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Secundum atrial septal defect (ASD2) detection is often delayed, with the potential for late diagnosis complications. Recent work demonstrated artificial intelligence-enhanced ECG analysis shows promise to detect ASD2 in adults. However, its application to pediatric populations remains underexplored. In this study, we trained a convolutional neural network (AI-pECG) on paired ECG-echocardiograms (≤ 2 days apart) to detect ASD2 from patients ≤ 18 years old without major congenital heart disease. Model performance was evaluated on the first ECG-echocardiogram pair per patient for Boston Children's Hospital internal testing and emergency department cohorts using area under the receiver operating (AUROC) and precision-recall (AUPRC) curves. The training cohort comprised of 92,377 ECG-echocardiogram pairs (46,261 patients; median age 8.2 years) with an ASD2 prevalence of 6.7%. Test groups included internal testing (12,631 patients; median age 7.4 years; 6.9% prevalence) and emergency department (2,830 patients; median age 7.5 years; 4.9% prevalence) cohorts. Model performance was higher in the internal test (AUROC 0.84, AUPRC 0.46) cohort than the emergency department cohort (AUROC 0.80, AUPRC 0.30). In both cohorts, AI-pECG outperformed ECG findings of incomplete right bundle branch block. Model explainability analyses suggest high-risk limb lead features include greater amplitude P waves (suggestive of right atrial enlargement) and V1 RSR' (suggestive of RBBB). Our findings demonstrate the promise of AI-pECG to inexpensively screen and/or detect ASD2 in pediatric patients. Future multicenter validation and prospective trials to inform clinical decision making are warranted.

RESUMEN

(1) Background: Mastery of auscultation can be challenging for many healthcare providers. Artificial intelligence (AI)-powered digital support is emerging as an aid to assist with the interpretation of auscultated sounds. A few AI-augmented digital stethoscopes exist but none are dedicated to pediatrics. Our goal was to develop a digital auscultation platform for pediatric medicine. (2) Methods: We developed StethAid-a digital platform for artificial intelligence-assisted auscultation and telehealth in pediatrics-that consists of a wireless digital stethoscope, mobile applications, customized patient-provider portals, and deep learning algorithms. To validate the StethAid platform, we characterized our stethoscope and used the platform in two clinical applications: (1) Still's murmur identification and (2) wheeze detection. The platform has been deployed in four children's medical centers to build the first and largest pediatric cardiopulmonary datasets, to our knowledge. We have trained and tested deep-learning models using these datasets. (3) Results: The frequency response of the StethAid stethoscope was comparable to those of the commercially available Eko Core, Thinklabs One, and Littman 3200 stethoscopes. The labels provided by our expert physician offline were in concordance with the labels of providers at the bedside using their acoustic stethoscopes for 79.3% of lungs cases and 98.3% of heart cases. Our deep learning algorithms achieved high sensitivity and specificity for both Still's murmur identification (sensitivity of 91.9% and specificity of 92.6%) and wheeze detection (sensitivity of 83.7% and specificity of 84.4%). (4) Conclusions: Our team has created a technically and clinically validated pediatric digital AI-enabled auscultation platform. Use of our platform could improve efficacy and efficiency of clinical care for pediatric patients, reduce parental anxiety, and result in cost savings.

Asunto(s)

Inteligencia Artificial , Estetoscopios , Humanos , Niño , Auscultación , Soplos Cardíacos/diagnóstico , Algoritmos , Ruidos Respiratorios/diagnóstico

RESUMEN

Double aortic arch associated with atresia of the left arch proximal to the left common carotid artery has been considered a theoretical possibility. To our knowledge, we report the first patient with this anatomy confirmed by surgical observation.

Asunto(s)

Aorta Torácica , Anillo Vascular , Humanos , Aorta Torácica/diagnóstico por imagen , Aorta Torácica/cirugía , Aorta Torácica/anomalías , Arteria Carótida Común/diagnóstico por imagen , Arteria Carótida Común/cirugía , Documentación

