RESUMEN
BACKGROUND: A superior cavopulmonary connection is commonly performed before the Fontan procedure in patients with a functionally univentricular heart. Data are limited regarding associations between a prior superior cavopulmonary connection and functional and ventricular performance late after the Fontan procedure. METHODS: We compared characteristics of those with and without prior superior cavopulmonary connection among 546 subjects enrolled in the Pediatric Heart Network Fontan Cross-Sectional Study. We further compared different superior cavopulmonary connection techniques: bidirectional cavopulmonary anastomosis (n equals 229), bilateral bidirectional cavopulmonary anastomosis (n equals 39), and hemi-Fontan (n equals 114). RESULTS: A prior superior cavopulmonary connection was performed in 408 subjects (75%); the proportion differed by year of Fontan surgery and centre (p-value less than 0.0001 for each). The average age at Fontan was similar, 3.5 years in those with superior cavopulmonary connection versus 3.2 years in those without (p-value equals 0.4). The type of superior cavopulmonary connection varied by site (p-value less than 0.001) and was related to the type of Fontan procedure. Exercise performance, echocardiographic variables, and predominant rhythm did not differ by superior cavopulmonary connection status or among superior cavopulmonary connection types. Using a test of interaction, findings did not vary according to an underlying diagnosis of hypoplastic left heart syndrome. CONCLUSIONS: After controlling for subject and era factors, most long-term outcomes in subjects with a prior superior cavopulmonary connection did not differ substantially from those without this procedure. The type of superior cavopulmonary connection varied significantly by centre, but late outcomes were similar.
Asunto(s)
Procedimiento de Fontan , Puente Cardíaco Derecho/métodos , Cardiopatías Congénitas/cirugía , Calidad de Vida , Adolescente , Niño , Preescolar , Estudios Transversales , Femenino , Cardiopatías Congénitas/fisiopatología , Pruebas de Función Cardíaca , Humanos , Masculino , Resultado del TratamientoRESUMEN
OBJECTIVES: This study was undertaken to determine the effects of creating a systemic-to-pulmonary venous atrial-level communication (fenestration) at the time of the Fontan procedure on late outcomes. BACKGROUND: Fenestrations are frequently performed during Fontan procedures, but late consequences are not well described. METHODS: Patient characteristics were compared between those with and without surgical fenestration among 536 subjects (mean age 11.9 years) enrolled in the Pediatric Heart Network Fontan Cross-Sectional Study. The status of the fenestration and the association of a currently patent fenestration with health status and measures of ventricular performance were investigated. RESULTS: Fenestration was performed in 361 patients (67%), and frequency differed by year and center (p < 0.001 for each). After adjustment for center, age at Fontan, year of Fontan, and prior superior cavopulmonary surgery, the fenestrated group had shorter length of Fontan hospital stay. At the time of cross-sectional testing 8 ± 3 years after Fontan, the fenestration remained open in 19% of subjects. Among those with confirmed fenestration closure, 59% were by catheter intervention and 1% by surgical intervention, and 40% had apparent spontaneous closure. Compared with those without evidence of a fenestration, subjects with a current fenestration were taking more medications (p = 0.02) and had lower resting oxygen saturation (median 89% vs. 95%, p < 0.001). Functional health status, exercise performance, echocardiographic variables, prevalence of post-Fontan stroke or thrombosis, and growth did not differ by current fenestration status. CONCLUSIONS: Surgical fenestration is associated with well-demonstrated early post-operative benefits. This cross-sectional study found few associations between a persistent fenestration and deleterious later outcomes.
