Tetralogy of fallot associated with a double aortic arch: palliation with a prosthetic tube graft

Tetralogy of Fallot associated with a double aortic arch is an extremely rare congenital anomaly. Among the 785 patients who underwent definitive repair of tetralogy of Fallot between 1969 and 1985 at our center, only 1-a 19-month-old girl-had a double aortic arch. This report describes her case history, which included successful palliative repair with a polytetrafluoroethylene (PTFE) tube graft.     

Is banding of the pulmonary trunk obsolete for infants with tricuspid atresia and double inlet ventricle with a discordant ventriculoarterial connection? Role of aortic arch obstruction and subaortic stenosis.

Banding the pulmonary trunk may exacerbate or promote the development of subaortic stenosis in patients with double inlet ventricle or tricuspid atresia with a dominant left ventricle and discordant ventriculoarterial connection and, therefore, may be an inappropriate palliative procedure for such patients. To examine this possibility, 102 consecutive infants were studied who presented with this anatomy between 1972 and 1987. Obstruction of the aortic arch was present in 52 patients. In 28 patients (17 with aortic arch obstruction), subaortic stenosis was already apparent at presentation. Of the remaining 74 patients, 19 received no palliative surgery and 55 underwent banding of the pulmonary trunk either with (n = 22) or without (n = 33) aortic arch repair. Outcome was significantly worse in patients with associated aortic arch obstruction. All such patients either died or developed subaortic stenosis by 3 years of age (survival free of subaortic stenosis 0 of 22 versus 22 of 33 for patients with isolated banding of the pulmonary trunk, p less than 0.001). After isolated banding, there was a lower ratio of the ventricular septal defect to ascending aorta diameters at presentation in the patients who developed subaortic stenosis than in the patients who did not (0.60 +/- 0.08 versus 1.03 +/- 0.15, p less than 0.001). Of the latter, 18 (95%) of 19 patients fulfilled criteria for a Fontan procedure at recatheterization. Thus, the presence of aortic arch obstruction is associated with rapid development of subaortic stenosis after banding of the pulmonary trunk. Alternative initial surgery, even though high risk, may be indicated. In the absence of such obstruction, banding the pulmonary trunk can be performed at reasonable risk and, provided that the ventricular septal defect is of adequate size, satisfactorily prepares most patients for a later Fontan procedure.     

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.     

Palliative stent graft placement combined with subsequent open surgery for retrograde ascending dissection intra-thoracic endovascular aortic repair

Thoracic endovascular aortic repair (TEVAR) is an effective strategy for type B dissection. Retrograde ascending dissection (RAD) intra-TEVAR is a rare complication on clinic. In this case, a 48-year-old Chinese man with Stanford type B aortic dissection suffered acute RAD during the TEVAR. And palliative stent grafts placement was performed in a local hospital, which earned the time for transfer and subsequent total arch replacement surgery in Zhongshan Hospital Fudan University. This report suggests that the palliative strategy may be an option for RAD in some specific situation.     

Late aneurysm of the distal aortic arch after repair of aortic interruption. A case report

Aneurysm formation after aortic coarctation repair is not a rare complication of post-coarctation of aorta repair. We describe the case of a 43-year-old woman who had undergone repair of an isolated interruption of the aortic arch 30 years earlier, who came to our hospital with progressive chest pain, cough and dyspnea. A giant aortic aneurysm was revealed in the distal aortic arch by CT study. The patient underwent aneurysmectomy with total aortic arch replacement using a Dacron graft through redo median sternotomy. An embryologic explanation of this patient's anomaly and the previous surgical procedure are discussed for defining this rare clinical condition.     

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.     

Hybrid procedure of bilateral pulmonary artery banding and bilateral ductal stenting in an infant with aortic atresia and interrupted aortic arch

We report an infant with aortic valve atresia, interrupted aortic arch, ventricular septal defect, confluent pulmonary arteries, bilateral arterial ducts, absent common carotid arteries, and anomalous coronary arteries arising from main pulmonary artery. Hybrid procedure consisting of bilateral pulmonary artery banding and bilateral arterial duct stenting was performed at 4 weeks of age. Hybrid procedure can be an alternative palliative approach in an infant with this complex cardiac anatomy. © 2014 Wiley Periodicals, Inc.     

