Pulmonary hypertension (PH) encompasses several heterogeneous groups of multiple diseases characterized by abnormal pulmonary arterial blood pressure elevation. Unrepaired atrial septal defect (ASD) may be associated with pulmonary arterial hypertension (PAH), indicating pulmonary vascular remodeling. Furthermore, unrepaired ASD could also be associated with other conditions, such as left heart disease or thromboembolism, contributing to the disease progression. We present a case of a 61-years-old woman with complex PH comprising several etiologies, which are PAH due to unrepaired Secundum ASD, mitral regurgitation caused by mitral valve prolapse as a group 2 PH, pulmonary embolism (PE) which progress to chronic thromboembolism PH (CTEPH) and post-acute sequelae of SARS Cov-2. We highlighted the importance of diagnostic investigation in PH, which is crucial to avoid misdiagnosis and inappropriate treatment that could be detrimental for the patient.
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