ABSTRACT
Background: Congenital heart disease (CHD) survival rate has improved dramatically due to advances in diagnostic and therapeutic techniques. However, concerning the unrepaired CHD population of moderate and severe complexity, the data regarding risk predictors and surgical outcomes are scarce. Our aim was to describe the surgical results and predictors of in-hospital outcomes in adult patients with moderate-to-severe complexity CHD that were not repaired in childhood. Methods: We conducted a retrospective cohort study that included 49 adult patients with moderate-to-complex CHD who were treated in a single medical centre. Clinical and echocardiographic variables were obtained on admission, after surgical procedures and during follow-up. Results: Most of the patients were female (66%). Left ventricular ejection fraction and right ventricular outflow tract fractional shortening were within the normal range. The median pulmonary artery systolic pressure was 37 (27-55) mm Hg. The median time was 118 (80-181) minutes for extracorporeal circulation and 76 (49-121) minutes for aortic cross-clamping. The most frequent complication was postoperative complete atrioventricular block (12.2%). In-hospital survival rate was 87.7%. The development of low cardiac output syndrome with predominant right ventricle failure in the postoperative period was the most important predictor of in-hospital death (P = 0.03). Conclusions: Deciding to treat adults with CHD is challenging in moderate and severe unrepaired cases. Adequate clinical, functional, and imaging evaluation is essential to determine each patient's suitability for surgical management and to achieve the best clinical outcome for this population.
Contexte: Grâce aux avancées réalisées en matière de techniques diagnostiques et thérapeutiques, la survie des patients atteints d'une cardiopathie congénitale s'est considérablement améliorée. Cependant, en ce qui concerne les personnes atteintes d'une cardiopathie congénitale non corrigée présentant une complexité modérée ou extrême, les données portant sur les facteurs de risque prédictifs ainsi que sur les résultats chirurgicaux sont rares. Notre objectif était de décrire les résultats chirurgicaux ainsi que les facteurs prédictifs des résultats obtenus en milieu hospitalier chez les patients adultes atteints d'une cardiopathie congénitale présentant une complexité modérée ou extrême qui n'a pas été corrigée pendant l'enfance. Méthodologie: Nous avons mené une étude de cohorte rétrospective comprenant 49 patients adultes atteints d'une cardiopathie congénitale modérée ou complexe qui ont reçu leurs traitements dans un seul centre médical. Les variables cliniques et échocardiographiques ont été obtenues au moment de l'admission, après les interventions chirurgicales et pendant la période de suivi. Résultats: Les patients étaient en majorité des femmes (66 %). La fraction d'éjection du ventricule gauche ainsi que la fraction de raccourcissement de la voie d'éjection ventriculaire droite sont demeurées dans les limites de la normale. La pression systolique médiane de l'artère pulmonaire a été de 37 mmHg (27-55 mmHg). Le temps médian écoulé pour la circulation extracorporelle a été de 118 minutes (80-181 minutes) et pour le clampage de la crosse aortique, de 76 minutes (49-121 minutes). Le bloc auriculo-ventriculaire postopératoire complet a été la complication la plus fréquente (12,2 %). Le taux de survie en milieu hospitalier a été de 87,7 %. Le développement du syndrome du faible débit cardiaque accompagné d'une insuffisance prédominante du ventricule droit durant la période postopératoire a constitué le principal facteur prédictif de décès à l'hôpital (p = 0,03). Conclusion: Il est difficile de traiter les adultes qui présentent une cardiopathie congénitale modérée ou sévère non corrigée. Il est essentiel que les évaluations cliniques, fonctionnelles et par imagerie soient réalisées de façon adéquate pour déterminer si une prise en charge chirurgicale convient aux patients et pour garantir les meilleurs résultats cliniques chez ces derniers.
