Coarctation of the aorta with hypoplastic aortic arch :Analysis of midterm and longterm outcomes with autologous pulmonary patch aortoplasty / 医学研究生学报

Objective Previously we have reported the early and midterm benifit of autologous pulmonary patch in repairing aortic coarctation of hypoplastic aortic arch.This study aimed to assess its reliability and midterm and longterm outcomes.Methods We retrospectivly analyzed 42 pediatric patients with coarctation of the aorta (CoA) with hypoplastic aortic arch undergoing surgical repair with autologous pulmonary patch from May 2009 to May 2017 in General Hospital of Guangzhou Military Command of PLA.All the patients were allocated into either senior group (＞ 1 years) or junior group (≤1 years) according to the age of operation.The trans-coarctation gradient,pulmonary pressure and aortic Z value change were compared between two groups before and after the repair.Results There were 8 cases had early postoperative complications.However,no death had been reported during the postoperative time and the followed up period ranged from 4 months to 106 months (40.0± 15.5) months).The average pressure gradient of coarctation segment for all the patients was (11.9±6.4) mmHg,including 5 cases more than 25 mmHg.The pressure gradient and mean pulmonary arterial pressure after operation were significantly lower than those before operation (P＜0.05),The postoperative aortic arch Z value was greater than the preoperative value (P＜0.05).Compared with the preoperative period,the Z value of proximal transverse arch increased significantly(-0.64±0.44) vs (1.27±0.66),P＜0.05.Compared with junior group,the senior group had higher preoperative and postoperative pulmonary artery pressure (P＜0.05),and longer CPB time,aortic block time,ventilation time,ICU time and hospital stay time (P＜0.05).However,patients in the junior group had a higher pressure gradient through the aorta arch(P＜0.05) and a smaller Z value transverse arch aortic proximal and isthmus(P＜0.05) during the long-term period.The time of selective cerebral perfusion had no statistical difference between the two groups (P＞ 0.05).Conclusion Early surgery for coarctation of aorta with hypoplastic aortic arch,autologous pulmonary patch aortoplasty is a relatively ideal option with better midterm and longterm outcomes.

Strategy of repairing coarctation of aorta with hypoplastic aortic arch using cardiopulmonary bypass in neonates / 中华胸心血管外科杂志

Objective To analysis the the strategy of repairing coarctation of aorta with hypoplastic aortic arch in neo-nate.Methods Collected consecutive 24 neonates suffered coarctation of aorta with hypoplastic aortic arch form January 2015 to March 2017,12 patients were underwent aortic arch reconstruction with extremely extended end-to-end anastomosis under cardiopulmonary bypass(CPB) through the media sternotomy approach(CPB group), while another 12 cases were under coarc-tation repair using end-to-end anastomosis or end-to-side anastomosis through left posterolateral thoracotomy approach ( control group).The postoperative blood pressure,echocardiography and cardiac CT scan were used to evaluate the result of the aortic arch rconstruction.The pressuregradientof the upper limb and lower limb,flow velocity of the anastomoses, aortic arch morphol-ogy,rate of the residual abstruction were compared between the two groups.Results Mechanical ventilation time[ CPB group (17 ±27)h vs.control group(44 ±52)h, P＜0.05], ength of stay in ICU[CPB group(3.75 ±1.36)days vs.control group (6.54 ±5.08)days, P＜0.05], all patients were followed up for 1－12 months,the aortic residual pressure[CPB group(14 ± 10)mmHg (1 mmHg＝0.133 kPa) vs.control group(26 ±17)mmHg,P ＜0.05) ], flow velocity of the anastomoses[CPB group(2.32 ±0.78)m/s, control group(1.55 ±0.99)m/s, P ＜0.05 ], pressuregradientof the upper limb and lower limb [CPB group (6.67 ±3.49)mmHg, control group(7.41 ±4.29)mmHg, P＞0.05].There was no died in two gruops.Con-clusion Anastomosis with end to end techinique under cardiopulmonary bypass may achieve better effect in neonates with co-arctation of aorta with hypoplastic aortic arch,it has better duplcity, operability and early curative effect,but the long-term effect need the multicenter and large sample-volume study to explore.

Síndrome de Turner en una adolescente / Turner syndrome in an adolescent

Se presenta el caso clínico de una adolescente ecuatoriana de 17 años de edad, blanca, quien nació producto de un embarazo normal y parto eutócico. Posterior al nacimiento se le realizó estudio genético que mostró la presencia de un cariotipo X0, por lo cual se le diagnosticó síndrome de Turner. A los 3 años fue operada de hipoplasia del arco aórtico con una evolución favorable. En la actualidad la paciente tiene baja talla con orejas de baja implantación, así como implantación alta del cabello en la nuca, tórax plano en forma de escudo, escaso bello pubiano y ausencia de menstruación

The case report of a 17 years Ecuadorian white adolescent is presented who was born from a normal pregnancy and eutocic delivery. After birth, a genetic study that showed the presence of a X0 Karyotype was carried out, reason why she was diagnosed Turner syndrome. She was operated of hypoplastic aortic arch when she was 3 years with a favorable clinical course. At the present time the patient has short height with ears of low implantation, as well as high implantation of the hair in the back of the neck, flat thorax in shield form, scarce pubic hair and amenorrhea

Diagnosis of aortic coarctation combined with hypoplastic aortic arch by echocardiography / 中华超声影像学杂志

Objective To study the diagnosis accuracy and features of echocardiography on aortic coarctation combined with hypoplastic aortic arch. Methods The echocardiographic characteristics of seven patients who were diagnosed as aortic coarctation combined with hypoplastic aortic arch were analyzed and compared with results of cardiac catheterization, CT scan and operation. The diagnostic features of echocardiography were summarized. Results Besides the echocardiographic characteristics of aortic coarctation,other specific echocardiographic characteristics of hypoplastic aortic arch were:distinct stenoses of transverse and decsending part of aortic arch were visualized in supra-sternal long-axis view; the range of hypoplastic part started from innominate artery beginning or left common carotid artery beginning; the ratio of diameter of hypoplastic aortic arch over that of descending aorta at diaphragm was less than 0. 5; the blood flow speed detected by Doppler at the site of aortic coarctation was in the normal range. The echocardiographic characteristics of the aortic coarctation combined with hypoplastic aortic arch were correspondent to the results of cardiac catheterization, CT scan and operation results. Conclusions There is high accuracy of echocardiography in diagnosis of aortic coarctation combined with hypoplastic aortic arch. Hypoplastic aortic arch should be paid more attention in diagnosis of aortic coarctation.