Interrupted aortic arch in an adult single-stage extra-anatomic repair

Interrupted aortic arch is a rare congenital malformation of the aortic arch that occurs in 3 per million live births. Defined as a loss of luminal continuity between the ascending and descending portions of the aorta, this anomaly entails a very poor prognosis without surgical treatment. To our knowledge, the world medical literature contains only 12 reports of isolated interrupted aortic arch diagnosed in adults. Nine of these patients underwent successful surgical repair, but 1 died during the early postoperative period. We describe a 10th successful surgical repair, which involved a 42-year-old woman who had an asymptomatic type B interrupted aortic arch (characterized by interruption between the left subclavian and left carotid arteries). We performed a single-stage extra-anatomic repair by placing a 16-mm extra-anatomic Dacron graft between the ascending and descending portions of the thoracic aorta and by interposing a 7-mm extra-anatomic Dacron graft between the 16-mm graft and the left subclavian artery. The patient recovered uneventfully and continued to do well 6 months later.     

Single-stage aortic valve-sparing root replacement and extra-anatomic bypass for aortic arch interruption in an adult

We describe the treatment of an extremely rare case of interrupted aortic arch, annuloaortic ectasia, and aortic regurgitation in a 34-year-old man who presented with dyspnea and palpitation. We performed a single-stage operation involving aortic root reconstruction and valve repair with concomitant extra-anatomic bypass from the ascending to the descending aorta with a Dacron tube graft. The patient made a full recovery. To the best of our knowledge, this is the first such report in the English medical literature.     

Aortic valve repair versus replacement in bicuspid aortic valve disease

BACKGROUND AND AIM OF THE STUDY: The study aim was to compare the results of aortic valve repair and replacement with biological valves in adult patients with aortic insufficiency (AI) caused by congenital bicuspid aortic valve (BAV) METHODS: Forty-four patients who had aortic valve repair were matched for age and left ventricular function to 44 patients who had aortic valve replacement (AVR) with biological valves. Patients were followed annually using echocardiography. The mean follow up was 2.6 +/- 2.1 years for the repair group, and 3.5 +/- 2.1 years for the replacement group. Follow up was complete. RESULTS: There was no operative or late death in either group. Early postoperative echocardiography showed trace or no AI in 35 patients and mild AI in nine who had repair, and trace or no AI in 38 patients and mild AI in five who had AVR. The mean peak systolic gradient was 16.2 +/- 7.6 mmHg for repair and 13.2 +/- 7.2 mmHg for AVR. Four patients who had valve repair and two who had AVR, needed repeat aortic valve surgery because of progressive AI or endocarditis. Freedom from reoperation at five years was 91 +/- 5% for repair and 94 +/- 6% for replacement (p = 0.2), while freedom from moderate or severe AI at five years was 79 +/- 8% for repair and 94 +/- 6% for replacement (p = 0.024). The peak systolic gradient at follow up was 11.7 +/- 6.8 mmHg after repair and 13.3 +/- 9.6 mmHg after AVR (p = 0.4). There were no thromboembolic complications in either group. CONCLUSION: Repair of BAV is feasible in certain patients with AI, but the hemodynamics and clinical outcomes do not appear to be superior to AVR with biological valves during the first five years of follow up.     

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.     

An interrupted suture technique for ascending aortic replacement concomitant to aortic valve repair

While running suture anastomoses represent the mainstay of thoracic aortic surgery, advantages may exist for interrupted techniques in selected cases, especially with ascending aortic replacement and concomitant aortic valve repair. Interrupted suture anastomoses precisely reconstruct the sinotubular geometry in dissections, and allow the proximal suture line to be placed close to the coronary arteries and aortic valve posts. With aneurysm extension into the aortic arch, interrupted suture techniques allow hemi-arch replacement without the need for aortic clamp removal and circulatory arrest. By 'sandwiching' all layers of the aortic wall between a Dacron graft on the inside and a Teflon strip on the outside, quite hemostatic anastomoses can be performed, even with poor-quality tissues. Any tearing of the aortic wall adjacent to individual sutures will not loosen the entire suture line, and can be repaired simply by extra suture placement. Thus, in selected situations, interrupted suture methods can be useful for ascending aortic replacement.     

Isolated interrupted aortic arch: in a 40-year-old adult

Interrupted aortic arch (IAA) is a scarce and generally lethal congenital malformation. Patients with complete IAA scarcely reach adult age without previous surgical intervention. In this case, we presented a 40-year-old isolated IAA case with hypertension and angina pectoris. IAA just distal to left subclavian artery and markedly developed collateral circulation was demonstrated via cardiac catheterization (CC) and multi-slice computed tomography angiography (MSCT).     

