Subject(s)
Humans , Male , Adolescent , Pulmonary Valve Stenosis/etiology , Pulmonary Valve Stenosis/diagnostic imaging , Lymphoma, Non-Hodgkin/complications , Lymphoma, Non-Hodgkin/diagnosis , Pulmonary Valve Stenosis/congenital , Thoracoscopy/methods , Chest Pain/complications , Echocardiography/methods , Radiography, Thoracic/methods , Trimethoprim, Sulfamethoxazole Drug Combination/administration & dosage , Drug Therapy/methods , Dyspnea/complications , Neoplasms/classificationSubject(s)
Child , Female , Humans , Postoperative Complications/surgery , Pulmonary Valve Stenosis/surgery , Transposition of Great Vessels/surgery , Echocardiography , Electrocardiography , Postoperative Complications/diagnosis , Postoperative Complications/etiology , Pulmonary Valve Stenosis/diagnosis , Pulmonary Valve Stenosis/etiology , ReoperationABSTRACT
FUNDAMENTO: A transposição das grandes artérias é a cardiopatia congênita cianogênica mais freqüente no período neonatal, correspondendo a 7 por cento de todas as cardiopatias congênitas. Dentre as operações para tratamento cirúrgico, a operação de Jatene, com correção arterial, é o tratamento escolhido. Durante a evolução pós-operatória tardia, alguns problemas foram observados, sendo o mais comum a ocorrência de estenose supravalvar na neopulmonar, independentemente do tipo da técnica cirúrgica utilizada. OBJETIVO: Estudar e analisar a prevalência da estenose, bem como descrever o tratamento cirúrgico e propor manobras técnicas para prevenir seu aparecimento. MÉTODOS: Dentre 553 pacientes operados, 409 tiveram alta hospitalar e 281 seguidos tardiamente; 59 (20,9 por cento) apresentaram diferentes graus de estenose supravalvar pulmonar e 21 gradiente médio superior a 60 mmHg, necessitando tratamento cirúrgico. Dependendo da localização e da anatomia da estenose, o tratamento cirúrgico constou de aplicação de diferentes técnicas, como ampliação das áreas de estenose com remendos de pericárdio bovino, ressecção de áreas estenóticas e anastomose término-terminal, substituição de remendos retraídos e de tubos sintéticos. RESULTADOS: Houve boa evolução em 20 pacientes, com óbito em um dos casos. CONCLUSÃO: Conclui-se que a estenose supravalvar pulmonar pós-operação de Jatene para transposição das grandes artérias teve prevalência de 20,9 por cento. Uma vez identificada e com indicação de tratamento, pode ser tratada cirurgicamente com baixa mortalidade, mediante diferentes técnicas cirúrgicas. Para prevenir a ocorrência de estenose, propõem-se ampla dissecção e liberação dos ramos pulmonares, anastomoses amplas, remendos amplos de pericárdio autólogo e cuidado na reconstrução da neoaorta, evitando compressão da neopulmonar.
BACKGROUND: The Transposition of the Great Arteries is the most frequent congenital cyanogenic cardiopathy in the neonatal period, corresponding to 7 percent of all congenital cardiopathies. Among the operations for surgical treatment, the Jatene operation, with arterial correction, is the treatment of choice. During the late postoperative evolution, some problems were observed, with the most common being the occurrence of supravalvular stenosis at the neopulmonary, regardless of the type of surgical technique used. OBJECTIVE: To study and analyze the prevalence of stenosis, as well as describe the surgical treatment and propose technical maneuvers to prevent its onset. METHODS: Of the 553 patients that underwent surgery, 409 were discharged from the hospital and 281 had late follow-up; 59 (20.9 percent) presented different degrees of supravalvular pulmonary stenosis and 21 had a mean gradient > 60 mmHg, needing surgical treatment. Depending on the location and anatomy of the stenosis, the surgical treatment consisted of the use of different techniques, such as the enlargement of stenosis areas with bovine pericardium patches, resection of stenotic areas and termino-terminal anastomosis, replacement of retracted patches and synthetic tubes. RESULTS: Twenty patients presented good evolution and only one patient died. CONCLUSION: It can be concluded that the supravalvular pulmonary stenosis, post-Jatene operation for Transposition of Great Arteries, had a prevalence of 20.9 percent; once identified and with indication for treatment, it can be treated surgically with low mortality levels, through different surgical techniques; to prevent the occurrence of stenosis, ample dissection and release of the pulmonary branches, double anastomoses, large patches of autologous pericardium and careful reconstruction of the aorta are proposed, which prevents the compression of the neopulmonary.
Subject(s)
Animals , Cattle , Child , Humans , Pulmonary Artery/surgery , Pulmonary Valve Stenosis/surgery , Transposition of Great Vessels/surgery , Brazil/epidemiology , Follow-Up Studies , Magnetic Resonance Spectroscopy , Pericardium , Postoperative Complications , Postoperative Period , Prevalence , Pulmonary Artery/pathology , Pulmonary Valve Stenosis/epidemiology , Pulmonary Valve Stenosis/etiology , Transposition of Great Vessels/complicationsABSTRACT
The authors report a 7-year-old girl with univentricular heart physiology who developed prolonged pleural effusion due to discrete narrowing of the proximal right pulmonary artery, and progressive cyanosis which resulted from leakage of the atrial baffle, multiple veno-venous collaterals after the lateral tunnel Fontan operation. Percutaneous balloon-expandable stent implantation was used to correct the right pulmonary artery stenosis with an excellent result. Cyanosis was improved by coil embolization of the collaterals and occlusion of the baffle leakage with Amplatzer septal occluder. This is the first successful report in Thailand.