RESUMEN

BACKGROUND: Access to pediatric sub-specialty training is a critical unmet need in many resource-limited settings. In Rwanda, only two pediatric cardiologists are responsible for the country's clinical care of a population of 12 million, along with the medical education of all pediatric trainees. To strengthen physician training opportunities, we developed an e-learning curriculum in pediatric cardiology. This curriculum aimed to "flip the classroom", allowing residents to learn key pediatric cardiology concepts digitally before an in-person session with the specialist, thus efficiently utilizing the specialist for additional case based and bedside teaching. METHODS: We surveyed Rwandan and US faculty and residents using a modified Delphi approach to identify key topics in pediatric cardiology. Lead authors from Rwanda and the USA collaborated with OPENPediatrics™, a free digital knowledge-sharing platform, to produce ten core topics presented in structured videos spanning 4.5 h. A mixed methods evaluation was completed with Rwandan pediatric residents, including surveys assessing knowledge, utilization, and satisfaction. Qualitative analysis of structured interviews was conducted using NVivo. RESULTS: Among the 43 residents who participated in the OPENPediatrics™ cardiology curriculum, 33 (77%) completed the curriculum assessment. Residents reported using the curriculum for a median of 8 h. Thirty-eight (88%) reported viewing the curriculum on their personal or hospital computer via pre-downloaded materials on a USB flash drive, with another seven (16%) reporting viewing it online. Twenty-seven residents viewed the course during core lecture time (63%). Commonly reported barriers to utilization included lack of time (70%), access to internet (40%) and language (24%). Scores on knowledge assessment improved from 66.2% to 76.7% upon completion of the curriculum (p < 0.001) across all levels of training, with most significant improvement in scores for PGY-1 and PGY-2 residents. Residents reported high satisfaction with the visuals, engaging presentation, and organization of the curriculum. Residents opined the need for expanded training material in cardiac electrocardiogram and echocardiogram and requested for slower narration by foreign presenters. CONCLUSION: Video-based e-learning via OPENPediatrics™ in a resource-limited setting was effective in improving resident's knowledge in pediatric cardiology with high levels of utilization and satisfaction. Expanding access to digital curriculums for other pediatric sub-specialties may be both an effective and efficient strategy for improving training in settings with limited access to subspecialist faculty.

Asunto(s)

Cardiología , Instrucción por Computador , Internado y Residencia , Cardiología/educación , Niño , Curriculum , Humanos , Rwanda

RESUMEN

OBJECTIVES: To assess the role of neonatal pulse oximetry screening and other bases of referral for patients of all ages with aortic coarctation referred to a tertiary care medical center. STUDY DESIGN: Medical records were reviewed for 200 consecutive patients diagnosed and treated for CoA in either 2006-2011 or 2015-2019, before and after mandated pulse oximetry screening, respectively. RESULTS: In both groups, ∼50% of patients were diagnosed within the first 5 days. Diagnosis by fetal echocardiography was more frequent in the 2015-2019 group (30.5% vs 20.5%; P < .03); obstruction often developed only as the ductus arteriosus closed. In each group, ∼25% of patients were diagnosed at age >1 year and 7% had an incidental diagnosis. Pulse oximetry screening was documented as abnormal in only 8 of 47 patients and was not performed in those with a fetal diagnosis. Evaluation of a murmur was the second most frequent basis for referral. Moderate to severe left ventricular dysfunction occurred mainly in infants in the first month, with a similar frequency in the 2 groups; these patients often had tachypnea or poor weight gain. Decreased femoral pulses or systemic hypertension were infrequently documented by referring physicians. Hypertension typically was ascribed to a renal or essential basis. Exercise symptoms occurred mainly in patients age >10 years. CONCLUSIONS: Although fetal echocardiography and neonatal pulse oximetry contribute to the diagnosis of coarctation, physical examination has an important complementary role. Evaluation of peripheral pulses on initial and early follow-up neonatal examinations, along with consideration of coarctation in any patient with hypertension, are needed to improve timely detection.

Asunto(s)

Coartación Aórtica , Hipertensión , Adulto , Coartación Aórtica/diagnóstico , Niño , Humanos , Lactante , Recién Nacido , Tamizaje Neonatal , Oximetría , Derivación y Consulta

RESUMEN

OBJECTIVE: A review of our center's experience before March 2011 showed that one half of 36 patients who had a baffling or reimplantation procedure to repair scimitar syndrome developed pulmonary vein obstruction. We analyzed the results of a new operation that enlarges the left atrium and avoids circuitous pathways or tension on the scimitar pulmonary vein. METHODS: Between April 2011 and November 2018, 22 patients underwent scimitar vein surgery; 11 had baffling or reimplantation and 11 only had the new operation that included resection of the atrial septum with removal of the muscular limbus. The left atrium was pulled down toward the scimitar vein and a V-shaped incision made at the scimitar vein atrial junction with the space filled with a pulmonary homograft. If the scimitar vein coursed adjacent to the atrium, a V-shaped incision was made into the scimitar vein and directly anastomosed to the atrium. A patch of autologous pericardium was used to septate the atrium and an additional patch placed anteriorly to augment the inferior vena cava. RESULTS: Of the 11 patients who had baffling or reimplantation, 5 developed pulmonary vein obstruction between 45 days and 9.5 months after surgery associated with baffle thrombosis or tension on the pulmonary vein. None of the 11 patients who only had the new procedure developed pulmonary vein obstruction during postoperative monitoring up to 3.6 years. CONCLUSIONS: Patients having only the multipatch procedure for repair of scimitar syndrome have not developed postoperative pulmonary vein obstruction in the short to intermediate term.