Predictors of left ventricular outflow obstruction following single-stage repair of interrupted aortic arch and ventricular septal defect

This study looked at echocardiographic predictors of left ventricular outflow obstruction after primary neonatal repair of interrupted aortic arch and ventricular septal defect. Results of this study indicate that the only significant independent predictor of left ventricular outflow obstruction is aortic valve diameter; all patients with an aortic valve diameter <4.5 mm (Z score <-5) subsequently developed obstruction, whereas patients with annuli >4.5 mm (Z score >-5) remained free from obstruction.     

Aortic valve replacement for aortic stenosis in persons aged 80 years and over

Seventy-one patients aged greater than or equal to 80 years (mean +/- standard deviation 82 +/- 2) with aortic stenosis or mixed stenosis and regurgitation underwent aortic valve replacement alone (n = 35, group 1) or in combination with a coronary artery bypass procedure without any other valve procedure (n = 36, group 2). Preoperatively, 91% had severe cardiac limitations (New York Heart Association class III or IV). Hospital mortality was 12.7% overall (9 of 71), 5.7% (2 of 35) for group 1 and 19.4% (7 of 36) for group 2. Perioperatively, 1 patient (1.4%) had a stroke. Survival from late cardiac death at 1 and 3 years was 98.2 and 95.5%, respectively, for all patients, 100% for patients who underwent isolated aortic valve replacement, and 96.3 and 91.2%, respectively, for patients who underwent aortic valve replacement plus coronary artery bypass. Eighty-three percent of surviving patients had marked symptomatic improvement. Freedom from all valve-related complications (thromboembolism, anticoagulant, endocarditis, reoperation or prosthetic failure) was 93.3 and 80.4% at 1 and 3 years, respectively. Thus, short- and long-term morbidity and mortality after aortic valve replacement for aortic stenosis in patients aged greater than or equal to 80 years are encouragingly low, although the addition of coronary artery bypass grafting increases short- and long-term mortality.     

Aortic root enlargement for aortic valve replacement in an achondroplastic dwarf

We present a case of aortic valve replacement combined with aortic root enlargement, performed on an achondroplastic dwarf with severe calcific aortic stenosis. There are no data about the incidence of valvular diseases in achondroplastic patients. To our knowledge, this is the 1st time that an aortic valve replacement associated with an aortic root enlarging procedure has been performed in this kind of patient. The aim of this report is to show that achondroplasia, in and of itself, is not a contraindication to aortic valve replacement.     

Aortic valve leaflet disruption techniques in transcatheter aortic valve replacement

With continued technological advancement and technical improvement of transcatheter aortic valve replacement (TAVR), it has become a desirable treatment option for aortic valve stenosis. Its minimally invasive approach compared to surgical aortic valve replacement offers the treatment to a broader patient population, mainly non-surgical candidates. A feared complication of TAVR is the occlusion of coronary artery ostium by the native aortic valve leaflet due to its displacement by the expanded transcatheter valve. Bioprosthetic or native aortic scallop intentional laceration to prevent iatrogenic coronary artery obstruction (BASILICA) is a technique developed to mitigate this risk by creating a lengthwise laceration of the left and/or right aortic valve leaflets prior to TAVR. Patient outcomes following TAVR with BASILICA have been promising. Meticulous preoperative examination, patient selection, and hemodynamic management are imperative. With continued refinement, BASILICA may further expand the application of TAVR to patients at high risk for coronary occlusion associated with the procedure.

