Classic form of hypoplastic left heart syndrome diagnosed post-natally: an autopsy report

Abstract

Hypoplastic left heart syndrome (HLHS) is a congenital heart disease, which, despite the current improved knowledge about its management and surgical treatment, is still associated with high mortality, especially in the early neonatal period and before the second stage of reconstruction surgery. The low rate of prenatal diagnosis and delayed diagnostic suspicion results in unsuccessful therapeutic intervention, even though the real impact of early diagnosis and intervention on mortality and quality of life of patients is still uncertain. Fortunately, this syndrome of challenging treatment is not that frequent. It involves a spectrum of obstructions to the blood flow within the left heart and is characterized by an inappropriate size of the left ventricle associated with a wide variety of valvular dysfunctions. Treatment ranges from heart transplantation to palliative surgical procedures. The authors describe a case of a newborn with HLHS, whose diagnosis was made after birth because of early respiratory failure. Despite the use of prostaglandin the newborn died. An autopsy was performed and the anatomical findings were described.

Hypoplastic Left Heart Syndrome: A Case Report

“…An autopsy case published in Sao Paulo, Brazil highlighted that HLHS is a lethal congenital heart disease and despite the availability of improved management and surgical treatment in developed countries, it is still associated with high mortality, especially in the early neonatal period and before the second stage of reconstruction surgery. 7 The HLHS, which is characterized anatomically by an insufficiently sized left ventricle to support the systemic circulation and varying degrees of valvular obstructions, involves a spectrum of blood flow obstructions inside the left heart. The main problems mentioned include hypoplasia of the aortic arch and ascending aorta, as well as atresia or stenosis of the mitral and aortic valves.…”

“…An autopsy case published in Sao Paulo, Brazil highlighted that HLHS is a lethal congenital heart disease and despite the availability of improved management and surgical treatment in developed countries, it is still associated with high mortality, especially in the early neonatal period and before the second stage of reconstruction surgery. 7 …”

A fatal combination of duodenal atresia with preduodenal portal vein, malrotation, and hypoplastic left heart syndrome: A case report

“…Without treatment this syndrome is fatal in 100% of cases, with 95% of patients dying during the first month of life. [9] Medical treatment is directed at maintaining ductal patency and balancing systemic and pulmonary blood flow; and possible manoeuvres include deliberate hypoventilation, low inspired oxygen concentration and additional carbon dioxide in an attempt to increase pulmonary vascular resistance (PVR) and limit pulmonary flow. PGE1 infusion may temporarily improve HLHS by reopening the ductus arteriosus, and it was attempted in our case too.…”