RESUMEN

The diagnosis of secundum atrial septal defect often is delayed. For 310 patients with hemodynamically significant secundum atrial septal defect undergoing closure over a 5-year period at a single medical center, this study reviews the symptoms prompting referral, limitations of physical examination and electrocardiography, and basis for initially missing the diagnosis.

Asunto(s)

Defectos del Tabique Interatrial/diagnóstico , Adolescente , Adulto , Niño , Preescolar , Diagnóstico Tardío/estadística & datos numéricos , Electrocardiografía , Femenino , Estudios de Seguimiento , Defectos del Tabique Interatrial/fisiopatología , Defectos del Tabique Interatrial/cirugía , Hemodinámica , Humanos , Lactante , Recién Nacido , Masculino , Persona de Mediana Edad , Examen Físico , Estudios Retrospectivos , Adulto Joven

RESUMEN

OBJECTIVES: To determine if patients evaluated using the pediatric chest pain standardized clinical assessment and management plan (SCAMP) in cardiology clinic were later diagnosed with unrecognized cardiac pathology, and to determine if other patients with cardiac pathology not enrolled in the SCAMP would have been identified using the algorithm. STUDY DESIGN: Patients 7-21 years of age, newly diagnosed with hypertrophic or dilated cardiomyopathy, coronary anomalies, pulmonary embolus, pulmonary hypertension, pericarditis, or myocarditis were identified from the Boston Children's Hospital (BCH) cardiac database between July 1, 2010 and December 31, 2012. Patients were cross-referenced to the SCAMP database or retrospectively assessed with the SCAMP algorithm. RESULTS: Among 98 patients with cardiac pathology, 34 (35%) reported chest pain, of whom 10 were diagnosed as outpatients. None of these patients were enrolled in the SCAMP because of alternate chief complaints (n = 4) or referral to BCH for management of the new diagnosis (n = 6). Each of these patients would have had an echocardiogram recommended by retrospective application of the SCAMP algorithm. Two other patients with cardiac pathology were among the 1124 patients assessed by the SCAMP. One patient initially diagnosed with noncardiac chest pain presented 18 months later and was diagnosed with myocarditis as an inpatient. One patient seen initially in the emergency department was subsequently diagnosed with pericarditis as an outpatient. CONCLUSIONS: Patients assessed by the chest pain SCAMP at BCH were not later diagnosed with cardiac pathology that was missed at the initial encounter. Nonenrolled outpatients with cardiac pathology and chest pain would have been successfully identified with the SCAMP algorithm.

Asunto(s)

Técnicas de Imagen Cardíaca/métodos , Dolor en el Pecho/diagnóstico , Manejo de la Enfermedad , Cardiopatías/diagnóstico , Medición de Riesgo/métodos , Adolescente , Dolor en el Pecho/epidemiología , Dolor en el Pecho/etiología , Niño , Diagnóstico Diferencial , Femenino , Estudios de Seguimiento , Cardiopatías/complicaciones , Cardiopatías/epidemiología , Humanos , Incidencia , Masculino , Massachusetts/epidemiología , Pronóstico , Estudios Retrospectivos , Factores de Tiempo , Adulto Joven

RESUMEN

BACKGROUND: Delayed diagnosis of critical congenital heart disease (CCHD) in neonates increases morbidity and mortality. The use of pulse oximetry screening is recommended to increase detection of these conditions. The contribution of pulse oximetry in a tertiary-care birthing center may be different from at other sites. METHODS: We analyzed CCHD pulse oximetry screening for newborns ≥ 35 weeks' gestation born at Brigham and Women's Hospital and cared for in the well-infant nursery during 2013. We identified patients with prenatal diagnosis of CCHD. We also identified infants born at other medical centers who were transferred to Boston Children's Hospital for CCHD and determined if the condition was diagnosed prenatally. RESULTS: Of 6838 infants with complete pulse oximetry data, 6803 (99.5%) passed the first screening. One infant failed all 3 screenings and had the only echocardiogram prompted by screening that showed persistent pulmonary hypertension. There was 1 false-negative screening in an infant diagnosed with interrupted aortic arch. Of 112 infants born at Brigham and Women's Hospital with CCHD, 111 had a prenatal diagnosis, and none was initially diagnosed by pulse oximetry. Of 81 infants transferred to Boston Children's Hospital from other medical centers with CCHD, 35% were diagnosed prenatally. CONCLUSIONS: In our tertiary-care setting, pulse oximetry did not detect an infant with CCHD because of effective prenatal echocardiography screening. Pulse oximetry will detect more infants in settings with a lower prenatal diagnosis rate. Improving training in complete fetal echocardiography scans should also improve timely diagnosis of CCHD.

