BACKGROUND The Norwood procedure with a modified Blalock–Taussig (MBT) shunt, the first palliative stage for single-ventricle lesions with systemic outflow obstruction, is associated with high mortality. The right ventricle–pulmonary artery (RVPA) shunt may improve coronary flow but requires a ventriculotomy. We compared the two shunts in infants with hypoplastic heart syndrome or related anomalies. METHODS Infants undergoing the Norwood procedure were randomly assigned to the MBT shunt (275 infants) or the RVPA shunt (274 infants) at 15 North American centers. The primary outcome was death or cardiac transplantation 12 months after randomization. Secondary outcomes included unintended cardiovascular interventions and right ventricular size and function at 14 months and transplantation-free survival until the last subject reached 14 months of age. RESULTS Transplantation-free survival 12 months after randomization was higher with the RVPA shunt than with the MBT shunt (74% vs. 64%, P = 0.01). However, the RVPA shunt group had more unintended interventions (P = 0.003) and complications (P = 0.002). Right ventricular size and function at the age of 14 months and the rate of nonfatal serious adverse events at the age of 12 months were similar in the two groups. Data collected over a mean (±SD) follow-up period of 32±11 months showed a nonsignificant difference in transplantation-free survival between the two groups (P = 0.06). On nonproportional-hazards analysis, the size of the treatment effect differed before and after 12 months (P = 0.02). CONCLUSIONS In children undergoing the Norwood procedure, transplantation-free survival at 12 months was better with the RVPA shunt than with the MBT shunt. After 12 months, available data showed no significant difference in transplantation-free survival between the two groups. (ClinicalTrials.gov number, NCT00115934.)

permissão concedida pela Elsevier para reprodução do material em português somente. Abreviações: 2D: bidimensional 3D: tridimensional Área A: área abaixo da curva da onda A da fluxo mitral Onda A: Fluxo mitral correspondente ao momento da contração atrial Onda a': Onda obtida no nível do anel mitral pelo Doppler tecidual durante a contração atrial Onda Ar: Velocidade de fluxo reverso pulmonar ou sistêmico durante a contração atrial VAo: Valva aórtica ASE: Sociedade Americana de Ecocardiografia AV: Atrioventricular SC: Superfície corpórea CC: Cardiopatia congênita Onda D: Componente diastólico do fluxo venoso pulmonar ou sistêmico dP/dt: Primeira derivado de pressão em relação ao tempo Área A: Área abaixo da curva da onda A do fluxo mitral (diástole precoce) Onda E: Fluxo mitral correspondente ao componente diastólico precoce Onda e': Onda obtida pelo Doppler tecidual no nível do anel valvar mitral durante a diástole precoce DDF: Diâmetro diastólico final VDF: Volume diastólico final FE: Fração de ejeção DSF: Diâmetro sistólico final VSF: Volume sistólico final VCI: Veia cava inferior TCIV': Tempo de contração isovolumétrica obtido ao Doppler tecidual TRIV: Tempo de relaxamento isovolumétrico obtido ao Doppler convencional TRIV': Tempo de relaxamento isovolumétrico obtido ao Doppler tecidual L: Comprimento AE: Átrio esquerdo VE: Ventrículo esquerdo RNM: Ressonância nuclear magnética VM: Valva mitral PFR SV : Taxa máxima de enchimento/volume ejetado VP: valva pulmonar AD: Átrio direito VD: Ventrículo direito Onda s': Onda obtida pelo Doppler tecidual no nível do anel valvar mitral durante a sístole Onda S: Componente sistólico do fluxo venoso pulmonar ou sistêmico ▲D: Fração de encurtamento TAPSE: Deslocamento do anel tricúspide durante a sístole VT: Valva tricúspide V: Volume ventricular VTI: Integral da velocidade x tempo

Infantile hemangiomas (IHs) occur in as many as 5% of infants, making them the most common benign tumor of infancy. Most IHs are small, innocuous, self-resolving, and require no treatment. However, because of their size or location, a significant minority of IHs are potentially problematic. These include IHs that may cause permanent scarring and disfigurement (eg, facial IHs), hepatic or airway IHs, and IHs with the potential for functional impairment (eg, periorbital IHs), ulceration (that may cause pain or scarring), and associated underlying abnormalities (eg, intracranial and aortic arch vascular abnormalities accompanying a large facial IH). This clinical practice guideline for the management of IHs emphasizes several key concepts. It defines those IHs that are potentially higher risk and should prompt concern, and emphasizes increased vigilance, consideration of active treatment and, when appropriate, specialty consultation. It discusses the specific growth characteristics of IHs, that is, that the most rapid and significant growth occurs between 1 and 3 months of age and that growth is completed by 5 months of age in most cases. Because many IHs leave behind permanent skin changes, there is a window of opportunity to treat higher-risk IHs and optimize outcomes. Early intervention and/or referral (ideally by 1 month of age) is recommended for infants who have potentially problematic IHs. When systemic treatment is indicated, propranolol is the drug of choice at a dose of 2 to 3 mg/kg per day. Treatment typically is continued for at least 6 months and often is maintained until 12 months of age (occasionally longer). Topical timolol may be used to treat select small, thin, superficial IHs. Surgery and/or laser treatment are most useful for the treatment of residual skin changes after involution and, less commonly, may be considered earlier to treat some IHs.