Left pulmonary artery coarctoplasty by using the right pulmonary artery flap near the arterial duct

Coarctation of the left pulmonary artery may coexist with tetralogy of Fallot or pulmonary atresia with ventricular septal defect. Various surgical methods, such as autologous pericardial patching and intraoperative pulmonary artery stenting, have been used for solving this condition. We proposed a simple and effective technique for treating patients with left pulmonary artery coarctation and pulmonary atresia with ventricular septal defect by using the right pulmonary artery flap for left pulmonary artery coarctoplasty, plus central shunt creation by V-Y plasty.     

Severe pulmonary valve incompetence late after debanding: repair by bicuspidization

We report a simple valve repair for severe pulmonary incompetence in a 25-year-old patient. The patient had been operated on twice before for ventricular septal defect and coarctation of the aorta. The first operation consisted of pulmonary artery banding and coarctectomy and end-to-end anastomosis at 4 months, followed by debanding and transinfundibular ventricular septal defect closure at 6 years of age. Massive pulmonary incompetence was due to destruction of one valve cusp with the right ventricular outflow tract and pulmonary artery dilated secondarily. Repair consisted of pulmonary valve bicuspidization and right ventricular remodelling.     

Experimental repair of ventricular septal defects using autologous right ventricular muscle flaps: preliminary report

Survival after repair of postinfarction ventricular septal defects remains poor, often due to extensive loss of contractile muscle in the septum or left ventricle. We evaluated whether a contractile flap of right ventricular muscle could be used to repair a similar ventricular septal defect to augment left ventricular performance in 7 fully instrumented mongrel dogs (weight, 23 to 28 kg). By using hypothermic bypass and cold fibrillatory arrest, a trapezoidal right ventricle flap was fashioned from the free wall of the mid to lower right ventricle, basing its widest portion anteriorly on the septum and left ventricle. A large, 2-cm-diameter core of septum was excised beneath this flap to simulate a postinfarct ventricular septal defect. The right ventricular flap was then invaginated through the defect and sewn to the left ventricular side of the septum with pledgeted sutures taken full thickness through the flap and septum in a "vest-over-pants" fashion. Contraction of the right ventricular flap was confirmed visually and by postbypass multiple gated acquisition scans. The right ventricular defect was closed with fascia lata. All dogs were weaned from bypass without inotropes. Precardiac and postcardiac outputs of 2.5 +/- 0.5 versus 2.3 +/- 0.4 L/min and left ventricular end-diastolic pressures of 4 +/- 2 versus 4 +/- 3 mm Hg were identical. No shunts were detected by oxygen saturation. Autopsies confirmed the integrity of the repair. We conclude that septal defects can be repaired by using contractile right ventricular muscle, thus preserving left ventricular function. This technique offers promise for repair of postinfarction ventricular septal defects by using autologous, already conditioned to contract, cardiac muscle, but its application in humans must await long-term testing.     

Pulmonary atresia, ventricular septal defect, and coronary-pulmonary artery fistula

We report 2 patients with pulmonary atresia, ventricular septal defect, and coronary-pulmonary fistula. The fistula originated from the left anterior descending artery in 1 patient, and from the right coronary artery in the other. Both patients survived staged correction in which right ventricular outflow was reconstructed with autologous fistula tissue. One patient with pulmonary hypertension suddenly died 9 months after surgery. Twenty-four patients with pulmonary atresia, ventricular septal defect, and coronary-pulmonary fistula have been reported previously.     

Autologous Pericardium Patch Aneurysm after Ventricular Septal Defect Closure and Arterial Switch Operation

Abstract   A neonate with L-transposition of the great arteries with ventricular septal defect underwent complete repair using fresh autologous pericardium to close the ventricular septal defect as well as to reconstruct the neo pulmonary artery sinuses. Four months later, the child came back with right ventricular inflow obstruction related to aneurysmal pericardial patch, severe tricuspid regurgitation, and severe supra-valvular pulmonic stenosis. At reoperation, there was a redundant, aneurysmal pericardial patch densely adherent to the septal and posterior leaflets of the tricuspid valve, which was damaged. The pericardial patch was replaced, the pulmonary artery enlarged, and tricuspid valve repaired. Postoperative course was uneventful, but residual moderate tricuspid regurgitation required intensive medical treatment.     