Asunto(s)

Cardiopatías Congénitas/sangre , Cardiopatías Congénitas/diagnóstico , Tamizaje Neonatal/métodos , Diagnóstico Prenatal/métodos , Femenino , Cardiopatías Congénitas/epidemiología , Humanos , Recién Nacido , Masculino , Oximetría/métodos , Embarazo

RESUMEN

BACKGROUND AND OBJECTIVES: Chest pain is a complaint for which children are frequently evaluated. Cardiac causes are rarely found despite expenditure of considerable time and resources. We describe validation throughout New England of a clinical guideline for cost-effective evaluation of pediatric patients first seen by a cardiologist for chest pain using a unique methodology termed the Standardized Clinical Assessment and Management Plans (SCAMPs). METHODS: A total of 1016 ambulatory patients, ages 7 to 21 years initially seen for chest pain at Boston Children's Hospital (BCH) or the New England Congenital Cardiology Association (NECCA) practices, were evaluated by using a SCAMPs chest pain guideline. Findings were analyzed for diagnostic elements, patterns of care, and compliance with the guideline. Results from the NECCA practices were compared with those of Boston Children's Hospital, a regional core academic center. RESULTS: Two patients had chest pain due to a cardiac etiology, 1 with pericarditis and 1 with an anomalous coronary artery origin. Testing performed outside of guideline recommendations demonstrated only incidental findings. Patients returning for persistent symptoms did not have cardiac disease. The pattern of care for the NECCA practices and BCH differed minimally. CONCLUSIONS: By using SCAMPs methodology, we have demonstrated that chest pain in children is rarely caused by heart disease and can be evaluated in the ambulatory setting efficiently and effectively using minimal resources. The methodology can be implemented regionally across a wide range of clinical practice settings and its approach can overcome a number of barriers that often limit clinical practice guideline implementation.

Asunto(s)

Dolor en el Pecho/diagnóstico , Ecocardiografía/normas , Electrocardiografía/normas , Cardiopatías/diagnóstico , Pediatría/métodos , Guías de Práctica Clínica como Asunto/normas , Adolescente , Atención Ambulatoria/métodos , Dolor en el Pecho/fisiopatología , Dolor en el Pecho/terapia , Niño , Manejo de la Enfermedad , Ecocardiografía/métodos , Electrocardiografía/métodos , Femenino , Estudios de Seguimiento , Adhesión a Directriz , Cardiopatías/fisiopatología , Cardiopatías/terapia , Humanos , Masculino , Radiografía Torácica/normas , Adulto Joven

RESUMEN

BACKGROUND: Scimitar syndrome is a rare congenital anomaly. We evaluated risk factors for postoperative pulmonary vein stenosis or death and predictive factors for survival without scimitar vein surgery in patients with scimitar syndrome. METHODS: The records of patients with scimitar syndrome evaluated at our medical center between 1964 and 2011 were reviewed. RESULTS: Scimitar syndrome was identified in 80 patients, with a median follow-up of 4.5 years. Patients presenting less than 1 year of age had a higher incidence of symptoms, aortopulmonary collaterals, coexisting congenital heart disease (CHD), extracardiac anomalies, and pulmonary hypertension. Of 36 patients having scimitar vein surgery, 18 had postoperative pulmonary vein obstruction that occurred with similar frequency after baffle or reimplantation procedures, early or late in the study period, and tended to be more common in infants (P = .10). Overall, 19 (24%) of 80 died. Multivariate risk factors for death included systolic pulmonary pressure >0.5 systemic level (P = .007) and left pulmonary vein stenosis (P = .009). Pulmonary artery systolic pressure <0.5 systemic level (P = .01) and absence of CHD excluding atrial septal defect (P = .01) were predictive factors in 28 patients who survived and did not have scimitar vein surgery; these patients had no or mild right ventricular dilation and a ratio of pulmonary-to-systemic flow <1.6 either at baseline, after coiling aortopulmonary collaterals or nonscimitar vein intervention. CONCLUSIONS: Postoperative pulmonary vein obstruction is common after scimitar vein surgery regardless of redirection technique. Pulmonary hypertension and left pulmonary vein stenosis are risk factors for death, whereas patients without significant pulmonary hypertension or associated CHD did well without scimitar vein surgery. These observations may guide management decisions in patients with scimitar syndrome.

