Background: Pediatric inflammatory multisystem syndrome (PIMS) is a complication of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in children that resembles Kawasaki syndrome and places them at high risk of cardiorespiratory instability and/or cardiac damage. This study aims to describe the clinical presentation and outcomes of patients with PIMS in Mexico City. Methods: This was an observational study of children hospitalized for PIMS based on the Centers for Disease Control and Prevention case definition criteria, in a single tertiary care pediatric center in Mexico City between May 1, 2020, and September 30, 2021. Demographic characteristics, epidemiological data, medical history, laboratory tests, cardiologic evaluations, treatment, and clinical outcomes were analyzed. Results: Seventy-five cases fulfilled the case definition criteria for PIMS [median age: 10.9 years, Interquartile range (IQR): 5.6-15.6]. Fifteen (20%) patients had a severe underlying disease, 48 (64%) were admitted to the intensive care unit, 33 (44%) required invasive mechanical ventilation and 39 (52%) received vasopressor support. The patients were clustered through latent class analysis based on identified symptoms: Cluster 1 had rash or gastrointestinal symptoms (n = 60) and cluster 2 were those with predominantly respiratory manifestations (n = 15). Two patients (2.7%) died, and both had severe underlying conditions. Five patients (6.7%), all from cluster 1, developed coronary aneurysms. Conclusion: There were a high proportion of patients with severe respiratory involvement and positive RT-PCR SARS-CoV-2 and very few cases of coronary aneurysms in our study which suggests that a high proportion of the children had severe acute COVID-19. The clinical manifestations and outcomes are comparable to previously reported international studies.
Abstract Objective: Implementing screening through pulse oximetry (PO) and a knowledge management model (KMM) for early detection of life-threatening congenital heart disease (CHD) in the neonatal period. Material and methods: Pilot study of PO implementation supported by clinical criteria performed in newborns at two public hospitals of Hidalgo State. Those who tested positive were referred for echocardiography and those diagnosed with critical CHD (CCHD) were referred to specialized hospitals for treatment. Results: 1748 newborns were screened: 29 positive, 62% with CHD and 13.8% with CCHD, one death, three referrals to palliative treatment. Conclusion: PO as a method of screening helps in early diagnosis of CHD added to clinical and echocardiography studies. KMM fosters innovation and resource management.
Resumen Objetivo: Implementar el tamizaje mediante la oximetría de pulso (OP) y un modelo de gestión del conocimiento (MGC) para la detección oportuna de cardiopatías congénitas (CC) que amenazan la vida en el período neonatal. Material y métodos: Estudio piloto de implementación de OP apoyado en criterios clínicos, realizado en recién nacidos (RN) de dos hospitales públicos de Hidalgo. Los pacientes que resultaron positivos fueron objeto de ecocardiografía (EC) y los diagnosticados con cardiopatías congénitas críticas (CCC) se refirieron a tratamiento. Resultados: Se tamizó a 1,748 RN (29 positivos), CC en 62% y CCC en 13.8 %, 1 muerte y 3 programados para operación paliativa. Conclusiones: La OP ayuda en el diagnóstico de CC en combinación con criterios clínicos y EC. Un MGC favorece la innovación y la gestión de recursos.
Humans , Male , Female , Infant, Newborn , Oximetry/methods , Neonatal Screening/methods , Heart Defects, Congenital/diagnosis , Echocardiography , Pilot Projects , Early Diagnosis , Mexico
Objective: Implementing screening through pulse oximetry (PO) and a knowledge management model (KMM) for early detection of life-threatening congenital heart disease (CHD) in the neonatal period. Material and methods: Pilot study of PO implementation supported by clinical criteria performed in newborns at two public hospitals of Hidalgo State. Those who tested positive were referred for echocardiography and those diagnosed with critical CHD (CCHD) were referred to specialized hospitals for treatment. Results: 1748 newborns were screened: 29 positive, 62% with CHD and 13.8% with CCHD, one death, three referrals to palliative treatment. Conclusion: PO as a method of screening helps in early diagnosis of CHD added to clinical and echocardiography studies. KMM fosters innovation and resource management.
