Peripheral pulmonary stenosis

Orpha code: 99084OMIM code:

Definition

Peripheral pulmonary stenosis is a rare congenital anomaly of the great arteries that may occur at single or multiple sites, in isolation or in association with other congenital heart defects (valvular pulmonary stenosis, atrial, or ventricular septal defects or tetralogy of Fallot) and genetic syndromes (Williams, Alagile syndrome). Clinical presentation is variable and includes heart murmurs, dyspnea, syncope, chest pain and pulmonary hypertension-associated symptoms.

Disease data
Classification

Morphological anomaly

Synonyms
Branch pulmonary artery stenosis
Pulmonary branch stenosis
Branch pulmonary artery stenosis
Pulmonary branch stenosis
ORPHA code
99084
OMIM code
-
ICD10 code
Q25.6
ICD11 code
-

No additional description.

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