Asunto(s)

Venas Pulmonares/anomalías , Enfermedad Veno-Oclusiva Pulmonar/etiología , Síndrome de Cimitarra/cirugía , Procedimientos Quirúrgicos Vasculares/efectos adversos , Adolescente , Adulto , Anciano , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Incidencia , Lactante , Recién Nacido , Masculino , Massachusetts/epidemiología , Persona de Mediana Edad , Pronóstico , Circulación Pulmonar , Venas Pulmonares/cirugía , Enfermedad Veno-Oclusiva Pulmonar/epidemiología , Enfermedad Veno-Oclusiva Pulmonar/fisiopatología , Estudios Retrospectivos , Síndrome de Cimitarra/diagnóstico , Síndrome de Cimitarra/mortalidad , Tasa de Supervivencia/tendencias , Factores de Tiempo , Resultado del Tratamiento , Procedimientos Quirúrgicos Vasculares/métodos , Adulto Joven

RESUMEN

OBJECTIVE: To review the short and medium term outcome of transcatheter pulmonary valve perforation (PVP) in patients with pulmonary atresia-intact ventricular septum and non-right ventricular dependant coronary circulation (PA/IVS non-RVDCC). BACKGROUND: PVP in patients with PA/IVS non-RVDCC has become more common in the past two decades. However, data on outcomes with this strategy are mixed. METHODS: Data were reviewed retrospectively for all patients with PA/IVS non-RVDCC treated from 1996- 2010 at our institution. Patients who had severe neonatal Ebstein malformation, or initial interventional management at another institution were excluded. RESULTS: PVP was attempted in 30 of 50 patients (60%); 26 (87%) of these had a successful procedure. Twenty-four patients (48%) had surgery without PVP. There were no deaths in the cohort. Complications of PVP included 5 (17%) myocardial perforations. Of those with successful PVP, 10 (38%) did not have surgery (PVP-NS) and 16 (62%) had surgery (PVP-S) prior to discharge. Tricuspid valve (TV) Z-score was larger in the PVP-NS than in PVP-S patients, with median TV diameter Z-scores of +0.7 (-0.9, 1.7) and -1.1 (-2.8, 2), respectively (P = 0.01). Time from PVP to either hospital discharge (PVP-NS group) or surgery (PVP-S group) was significantly different between groups: 15 (7, 22) and 8 days (0, 46), respectively (P = 0.01). There were no differences in the number of trials or lowest arterial PaO2 off prostaglandins between groups. All patients in the PVP-NS group had a biventricular circulation at a median follow-up of 4.3 years. CONCLUSIONS: The results of a collaborative approach to treating neonates with PA/IVS non-RVDCC are excellent. Smaller TV size is associated with greater likelihood of surgery prior to discharge, and may serve as a surrogate for early RV inadequacy.

Asunto(s)

Cateterismo Cardíaco/métodos , Procedimientos Quirúrgicos Cardíacos/métodos , Cardiopatías Congénitas/cirugía , Atresia Pulmonar/cirugía , Boston , Cateterismo Cardíaco/efectos adversos , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Estudios de Cohortes , Ecocardiografía Doppler/métodos , Femenino , Estudios de Seguimiento , Cardiopatías Congénitas/diagnóstico por imagen , Hospitales Pediátricos , Humanos , Recién Nacido , Masculino , Procedimientos Quirúrgicos Mínimamente Invasivos/efectos adversos , Procedimientos Quirúrgicos Mínimamente Invasivos/métodos , Atresia Pulmonar/diagnóstico por imagen , Estudios Retrospectivos , Medición de Riesgo , Índice de Severidad de la Enfermedad , Resultado del Tratamiento

RESUMEN

BACKGROUND: Chest pain is a common reason for referral to pediatric cardiologists. Although pediatric chest pain is rarely attributable to serious cardiac pathology, extensive and costly evaluation is often performed. We have implemented a standardized approach to pediatric chest pain in our pediatric cardiology clinics as part of a broader quality improvement initiative termed Standardized Clinical Assessment and Management Plans (SCAMPs). In this study, we evaluate the impact of a SCAMP for chest pain on practice variation and resource utilization. METHODS AND RESULTS: We compared demographic variables, clinical characteristics, and cardiac testing in a historical cohort (n=406) of patients presenting to our outpatient division for initial evaluation of chest pain in the most recent pre-SCAMP calendar year (2009) to patients enrolled in the chest pain SCAMP (n=364). Demographic variables including age at presentation, sex, and clinical characteristics were similar between groups. Adherence to the SCAMP algorithm for echocardiography was 84%. Practice variation decreased significantly after implementation of the SCAMP (P<0.001). The number of exercise stress tests obtained was significantly lower in the SCAMP-enrolled patients compared with the historic cohort (∼3% of patients versus 29%, respectively; P<0.001). Similarly, there was a 66% decrease in utilization of Holter monitors and 75% decrease in the use of long-term event monitors after implementation of the chest pain SCAMP (P=0.003 and P<0.001, respectively). The number of echocardiograms obtained was similar between groups. CONCLUSIONS: Implementation of a SCAMP for evaluation of pediatric chest pain has lead to a decrease in practice variation and resource utilization. (J Am Heart Assoc. 2012;1:jah3-e000349 doi: 10.1161/JAHA.111.000349.).