Repair of hemitruncus with autologous arterial ring and valved bioconduit

We present a technical variant to reconstruct the right outflow tract in truncus type A3 (single pulmonary artery branch originating from the ascending aorta with common arterial valve and ventricular septal defect) with interposition of a ring of autologous arterial tissue and a bioconduit between the right ventricular infundibulum and the pulmonary artery branches. The final result is shown by postoperative angiogram which demonstrates an anatomically adequate reconstruction of the right ventricular outflow tract.     

Anomalous origin of the pulmonary artery from the right coronary artery

A 5-year-old boy who had an anomalous origin of a stenotic pulmonary artery from the right coronary artery with ventricular septal defect and pulmonary atresia is reported. The diagnosis was made at operation. Successful repair was achieved for the complex anomaly by using a pulmonary artery homograft as a conduit and closing the ventricular septal defect.     

One-stage unifocalization and palliative right ventricular outflow tract reconstruction

We report a successful case of one-stage unifocalization concomitant with palliative right ventricular outflow tract reconstruction for pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries and central pulmonary arterial absence. Through a median sternotomy, one-stage unifocalization was accomplished using autologous pericardial conduit and no prosthetic material. To achieve an adequate pulmonary blood flow, the right ventricular outflow tract was reconstructed so that it would be 70% of the normal pulmonary annular diameter, while the ventricular septal defect was left open. One-stage unifocalization concomitant with palliative right ventricular outflow tract reconstruction is considered to be a good surgical option for selected patients with these diseases.     

Staged Approach for Congenitally Corrected Transposition of the Great Arteries Associated with Dextrocardia and Nonconfluent Pulmonary Artery

Abstract   We completed double switch operation (Senning plus Rastelli procedure; SLL) after staged palliative operations for SLL, dextrocardia, congenitally corrected transposition of the great arteries ventricular septal defect, atrial septal defect, and nonconfluent pulmonary artery. The patient had undergone three preparatory operations including central pulmonary artery reconstruction with autologous pericardium and morphologic right ventricle-pulmonary artery shunt and then double switch operation was performed. During the double switch operation, the previous conduit which was established as right ventricle-pulmonary artery shunt in the third palliation was replaced by a larger conduit. This conduit was used as Rastelli external conduit to avoid coronary artery injury. When systemic venous chamber was reconstructed, suture line pouch technique was used, in which dog ears were made at the sites of superior and inferior vena cava orifices, respectively, to prevent systemic venous return obstruction. Pulmonary venous chamber was reconstructed without any augmentation, which reduced the possibility of postoperative arrhythmia. Systemic and pulmonary venous pathway obstructions were not observed after the operation. Postoperative arrhythmia was not detected.     

Surgical repair of double-chambered right ventricle with or without ventriculotomy

Double-chambered right ventricle is an uncommon congenital anomaly consisting of obstruction of the trabecular zone of the ventricle by an elevated, hypertrophied moderator band, with or without additional muscle bundles(s). During an 18 month period (August 1984 to February 1986) 12 patients underwent repair of double-chambered right ventricle. Their ages ranged from 13 months to 12 years (mean 4.4 years). Associated lesions were ventricular septal defect in 11 patients (three closed spontaneously), atrial septal defect in two, left superior vena cava in three, aortic valve prolapse in one, and mild subaortic stenosis in two. Right ventricular outflow tract gradients were from 30 to 165 mm Hg (mean 77.2 +/- 37.3 mm Hg). The initial six patients (Group 1) were treated in the "classical" manner with a right ventriculotomy for muscle resection and ventricular septal defect closure (4/6). All patients survived. Five of the six required pharmacologic support for signs of mild to moderate right ventricular failure. The right ventricular outflow tract gradient postoperatively was 0 mm Hg in five patients and 25 mm Hg in one patient. Oxygen saturations were checked in the right atrium and pulmonary artery in all six patients, and one patient had a step-up of more than 10%. In the next six patients (Group 2) a previously unreported right atrial-pulmonary artery approach was used to avoid a ventriculotomy. The right ventricular outflow tract gradient was 0 mm Hg in five patients and 5 mm Hg in the sixth. A ventricular septal defect was closed in four of the six patients. There were no deaths, and no patient required inotropic support. Follow-up has been from 2 to 20 months. There have been no late deaths, and all patients are asymptomatic. One patient in Group 1 still has signs of a residual ventricular septal defect at 1 year. No patient has echocardiographic evidence of right ventricular outflow tract obstruction. All patients have remained in sinus rhythm with right bundle branch block. No patient has been recatheterized. Repair of double-chambered right ventricle is easily performed without a ventriculotomy. The atrial exposure of the ventricular septal defect is excellent. The muscle bundle(s) can be visualized easily through an initial transverse pulmonary arteriotomy with completion of resection through the tricuspid valve.     

