The latter diagnosis should be made by an expert clinician such as a paediatric cardiologist. Left-sided obstructive defects such as aortic stenosis/atresia and mitral stenosis/atresia may occur together but do not necessarily qualify as a diagnosis of HLHS. Some common genetic syndromes associated with HLHS include Turner syndrome, Noonan syndrome and the common trisomies. Family members may have subtle variants of left-sided heart defects, which may require echocardiograms for diagnosis. HLHS can occur in association with genetic conditions, though in >75% of cases it seems to be an isolated condition. The final, specific diagnosis is readily made by echocardiography. Other cases can be suspected because of absence of pulses. Some cases, especially in the presence of a widely open ductus arteriosus, might be missed in the early newborn period and can become clinically obvious only after the ductus closes, which can happen after discharge from the nursery.Īs with other severe heart disease, newborn screening via pulse oximetry – which is based on the non-invasive detection of low peripheral oxygen saturation – can detect many cases before they become clinically obvious. In HLHS, the norm is early-onset heart failure (cardiogenic shock). Prenatal diagnoses should be confirmed postnatally for example, by echocardiography. HLHS can be suspected and, in expert hands, diagnosed prenatally. Clinically, the heart is unable to sustain the systemic circulation, especially after the ductus closes, resulting in heart failure and cardiogenic shock.Underdeveloped ascending aorta, sometimes with hypoplastic aortic arch and descending aorta.A small, narrowed or atretic mitral valve and/or aortic valve.
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