RESUMEN
Aortico-left ventricular tunnel and left ventricular non-compaction are rare congenital cardiac anomalies with varied clinical presentations and sparsely described co-existence in the medical literature. Owing to the limited information about these diagnoses in tandem, we present four cases of aortico-left ventricular tunnel and left ventricular non-compaction from our institution and discuss the clinical presentation, management, and follow-up.
Asunto(s)
Anomalías Múltiples , Aorta Torácica/anomalías , Cardiopatías Congénitas/diagnóstico por imagen , Ventrículos Cardíacos/anomalías , Malformaciones Vasculares/diagnóstico por imagen , Adolescente , Adulto , Aorta Torácica/diagnóstico por imagen , Ecocardiografía , Femenino , Ventrículos Cardíacos/diagnóstico por imagen , Humanos , Recién Nacido , Masculino , Embarazo , Ultrasonografía PrenatalRESUMEN
An infant with coarctation of the aorta and Williams syndrome was noted to have petechiae in cardiology clinic prior to planned surgical intervention. Workup revealed acquired von Willebrand syndrome secondary to the high shear force generated by the aortic coarctation. He was treated with intra- and postoperative Humate P; there were no postoperative bleeding complications. His acquired von Willebrand syndrome resolved postoperatively.
Asunto(s)
Anomalías Múltiples , Coartación Aórtica/diagnóstico , Síndrome de Williams/diagnóstico , Enfermedades de von Willebrand/diagnóstico , Factor de von Willebrand/metabolismo , Coartación Aórtica/sangre , Coartación Aórtica/cirugía , Ecocardiografía , Humanos , Recién Nacido , Masculino , Procedimientos Quirúrgicos Vasculares/métodos , Síndrome de Williams/sangre , Síndrome de Williams/cirugía , Enfermedades de von Willebrand/cirugíaRESUMEN
In repaired tetralogy of Fallot (rTOF), pulmonary insufficiency results in varying degrees of right ventricle (RV) dilation. A subset of patients is diagnosed at initial cardiac magnetic resonance imaging (CMR) with a massively dilated RV, far beyond pulmonary valve replacement (PVR) criteria, which is unlikely to return to normal size after PVR. This study aimed to identify risk factors for massive RV dilation at initial CMR. This nested case-control study included all patients at our institution with rTOF and massive RV dilation (indexed RV end-diastolic volume [RVEDVi] ≥200 ml/m2) on initial CMR. Patients were matched by age at first CMR, gender, and type of repair with rTOF controls with RVEDVi<200 ml/m2. In 39 cases (median RVEDVi 227 ml/m2, interquartile range [IQR] 213 to 250) and 73 controls (median RVEDVi 155 ml/m2, IQR 130 to 169), repair at >6 months of age, longer QRS duration, and non-Caucasian race were significantly associated with massive RV dilation on univariate analysis. In multivariate analysis, repair at >6 months of age (adjusted odds ratio [AOR] 2.90, 95% confidence interval [CI] 1.12 to 7.55, pâ¯=â¯0.03), longer QRS duration (AORâ¯=â¯1.03, 95% CI 1.01 to 1.05, pâ¯=â¯0.005), and non-Caucasian race (AORâ¯=â¯7.84, 95% CI 1.76 to 34.8, pâ¯=â¯0.01) remained independently associated with massive RV dilation. Era of repair, history of systemic to pulmonary shunt palliation, genetic anomaly, and additional cardiac lesions did not differ between groups. In conclusion, these risk factors identify a subset of patients who may benefit from earlier CMR evaluation to avoid massive irreversible RV dilation.