Background:Intact-atrial-septum (IAS) and left-ventriculocoronary-arterial-fistula (LVCAF) are the significant risk factors in hypoplastic-left-heart-syndrome (HLHS) and the prognosis of their association is considered grim. Although there has been no survival report of patient with this association, prenatal diagnosis and postnatal aggressive approach may have potential to save patient.
Method and Results: We report a case of prenatally diagnosed HLHS with IAS and LVCAF who were treated aggressively with surgical atrial septostomy and bilateral pulmonary-arterial-banding immediately after birth followed by planned extra-corporeal-membrane-oxygenation support. Echocardiogram clearly showed the change of flow direction of left coronary artery before and after atrial septostomy (from retrograde to antegrade). Although the patient died on 5th postoperative day due to sepsis, hemodynamic markers were stable until the deterioration of sepsis. Postmortem histology revealed no myocardial ischemia nor significant change of pulmonary arteriolar lumen, suggesting prenatal planning and aggressive approach may lead to a survival of this ultimate association.
Conclusion: Prenatal planning and postnatal aggressive approach may lead to the survival of patient with this association in future.