Objetivo: Implementar el tamizaje mediante la oximetría de pulso (OP) y un modelo de gestión del conocimiento (MGC) para la detección oportuna de cardiopatías congénitas (CC) que amenazan la vida en el período neonatal. Material y métodos: Estudio piloto de implementación de OP apoyado en criterios clínicos, realizado en recién nacidos (RN) de dos hospitales públicos de Hidalgo. Los pacientes que resultaron positivos fueron objeto de ecocardiografía (EC) y los diagnosticados con cardiopatías congénitas críticas (CCC) se refirieron a tratamiento. Resultados: Se tamizó a 1,748 RN (29 positivos), CC en 62% y CCC en 13.8 %, 1 muerte y 3 programados para operación paliativa. Conclusiones: La OP ayuda en el diagnóstico de CC en combinación con criterios clínicos y EC. Un MGC favorece la innovación y la gestión de recursos.
Heart Defects, Congenital/diagnosis , Neonatal Screening/methods , Oximetry/methods , Early Diagnosis , Echocardiography , Female , Humans , Infant, Newborn , Male , Mexico , Pilot Projects
Resumen Objetivo: Determinar la prevalencia y espectro de las enfermedades que predisponen la muerte súbita cardiaca en niños mexicanos e identificar los principales signos y síntomas tempranos que pueden permitir al personal de salud sospechar acerca de estas enfermedades y referir a los pacientes a un hospital de tercer nivel de manera temprana. Métodos: La incidencia, prevalencia y prevalencia de periodo, así como los primeros síntomas, los datos clínicos y el seguimiento, se describen en todos los niños con enfermedades que predisponen a la muerte súbita cardiaca en el Hospital Infantil de México. Resultados: Cincuenta y nueve pacientes de 8 ± 5 años, 40 con miocardiopatías y 19 con enfermedades arritmogénicas hereditarias. La prevalencia del periodo fue de 9.5/1,000 pacientes/año. Los primeros síntomas más comunes fueron disnea, palpitaciones y síncope. En 9 casos se encontró un patrón de herencia mendeliana. Tres pacientes fallecieron de muerte súbita cardiaca durante el periodo de estudio. Conclusión: Las enfermedades que predisponen a la muerte súbita cardiaca en los niños no son muy conocidas por la comunidad médica y general. Todo niño con disnea, palpitaciones y/o síncope debe referirse para la búsqueda intensiva de estas enfermedades. Una evaluación cardiológica completa en todos los miembros de la familia está indicada.
Abstract Objective: To determine the prevalence and spectrum of diseases that predispose to sudden cardiac death in Mexican children, and to identify the main early signs and symptoms that can enable the health personnel to suspect these diseases and to refer the patients to a tertiary hospital in a timely manner. Methods: Incidence, prevalence, and period prevalence, as well as early symptoms, clinical data, and follow-up were recorded on all children found with diseases that predispose to sudden cardiac death in The Children's Hospital of Mexico. Results: The study included 59 patients, with a mean age of 8 ± 5 years old, with 40 cardiomyopathies, and 19 with inherited arrhythmogenic diseases. The period prevalence was 9.5/1,000 patients/year. The most common early symptoms were dyspnoea, palpitations, and syncope. A Mendelian inheritance pattern was found in 9 cases. Three patients died of sudden cardiac death during the period of the study. Conclusion: Diseases that predispose to sudden cardiac death in children are not very well known by the general medical community. Every child with dyspnoea, palpitations and/or syncope, should be referred for the intensive search of these diseases. A complete cardiological evaluation in all members of the family is indicated.
