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Pulmonary Valve Stenosis with Atrial Septal Defect

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Synonyms

Pulmonic stenosis with interatrial communication; Triologie de Fallot

Definition and Characteristics

When pulmonary stenosis (PS) and atrial septal defect (ASD) occur together, the interatrial shunt could be patent foramen ovale, ostium secundum ASD, or ASDs of the ostium primum or sinus venous variety. Obstruction to the right ventricle (RV) outflow is usually caused by stenosis of the pulmonic valve (PV) and less commonly of the pulmorary artery (PA) and its branches. When a true ASD is present, coexisting PS is almost always valvular. In PV stenosis with intact ventricular septum, the coexisting right to left shunt (RTLS) is usually via a patent foramen ovale (PFO) rather than a true ASD. In patients with significant PV stenosis and RTLS through a PFO, clinical features include exertional syncope, chest pain, central cyanosis, erythema of fingers and toes, clubbing of digits, underdevelopment, moon face, a sustained and strong RV impulse, and a systolic pulmonic murmur...

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  • DOI: 10.1007/978-3-540-29676-8_1501
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References

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© 2009 Springer-Verlag GmbH Berlin Heidelberg

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Ma, L.K., Ma, P.T.S., Leung, A.K.C. (2009). Pulmonary Valve Stenosis with Atrial Septal Defect. In: Lang, F. (eds) Encyclopedia of Molecular Mechanisms of Disease. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-29676-8_1501

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