Enlarging angioplasty of the aortic arch with an pulmonary artery autograft patch. Interest in a single-stage repair of the syndrome of coarctation and interruption of the aortic arch]

Single stage repair of syndromes of coarctation and interruption of the aortic arch is a routine procedure in many surgical centres with good immediate results. The classical technique of aortic repair is based on the principles of Crafoord's extended resection anastomosis. Recoarctation is not an unusual long-term complication. A technique of enlarging angioplasty of the aorta using a patch of pulmonary artery has been developed and used in 22 neonates with obstruction of the aortic arch associated with ventricular septal defect with an average age and body weight of 15 days and 2.9 Kg respectively. The ventricular septal defect was closed surgically during the same procedure. Total circulatory arrest was not used in these children and all had aortic repairs with selective cerebral perfusion with moderate hypothermia (28-30 degrees C). This technique was used without any procedure-related early morbidity. No early or late deaths were observed in this series. Two patients were reoperated during the first year after the initial procedure: one for residual ventricular septal defect and the other for supraventricular pulmonary stenosis. Two patients, one of whom was reoperated, developed supraventricular pulmonary stenosis with a gradient of over 60 mmHg. These stenoses were observed in the first cases operated and were essentially due to the technique of pulmonary artery reconstruction. Over a median follow-up period of 10 months, no recoarctations were observed: the Doppler ultrasound study showed an isolated mean systolic gradient of 6 +/- 12 mmHg. The authors conclude that angioplasty of the aortic arch with an enlarging patch of pulmonary artery autograft during single stage surgery of syndromes of coarctation and interruption of the aortic arch provides a harmonious and durable repair of the aortic arch.     

Surgical implications of right aortic arch with isolation of left subclavian artery.

Few cases have been reported of the combination of right aortic arch with isolation of the left subclavian artery in childhood because of the absence of symptoms from this pairing of defects and the difficulty of its recognition by routine examination. All the reports have been associated with congenital heart disease. We report 4 cases. In one of them, the diagnosis was made during an attempt to create a left Blalock-Taussig anastomosis and the technique was modified. The diagnosis can be made before operation with an aortogram. This procedure should be carried out to show the aortic arch branches in cases where palliative surgery is contemplated.     

Surgical implications of right aortic arch with isolation of left subclavian artery.

Few cases have been reported of the combination of right aortic arch with isolation of the left subclavian artery in childhood because of the absence of symptoms from this pairing of defects and the difficulty of its recognition by routine examination. All the reports have been associated with congenital heart disease. We report 4 cases. In one of them, the diagnosis was made during an attempt to create a left Blalock-Taussig anastomosis and the technique was modified. The diagnosis can be made before operation with an aortogram. This procedure should be carried out to show the aortic arch branches in cases where palliative surgery is contemplated.     

The implications of common brachiocephalic trunk on associated congenital cardiovascular defects and their management

A common brachiocephalic trunk is an anatomic variant in which both common carotid arteries and the right subclavian artery arise from the aortic arch via a single trunk. The impact of this condition on associated congenital cardiac malformations is presently unknown. Out of a total of 1480 cardiac catheterizations performed in children over a period of 10 years, we discovered 48 patients (3.2%) to have a common brachiocephalic trunk, of whom 98% had associated congenital cardiac malformations. A spectrum of associated lesions was identified, including left-to-right shunts in 19 patients, right-sided anomalies in 18 patients, left-sided obstructive lesions in 12 patients, and coronary arterial abnormalities in 10 patients, eight of whom had other cardiac defects. Genetic syndromes were present in one-fifth of the cases. When found with left-sided malformations, the common trunk was associated with persistent hypoplasia of the aortic arch, likely related to diminished flow through the arch during development. In each of four patients in whom the brachiocephalic trunk had been used during construction of a palliative shunt, we observed inadequate growth and deformation of the pulmonary arteries. Thus, angiographic identification of a common brachiocephalic trunk may be a marker for the presence of accompanying congenital cardiac defects and coronary arterial abnormalities. Understanding the pathophysiologic effects of the common trunk is important when planning the palliative or corrective procedures, and when assessing the potential benefit of the surgical repair over the long term.     

Endovascular repair of a thoracic arch aneurysm with a fenestrated stent-graft

Purpose: To report a new endovascular technique for thoracic aortic arch aneurysm repair with a fenestrated stent-graft. Case Report: A 75-year-old man presented with a 5.5-cm thoracic aneurysm involving the inner curve and left anterior lateral aspect of the aortic arch. The innominate and left carotid arteries shared a common trunk, and the right vertebral artery was dominant. Endovascular repair was performed utilizing a fenestrated stent-graft that allowed perfusion of the innominate and left carotid arteries but occluded the left subclavian artery. At 8 months post procedure, the patient is well; imaging documented the continued exclusion of the aneurysm and perfusion of the common trunk branches. Conclusion: Fenestrated endovascular grafts for specific thoracic aortic arch aneurysm can be used safely in the high-risk patient.     

