Colín-Ortiz José L, López-Andrade Cecilia E
Departamento de Cardiología Pediátrica, Instituto Nacional de Pediatría, Ciudad de México, México.
Arch Cardiol Mex. 2024 Nov 21;95(2):143-53. doi: 10.24875/ACM.24000057.
Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital heart disease characterized by a wide morphological spectrum that can be associated with abnormalities in the coronary circulation such as sinusoids, fistulas, stenosis or atresia. Some patients do not present ventriculo-coronary fistulas or intramyocardial sinusoids, other patients do present ventriculo-coronary connections, but only some of the latter will have right ventricle-dependent coronary circulation (RVDCC); timely establishment of the diagnosis of RVDCC is essential, since the prognosis of these patients is generally fatal. There are reports of patients with this type of coronary circulation (RVDCC) who were undergone to univentricular physiology, but this treatment option remains controversial, so the purpose of this manuscript is to share three cases with PA-IVS and RVDCC, their outcome and the reflections they provide us.
We conducted a cross-sectional, descriptive, observational study of patients with PA-IVS and RVDCC without atresia of the coronary ostium during the study period.
Three patients were identified, the clinical and angiographic characteristics and their evolution are described, and a new technique or maneuver for transient percutaneous decompress of the right ventricle is described theoretically for those cases where there is doubt about RVDCC during the angiographic study.
An accurate diagnosis regarding the existence of RVDCC is vital. In some cases, the interpretation of angiography for the diagnosis of RVDCC can be difficult to pinpoint and in many cases, it can be operator dependent, however we consider that is necessary to have a technique or maneuver that can objectively and without doubts determine RVDCC in those cases where angiography is not totally conclusive and thus be able to offer the best therapeutic option.
室间隔完整的肺动脉闭锁（PA-IVS）是一种罕见的先天性心脏病，其形态学范围广泛，可伴有冠状动脉循环异常，如窦状隙、瘘管、狭窄或闭锁。一些患者不存在心室-冠状动脉瘘或心肌内窦状隙，其他患者存在心室-冠状动脉连接，但只有部分后者会出现右心室依赖型冠状动脉循环（RVDCC）；及时确立RVDCC的诊断至关重要，因为这些患者的预后通常是致命的。有报道称患有这种冠状动脉循环类型（RVDCC）的患者接受了单心室生理治疗，但这种治疗选择仍存在争议，因此本手稿的目的是分享三例PA-IVS和RVDCC患者、他们的结局以及带给我们的思考。
我们对研究期间冠状动脉口无闭锁的PA-IVS和RVDCC患者进行了横断面、描述性、观察性研究。
确定了三名患者，描述了他们的临床和血管造影特征及其病情演变，并从理论上描述了一种用于在血管造影研究中对RVDCC存在疑问的病例进行右心室临时经皮减压的新技术或操作。
关于RVDCC存在与否的准确诊断至关重要。在某些情况下，用于诊断RVDCC的血管造影解释可能难以精确确定，而且在很多情况下可能依赖于操作者，然而我们认为有必要拥有一种技术或操作，能够在血管造影不完全确定的情况下客观且毫无疑问地确定RVDCC，从而能够提供最佳治疗选择。
