Surgical therapy of thoracic aortic dissection]

Considerable surgical progress of treating aortic dissection has been achieved during the past decade. The emergency indication for acute dissection of the ascending aorta (type A according to the Stanford classification) is unquestioned while surgical treatment for acute dissection of the descending aorta (type B dissection) is mainly reserved for complicated cases. The major complication of acute operations--fatal hemorrhage from the suture line and secondary multi-organ failure--have been successfully reduced by a progress of cardiopulmonary bypass techniques, the introduction of cold cardioplegic myocardial protection, the development of modern suture materials and glues and last not least by a continuous intensive monitoring. Especially the introduction of the so-called french glue safely enabled both the closure of the false lumen as well as the strong reinforcement of the diseased aortic wall and seems to offer a reliable alternative to the application of multi-layered teflon strips. Since the principle of all reconstructive approaches in case of dissection consists of closure of dissected layers and the limited replacement of the segment that is susceptible to a rupture the exact readaptation and reinforcement of the diseased aortic wall represents a fundamental operative step. In type A operations the supracoronary aortic prosthetic replacement or the combined replacement of ascending aorta plus aortic valve followed by the reattachment of coronary arteries has become the standard operative technique. In fact, independently from the location of the primary intimal tear the operation has been traditionally limited to replace the ascending aorta in order to remove an aortic segment that is most likely to rupture. Yet an increasing number of follow-up investigations has demonstrated recurrence of dissection or an aneurysmatical dilatation of the false lumen in about 20% of patients treated with ascending aortic replacement. Consequently, repair of the aortic transverse arch and the radical elimination of the intimal entry is now favoured by an increasing number of surgeons. In addition to these various perioperative and intraoperative adjuncts the introduction of new imaging techniques, especially computerized tomography, magnetic resonance imaging and transesophageal echocardiography allowed to establish adequate therapeutical concepts on a more rational basis. Transesophageal echocardiography as a mobile diagnostic device enables investigators to perform a bed-side dynamic visualization of both the location and extent of a dissection, the evaluation of ventricular performance and aortic competence. Treatment of acute type B dissection is mainly conservative unless complications like intractable pain, aneurysmatic enlargement of the false lumen, ischemia of visceral organs or even rupture occur.(ABSTRACT TRUNCATED AT 400 WORDS)     

New technique for single-staged repair of aortic coarctation and coexisting cardiac disorder

The management of adults with aortic coarctation and a coexisting cardiac disorder is still a surgical challenge. Single-staged procedures have lower postoperative morbidity and mortality rates than do 2-staged procedures. We present our experience with arch-to-descending aorta bypass grafting in combination with intracardiac or ascending aortic aneurysm repair.From October 2004 through April 2010, 5 patients (4 men, 1 woman; mean age, 45.8 ± 9.4 yr) underwent anatomic bypass grafting of the arch to the descending aorta through a median sternotomy and concomitant repair of an intracardiac disorder or an ascending aortic aneurysm. Operative indications included coarctation of the aorta in all cases, together with severe mitral insufficiency arising from damaged chordae tendineae in 2 patients, ascending aortic aneurysm with aortic regurgitation in 2 patients, and coronary artery disease in 1 patient. Data from early and midterm follow-up were reviewed.There was no early or late death. Follow-up was complete for all patients, and the mean follow-up period was 34.8 ± 18 months (range, 18 mo-5 yr). All grafts were patent. No late graft-related sequelae or reoperations were observed.For single-staged repair of aortic coarctation with a coexistent cardiac disorder, we propose arch-to-descending aorta bypass through a median sternotomy as an alternative for selected patients.     

Management of intramural hematoma of the ascending aorta and aortic arch: the risks of limited surgery

We present the case of a 57-year-old woman who had an intramural hematoma of the ascending aorta and aortic arch. After initial blood pressure control and imaging studies, the patient underwent limited surgical repair that consisted of ascending aortic replacement. One week postoperatively, the aortic arch hematoma progressed to a full dissection that extended into the proximal descending aorta. Emergent aortic arch replacement was required. Current world medical literature regarding thoracic aortic intramural hematoma is presented. This case supports the treatment of intramural hematomas of the ascending aorta and arch by surgical replacement of both segments with a Dacron graft, with the patient under deep hypothermic circulatory arrest.     