ABSTRACT
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Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Adolescent , Vascular Closure Devices , Aortopulmonary Septal Defect/surgery , Endocardial Cushion Defects/surgery , Heart Defects, Congenital/surgery , Angiography/methodsSubject(s)
Aortography/methods , Aortopulmonary Septal Defect/diagnostic imaging , Aortopulmonary Septal Defect/surgery , Cardiac Catheterization/instrumentation , Computed Tomography Angiography/methods , Septal Occluder Device , Adolescent , Aortopulmonary Septal Defect/physiopathology , Cardiac Catheterization/methods , Child , Child, Preschool , Cohort Studies , Female , Follow-Up Studies , Humans , Infant , Male , Minimally Invasive Surgical Procedures/methods , Patient Selection , Retrospective Studies , Treatment OutcomeABSTRACT
BACKGROUND: Surgical repair of common arterial trunk (CAT) by means of a homograft conduit has become a standard practice. We report our experience in the correction of this heart disease with a handmade bovine pericardial-valved woven Dacron conduit as an alternative procedure to homografts, with a focus on early, mid-term, and long-term results. METHODS: We designed a retrospective study that included 15 patients with a mean age of 1.5 years (range: three months to eight years), who underwent primary repair of simple CAT. Right ventricular outflow tract was reconstructed in all the cases with this handmade graft that was explanted at the time of its biological stenotic degeneration. A peeling procedure was performed at this time, in order to reconstruct the right ventricle-to-pulmonary artery continuity. RESULTS: Overall mortality was 13.3% (one death at the early postoperative primary repair and the other at the mid-term postoperative peeling reoperation). Actuarial survival rate was 93.3%, 86.7%, and 86.7% at 5, 10, and 15 years, respectively. All of the 14 survivors developed stenosis of the handmade conduit at the mid-term period (8 ± 3 years), but after the peeling procedure, 13 survivors remain asymptomatic to date. CONCLUSIONS: Primary repair of common arterial trunk using a handmade conduit can be performed with very low perioperative mortality and satisfactory mid-term and long-term results, which can be favorably compared with those reported with the use of homografts. When graft obstruction develops, peeling procedure is a good option because it does not affect the overall survival, although long-term outcomes warrant further follow-up.
Subject(s)
Pericardium/transplantation , Polyethylene Terephthalates , Prostheses and Implants , Truncus Arteriosus, Persistent/surgery , Truncus Arteriosus/surgery , Animals , Cattle , Female , Follow-Up Studies , Humans , Infant , Male , Prosthesis Design , Pulmonary Artery/surgery , Reoperation , Retrospective Studies , Survival Rate/trends , Transplantation, Homologous , Truncus Arteriosus/abnormalities , Truncus Arteriosus, Persistent/mortalityABSTRACT
INTRODUCTION: Surgical treatment is accepted as conventional treatment of patients with acute aortic syndrome associated to ischemic complications. Promising results of thoracic endovascular aortic repair (TEVAR) has expanded its indication to a variety of thoracic aorta pathologies. OBJECTIVES: The objective of this study was to evaluate the safety and efficacy of TEVAR during the in-hospital period and at mid-term follow-up, and compare with medical and surgical treatment in patients who presented with acute aortic syndrome (AAS) type B. MATERIAL AND METHODS: Of 113 patients that fulfilled the inclusion criteria, 58 underwent endovascular treatment (Group 1), 34 had medical treatment (Group 2), and 21 had surgical treatment (Group 3). RESULTS: Global and cardiovascular in-hospital mortality was superior in Group 3 (p = 0.0001). Mean follow-up was 30.4 ± 12.2 months. The survival rate free from cardiovascular death was 92.5, 60.5, and 42.9, in Group 1, 2, and 3, respectively, at 36 months. CONCLUSIONS: Our results suggest that endovascular treatment is feasible, safe, and associated with a reduction of in-hospital and mid-term major adverse cardiovascular events, and superior survival rate compared with surgical and medical treatment.
ABSTRACT
Heart development consists in a group of complex and specific morfogenetic interactions, that requires the proper activity of each factor implicated in this process. Congenital heart defects (CHD) are a group of multifactorial complex diseases with environmental and genetic factors playing important roles. There is not an exact relation between molecular mechanisms and morphological defects in CHD, because in most of the cases the proper development of an anatomical structure implies the adequate function of several pathways that may depend of the action of different genes. This review summarizes the genetic factors implied in the normal heart development and the most common gene mutations associated with CHD.
