Vlachakis Panayotis K, Nyktari Eva, Apostolos Anastasios, Theofilis Panagiotis, Karakasis Paschalis, Synetos Andreas, Toutouzas Konstantinos, Tsioufis Costas, Drakopoulou Maria
First Cardiology Department, Athens Medical School, Hippokration General Hospital, National and Kapodistrian University of Athens, Athens, Greece.
CMR Unit, Onassis Cardiac Surgery Center, Kallithea, Greece.
JACC Case Rep. 2025 Jun 25;30(16):104249. doi: 10.1016/j.jaccas.2025.104249.
A 21-year-old male with complex congenital heart disease (pulmonary atresia with intact ventricular septum and major aortopulmonary collateral arteries [MAPCAs]) was seen in our outpatient clinic. Diagnosed shortly after birth, the patient underwent Rashkind atrial septostomy and was deemed amenable to surgical correction/partial unifocalization or palliative intervention.
On examination, the patient was cyanotic with no signs of heart failure. Multimodality imaging showed MAPCAs from the aorta to the pulmonary circulation with a variable degree of stenosis suggestive of segmental pulmonary hypertension (PH). The decision of the multidisciplinary meeting was for a conservative approach and close monitoring with the potential of palliative interventions if there is a deterioration in patients' functional status.
Segmental PH presents a complex scenario, necessitating multimodality imaging for therapeutic considerations.
TAKE-HOME MESSAGE: In cyanotic congenital heart disease, the presence of MAPCAs and segmental PH presents a complex scenario, necessitating multimodality imaging.
一名21岁患有复杂先天性心脏病（室间隔完整的肺动脉闭锁及主要体肺侧支动脉[MAPCAs]）的男性患者前来我院门诊就诊。该患者出生后不久即被诊断，接受了拉什金德房间隔造口术，并被认为适合进行手术矫正/部分单灶化或姑息性干预。
经检查，患者面色青紫，无心力衰竭迹象。多模态成像显示存在从主动脉到肺循环的MAPCAs，伴有不同程度的狭窄，提示存在节段性肺动脉高压（PH）。多学科会诊决定采取保守治疗方法并密切监测，若患者功能状态恶化则可能进行姑息性干预。
节段性PH情况复杂，治疗时需要多模态成像。
在青紫型先天性心脏病中，MAPCAs和节段性PH的存在情况复杂，需要多模态成像。
