Surgery for critical congenital aortic stenosis during the first three months of life

Open commissurotomy for critical aortic stenosis has been performed in a consecutive series of 28 neonates and infants below 3 months of age (average age 1 month) including 5 patients with severe organic mitral valve disease in need of concomitant correction. In the majority of cases, aortic stenosis was due not only to fused commissures but also to excessive immature valve tissue protruding into the valve area. Therefore a technique of extended commissurotomy has been adopted resecting such nodules responsible for a secondary level stenosis. All operations were done under deep hypothermia (17 degrees C), circulatory arrest (33 +/- 11 min) and cardioplegia using the Bretschneider solution (35-50 ml/kg). Hospital mortality was 18% (5) including 1 infant dying at 2 months of thrombosed mitral prosthesis inserted at a secondary operation. Severe organic mitral valve disease proved to be the only significant risk factor for early mortality. During the follow-up period of up to 10 years (average 5 years), 1 child with a hypoplastic left heart died and 2 children had to undergo reoperation for residual and recurrent stenosis, respectively. Actuarial survival for the present series is 78% at 10 years while reoperation free survival for the aortic valve is 64%. It is concluded that careful and if necessary extended open commissurotomy still represents the method of choice in this age group. Alternative methods such as transventricular or percutaneous dilatation did not show a lower risk until now and long-term results are not convincing as yet.     

Repair of aortic coarctation in the first year of life

Twenty-five infants under 1 year of age (mean, 10.3 weeks and 4.0 kg) underwent coarctation repair. Eight had ventricular septal defect (VSD), 3 had transposition of the great arteries with VSD, and 5 had severe tubular hypoplasia. One infant required mitral valve replacement, and 1 required repair of total anomalous pulmonary venous return. Fifteen had repair by primary anastomosis. Seven underwent Dacron or subclavian aortoplasty; the advantages and technique of angioplasty are reviewed. Three patients required bypass grafts. Seventeen patients survived operation. All 5 patients who had severe tubular hypoplasia died postoperatively. The mortality for repair of coarctation with VSD by simultaneous pulmonary artery banding was high; for coarctation with VSD we currently recommend repair without banding, followed by VSD closure if indicated. Three infants have been treated successfully in this manner, with early VSD closure in 1 and regression of the VSD during follow-up in 2. The 17 survivors have been followed for a mean of 41 months with 3 late deaths. Of the 17 survivors, all of whom had a primary anastomosis, 3 have residual gradients. Of the 11 survivors who had preoperative hypertension, 6 are still hypertensive; 3 of these have a gradient between the upper and lower extremities. It is striking that 3 have persistent hypertension despite repair under the age of 1 year.     

Combined repair of adult coarctation of the aorta and aortic valvular stenosis

A 57-year-old man with a high-grade aortic stenosis and aortic coarctation was treated with concomitant valve replacement and insertion of a conduit from the ascending aorta to the retrocardiac descending aorta via the left pleura. Because heart failure has been reported shortly after cardiopulmonary bypass using this technique, the conduit was initially clamped until the postrepair haemodynamics was stable. Computed tomography after 14 months verified patency of the shunt.     

Successful repair of TGA with ventricular septal defect, the outflow tract stenosis of the left ventricle and coarctation of the aortic arch]

A 9-month-old boy after the coarctectomy at 5 months old was successfully repaired by Senning procedure with closure of ventricular septal defect and resection of the membranous septal aneurysm causing the outflow tract stenosis of the left ventricle. Retrospectively, this case might have been possible to do Jatene procedure, however, attempts to resect the left ventricular outflow tract stenosis often failed to relieve stenosis completely. Therefore, the intraatrial switch operation seems to be better than the arterial switch operation when there is a possibility to remain left ventricular outflow tract stenosis. True estimation for these cases must await more case studies.     

Successful repair of transposition of the great arteries with right aortic arch and coarctation of the aorta

A one-month-old girl with a diagnosis of situs solitus, d-transposition of the great arteries, coarctation of the aorta, and right aortic arch underwent a successful rapid two-stage repair. The combined anatomy of coarctation and right aortic arch is relatively rare, and even rarer in combination with transposition of the great arteries. We report the first successful surgical intervention to correct these anomalies.     

Ebstein's anomaly of tricuspid valve with aortic stenosis and coarctation of aorta: Successful single-stage repair of a rare adult congenital heart disease

Ebstein's anomaly of the tricuspid valve is infrequently associated with left heart anomalies. The association of aortic stenosis in Ebstein's anomaly has been reported to be extremely rare and the association of coarctation of aorta is even rarer especially in adults. The combination of all three of these lesions is virtually unknown without any references in literature. We report here an unusual case of Ebstein's anomaly of the tricuspid valve and severe aortic stenosis with coarctation of aorta in an adult who presented to us with exertional dyspnoea in the third decade and underwent a successful single-stage intracardiac repair.     