Asunto(s)
Procedimiento de Fontan/métodos , Cardiopatías Congénitas/cirugía , Hemodinámica/fisiología , Adolescente , Anastomosis Quirúrgica/métodos , Cateterismo Cardíaco/métodos , Niño , Estudios de Cohortes , Intervalos de Confianza , Estudios Transversales , Femenino , Estudios de Seguimiento , Procedimiento de Fontan/efectos adversos , Atrios Cardíacos/cirugía , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/mortalidad , Pruebas de Función Cardíaca , Humanos , Cuidados Intraoperatorios/métodos , Tiempo de Internación , Modelos Lineales , Modelos Logísticos , Masculino , Análisis Multivariante , Cuidados Posoperatorios/métodos , Venas Pulmonares/cirugía , Medición de Riesgo , Resultado del TratamientoRESUMEN
Prenatal diagnosis of congenital heart disease (CHD) is increasingly common. However, the current impact of prenatal diagnosis on neonatal outcomes is unclear. Between January 2004 and January 2008, a retrospective chart review of infants who underwent surgical repair of CHD before discharge at our institution was conducted. Obstetric and perioperative variables were recorded. Of 439 neonates, 294 (67%) were diagnosed prenatally (PREdx). Infants with PREdx had a lower mean birth weight (3.0 +/- 0.6 vs. 3.1 +/- 0.6 kg, p = 0.002) and gestational age (37.9 +/- 2.1 vs. 38.6 +/- 2.4 wk, p < 0.001) than those with postnatal diagnosis (POSTdx). Severe lesions were more likely to be PREdx: Neonates with single-ventricle (SV) physiology (n = 130 patients [31.2%]) had increased odds of PREdx (n = 113/130, odds ratio [OR] 4.7; 95% confidence interval [CI] 2.7-8.2, p < 0.001). PREdx was associated with decreased preoperative intubation (OR 0.62; 95% CI 0.42-0.95, p = 0.033), administration of antibiotics (OR 0.23; 95% CI 0.15-0.36, p < 0.001), cardiac catheterization (OR 0.54; 95% CI 0.34-0.85, p = 0.01), and emergency surgery (OR 0.18; 95% CI 0.06-0.5, p < 0.001) compared with POSTdx infants. There was no difference in APGAR scores, preoperative pH, day of life of surgery, operative complications, hospital length of stay, or overall mortality in the PREdx versus POSTdx groups, even when controlling for lesion severity. PREdx was not independently associated with neonatal mortality, despite having included more severe cardiac lesions. PREdx was significantly associated with decreased neonatal morbidity in terms of decreased use of preoperative ventilator, administration of antibiotics, cardiac catheterization, and emergency surgery.
Asunto(s)
Cardiopatías Congénitas/diagnóstico , Diagnóstico Prenatal , Peso al Nacer , Distribución de Chi-Cuadrado , Femenino , Edad Gestacional , Cardiopatías Congénitas/mortalidad , Cardiopatías Congénitas/cirugía , Humanos , Mortalidad Infantil , Recién Nacido , Tiempo de Internación/estadística & datos numéricos , Modelos Logísticos , Masculino , Embarazo , Resultado del Embarazo , Prevalencia , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Índice de Severidad de la EnfermedadRESUMEN
BACKGROUND: Patients after the Fontan procedure are at risk for suboptimal functional health status, and associations with laboratory measures are important for planning interventions and outcome measures for clinical trials. METHODS AND RESULTS: Parents completed the generic Child Health Questionnaire for 511 Fontan Cross-Sectional Study patients 6 to 18 years of age (61% male). Associations of Child Health Questionnaire Physical and Psychosocial Functioning Summary Scores (FSS) with standardized measurements from prospective exercise testing, echocardiography, magnetic resonance imaging, and measurement of brain natriuretic peptide were determined by regression analyses. For exercise variables for maximal effort patients only, the final model showed that higher Physical FSS was associated only with higher maximum work rate, accounting for 9% of variation in Physical FSS. For echocardiography, lower Tei index (particularly for patients with extracardiac lateral tunnel connections), lower indexed end-systolic volume, and the absence of atrioventricular valve regurgitation for patients having Fontan procedure at age <2 years were associated with higher Physical FSS, accounting for 14% of variation in Physical FSS. For magnetic resonance imaging, ratio of lower mass to end-diastolic volume and midquartiles of indexed end-systolic volume (nonlinear) were associated with higher Physical FSS, accounting for 11% of variation. Lower brain natriuretic peptide was significantly but weakly associated with higher Physical FSS (1% of variation). Significant associations for Psychosocial FSS with laboratory measures were fewer and weaker than for Physical FSS. CONCLUSIONS: In relatively healthy Fontan patients, laboratory measures account for a small proportion of the variation in functional health status and therefore may not be optimal surrogate end points for trials of therapeutic interventions.