Clinical results of arterial switch operation for double-outlet right ventricle with subpulmonary VSD.

OBJECTIVE: An arterial switch operation is considered a good alternative for the repair of double-outlet right ventricle (DORV) with atrioventricular concordance connection and subpulmonary ventricular septal defect (VSD) when intraventricular rerouting is not feasible. The clinical results of an arterial switch operation with ventricular septal defect closure for this anomaly were studied. METHODS: Between 1986 and 1997, 27 patients ranging from 10 days to 5 years of age (mean 0.4 years) underwent an arterial switch operation with ventricular septal defect closure for the correction of double outlet right ventricle with subpulmonary VSD. The 50% rule was used to define double-outlet right ventricle. Arch anomalies were associated in nine cases, and were corrected either previously or simultaneously. A subarterial muscle resection was performed in 14 without any subsequent stenosis of the ventricular outflow tract. The relationship of the great arteries was mostly anteroposterior in 15 and mostly side by side in 12. The left coronary artery (main trunk or circumflex artery) courses behind the pulmonary artery in 15/27 (six/15 in the anteroposterior relation and ten/12 in the side by side relation). The Lecompte maneuver was used to reconstruct the pulmonary artery in all but five cases with a side by side relationship of the great arteries. RESULTS: There was one operative death (3.7%) and three late deaths. The actuarial survival rate was 83 +/- 8% at 9 years. Right ventricular outflow tract obstruction including peripheral pulmonary stenosis developed in seven cases operated on in the early era. The reoperation free rate was 46 +/- 20% at 9 years. CONCLUSION: Although double-outlet right ventricle with subpulmonary VSD has complex features, including an aortic arch obstruction and coronary artery anomalies, an optimal definitive surgical repair using an arterial switch operation can be performed safely with a thorough understanding of this variable anomaly. The prevention of right ventricular outflow tract obstruction at the time of an arterial switch operation may thus help improve the rate of late morbidity.     

Surgical management of the conal (supracristal) ventricular septal defect

Surgical management of the conal (supracristal) ventricular septal defect differs significantly from the management of the perimembranous (infracristal) ventricular septal defect. The absence of a portion of the conal septum can lead to prolapse of the right cusp of the aortic valve, which predisposes these patients to aortic insufficiency. Between January 1980 and December 1989, 36 children with conal ventricular septal defect underwent intracardiac repair. Diagnosis was by echocardiography, cardiac catheterization, and intraoperative exploration. Preoperative evaluation showed that 26 patients (72%) had aortic valve prolapse and 16 (44%) had aortic insufficiency. Pulmonary-to-systemic flow ratios ranged from 1:1 to 3.5:1 (mean 2.0:1.0). Ten patients (27%) were believed to have clinical congestive heart failure. Age at the time of operation ranged from 2 weeks to 18 years (mean 5.5 years). Operative exposure was through the pulmonary artery (26), aorta (4), right ventricle (3), or right atrium (3). Simultaneous aortic valve suspension for aortic insufficiency was performed in four patients. Operative survival was 100%. Follow-up is complete in all patients and ranges from 0.5 to 9 years (mean 4.3 years). All patients are in normal sinus rhythm. No residual ventricular septal defects have been identified. Twenty-three of 36 patients (64%) have no evidence of aortic insufficiency; 12 of 36 (33%) have trivial or mild aortic insufficiency. One patient with initial severe aortic insufficiency underwent repeat aortic valvuloplasty 3 years after ventricular septal defect closure and aortic valve suspension. No patients have required aortic valve replacement. Surgical management of the conal ventricular septal defect differs from that of the perimembranous ventricular septal defect in two critical aspects. The operative approach should be through the pulmonary artery. This allows the best exposure of the remaining conal septum and the pulmonary and aortic valve leaflets, facilitating closure of the defect without injury to the valves or conduction system. Conal ventricular septal defects should undergo early closure, regardless of shunt volume, to prevent progressive aortic insufficiency.     

