ABSTRACT
La double discordance est une cardiopathie congénitale complexe extrêmement rare, elle se caractérise par une discordance auriculo-ventriculaire suivie d'une autre discordance ventriculo-artérielle. Son diagnostic est posé généralement à l'âge adulte par des examens d'imagerie. Son pronostic dépend essentiellement des autres malformations congénitales cardiaques associées, des arythmies et des troubles de la conduction, ainsi que de la fonction systolique du ventricule droit en position systémique. Nous rapportons le cas d'un patient âgé de 23 ans, porteur d'une cardiopathie congénitale complexe cyanogène. Il s'agit d'une double discordance associée à une large communication interventriculaire sous aortique avec un shunt inversé et une hypoplasie de l'artère pulmonaire. Ce diagnostic a été confirmé par l'imagerie multimodale. Non opéré, le patient a vu son stade fonctionnel s'aggraver rapidement avec une dyspnée qui est passée d'un stade I à un stade III-IV de la New York Heart Association (NYHA), et apparition de signes hypoxiques et congestifs. A travers cette observation, nous avons précisé les caractéristiques anatomocliniques de cette cardiopathie congénitale complexe cyanogène, son exploration, ses options thérapeutiques, ainsi que le rôle péjoratif de son association avec d'autres malformations congénitales.
Double discordance is an extremely rare complex congenital heart disease; It is characterized by atrioventricular discordance followed by another ventriculo-arterial discordance. Its diagnosis is usually made in adulthood by imaging examinations. Its prognosis depends mainly on other associated congenital heart defects, arrhythmias and disorders of the conduction, as well as systolic function of the right ventricle in the systemic position. We report the case of a 23-year-old patient with a complex cyanogen heart disease.It is a double discordance associated to an interventricular communication with reverse shunt and an hypoplasia of the pulmonary artery.This Diagnosis was confirmed in multimodal imaging. Non-operated, the patient saw his functional status worsen rapidly with dyspnea which went from stage I to stage III-IV of the New York Heart Association (NYHA), and appearance of hypoxic and congestive signs. Through this observation, we have clarified the anatomoclinical characteristics of this complex congenital cyanogens heart disease, the necessary explorations and the different therapeutic options, as well as the pejorative role of its association with other congenital malformations.
Subject(s)
Humans , Female , Pulmonary Artery , Therapeutics , Transposition of Great Vessels , Heart Defects, Congenital , Arrhythmias, Cardiac , Congenital AbnormalitiesABSTRACT
@#Objective To summarize the experience of right ventricular outflow tract reconstruction with self-made single-valve conduit for the treatment of complex congenital heart disease, and to explore the key points of operation, and to evaluate the short-term and medium-term results of the treatment. Methods We retrospectively analyzed the clinical data of 65 patients with complex congenital heart disease treated by self-made single-valve conduit from January 2006 to June 2018. There were 42 males and 23 females aged 5-23 (9.9±4.2) years with weight 15-65 (26.2±9.9) kg. There were 19 patients with single valve artificial blood vessel (an artificial vascular group), and 46 patients with single valve bovine pericardium tube (a bovine pericardial tube group). There were 48 patients of ventricular septal defect (VSD) with pulmonary atresia (PA), 10 patients of corrective transposition of great artery with pulmonary artery stenosis, 5 patients of tetralogy of Fallot with single coronary artery malformation, 2 patients of double outlet of right ventricle with pulmonary artery stenosis and single coronary artery malformation. Results Two patients died early after operation, both of them were VSD/PA patients who underwent radical treatment of extravascular prosthesis. The right ventricular outflow tract pressure difference was 13-37 (25.2±4.9) mm Hg in the artificial vascular group and 5-23 (10.5±3.3) mm Hg in the bovine pericardial tube group. No obvious reflux was found at discharge. The patients were followed up for 8 months to 13 years. One patient was lost. During the follow-up period, there were 5 patients of moderate tricuspid regurgitation, 32 patients of moderate intraductal regurgitation, 7 patients of severe right ventricular outflow tract obstruction, 11 patients of moderate obstruction and 25 patients of mild obstruction. There was no late death. Heart function classification of all patients was in New York Heart Association classⅠtoⅡ. Conclusion The self-made single-valve conduit used to reconstruct right ventricular outflow tract shows good clinical effect. Using bovine pericardium tube has less pressure difference of outflow tract in comparison with artificial blood vessel. Bovine pericardium tube is more recommended for young and low weight patients.
