Adult aortic coarctation discovered incidentally after the rupture of sinus of Valsalva aneurysm: combined surgical and interventional approach

Combination of ruptured sinus of Valsalva aneurysm (SVA), and a coexisting asymptomatic adult aortic isthmic coarctation is extremely rare. The timing and sequence of surgical and/or interventional repair of these two pathologies are controversial. We present a case of a 37-year-old male who was admitted to our department because of severe acute congestive heart failure and signs of ruptured aneurysm of the SV into the right ventricle. Transthoracic and transoesophageal echocardiography confirmed the communication between an important right coronary SVA and right ventricle, bicuspid aortic valve, mild aortic regurgitation, and revealed severe aortic coarctation. Because of the severe dilation of right sinus of Valsalva a surgical repair of the ruptured aneurysm was performed. Aortic coarctation was treated four weeks later by a percutaneous stent-graft implantation. This case report supports the concept that hybrid approach is feasible in patients with ruptured SVA and aortic coarctation in adulthood.     

自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并弓发育不良

目的 探讨缩窄段切除加自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并主动脉弓发育不良的方法和效果.方法 2007年5月至2009年12月,14例主动脉缩窄合并主动脉弓发育不良病婴行主动脉缩窄段切除加自体肺动脉补片主动脉弓成形手术,其中男9例,女5例;年龄23天至17个月,中位值4.33月龄;平均体重(6.14±2.36)kg.所有病婴均诊断为合并室间隔缺损的主动脉缩窄,同时存在主动脉弓发育不良.手术在深低温体外循环下完成,其中8例采用选择性脑灌注技术,6例停循环.主动脉成形采用新鲜的自体肺动脉补片.同期修补合并的室间隔缺损.结果 围术期死于循环衰竭1例.13例生存,其中1例合并低心排血量综合征,经相应治疗恢复.术后超声心动图检查主动脉弓无残余梗阻.随访4个月至3年.超声心动图示主动脉弓压力阶差均<16 mm Hg(1 mm Hg=0.133 kPa),随访期间主动脉弓降部血流速度与出院时无明显变化.CT扫描显示主动脉弓几何构形正常;术后半年原左主支气管受压者症状明显改善或完全消失.无主动脉夹层动脉瘤发生.结论 缩窄段切除加自体肺动脉补片主动脉弓成形是治疗婴儿主动脉缩窄合并主动脉弓发育不良理想的手术方法.

Abstract：

Objective To discuss the operative techniques and results of coarctation resection plus aortoplasty with pulmonary autograft patch for coarctation of the aorta combined with hypoplastic aortic arch in infant.Methods Between May 2007 and Dec 2009,14 cases including 9 males and 5 females with caorctation of the aorta and hypoplastic aortic arch underwent coarctation resection plus aortoplasty with pulmonary autograft patch in our hospital.The age ranged from 23 days to 17 months,with a median of 4.33 months.The mean body weight was (6.14 ±2.36) kg.All patients were diagnosed as aortic coarctation combined with VSD and hypoplastic aortic arch.The surgery was performed under deep hypothermia cardiopulmonary bypass with selective cerebral perfusion in 8 cases and circulation arrest in 6 cases.Fresh pulmonary autograft patch harvested from the main pulmonary artery was used for aortoplasty.The associated VSD was repaired in the same stage.Results All patients survived except one died from circulatory failure during the perioperative period.Low cardiac output syndrome occurred in another case who was cured afterwards by correspondent treatments.No residual obstruction was detected by echocar-diography after the operation.Follow-up was carried out in 13 cases from 4 months to 3 years.Echocardiographic examination showed that the pressure gradient across the aortic arch was less than 16 mm Hg in all cases.The blood velocity at the descending aortic arch was not significantly changed during the follow-up period as compared with that of the immediate after operation.Computed tomography showed that the morphology of aortic arch was normal.The left bronchus compression was relieved obviously or totally disappeared in patients who suffered from left bronchus stenosis before operation,and no aortic aneurysm were detected in these patients.Conclusion Conclusion Coarctation resection plus aortoplasty with pulmonary autograft patch is the optimal surgical method for treating coarctation of the aorta combined with hypoplastic aortic arch in infant.     

