Surgical management of tunnel-like subaortic stenosis via ventricular septal defect in a patient with the interrupted aortic arch

A 24-day-old male with interrupted aortic arch (type B), ventricular septal defect, and tunnel-like subaortic stenosis underwent a one-stage surgical treatment The operative procedure comprised reconstruction of the aortic arch, transatrial excision of the subaortic fibromuscular tissue via the ventricular sepatal defect, and patch closure of the defect. The patient tolerated the procedure well and the postoperative echocardiography demonstrated a residual pressure gradient across the left ventricular outflow tract of 20 mmHg. Our result suggests that the transatrial surgical management of subaortic stenosis via the ventricular sepatal defect produces a safe and promising surgical option.     

Modified Van Praagh's operation for interrupted aortic arch with severe subaortic stenosis in a neonate

A 26 day old neonate with Type B interrupted aortic arch, a ventricular septal defect and severe subaortic stenosis underwent a successful modified Van Praagh's operation, consisting of insertion of a graft between the main pulmonary artery and descending aorta, ligation of the patent ductus arteriosus and main pulmonary artery banding distal to the graft. This palliative procedure was effective and easily performed without the aid of cardiopulmonary bypass even in this severely ill neonate with such a complex defect.     

Surgical management of severe aortic coarctation and interrupted aortic arch in neonates.

Forty-four infants, 2 to 90 days of age, with severe obstructive lesions of the aortic arch, underwent emergency surgical correction between Jan. 1, 1966, and April 1, 1975. The typical clinical presentation was severe congestive heart failure and acidemia. Resection of an aortic coarctation with end-to-end anastomosis was performed in 31 patients. Eight (26 per cent) died after the operation. Since 1969, the mortality rate has been reduced to 14 per cent (3 of 22 patients) even though the incidence of major associated cardiac lesions has remained essentially constant (56 per cent from 1966 through 1969, 64 per cent from 1970 through March, 1975). This suggests that the higher survival rate has resulted from improved surgical techniques and postoperative care. The mortality rate in the infants operated upon during the second and third months of life was twice as high as that in those operated upon before the age of 1 month. Eight patients with Type A interrupted aortic arch were operated upon and 5 survived. Five patients with Type B aortic arch were operated upon and 3 survived.     

Primary repair of interrupted aortic arch and severe aortic stenosis in neonates

Two infants, aged 36 days old (Case 1) and 18 days old (Case 2) with interrupted aortic arch types B and A, respectively, and with severe aortic stenosis, were successfully operated on by use of pulsatile cardiopulmonary bypass. The great arteries were normally related in Case 1 and were transposed in Case 2. Repair involved the following procedure: ligation of the patent ductus arteriosus, restoration of aortic continuity with an 8 mm polytetrafluoroethylene graft, placement of an internal patch to tunnel all left ventricular blood from the left ventricle through the ventricular septal defect into the pulmonary artery in Case 1 and patch closure of the ventricular septal defect in Case 2, transection of the main pulmonary artery, anastomosis between the proximal pulmonary artery and the ascending aorta, and interposition of a valved conduit between the right ventricle and the distal pulmonary artery. The operative field could be approached easily through a median sternotomy. Postoperative cardiac catheterization revealed satisfactory anatomical and hemodynamic results in both cases.     

Surgical treatment of persistent fifth aortic arch associated with interrupted aortic arch

This study describes two cases of the rare congenital anomaly, persistent fifth aortic arch. Both cases involve boys (1 at 9 years of age and another at 7 months of age). To detect persistent fifth aortic arch with interrupted aortic arch, the following methods were used: echocardiogram, angiocardiography, and magnetic resonance imaging. In both cases the blood pressure between the upper and lower limbs differed. To relieve the obstruction of blood flow, each case was surgically repaired using patching or conduit interposition. Postoperative courses were uneventful. Two-year to 5-year follow-up examinations were positive showing that the anastomosis was unobstructed and the velocity of blood flow to the descending aorta was normal.     