Subject(s)
Heart Defects, Congenital/genetics , Heart Diseases/congenital , Heart Diseases/genetics , Heart/anatomy & histology , HumansABSTRACT
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)
Abnormalities, Multiple/diagnosis , Brain/abnormalities , Heart Defects, Congenital/therapy , Brain/pathology , Fatal Outcome , Humans , Infant , MaleABSTRACT
Congenital cardiopathies are the most frequent congenital malformations. Reports of its prevalence around the world range from 2.1 to 12.3 for every 1000 newborns. Prevalence in our country remains unknown, but it probably occupies sixth place for mortality in infants less than a year old, and third place for mortality in those aged between 1 and 4 years. Based on birthrate, it is calculated that 10 to 12 000 infants in our country have some cardiac malformation. To understand the magnitude of the problem, it is important to identify the global number of newborns with some congenital cardiopathy each year and the type of malformation that they have, in order to determine the necessary resources and to plan their distribution. The main objective of regionalization is the justification of the resources with an emphasis in the specialized medical services to provide the best results for the patients. Hence, reason, based on the resources of each state, as well as their natality and infant mortality rates related to congenital cardiovascular pathology, we should proceed to regionalize the attention, and to simultaneously create a trustworthy database of the congenital cardiopathies. This should have many benefits, such as increase the number of total attended cases, improve the quality of attention, use appropriately the existent resources, and -surely- decrease the infant mortality.
Subject(s)
Health Resources/organization & administration , Heart Defects, Congenital/therapy , Heart Defects, Congenital/epidemiology , Humans , Infant, Newborn , Mexico , Risk AssessmentABSTRACT
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
Las malformaciones congénitas más frecuentes son las cardiopatías congénitas. La prevalencia reportada a nivel mundial va de 2.1 a 12.3 por 1000 recién nacidos. En nuestro país, se desconoce su prevalencia real; como causa de muerte infantil, se ubica en el sexto lugar en menores de un año y como la tercera causa en los niños entre uno y cuatro años; con base en la tasa de natalidad, se calcula que alrededor de 10 mil a 12 mil niños nacen con algún tipo de malformación cardiaca. Es de suma importancia conocer la magnitud del problema, identificar el número de niños que nacen cada año con una cardiopatía congénita y de manera desglosada por el tipo de la malformación; lo que permitiría determinar con mayor exactitud los recursos necesarios y planear su distribución. La regionalización tiene como objetivo la racionalización de los recursos con énfasis en servicios médicos muy especializados con la finalidad de lograr un mejor resultado para los pacientes. Por lo que, de manera simultánea a la creación de una base de datos fidedigna de las cardiopatías congénitas y, con base a la tasas de natalidad y mortalidad infantil secundaria a patología cardiovascular congénita y recursos en cada Estado, se debería proceder a regionalizar la atención. Lo anterior tendría diversos beneficios, ya que permitiría aumentar el número de casos atendidos, mejorar la calidad de la atención, aprovechar adecuadamente los recursos existentes y, seguramente, obtener una disminución de la mortalidad infantil.
Congenital cardiopathies are the most frequent congenital malformations. Reports of its prevalence around the world range from 2.1 to 12.3 for every 1000 newborns. Prevalence in our country remains unknown, but it probably occupies sixth place for mortality in infants less than a year old, and third place for mortality in those aged between 1 and 4 years. Based on birthrate, it is calculated that 10 to 12 000 infants in our country have some cardiac malformation. To understand the magnitude of the problem, it is important to identify the global number of newborns with some congenital cardiopathy each year and the type of malformation that they have, in order to determine the necessary resources and to plan their distribution. The main objective of regionalization is the justification of the resources with an emphasis in the specialized medical services to provide the best results for the patients. Hence, reason, based on the resources of each state, as well as their natality and infant mortality rates related to congenital cardiovascular pathology, we should proceed to regionalize the attention, and to simultaneously create a trustworthy database of the congenital cardiopathies. This should have many benefits, such as increase the number of total attended cases, improve the quality of attention, use appropriately the existent resources, and -surely- decrease the infant mortality.
Subject(s)
Humans , Infant, Newborn , Health Resources/organization & administration , Heart Defects, Congenital/therapy , Heart Defects, Congenital/epidemiology , Mexico , Risk AssessmentABSTRACT
We report the case of an infant 3 months old with a rare association, tetralogy of Fallot with total anomalous pulmonary veins connection the diagnosis was made by echocardiography and a successful complete reparation was achieved.
Subject(s)
Humans , Infant , Male , Pulmonary Veins/abnormalities , Tetralogy of Fallot , Abnormalities, Multiple , Abnormalities, Multiple , Cardiac Catheterization/methods , Pulmonary Veins , Pulmonary Veins , Tetralogy of FallotABSTRACT
We report the case of an infant 3 months old with a rare association, tetralogy of Fallot with total anomalous pulmonary veins connection the diagnosis was made by echocardiography and a successful complete reparation was achieved.