Imaging of the Postsurgical Aorta in Marfan Syndrome

Marfan syndrome is a heritable multisystem connective tissue disease and is the most common genetic cause of aortic disease. Guidelines for surgical repair and recommendations for surveillance imaging aim to prevent aneurysm rupture, aortic dissection, and death. Options for proximal aorta repair include the modified Bentall procedure and valve-sparing aortic root repairs. Hemiarch and total arch replacement are options for aortic arch repair, and the elephant trunk procedure is a special two-staged total arch repair facilitating future descending aorta repair. Endovascular repair is not currently considered an acceptable long-term treatment option in Marfan syndrome but may be performed when open repair is not feasible or in the acute setting as a bridge to definitive surgical treatment. After an initial surgery, patients remain at risk for new aortic dissection, propagation of a pre-existing aortic dissection, and new or enlarging aortic aneurysm. Anastomotic pseudoaneurysm is a potential postsurgical complication at multiple sites including proximal and distal aortic anastomoses, coronary anastomoses, and intercostal and visceral artery patches. Patients undergoing endovascular repair are at increased risk of endoleak and aortic dissection. CT and MR angiography are the main imaging modalities for surveillance of the aorta in Marfan syndrome and are capable of demonstrating these complications, as well as expected postsurgical appearances. 4D flow MRI is an emerging technique providing hemodynamic information that may risk-stratify aneurysms better than size alone and help predict future aortic events.     

Quantitative analysis of hypertrophy in cardiac chambers in cyanotic tetralogy of Fallot

Although early total corrective repair for cyanotic tetralogy of Fallot is now safely performed at many institutions, long-term complications after surgical repair have been demonstrated. Therefore, the optimal procedure and timing for surgical treatment remain controxersial. In the present study, we conducted a quantitative analysis of the hypertrophy of all four chambers of 87 autopsied hearts of cyanotic tetralogy of Fallot and 71 normal control hearts utilizing the myocardial mass index, and evaluated the progression of lesions with advancing age. In cyanotic tetralogy of Fallot, hypertrophy of the right ventricle progresses immediately after birth, with that of the right atrium developing soon after. The left side of the heart is normal or slightly atrophied which could be corrected by sufficient palliative intervention or total corrective repair. The growth curves of both ventricles were parallel to those of normal hearts for the period studied. Pulmonary atresia, palliative operation, and total corrective repair have been shown to have some influence on the morphological characteristics of hearts of cyanotic tetralogy of Fallot.     

Profound hypothermic circulatory arrest in management of aortic aneurysms

A total of 15 patients having aneurysms of aorta were operated from June 1997 to December 1998 using deep hypothermic circulatory arrest as a modality of brain protection. There were 12 males and 3 females. The age ranged from 19 years to 74 years and the mean age was 44.9 years. Nine patients had aneurysms of ascending aorta (group I), one had aneurysm of ascending aorta and arch of aorta (group II), four had aneurysm of the distal aortic arch (group III) and one patient had thoracoabdominal aortic aneurysm (group IV). In group I, six patients underwent Bentall procedure, two underwent Wheat procedure and one patient had repair of pseudoaneurysm of ascending aorta. The only patient in group II had his ascending aorta and arch replaced, with reimplantation of left common carotid and innominate artery. In group III, three patients had interposition Gelseal graft and one had repair of the tear in distal aortic arch. The lone patient in group IV had interposition Gelseal graft of thoracoabdominal aorta. The hypothermic circulatory arrest was used in all of them for brain and/or spinal cord protection. Retrograde cerebral perfusion was used in two patients. There were two (13%) operative deaths. One patient died of cerebrovascular accident on eighth post-operative day and second died of inadequate surgical repair. There was one instance of left hemiparesis secondary to an infarct in right frontoparietal region. To conclude, hypothermic circulatory arrest could provide an adequate brain protection for aortic aneurysm surgery. Retrograde cerebral perfusion could be an adjuvant when the anticipated time of hypothermic circulatory arrest is likely to exceed 45 minutes.     

Hybrid repair of aortic arch aneurysm

Aneurysms of the transverse aortic arch requiring surgery most often affects elderly patients with multiple co-morbidities and represents a significant challenge to both patient and surgeon. The hybrid approach developed in recent years (debranching followed by endovascular repair) may improve the morbidity and mortality of the population risk. We present the case report of a 72-year-old man with aortic arch aneurysm arising at the origin of the left subclavian artery involving whole caudal segment of an aortic arch with concomitant single vessel coronary disease. The hybrid procedure was carried out in two stages, first (open surgical approach) performing an extra-anatomic bypass – debranching combining with concomitant coronary artery bypass procedure without heart–lung machine and following day deploying the aortic endograft. Postoperative period was uneventful. On the 15th day after hybrid procedure, the patient was discharged in a stabilized condition for ambulatory care. This approach may be an alternative to standard open procedures in high-risk patients with promising midterm results.     

