Truncus Arteriosus With Patent Ductus Arteriosus and Normal Aortic Arch

In hearts with a common arterial trunk (truncus arteriosus), there is almost always an inverse development of the aortic arch and the ductus arteriosus. Truncus with a normal aortic arch and a patent ductus is a rare echocardiographic and surgical finding. In this report, we describe 2 neonates in whom truncus arteriosus with a normal aortic arch and a medium or large patent ductus was diagnosed by preoperative echocardiography (without catheterization) and confirmed intraoperatively.     

Repair of persistent truncus arteriosus with interrupted aortic arch.

OBJECTIVE: The aim of our study was to analyse experience with repair of truncus arteriosus with interrupted aortic arch. METHODS: Between 1993 and 2004, eight consecutive patients underwent repair of truncus arteriosus with interrupted aortic arch. The median age was 6.5 days (range 1-85 days) and median weight was 3.2 kg (range 2.6-4.8 kg). Five patients had type A and 3 patients had type B aortic arch interruption. The repair was performed in deep hypothermia with circulatory arrest in 4 patients and isolated selective low-flow perfusion of the head and the heart in the last 4 patients. The repair consisted in aortic arch reconstruction by direct anastomosis between descending and ascending aorta, closure of ventricular septal defect and reconstruction of the right ventricular to pulmonary artery continuity using a valved conduit. RESULTS: One (12.5%) patient died from sepsis and hepato-renal failure 18 days after surgery. Seven (87.5%) patients were followed up for 2.0-11.7 years (median 2.6 years). No patient died after the discharge from hospital. In 4 patients 1-3 reinterventions were required 0.6-10.0 years after repair. Reoperations were performed for conduit obstruction in 2 patients, aortic regurgitation in 2 patients, right pulmonary artery stenosis in 2 patients and airway obstruction in 1 patient. In 2 patients concommitant aortic valve and conduit replacement was required. Balloon angioplasty for aortic arch obstruction was necessary in 1 patient, and for bilateral pulmonary branch stenosis in 1 patient. Five (28.6%) surviving patients are in NYHA class I and 2 (71.4%) patients are in NYHA class II. CONCLUSIONS: Primary repair of persistent truncus arteriosus with interrupted aortic arch can be done with low mortality and good mid-term results. Aortic arch reconstruction in isolated low-flow perfusion of the head and the heart influences favourably the postoperative recovery. The main postoperative problems are associated with conduit obstruction and aortic insufficiency.     

Truncus arteriosus with interrupted aortic arch: successful repair using modified cardiopulmonary bypass and surgical techniques

A neonate with truncus arteriosus (type 1) and interrupted aortic arch (type A) associated with an excessively large gap between interrupted aortic segments underwent successful reconstruction of the aortic arch with a partial subclavian flap aortoplasty plus bovine jugular patch (Contegra 200, Medtronic, Inc, Minneapolis, MN) using continuous selective low-flow cardiopulmonary bypass without circulatory arrest. The ventricular septal defect was closed with a Dacron (IMPRA, Inc, Tempe, AZ) patch, and a Contegra prosthesis was used to re-establish right ventricle to pulmonary artery continuity. At an 18-month follow-up, the patient was free of symptoms with normal development. Echocardiography revealed a widely patent aortic arch and an unobstructed right ventricle to pulmonary artery connection.     

Right aortic arch and isolated left innominate artery from a left sided patent ductus arteriosus: a very rare aortic arch anomaly

Malformation of the aortic arch system has been described in details by Stewart et al. in 1964. Innominate artery originating via the ductus arteriosus from the pulmonary artery is a very rare type of congenital aortic arch anomaly that has been seldomly reported. We report the case of an aortic arch anomaly revealed by a pulmonary hypertension because of left to right shunt. Surgical procedure was performed through a median sternotomy, without cardiopulmonary bypass. After section. of the ductus arteriosus, the left innominate artery was extensively dissected and mobilized to be implanted on the left side of the ascending aorta under lateral clamping.     

Successful surgical repair of truncus arteriosus with interrupted aortic arch in infancy by an anterior approach

A 98-day-old infant was successfully operated on for truncus arteriosus (type I) with interrupted aortic arch (type B) using a one-stage anterior approach. The interrupted aortic arch was reconstructed by direct anastomosis between the ascending and descending aorta. The ventricular septal defect was closed, and a 12-mm porcine valved conduit was placed on the right side of the ascending aorta to establish continuity between the right ventricle and pulmonary artery. Surgical procedures for the repair of this malformation are the focus of discussion.     

Left aortic arch with right descending aorta and right ligamentum arteriosum. A rare form of vascular ring.