Subject(s)
Pulmonary Veins/abnormalities , Tetralogy of Fallot/diagnosis , Abnormalities, Multiple/diagnosis , Abnormalities, Multiple/surgery , Cardiac Catheterization/methods , Humans , Infant , Male , Pulmonary Veins/diagnostic imaging , Pulmonary Veins/surgery , Tetralogy of Fallot/surgery , UltrasonographyABSTRACT
OBJECTIVES: Evaluate long-term evolution of patients submitted to surgery for coarctation of the aorta. Compare event free survival in younger vs older patients at the time of surgical correction. METHODS: We reviewed the clinical records of patients operated for coarctation of the aorta from January 1 1980 to December 31 1994. The mean follow-up ranged from 9 to 23 years (mean 10.9 y). Final events registered were recoarctation, death, systemic hypertension, endocarditis, stroke, aneurisms. RESULTS: Two hundred and sixteen patients were found with mean age 13 +/- 12 y male gender was most frequent (61%). Recoarctation was found in 13 patients (6.02%), persistent hypertension in 14.1%. Event free survival at 10 years was 86.2%. In patients less than 10 y was 89% vs 80.2% in older patients. Hypertension free survival in patients less than 10 y was 98.3% vs 80.1% in older patients (p < 0.001). CONCLUSIONS: Our data confirms that surgical treatment for coarctation of the aorta is associated with low morbidity and mortality at long-term with reduced rate of recoarctation (7%). Early correction (< 10 y) is associated with a better long term survival. Hypertension and use of pharmacologic treatment are reduced after surgery and persist in the long-term evolution.
Subject(s)
Aortic Coarctation/surgery , Adolescent , Adult , Aortic Coarctation/mortality , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Infant , Male , Middle Aged , Retrospective Studies , Survival Rate , Time FactorsABSTRACT
Kawasaki's disease is by now the first cause of pediatric acquired cardiopathies in many countries, even more than rheumatic fever. Probably the most common complication of this disease is coronary affection, which often causes stenosis. Treatment of the acute and chronic coronary events in children is based on the knowledge acquired from the disease in adults. The increasing experience in pediatric patients with this pathology has led to better ways of handling and treating this disease.
Subject(s)
Coronary Stenosis/surgery , Mucocutaneous Lymph Node Syndrome/complications , Myocardial Revascularization , Adolescent , Coronary Stenosis/etiology , Humans , MaleABSTRACT
Kawasaki's disease is by now the first cause of pediatric acquired cardiopathies in many countries, even more than rheumatic fever. Probably the most common complication of this disease is coronary affection, which often causes stenosis. Treatment of the acute and chronic coronary events in children is based on the knowledge acquired from the disease in adults. The increasing experience in pediatric patients with this pathology has led to better ways of handling and treating this disease.
Subject(s)
Adolescent , Humans , Male , Coronary Stenosis , Myocardial Revascularization , Mucocutaneous Lymph Node Syndrome , Coronary StenosisABSTRACT
OBJECTIVES: Evaluate long-term evolution of patients submitted to surgery for coarctation of the aorta. Compare event free survival in younger vs older patients at the time of surgical correction. METHODS: We reviewed the clinical records of patients operated for coarctation of the aorta from January 1 1980 to December 31 1994. The mean follow-up ranged from 9 to 23 years (mean 10.9 y). Final events registered were recoarctation, death, systemic hypertension, endocarditis, stroke, aneurisms. RESULTS: Two hundred and sixteen patients were found with mean age 13 +/- 12 y male gender was most frequent (61%). Recoarctation was found in 13 patients (6.02%), persistent hypertension in 14.1%. Event free survival at 10 years was 86.2%. In patients less than 10 y was 89% vs 80.2% in older patients. Hypertension free survival in patients less than 10 y was 98.3% vs 80.1% in older patients (p < 0.001). CONCLUSIONS: Our data confirms that surgical treatment for coarctation of the aorta is associated with low morbidity and mortality at long-term with reduced rate of recoarctation (7%). Early correction (< 10 y) is associated with a better long term survival. Hypertension and use of pharmacologic treatment are reduced after surgery and persist in the long-term evolution.