Humans , Male , Female , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Arrhythmias, Cardiac/epidemiology , Death, Sudden, Cardiac/epidemiology , Dyspnea/epidemiology , Cardiomyopathies/epidemiology , Arrhythmias, Cardiac/complications , Syncope/epidemiology , Incidence , Prevalence , Follow-Up Studies , Longitudinal Studies , Death, Sudden, Cardiac/etiology , Hospitals, Pediatric , Mexico/epidemiology , Cardiomyopathies/complications
The incidence of total anomalous pulmonary venous connection (TAPVC) in the Caucasian population is 2.5/100,000 live births (LB), and the incidence in the Hispanic population is 19.8/100,000 LB. Without knowing the exact etiology for the development of congenital heart disease, our objective was to determine the maternal factors associated with the development of TAPVC. METHODS: 55 mother-child binomials with isolated TAPVC (group I) and 152 healthy mother-child binomials (group II) were included. Both groups had no maternal history of addiction, pre-eclampsia, or type 1, 2 or gestational diabetes mellitus. Complete clinical histories were obtained for the women in both groups and perinatal and birth data were recorded. In addition, genealogies across three generations were constructed to determine affected first- or second-degree relatives with complex congenital heart disease. RESULTS: Among the maternal characteristics analyzed, women in group I had a higher number of pregnancies before gestation of the index case (p = <0.05), and the Body Mass Index (BMI) before pregnancy was higher compared to Group II (p < 0.05), with an adjusted risk of OR = 3.6 (p = 0.011). The family history showed a higher prevalence in the group of patients with TAPVC compared to healthy children (p < 0.05). CONCLUSION: Maternal obesity before pregnancy is a risk factor for the development of CATVP in children in the Mexican population.
Obesity/epidemiology , Scimitar Syndrome/epidemiology , Adult , Body Mass Index , Child, Preschool , Female , Heart Defects, Congenital , Humans , Infant, Newborn , Male , Mexico/epidemiology , Obesity/pathology , Parity , Pregnancy , Risk Factors , Scimitar Syndrome/pathology
OBJECTIVE: To determine the prevalence and spectrum of diseases that predispose to sudden cardiac death in Mexican children, and to identify the main early signs and symptoms that can enable the health personnel to suspect these diseases and to refer the patients to a tertiary hospital in a timely manner. METHODS: Incidence, prevalence, and period prevalence, as well as early symptoms, clinical data, and follow-up were recorded on all children found with diseases that predispose to sudden cardiac death in The Children's Hospital of Mexico. RESULTS: The study included 59 patients, with a mean age of 8 ± 5 years old, with 40 cardiomyopathies, and 19 with inherited arrhythmogenic diseases. The period prevalence was 9.5/1,000 patients/year. The most common early symptoms were dyspnoea, palpitations, and syncope. A Mendelian inheritance pattern was found in 9 cases. Three patients died of sudden cardiac death during the period of the study. CONCLUSION: Diseases that predispose to sudden cardiac death in children are not very well known by the general medical community. Every child with dyspnoea, palpitations and/or syncope, should be referred for the intensive search of these diseases. A complete cardiological evaluation in all members of the family is indicated.