The combination of left aortic arch, right descending aorta, and right patent ductus arteriosus is a rare form of aortic arch anomaly. Only 11 such cases have been previously reported. A 6-month-old infant with this anomaly manifested severe respiratory difficulty and feeding problems in the early newborn period. The aortic arch abnormalities were confirmed by angiocardiography, and operative correction was successful. This is the first case reported in which the patient developed severe respiratory and feeding difficulties due to this type of a vascular ring.     

Endograft and external cinch to control hemorrhage in acute type A aortic dissection

Acute DeBakey I or II aortic dissection (AD) is a surgical emergency with significant mortality if not repaired immediately. We present the case of a 49-year-old man with acute type I AD, who initially underwent zone 2 arch replacement for a primary arch tear. A calcified ductus arteriosus was noted during arch reconstruction. He exhibited exsanguinating hemorrhage from the proximal descending thoracic aorta upon an initial attempt to wean off cardiopulmonary bypass. Hemostasis was achieved with retrograde transfemoral thoracic endovascular aortic repair and transmediastinal external cinch around the descending aorta to obliterate false lumen flow.     

Successful palliation using partial cardiopulmonary bypass in a two-day-old infant with type B interruption of the aortic arch.

Partial cardiopulmonary bypass from the pulmonary artery to the descending aorta was used successfully for resuscitation and aortic arch reconstruction in a 2-day-old infant with type B interrupted aortic arch. Partial cardiopulmonary bypass interrupted the fatal progression to acidosis and death caused by hypoperfusion of structures distal to the ductus arteriosus in this "ductus dependent" infant, and this approach provided time for an accurate dissection and anastomosis. The natural history, pathophysiology, and currently available surgical alternatives are considered in suggesting a possible role for partial bypass in the treatment interrupted aortic arch.     

Aneurysm of the ductus diverticulum in adults The diagnostic value of three-dimensional computed tomographic scanning

Ductus arteriosus aneurysm is rare in adults and preoperative diagnosis has not been usually done. We report 2 cases of adult type ductus arteriosus aneurysm. In both cases, 3D computed tomographic scanning showed a saccular aneurysm originating from the distal aortic arch toward the left pulmonary artery, which had a notching in the orifice of the aortic side. They were successfully treated surgically though one was a ruptured aneurysm to the left pulmonary artery. In these cases, 3D-CT scan was of great value in the preoperative diagnosis of the ductus arteriosus aneurysm.     

Aneurysm of the ductus diverticulum in adults <Emphasis Type="Italic">The diagnostic value of three-dimensional computed tomographic scanning</Emphasis>

Ductus arteriosus aneurysm is rare in adults and preoperative diagnosis has not been usually done. We report 2 cases of adult type ductus arteriosus aneurysm. In both cases, 3D computed tomographic scanning showed a saccular aneurysm originating from the distal aortic arch toward the left pulmonary artery, which had a notching in the orifice of the aortic side. They were successfully treated surgically though one was a ruptured aneurysm to the left pulmonary artery. In these cases, 3D-CT scan was of great value in the preoperative diagnosis of the ductus arteriosus aneurysm.     

Thymoma concomitant with a right aortic arch

A 63-year-old woman was referred to our hospital because screening had detected an anterior mediastinal tumor with a right aortic arch. She underwent typical total thymectomy via a median sternotomy but developed left recurrent laryngeal nerve (RLN) palsy postoperatively. The pathology examination revealed that the tumor was a stage I thymoma. This is the first report of a thymoma with a right aortic arch. The left RLN goes around the left ductus arteriosus, which connects the origin of the left subclavian artery to the left pulmonary artery. The RLN was likely to be injured in the neighborhood of the left ductus arteriosus when the tumor and thymus were dissected over the pulmonary artery. During operations for an anterior mediastinal tumor with a right aortic arch, we should be attentive to the location of the tumor, the left ductus arteriosus, and the left RLN.     

Adult aortic arch atresia.

Atresia in the aortic arch is a rare and severe congenital cardiovascular anomaly. Without surgical therapy, only a few patients can survive to adulthood. A 29-year-old woman with atresia of the aortic arch (Celoria-Patton Type A) without any intracardiac shunt underwent primary surgical correction involving reconstruction of the aortic arch with prosthetic interposition between the transverse aortic arch and the descending aorta and division of the persistent ductus arteriosus. Her postoperative course was uneventful, and she is now doing well at three years to date after surgical treatment.     