A giant post-dissection aneurysm of the ascending aorta in an octogenarian

Aneurysm formation is a well-known complication of untreated long-standing dissection of the aorta. Despite numerous advances in cardiac surgery, intrinsic diseases of the ascending aorta and aortic arch requiring surgical therapy, remain a technical challenge. However, surgery is the only option for effective treatment of ascending aorta aneurysm but carries an increased risk of severe morbidity and mortality, particularly in the elderly. We report on the successful repair of a giant ascending aorta aneurysm due to chronic dissection in an elderly woman with dyspnoea as main symptom.     

Hybrid treatment for complex aortic problems combining surgery and stenting in the integrated operating theater

OBJECTIVES: Conventional surgical treatment of complex aortic pathologies involving several thoracoabdominal aortic segments necessitates extended incisions or subsequent surgeries, resulting in significant mortality and morbidity rates. The combination of surgery and simultaneous stenting in the operating theater may reduce the surgical trauma. METHODS: A total of nine patients (62 +/- 10 years, range 44-70) underwent a combined surgical and endovascular treatment of thoracic or thoracoabdominal aortic aneurysms or chronic dissection. Five patients were treated with viscero-renal artery translocation followed by transfemoral stenting of the entire thoracoabdominal aorta. Two patients underwent debranching of the supraaortic vessels followed by immediate transfemoral stenting of the aortic arch, and two patients with a history of an ascending aortic aneurysm repair were treated with open surgical debranching of the supraaortic trunks and repair of the ascending aorta and aortic arch with elephant trunk technique. Preoperatively, magnetic resonance imaging was used to check supraaortic and intracranial vessels as well as the completeness of the Circle of Willisi prior to arch stenting and/or supraaortic vessel surgery. Cerebrospinal fluid drainage and induced mild hypertension have been used for one-step thoracoabdominal aortic stenting. RESULTS: Thirty-day mortality rate and incidence of paraplegia was 0%. There was a single reversible perioperative stroke after aortic arch stenting. One patient required temporary renal replacement therapy using continuous arterio-venous hemofiltration. There was one early reoperation at the superior mesenteric artery after viscero-renal translocation. Four type I endoleaks occurred in three patients requiring two interventions. All patients have been discharged to home. CONCLUSION: The innovative combination of simultaneous conventional surgery and stenting reduces the operative burden for patients with complex aortic pathologies involving several segments of the thoracic and thoracoabdominal aorta. Arch debranching and viscero-renal artery translocation may avoid the use of thoracoabdominal incisions, cardiopulmonary bypass techniques, deep hypothermia, and circulatory arrest.     

Ascending thoracic aorta dimension and outcomes in acute type B dissection (from the International Registry of Acute Aortic Dissection [IRAD])

It is not well known if the size of the ascending thoracic aorta at presentation predicts features of presentation, management, and outcomes in patients with acute type B aortic dissection. The International Registry of Acute Aortic Dissection (IRAD) database was queried for all patients with acute type B dissection who had documentation of ascending thoracic aortic size at time of presentation. Patients were categorized according to ascending thoracic aortic diameters ≤4.0, 4.1 to 4.5, and ≥4.6 cm. Four hundred eighteen patients met inclusion criteria; 291 patients (69.6%) were men with a mean age of 63.2 ± 13.5 years. Ascending thoracic aortic diameter ≤4.0 cm was noted in 250 patients (59.8%), 4.1 to 4.5 cm in 105 patients (25.1%), and ≥4.6 cm in 63 patients (15.1%). Patients with an ascending thoracic aortic diameter ≥4.6 cm were more likely to be men (p = 0.01) and have Marfan syndrome (p <0.001) and known bicuspid aortic valve disease (p = 0.003). In patients with an ascending thoracic aorta ≥4.1 cm, there was an increased incidence of surgical intervention (p = 0.013). In those with an ascending thoracic aorta ≥4.6 cm, the root, ascending aorta, arch, and aortic valve were more often involved in surgical repair. Patients with an ascending thoracic aorta ≤4.0 were more likely to have endovascular therapy than those with larger ascending thoracic aortas (p = 0.009). There was no difference in overall mortality or cause of death. In conclusion, ascending thoracic aortic enlargement in patients with acute type B aortic dissection is common. Although its presence does not appear to predict an increased risk of mortality, it is associated with more frequent open surgical intervention that often involves replacement of the proximal aorta. Those with smaller proximal aortas are more likely to receive endovascular therapy.     

