Emergency surgical repair of acute aortic arch dissection. Apropos of 14 cases

Among 70 patients operated upon for acute dissection of the ascending aorta between 1977 and 1984, 14 (age range 40-72 years) benefited from emergency aortic arch repair motivated by the presence in, or extension to, this segment of the portal of entry, or by lesions of the supra-aortic main vessels. The operation was performed under cardiopulmonary bypass in all cases. Cerebral protection was ensured in 9 patients by deep hypothermia alone or with circulatory arrest for a mean period of 5 minutes (7 cases), and in 5 patients by continuous brain perfusion (carotid blood flow 4 ml/kg/min; perfusion pressure 70 mmHg). Repair consisted of ascending aorta replacement combined with gluing of the arch whenever possible, or bevelled resection in the concavity of the arch, or complete aortic arch replacement with reimplantation of the cervico-cerebral vessels when necessary. In every case the gelatin-resorcin-formalin glue was used to reinforce the aortic wall or the areas with sutures. Operative mortality was nil. Hospital mortality was 28.5%. In patients operated upon under deep hypothermia alone or associated with circulatory arrest the main complications were neurological (4 cases) and respiratory (3 cases) disorders; these were responsible for the death of 3 patients. A fourth patient died of mediastinitis. No neurological or respiratory complication occurred in patients who had brain perfusion. The mean follow-up period was 37 months (range 7-84 months). Late mortality was nil. All but one patient (sequelae of paraplegia) are in excellent clinical condition. Post-operative angiography alone or combined with computerized tomography showed satisfactory blood distribution in all cases and no aneurysm formation on the dissected aorta despite a persistent false lumen.     

Determinants of survival following repair of interrupted aortic arch in infancy.

Between January 1971 and March 1987, surgery was performed in 26 infants with interrupted aortic arch. At operation the 14 boys and 12 girls weighted between 1.71 and 4.23 kg (mean +/- SD = 3.1 +/- 0.63 kg) and ranged in age from 2 to 90 days (13 +/- 18 days). The interruption was distal to the left subclavian artery in 4 (15%), between the left carotid and subclavian arteries in 20 (77%) and between the brachiocephalic (innominate) and left carotid arteries in 2 (8%). Associated complex cardiac lesions in 8 patients included complete transposition (2), common arterial trunk (2), aortopulmonary window (2), double inlet left ventricle (1) and tricuspid atresia (1). The remaining patients had an isolated ventricular septal defect. The arch was reconstructed with a prosthetic conduit in 14 patients; by a direct anastomosis in 6; using the subclavian artery in 3; and with the pulmonary trunk and the arterial duct in 2. Twenty patients (77%) underwent palliative surgery as the first stage of management, and banding of the pulmonary trunk was also performed in 16 of these. Five patients (19%) underwent primary complete repair of the interruption and intracardiac anomalies. One patient (4%) died soon after thoracotomy for palliative surgery. Of the 15 (57%, 70% confidence limits CL = 46-69%) early deaths, 7 occurred in patients with complex associated defects and 4 occurred when single stage repair was attempted. Survival following first-stage palliative surgery for arch interruption with isolated ventricular septal defect was 64% (9/14) [70% CL = 47-79%]. All of these patients subsequently underwent complete repair. Chi-squared and t-tests showed the year of operation and the type of operation (two-stage repair) to be associated with improved survival. It is concluded that a two-stage repair of interrupted aortic arch offers a reasonable alternative to primary complete correction and will lead to satisfactory subsequent repair in most cases.     

Hypoplastic right retro-oesophageal aortic arch: similarities to interrupted aortic arch.

At echocardiography a dysmorphic neonate was found to have a hypoplastic mitral valve, left ventricle, and ascending aorta with interruption of the aortic arch between the left common carotid and the left subclavian arteries--the left subclavian artery arose from a normal sized left descending aorta. Cardiac catheterisation and subsequent necropsy confirmed this arrangement but also showed a tortuous right retro-oesophageal aortic arch. This arch was severely hypoplastic distal to the right subclavian artery. These cardiovascular anomalies occurred in the child of a family in which other members showed the velo-cardio-facial syndrome, a condition known to be associated with right aortic arch. This aortic arch arrangement may be impossible to distinguish from simple aortic interruption by echocardiography alone.     

Interruption of the aortic arch and malformative cardiac lesions requiring repair under extracorporeal circulation. Apropos of 3 cases

Interruption of the aortic arch is practically always associated with intracardiac malformations of variable complexity, at the least, a ventricular septal defect. Surgery is usually performed in two stages: aortic repair and pulmonary artery banding after intravenous prostaglandin administration. The second stage comprises debanding and repair of the intracardiac lesions under cardiopulmonary bypass. However, in some cases, interruption of the aortic arch is associated with intracardiac lesions which necessitate correction under cardiopulmonary bypass from the onset, this was the situation in two of the three cases described by the authors: aorto-pulmonary window, a lesion which can only be corrected under circulatory arrest and deep hypothermia. One of these two children, operated in the neonatal period, did not survive: the other, operated at 6 weeks, had an excellent result. In the third case, the association of tricuspid atresia and a restrictive ventricular septal defect necessitated enlargement of the septal defect and therefore, open heart surgery under circulatory arrest; the results were favourable.     

