-5, 2016 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-27211949

RESUMO

Hemoglobin SC (HbSC) disease is a hemoglobinopathy that may produce sickling under conditions of hypoxemia, dehydration, and acidosis. We present a case of HbSC disease and tricuspid atresia, type IB. We describe management by cardiopulmonary bypass CPB using exchange transfusion at initiation of bypass and fractionation of collected blood, allowing platelet and plasma apheresis, as an option for patients unable to undergo this procedure off pump.


Assuntos
Ponte Cardiopulmonar/métodos , Doença da Hemoglobina SC/complicações , Atresia Tricúspide/cirurgia , Anticoagulantes/administração & dosagem , Transfusão de Sangue Autóloga , Pré-Escolar , Cianose , Transfusão Total , Feminino , Técnica de Fontan , Parada Cardíaca Induzida , Heparina/administração & dosagem , Humanos , Hipotermia Induzida , Cuidados Paliativos , Plasmaferese , Plaquetoferese , Cuidados Pré-Operatórios , Atresia Tricúspide/complicações
17.
J Card Surg ; 31(4): 237-41, 2016 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26917191

RESUMO

Hypoxemia is a well-known trigger for precipitating a sickling crisis in patients with sickle cell disease. Patients undergoing staged single ventricle palliation have hypoxemia during the initial stages of the Fontan pathway. The successful completion of staged single ventricle palliation in a child with a combination of homozygous sickle cell disease and a single ventricle physiology that tolerate prolonged hypoxemia during earlier stages of Fontan pathway is described.


Assuntos
Anemia Falciforme/complicações , Ventrículos do Coração/anormalidades , Criança , Ecocardiografia , Feminino , Técnica de Fontan/métodos , Ventrículos do Coração/cirurgia , Humanos , Hipóxia , Complicações Intraoperatórias , Cuidados Paliativos , Atresia Tricúspide/complicações , Atresia Tricúspide/diagnóstico por imagem , Atresia Tricúspide/cirurgia
18.
Tex Heart Inst J ; 43(6): 546-549, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28100981

RESUMO

Isolated left subclavian artery is one of the rarer aortic arch anomalies. It has been associated with other congenital heart diseases, typically tetralogy of Fallot, double-outlet right ventricle, and atrial and ventricular septal defects. Its significant clinical implications include a left-to-right shunt from the vertebrobasilar system, which causes pulmonary overcirculation and subclavian steal. We present an unusual case of a premature infant who was diagnosed prenatally with congenital complete atrioventricular block and tricuspid atresia and was found to have an isolated left subclavian artery postnatally. The patient underwent implantation of a permanent single-chamber epicardial pacing system. To our knowledge, this combination of lesions has not been reported-and in our case, it influenced our surgical planning.


Assuntos
Anormalidades Múltiplas , Bloqueio Atrioventricular/congênito , Bloqueio Cardíaco/congênito , Artéria Subclávia/anormalidades , Atresia Tricúspide/complicações , Malformações Vasculares/complicações , Bloqueio Atrioventricular/diagnóstico , Bloqueio Atrioventricular/cirurgia , Estimulação Cardíaca Artificial , Angiografia por Tomografia Computadorizada , Ecocardiografia Doppler em Cores , Desenho de Equipamento , Feminino , Técnica de Fontan , Idade Gestacional , Bloqueio Cardíaco/diagnóstico , Bloqueio Cardíaco/cirurgia , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Marca-Passo Artificial , Artéria Pulmonar/anormalidades , Artéria Pulmonar/diagnóstico por imagem , Reimplante , Artéria Subclávia/diagnóstico por imagem , Artéria Subclávia/cirurgia , Resultado do Tratamento , Atresia Tricúspide/diagnóstico por imagem , Atresia Tricúspide/cirurgia , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/cirurgia
19.
Cardiol Young ; 26(1): 209-13, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26194906

RESUMO

BACKGROUND: Those with cyanotic heart disease have an elevated bleeding risk but also are hypercoaguable. Treating haemodynamically significant thrombi in this unique cohort poses a monumental challenge. Case A 29-year-old women with tricuspid atresia and left pulmonary artery atresia presented with superior caval vein syndrome. She had a right modified Blalock-Taussig shunt as a neonate. A left modified Blalock-Taussig shunt performed later failed to establish flow to her left lung. At age 5, she had a Fontan procedure to the right lung but could not tolerate the physiology and had a low cardiac output syndrome. The Fontan was taken down and she was left with a Glenn anastamosis to the right pulmonary artery. She did well for years until she had dyspnea, upper extremity oedema and "facial fullness". On examination she was tachycardic, hypotensive, and more desaturated than baseline. She also had facial plethora. Decision-making Echocardiogram showed a large 9 × 3 mm nearly occlusive thrombus in the superior caval vein at the bifurcation of the left and right innominate veins. An emergent venogram confirmed the location and size of the thrombus. Given the thrombus burden and potential for distal embolisation through the Glenn to the single functional lung, we chose to treat the patient with thrombolytics. She had uncomplicated ICU course and was sent home on warfarin. Follow-up echocardiogram showed complete resolution of clot. CONCLUSION: This case shows the importance of history and physical exam in caring for this complex cohort of adult patients with CHD.


Assuntos
Fibrinolíticos/uso terapêutico , Complicações Pós-Operatórias/tratamento farmacológico , Complicações Pós-Operatórias/etiologia , Síndrome da Veia Cava Superior/tratamento farmacológico , Síndrome da Veia Cava Superior/etiologia , Atresia Tricúspide/complicações , Adulto , Feminino , Técnica de Fontan , Humanos
20.
Masui ; 64(4): 416-20, 2015 Apr.
Artigo em Japonês | MEDLINE | ID: mdl-26419108

RESUMO

With the advance of diagnosis and therapeutic management for congenital heart disease, increasing numbers of the non-cardiac surgery were performed on patients with post-cardiac surgery, such as the radical operations and Fontan operation. However, there are adult patients who have not received Fontan operation lacking indication. We experienced a case of adult surgical patient for laparoscopic cholecystectomy complicated with tricuspid atresia who was not indicated for Fontan surgery. A 28-year-old man diagnosed with cholecystolithiasis was scheduled for laparoscopic cholecystectomy. He had undergone bidirectional Glenn and Blalock-Taussig shunt, but not undergone Fontan surgery for hypoplasia of the pulmonary artery and low left ventricular function. To reduce the influence of intraabdominal pressue on hemodynamics, the insufflations pressure was maintained at 8 mmHg during pneumoperitoneum. To assist ventricular function, dopamine and carperitide were administered perioperatively. We should carefully manage such a patient during perioperative period by understanding the hemodynamic state and considering the influence of pneumoperitoneum and posture.


Assuntos
Anestesia Geral/métodos , Técnica de Fontan , Atresia Tricúspide/cirurgia , Adulto , Colecistectomia Laparoscópica , Humanos , Masculino , Dor Pós-Operatória/tratamento farmacológico , Pancreatite/complicações , Pancreatite/cirurgia , Atresia Tricúspide/complicações
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