Surgical correction of pulmonary atresia and ventricular septal defect with large systemic-pulmonary collaterals

A two-stage surgical procedure was performed in a young adult with pulmonary atresia and a ventricular septal defect with upper lobe pulmonary artery flow from confluent central pulmonary arteries and lower lobe pulmonary blood flow originating from two large systemic-pulmonary collaterals. Initially a Dacron Y graft was anastomosed between the ascending aorta and the collaterals and a graft to the left pulmonary artery. At the second operation, continuity was established between the right ventricle and the Y graft by using a valved Dacron conduit and the ventricular septal defect was closed.     

A case report of successful repair for ruptured aneurysm of sinus of Valsalva (I VSD) with aortic regurgitation

A nineteen-year-old female who had history of infectious endocarditis underwent surgical repair for ruptured aneurysm of sinus of Valsalva with aortic regurgitation. Through aortotomy mild degree of prolapse of right coronary cusp and perforation of left coronary cusp sized 6 mm in diameter were recognized and the latter was thought to be the dominant lesion resulting in severe aortic regurgitation. Following direct closure of ruptured aneurysm and patch closure of ventricular septal defect, perforated left coronary cusp was repaired with autologous pericardium. Post operative course was uneventful and she is now doing well.     

Pediatric lung transplantation for pulmonary hypertension and congenital heart disease.

Five children underwent lung transplantation for end-stage pulmonary hypertension and respiratory insufficiency associated with congenital heart disease. One (17 mo) had pulmonary hypertension with a patent ductus arteriosus and required two periods of preoperative extracorporeal membrane oxygenation before successful bilateral sequential lung transplantation. One (21 mo) required bilateral lung transplantation for pulmonary hypertension and bronchopulmonary dysplasia associated with iatrogenic injury to the left pulmonary artery. This child also had patent ductus arteriosus ligation and preoperative catheter closure of an atrial septal defect. Extracorporeal membrane oxygenation was required for early postoperative pulmonary support. One child underwent right single-lung transplantation and closure of an atrial septal defect for pulmonary hypertension. Two patients had single-lung transplantation for Eisenmenger's syndrome: 1 with muscular inlet ventricular septal defect closure, atrial septal defect closure, and right single-lung transplantation; 1 with ventricular septal defect closure, patent ductus arteriosus ligation, right ventricular outflow tract patch repair, and single-lung transplantation. All patients survived operation, with one late death (lymphoproliferative disease). The 4 survivors are all ambulatory without oxygen and have evidence of normal pulmonary artery pressure 9 to 12 months after transplantation.     

Pericardial collar modification for Ross procedure

Although the surgical technique of the Ross operation has been improved over the years, there are still some technical difficulties: (1) The possibility of damage to first septal artery during harvesting and reconstruction of right ventricular outflow tract (RVOT). (2) Weak posterior anastomotic area during RVOT reconstruction. (3) Persistent bleeding from septal dissection site of pulmonary autograft. To deal with these difficulties, we used a 1-cm wide pericardial strip (pericardial collar) which was sutured to epicardium at the posterior and septal edge of the RVOT. The conduit used for the reconstruction of pulmonary outflow was then sutured to this pericardial strip at the posterior part of the anastomosis. We found this technique very useful to create a safe and strong margin for the posterior suture line. Persistent bleeding from septal dissection site can be also avoided using this modification by diverting the bleeding site into the right ventricular cavity.     