ABSTRACT
Resumen Se trata de un estudio de revisión de datos publicados en literatura médica relacionada con el cribado usado para la detección temprana de cardiopatías congénitas complejas en recién nacidos aparentemente sanos en diversas ciudades del mundo, incluyendo los reportados en México, desde aquellos en los que se realiza el cribado por el conocimiento de la fisiopatologìa de datos indirectos de hipoxia, observación de cianosis diferencial y la consecuente diferencia en los valores de la pulsioximetría pre y posductal derivada de la dependencia ductal y/o de comunicación interauricular de diversas cardiopatías congénitas graves, hasta aquellos estudios de investigación realizados de forma masiva y reportados como multicéntricos, justificando la utilidad de la práctica para su implementación cotidiana y obligada de forma extensa a nivel internacional. Asimismo se citan tópicos legislativos en nuestro país como parte de los esfuerzos para establecer la obligatoriedad del cribado en toda la República Mexicana.
Abstract A review is presented of data published in medical literature related to the screening used for the early detection of complex congenital heart disease in apparently healthy newborns in several cities of the world, including those reported in Mexico. The screening wasperformed due to the knowledge of the pathophysiology of indirect hypoxia data, observation of differential cyanosis and the consequent difference in the values of pre- and post-ductal pulse oximetry derived from the ductal and/or atrial septal defect dependence of several severe congenital heart diseases. Multicentre research studies have also been carried out on a massive scale, thus justifying the usefulness of the practice for its daily implementation and at international level. Additionally, legislative topics are cited in our country as part of the efforts to establish the mandatory nature of the screening throughout the Mexican Republic.
Subject(s)
Humans , Infant, Newborn , Oximetry/methods , Neonatal Screening/methods , Heart Defects, Congenital/diagnosis , Early Diagnosis , Heart Defects, Congenital/physiopathology , Hypoxia/diagnosis , MexicoABSTRACT
@#Objective To investigate the surgery experience of modified intra/extracardiac conduit total cavopulmonary connection (TCPC). Methods We retrospectively analyzed clinical data of 47 patients of complex congenital heart disease undergoing intra/extracardiac conduit total cavopulmonary connection in our hospital between January 2008 and December 2015. There were 29 males and 18 females with a median age of 7 years (range 4 to 9 years) and median body weight of 22 kg (range 14 to 38 kg). The heart echocardiography and cardiac imaging confirmed diagnosis suitable for TCPC surgery. Results There was no early death in the whole group. The mean pulmonary arterial pressure was 16 (12–20) mm Hg and the ventilation time was 14 (7–97) h. The main complications were intractable pleural effusion in 7 patients, low cardiac output syndrome in 3 patients, repeated supraventricular tachycardia in 1 patient. All the patients recovered after treatment. At the end of discharge, the percutaneous oxygen saturation was 85%–96% (92.6%±3.3%). The echocardiography showed the conduit pressure was 0–2 mm Hg. Patients were followed up for 1 to 7 years. Three patients were lost. One patient had intestinal nutrition loss, receving repeated pleural effusion, the treatment was ineffective, died after 4 years. Four patients of repeated pleural effusion improved after treatment. One patient repeated attacks supraventricular tachycardia within 1 year, controlled by amiodaronum, already stopped about 28 months. No recurrence occurred. All survivors were in New York Heart Association (NYHA) functional class Ⅰ or Ⅱ, with good activity tolerance. Conclusion The modified intra/extracardiac conduit TCPC combines the advantages of both the lateral tunnel and the extracardiac conduit. The operation is simple, used in the treatment of complex congenital heart disease. The short-term and mid-term results are encouraging.
ABSTRACT
Objective To discuss the surgical strategy for children with complex congenital heart disease (CHD) and end-stage liver disease (ESLD).Methods We reported two eases of pediatric liver transplantation in patients with complex CHD and ESLD.Medical data including operation procedure,ICU management and outcomes were reviewed retrospectively.Also we reviewed the literature on the topic of clinical outcomes resulted from different surgery options.Results The first case was a seven-month-old male patient with biliary atresia and complex CHD (unroofed coronary sinus syndrome,persistent left superior vena cava,patent foramen ovale,and peripheral pulmonary stenosis).Liver transplantation was successfully performed without corrective heart surgery.The operation time was 6 h and 35 min.The patient suffered acute cardiac dysfunction and significant hypoxemia after extubation,then pneumonia developed,and eventually the patient died on post-operative day 12.The second case was a seven-month-old male patient with biliary atresia and complex CHD (ventricular septal defect,patent foramen ovale,patent ductus arteriosus,pulmonary stenosis).Liver transplantation was performed on the same day following total correction of cardiac defects by open-heart surgery.The operation time was 16 h and 15 min.The patient was extubated after 60 h ventilation,and was transferred to ward from ICU on post-operative day 6 with stable cardiopulmonary function.However,hepatic artery occlusion occurred on early postoperative stage,and consequently the patient received the second liver transplantation for ischemic biliary complication on post-operative day 40.The second liver transplantation procedure was uneventful.The liver graft recovered smoothly with stable hemodynamics.Conclusion Children with complex CHD undergoing liver transplantation are at an increased perioperative risk.The surgical strategy for each patient must be tailored individually according to specific cardiovascular status and limited hepatic reserve.