Surgical management of idiopathic hypertrophic subaortic stenosis

Ten of 11 patients were successfully operated upon for severely symptomatic idiopathic hypertrophic subaortic stenosis (IHSS). The operative techniques utilized were myotomy in 9 patients, myotomy and mitral valve replacement in 1 patient, and mitral valve replacement alone in 1 patient. Intraoperative and late postoperative measurement of gradients have shown abolition of or a marked decrease in gradients in 8 patients studied. With 1 exception, all 10 survivors are now in N.Y.H.A. Functional Class I or II. One patient suffers from persistent left heart failure and remains in Class IV. Significant mitral insufficiency was abolished by operation in all but 1 patient. Myotomy is an effective and safe operative technique in the treatment of IHSS. Mitral valve replacement should be undertaken if myotomy does not relieve obstruction.     

Surgical management of complex and tunnel-like subaortic stenosis

Background: Relief of primary or secondary subaortic stenosis (SAS) remains a surgical challenge. Heart block, aortic valve regurgitation and recurrent obstruction have been persistent problems. Methods: Forty six patients who underwent surgery for complex and tunnel-like SAS between January 1990 and November 1998 were reviewed. In 45 of the 46 patients SAS developed following repair of a primary congenital heart defect and only one patient presented with de novo tunnel-like SAS. Fifteen of the 45 patients had undergone repair of double-outlet right ventricle (DORV) and the remaining 30 had undergone repair of a variety of defects. The median age at the time of surgery was 5 years. The modified Konno procedure was performed in 15 patients, Konno procedure in three, Ross–Konno procedure in two and resection of the conal septum in 12 patients. Five patients with DORV underwent replacement of the intraventricular baffle and two patients underwent an aortic valve-preserving procedure in conjunction with mitral valve replacement. Results: There were no deaths. None of the patients had an exacerbation of aortic regurgitation and none developed complete heart block. The median follow-up was 3 years (range 1 month–8.5 years). Two patients developed recurrent SAS defined as a gradient of 40 mmHg or greater diagnosed by transthoracic echocardiography. Freedom from SAS at 1, 3 and 5 years was 100, 94 and 86%, respectively. Conclusions: We favor the modified Konno procedure and conal resection to the Konno or the Ross procedure, since insertion of a prosthetic valve or homograft is avoided and aortic valve function is preserved. Excellent relief of tunnel-like SAS can be achieved without damage to the conduction tissue.     

Surgical treatment of coarctation of the aorta and interrupted aortic arch

From December, 1984, through April, 1987, ten infants with coarctation of the aorta and six with interrupted aortic arch underwent staged repair of aorta and other cardiac lesions. Simultaneous pulmonary artery banding was performed in six of 8 patients with ventricular septal defect (VSD) and in all of seven patients with complex cardiac lesions. With first operation, there were no operative deaths and two late deaths. Eight of 14 survivors underwent total correction of associated lesions at three to 17 months after initial operation. VSD was closed in five patients with one operative death. One patient required pulmonary artery debanding alone because of decrease of VSD size. The Damus-Kaye-Stansel operation was performed successfully in one patient with Taussig-Bing anomaly and the Jatene operation was done in one with transposition of the great arteries. Based on these results, we prefer staged repair with pulmonary artery banding for coarctation or interruption of the aorta associated with complex cardiac lesions.     

Surgical treatment of coarctation and interrupted aortic arch complex in infants

One hundred forty-two consecutive neonates or early infants with coarctation and interrupted aortic arch complex who underwent biventricular repair at the Fukuoka Children's Hospital between January 1991 and December 2000 were reviewed. One-stage repair was performed in 33 patients (35%) with coarctation complex and in 41 patients (85%) with interrupted aortic arch complex. The overall mortality rate was 6.1% in one-stage repair and 6.6% in two-stage repair of coarctation complex patients and 9.8% in one-stage repair and 28.6% in two-stage repair of interrupted aortic arch complex patients. The recoarctation rate was 5.3% in coarctation complex and 2.1% in interrupted aortic arch complex. All patients with recoarctation underwent successful catheter intervention and required no reoperation. In conclusion, one-stage repair of interrupted aortic arch and coarctation complex with the anterior approach resulted in good outcomes. Then descending aorta cannulation through a median sternotomy combined with the cerebral perfusion technique enables complete avoidance of circulatory arrest and is a useful technique. However, a two-stage procedure can be useful in the patients whose condition has deteriorated substantially or in whom intracardiac anomalies are severe.     