Asunto(s)
Tolerancia al Ejercicio/fisiología , Procedimiento de Fontan , Estado de Salud , Cardiopatías Congénitas/cirugía , Complicaciones Posoperatorias/fisiopatología , Disfunción Ventricular/fisiopatología , Adolescente , Volumen Cardíaco , Niño , Estudios Transversales , Ecocardiografía , Prueba de Esfuerzo , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Péptido Natriurético Encefálico/sangre , Complicaciones Posoperatorias/diagnóstico por imagen , Complicaciones Posoperatorias/patología , Encuestas y Cuestionarios , Disfunción Ventricular/diagnóstico por imagen , Disfunción Ventricular/patologíaRESUMEN
OBJECTIVES: Cardiac catheterization has a low diagnostic yield before a Fontan operation, and magnetic resonance imaging and computed tomography are reliable alternatives to invasive angiography. A new diagnostic algorithm to avoid cardiac catheterization in "low-risk" subjects before a Fontan operation is proposed. METHODS: The proposed algorithm would identify "high-risk" subjects on the basis of risk factors on medical history, echocardiography, and noninvasive angiography. The efficacy of this algorithm in screening for subjects deemed to be inoperable after catheterization was evaluated retrospectively in 151 children. For this analysis, results of conventional angiography (assumed to be equivalent to noninvasive angiography) were used. RESULTS: According to the algorithm, 95 (63%) of 151 subjects had no risk factors ("low risk") whereas 56 (37%) of 151 had 1 risk factor or more ("high risk"). Nine (6%) of 151 subjects were found to be inoperable after catheterization and all 9 were correctly classified as high risk by the algorithm. In the 135 of 151 subjects who underwent a Fontan operation, the algorithm predicted an adverse postoperative outcome with a sensitivity of 51% and specificity of 78%. However, this prediction was not improved by including elevated pulmonary artery pressure or ventricular filling pressure as additional risk factors. CONCLUSIONS: The proposed algorithm effectively screened for subjects who were deemed unsuitable for a Fontan procedure. In addition, omitting preoperative invasive hemodynamic assessment did not impair prediction of adverse postoperative outcomes. Prospective evaluation of such a noninvasive diagnostic strategy before the Fontan operation is warranted.
Asunto(s)
Procedimiento de Fontan , Cardiopatías Congénitas/diagnóstico , Ventrículos Cardíacos/anomalías , Algoritmos , Angiografía , Cateterismo Cardíaco , Preescolar , Ecocardiografía , Femenino , Procedimiento de Fontan/efectos adversos , Procedimiento de Fontan/mortalidad , Cardiopatías Congénitas/cirugía , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Masculino , Valor Predictivo de las Pruebas , Circulación Pulmonar , Factores de Riesgo , Sensibilidad y EspecificidadRESUMEN
OBJECTIVES: To test the hypothesis that long-term survivors of low-risk Kawasaki disease (KD) have ongoing vascular inflammation and dysfunction and a higher risk of accelerated atherosclerosis than healthy control subjects. STUDY DESIGN: Twenty-eight patients with KD (7-20 years after acute illness) and 27 age-matched healthy control subjects were examined for medical and dietary history, serum markers of atherosclerotic risk and inflammation, carotid intimal-medial thickness (CIMT) with vascular ultrasound scanning and arterial stiffness with applanation tonometry. RESULTS: Patients and control subjects were similar in age, sex, body mass index, waist-to-hip ratio, blood pressure, cigarette smoking, family history, diet, high-density lipoprotein cholesterol level, lipoprotein (a) level, homocysteine level, glucose level, insulin level, CIMT, arterial stiffness, C-reactive protein level, and inflammatory cytokine level. Levels of total cholesterol and apolipoprotein B were significantly higher in patients with KD than in control subjects. CONCLUSIONS: There was no evidence of increased atherosclerosis. Small but significant differences in cholesterol and apolipoprotein B levels could suggest increased future risk for atherosclerosis and warrant further study.
