Open AccessNovel diagnostic and therapeutic approaches to pulmonary hypertension due to the unilateral absence of a pulmonary artery
Author(s) -
Nagamatsu Yuki,
Goda Ayumi,
Ito Junnosuke,
Takeuchi Kaori,
Kikuchi Hanako,
Kariyasu Toshiya,
Machida Haruhiko,
Inami Takumi,
Kohno Takashi,
Soejima Kyoko,
Satoh Toru
Publication year - 2021
Publication title -
esc heart failure
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.787
H-Index - 25
ISSN - 2055-5822
DOI - 10.1002/ehf2.13459
Subject(s) - medicine , pulmonary artery , pulmonary hypertension , cardiology , radiology , medical diagnosis , hemodynamics , heart failure , differential diagnosis , pulmonary wedge pressure , pathology
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).