Surgical treatment for graft stenosis after repair of an interrupted aortic arch: Report of two cases

We report herein two cases of patients who underwent successful reoperation for graft stenosis after repair of an interrupted aortic arch (IAA). The first patient was a 10-year-old girl who suffered from upper limb hypertension 9 years after her initial operation. Cardiac catheterization revealed a pressure gradient of 55 mmHg across the repaired arch. At reoperation, a left subclavian turndown anastomosis was performed, following which the hypertension resolved and a car-diac catheterization done 5 years later demonstrated sufficient growth of the restored arch with no significant gradient. The second patient was a 17-year-old boy who suffered from general fatigue and intermittent hypertension 12 years after his initial operation. Cardiac catheterization revealed a gradient of 60 mmHg across the repaired arch. He underwent an extraanatomic ascending to descending aortic bypass employing an additional 18-mm graft, and a postoperative cardiac catheterization showed no gradient between the ascending and descending aorta. Our experience has shown that IAA should be repaired without prosthetic grafts if possible. Although extraanatomic bypass is useful for reducing the operative risks at reoperation, a large graft should be used to avoid the need for a third operation. For young children expected to outgrow a second graft, performing an endogenous anastomosis, such as a left subclavian turndown anastomosis, should be considered as an alternative. Received: June 14, 1999 / Accepted: March 24, 2000     

Pulmonary agenesis with interrupted aortic arch.

This report describes a unique case of an infant with right pulmonary agenesis associated with interrupted aortic arch, ventricular septal defect, and aortic valvular stenosis. Blalock-Park arterial anastomosis and pulmonary arterial banding were performed without incidence. These techniques were useful for this complicated heart defect with pulmonary agenesis. The postoperative course of the patient was uneventful.     

Anesthetic management of apicoaortic bypass in patients with severe aortic stenosis

Apicoaortic bypass (AAB), or apicoaortic conduit insertion, is a conventional surgical method that has been regaining attention due to the aging population and the increasing number of repeat surgeries. The indication for the procedure has been extended as an alternative for aortic stenosis when the usual sternotomy or aortic clamping is considered to be difficult, e.g., in patients with severe calcification of the ascending aorta (porcelain aorta), or in patients with a patent coronary artery bypass graft located adjacent to the posterior surface of the sternum. Herein, we report our recent anesthetic management of three patients undergoing AAB. Once the apicoaortic conduit is inserted, blood from the left ventricle is ejected via two routes, the narrowed native aortic valve and the apicoaortic conduit. Thus, it is necessary to elucidate any change in blood flow after the withdrawal of the extracorporeal circulation, by using intraoperative transesophageal echocardiography. Furthermore, if a rigid apical connector is not used, anastomosis of the cardiac apex and conduit is conducted under ventricular fibrillation without the infusion of cardioplegic solution; thus, patients are deemed likely to suffer increased myocardial damage. As a rigid apical connector was not used in the three present patients, the administraction of adequate catecholamines was needed for the withdrawal of the extracorporeal circulation. In addition, because those undergoing AAB often have extremely poor cardiac reserve preoperatively owing to the administration of adequate catecholamines was needed for the withdrawal of the extracorporeal circulation. In the three present patients, anesthetic management was successful, and there were no intraoperative or immediate postoperative complications.     

Surgical management of diffuse subaortic stenosis: an integrated approach

An integrated approach to the surgical management of diffuse subaortic stenosis has been designed to provide adequate relief of left ventricular outflow tract obstruction whatever the anatomical features encountered at operation. This approach was used in 22 patients with tunnel subaortic stenosis (19 patients) or diffuse hypertrophic obstructive cardiomyopathy (3 patients). The obstructive tissue was resected through an aortoseptal approach. In 18 patients, associated hypoplasia of the aortic orifice necessitated aortic valve replacement using the Konno procedure; in 4 patients with a normal-sized aortic orifice, the native aortic valve was preserved. There were two early deaths and one late death (all after a Konno operation). Long-term adequate relief of left ventricular outflow tract obstruction was achieved in all survivors. Operation for diffuse subaortic stenosis should be performed with two main goals: (1) to obtain complete relief of the left ventricular outflow tract obstruction by the appropriate procedure and (2) to preserve the native aortic valve whenever possible, particularly in young patients.