Subject(s)
Blood Vessel Prosthesis Implantation , Child , Cyanosis/etiology , Embolization, Therapeutic , Female , Fontan Procedure/adverse effects , Heart Septum/surgery , Heart Ventricles/abnormalities , Humans , Postoperative Complications , Pulmonary Valve Stenosis/etiology , StentsSubject(s)
Humans , Female , Adult , Lentigo , Arrhythmias, Cardiac , Deafness , Pulmonary Valve Stenosis/etiology , Failure to Thrive , Genitalia , Genitalia, Female , HypertelorismABSTRACT
La estenosis pulmonar valvular crítica en lactantes es una emergencia. El objetivo de este estudio es conocer nuestra casuística en dos centros ( Costa Rica y Guatemala). Método: se revisaron los expedientes de 24 lactantes menores de 4 meses, 12 de ellos menores de un mes, que se presentaron con estenosis pulmonar valvular crítica y que fueron sometidos a valvuloplastía. La edad varió de un ...
Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Pulmonary Valve Stenosis/diagnosis , Pulmonary Valve Stenosis/etiology , Pulmonary Valve Stenosis/therapy , Costa RicaABSTRACT
Se presenta un caso de síndrome de Alagille o de escasez de conductos biliares interlobulares; se realiza la descripción de la clínica e histología de nuestro paciente, así como una revisión de esta patología, destacando el interés del tema debido a la escasa frecuencia de observación
Subject(s)
Humans , Male , Child , Alagille Syndrome/diagnosis , Cholestasis/etiology , Cholestasis, Intrahepatic/etiology , Pulmonary Valve Stenosis/etiology , Prognosis , Alagille Syndrome/pathology , Alagille Syndrome/drug therapy , Spinal Dysraphism/etiology , Xanthomatosis/etiologyABSTRACT
A sindrome de Noonan foi descrita em 1963 por Noonan e Ehmke em pacientes portadores de estenose valvular pulmonar associado a baixa estatura, hipertelorismo, retardo mental moderado entre outras alteracöes. O diagnostico é clínico, baseado no achado dos caracteres fenotipos associado a ausência de alteracöes no cariotipo. A transmissäo parece ser do tipo auto-somica dominante. Também denominada de fenotipo de Turner, sindrome de Ullrich, sindrome de Ullrich-Noonan, sindrome de Bonnevie-Ullrich entre outros
Subject(s)
Humans , Noonan Syndrome/diagnosis , Pulmonary Valve Stenosis/diagnosis , Pulmonary Valve Stenosis/etiologySubject(s)
Adult , Humans , Male , Noonan Syndrome/complications , Pulmonary Valve Stenosis/etiologyABSTRACT
Dois pacientes portadores de Transposiçäo de grande artérias submetidos a cirurgia de Jatene aos seis dias e aos seis meses de idade apresentaram no período pós-operatório estenose na anastomose aorto-pulmonar, diagnosticada aos 3§ e 6§ anos de evoluçäo. Procedeu-se a angioplastia transluminal percultânea da referida obstruçäo, com sucesso, seis meses e imediatamente após o diagnóstico, respectivamente. Houve reduçäo dos gradientes de pressäo, respectivamente de 83 mmHg para 24 mmHg, e de 76 mmHg para 13 mmHg, com nítda melhora do aspecto angiográfico. Nenhum dos pacientes apresentou complicaçöes durante ou após o procedimento. Esta aplicaçäo da angioplastia trasluminal percutânea, inédita na literatura, deve ser cogitada como alternativa a correçäo cirúrgica da estenose do "novo" tronco pulmonar da cirurgia de Jatene
Two patients with transposition of the great arteries submitted to Jatene surgery at ages of 6 days and 6 months respectively, presented in the postoperative period stenosis of the aorto-pulmonary anastomosis. The diagnosis was made three and six years after the surgery. In both patients successful percutaneous transluminal angioplasty (PTA) was performed, respectively six months and immediately after the diagnosis. Pressure gradients decreased from 83 mmHg to 24 mmHg in the first case, and from 76 mmHg to 13 mmHg in the other case, with clear improvement of the angiographic image. Complications of the procedure did not occur in any case. This application for PTA was not previously reported and should be considered as an altennative to the surgical correction of the new pulmonary trunk stenosis in the Jatene surgery
Subject(s)
Humans , Male , Child, Preschool , Pulmonary Valve Stenosis/therapy , Angioplasty, Balloon , Pulmonary Valve Stenosis/etiology , Follow-Up Studies , Cyanosis/complications , Transposition of Great Vessels/complicationsABSTRACT
Las estenosis pulmonares congénitas con tabique interventricular intacto se dividen en formas valvulares (la más frecuente), subvalvulares (infundibular o por existencia de fascículos musculares anómalos), y supravalvulares, de los grandes troncos o periféricos de las arterias pulmonares segmentarias. Corresponden aproximadamente al 10% de todas las cardiopatías congénitas. El gradiente tensional pre y postestenótico, siempre que sea mayor de 50 mmHg, definirá la oportunidad operatoria. Los resultados quirúrgicos, especialmente de las formas valvulares y subvalvulares son buenos, tanto por el resultado hemodinámico como por el bajo índice de mortalidad