Repair of Truncus Arteriosus and Interrupted Aortic Arch

A total of seven patients with truncus arteriosus and interrupted aortic arch (IAA) comprises our surgical experience in this condition. All underwent primary complete repair via median sternotomy between June 1985 and December 1989. Median age at repair was 8 days and median weight, 3.2 kg. Anatomy of these seven patients was truncus arteriosus type "1 1/2" in five patients and type II in two patients, IAA type B in six patients and type A in one patient. Aortic arch was reconstructed by direct anastomosis of ascending aorta and descending aorta. Right ventricle to pulmonary artery continuity was established with a porcine valved conduit in four patients, aortic homograft in two, and aortic homograft monocusp patch in one. Three patients have required five reoperations (three in one patient). One reoperation was due to compression of the left main bronchus from the reconstructed aorta, one was due to obstruction of the aorta at the site of IAA repair, and one was due to compression of the left main bronchus, right pulmonary artery, and residual stenosis across the hypoplastic ascending aorta. There were no early or late deaths and all seven survivors are currently well with a mean follow-up of 29 months from initial repair.     

Application of an extended aortic arch anastomosis for staged repair of type A interruption: a case report of a 13-day-old neonate

A 2-day-old girl was found to have a type A interruption of aortic arch, ventricular septal defect and patent ductus arteriosus with severe metabolic acidosis. Following aggressive medical treatment with Prostaglandin E1 and inotropic agents, she underwent arch reconstruction by extended aortic arch anastomosis and pulmonary arterial banding on her 13th day. Six months after first stage repair, a subarterial infundibular VSD was closed transpulmonary and reconstruction of pulmonary artery was done by end-to-end anastomosis. She shows normal development, strong femoral pulse and no neurologic sequelae after 8 months postoperatively.     

The right-sided window ductus: a rare anatomical entity

A rare type of patent ductus arteriosus called window ductus was identified in association with a right aortic arch in a 1-year-old child who was referred for closure of a membranous ventricular septal defect.     

Rastelli and Norwood combination for the treatment of type I truncus arteriosus and hypoplastic aortic arch: a case report

Although truncus arteriosus is often treated with low mortality and morbidity rates, truncal valve patency and aortic arch and coronary artery anomalies are factors that can contribute to a worse outcome. In this report, we present our experience with the combination of Rastelli and Norwood procedures for the treatment of Type I truncus arteriosus that was complicated by a hypoplastic aortic arch.     

Truncus arteriosus with coarctation of persistent fifth aortic arch

Truncus arteriosus is a rare form of congenital heart defect. Among cases, 10% to 20% are associated with arch anomalies, such as interruption, coarctation, or patent ductus arteriosus. We treated a rare case of combined truncus with complex arch anomaly. The patient was a 2-month-old girl weighing 3.8 kg. Echocardiogram and computed tomogram showed interrupted aortic arch, truncus arteriosus, persistent fifth aortic arch, and its coarctation. One-stage total correction was performed under cardiopulmonary bypass without circulatory arrest. The operation consisted of a Rastelli procedure with a 12-mm valved conduit, coarctoplasty, and intraventricular baffling. The patient recovered well without complications.     

Complete repair of concomitant interrupted aortic arch and partial anomalous pulmonary venous connection

We report a rare case of concomitant presentation of partial anomalous pulmonary venous connection and type A interrupted aortic arch in a 4-week-old, 2.1 kg newborn. She presented in extremis and was found to have a closed ductus arteriosus with the entire left pulmonary venous return obstructed at its connection with the innominate vein. Emergent operative repair was performed in one-stage, consisting of aortic arch reconstruction and anomalous vein translocation. Postoperative recovery was unremarkable and the patient was discharged from hospital 12 days after operation.     

Surgical treatment of vascular ring

Seven patients with vascular ring have been operated upon since 1965 at the Keio University Hospital with one operative death and three late deaths. Four patients had double aortic arch and 3 had right aortic arch with left ductus arteriosus. The lesser arch was divided in the four cases of double aortic arch. Ductus or ligamentum was divided in the three cases of right aortic arch with left ductus arteriosus or ligamentum arteriosum. Respiratory distress seems to constitute major cause of post-operative death and complete relief from respiratory and swallowing difficulties was achieved in only two out of the seven cases. Associated anomalies of the face and nasolaryngeal regions with vascular ring were found in three of the 6 infants. Postoperative results seemed to be less satisfactory in those with these associated anomalies. A proposal is made to call such association as vascular ring-facial syndrome.     

Aneurysm of a patent ductus arteriosus in an adult

An adult patient with a spontaneous aneurysm of a patent ductus arteriosus was successfully treated. The lumen of the aneurysm communicated with the distal aortic arch and the pulmonary artery. Closure of the pulmonary arterial orifice of the ductus arteriosus and replacement of the inferior wall of the distal aortic arch and proximal descending aorta with prosthetic graft were performed under partial cardiopulmonary bypass using a centrifugal pump. The postoperative recovery was uneventful.