Karmali Rehan, Mazumder Samia, Berglund Felix, Fuchs Margaret M
Department of Internal Medicine, Cleveland Clinic, 9500 Euclid Ave, NA10, Cleveland, OH 44195, USA.
Department of Cardiovascular Medicine, Heart, Vascular, and Thoracic Institute, Cleveland Clinic, 9500 Euclid Ave, Cleveland, OH 44195, USA.
Eur Heart J Case Rep. 2023 Apr 3;7(4):ytad154. doi: 10.1093/ehjcr/ytad154. eCollection 2023 Apr.
d-Transposition of the great arteries (d-TGA) is a congenital cardiac defect that is typically fatal. Those patients who survive without surgical repair and who are rare in number, need adequate intracardiac shunting and will suffer from chronic cyanosis. Here, we present a rare case of an adult with cyanotic congenital heart disease (CHD) who developed infective endocarditis (IE) and also our approach to the medical decision-making process in this uncommonly encountered dilemma.
A 52-year-old female with unrepaired d-TGA with tricuspid atresia, hypoplastic right ventricle, unrestricted atrial septal defect, ventricular septal defect, and sub-valvular as well as valvular pulmonic stenosis with a hypoplastic, bicuspid pulmonary valve presented with abdominal pain and hypoxia and was found to have an acute renal infarct. Transthoracic echocardiogram (TTE) revealed a large mobile mass on the mitral valve. Blood cultures grew and she was diagnosed with streptococcal native mitral valve IE complicated by a renal embolus. Her large left-sided vegetation and embolic phenomenon favoured surgery. However, a right heart catheterization showed intracardiac pressures, likely a result of multi-level obstruction relating to sub-valvular and valvular pulmonary stenosis protecting the pulmonary vasculature from over-circulation and pulmonary hypertension. Cardiac surgery posed a significant risk of destabilizing her delicately balanced haemodynamics. Hence, she was treated with ceftriaxone for 4 weeks. A repeat TTE 8 weeks later showed a resolution of the vegetation.
A decision for surgery vs. medical treatment for IE in adult patients with compensated CHD should be made following a multi-disciplinary assessment of each patient's unique cardiac haemodynamics and after shared decision-making with the patient.
大动脉 d 转位（d-TGA）是一种典型的致命性先天性心脏缺陷。那些未经手术修复而存活下来的患者数量稀少，需要足够的心内分流，且会遭受慢性紫绀。在此，我们报告一例罕见的成年紫绀型先天性心脏病（CHD）患者发生感染性心内膜炎（IE）的病例，以及我们在这种罕见困境中进行医疗决策的方法。
一名 52 岁女性，患有未经修复的 d-TGA，伴有三尖瓣闭锁、右心室发育不全、无限制房间隔缺损、室间隔缺损、瓣下及瓣膜性肺动脉狭窄，以及发育不全的二叶式肺动脉瓣。患者因腹痛和缺氧就诊，发现有急性肾梗死。经胸超声心动图（TTE）显示二尖瓣上有一个大的活动团块。血培养结果阳性，她被诊断为链球菌性原发性二尖瓣 IE 并发肾栓塞。她左侧的大赘生物和栓塞现象支持手术治疗。然而，右心导管检查显示心内压力升高，这可能是由于瓣下和瓣膜性肺动脉狭窄导致的多级梗阻，从而保护肺血管免受过度循环和肺动脉高压的影响。心脏手术有显著风险使她微妙平衡的血流动力学失稳。因此，她接受了 4 周的头孢曲松治疗。8 周后复查 TTE 显示赘生物消退。
对于患有代偿性 CHD 的成年 IE 患者，手术与药物治疗的决策应在对每位患者独特的心脏血流动力学进行多学科评估后，并与患者共同决策后做出。