Assessment of perfusion toward the aortic valve using the new dispersion aortic cannula during coronary artery bypass surgery

When there is an echocardiographic diagnosis of severe mobile atherosclerotic plaque in the aortic arch or descending aorta, perfusion toward the aortic arch during cardiopulmonary bypass may create a high risk of embolic neurologic injury. Other perfusion methods, such as cannulation of the femoral or axillary arteries, are not always possible, due to atherosclerosis. The ascending aorta may be an alternative site for perfusion, since it is less frequently diseased. We assessed a new technique of perfusion toward the aortic valve using a new cannula designed for this purpose (Dispersion aortic cannula). Our study included 100 consecutive patients, 72 men and 28 women, with an average age of 68 +/- 1.0 years (range, 39-89 years). There were no complications related to insertion of the cannula or perfusion. The ascending aorta could be cross-clamped and side-clamped without perfusion problems. Three deaths occurred; none was related to the cannulation technique. No intra-operative stroke occurred. Two patients suffered neurologic events, one on day 1 and the other on day 6; both had been fully alert after surgery. Perfusion toward the aortic valve appears to be safe and hemodynamically effective. This cannulation technique appears to be an acceptable alternative to present methods. Comparative studies will be needed to determine whether this alternative technique is effective in patients with severe aortic arch disease.     

Hybrid endovascular and off-pump open surgical treatment for synchronous aneurysms of the aortic arch, brachiocephalictrunk, and abdominal aorta

A 71-year-old patient was admitted for synchronous aneurysms of the aortic arch, brachiocephalic trunk, and juxtarenal abdominal aorta involving the iliac arteries. The patient first underwent open surgical repair of the juxtarenal abdominal aortic aneurysm by means of aorto-bifemoral bypass. Three months later, he underwent off-pump surgical repair of the aneurysm of the brachiocephalic trunk and bypass grafting from the ascending aorta to the brachiocephalic trunk and the left common carotid artery, followed by successful exclusion of the aneurysm of the aortic arch by deployment of a Zenith TX1 custom-made endograft, inserted through a limb of the aorto-bifemoral graft. Combined endovascular and open surgical treatment is an appealing new alternative to open surgical repair for complex aortic diseases. Debranching of the aortic arch enables endovascular grafting in this area, thereby avoiding cardiopulmonary bypass and circulatory arrest. Staged and simultaneous procedures should be considered for the treatment of complex aortic diseases even in poor-risk patients; however due to the investigative characteristics of these procedures, patient selection and postoperative follow-up should be carried out with utmost attention.     

Coarctation and related aortic arch anomalies in hypoplastic left heart syndrome

The aortic arches of 34 specimens with hypoplastic left heart syndrome were studied in order to establish the frequency, the nature and the clinical implications of aortic arch anomalies. A localized aortic coarctation was present in 23 specimens. The coarcation was located preductally in 2 and paraductally in 21 cases. The degree of obstruction caused by the coarctation varied considerably. In only 6 cases (1 preductal and 5 paraductal) was the obstruction judged to be severe. One of these cases had an additional aneurysm of the aortic wall proximal to the coarctation. An aberrant relation of the ductus arteriosus and the aortic arch was found in 2 specimens without localized coarctation. In the remaining 9 specimens the aortic arch appeared normal. The aortic arch anomalies were mostly present in specimens with a severely hypoplastic ascending aorta. Clinicians, when preparing infants with hypoplastic left heart syndrome for surgical palliation, should always suspect associated aortic arch anomalies, especially when there is severe hypoplasia of the ascending aorta. Coarctation of the aorta will require additional surgical treatment.     

Surgical strategies for severely atherosclerotic (porcelain) aorta during coronary artery bypass grafting

Porcelain aorta (PA) is an asymptomatic atherosclerotic disease, characterized by circumferential calcification throughout the whole perimeter of the aorta. It is seen in 2% to 9.3% of patients undergoing elective coronary artery bypass grafting (CABG) and makes manipulation of the ascending aorta impossible. It has been clearly shown that most emboli seen and detected during the CABG procedure occur during aortic cross-clamping and aortic side-clamping. Manipulation of porcelain or a severely atherosclerotic aorta increases the risk of perioperative stroke. The incidence of stroke after CABG is between 0.48% and 2.9%, and the risk is correlated with the extent and severity of the atherosclerotic disease. A conventional CABG procedure involves successive steps that include cannulation of the ascending aorta, application of a cross-clamp to the aorta, and partial clamping of the aorta to create the proximal anastomosis. Therefore in procedures that involve cannulation, clamping, or proximal anastomosis, and where aortic manipulation is inevitable, preassessment of the atherosclerotic aortic plaques is crucial. Although many surgeons still rely on intraoperative manual aortic palpation, this approach has very low sensitivity and underestimates the severity of the atherosclerotic illness. Imaging methods including preoperative computed tomography or intraoperative epiaortic ultrasonography enable modification of the surgical technique according to the severity of atherosclerosis. Various surgical techniques have been described to reduce the risk of atheroembolism that may lead to cerebrovascular events in patients with severely atherosclerotic ascending aorta. Anaortic or “no-touch” techniques that do not utilize aortic manipulation may significantly decrease the development of neurological complications by avoiding aortic maneuvers known to cause emboli. In cases where severe atherosclerotic disease or other factors preclude safe use of the ascending aorta, modifications in the surgical techniques, such as switching to different cannulation sites including the axillary/subclavian, femoral and innominate arteries, or using hypothermic ventricular fibrillation and in-situ pedicled arterial grafts, or performing proximal anastomoses at alternative anatomical locations will enable CABG operations to be performed safely with low morbidity and mortality rates in patients with porcelain aortas.     

