Novel diagnostic and therapeutic approaches to pulmonary hypertension due to the unilateral absence of a pulmonary artery

• Main Authors: Yuki Nagamatsu, Ayumi Goda, Junnosuke Ito, Kaori Takeuchi, Hanako Kikuchi, Toshiya Kariyasu, Haruhiko Machida, Takumi Inami, Takashi Kohno, Kyoko Soejima, Toru Satoh
• Format: Article
• Language: English
• Published: Wiley 2021-08-01
• Series: ESC Heart Failure
• Subjects: Unilateral absence of a pulmonary artery; Pulmonary hypertension; 4D computed tomography; Upfront triple combination therapy
• Online Access: https://doi.org/10.1002/ehf2.13459
• ISSN: 2055-5822

Abstract We report the case of a 64‐year‐old female diagnosed with severe pulmonary hypertension due to the unilateral absence of a pulmonary artery. The four‐dimensional computed tomography scan is a useful modality for revealing detailed anatomical findings for differential diagnoses and surgical decision‐making. The patient had severe pulmonary hypertension with a mean pulmonary artery pressure (PAP) of 74 mmHg and was treated with triple upfront combination therapy, leading to significant improvement in pulmonary haemodynamics (to 27 mmHg in mean PAP) and functional capacity (WHO functional class, from III to II; 6‐min walk distance, from 211 to 276 m).