Respir Case Rep. 2021; 10(2): 92-94 | DOI: 10.5505/respircase.2021.45722

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

Wang Kin Wong¹, Wan-Jing Ho², Jaw-Ji Chu³, Shue-Fen Luo^4
1Chang Gung Üniversitesi Týp 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 Ýmmünoloji Bölümü, Tao-Yuan, Tayvan

Mikst konnektif doku hastalýðý olan 43 yaþýndaki kadýnda, pulmoner arteriyel hipertansiyon araþtýrmalarý sýrasýnda, yaþayan bir yetiþkinde, pulmoner arterden orijin alan sol koroner arter anomalisi saptandý. Konjenital anomalinin cerrahi tedavisi öncesi ve sonrasý, ciddi pulmoner hipertansiyon yönetim stratejisini açýklýyoruz.

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

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

Wang Kin Wong¹, Wan-Jing Ho², Jaw-Ji Chu³, Shue-Fen Luo^4
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.

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