Echocardiographic assessment of interrupted aortic arch

BACKGROUND: In patients with interrupted aortic arch echocardiography provides detailed information about the anatomy of the aortic arch and the associated cardiac anomalies. Only a few reports have evaluated the reliability of this non-invasive diagnostic procedure by correlation with angiographic and surgical findings. METHODS: From 1988 through 1993, 45 infants with interrupted arch underwent surgical repair (mean age 13.02 days). Of the patients, 33 had interruption of the arch between the left common carotid and subclavian arteries; 25 patients had a ventricular septal defect, and the remaining 20 had coexisting complex congenital heart defects. Preoperative diagnosis was made exclusively by echocardiography in 25 of the patients. Accuracy of echocardiographic diagnosis was evaluated retrospectively by comparing preoperative studies with angiography and surgical reports. We then investigated whether the morphologic features of the interrupted arch might influence surgical procedure or outcome. RESULTS: Intracardiac anatomy was accurately diagnosed by echocardiography in all cases; in 2 patients angiography provided additional information concerning the morphology of the aortic arch. Operative notes described differences in morphology of the arch in 7 patients, but these did not influence the surgical procedure. Direct anastomosis of the interrupted segments was possible in 38 patients, and 36 patients underwent primary intracardiac repair. Echocardiographic measurements revealed that the diameter of the ascending aorta was related to the number of vessels originating from the proximal aortic arch. The distance between the interrupted segments was significantly different according to the site of interruption, but not between cases with an isolated ventricular septal defect versus those with complex heart disease. It did not influence the method of arch repair, nor was it related to recurrent or residual obstruction. CONCLUSION: Preoperative echocardiography offers accurate and complete diagnosis in the critically ill neonate with interrupted aortic arch and associated intracardiac abnormalities.     

不同年龄段患儿主动脉弓中断一期根治外科治疗的对比观察

目的：对比分析婴幼儿及儿童主动脉弓中断的外科一期根治手术疗效,观察大龄患儿合并重度肺动脉高压对手术效果的影响。方法：15例（男性9例,女性6例）患儿一期主动脉弓中断根治。婴儿组（5 d～2.9岁）9例,体质量3.7～16.3 kg;儿童组（3.1～11.5岁）6例,其中2例年龄〉10岁,体质量13.7～30 kg。所有患儿均有非限制性室间隔缺损伴有重度肺动脉高压,并经未闭动脉导管向下肢动脉供血。全组患儿经超声心动图明确诊断,7例行CT检查,2例行心导管测压,不吸氧时右上肢饱和度均≥95%,胸X线片肺血明显增加。均于深低温半身停循环,经无名动脉选择性脑区域灌注,重建主动脉弓后,恢复全身循环,并修复心内畸形。术后呼吸机辅助呼吸,镇静镇痛药物及合理的血管活性药物,常规吸入NO或伊洛前列素,必要时应用波生坦治疗,血流动力学及氧代谢平稳后停止呼吸机辅助呼吸。结果：全组无肾衰竭及神经系统并发症。手术死亡1例（6.7%）,为3个月婴儿,术中停机困难,经（ECMO）辅助循环无效;儿童组乳糜胸一例,胸导管结扎后治愈。两组间体外循环、选择性脑灌注、主动脉阻断及术后呼吸机辅助时间差异均无统计学意义;术后婴儿组平均肺动脉压下降至（32.0±9.86）mmHg（1 mmHg=0.133 kPa）,低于儿童组的（36.7±17.09）mmHg;而儿童组ICU停留时间（5.3±2.06）d,低于婴儿组（7.5±2.95）d;但是两组间差异无统计学意义（P〉0.05）。全组随访4个月～2年,术后早中期效果良好。结论：合并重度肺动脉高压的主动脉弓中断儿童虽错过最佳手术时机,但如果右上肢饱和度≥95%,胸X线片肺血明显增多,仍可手术一期根治,并取得良好临床效果。     

Posterior pericardial ascending-to-descending aortic bypass for interrupted aortic arch in an adult

Interrupted aortic arch (IAA) in the adult population is an extremely rare condition. In this case report, we present a 35-year old male patient with Type A interrupted aortic arch (IAA) and discuss the successful surgical treatment.     