Arrhythmias, Cardiac/epidemiology , Cardiomyopathies/epidemiology , Death, Sudden, Cardiac/epidemiology , Dyspnea/epidemiology , Adolescent , Arrhythmias, Cardiac/complications , Cardiomyopathies/complications , Child , Child, Preschool , Death, Sudden, Cardiac/etiology , Female , Follow-Up Studies , Hospitals, Pediatric , Humans , Incidence , Infant , Infant, Newborn , Longitudinal Studies , Male , Mexico/epidemiology , Prevalence , Syncope/epidemiology
Resumen ANTECEDENTES Los tumores cardiacos fetales son hallazgos esporádicos en el ultrasonido fetal con una incidencia de 1 caso por cada 10,000 nacidos vivos. Los rabdomiomas son los tumores cardiacos más comunes en la vida intrauterina (60-86%) seguidos por los fibromas y teratomas. Casi siempre tienen regresión espontánea, pueden ocasionar alteraciones hemodinámicas e incrementar la morbilidad y mortalidad perinatal; además de su importante asociación con el complejo de la esclerosis tuberosa, enfermedad genésica que cursa con epilepsia y déficit cognitivo. OBJETIVO Reportar la primera serie mexicana de casos de detección prenatal de rabdomiomas. MATERIALES Y MÉTODOS Estudio retrospectivo consistente en la revisión de los expedientes de todos los fetos con diagnóstico prenatal de tumores cardiacos registrados de enero de 2007 a diciembre de 2017 en el Hospital Ángeles Lomas. A todas las pacientes se les realizó ultrasonido de segundo nivel y ecocardiografía avanzada prenatal y postnatal. RESULTADOS Se estudiaron siete casos, la mayoría con tumoraciones únicas. En un caso hubo alteraciones hemodinámicas durante el periodo fetal que le ocasionaron la muerte. En dos casos se confirmó esclerosis tuberosa. CONCLUSIONES Se trata de la primera serie de casos mexicanos de tumores cardiacos fetales. Los hallazgos fueron similares a los reportados en la bibliografía mundial, excepto que se encontraron más casos de tumores únicos que de múltiples. El tamaño del tumor, el número y la localización pueden predecir el pronóstico perinatal y, en especial, la posibilidad de esclerosis tuberosa.
Abstract BACKGROUND Fetal cardiac tumors are sporadic findings in fetal ultrasound with an incidence of 1 case per 10,000 live births. Rhabdomyomas are the most common cardiac tumors in intrauterine life (60-86%) followed by fibroids and teratomas. They almost always have spontaneous regression, can cause hemodynamic alterations and increase perinatal morbidity and mortality; in addition to its important association with the tuberous sclerosis complex, a genetic disease that presents with epilepsy and cognitive deficit. OBJECTIVE To report the first Mexican series of cases of prenatal detection of rhabdomyomas. MATERIALS AND METHODS Retrospective study consisting of the review of the records of all fetuses with prenatal diagnosis of cardiac tumors registered from January 2007 to December 2017 at the Ángeles Lomas Hospital. All patients underwent second-level ultrasound and advanced prenatal and postnatal echocardiography. RESULTS Seven cases were studied, most of them with single tumors. In one case there were hemodynamic alterations during the fetal period that caused his death. In two cases, tuberous sclerosis was confirmed. CONCLUSIONS This is the first series of Mexican cases of fetal cardiac tumors. The findings were similar to those reported in the world literature, except that more cases of single tumors were found than multiple tumors. The size of the tumor, the number and the location can predict the perinatal prognosis and, especially, the possibility of tuberous sclerosis.
Resumen: El arco aórtico derecho puede estar asociado a subclavia izquierda aberrante, en algunos casos esta se origina de una dilatación aneurismática que se conoce como divertículo de Kommerell. Se presentan 2 casos de anillo vascular formado por un arco aórtico derecho, subclavia izquierda anómala con divertículo de Kommerell y persistencia del conducto arterioso izquierdo con una revisión de la literatura acerca del desarrollo embriológico y los métodos de imagen que ayudan al diagnóstico de esta rara anomalía vascular.
Abstract: The right-side aortic arch may be associated with aberrant left subclavian artery, in some cases this artery originates from an aneurismal dilation of the aorta called Kommerell's diverticulum. A report is presented on 2 cases of vascular ring formed by a right-side aortic arch, anomalous left subclavian artery, Kommerell's diverticulum and left patent ductus arteriosus. A review the literature was also performed as regards the embryological development and the imaging methods used to help in the diagnosis of this rare vascular anomaly.
Humans , Aorta, Thoracic/abnormalities , Subclavian Artery/abnormalities , Abnormalities, Multiple/diagnostic imaging , Diverticulum/complications , Cardiovascular Abnormalities/complications , Aneurysm/complications , Aorta, Thoracic/diagnostic imaging , Subclavian Artery/diagnostic imaging , Cardiovascular Abnormalities/diagnostic imaging , Vascular Ring/etiology , Vascular Ring/diagnostic imaging , Aneurysm/diagnostic imaging
The right-side aortic arch may be associated with aberrant left subclavian artery, in some cases this artery originates from an aneurismal dilation of the aorta called Kommerell's diverticulum. A report is presented on 2 cases of vascular ring formed by a right-side aortic arch, anomalous left subclavian artery, Kommerell's diverticulum and left patent ductus arteriosus. A review the literature was also performed as regards the embryological development and the imaging methods used to help in the diagnosis of this rare vascular anomaly.