Tetralogy of Fallot. Risk factors associated with complete repair.

Ninety-six consecutive total repairs of the tetralogy of Fallot are reviewed. There was an overall hospital mortality of 8-3 per cent and a total incidence of low output cardiac failure of 18-8 per cent, and this was the principal cause of death and the most important source of postoperative morbidity. In 49 cases there had been a previous palliative shunt procedure and there was a strikingly lower mortality and a highly significant lower morbidity in this group. Other factors which correlated in a positive fashion with increased mortality and morbidity were chronic hypoxia (as evidenced by polycythaemia), age below 5 years, severe postoperative right ventricular hypertension, and (to a lesser extent) extensive right ventricular outflow tract reconstruction. Data are presented to support the hypothesis that a palliative shunt procedure should be considered in the severely polycythaemic child with a surgically 'unfavourable' right ventricular outflow. This policy carries a low early mortality in our hands (5-4%), and is associated with a low mortality (3-9%) at a subsequent repair. This compares with a mortality of 12-8 per cent for primary repair, and the incidence of low output cardiac failure is five times as high in the primary repair as compared to the previously shunted group.     

Tetralogy of Fallot. Risk factors associated with complete repair.

Ninety-six consecutive total repairs of the tetralogy of Fallot are reviewed. There was an overall hospital mortality of 8-3 per cent and a total incidence of low output cardiac failure of 18-8 per cent, and this was the principal cause of death and the most important source of postoperative morbidity. In 49 cases there had been a previous palliative shunt procedure and there was a strikingly lower mortality and a highly significant lower morbidity in this group. Other factors which correlated in a positive fashion with increased mortality and morbidity were chronic hypoxia (as evidenced by polycythaemia), age below 5 years, severe postoperative right ventricular hypertension, and (to a lesser extent) extensive right ventricular outflow tract reconstruction. Data are presented to support the hypothesis that a palliative shunt procedure should be considered in the severely polycythaemic child with a surgically 'unfavourable' right ventricular outflow. This policy carries a low early mortality in our hands (5-4%), and is associated with a low mortality (3-9%) at a subsequent repair. This compares with a mortality of 12-8 per cent for primary repair, and the incidence of low output cardiac failure is five times as high in the primary repair as compared to the previously shunted group.     

Mini-access open arch repair

BackgroundThe use of minimally invasive approaches is scarce in open aortic arch repair because of its perceived high operative risk and technical difficulty.MethodsThis study enrolled 59 consecutive patients (aged 58.2±13.2 years) undergoing elective arch replacement either through upper hemi-sternotomy (n=58) or mini-thoracotomy (n=1) between 2015 and 2020. Of these, 44 underwent hemiarch replacement and 15 underwent total arch replacement. Moderate hypothermic circulatory arrest was used for all patients while antegrade cerebral perfusion was selectively used for total arch repair. For more efficient distal aortic anastomosis in limited spaces, inverted graft anastomosis was utilized whenever possible.ResultsHemi-sternotomy involved upper sternal separation down to the second, third, and fourth intercostal spaces in 1 (1.7%), 30 (50.8%), and 27 (45.8%) patients, respectively. Concomitant cardiac procedures included root replacement in 19 patients (32.2%) and aortic valve replacement in 21 patients (35.6%). Circulatory arrest, cardiac ischemic, cardiopulmonary bypass, and total procedural times were 8.9±3.4, 91.1±31.1, 114.6±46.2, and 250.3±79.5 min, respectively for total arch repair, and 25.0±12.1, 72.3±16.6, 106.0±16.9, and 249.1±41.7 min, respectively for hemiarch repair. Conversion to full-sternotomy was required in 1 patient (1.7%) due to bleeding. There was one case of mortality (1.7%) attributable to low-cardiac output syndrome following hemiarch repair concomitantly with Bentall procedure. Major complications included requirement for mechanical support in 1 (1.7%), temporary neurologic deficit in 1 (1.7%), newly initiated dialysis in 3 (5.1%), and re-exploration due to bleeding in 2 (3.4%).ConclusionsMini-access open arch repair is technically feasible and achieved excellent early outcomes.     

Implantation of endovascular stents for hypoplasia of the transverse aortic arch

Hypoplasia of the transverse aortic arch is commonly associated with aortic coarctation. Persistent or recurrent obstruction can occur at this level after successful repair of the native coarcted segment. The purpose of this report is to present a new technique to treat such lesions, namely with implantation of a balloon-expandable stent. This approach was used successfully in 4 children with such hypoplasia occurring after repair of coarctation. Implantation led to both anatomical and physiological relief of obstruction in all. The patients tolerated the procedure, and there were no major adverse events.