Aneurysm of the ascending aorta in its coarctation

In long-term period after resection of aorta coarctation, 9-14% patients developed aortic aneurysms, one third of them localized in the ascending aorta. From 146 patients operated on for aneurysm of the ascending aorta, 3 had aortic coarctation. In 2 patients aneurysms of the ascending aorta formed late after resection of the coarctation, the third patient was hospitalized with clinical picture of cardiac insufficiency at terminal stage due to coarctation of the aorta and a giant aneurysm of the ascending aorta with significant aortic insufficiency. The patients underwent successful surgeries: 1) ascending aorta grafting; 2) aortic valve and ascending aorta grafting by Bentallo de Bono method; 3) aortic valve and ascending aorta grafting by Bentallo de Bono method with bypass of descending aorta from the conduit. Cystic medianecrosis and two-volume aortic valve were revealed in all the patients. It is concluded that patients after surgery for coarctation of the aorta require long-term follow-up to defect cardiovascular complications early.     

Abdominal aortic coarctation with splanchnic arterial occlusion

Abdominal aortic coarctation is found in only 2% of aortic coarctation and is usually manifested by renovascular hypertension. Splanchnic arterial occlusive lesions occur in 22% of these patients and are exceptionally symptomatic. We present a case report of a young patient with abdominal aortic coarctation causing hypertension and visceral angina. The aetiopathogeny and treatment are discussed.     

Coarctation of the aorta with right aortic arch: surgical technique and new classification.

BACKGROUND: The combination of right aortic arch and coarctation of the aorta has seldom been reported. This rare abnormality occurs as an isolated lesion or in association with other congenital defects, such as mixed gonadal dysgenesis and Turner's syndrome. METHODS: The medical records of 2 patients who underwent operation for right aortic arch and aortic coarctation in our institution were reviewed together with case reports in the literature. Various surgical options have been reported: synthetic grafts, subclavian flap, and end-to-end anastomosis. End-to-end anastomosis is our method of choice for coarctation of the aorta and for right aortic arch with coarctation, even though an excessive amount of dissection is needed. RESULTS: Both patients from our institution are doing well, with no sign of recoarctation in either patient. Our experience and recent advances in the understanding of the anatomy of this lesion led us to develop an alternative simplified classification for right aortic arch, which is presented here. CONCLUSIONS: Right aortic arch and coarctation of the aorta is a rare morphologic combination. On the basis of our experience with repair of coarctation of the aorta and our review of the literature, we think that end-to-end anastomosis is the operative technique of choice with the best long-term results. Our simplified classification is easy to understand when dealing with right aortic arch.     

Right aortic arch with mirror-image branching and coarctation of the aorta--a case report]

The right aortic arch with coarctation of the aorta was reported. A 56-year-old woman admitted to the hospital because of headache and hypertension. Cardiac catheterization revealed the right aortic arch with coarctation of the aorta and 80 mmHg pressure gradient across the coarctation. The bypass operation with a 14 mm Dacron graft between the ascending to descending aorta was performed. There was no peak systolic pressure gradient between the ascending and descending aorta after bypass operation. This patient is the fourth case report with both mirror-image type right aortic arch and coarctation of the aorta.     

Single-stage repair of aortic coarctation with concomitant bicuspid aortic valve and ascending aortic aneurysm

The optimal surgical management of patients presenting with an aortic coarctation together with other cardiovascular disorders is unclear. In this study, we report the case of an adult male with an aortic coarctation associated with a bicuspid aortic valve and an ascending aortic aneurysm. The patient underwent single-stage repair involving the Bentall technique and total arch replacement combined with stented elephant trunk implantation, which was performed through median sternotomy. We consider this procedure to be a suitable alternative for treating these complex cases.     

