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Pulmonary Arterial Hypertension in a Patient with Connective Tissue Disease and ALCAPA Syndrome [Respir Case Rep]
Respir Case Rep. 2021; 10(2): 92-94 | DOI: 10.5505/respircase.2021.45722

Pulmonary Arterial Hypertension in a Patient with Connective Tissue Disease and ALCAPA Syndrome

Wang Kin Wong1, Wan-Jing Ho2, Jaw-Ji Chu3, Shue-Fen Luo4
1School of Medicine, Chang Gung University, Chang Gung Memorial Hospital, Tao-Yuan, Taiwan
2Department of Cardiology, Chang Gung Memorial Hospital, Chang Gung University, Tao-Yuan, Taiwan
3Chang Gung Üniversitesi, Chang Gung Memorial Hastanesi, Kardiotorasik ve Vasküler Cerrahi Bölümü, Tao-Yuan, Tayvan
4Department of Rheumatology, Allergy and Immunolo-gy, Chang Gung Memorial Hospital, Chang Gung Uni-versity, Tao-Yuan, Taiwan

A 43-year-old female with mixed connective tissue disease was incidentally discovered to be an adult survivor of an anomalous left coronary artery originating from the pulmonary artery during a work-up for her pulmonary hypertension. We describe here the management strategy of severe pulmonary hypertension prior to and after the surgical correction of the congenital anomaly.

Keywords: Pulmonary hypertension, pulmonary arterial hypertension, anomalous left coronary ar-tery originating from the pulmonary artery, ALCAPA, connective tissue disease.

Konnektif Doku Hastalığı ve ALCAPA Sendromu olan Bir Hastada Pulmoner Arteriyal Hipertansiyon

Wang Kin Wong1, Wan-Jing Ho2, Jaw-Ji Chu3, Shue-Fen Luo4
1Chang Gung Üniversitesi Tıp Fakültesi, Chang Gung Memorial Hastanesi, Tao-Yuan, Tayvan
2Chang Gung Üniversitesi, Chang Gung Memorial Hastanesi, Kardiyoloji Bölümü, Tao-Yuan, Tayvan
3Department of Cardiothoracic and Vascular Surgery, Chang Gung Memorial Hospital, Chang Gung Universi-ty, Tao-Yuan, Taiwan
4Chang Gung Üniversitesi, Chang Gung Memorial Hastanesi, Romatoloji, Allerji ve İmmünoloji Bölümü, Tao-Yuan, Tayvan

Mikst konnektif doku hastalığı olan 43 yaşındaki kadında, pulmoner arteriyel hipertansiyon araştırmaları sırasında, yaşayan bir yetişkinde, pulmoner arterden orijin alan sol koroner arter anomalisi saptandı. Konjenital anomalinin cerrahi tedavisi öncesi ve sonrası, ciddi pulmoner hipertansiyon yönetim stratejisini açıklıyoruz.

Anahtar Kelimeler: Pulmoner arteriyal hipertansiyon, pulmoner hipertansiyon, pulmoner arterden kaynak-lanan sol koroner arter anomalisi, ALCAPA, konnektif doku hastalığı.







Wang Kin Wong, Wan-Jing Ho, Jaw-Ji Chu, Shue-Fen Luo. Pulmonary Arterial Hypertension in a Patient with Connective Tissue Disease and ALCAPA Syndrome. Respir Case Rep. 2021; 10(2): 92-94

Corresponding Author: Wan-Jing Ho, Taiwan
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