The usefulness of staged extra-anatomic bypass for coarctation of the aorta with arch hypoplasia, from premature neonate to adult.

Various surgical techniques have been developed for coarctation of the aorta. However, coarctation repair in neonates with arch hypoplasia remains challenging. We herein report a case in which a premature neonate under 1500 g with coarctation of the aorta and arch hypoplasia underwent an extra-anatomical bypass at 18 days old. A second extra-anatomical bypass was performed at 3 years of age, and a third extra-anatomic bypass for recurrent coarctation was performed in adulthood. By increasing the size of the graft as the patient grows, extra-anatomic bypass can be a useful surgical option for premature neonates with coarctation and arch hypoplasia.

Pseudoaneurysm after aortopulmonary window repair and bilateral lung transplantation for eisenmenger syndrome: a case report.

BACKGROUND: Aortopulmonary window (APW) is a rare congenital cardiac anomaly characterized by communication between the main pulmonary artery and ascending aorta. There are various surgical techniques, and the short- and long-term results are excellent if the surgical repair is performed early in life. To our knowledge, there have been no reports of pseudoaneurysm after APW repair. Herein, we present a case of a 30-year-old woman with an ascending aortic pseudoaneurysm found at the site of APW repair nine months after the APW repair and bilateral lung transplantation. CASE PRESENTATIONS: A 30-year-old woman presented with APW and Eisenmenger syndrome. The patient underwent APW repair and bilateral lung transplantation. We transected the communication between the aorta and pulmonary artery and closed the aortic side directly with strips of felts. Nine months after the surgery, the patient complained of chest pain. Cardiac computed tomography revealed an ascending aortic pseudoaneurysm at the anastomotic site. Emergent graft replacement of the ascending aorta was performed and the postoperative course was uneventful. CONCLUSIONS: We have presented a case of a pseudoaneurysm at the anastomotic site after APW repair and bilateral lung transplantation. The choice of surgical technique should be based on the patient's background requiring lung transplantation, and in these cases close postoperative follow-up is required.

Novel surgical technique for anomalous origin of a left coronary artery from the right sinus of Valsalva with a single coronary orifice.

Anomalous origin of a left coronary artery from the right sinus of Valsalva with a single coronary orifice is a rare congenital anomaly, which has been associated with myocardial ischemia and sudden death. Surgical repair is recommended upon its detection. A 14-year-old boy was diagnosed with anomalous origin of a left coronary artery from the right sinus of valsalva with a single coronary orifice after a syncope attack. The patient underwent relocation of the left coronary orifice. The postoperative course was uneventful, with no ventricular arrhythmia or syncope. The patient did not develop cardiac ischemia or infarction on exercise Tc-99 m myocardial scintigraphy 8 months after the procedure.

Bilateral pulmonary artery banding for transposition of the great arteries complex with coarctation.

Surgical approaches for transposition of the great arteries with aortic arch obstruction include primary repair and two-stage repair. However, neither approach provides a satisfactory outcome. We report a case of patient who underwent two-stage repair, wherein arterial switch operation combined with aortic arch reconstruction was preceded by bilateral pulmonary artery banding; this yielded good outcomes. This approach safely avoids primary repair in the neonatal period and allows for the opportunity to evaluate right ventricle outlet tract stenosis before the definitive repair.

Management of neonatal cardiac rhabdomyoma obstructing the aortic valve.

Rhabdomyomas associated with tuberous sclerosis are common cardiac tumors in children, and no surgical intervention is needed in most cases. However, when the tumor causes left ventricular outlet tract obstruction (LVOTO), immediate surgical intervention is indicated. Here, we report a newborn who was diagnosed antenatally with multiple cardiac rhabdomyomas, one of which blocked the aortic valve during systole. The tumor was resected early in the postnatal period with excellent outcomes. Until surgery, we maintained ductus arteriosus patency and systemic circulation using prostaglandin E1, which helped to reduce the risk of sudden death due to LVOTO. Postoperative two-dimensional echocardiography at discharge showed that surgery was effective in resolving LVOTO.

Coarctation of Aorta With Circumflex Aorta: Risk of Bronchial Compression and Recoarctation.