Paradigmenwechsel in der Chirurgie der komplexen thorakalen Aortenerkrankung

One of the main issues in complex thoracic aortic disease, requiring the replacement of the ascending aorta, the entire aortic arch and the descending aorta, is the vast amount of surgery necessary to cure the patient. Though one-stage repair is feasible by a clamshell thoracotomy, the associated surgical trauma and perioperative morbidity limit this approach to younger patients only. Classic surgical repair consist of a two-stage strategy, whereby, in the first step, the ascending aorta and the aortic arch are replaced via a midline sternotomy. In the second step, via a lateral thoracotomy, the descending aorta is replaced. The two stages may sum up to a mortality of 20%; furthermore, the waiting period between the stages is associated with a mortality rate of 10% of its own. Additionally, the two-stage strategy has an inherent limitation, due to the comorbidity and advanced age of the majority of patients. Therefore, the second stage cannot be offered to up to 30% of patients. New developments and improvements in aortic surgery were introduced to overcome these shortcomings and to simplify the surgical repair. The "elephant trunk" principle, introduced by Borst et al. in 1983, was an important step to facilitate surgical repair, but still required the second step. With the introduction of endovascular repair of thoracic aortic disease with stent grafts implanted retrograde via the femoral artery, new therapeutic concepts emerged. In the late 1990s, two Japanese groups reported first trials to stabilize the free-floating "elephant trunk" prosthesis by implantation of nitinol stent grafts into the vascular graft. The applied devices were purely custom-made and nonstandardized. The availability of industrially made and CE-marked stent-graft devices raised the possibility to apply them in open aortic arch surgery. The experience with stent-graft devices implanted antegrade into the descending aorta (Medtronic Talent) was reported first by the Essen and the Vienna group. The experience gained with these devices revealed the limitations of the devices designed for pure retrograde aortic delivery. This required a complete redesign and new construction of the stent graft itself as well as the introducer system. In a preliminary series of 14 patients the required stent-graft properties were presented in detail and resulted in the first industrially manufactured standardized and CE-marked Hybrid stent graft (Essen 1 prosthesis, E-vita Open, Jotec), especially made for antegrade open stent grafting of the descending aorta. This device consists of a stent graft with an integrated Dacron vascular prosthesis, enabling for direct and continuous aortic arch replacement after stent grafting of the descending aorta. From 01/2005 to 03/2006, this hybrid prosthesis was implanted in 16 patients (one aneurysm and 15 aortic dissections). In all cases, the underlying pathology within the thoracic aspect of the aorta could be excluded in a one-stage approach. In case of aortic dissection, thrombosis of the false lumen was detectable by transesophageal echocardiography already at the end of surgery. Though long-term results using this new method are not yet available, the initial promising results postoperatively are encouraging toward true one-stage repair by combining classic aortic surgery with open antegrade stent grafting utilizing the newly designed hybrid prosthesis. While surgical trauma is markedly reduced, this treatment option can be offered to elderly patients as well.     

Infectious aneurysm of the ascending aorta – Successful conservative treatment in a high-risk patient

Infectious aortic aneurysm is a rare disease requiring early and comprehensive management to prevent the development of serious complications. While surgical repair is still the gold standard, an endovascular approach is an alternative for some patients. Conservative management is traditionally associated with the worst prognosis. We report a case of an infectious aneurysm of the ascending aorta with an atypical clinical manifestation and a complicated diagnostic process, which resulted in successful comprehensive conservative management of a high-risk patient who rejected radical surgical treatment.     