RESUMEN

OBJECTIVES: Chest pain is a common reason for referral to pediatric cardiologists and often leads to an extensive cardiac evaluation. The objective of this study is to describe current management practices in the assessment of pediatric chest pain and to determine whether a standardized care approach could reduce unnecessary testing. PATIENTS AND METHODS: We reviewed all patients, aged 7 to 21 years, presenting to our outpatient pediatric cardiology division in 2009 for evaluation of chest pain. Demographics, clinical characteristics, patient outcomes, and resource use were analyzed. RESULTS: Testing included electrocardiography (ECG) in all 406 patients, echocardiography in 175 (43%), exercise stress testing in 114 (28%), event monitoring in 40 (10%), and Holter monitoring in 30 (7%). A total of 44 (11%) patients had a clinically significant medical or family history, an abnormal cardiac examination, and/or an abnormal ECG. Exertional chest pain was present in 150 (37%) patients. In the entire cohort, a cardiac etiology for chest pain was found in only 5 of 406 (1.2%) patients. Two patients had pericarditits, and 3 had arrhythmias. We developed an algorithm using pertinent history, physical examination, and ECG findings to suggest when additional testing is indicated. Applying the algorithm to this cohort could lead to an ∼20% reduction in echocardiogram and outpatient rhythm monitor use and elimination of exercise stress testing while still capturing all cardiac diagnoses. CONCLUSIONS: Evaluation of pediatric chest pain is often extensive and rarely yields a cardiac etiology. Practice variation and unnecessary resource use remain concerns. Targeted testing can reduce resource use and lead to more cost-effective care.

Asunto(s)

Dolor en el Pecho/diagnóstico , Dolor en el Pecho/terapia , Pediatría/normas , Adolescente , Dolor en el Pecho/fisiopatología , Niño , Manejo de la Enfermedad , Electrocardiografía/métodos , Electrocardiografía/normas , Prueba de Esfuerzo/métodos , Prueba de Esfuerzo/normas , Femenino , Estudios de Seguimiento , Humanos , Angiografía por Resonancia Magnética/métodos , Angiografía por Resonancia Magnética/normas , Masculino , Pediatría/métodos , Estudios Retrospectivos

RESUMEN

INTRODUCTION: Chest pain in children is common, but rarely heralds serious underlying cardiac pathology. Despite this, the anxiety of missing a potentially life threatening condition creates a large burden of referrals and diagnostic testing. We evaluated patients diagnosed with 1 of 9 serious cardiac diseases and detailed the clinical signs and symptoms of the patients presenting with chest pain. METHODS: Patients diagnosed between the ages of 7 and 21 years from January 2000 to December 2009 at Children's Hospital Boston (CHB) were identified from a database using diagnostic and billing codes for aortic dissection, coronary anomalies, dilated cardiomyopathy, hypertrophic cardiomyopathy, myocarditis, pericarditis, pulmonary embolus, pulmonary hypertension, and Takayasu arteritis. Patients with previously diagnosed congenital or acquired heart disease were excluded. RESULTS: Four hundred eighty-four patients were included and 35% presented with chest pain. Forty-one (24%) of these patients with chest pain were diagnosed in the outpatient cardiology clinic, while the remaining 130 patients (76%) were diagnosed in the emergency department (ED) or inpatient setting. Coronary artery anomalies were the most common diagnosis made in cardiology clinic, and 16 of the 23 (70%) patients with serious coronary anomalies had exercise-induced chest pain. Patients presenting to the ED or inpatient units tended to have other important nonspecific symptoms (35-44%), high-risk past medical histories (12%), physical examination findings (32%), and electrocardiogram (ECG) abnormalities (78%) that heighten clinical suspicion of cardiac disease. CONCLUSIONS: Identifying underlying cardiac pathology in the CHB outpatient cardiology department in patients presenting with chest pain is rare, with only 41 cases over a 10-year period. The presence of exertional chest pain was important in identifying patients with coronary artery anomalies. A detailed history and physical examination, along with a critical review of an ECG, seem to identify those patients with rare diseases who need further diagnostic testing.