Since the very first human transcatheter aortic valve replacement (TAVR) procedure in 2002, more than 200,000 TAVRs have been performed worldwide.^[1] Though it was reserved for select non-surgical candidates in the early stages of development, its application is expanding considerably in recent years. Large randomized controlled studies have compared outcomes of TAVR versus surgical aortic valve replacement (SAVR) for intermediate and high-risk patients and found no significant difference in primary outcome of morbidity from major stroke or mortality at one year and two years, respectively.^[2,3] Furthermore, a study of similar design involving low-risk patients found that TAVR patients had significantly lower rate of death, stroke, or rehospitalization at one year.^[4] Another evolving application of TAVR is to salvage a failing bioprosthetic valve, known as valve-in-valve (ViV) procedure.^[5] Minimally invasive aspect of TAVR confers a major advantage over SAVR, and the patient outcomes have been promising thus far. Despite its advantages, TAVR is associated with several notable risks. Firstly, without follow-up data from past six years, the durability of TAVR valves is uncertain. Therefore, current recommendations still favor SAVR for younger patients with longer life expectancy.^[6,7] However, one of the most consequential intraoperative complications of TAVR is the occlusion of coronary artery. With the placement of the prosthetic valve, an existing aortic valve leaflet may displace outward and directly occlude the adjacent coronary ostium or block the entire sinus of Valsalva, resulting clinically significant coronary obstruction. The left coronary ostium is much more frequently affected than the right coronary ostium, likely due to its lower height of the ostium in relation to the leaflet.^[8] Most cases of coronary obstruction manifest as severe hypotension immediately following the deployment of the prosthetic valve.^[8–10] The incidence is less than 1% in TAVR performed on native aortic valves, but it increases over 3-fold in ViV TAVR procedures.^[11,12] Majority of patients with TAVR-related coronary obstruction in the past have received emergent percutaneous coronary intervention and a much smaller number received coronary artery bypass grafting.^[8] Despite these measures, the 30-day mortality of coronary obstruction in patients undergoing ViV TAVR is reported to be 48.6%, which is likely much higher than in patients with coronary obstruction during native valve TAVR.^[8,11]Given the high rate of catastrophic outcomes associated with coronary obstruction, efforts must be devoted to preventive strategies. Several factors have been identified which include pre-existing surgical bioprosthetic aortic valve, older age, female gender, no history of coronary artery bypass grafting, and higher risk profile according to the logistic European System for Cardiac Operative Risk Evaluation (logEuroSCORE).^[10] The patient characteristics are likely significant due to the anatomic variability associated with them which include significantly smaller aortic annulus areas, sinus of Valsalva diameters, sino-tubular junction diameters, and lower left coronary artery heights as.^[10] In ViV patients, the prior surgical valve location, manipulation of the aortic root, and implantation of coronary grafts may affect these parameters considerably.^[12] With knowledge of these factors contributing to the risk of coronary obstruction, meticulous protocols are applied to stratify patient risk, as well as to plan the procedure appropriately.^[13,14]Bioprosthetic or native aortic scallop intentional laceration to prevent iatrogenic coronary artery obstruction (BASILICA) is a novel technique developed to create a lengthwise laceration down the middle of an aortic valve leaflet from the base to the tip.^[15] The goal of this technique is to prevent coronary obstruction by creating a separation in the existing valve leaflet through which the coronary artery can remain open and perfused when the leaflet is displaced outward by the transcatheter valve and the details have been previously described.^[14,15] Briefly, this is accomplished by traversing an electrified guidewire through the mid-base of the valve leaflet of interest. The guidewire is then captured by a ring snare pre-positioned at the aortic outflow tract through a separate catheter. Then the guidewire and snare are both manipulated to position the cutting segment of the electrified guidewire in contact with the valve leaflet, which creates the valve laceration with electrocautery (Figure 1).Open in a separate windowFigure 1Schematic illustration (A) and transesophageal echocardiography (B) of mid-esophageal long axis view showing the electrocautery apparatus (arrow) in place for BASILICA.BASILICA: bioprosthetic or native aortic scallop intentional laceration to prevent iatrogenic coronary artery obstruction.Initial testing of BASILICA in swine revealed successful laceration of the aortic valve leaflet without collateral injury on necropsy, except in the first trial while refining the technique.^[15] Clinical trial was subsequently performed and showed promising results.^[16] Of 30 patients, all subjects were free of coronary obstruction, despite being categorized as high risk based on preoperative evaluation.^[16] Due to the relatively small sample size, several questions remained unanswered regarding the safety of BASILICA, such as the risk of stroke and major vascular complications. However, many centers began to perform BASICILA over the recent years, including here at UC Davis Medical Center. As a result, the multicenter international BASILICA registry has accumulated data on 214 patients who underwent the procedure at 25 centers in North America and Europe.^[17] Analysis of the registry revealed that leaflet laceration was performed successfully in 94.4% of the patients and among those patients, 4.7% of patients experienced partial or complete coronary occlusion.^[17] The patients with coronary obstruction were successfully treated with coronary stents, but one patient died from cardiogenic shock despite the interventions. Considering the previously established mortality ranging between 40% and 50% in patients who experience coronary obstruction due to TAVR without BASILICA, the mortality of 10% in patients who experience coronary obstruction with BASILICA bolsters the benefits of this procedure.^[18] The authors’ explanation for this finding is that the obstruction resulting after BASILICA is not flow-limiting and the space created by BASILICA often allows placement of coronary stents through the struts of the transcatheter valve, instead of the traditional “snorkel” stent.^[17] Overall, the 30-day mortality and incidence of stroke reported through this registry were 2.8% and 2.8%, respectively.^[18] This is similar to the data of all patients undergoing TAVR reported by the Society of Thoracic Surgeons/American College of Cardiology Transcatheter Valve Therapy Registry.^[18] Considering that patients who underwent BASILICA are at higher risk than the average TAVR patients, this result supports the implementation of BASILICA for this subset of patients.BASILICA requires meticulous planning and certain patient factors make the procedure less feasible. A large portion of the failed BASILICA were attributed to failure to traverse the electrified guidewire through the leaflet due to the extensive calcification. Hence, it may be a relative contraindication for the procedure. Anesthetic planning is also an important component in taking care of these hemodynamically fragile TAVR patients. This is highlighted by the authors of the BASILICA registry analysis, who attributed one of the deaths to cardiogenic shock resulting from the anesthesia induction.^[18] Although moderate sedation may be considered, there is a strong preference for general anesthesia (GA) with endotracheal intubation. In case of severe hemodynamic compromise that requires emergent surgical intervention or mechanical circulatory support, GA with a secure airway is necessary. Furthermore, transesophageal echocardiogram (TEE) can be performed much more safely with a secured airway with an endotracheal tube. Particularly in this patient population, critical intraoperative complications, such as wall motion abnormalities can be detected early using TEE to direct clinical management.