Segmental aortic hypoplasia or how to interpret the flow concept

The development of elastin lamellae in the media of elastic type arteries such as the aorta appears to be modulated by mechanical factors such as flow. On that basis, decreased aortic flow during fetal development is held responsible for the occurrence of tubular hypoplasia of the aortic arch. Underdevelopment of the ascending aorta in aortic atresia likewise is considered due to diminished retrograde flow from the aortic arch. The present study has examined this hypothesis by comparing the number of elastic lamellae in hypoplastic segments of the aortic arch and ascending aorta with corresponding segments from normal aorta. In both situations the underdeveloped segments show a paucity of cellular and supportive connective tissue constituents, leading to a densely packed layer of elastic lamellae and, thus, contributing to the diminished dimensions. A major difference, however, was noted with respect to the number of elastic lamellae. In the hypoplastic ascending aorta in aortic atresia, the number was not different from that encountered in normal specimens. In tubular hypoplasia, on the other hand, the number of elastic lamellae was significantly lower than in the corresponding segments of normal specimens (P less than 0.01). Taking the stance that flow is a major factor in proper development of the aortic media, it seems that the findings in tubular hypoplasia fit well with a chronic lack of tension from early development and, hence, relate directly to the cardiac malformation. The findings in the ascending aorta of aortic atresia are less easy to understand from this point of view.(ABSTRACT TRUNCATED AT 250 WORDS)     

Endovascular Therapy for Thoracic Aortic Aneurysms: State of the Art in 2012

Conventional surgery for thoracic aortic pathology involves replacing the affected segment of aorta with an interposition graft and often requires the use of extracorporeal circulatory support with or without deep hypothermic circulatory arrest. Although operative results have improved consistently over 60 years, patients with extensive aneurysms face a considerable risk with conventional surgery, particularly when burdened with multiple comorbidities. Thoracic endovascular aortic repair (TEVAR) was first performed in 1994 and has become a well-established alternative therapy for many thoracic aortic pathologies. TEVAR is most frequently performed through a small groin incision to access the common femoral artery. Wires and catheters are used to deliver and deploy the stent graft in the thoracic aorta under fluoroscopic control. Occasionally, TEVAR is performed as part of a complex hybrid procedure including one stage of conventional open surgery that may utilize a thoracic incision and cardiopulmonary bypass support. The less invasive nature of TEVAR offers the potential for lower mortality and peri-procedural morbidity. Although long-term results of TEVAR are still being gathered, mid-term results are excellent and most late vascular complications can be treated with additional transcatheter procedures. Recent development of fenestrated and branched stent grafts is expanding the application of endovascular therapies to complex aortic pathologies involving the thoracoabdominal aorta and aortic arch. Although conventional techniques continue to be the gold standard for treatment of ascending aortic pathology, recent reports have proven TEVAR to be a viable alternative in specific situations. Design improvements continue to expand the indications for TEVAR, and technological advancements in the field of imaging facilitate safer and more accurate planning, delivery, and assessment of patients with thoracic aortic aneurysms. Hybrid operating rooms provide the optimal environment with state of the art imaging technology for the cardiovascular team to perform TEVAR or alternative hybrid procedures.     

Modified bypass procedure and apicoaortic conduit. Management of coronary artery disease,aortic valve stenosis and porcelain aorta

BACKGROUND: In case of severely calcified ascending aorta, modified operative strategies are required in order to avoid manipulations of the aorta and minimize subsequent cerebral vascular accidents. CASE REPORT: A 73-year-old woman, with a coronary two-vessel disease and aortic stenosis was scheduled for coronary artery bypass grafting and aortic valve replacement. Due to severed calcification of the ascending aorta including the transverse arch, neither cannulation, clamping nor incision of the aorta or its replacement was feasible. Therefore bypass operation was performed using a modified approach. After 1 month, implantation of a valved conduit between the left ventricular apex and the descending aorta through a lateral thoracotomy followed. CONCLUSION: Only in few cases the surgical treatment of a coronary artery disease in combination with left ventricular outflow tract obstruction and heavily calcified ascending aorta has been described. Undoubtedly, creation of an apicoaortic connection is today only indicated in the adult population in a small collective with multiple previous operations or porcelain aorta.     