Asunto(s)

Dolor en el Pecho/etiología , Electrocardiografía , Cardiopatías/diagnóstico , Adolescente , Boston , Servicio de Cardiología en Hospital , Niño , Servicio de Urgencia en Hospital , Prueba de Esfuerzo , Femenino , Cardiopatías/complicaciones , Hospitales Pediátricos , Humanos , Masculino , Anamnesis , Servicio Ambulatorio en Hospital , Examen Físico , Valor Predictivo de las Pruebas , Pronóstico , Adulto Joven

RESUMEN

We report 4 teenage patients who, after successful palliation of severe aortic valve stenosis by balloon aortic valvuloplasty in early infancy, presented within the previous 3 years with significant left ventricular (LV) diastolic heart failure. All patients had remained asymptomatic until their teenage years and had normal or hyperdynamic LV systolic function on presentation, with limited residual aortic valve stenosis and regurgitation. All underwent echocardiography, cardiac catheterization, and cardiac magnetic resonance evaluation. One notable common feature in the 4 patients was the presence of a confluent layer of LV subendocardial hyperenhancement demonstrated by late gadolinium enhancement technique. Histopathology was available for 2 patients, which documented significant LV endocardial fibroelastosis. One patient who underwent endocardial fibroelastosis resection in conjunction with aortic valve replacement had late clinical improvement. In conclusion, this group of patients may represent an important emerging clinical entity and merits close clinical surveillance, with prospective assessment of diastolic function.

Asunto(s)

Estenosis de la Válvula Aórtica/congénito , Cateterismo , Disfunción Ventricular Izquierda/etiología , Disfunción Ventricular Izquierda/cirugía , Adolescente , Estenosis de la Válvula Aórtica/cirugía , Medios de Contraste , Diástole , Progresión de la Enfermedad , Ecocardiografía , Humanos , Imagen por Resonancia Magnética , Masculino

Asunto(s)

Aorta Torácica/anomalías , Aorta Torácica/diagnóstico por imagen , Tronco Braquiocefálico/anomalías , Tronco Braquiocefálico/diagnóstico por imagen , Niño , Preescolar , Femenino , Humanos , Masculino , Tomografía Computarizada por Rayos X

RESUMEN

BACKGROUND: Clinical practice is discrepant regarding routine enlargement of the interatrial communication (IAC) in patients with right-sided atrioventricular valve atresia. We determined the percentage and risk factors of those who develop a restrictive IAC. METHODS: Medical records were reviewed for patients treated from 1985 to 2006, including those admitted in the first 6 weeks of life (group A), and those referred at a later age (group B), some of whom had routine atrial septal procedures. In group A, we analyzed the initial postnatal echocardiogram. RESULTS: Group A consisted of 79 patients, 16 of whom had an atrial septectomy as part of the initial surgical procedure. Of the remaining 63 patients, 9 (14%) developed atrial septal restriction. In group A, an atrial septal aneurysm (ASA) (OR 16, P = .006) and IAC diameter < 5 mm (OR 13, P = .009) were associated with atrial septal restriction. Atrial septal restriction occurred in 80% of patients with both features, 20% with IAC > or = 5 mm and ASA, 18% with IAC < 5 mm and no ASA, and 2% with neither feature. Group B consisted of 95 patients, 27 of whom had an atrial septal procedure. Of the remaining 68 patients, 11 (16%) developed atrial septal restriction. CONCLUSION: Routine enlargement of the IAC is not necessary in patients with right-sided atrioventricular valve atresia. Patients with ASA or IAC < 5 mm are at increased risk for development of atrial septal restriction.

Asunto(s)

Foramen Oval Permeable/patología , Atresia Tricúspide , Adolescente , Tabique Interatrial/patología , Tabique Interatrial/cirugía , Niño , Preescolar , Ecocardiografía , Estudios de Seguimiento , Foramen Oval Permeable/diagnóstico por imagen , Foramen Oval Permeable/cirugía , Hemodinámica , Humanos , Lactante , Recién Nacido , Factores de Riesgo , Atresia Tricúspide/diagnóstico por imagen , Atresia Tricúspide/fisiopatología