Asunto(s)
Aterosclerosis/epidemiología , Síndrome Mucocutáneo Linfonodular/metabolismo , Síndrome Mucocutáneo Linfonodular/patología , Proteínas de Fase Aguda/metabolismo , Adolescente , Adulto , Aterosclerosis/diagnóstico , Biomarcadores/metabolismo , Arterias Carótidas/patología , Estudios de Casos y Controles , Citocinas/sangre , Femenino , Humanos , Mediadores de Inflamación/sangre , Lípidos/sangre , Masculino , Síndrome Mucocutáneo Linfonodular/complicaciones , Factores de Riesgo , Adulto JovenRESUMEN
BACKGROUND: Despite improvements in outcomes after completion of the Fontan circulation, long-term functional state varies. We sought to identify pre- and postoperative characteristics associated with overall function. METHODS AND RESULTS: We analyzed data from 476 survivors with the Fontan circulation enrolled in the Pediatric Heart Network Fontan Cross-sectional Study. Mean age at creation of the Fontan circulation was 3.4 plus or minus 2.1 years, with a range from 0.7 to 17.5 years, and time since completion was 8.7 plus or minus 3.4 years, the range being from 1.1 to 17.3 years. We calculated a functional score for the survivors by averaging the percentile ranks of ventricular ejection fraction, maximal consumption of oxygen, the physical summary score for the Child Health Questionnaire, and a function of brain natriuretic peptide. The mean calculated score was 49.5 plus or minus 17.3, with a range from 3 to 87. After adjustment for time since completion of the circulation, we found that a lower score, and hence worse functional state, was associated with: right ventricular morphology (p less than 0.001), higher ventricular end-diastolic pressure (p equals 0.003) and lower saturations of oxygen (p equals 0.047) prior to completion of the Fontan circulation, lower income for the caregiver (p equals 0.003), and, in subjects without a prior superior cavopulmonary anastomosis, arrhythmias after completion of the circulation (p equals 0.003). The model explained almost one-fifth (18%) of the variation in the calculated scores. The score was not associated with surgical centre, sex, age, weight, fenestration, or the period of stay in hospital after completion of the Fontan circuit. A validation model, using 71 subjects randomly excluded from initial analysis, weakly correlated (R equals 0.17, p equals 0.16) with the score calculated from the dataset. CONCLUSIONS: Right ventricular morphology, higher ventricular end-diastolic pressure and lower saturations of oxygen prior to completion of the Fontan circuit, lower income for the provider of care, and arrhythmias after creation of the circuit, are all associated with a worse functional state. Unmeasured factors also influence outcomes.
Asunto(s)
Procedimiento de Fontan , Cardiopatías Congénitas/cirugía , Recuperación de la Función , Volumen Sistólico/fisiología , Función Ventricular/fisiología , Adolescente , Niño , Estudios Transversales , Femenino , Estudios de Seguimiento , Cardiopatías Congénitas/fisiopatología , Humanos , Masculino , Periodo Posoperatorio , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
OBJECTIVE: To investigate the incidence of aortic root dilatation in pectus excavatum. DESIGN: Retrospective medical record review and echocardiographic reanalysis. SETTING: Morgan Stanley Children's Hospital of New York-Presbyterian. PARTICIPANTS: Surgical candidates with pectus excavatum (n = 37) and age-matched controls (n = 44) referred for an echocardiogram from 1994 to 2002. INTERVENTIONS: Two-dimensional and color Doppler transthoracic echocardiograms. OUTCOME MEASURES: The aortic annulus and root were measured and z scores were calculated and compared. Medical records were reviewed for genetic evaluation. RESULTS: Patients with pectus excavatum and age-matched controls were reanalyzed. There was no difference in age, weight, height, or body surface area between patients and controls. There were no differences in the mean aortic annulus diameter, mean aortic annulus z score, or mean aortic root measurements. However, the aortic root z score was significantly higher in the pectus excavatum group compared with the controls: 0.9 (SD, 1.06) vs 0.0 (SD, 1.25) (P = .001). There were more patients with an aortic root z score of 2 or greater in the pectus excavatum group (9 of 37 patients) than in the control group (0 of 43 controls), with a calculated odds ratio of 29.7 (95% confidence interval, 1.10-1.59). Genetic evaluation was performed in 5 patients with a pectus excavatum and dilated aortic root; 2 of them received diagnoses of Marfan syndrome. CONCLUSIONS: Aortic root dilatation is more common in patients with pectus excavatum than in a control population. Echocardiographic screening may be useful in the identification of aortic root dilatation in patients with isolated pectus excavatum.