Fate of hypertension after repair of coarctation of the aorta in adults

BACKGROUND: Unrepaired aortic coarctation is known to have a detrimental effect on survival. The benefit of coarctation repair on systolic hypertension in adults has been questioned. This retrospective study was conducted to evaluate the impact of repair of aortic coarctation on systolic hypertension in adults. METHODS: Repair of aortic coarctation was performed in 84 patients aged 16-54 (mean 29) years. All patients were hypertensive before surgical intervention (mean systolic blood pressure 162 mmHg; mean diastolic blood pressure 93 mmHg). All patients underwent echocardiography and/or cardiac catheterization. The peak mean systolic gradient across the coarctation was 60 mmHg. The patients were followed after coarctation repair for between 1 and 12 (mean 5.2) years. RESULTS: There was significant regression of hypertension (P < 0.001) in all patients. Thirty-five patients (42 per cent) did not need any antihypertensive medication 3 months after surgery. The prevalence of hypertension at the last follow-up (after mean 5.2 years) was 31 per cent. CONCLUSION: Surgical repair of coarctation of the aorta in adults leads to regression of systolic hypertension and a decreased requirement for antihypertensive medication.     

分期介入治疗主动脉缩窄合并右锁骨下动脉瘤1例

主动脉缩窄(CoA)是指主动脉局限狭窄,管腔缩小,造成血流量减少.主动脉缩窄可以单独出现,也可以合并二叶式主动脉瓣、室间隔缺损、动脉导管未闭、大动脉转位等病变.主动脉狭窄合并右锁骨下动脉瘤非常罕见,本文报导一例主动脉缩窄合并右锁骨下动脉及降主动脉瘤,一期应用定制的Armada 35球囊扩张导管及覆膜支架治疗主动脉狭窄,...     

Internal mammary artery dilatation in a patient with aortic coarctation,aortic stenosis,and coronary disease. Case report

The ideal surgical approach is unclear in adult patients with coarctation of the aorta that is associated with other cardiovascular pathologies that require intervention. Standard median sternotomy allows simultaneous, coronary revascularization surgery, valve replacement and repair of aortic coarctation. However the collateral circulation and the anatomy of the mammary arteries must be determined, to avoid possible complications. We report a case of a 69 year-old man with aortic coarctation, aortic stenosis, coronary artery disease and internal mammary artery dilatation who underwent concomitant surgical procedures through a median sternotomy.     

Repair of Coarctation of the Aorta in Neonates and Young Infants

Abstract In repair of coarctation in neonates or young infants, inadequate removal of ductal tissue, failure to address hypoplasia of the aortic arch, and suture line tension have been reported to be important factors of residual or early recurrent stenosis at the coarctation repair site. In a consecutive series of neonates and young infants with coarctation, who were all operated without delay with extended resection, the clinical outcome regarding the development of restenosis and hypertension was studied. In addition, the resected specimens were investigated regarding the completeness of resection of ductal tissue. Twenty-five consecutive neonates and young infants (median age 22 days, range 5 to 39 days) who underwent surgical correction of coarctation were reviewed; the resected specimens were examined histologically to document the extent of ductal tissue in the aortic wall. Fifteen patients had a preductal coarctation with associated cardiovascular anomalies including a hypoplastic aortic arch (n = 11). The remaining 10 patients had a paraductal coarctation without associated intracardiac anomalies. In all patients, the isthmus was bypassed and an end-to-side anastomosis was constructed between the descending aorta and the undersurface of the proximal aortic arch (n = 13) or the distal ascending aorta (n = 12). In 13 patients without marked hypoplasia of tbe aortic arch, the coarctation repair was performed through a left thoracotomy. In the remaining 12 patients, the coarctation was repaired through a median sternotomy with CPB and hypothermic circulatory arrest, on the basis of an associated hypoplastic aortic arch (n = 4), hypoplastic aortic arch with intracardiac anomalies (n = 7), or a “bovine” innominate artery (n = 1). There was no perioperative or late mortality. At a median follow-up of 15 months, 1 patient (4%) developed a recurrent stenosis at the coarctation repair site; in the remaining 24 patients, echocardiograpby showed a widely patent anastomosis with no evidence of a hemodynamically significant gradient. None of the patients had hypertension. Histologic examination of the resected specimens demonstrated the presence of ductal tissue in the descending aorta with maximal extension into its lateral wall (mean 5.2 mm). In all specimens of the paraductal subtype, there was also extension of ductal tissue into the lateral wall of tbe isthmus (mean 3.9 mm). We conclude that: (1) in the absence of marked hypoplasia of the proximal aortic arch, coarctation can be repaired with low mortality and morbidity via a left thoracotomy; (2) in the presence of marked hypoplasia of the proximal aortic arch and/or if associated intracardiac defects also need to be repaired, we advocate repair of the coarctation and associated defects through a median sternotomy with circulatory arrest; (3) in view of the absence of postoperative hypertension in this series, early repair of aortic coarctation is recommended; and (4) because ductal tissue may extend not only into the descending aorta but also into the isthmus, complete excision of the coarctation and bypass of the isthmus are valuable techniques to avoid secondary constriction of the aorta by ductal tissue.     

