Which prenatal finding best characterizes pulmonary atresia with intact ventricular septum?

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Multiple Choice

Which prenatal finding best characterizes pulmonary atresia with intact ventricular septum?

Pulmonary atresia with intact ventricular septum is defined by obstruction of the right ventricular outflow tract at the level of the pulmonary valve, with no communication between the ventricles. In this scenario the pulmonary valve is either absent or severely narrowed, and the right ventricle is often small because its inflow and outflow are restricted. The ventricular septum remains intact, so there is no VSD. In fetal imaging this constellation—absent or severely restricted pulmonary valve flow, a very small right ventricle, and intact ventricular septum—best characterizes this condition. The other patterns describe different lesions: a normal valve with a VSD implies a different anatomy; a normal RV and valve is not pathological; a VSD with PDA denotes a VSD present with additional ductal-dependent pulmonary flow, which also does not fit PA/IVS.

Pulmonary atresia with intact ventricular septum is defined by obstruction of the right ventricular outflow tract at the level of the pulmonary valve, with no communication between the ventricles. In this scenario the pulmonary valve is either absent or severely narrowed, and the right ventricle is often small because its inflow and outflow are restricted. The ventricular septum remains intact, so there is no VSD.

In fetal imaging this constellation—absent or severely restricted pulmonary valve flow, a very small right ventricle, and intact ventricular septum—best characterizes this condition. The other patterns describe different lesions: a normal valve with a VSD implies a different anatomy; a normal RV and valve is not pathological; a VSD with PDA denotes a VSD present with additional ductal-dependent pulmonary flow, which also does not fit PA/IVS.

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