Objective To summarize the clinical characteristics and the effect of pulmonary endarterectomy(PEA)in CTEPH patients with unilateral main pulmonary artery occlusion.Methods Of 160 CTEPH patients operated between Janua...Objective To summarize the clinical characteristics and the effect of pulmonary endarterectomy(PEA)in CTEPH patients with unilateral main pulmonary artery occlusion.Methods Of 160 CTEPH patients operated between January2004 and March 2018 at our center,13(8.1%)had complete main pulmonary artery occlusion.Patients were included if the ventilation/perfusion(V/Q)scan revealed nonperfusion of an entire lung and the pathological examination showed chronic thromboembolic.展开更多
INTRODUCTION Unilateral Absence of Pulmonary Artery(UAPA)is a rare complicated congenital heart disease firstly described by Frentzel in 1868.An absent pulmonary artery is caused by the involution of the proximal sixt...INTRODUCTION Unilateral Absence of Pulmonary Artery(UAPA)is a rare complicated congenital heart disease firstly described by Frentzel in 1868.An absent pulmonary artery is caused by the involution of the proximal sixth aortic arch and persistence of the connection of the intrapulmonary pulmonary artery to the distal sixth aortic arch in embryonic development period.Patent ductus arteriosus which connects the pulmonary arteries with systemic circulation artery might get atresia gradually after birth with lack of blood flow for this side of pulmonary arteries and pulmonary hypertension in the other side.There are no evidences showing the development of the pulmonary vessels especially in the position of the hilus of the lung and the distribution of the pulmonary arteries on the regular examination,such as echocardiogram and CT scan.Our team has taken enhanced CT scan and three-dimensional reconstruction to confirm the diagnosis and to assess the surgical indications.We adopted utwosegment”technique to reconstruct the absent pulmonary artery for a 3-month-old infant followed by significant alleviation of pulmonary hypertension and excellent rehabilitation.展开更多
目的分析中国单侧肺动脉缺如（unilateral absence of a pulmonary artery,UAPA）患者的临床特点、诊断方法和治疗策略。方法检索清华同方和万方这两个汉语数据库,查得1988~2009年发表的关于UAPA的中文文章52篇,从临床表现、各种检查、...目的分析中国单侧肺动脉缺如（unilateral absence of a pulmonary artery,UAPA）患者的临床特点、诊断方法和治疗策略。方法检索清华同方和万方这两个汉语数据库,查得1988~2009年发表的关于UAPA的中文文章52篇,从临床表现、各种检查、诊断、误诊、治疗和随访等方面分析这些文章。我们报道1例UAPA患者。结果 52篇中文文献报道234例UAPU患者,加上我们报道的1例共235例,其中单纯性UAPA患者29例;合并其他心血管畸形的UAPA患者201例;5例患者没有注明是否合并其他心血管畸形。单纯性UAPA患者年龄3~61岁,平均（29.2±14.3）岁,前3个主要症状：反复呼吸道感染史（48.3%）、呼吸困难（41.4%）和咯血（34.5%）。确诊UAPA的主要方法是右心室和（或）肺动脉造影,其次还有增强电子束CT、多排CT肺动脉造影、超声心动图（UCG）、MRI和核素肺通气-灌注扫描。手术治疗者较少,主要针对咯血。合并其他心血管畸形的UAPA患者年龄2~30岁,平均（9.4±8.2）岁,主要合并法洛四联征143例（71.1%）。前3个主要症状：呼吸困难、活动受限和蹲踞。确诊方法同单纯性UAPA患者。手术主要针对合并的心血管疾病。结论中文文献报道的UAPA有一定的特点。患者的临床表现缺乏特异性,但如果出现胸部畸形、患侧呼吸音低、心脏杂音和肺动脉高压,应考虑UAPA。用右心室和（或）肺动脉造影等方法确诊。UAPA患者容易误诊,特别是单纯性UAPA患者。在我国,目前手术治疗UAPA主要针对合并的其他心血管病畸形或咯血。展开更多
文摘Objective To summarize the clinical characteristics and the effect of pulmonary endarterectomy(PEA)in CTEPH patients with unilateral main pulmonary artery occlusion.Methods Of 160 CTEPH patients operated between January2004 and March 2018 at our center,13(8.1%)had complete main pulmonary artery occlusion.Patients were included if the ventilation/perfusion(V/Q)scan revealed nonperfusion of an entire lung and the pathological examination showed chronic thromboembolic.
基金supported by National Key Research and Development Plan(No.2018YFC1002600)National Natural Science Foundation of China(No.U1401255)Guangdong Provincial Key Laboratory of South China Structural Heart Disease(No.2012A061400008)
文摘INTRODUCTION Unilateral Absence of Pulmonary Artery(UAPA)is a rare complicated congenital heart disease firstly described by Frentzel in 1868.An absent pulmonary artery is caused by the involution of the proximal sixth aortic arch and persistence of the connection of the intrapulmonary pulmonary artery to the distal sixth aortic arch in embryonic development period.Patent ductus arteriosus which connects the pulmonary arteries with systemic circulation artery might get atresia gradually after birth with lack of blood flow for this side of pulmonary arteries and pulmonary hypertension in the other side.There are no evidences showing the development of the pulmonary vessels especially in the position of the hilus of the lung and the distribution of the pulmonary arteries on the regular examination,such as echocardiogram and CT scan.Our team has taken enhanced CT scan and three-dimensional reconstruction to confirm the diagnosis and to assess the surgical indications.We adopted utwosegment”technique to reconstruct the absent pulmonary artery for a 3-month-old infant followed by significant alleviation of pulmonary hypertension and excellent rehabilitation.
文摘目的分析中国单侧肺动脉缺如（unilateral absence of a pulmonary artery,UAPA）患者的临床特点、诊断方法和治疗策略。方法检索清华同方和万方这两个汉语数据库,查得1988~2009年发表的关于UAPA的中文文章52篇,从临床表现、各种检查、诊断、误诊、治疗和随访等方面分析这些文章。我们报道1例UAPA患者。结果 52篇中文文献报道234例UAPU患者,加上我们报道的1例共235例,其中单纯性UAPA患者29例;合并其他心血管畸形的UAPA患者201例;5例患者没有注明是否合并其他心血管畸形。单纯性UAPA患者年龄3~61岁,平均（29.2±14.3）岁,前3个主要症状：反复呼吸道感染史（48.3%）、呼吸困难（41.4%）和咯血（34.5%）。确诊UAPA的主要方法是右心室和（或）肺动脉造影,其次还有增强电子束CT、多排CT肺动脉造影、超声心动图（UCG）、MRI和核素肺通气-灌注扫描。手术治疗者较少,主要针对咯血。合并其他心血管畸形的UAPA患者年龄2~30岁,平均（9.4±8.2）岁,主要合并法洛四联征143例（71.1%）。前3个主要症状：呼吸困难、活动受限和蹲踞。确诊方法同单纯性UAPA患者。手术主要针对合并的心血管疾病。结论中文文献报道的UAPA有一定的特点。患者的临床表现缺乏特异性,但如果出现胸部畸形、患侧呼吸音低、心脏杂音和肺动脉高压,应考虑UAPA。用右心室和（或）肺动脉造影等方法确诊。UAPA患者容易误诊,特别是单纯性UAPA患者。在我国,目前手术治疗UAPA主要针对合并的其他心血管病畸形或咯血。
