Hypoplastic left heart syndrome (HLHS) with intact or very restrictive atrial septum is a highly lethal combination. We review our 13-year institutional experience treating this high-risk subgroup of patients with emergent catheter therapy. Infants with HLHS requiring catheter septostomy within the first 2 days of life were compared with a matched control group with adequate interatrial communication. Preoperative, early postoperative, and medium-term survival were evaluated. Earlier experience was compared with recent results to assess the effect of changes in catheterization and surgical and intensive care unit management strategies over the study period. From 1990 to 2002, 33 newborns with HLHS (11% of newborns with HLHS managed during this period) underwent urgent/semiurgent catheterization to create or enlarge an interatrial communication before surgical palliation. Preoperative and early postoperative mortality were high (48%) compared with control HLHS patients, regardless of prenatal diagnosis and despite successful catheter-based atrial septostomy with clinical stabilization. Mortality trended down during the later part of the study period. Those who survived the neonatal period had late survival, pulmonary artery pres...Continue Reading
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Implantation of stents to ensure an adequate interatrial communication in patients with hypoplastic left heart syndrome
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Preoperative pulmonary hemodynamics and assessment of operability: is there a pulmonary vascular resistance that precludes cardiac operation?
Vasoreactive response to maternal hyperoxygenation in the fetus with hypoplastic left heart syndrome
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The use of reconstructive surgery to improve quality of life and survival in prenatal hypoplastic left heart syndrome
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Technical challenges of atrial septal stent placement in fetuses with hypoplastic left heart syndrome and intact atrial septum
Prenatal diagnosis of CHDs: a simple ultrasound prediction model to estimate the probability of the need for neonatal cardiac invasive therapy
Left atrial decompression through unidirectional left-to-right interatrial shunt for the treatment of left heart failure: first-in-man experience with the V-Wave device
Hypoplastic left heart syndrome and the nutmeg lung pattern in utero: a cause and effect relationship or prognostic indicator?
Risk factors for interstage death after stage 1 reconstruction of hypoplastic left heart syndrome and variants
Catheter-based decompression of the left atrium in patients with hypoplastic left heart syndrome and restrictive atrial septum is safe and effective
Fetal transesophageal echocardiography: clinical introduction as a monitoring tool during cardiac intervention in a human fetus
Therapeutic ultrasound to noninvasively create intracardiac communications in an intact animal model
Prenatal diagnosis of atrial restriction in hypoplastic left heart syndrome is associated with decreased 2-year survival
Balloon atrial septostomy by a right internal jugular venous approach in a newborn with hypoplastic left heart syndrome with a restrictive atrial septum
Early outcomes from a new regional programme for the surgical management of hypoplastic left heart syndrome
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