Coarctation of the aorta with a circumflex aorta is a very rare congenital heart anomaly. This report describes the treatment of 3 patients with this condition. All patients underwent direct anastomosis for aortic arch repair. However, postoperatively, in 2 patients, left main bronchial compression with gradual traction of the descending aorta toward the left side developed. The third patient experienced recoarctation of the aorta resulting from excessive tension. Surgical strategies for coarctation of the aorta should consider the positional relationship of the descending aorta and aortic arch, such as cases of a circumflex aorta.

Aortic-to-Right Ventricle Shunting for Rare Cardiovascular Conditions.

Pulmonary atresia with hypoplastic right ventricle and ventricular septal defect with right ventricle-dependent coronary circulation are very rare congenital heart anomalies. This report describes the case of a patient in whom aortic-right ventricle shunting was surgically established with a satisfactory postoperative course. Aortic-right ventricle shunting can enhance oxygenation of the sinusoidal communication and reduce the incidence of myocardial ischemia without influencing the left ventricular volume load through the ventricular septal defect despite preservation of the septal defect.

Reconstruction in left main coronary artery atresia with bypass graft obstruction.

Paediatric coronary artery bypass surgery with internal thoracic artery grafting is the optimal choice for left main coronary artery atresia; we report successful reconstruction in a patient with bypass graft obstruction. The pulmonary trunk was transected to expose the left main coronary stem, which was opened beyond the obstruction and the incision extended through the left main coronary artery to the circumflex bifurcation and left anterior descending artery. A funnel-shaped coronary ostium was created with a glutaraldehyde-treated autologous pericardium onlay patch. Surgical reconstruction is a good alternative for left main coronary artery atresia with graft obstruction and preserves the remaining internal thoracic artery for future use.

Anticoagulation Therapy After the Fontan Procedure.

The optimum postoperative anticoagulation therapy type and duration after the Fontan procedure remains unclear. This study aimed to evaluate whether our approach of administering only oral antiplatelets without warfarin due to serious bleeding complication risks in children is reasonable. We retrospectively reviewed the data of 249 patients who underwent the Fontan procedure. Total cavopulmonary connection with extracardiac conduit was performed in 230 patients (92%), the lateral tunnel Fontan procedure was performed in 18 patients (8%), and intraatrial conduit Fontan procedure was performed in one patient. Aspirin administration (initial dose: 5 mg/kg/day) was continued in all patients. Only 29 patients (11%) received combined administration of aspirin and warfarin for various reasons, and warfarin was discontinued in 20 (71%) of these within a median of 16 months postoperatively. Sixteen patients at high risk of thromboembolism were started on warfarin. Hospital mortality was 0%, with no thromboembolic or bleeding event-related early death. Nine late deaths, not associated with bleeding nor thromboembolism, occurred except one resulting from cardiac infarction 1.9 years after the procedure. During hospitalization, five patients experienced graft thromboembolism, two of which underwent graft exchange, and the others were started on warfarin. In the late phase (3.1 ± 6.6 years), cerebral infarction (n = 3), peripheral pulmonary artery occlusion (n = 2), gastrointestinal bleeding (n = 5), and respiratory hemorrhage (n = 4) occurred. Eight of nine patients with bleeding events received only aspirin. Thromboembolic events occurred in six patients within the first year after the procedure. Bleeding events occurred at 8 ± 5.5 years after the procedure. Expected freedom from a thromboembolic event was 99.8% at 1 year and 93.4% at 20 years. Expected freedom from a bleeding event was 100% at 1 year and 88.4% at 20 years. In children at low risk of thromboembolism after the Fontan procedure, continuous administration of an oral antiplatelet agent alone without warfarin seems to represent a reasonable prophylactic approach.

Norwood procedure for hypoplastic left heart syndrome associated with valvular pulmonary stenosis.

Association between hypoplastic left heart syndrome and valvular pulmonary stenosis is very rare. Severity of valvular pulmonary stenosis in this setting limits management options. Consequently, patients with this condition are considered poor candidates for Norwood stage one reconstruction. Herein, we describe a newborn with hypoplastic left heart syndrome and significantly dysplastic pulmonary valve who successfully underwent the Norwood procedure with neoaortic valve reconstruction. Therefore, the Norwood procedure with neoaortic valve reconstruction might be an option for this difficult condition.