RESUMEN

OBJECTIVES: The purpose of this research was to investigate the causes and symptoms of superior vena cava (SVC) obstruction or occlusion and report on the long-term results of transcatheter therapy. BACKGROUND: Information on transcatheter therapy for SVC obstruction is limited. METHODS: Superior vena cava catheterization interventions between August 1984 and April 2006 were reviewed. Patients were divided into 2 subgroups depending on whether or not they had previously undergone congenital cardiac surgery. RESULTS: Sixty-three patients with median age of 3.7 years (range 1 month to 42 years) and weight of 13.3 kg (range 3 to 114 kg) were treated. Fifty patients (79%) were symptomatic, although only 50% had symptoms suggestive of SVC obstruction. Superior vena cava syndrome was more common in the non-cardiac surgical group (52% vs. 10%, p = 0.001). The mean gradient and SVC diameter improved from 10.8 +/- 5.8 mm Hg to 2.6 +/- 2.2 mm Hg (p < 0.001) and 3.1 +/- 2.7 mm to 9.1 +/- 3.8 mm, respectively (p < 0.001). The obstruction was adequately relieved in all 36 patients receiving stents and in 21 of 27 patients (78%) who had balloon dilation alone. Complications occurred in 12 patients (19%), all of whom had previously undergone cardiac surgery; 10 of these patients were successfully treated in the catheterization laboratory. Freedom from re-intervention did not differ between patients undergoing balloon dilation or stent implantation, but was longer in patients age >5 years at the time of intervention. CONCLUSIONS: Superior vena cava-related symptoms occur in only 50% of patients with hemodynamically significant SVC obstruction. Endovascular therapy is successful in relieving the stenosis and associated symptoms with good long-term results.

Asunto(s)

Cateterismo Venoso Central/estadística & datos numéricos , Síndrome de la Vena Cava Superior/terapia , Adolescente , Adulto , Distribución por Edad , Presión Sanguínea , Boston/epidemiología , Estimulación Cardíaca Artificial/estadística & datos numéricos , Enfermedades Cardiovasculares/epidemiología , Enfermedades Cardiovasculares/terapia , Cateterismo/estadística & datos numéricos , Causalidad , Niño , Preescolar , Comorbilidad , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Análisis Multivariante , Modelos de Riesgos Proporcionales , Stents/estadística & datos numéricos , Síndrome de la Vena Cava Superior/epidemiología , Síndrome de la Vena Cava Superior/fisiopatología , Análisis de Supervivencia , Resultado del Tratamiento

RESUMEN

BACKGROUND: The improved survival of very low-birth-weight (<1,500 g) infants justifies more aggressive attempts to treat underlying congenital heart disease than in the past. METHODS: We retrospectively reviewed all catheterizations performed at our institution between January 1, 1990 and June 1, 2004 in infants weighing <1,500 g. We performed a 3:1 case-control study. Comparisons were randomly selected from a group of patients catheterized within 6 months of the cases and weighing 2-3 kg. All catheterization data, angiograms, and hospital charts were reviewed. RESULTS: Eighteen patients weighing <1,500 g underwent catheterization. Fifty-four patients were selected as comparisons. There were no significant differences in the age at catheterization, procedure time, fluoroscopy time, or contrast amount (cc/kg). The lower-birth-weight infants were more likely to be premature (median age 29 vs. 37 weeks, P < 0.001), and to have left-sided obstructive lesions including aortic stenosis or coarctation. The comparison patients were more likely to be postoperative (28% vs. 0%, P = 0.02), and included a higher number with hypoplastic left heart syndrome. There was an increased incidence of interventions performed in the lower-birth-weight infants (83% vs. 41%, P = 0.002). There was a difference in the interventions performed between the two groups: the comparisons had more atrial septal procedures, and the lower-birth-weight infants had more coarctation dilations and aortic valve dilations. There were no significant differences in the acute success rate of the procedures (100% vs. 95%), overall complication rate (56 vs. 57%), incidence of blood transfusions (44 vs. 30%), or major complications (11 vs. 13%) between the lower-birth-weight and comparison groups respectively. There was a trend towards higher survival rate in the comparison group in this small study population, but it did not reach significance (80 vs. 61%, P = 0.13). CONCLUSIONS: Cardiac catheterization in neonates <1,500 g is more likely to include percutaneous intervention, especially on the left side, but is generally successful with a complication rate similar to procedures performed in larger infants. Although these procedures are rare, improved miniaturization of equipment would facilitate safer interventions.

Asunto(s)

Angioplastia Coronaria con Balón/métodos , Cateterismo Cardíaco/métodos , Cardiopatías Congénitas/cirugía , Recién Nacido de muy Bajo Peso , Angioplastia Coronaria con Balón/efectos adversos , Cateterismo Cardíaco/efectos adversos , Estudios de Casos y Controles , Humanos , Recién Nacido , Estudios Retrospectivos