Abnormalities, Multiple , Aneurysm/complications , Aorta, Thoracic/abnormalities , Cardiovascular Abnormalities/complications , Diverticulum/complications , Subclavian Artery/abnormalities , Vascular Ring/etiology , Abnormalities, Multiple/diagnostic imaging , Aneurysm/diagnostic imaging , Aorta, Thoracic/diagnostic imaging , Cardiovascular Abnormalities/diagnostic imaging , Humans , Subclavian Artery/diagnostic imaging , Vascular Ring/diagnostic imaging
The use of prenatal ultrasonography has improve the detection of fetal abnormalities, which affects the perception about the natural history and evolution of them, changing the management of the fetus and neonate. Today, it is possible to perform an early prenatal diagnosis, and be treated even intrauterine with high rates of success. Such is the case of Congenital Cystic Adenomatoid Malformation (CCAM), characterized by abnormal proliferation and dilatation of the terminal structures of the airway, generating multiple cysts of diferent sizes and locations. The CCAM can be asymptomatic until adulthood, but sometimes it is presented as an adverse perinatal outcome, manifested with ascites, hydrothorax, hydrops and hypoplasia of the affected lung. We report a case of a pregnant women, 32-year-old, diagnosed on the 18 week pregnancy by ultrasound Type 3 Congenital Cystic Adenomatoid Malformation disease. Treated with an intrauterine intervention percutaneous fetal sclerotherapy successfully.
Cystic Adenomatoid Malformation of Lung, Congenital/therapy , Fetal Therapies/methods , Sclerotherapy/methods , Ultrasonography, Prenatal/methods , Adult , Cystic Adenomatoid Malformation of Lung, Congenital/diagnosis , Female , Humans , Pregnancy , Treatment Outcome
La persistencia del quinto arco aórtico es una rara anomalía congénita vascular que consiste en la presencia de una comunicación entre la aorta ascendente y la aorta descendente a través de un conducto arterial; se diagnostica de manera incidental. Informamos de un caso asociado a persistencia de conducto arterioso.
Persistent fifth aortic arch is a rare congenital vascular anomaly, with no clinical impact, so diagnosis is usually an incidental finding ocasionally associated with other congenital heart defects. We report a case of persistent fifth aortic arch associated with patent ductus arteriosus.
Child , Female , Humans , Abnormalities, Multiple , Aorta, Thoracic/abnormalities , Ductus Arteriosus, Patent/complications , Abnormalities, Multiple/diagnosis , Ductus Arteriosus, Patent/diagnosis
Persistent fifth aortic arch is a rare congenital vascular anomaly, with no clinical impact, so diagnosis is usually an incidental finding occasionally associated with other congenital heart defects. We report a case of persistent fifth aortic arch associated with patent ductus arteriosus.