ABSTRACT
Interrupted Aortic Arch (lAA) is the absence or discontinuation of a portion of the aortic arch. There are three types of Interrupted Aortic Arch,and they are classified according to the site of the interruption. lsolated IAA appears commonly in type B lAA,where the interruption occurs between the left carotid artery and the left subclavian artery.In such case there is no associated cardiac anomaly and the patient may not exhibit lethal form of presentation unless there is duct dependent circulation or ventricular septal defect. lsolated interrupted arch is relatively rare congenital anomaly and very few cases were reported in literature so far.
ABSTRACT
Se presenta el caso de un lactante de dos meses con una cardiopatía congénita compleja del tipo coartación aórtica con comunicación interventricular asociada a un trastorno de migración celular cerebral. El manejo consistió en la corrección de la cardiopatía congénita en dos tiempos quirúrgicos sin haber profundizado en la evaluación neurológica preoperatoria. El paciente desarrolló múltiples complicaciones perioperatorias que incluyeron paro cardiaco en dos ocasiones, reconexiones consecutivas a circulación extracorpórea, tamponamiento cardiaco, quilotórax y choque séptico. Concomitante a las complicaciones postoperatorias, se realizó una evaluación neurológica secundaria bajo un protocolo de abordaje neurológico que consistió en electroencefalografía, resonancia nuclear magnética y tomografía por emisión de positrón (SPECT). De esta forma se detectó paquigiria con microgiria y se sospechó trastorno de migración celular cerebral. La evolución final fue hacia el deceso por falla multisistémica y la autopsia confirmó la patología neural, así como el pobre pronóstico para la función y la vida. De haberse considerado en el preoperatorio un estudio integral que incluyera el binomio corazón-cerebro, el planteamiento terapéutico podría haberse modificado.
A case of a two month infant with complex congenital heart disease (aortic coarctation with ventricular septal defect) associated to a cellular brain migration failure is presented. The management strategy consisted on the correction of congenital heart disease by means of a two-stage surgery without a further preoperative evaluation of the neurological status. The patient developed several perioperative complications such as two episodes of cardiac arrest, reconnection to cardiopulmonary bypass, cardiac tamponade, chilothorax and septic shock. A neurological protocol consisting in electroencephalography, brain magnetic resonance and Single Photon Emission Computed Tomography (SPECT) was practiced during the postoperative period, which detected microgyria with paquigyria and a cellular brain migration failure was suspected. The final outcome was death due to multisystemic failure and the autopsy confirmed the neurological disease, as well as poor life function prognosis. Should the heart-brain binomial had been considered in an integral preoperative evaluation, the therapeutical approach could have been modified.
Subject(s)
Humans , Infant , Male , Abnormalities, Multiple/diagnosis , Brain/abnormalities , Heart Defects, Congenital/therapy , Brain/pathology , Fatal OutcomeABSTRACT
Objective To summarize the clinical experiences of total cavopulmonary connection (TCPC), investigate the applications and short and mid term effects. Methods From 1999 to 2007,31 patients with complex congenital heart disease underwent TCPC. All patients were divided into child group(≤16 years old ,n =21 ) and a-dult group( n = 10 ), including 15 cases of functional univentricle, 8 cases of complete atrioventricular septal defect with double outlet of right ventricle, 3 cases of transposition with double outlet right ventricle, 3 cases of tricuspid atresia, 1 case of double outlet of right ventricle with hypoplastic left heart. 1 case of corrected transposition of the great arteries with TOF. Among them, 1 patient had cortriatriatum, 10 patients had left superior vena cave, moderate or severe mitral valve insufficiency occurred in 1, moderate atrioventricular valve insufficiency occurred in 3.28 pa-tients underwent TCPC with cardiopulmonary bypass ( CPB ) and 3 patients without CPB ;Of them, 21 patients were treated with extracardiac conduit and 10 cases with intratrial lateral tunnel. Results There was no early death,post-operative death or large quantities of pleural effusion in child group;but two deaths and three patients with large quantities of pleural effusion in adult group. One case caught up in inferior venous stasis and was cured. Follow- up was carded out from 6 months to 6 years. 26 patients were in NYHA class Ⅰ ,2 patients in NYHA class Ⅱ and only one patient in NYHA class Ⅲ. Conclusions TCPC is effectively used for complex congenital heart disease which can not be corrected in anatomy;serious postoperative complications often occur in adults. Atrioventricular valve in-sufficiency decisively affects the short and midterm effects, even the long-term effects.