Hybridraumtechnologie als Voraussetzung f��r die moderne Therapie der Aortendissektion

With the establishment of a hybrid room 7 years ago, it was possible for the first time to unite a full range of diagnostics and surgical therapy under the sterile conditions of an operating theatre in life-threatening aortic dissection. Thus, the early phase associated with high mortality rates (3%-5% per hour) could be significantly reduced from 8 h to 4 h. Multidisciplinary teams consisting of a cardiac surgeon, a cardiologist and an anaesthetist enable competent and rapid life-saving measures. In the case of acute and persistent visceral and/or peripheral malperfusion over many hours, primary endovascular reconstitution of perfusion precedes delayed surgical replacement of the ascending aorta with or without the aortic arch. Additional strategic and technical surgical developments have helped reduce overall hospital mortality from 15%-20% to 10%-15%. Though expensive to build, a high-technology hybrid room enables interdisciplinary specialization and concentration, as demonstrated by the exponential growth in the development of transcatheter aortic valve implants or the endovascular treatment of aortic disease.     

Double Aortic Arch with Aortic Atresia and Left-Sided Type B Interruption

Aortic valve atresia with interruption of the aortic arch is an extremely rare anomaly; only eleven cases of this anomaly have been reported to date. In the absence of additional sources of blood flow to the ascending aorta, aortic valve atresia with interruption of the aortic arch is fatal. We present, to the best of our knowledge, the first case of a live birth with aortic valve atresia and interrupted left aortic arch (type B) without evidence of an aorticopulmonary communication or ductal supply to the native ascending aorta. Instead, blood flow to the native aortic root was derived from a persistent right embryonic dorsal aorta.     

Stentversorgung der thorakalen Aorta

Considering the demographic changes in our society and the proliferation of imaging-based improved diagnostics, both acute and chronic aortic diseases attract increasing attention and require dedicated care. Cardiac as well as vascular surgery used to represent the gold standards for therapeutic management of pathologies of the ascending aorta and the arch; however, the technological evolution of endoluminal strategies has had a serious impact on the treatment of the descending aorta, the aortic arch in combination with vascular debranching or bypass, and in selected cases even on managing pathologies of the ascending aorta. Although several case series and meta-analyses of published observations hint towards superiority of endografting in comparison to open surgical repair, the affected usually multimorbid patients with highly complex aortic disease should be subjected to an individual evaluation by a team of cardiologists, cardiac and vascular surgeons as well as imaging specialists; a dedicated individualized treatment concept in highly experienced centers of excellence is likely to provide the best results for such challenging patients.     

Surgical correction of ascending type a thoracic aortic dissection: simultaneous endoluminal exclusion of the arch and distal aorta.

PURPOSE: To describe repair of an ascending type A dissection combining an open ascending tube graft with simultaneous great vessel transposition and antegrade deployment of an endoluminal graft across the arch and into the descending thoracic aorta. CASE REPORT: A 50-year-old man was evaluated at an outside hospital and transferred to our service for treatment of an ascending aortic dissection with associated lower extremity ischemia. Imaging identified an aortic dissection extending from the aortic root to the aortic bifurcation and into the right common iliac artery. A hybrid procedure incorporating both open and endovascular techniques successfully repaired the dissection and aneurysm and restored blood flow to the extremity. CONCLUSIONS: Although less invasive procedures are sometimes appropriate for repair in the descending thoracic aorta, surgical correction of an ascending dissection and endoluminal exclusion of the arch and distal aorta may form the basis of future treatment strategies for complex aortic pathologies, possibly eliminating the need for hypothermic cardiac arrest.     

Anomalous origin of the left pulmonary artery from the ascending aorta in two children with pulmonary atresia, sub-aortic ventricular septal defect and right-sided major aorto-pulmonary collateral arteries

We report two rare cases of an anomalous origin of the left pulmonary artery (AOLPA) from the ascending aorta, associated with pulmonary atresia, a ventricular septal defect and a left aortic arch. The cases are unusual because AOLPA is more commonly associated with a right aortic arch and it is more usual for the right pulmonary artery to originate anomalously from the ascending aorta. The pulmonary blood supply to the right lung in both patients was absent and provided instead by major aorto-pulmonary collateral arteries which were stenosed at multiple levels. The AOLPA in both patients originated from the postero-lateral aspect of the ascending aorta just distal to the sino-tubular junction. Only one patient showed the more common association of an unusual aortic arch branching pattern in the form of an anomalous right subclavian artery. Neither patient was in heart failure and the chest X-ray in both revealed differential pulmonary perfusion with prominent vascularity of the left lung. Cardiac catheterisation showed systemic pressures within the anomalous left pulmonary artery. Karyotyping revealed normal chromosomes, and fluorescent in-situ hybridisation done in one patient was negative for chromosome 22q11.2 microdeletion. Both patients have been managed conservatively.