主动脉缩窄合并心脏复杂畸形1例分析

患儿，女，4岁，以“发现心脏杂音4年”入院。活动无明显受限，易感冒，口唇无紫绀。血压：左上肢为110mmHg／65mmHg，左下肢130mmHg／75mmHg。心前区隆起，可触及震颤，胸骨左缘第2肋间可闻及连续性机器轰鸣样杂音，胸骨左缘3、4肋间可闻及收缩期3／6级喷射样杂音，P2〉A2。彩超示：①室间隔膜周部10mm缺损；②房间隔中央6mm缺损；③降主动脉与左肺动脉间可见6mm×8mm管状通道；     

A case of simple coarctation]

We report a successful surgical repair of the simple coarctation of a 80-day-old girl by extended end-to-end aortic arch reconstruction. She was admitted to our hospital at the age of 4 days because of poor pulsation of femoral arteries. The systolic blood pressure gradient between the arm and the leg was 30 mmHg. Echocardiography on admission revealed a simple coarctation and patent foramen ovale, with the mildly impaired left ventricular contraction (left ventricular fractional shortening was 23%). Although aortography demonstrated an isolated interrupted segment at the aortic isthmus with collaterals (type A classification of Celoria-Patton), the tubular connection between the distal arch and the descending aorta, of which intralumen was obstructed with abundant ductal tissues, was found at operation. The obstruction of the lumen of aortic isthmus in our case, which was originally patent, might be caused by ductal closure and present as a simple coarctation.     

Balloon dilatation of aortic coarctation]

Endovascular balloon dilatation of coarctation of the aorta is a manipulation for radical correction of the anomaly with an evident hemodynamic effect in patients with the isolated form, in combination of coarctation of the aorta with aortic stenosis, and in atypical forms of the anomaly. Up to 90% of good immediate results were produced among these patients. Balloon dilatation may also be conducted in recoarctation after the traditional surgical management. Being devoid of some of the negative factors of the accepted methods of treatment, endovascular angioplasty is a sparing and a relatively safe method. Paradoxical hypertension does not develop as a rule. Rare complications in the zone of the approach were encountered. Balloon dilatation may be combined with endovascular correction of other anomalies, and may be repeated many times. In recoarctation and atypical forms of the anomaly balloon dilatation helps in avoiding complex and dangerous operations using the traditional methods. Coarctation in the form of a diaphragm is the most "favourable" type of the anomaly for balloon dilatation. The operation is also indicated in coarctation in patients with hypoplasia of the arch and isthmus, despite its palliative character.     

Surgical treatment of coarctation of the aorta]

Repair of coarctation of the aorta was performed in 37 cases (31 patients, 6 patients of reoperation) ranging from 4 days old to 15 years old. Subclavian flap repair were performed in 15, resection and end-to-end anastomosis in 14, patch aortoplasty in 6, and interposition graft in 2. Subclavian flap angioplasty or end-to-end anastomosis is considered the procedure of choice in infants. However, the incidence of reoperation significantly increased in patients younger than age one month at initial subclavian flap repair. Mechanism of recurrent coarctation may be possibly related to retention of abnormal tissue, which is possibly ductal and/or intimal shelf, with the potential for proliferation and luminal narrowing. We suggest that in applicable case end-to-end anastomosis rather than subclavian flap angioplasty may be the surgical technique of choice in infants less than one month of age, and the most common reoperation technique was patch aortoplasty in re-stenosed cases.     

Selection of surgical method in aortic coarctation]

An experience with 64 operations for aortic coarctation is summarized. The author suggests some simple mathematical calculations allowing in each particular case a selection of an optimum variant of surgical intervention. The main task is to restore a normal diameter of the aorta. Of 64 operated subjects 2 patients died, both of them were operated upon in later terms.     

Aortic coarctation,vascular ring,and right aortic arch with aberrant subclavian artery

Right aortic arch, in all situations, is relatively rare. In association with coarctation and vascular compression, it is extremely rare. We present a patient with a right aortic arch and an aberrant left subclavian artery, in addition to coarctation. This was dealt with through a left thoracotomy by dividing the ligamentum arteriosum and placing a Dacron graft from the ascending aorta to the descending aorta.     