Acute post-traumatic coarctation of the abdominal aorta.

An unusual case of coarctation of the abdominal aorta following trauma in reported. The resultant supratenal aortic constriction was associated with severe hypertension and elevated plasma renin concentration. Gradual aortic dilation at the suture site was accompanied by remission of hypertension and return of plasma renin concentration to normal. Correlation of laboratory and hemodynamic changes resulting from this unusual case of abdominal coarctation is discussed.     

Delayed presentation of massive haemoptysis from aortic aneurysm after aortic coarctation repair (a case report)

IntroductionMassive haemoptysis refers to coughing and losing a huge amount of blood in a 24-hour period. It's a life-threatening condition with high mortality rate.Case presentationWe report a rare case of massive haemoptysis in a 60-year-old female patient who had aortic coarctation repair 30 years ago. Her Computed tomography (CT) angiography showed huge aneurysmal dilatation and dissection of the descending thoracic aorta at the site of the repair. Thoracic endovascular aortic repair (TEVAR) was done, but the patient had recurrent massive haemoptysis due to extension of the aneurysm to the aortic arch. The patient then underwent one stage surgical right to left carotid artery shunt followed by TEVAR to the aortic arch covering the left common carotid artery. The procedure was successful, and haemoptysis was controlled without any complications.DiscussionIn this case the high index of suspicion for thoracic aortic aneurysm in patients presenting with haemoptysis and prior history of coarctation repair were demonstrated.Conclusionmassive haemoptysis in patients who had aortic coarctation repair is an alarming sign, and surgical intervention is required. TEVAR has become one of the best approaches for managing aortic aneurysm and has replaced open repair.     

Successful one stage repair of coarctation of the aorta and aneurysm of the ascending aorta in a child

A case of successful one stage repair of aortic coarctation and ascending aortic aneurysm in a 10 year old boy with cystic medial necrosis and congenital bicuspid aortic valve is presented. The patient underwent correction of both lesions at the same operation through two separate incisions. The coarctation was repaired first. The aortic valve was found to be hemodynamically normal and was not replaced. The patient leads a normal life at five and half years after operation and is playing ice hockey with no limitations. Close and long-term follow-up is considered essential in view of the potential complications.     