Abnormalities, Multiple , Aorta, Thoracic/abnormalities , Ductus Arteriosus, Patent/complications , Abnormalities, Multiple/diagnosis , Child , Ductus Arteriosus, Patent/diagnosis , Female , Humans
Background: Currently, there is a spreading worldwide tendency to characterize health issues and to propose alternative solutions via the creation of computerized databases. The aim of this study was to present the results in a computerized database of pediatric cardiac surgeries developed under the auspices of the Mexican Association of Specialists in Congenital Heart Diseases (Asociación Mexicana de Especialistas en Cardiopatías Congénitas A.C) and coordinated by the collegiate group of Pediatric Cardiology and Surgery as petitioned by the National Institutes of Health and High Specialty Hospitals Coordinating Commission. Methods: We analyzed all cases registered in the database during a 1-year observation period (August 1, 2011 to July 31, 2012) by all major Health Ministry-dependent institutes and hospitals offering surgical services related to pediatric cardiopathies to the non-insured population. Results: Seven institutions participated voluntarily in completing the database. During the analyzed period, 943 surgeries in 880 patients with 7% reoperations (n = 63) were registered. Thirty-eight percent of the surgeries were performed in children <1 year of age. The five most common cardiopathies were patent ductus arteriosus (n = 96), ventricular septal defect (n = 86), tetralogy of Fallot (n = 72), atrial septal defect (n = 68), and aortic coarctation (n = 54). Ninety percent of surgeries were elective and extracorporeal circulation was used in 62% of surgeries. Global mortality rate was 7.5% with the following distribution in the RACHS-1 score categories: 1 (n = 4, 2%), 2 (n = 19, 6%), 3 (n = 22, 8%), 4 (n = 12, 19%), 5 (n = 1, 25%), 6 (n = 6, 44%), and non-classifiable (n = 2, 9%). Conclusions: This analysis provides a representative view of the surgical practices in cardiovascular diseases in the pediatric population at the national non-insured population level. However, incorporating other health institutions to the national registry database will render a more accurate panorama of the national reality in surgical practices in the population <18 years of age.
The saccular or fusiform dilatation of the ductus arteriosus is called aneurysm (DAA). It is diagnosed in the second trimester during a structural ultrasound. Even though the reported incidence is 2.2%, it remains infrequent, because it is not searched routinely. The pathogenesis is uncertain, resulting from an increase on the circulating blood volume thus in the cardiac afterload, associated to a thinning of the vessel wall, for an inadequate intima development. Ductus arteriosus aneurysm can be classified according by their diameter as small (<7 mm.) and large (> or =8 mm). The smaller commonly closes spontaneously in 70% of the cases, unlike the larger one, they are associate in a greater number of complications, such as thrombosis, embolism, infection, compression effects of adjacent structures and spontaneous rupture. The absence of spontaneous closure, according to the symptoms and possible complications, a neonatal surgical closure should be considered. We report a case of a 35-year-old patient, diagnose on the 35 week pregnancy, a ductus arteriosus aneurysm by ultrasound with an image of the three vessels performed communicating, saccular dilatation of 7-8 mm. and a turbulent Doppler flow.
Ductus Arteriosus , Heart Aneurysm , Adult , Female , Heart Aneurysm/diagnostic imaging , Humans , Pregnancy , Ultrasonography, Prenatal
BACKGROUND: Currently, there is a spreading worldwide tendency to characterize health issues and to propose alternative solutions via the creation of computerized databases. The aim of this study was to present the results in a computerized database of pediatric cardiac surgeries developed under the auspices of the Mexican Association of Specialists in Congenital Heart Diseases (Asociación Mexicana de Especialistas en Cardiopatías Congénitas A.C) and coordinated by the collegiate group of Pediatric Cardiology and Surgery as petitioned by the National Institutes of Health and High Specialty Hospitals Coordinating Commission. METHODS: We analyzed all cases registered in the database during a 1-year observation period (August 1, 2011 to July 31, 2012) by all major Health Ministry-dependent institutes and hospitals offering surgical services related to pediatric cardiopathies to the non-insured population. RESULTS: Seven institutions participated voluntarily in completing the database. During the analyzed period, 943 surgeries in 880 patients with 7% reoperations (n=63) were registered. Thirty-eight percent of the surgeries were performed in children <1 year of age. The five most common cardiopathies were patent ductus arteriosus (n=96), ventricular septal defect (n=86), tetralogy of Fallot (n=72), atrial septal defect (n=68), and aortic coarctation (n=54). Ninety percent of surgeries were elective and extracorporeal circulation was used in 62% of surgeries. Global mortality rate was 7.5% with the following distribution in the RACHS-1 score categories: 1 (n=4, 2%), 2 (n=19, 6%), 3 (n=22, 8%), 4 (n=12, 19%), 5 (n=1, 25%), 6 (n=6, 44%), and non-classifiable (n=2, 9%). CONCLUSIONS: This analysis provides a representative view of the surgical practices in cardiovascular diseases in the pediatric population at the national non-insured population level. However, incorporating other health institutions to the national registry database will render a more accurate panorama of the national reality in surgical practices in the population <18 years of age.