Delayed filling of the anterior interventricular artery. Apropos of 9 cases

Selective coronary angiography has shown that typical angina pectoris may occur in the absence of atheromatous coronary stenosis. Other causes of these attacks of pain have been found: coronary spasm, small vessel disease, abnormal dissociation of haemoglobin or metabolic disturbances of the myocardial cell. Of all the patients undergoing coronary angiography in 1984 at the Centre Cantini, 9 had no classical coronary lesions but delayed filling of the left anterior descending artery. This syndrome was described for the first time in 1972 by Tambe as the "slow flow velocity syndrome". The aim of this study was to analyse the clinical, ECG and haemodynamic profiles of those patients. Five of them also underwent stress Thallium myocardial scintigraphy. An ergometrine provocation test was performed afterwards under ECG control. Delayed filling was appreciated by comparison with the other vessels and also by measuring the filling time which was two or three times longer than in a control series of 9 patients with angina and normal coronary arteries. The difference was statistically significant. These findings were only observed in strictly normal coronary vessels; they were reproducible and unaffected by the administration of nitrate derivatives. In our series all 9 patients were men with an average age of 51.4 years. One patient was asymptomatic and had a history suggestive of myocardial infarction, and 4 others had typical angina of effort: all had abnormal exercise stress tests. The other 3 patients had spontaneous atypical chest pain, normal resting ECG and a negative exercise stress test (impossible in one case). The five stress Thallium scintigraphies showed myocardial perfusion defects.(ABSTRACT TRUNCATED AT 250 WORDS)     

Aortic valve replacement in an adult 20 years after a single-stage extra-anatomic repair of an interrupted aortic arch

Interrupted aortic arch is a rare congenital malformation, which is defined as a loss of luminal continuity between the ascending and descending aorta. Usually, there is a considerable distance between the ascending and descending parts of the aorta. According to the classification system of Celoria and Patton, three subtypes have to be differentiated. We describe a single-stage, extra-anatomic repair in an adolescent patient with a rare type C (the interruption is proximal to the left common carotid artery) interrupted aortic arch and his subsequent aortic valve replacement 19 years later.     

Cervical aortic arch with ventricular septal defect. A differential diagnosis from interrupted aortic arch.

A case of right cervical aortic arch with a large ventricular septal defect and a bicuspid aortic valve is reported. The angiographic diagnosis was interruption of the aortic arch type II, with aberrant right subclavian artery, a closed ductus arteriosus and retrograde vertebral-subclavian flow to the descending aorta. The cervical arch, demonstrated post mortem, was located high and deep in the neck. This differential diagnosis may have potential importance, as reported experience shows that a surgical aortic anastomosis is not required in cervical aortic arch.     

Cervical aortic arch. Apropos of a case

A new case of left cervical aorta is described, affecting a 12-year-old girl. Haemodynamic, CT scan and radiological investigations are described. The only associated abnormality in the present case was hypoplasia of the left vertebral artery. There were two spicules on the medial surface of the descending aorta, possibly embryonic rests. A review is made of the literature and there is discussion of the contribution of different diagnostic techniques to determination of the embryological origin or the abnormality and its possible treatment.     

“Open” approach to aortic arch aneurysm repair

Aortic arch aneurysm is a relatively rare entity in cardiac surgery. Repair of such aneurysms, either in isolation or combined with other cardiac procedures, remains a challenging task. The need to produce a relatively bloodless surgical field with circulatory arrest, while at the same time protecting the brain, is the hallmark of this challenge. However, a clear understanding of the topic allows a better and less morbid approach to such a complex surgery.Literature has shown the advantage of selective cerebral perfusion techniques in comparison with only circulatory arrest. Ability to perfuse the brain has allowed circulatory arrest temperatures at moderate hypothermia without the need for deep hypothermia. Even though cannulation site selection appears to be a minor issue, literature has shown that the subclavian/axillary route has the best outcomes and that femoral cannulation should only be reserved for no access patients. Although different techniques for arch anastomosis have been described, we routinely perform the distal first technique as we find it to be less cumbersome and easiest to reproduce.In this review our aim is to outline a systematic approach to aortic arch surgery. Starting with indications for intervention and proceeding with approaches on site of cannulation, approaches to brain protection with hypothermia and selective cerebral perfusion and finally surgical steps in performing the distal and arch vessels anastomosis.     

Truncus arteriosus communis associated with interrupted aortic arch: a report on two uncommon cases.

The paper presents two infants with the A-4 type of truncus arteriosus communis (according to Van Praagh's classification). One patient who survived a surgical procedure demonstrated a rare variant of aortic arch interruption to the left off the left subclavian artery (type A according to Celoria and Patton), whereas the second presented an uncommon anomaly in which the right subclavian artery originated from the descending aorta with associated severe truncal valve incompetency. The authors describe the clinical picture along with the surgical treatment of the first infant who being six days old was subjected to a correction employing the wide patent ductus arteriosus to reconstruct the aortic arch, following the method described by Gomes and McGoon. Subsequently an aortic homograft was implanted in order to connect the right ventricle and the pulmonary artery.     

Isolated type a interrupted aortic arch: in an asymptomatic 19-year-old man

Interrupted aortic arch, characterized by luminal and anatomic discontinuity between the ascending and descending aorta, is a very rare congenital malformation. The condition is typically diagnosed in neonates and is highly fatal if left untreated. Herein, we report the unusual diagnosis of an isolated type A interrupted aortic arch in a hypertensive, asymptomatic 19-year-old man.     

Complete interruption of the aortic arch in adults

We describe a case of total interruption of the aortic arch (Steidele Complex) that survived without surgical operation and reached adulthood. The patient first presented with stroke. The diagnosis was made by cardiac catheterization. The presence of fixed pulmonary arterial hypertension excluded the possibility of surgical correction.