The neural crest as a possible pathogenetic factor in coarctation of the aorta and bicuspid aortic valve

Patients (n = 109) operated on for coarctation of the aorta were analyzed for occurrence of associated cardiac and noncardiac anomalies. Attention was also paid to the prevalence of cardiac anomalies in the relatives of these patients. Of the patients with coarctation of the aorta, 57 (52%) had a bicuspid aortic valve. Forty-three (39%) of the 109 patients had one or more noncardiac anomalies. In 29 (27%) patients the noncardiac anomaly involved the head/neck structures. Noncardiac anomalies were much more prevalent in patients with coarctation and bicuspid aortic valve, especially anomalies involving the head/neck structures: 44% compared to 8% of patients with a normal aortic valve. Congenital cardiac malformations were present in relatives in the first or second degree of 18% of the patients. Bicuspid aortic valve was more prevalent in patients with an affected relative (75%) than in patients with unaffected relatives (47%). Recent studies showed that the neural crest plays an important role in the development of cardiac and a variety of noncardiac structures. The cardiac structures derived from the neural crest involve the outflow tract of the heart and the aortic arch system. Maldevelopment of neural crest cells could therefore be responsible for the combined occurrence of outflow tract (e.g., bicuspid aortic valve), aortic arch (e.g., coarctation), and noncardiac anomalies. This study supports the concept that some anomalies of the aortic arch system, including aortic coarctation, are cardiovascular manifestations of a spectrum of anomalies involving the head and neck region that may be due to a genetic-environmental disorder of the neural crest.     

Early repair of coarctation of the aorta.

BACKGROUND: Repair of coarctation of the aorta with hypoplasia and elongation of the proximal aortic arch is a technically demanding procedure with a substantial rate of recurrent stenosis at the coarctation repair site. In addition, a high incidence of hypertension has been reported in patients who underwent repair beyond infancy. PATIENTS AND METHODS: Between January 1991 and June 1997, 52 patients (34 neonates and 18 infants with a median age of 37 days; range 2 days to 8 months) with a mean peak systolic upper to lower extremity resting gradient of 33.5 +/- 18.9 mmHg underwent repair of aortic coarctation. The echocardiographically measured median diameter of the aortic arch immediately distal to the innominate artery was 5.4 mm (range 4.0 to 8.1 mm). Eight patients (15%) were considered hypertensive. In 41 patients, through a left thoracotomy, an end-to-side anastomosis was constructed between the descending aorta and the undersurface of the proximal aortic arch. In 12 of these patients (who all had a hypoplastic and elongated aortic arch) this procedure was preceded by the construction of an extended side-to-side left carotid-subclavian arterioplasty. The remaining 11 patients, all with hypoplasia of the aortic arch, had concomitant complete repair of intracardiac anomalies through a median sternotomy. In 8 of these patients, in addition to anastomosis of the descending aorta to the undersurface of the proximal aortic arch, the ascending aorta and aortic arch were augmented with a pulmonary homograft patch. RESULTS: One neonate with associated Shone's syndrome died (2%) on the first postoperative day. There was no late mortality. Early postoperative complications included recurrent laryngeal nerve injury in 1 patient and prolonged chest tube drainage in 4 patients. At a median follow-up of 55 months (range 15 to 92 months), only 3 patients (5. 7%) developed a recurrent stenosis at the coarctation repair site. The remaining 48 patients are free of recurrent stenosis by echocardiography and clinical examination. None of the patients had systemic hypertension. CONCLUSIONS: 1. Coarctation repair consisting of resection of all ductal tissue with end-to-side anastomosis of the descending aorta to the undersurface of the (proximal) aortic arch, if necessary combined with a side-to side left carotid-subclavian arterioplasty, may lead to excellent results. 2. This technique can be applied with low mortality and morbidity via a left thoracotomy if the proximal aortic arch is at least 5 mm in diameter. 3. In view of the low mortality, the low incidence of restenosis at the coarctation repair site, and the absence of substantial morbidity including the development of hypertension, we advocate repair of aortic coarctation at neonatal age or in early infancy to avoid the detrimental sequelae of delayed repair of coarctation, in particular hypertension.     