Repair of large muscular ventricular septal defects in infants employing left ventriculotomy

The approach to muscular ventricular septal defect has been controversial. Traditional approaches have included pulmonary artery banding or right ventriculotomy. Left ventriculotomy has been employed in older children because of the ease of exposure in the absence of trabeculations, but concern has been raised regarding potential loss of left ventricular function. Four infants less than 4 months of age underwent repair of a large muscular ventricular septal defect by left ventriculotomy. One infant had no associated defects; 2 had undergone previous coarctation repair (1 with pulmonary artery banding); and 1 had a large secundum atrial septal defect. All had congestive heart failure refractory to maximal medical management. All patients survived, and there was no myocardial dysfunction. Echocardiographic indices of ventricular function 3 to 24 months postoperatively were normal (mean shortening fraction, 32.5%). Left ventriculotomy with patch closure of the ventricular septal defect provides a simple approach to a large muscular ventricular septal defect in infants with heart failure.     

Early results for anatomic correction of transposition of the great arteries and for double-outlet right ventricle with subpulmonary ventricular septal defect

Fifty patients, 21 with transposition of the great arteries plus ventricular septal defect, four with double-outlet right ventricle plus ventricular septal defect, and 25 with transposition of the great arteries plus intact ventricular septum, have undergone anatomic correction. There were four hospital deaths, all in the patients with ventricular septal defects (overall mortality rate 8%). The Lecompte maneuver was used in all patients, and in the last 25 patients the coronary arteries were transposed to medially hinged trapdoor flaps created in the neoaorta. In 10 patients (20%) intraoperative adjustments were necessary to the great vessels and coronary arteries to achieve unobstructed coronary artery flow. There have been no late deaths. Postoperative cardiac catheterization in 19 patients has revealed stenosis on the new main pulmonary artery suture line to be the main early problem. Anatomic correction of transposition (with or without ventricular septal defect) and double-outlet right ventricle septal defect would seem to be a good operative alternative to the Mustard or Senning operation, with the advantage of incorporating the left ventricle into the systemic circulation.     

Exclusive double outlet right ventricle with atrioventricular concordance and pulmonary stenosis. Results of reconstructive surgery

Double outlet right ventricle associated with atrioventricular concordance, pulmonary stenosis and situs solitus of the atria is a subset of double outlet right ventricle related through the surgical treatment. From 1974 to 1985, 14 patients, 5 males, 9 females (mean age 8.9 years, range 13 months-22 years) were operated upon. All patients had infundibular stenosis and normal or large pulmonary arteries. The apex of the heart was to the right in 2 patients, the right and left ventricles were superior and inferior in 2 patients and 1 patient had both anomalies. The ventricular septal defect was subaortic in 11 patients (aorto-mitral discontinuity in 5) and non-committed in 3 patients. Three patients had 2 ventricular septal defects. The aorta was anterior in 3 patients and to the right of the pulmonary artery in 11 patients. All patients, through a transventricular and transatrial approach, had a reconstructive surgery. In 3 patients, an aortic homograft valved conduit was used. One patient had the ventricular septal defect enlarged. There was one early death (7.1%) from high residual right ventricle pressure and no late death. One patient had a transient atrioventricular block. One patient was reoperated upon for a residual ventricular septal defect. All survivors had a good clinical result. Re-evaluation in 8 patients confirmed excellent haemodynamics: the right ventricle to pulmonary artery pressure gradient decreased from 80 mm Hg (range 60-95) preoperatively to 24 mm Hg (range 3-32) postoperatively.(ABSTRACT TRUNCATED AT 250 WORDS)     

室上嵴上室间隔缺损的外科治疗

本文报道５６例室上嵴上室间隔缺损的外科治疗。男３４例，女２２例，年龄３～３９岁。１９例合并主动脉瓣叶脱垂、主动脉瓣关闭不全或佛氏窦瘤破裂。经右房切口修补室缺２例，右室漏斗切口４７例，肺动脉切９７例。直接修补缺损２６例，补片修补３０例。主动脉瓣成形术１例，主动脉瓣置换术５例。５４例全愈出院，死亡２例。作者认为室上嵴上空间隔缺损应尽早手术，以免并发主动脉瓣损害和佛氏窦瘤破裂；中度主动脉瓣关闭不全可行主动脉瓣成形术，采用肺动脉切口优于右室漏斗部切口。