Aortic coarctation with hypoplastic aortic arch. Results of extended end-to-end aortic arch anastomosis

Between 1980 and 1986, 80 infants (less than or equal to 3 months old) with symptomatic aortic coarctation and associated severe tubular hypoplasia of the transverse aortic arch underwent surgical treatment. Extended end-to-end aortic arch anastomosis was used in an attempt to correct both the isthmic stenosis and the hypoplasia of the transverse arch. After complete excision of the coarctation tissue, a long incision was made in the inferior aspect of the aortic arch, which was then anastomosed to the obliquely trimmed distal aorta. Pure coarctation was present in 17 patients (group I); 24 infants had an additional ventricular septal defect (group II), and 39 patients had associated complex heart disease (group III). The overall early mortality rate was 26% (confidence limits 21% to 32%) (18% in group I, 17% in group II, and 36% in group III). The early risk declined with time and was 18% (confidence limits 12% to 26%) for the last 2 years (seven deaths in 39 patients). Follow-up was 100% for a mean of 19 months. Actuarial survival rate at 3 years was 82% for group I, 78% for group II, and 32% for group III. Recurrent coarctation (gradient greater than or equal to 20 mm Hg) occurred in six operative survivors (10%, confidence limits 6% to 16%) and necessitated reoperation in three. Freedom from recoarctation at 4 years was 88%. Because extended end-to-end aortic arch anastomosis provides adequate correction of the aortic obstruction and entails a low risk of restenosis, it is our procedure of choice in infants with coarctation and severe hypoplasia of the aortic arch.     

Aortic arch reconstruction with pulmonary autograft patch in coarctation and interruption of the aorta

The surgical management of the aortic arch pathologies is still subject to discussion. Primary end-to-end anastomosis has some complications such as bronchial compression, tension in the suture lines, and probability of recurrence. On the other hand, patch aortoplasties combined with end-to-end anastomosis carry the risk of aneurysm formation and recurrence. Considering the growth potential, pulmonary autograft patch use in aortic arch reconstructions has recently been introduced into clinical practice. In this study, we present the early findings of combined end-to-end anastomosis and pulmonary autograft patchplasty procedure in six patients. According to our experience the technique applied in this report seems to be more advantageous than other conventional approaches.     

Aortic root aneurysm in an adult patient with aortic coarctation: a single-stage approach

Coarctation of the aorta is a common congenital defect that may be undiagnosed until adulthood. Moreover, coarctation is associated with congenital and acquired cardiac pathology that may require surgical intervention. The management of an adult patient with aortic coarctation and an associated cardiac defect poses a great technical challenge since there are no standard guidelines for the therapy of such a complex pathology. Several extra-anatomic bypass grafting techniques have been described, including methods in which distal anastomosis is performed on the descending thoracic aorta, allowing simultaneous intracardiac repair. We report here a 37-year old man who was diagnosed with an aortic root aneurysm and aortic coarctation. The patient was treated electively with a single-stage approach through a median sternotomy that consisted of valve-sparing replacement of the aortic root and ascending-to-descending extra-anatomic aortic bypass, using a 18-mm Dacron graft. Firstly, the aortic root was replaced with the Yacoub remodelling procedure, and then the distal anastomosis was performed to the descending aorta, behind the heart, with the posterior pericardial approach. The extra-anatomic bypass graft was brought laterally from the right atrium and implanted in the ascending graft. Postoperative recovery was uneventful and a control computed tomographic angiogram 1 month after complete repair showed good results.     

Aortic coarctation and postcoarctation mycotic aneurysm in a woman 20 weeks pregnant: anesthetic management

A 28-year-old woman, 20 weeks pregnant, was diagnosed with aortic coarctation and postcoarctation mycotic aneurysm. After anesthetic induction, blood pressure was monitored in the radial artery of the right arm and the femoral artery of the right leg for two purposes: to verify hemodynamic stability as required in this type of operation and to determine the pressure gradient between the upper and lower limbs, which was approximately 40 mm Hg. To prevent spinal cord ischemia, an intradural catheter was inserted into the fourth and fifth lumbar space for spinal fluid drainage. A double lumen tube was used for intubation so that the left lung could be blocked, and a centrifugal pump was used instead of extracorporeal circulation. The aneurysm was resected through a left thoracotomy and an aortic prosthesis was placed. Satisfactory outcome was indicated by resolution of the pressure gradient, and fetal viability was verified by ultrasound. The mother suffered no neurological complications and the pregnancy continued to term uneventfully. We review the anesthetic procedure to follow in such cases.