Ascending-distal abdominal aorta bypass for treatment of hypoplastic aortic arch and atypical coarctation in the adult

Repair of aortic coarctation is usually an easy operation. However, it can be very difficult under certain circumstances. These include operating on an adult or operating when specific anatomical variations, such as hypoplasia of the transverse aortic arch or calcification of the coarctation area, are present. We recommend that in such cases the situation be handled using ascending aorta-lower abdominal aorta bypass grafts rather than conventional resection and anastomosis of the coarctation itself. The cases of 2 patients are presented in whom a hypoplastic aortic arch associated with atypical coarctation was repaired using such a procedure.     

Transverse arch hypoplasia may predispose patients to aneurysm formation after patch repair of aortic coarctation

BACKGROUND: Thoracic aortic aneurysm after patch repair of aortic coarctation is a potentially lethal complication. We hypothesized that transverse arch hypoplasia in association with patch repair of aortic coarctation predisposes aneurysm formation. METHODS: A retrospective analysis was performed on all patients undergoing isolated aortic coarctation repair at the University of Virginia Health Systems between 1970 and 1995. Of 244 repairs, 38 patients underwent patch repair. These 38 patients were divided into two groups. The aneurysm group (n = 15) had aortic aneurysms develop at the repair site, which required aneurysmectomy. The nonaneurysm group (n = 23) did not have any aneurysms develop. Univariate analysis and Fisher's exact text were used to identify factors that independently predict aneurysm formation. RESULTS: Univariate analysis demonstrated aortic arch hypoplasia associated with patch repair independently predicts future aneurysm formation (p < 0.01). Patients who had an aneurysm develop also had a similar incidence of bicuspid aortic valves, mild restenosis, and late hypertension compared with patients in the nonaneurysm group. CONCLUSIONS: Aneurysm formation after patch repair of aortic coarctation develops into a subgroup of patients. Aortic arch hypoplasia associated with coarctation independently predicts future aneurysm formation.     

婴幼儿主动脉缩窄合并心内畸形的外科治疗

目的 探讨婴幼儿主动脉缩窄合并心内畸形的外科治疗经验.方法 2000年1月至2006年12月,84例主动脉缩窄合并心内畸形患儿接受了外科手术治疗,手术年龄1个月～3岁(平均13.5个月),体重3.3～15.0 kg(平均7.3 kg).12例合并复杂心内畸形,72例合并室间隔缺损和其他简单心内畸形,23例伴有主动脉弓发育不良.一期手术62例,49例正中开胸同时矫治主动脉缩窄和心内畸形,13例左侧开胸矫治主动脉缩窄,正中开胸修补心内畸形;分期手术22例.主动脉缩窄的手术方式包括补片成形42例,切除端端吻合30例,锁骨下动脉翻转6例,血管旁路3例,球囊扩张1例.在49例正中切口一期手术中,43例应用选择性脑灌注加下半身停循环,4例应用全身低流量灌注,2例应用深低温停循环.结果 围手术期死亡8例,死亡率9.5%,其中3例为术前漏诊主动脉缩窄.结论 婴幼儿主动脉缩窄合并心内畸形的外科治疗可获得良好的近期疗效,绝大部分患儿可采取正中切口一期手术.选择性脑灌注和下半身停循环可以有效地保护脑和重要脏器.     

Late pseudoaneurysm formation after patch angioplasty of coarctation of the aorta; report of a case

We report a case of a 13-year-old boy presenting with pseudoaneurysm associated with a knitted Dacron patch used to repair a coarctation of the aorta. At the age of 3 months, he had undergone patch angioplasty for a coarctation of the aorta, which develops following patent ductus arteriosus division at 2 months of age. He was treated by distal aortic arch replacement using 16 mm woven Dacron tube graft in an end-to-end fashion with open proximal anastomosis under deep hypothermic circulatory arrest. The aneurysm was in the aortic wall opposite the patch graft. There was no evidence of infection or dilatation of the patch graft. This case illustrates that repair of aortic coarctations with Dacron patches cannot be recommended.