Asunto(s)
Aorta Torácica/diagnóstico por imagen , Tórax en Embudo/diagnóstico por imagen , Síndrome de Marfan/diagnóstico por imagen , Estudios de Casos y Controles , Distribución de Chi-Cuadrado , Niño , Dilatación Patológica , Ecocardiografía , Femenino , Humanos , Masculino , Estudios RetrospectivosRESUMEN
BACKGROUND: This study aimed to evaluate the impact of prenatal diagnosis on parental understanding of congenital heart disease (CHD) in newborns. METHODS: Consenting parents of newborns with CHD answered questions about the cardiac lesion, surgical repair, follow-up management, risk for CHD in future children, and maternal education before neonatal intensive care unit (NICU) discharge. A total understanding score was calculated (0-10) as the sum of five subscores: physician score, CHD score, surgery score, follow-up score, and reproduction score. Each category was scored as 0 (none correct), 1 (some correct), or 2 (all correct). The prenatal and postnatal diagnoses scores were compared. RESULTS: From June 2006 to November 2006, 50 families completed the questionnaire. Of these 50 families, 26 reported a prenatal diagnosis. The mean infant age when the parents were approached was 17.3 +/- 13.3 days. The summary understanding score for the entire group was 6.3 +/- 2.4 of 10. Multivariate regression analysis demonstrated a difference in scores between prenatal and postnatal diagnosis groups (p = 0.02) when control was used for maternal education. Prenatal diagnosis and maternal education (p < 0.01) had independent effects on the score. CONCLUSION: Prenatal diagnosis increases parental understanding of neonatal CHD. Nevertheless, parental understanding remains suboptimal.
Asunto(s)
Cardiopatías Congénitas/psicología , Padres/psicología , Estudios Transversales , Femenino , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/terapia , Humanos , Recién Nacido , Unidades de Cuidado Intensivo Neonatal , Masculino , Proyectos Piloto , Embarazo , Diagnóstico Prenatal , Análisis de Regresión , Encuestas y CuestionariosRESUMEN
OBJECTIVES: We characterized a large cohort of children who had a Fontan procedure, with measures of functional health status, ventricular size and function, exercise capacity, heart rhythm, and brain natriuretic peptide (BNP). BACKGROUND: The characteristics of contemporary Fontan survivors are not well described. METHODS: We enrolled 546 children (age 6 to 18 years, mean 11.9 years) and compared them within pre-specified anatomic and procedure subgroups. History and outcome measures were obtained within a 3-month period. RESULTS: Predominant ventricular morphology was 49% left ventricular (LV), 34% right ventricular (RV), and 19% mixed. Ejection fraction (EF) was normal for 73% of subjects; diastolic function grade was normal for 28%. Child Health Questionnaire mean summary scores were lower than for control subjects; however, over 80% of subjects were in the normal range. Brain natriuretic peptide concentration ranged from <4 to 652 pg/ml (median 13 pg/ml). Mean percent predicted peak O2 consumption was 65% and decreased with age. Ejection fraction and EF Z score were lowest, and semilunar and atrioventricular (AV) valve regurgitation were more prevalent in the RV subgroup. Older age at Fontan was associated with more severe AV valve regurgitation. Most outcomes were not associated with a superior cavopulmonary connection before Fontan. CONCLUSIONS: Measures of ventricular systolic function and functional health status, although lower on average in the cohort compared with control subjects, were in the majority of subjects within 2 standard deviations of the mean for control subjects. Right ventricular morphology was associated with poorer ventricular and valvular function. Effective strategies to preserve ventricular and valvular function, particularly for patients with RV morphology, are needed.
Asunto(s)
Procedimiento de Fontan , Cardiopatías Congénitas/cirugía , Adolescente , Factores de Edad , Niño , Preescolar , Estudios de Cohortes , Estudios Transversales , Ejercicio Físico/fisiología , Femenino , Estado de Salud , Corazón/fisiología , Cardiopatías Congénitas/sangre , Cardiopatías Congénitas/patología , Cardiopatías Congénitas/fisiopatología , Ventrículos Cardíacos/patología , Humanos , Masculino , Péptido Natriurético Encefálico/sangre , Factores Sexuales , Resultado del Tratamiento , Función Ventricular/fisiologíaRESUMEN
A covered stent was used for percutaneous closure of an unroofed coronary sinus in an infant with congestive heart failure secondary to increased pulmonary flow. Prior to the stent deployment, the location of the entrance of the coronary vein into the coronary sinus was demonstrated with a selective left coronary artery angiogram to facilitate placement of the stent. The procedure was well tolerated without complications. The infant's symptoms improved significantly after the procedure.
