El Louali F, Villacampa C, Aldebert P, Dragulescu A, Fraisse A
Service de cardiologie pédiatrique, pôle de pédiatrie, hôpital de Timone-Enfants, 264, rue Saint-Pierre, 13385 Marseille cedex 5, France.
Arch Pediatr. 2011 Mar;18(3):331-7. doi: 10.1016/j.arcped.2010.12.015. Epub 2011 Feb 2.
Pulmonary atresia and critical pulmonary stenosis with intact ventricular septum includes a wide spectrum of cardiopathies with great morphological heterogeneity. The pulmonary valve may be completely atretic or may contain a puncture hole if stenosis is present. The obstruction may be membranous and/or muscular. All components of the right ventricle can be affected, even the coronary circulation with ventriculocoronary connections and stenosis or atresia of the pulmonary arteries. Prenatal diagnosis is made when the right ventricle is hypoplastic and hypertrophic. The pulmonary valve is thickened and the pulmonary artery is perfused retrogradely through the ductus arteriosus. Right ventriculocoronary connections may sometimes be seen with fetal echocardiography. Postnatal survival depends on the patency of the ductus arteriosus, requiring prostaglandin E1 infusion. When hypoplastic right ventricle and/or ventricle-dependent coronary circulation exists, biventricular circulation is not possible. In these cases, surgical treatment is palliative. In cases with well-developed right ventricle, transcatheter therapy is usually provided with perforation and balloon dilation of the pulmonary valve. In cases of muscular obstruction of the right ventricle outflow tract, surgery may be considered as first-line therapy. In case of prenatal diagnosis, the medical termination of pregnancy is possible when severe right ventricular hypoplasia exists, precluding biventricular circulation. Postnatally, the prognosis of the patients is highly variable, mainly related to the size of the right cavities and the presence of coronary anomalies.
室间隔完整的肺动脉闭锁及重度肺动脉狭窄包括一系列形态学异质性很大的心脏病。肺动脉瓣可能完全闭锁，若存在狭窄则可能有一个穿孔。梗阻可能是膜性和/或肌性的。右心室的所有组成部分都可能受到影响，甚至包括伴有心室冠状动脉连接以及肺动脉狭窄或闭锁的冠状动脉循环。当右心室发育不良且肥厚时可作出产前诊断。肺动脉瓣增厚，肺动脉通过动脉导管逆行供血。胎儿超声心动图有时可发现右心室冠状动脉连接。出生后的存活取决于动脉导管的通畅情况，需要输注前列腺素E1。当存在右心室发育不良和/或心室依赖型冠状动脉循环时，双心室循环是不可能的。在这些情况下，手术治疗是姑息性的。对于右心室发育良好的病例，通常采用经导管治疗，即对肺动脉瓣进行穿孔和球囊扩张。对于右心室流出道肌性梗阻的病例，手术可被视为一线治疗方法。在产前诊断的情况下，当存在严重的右心室发育不良且无法进行双心室循环时，可考虑医学性终止妊娠。出生后，患者的预后差异很大，主要与右心腔的大小以及冠状动脉异常的存在有关。