ABSTRACT
La esencia del dextroisomerismo en el corazón es la presencia bilateral de atrios con orejuelas de morfología derecha. Generalmente forma parte de un grupo de cardiopatías congénitas complejas. El objetivo de nuestro estudio es hacer una descripción de los hallazgos clínicos y ecocardiográficos en los pacientes con dextroisomerismo atrial que han sobrevivido a la vida adulta. Se estudiaron 11 pacientes adultos con cardiopatías congénitas complejas y dextroisomerismo. A todos los pacientes se les realizó historia clínica completa, ecocardiografía transtorácica y transesofágica. La clase funcional según New York Heart Association al inicio del estudio fue: I en un paciente, II en 5 y III en 5. Los estudios de laboratorio mostraron poliglobulia importante. En tres casos se encontraron cuerpos de Howell Jolly El electrocardiograma de superficie mostró marcapaso migratorio en el 54.5%. Seis (54.5%) pacientes estuvieron en dextrocardia y 5 (45.5%) en levocardia. Seis pacientes (54.5%) tuvieron defectos de la tabicación atrioventricular, 4 con doble entrada (36.4%) y uno con ausencia de conexión auriculoventricular derecha (9.1%). La conexión ventriculoarterial más frecuente fue la doble salida en 8 (72.76%). Seis casos tuvieron estenosis pulmonary uno atresia pulmonar. El retorno venoso pulmonar fue al lado izquierdo del atrio común en tres casos (27.2%), al lado derecho del atrio común en cuatro (36.4%), al techo del atrio común a través de un colector en 3 casos (27.4%) y vía vena vertical en uno (9.0%). Ninguno de ellos presentó obstrucción. Se encontró insuficiencia de la válvula atrioventricular en 7 casos (63.6%). Tres pacientes (27.3%) fueron llevados a cirugía para colocación de fístula. Su clase funcional mejoró. Se trata de una serie de casos en pacientes adultos con dextroisomerismo atrial y cardiopatías congénitas complejas que a pesar de ello llegaron a la vida adulta. La valoración clínica y ecocardiográfica nos permiten en la actualidad establecer el diagnóstico, tomar decisiones terapéuticas y hacer el seguimiento. Aún falta por determinar qué diferencias tendrán con el resto de la población cuando se presenten los padecimientos crónicos degenerativos.
The most important feature of right atrial isomerism in the heart is the presence of both atria with morphologically right atrial appendages. The main aim of this study was to do a description of clinical and echocardiographic findings in adult patients with right atrial isomerism. A total of eleven consecutive patients were identified with a diagnosis of right atrial isomerism and complex congenital heart disease. A complete clinical history and transthoracic and/or transesophageal echocardiography were performed in all patients. Also a cardiac catheterization was realized in seven patients. The functional class according the New York Heart Association was I in one patient, II in 5 and III in 5. The laboratory studies showed severe polyglobulia. In 3 cases Howell-Jolly bodies were present. The electrocardiogram showed migratory pacemaker in the 54.5% of cases. The cardiac position was dextrocardia in six patients (54.5%) and levocardia in five (45.5%). Six patients (54.5%) had complete atrioventricular septal defects, 4 (36.4%) double inlet and one absence of a right atrioventricular connection (9.1%). The most frequent ventriculoarterial connection was double-inlet in 8 (72.76%) patients. In 4 cases hypopla-sia of the pulmonary artery was found. Six patients had severe pulmonary stenosis and one pulmonary atresia. The pulmonary venous drainage was into the left-sided atrium in 3 cases (27.2%), into the right-sided atrium in 4 (36.4%), into the midline of the atrium through a collector in 3 cases (27.4%) and by vertical vein in one (9.0%). Insufficiency of the atrioventricular valve was found in 7 cases (63.6%). Three patients (27.3%) went to surgery for implantation of a fistula. It is a series of adult patients with right atrial isomerism and complex congenital heart disease that survived to adult live. The clinical and echocardiographic assessment actually allows establishing the correct diagnosis, to take therapeutic decisions and to do the follow-up in all patients. Till now we could not yet determine the differences of these patients in relation to the general population when they develop chronic degenerative disease.
Subject(s)
Adolescent , Adult , Female , Humans , Male , Heart Atria/abnormalities , Heart Atria , Heart Defects, Congenital , Heart Atria/physiopathology , Heart Defects, Congenital/physiopathologyABSTRACT
Objective To evaluate the application of MSCT angiography in complex congenital heart disease.Methods Images of patient with complex congenital heart disease were acquired by MSCT with dedicated parameters.Target vas in ROI was identified with plain scanning and then contrast scanning were followed.The preset CT value of the target vas was 160 Hu.The computer would expose automatically when the contrast agent reached the value.Results The target vas was scanned continuously when the contrast agent reached the preset value after "bonus injection" of the vain.Then the acquired data were processed into a clear 3D image of cardiac vas.Conclusion 3D CT angiography is a safe,effective and noninvasive imaging modality for complex congenital heart disease.