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Our cases suggest that the mixture of PAPVC and pulmonary comorbidities may trigger the development of severe pulmonary arterial hypertension. The initiation of pulmonary arterial hypertension-targeted combination treatment disclosed become a secure and effective technique for patients with PAPVC-associated severe pulmonary arterial hypertension. © The Author(s) 2020.It happens to be usually accepted that severe types of pulmonary art