Aortic valve replacement for aortic stenosis in nonagenarians

We reviewed certain clinical and morphologic findings in 9 patients who had aortic valve replacement (AVR) for aortic stenosis (AS) when >or=90 years of age. All had AVR from February 2000 to April 2006. The aortic valve areas ranged from 0.41 to 1.00 cm2, and the transvalvular peak systolic gradients ranged from 20 to 110 mm Hg. The left ventricular ejection fractions were >or=50% in 6 of the 9 patients. The aortic valve was congenitally bicuspid in 3 patients, and the operatively excised valves in them weighed 4.20, 5.73, and 9.75 g, respectively (mean 6.56). The other 6 patients had 3-cuspid valves without commissural fusion, and the operatively excised valves in them weighed 0.43, 0.94, 1.08, 1.51, 1.98, and 4.43 g, respectively (median 1.30, mean 1.73). Coronary artery bypass grafting (CABG) was performed at the time of AVR in 8 of the patients. One patient died a day postoperatively and 2 others died 874 and 1,011 days, respectively, after operation. Two were in skilled nursing units postoperatively for several weeks. In conclusion, AS can be severe in nonagenarians and may be superimposed on a congenitally bicuspid aortic valve.     

Surgical repair of a pseudoaneurysm of the ascending aorta after aortic valve replacement

We report the case of a patient with a pseudoaneurysm of the ascending aortic clinically diagnosed 5 months after surgical replacement of the aortic valve. Diagnosis was confirmed with the aid of two-dimensional echocardiography and helicoidal angiotomography. The corrective surgery, which consisted of a reinforced suture of the communication with the ascending aorta after opening and aspiration of the cavity of the pseudoaneurysm, was successfully performed through a complete sternotomy using extracorporeal circulation, femorofemoral cannulation, and moderate hypothermia, with no aortic clamping.     

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.     

Aortic valve repair for congenital abnormalities of the aortic valve

BACKGROUND: Due to shortcomings of any valve replacement, repairing and retaining the native valve may be beneficial for congenital aortic valve disease. METHODS: Retrospective review of data and follow-up of aortic valve repair from a single institution. RESULTS: From 1993 to 2001, 56 patients underwent aortic valve repair [median age 13.4 years (range 1 day to 45 years)]. The predominant aortic valve lesion was mixed aortic stenosis/aortic insufficiency 25 (45%), aortic insufficiency 24 (43%) and aortic stenosis 7 (13%). Repair techniques included sub-commissural plication 36 (64%), commissurotomy 24 (43%), cusp plication 15 (27%), pericardial patch cusp extension 8 (14%) and resuspension of commissures 4 (7%). Most patients (88%) required a combination of techniques; 61% required additional procedures. Hospital survival was 55/56 [98%; (95% CI 91-100%)] no patient was discharged on anticoagulation for aortic valve pathology. Fifty-three patients [95%; (95% CI 85-98%)] remain alive after a median follow-up of 37 months; four survivors required aortic valve replacement and two required repeat aortic valve repair [84%; (95% CI 72-91%) reintervention-free survival]. CONCLUSIONS: (1) In this study, aortic valve repair for congenital abnormalities avoided reoperation in the majority of patients, avoided anticoagulation and retained growth potential of the valve. (2) Repeat aortic valve repair or replacement was used to treat subsequent valve deterioration.     

Pseudoaneurysm of the ascending thoracic aorta years after aortic valve replacement

Aortic pseudoaneurysm (PSA) formation post aortic valve replacement is one of the rare and devastating complications if left untreated. Aortic PSA can occur few weeks or month after surgery. The spectrum of symptoms varies from dyspnea, wide pulse pressure, and chest pain. However, in rare cases, aortic root PSA could be asymptomatic. Aortic PSA mortality could be as high as 70%, if left untreated. Therefore, High clinical suspicion and prompt surgical intervention is imperative. In this case, the patient presented with dyspnea years after Bentall procedure, and imaging studies revealed para-aortic PSA that was treated surgically.