Asunto(s)
Cardiopatías Congénitas/terapia , Stents , Anomalías Múltiples , Cateterismo Cardíaco , Angiografía Coronaria , Diagnóstico Diferencial , Ecocardiografía , Electrocardiografía , Cardiopatías Congénitas/diagnóstico , Humanos , Lactante , Masculino , Radiografía Torácica , SíndromeRESUMEN
BACKGROUND: Most studies of coronary artery involvement and associated risk factors in Kawasaki disease have used the Japanese Ministry of Health dichotomous criteria. Analysis of serial normalized artery measurements may reveal a broader continuous spectrum of involvement and different risk factors. METHODS AND RESULTS: Clinical, laboratory, and echocardiographic measurements obtained at baseline and 1 week and 5 weeks after presentation were examined in 190 Kawasaki disease patients as part of a clinical trial of primary therapy with pulse steroids in addition to standard intravenous immunoglobulin. Maximum coronary artery z score normalized to body surface area was significantly greater than normal at all time points, decreasing significantly over time from baseline. A maximal z score > or = 2.5 at any time was noted in 26% of patients. Japanese Ministry of Health dimensional criteria were met by 23% of patients. Significant independent factors associated with greater z score at any time included younger patient age, longer interval from disease onset to treatment with intravenous immunoglobulin, lower serum IgM level at baseline, and lower minimum serum albumin level. z scores of the proximal right coronary artery were higher than those in the left anterior descending branch. CONCLUSIONS: Analyses of serial normalized coronary artery measurements in optimally treated Kawasaki disease patients demonstrated that for most patients, measurements are greatest at baseline and subsequently diminish; baseline measurements appear to be good predictors of involvement during early follow-up. When a more precise assessment is used, risk factors for coronary artery involvement are similar to those defined with arbitrary dichotomous criteria.
Asunto(s)
Enfermedad Coronaria/epidemiología , Enfermedad Coronaria/fisiopatología , Síndrome Mucocutáneo Linfonodular/complicaciones , Niño , Enfermedad Coronaria/prevención & control , Vasos Coronarios/anatomía & histología , Vasos Coronarios/fisiología , Vasos Coronarios/fisiopatología , Humanos , Síndrome Mucocutáneo Linfonodular/terapia , Valores de Referencia , Factores de RiesgoRESUMEN
The natural history of aortic cuspal prolapse and aortic regurgitation (AR), studied most commonly in subpulmonic ventricular septal defect (VSD), has not been well defined in isolated, unrepaired VSD diagnosed during infancy. This study aimed to define the incidence and progression of aortic cuspal prolapse and AR in patients with subaortic VSDs diagnosed at <1 year of age who had no aortic cuspal prolapse or AR at presentation and did not require surgery within the first year of life. Patients had yearly follow-up, and data regarding clinical course, physical examination, and echocardiography were obtained. Comparisons were made between patients who developed aortic cuspal prolapse and AR and those who did not. One hundred patients, with a mean age at VSD diagnosis of 0.1 +/- 0.5 years, followed for a mean of 7.1 +/- 10.1 years, were studied. Aortic cuspal prolapse developed in 14 patients (14%) at a mean age of 7.1 +/- 6 years (range 0.4 to 18.4). AR murmurs were heard in 6 patients (6%) at a mean age of 5.1 +/- 3.1 years, all of whom had aortic cuspal prolapse and underwent VSD closure and aortic valvuloplasty. In conclusion, aortic cuspal prolapse and clinical AR are not uncommon in patients with subaortic VSDs. Long-term follow-up of patients with subaortic VSDs should include the serial evaluation of aortic valve anatomy and function.