Introducción. La atresia pulmonar con comunicación interventricular es una cardiopatía compleja con una incidencia aproximada de 2% entre todas las cardiopatías congénitas. Se asocia con frecuencia al síndrome de deleción 22q11. Tiene una amplia variabilidad anatómica que es necesario precisar con exactitud para poder establecer un plan médico quirúrgico individualizado. Caso clínico. Se presenta el caso de un paciente de 2 años con atresia pulmonar y comunicación interventricular asociadas a dos grandes colaterales aortopulmonares. Este paciente fue corregido mediante conexión de ventrículo derecho a arteria pulmonar, durante la cual fue posible ligar una de las colaterales; la restante fue embolizada mediante un dispositivo trascateterismo con oclusión total. Conclusiones. El manejo de las arterias colaterales asociadas a atresia pulmonar con comunicación interventricular es complejo pero accesible al tratamiento trascateterismo mediante el implante de dispositivos con mínima morbimortalidad.
Background. Pulmonary atresia with ventricular septal defect (VSD) is a complex heart disease with an incidence of ~2% of all congenital heart diseases. It is frequently associated with 22q11 deletion syndrome. Due to the extensive anatomic variability, it is necessary to accurately establish an individualized surgical/medical plan. Case report. We report the case of a 2-year-old patient with pulmonary atresia and two associated mayor aortopulmonary collateral arteries. This patient underwent right ventricular-pulmonary artery connection at which time it was possible to ligate one of the collaterals. The remaining were embolized by transcatheter device with total occlusion. Conclusions. Management of collateral arteries associated with pulmonary atresia with VSD is complex but is accessible with transcatheter treatment with device implantation with minimal morbidity.
Children born with a left ventricular outflow tract obstruction (LVOTO) can present with symptoms of left ventricular (LV) failure while ejection fraction (EF) is normal. A more sensitive parameter of systolic function might be obtained with speckle tracking echocardiography, which describes ventricular longitudinal deformation in strain values. It is presumed that despite a normal or only slight decrease in ejection fraction, patients with a LVOTO demonstrate aberrations in the longitudinal deformation of the left ventricle. In addition, it is expected that after a successful intervention, longitudinal deformation returns to normal values. Standard trans-thoracic echocardiography was performed on 33 consecutive patients with a LVOTO, either an isolated aortic coarctation (AoCo) or an isolated aortic stenosis (AoSt). Before intervention a significant decrease in strain values was observed compared with the control group (N = 40), with an additional decrease in strain values in the first week after intervention (N = 16). Strain values recovered after a mean follow-up period of 42 wk (N = 9), though normal values were never reached. In addition, patients with an AoCo had a smaller decrease in strain values compared with patients with AoSt. All strain values were measured with a concomitant ejection fraction between normal limits. It is concluded that patients with a congenital LVOTO have decreased ventricular systolic function measured as strain values, whereas their ejection fraction is within the normal range. Therefore, as ejection fraction may not be an accurate measure, speckle tracking-based strain may be significant in the identification of subtle changes in longitudinal deformation and may create opportunities for patients to benefit from early treatment for heart failure.
Aortic Coarctation/complications , Aortic Coarctation/diagnostic imaging , Aortic Valve Stenosis/complications , Aortic Valve Stenosis/diagnostic imaging , Elasticity Imaging Techniques/methods , Ventricular Outflow Obstruction/diagnostic imaging , Ventricular Outflow Obstruction/etiology , Child , Echocardiography/methods , Female , Humans , Male , Prognosis , Reproducibility of Results , Sensitivity and Specificity , Stroke Volume