Successful repair of graft failure and abdominal aortic aneurysm following thoraco-abdominal bypass for atypical aortic coarctation

A 54-year-old woman, who underwent descendo-abdominal aortic bypass grafting for atypical aortic coarctation complicated with Takayasu’s arteritis 37 years previously, was referred to our hospital for treatment of a pseudoaneurysm due to rupture of the graft. Preoperative computed tomography scan also demonstrated an abdominal aortic aneurysm. First, an endovascular stent-graft repair of the pseudoaneurysm was performed, then the abdominal aortic aneurysm was repaired with the aid of cardio-pulmonary bypass. Proper surgical planning was important to treat this rare development accompanied by aberrant circulation.     

Extraanatomic aortic bypass for repair of aortic coarctation

We describe two adult patients who underwent extraanatomic ascending aorta-to-descending thoracic aorta bypass grafting for repair of aortic coarctation through a median sternotomy and posterior pericardial approach. Of the two patients, one presented with coarctation and concurrent cardiovascular disorders, and the other, with residual coarctation. Cardiopulmonary bypass was established with double arterial cannulation in the aorta or axillary artery and the femoral artery ensure adequate perfusion proximal and distal to coarctation and bicaval cannulation. The heart was retracted cephalic and superiorly, and the descending thoracic aorta was exposed through the posterior pericardium. After achieving distal anastomosis, the graft was directed anterior to the inferior vena cava and lateral to the right atrium, and anastomosed to the right lateral aspect of the ascending aorta. A 14-mm graft was used. In one patient receiving concomitant procedures, mitral valve repair and replacement of the ascending aorta was performed after the distal anastomosis.     

One-stage repair of an aortic dissection and aortic coarctation

Ascending aortic dissection with aortic coarctation has a high mortality. There are few reports of successful surgical management of the combined condition. We report a case of a successful one-stage repair of type A aortic dissection with aortic coarctation, using an extra-anatomic bypass to connect the ascending to the abdominal aorta.     

Successful vaginal delivery for a parturient with complete aortic coarctation

This report describes the case of a parturient with complete coarctation of the aorta that was diagnosed during pregnancy. The use of invasive monitoring and the choice of labor analgesia for vaginal delivery are reviewed. Management of patients with complex cardiac anomalies requires consultation among cardiologist, obstetricians, and anesthesiologists to ensure good outcome for the mother and baby.     

Single-stage repair of aortic coarctation with concomitant bicuspid aortic valve and ascending aortic aneurysm

The optimal surgical management of patients presenting with an aortic coarctation together with other cardiovascular disorders is unclear. In this study, we report the case of an adult male with an aortic coarctation associated with a bicuspid aortic valve and an ascending aortic aneurysm. The patient underwent single-stage repair involving the Bentall technique and total arch replacement combined with stented elephant trunk implantation, which was performed through median sternotomy. We consider this procedure to be a suitable alternative for treating these complex cases.     

Successful repair of supravalvular aortic stenosis with ostial stenosis of the left coronary artery using Brom’s three patch technique

Stenotic lesion of the left coronary artery is an unnoticed but complicating feature of supravalvular aortic stenosis (SAS). We present successful repair of SAS with left coronary ostial stenosis. A 9-year-old girl was diagnosed as Williams syndrome associated with SAS. She had no symptoms of angina but cardiac catheterization revealed severe stenosis of the left coronary artery ostium. We adopted Brom’s three patch technique, which could enlarge the aortic root and ostial lesion of left coronary artery inclusively. This method is also ideal regarding restoration of the aortic root geometry.     

Somatosensory-evoked potentials during aortic coarctation repair.

OBJECTIVE: To determine the incidence of somatosensory-evoked potential (SSEP) changes and the interventions based on these changes during aortic coarctation repair. DESIGN: Retrospective review. SETTING: Single-institution, university hospital. PARTICIPANTS: Eighty-four children who had undergone surgical repair of aortic coarctation from January 1984 to May 1996. INTERVENTIONS: SSEPs were monitored in all patients throughout the procedure. A persistent decrease in amplitude greater than 50% from baseline was considered significant. Duration of SSEP changes in relation to the time course of surgical repair and whether a surgical or anesthetic intervention resulted from a change in SSEPs were documented. MEASUREMENTS AND MAIN RESULTS: Eighty-four patients underwent 87 surgical procedures. SSEP changes occurred in 40% of the procedures: 38.5% with repair and 15% with test clamp, with 9% occurring during both test clamp and repair. Interventions, which included repositioning the aortic cross-clamp, elevating blood pressure, and aborting surgery, occurred in 26.4% of all procedures based on SSEP changes. No patient sustained a neurologic deficit. CONCLUSION: This is the largest series to date describing the use of SSEPs in aortic coarctation repair. These SSEP changes were often immediately amenable to changes in surgical and anesthetic management. SSEP changes and interventions based on these changes occurred with a considerable frequency.