Asunto(s)
Insuficiencia de la Válvula Aórtica/etiología , Prolapso de la Válvula Aórtica/etiología , Defectos del Tabique Interventricular/complicaciones , Insuficiencia de la Válvula Aórtica/diagnóstico por imagen , Insuficiencia de la Válvula Aórtica/epidemiología , Insuficiencia de la Válvula Aórtica/cirugía , Prolapso de la Válvula Aórtica/diagnóstico por imagen , Prolapso de la Válvula Aórtica/epidemiología , Prolapso de la Válvula Aórtica/cirugía , Cateterismo Cardíaco , Progresión de la Enfermedad , Ecocardiografía Doppler en Color , Femenino , Estudios de Seguimiento , Defectos del Tabique Interventricular/diagnóstico por imagen , Defectos del Tabique Interventricular/epidemiología , Defectos del Tabique Interventricular/cirugía , Implantación de Prótesis de Válvulas Cardíacas , Humanos , Incidencia , Lactante , Recién Nacido , Masculino , New York/epidemiología , Índice de Severidad de la EnfermedadRESUMEN
OBJECTIVES: To test the hypothesis that left ventricular (LV) dilation associated with pressure-restrictive ventricular septal defect (VSD) often remains stable or regresses spontaneously, calling into question the role of interventional management for such defects. STUDY DESIGN: We analyzed 96 serial echocardiograms from 33 unoperated patients with a moderate-to-large VSD with LV dilation (LV end-diastolic dimension [LVED] z score >2.0) at enrollment who were followed for more than 2 years. Records of 125 surgical patients also were reviewed. Patients were evaluated for evidence of persistent or progressive LV dilation; signs or symptoms of congestive heart failure (CHF), failure to thrive (FTT), or pulmonary hypertension (PAH); as well as acquired ventricular outflow obstruction or aortic regurgitation. LVED z scores at enrollment versus latest follow-up were compared using paired t tests. A random-effects model with random intercept and slope was fitted to account for repeated observations for each patient. RESULTS: Mean age at enrollment was 4.6 +/- 3.2 years, and mean follow-up was 7.8 +/- 4 years (range, 2.8 to 22 years), during which mean LVED z score decreased from 3.0 +/- 0.6 to 1.2 +/- 1.3 (P < .01). LVED z score decreased in 29 of the 33 patients, and decreased to <2 in 26 of these 29 (79%). CONCLUSIONS: Most patients with pressure-restrictive VSD with moderate-to-severe LV dilation without CHF, FTT, or PAH will experience spontaneous resolution of LV dilation and can avoid cardiac surgery or catheter-based intervention.
Asunto(s)
Defectos del Tabique Interventricular/patología , Ventrículos Cardíacos/patología , Niño , Preescolar , Dilatación Patológica , Defectos del Tabique Interventricular/diagnóstico por imagen , Defectos del Tabique Interventricular/fisiopatología , Defectos del Tabique Interventricular/terapia , Humanos , Lactante , Remisión Espontánea , Volumen Sistólico , Ultrasonografía Doppler , Presión VentricularRESUMEN
OBJECTIVE: To test the hypothesis that chronic beta-blocker therapy in pediatric patients with Marfan syndrome alters the rate of aortic root dilation. Beta-blockade has been advocated as preventive therapy for Marfan syndrome based on reports indicating a decreased rate of aortic root dilation in treated patients. STUDY DESIGN: Patients with Marfan syndrome (n = 63) followed at Children's Hospital of Pittsburgh or Children's Hospital of New York-Presbyterian who had > or =18 months of echocardiographic follow-up were studied. All clinical data and 213 serial echocardiograms were reviewed, and aortic root dimensions were measured. Patients were divided into 2 groups for comparison: untreated (n = 34) and treated (n = 29). RESULTS: At study entry, the 2 study groups were comparable in terms of age, sex, body surface area (BSA), aortic root measurements, heart rate, and corresponding z scores. Follow-up duration in each group was similar. At last follow-up, heart rates and heart rate z scores were lower in the treated group. Rates of change of aortic root measurements (P = .52) and the corresponding z scores were not statistically different between the 2 group at the study's end. CONCLUSIONS: This study suggests that that beta-blocker therapy does not significantly alter the rate of aortic root dilation in children with Marfan syndrome. Based on these data, the recommendation of lifetime beta-blocker therapy instituted during childhood should be reassessed.