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.     

A successful surgical treatment for original Taussig-Bing malformation 13 years after banding of the pulmonary artery

A 13-year-old girl with original Taussig-Bing malformation underwent successful intraventricular rerouting. In this congenital heart disease, the large pulmonary flow elevates pulmonary arterial pressure and progress the pulmonary vascular diseases in early infancy. Banding of the pulmonary artery helps to prevent the irreversible vascular changes, while that may result in further hypertrophy of the right ventricle. Ideally, radical repair should be performed as soon as possible after definitive diagnosis. Although, she had banding operation of the pulmonary artery at 8 months of age, there was no difference between the right ventricle and the aortic pressure. The subpulmonary space was large enough to make the tunnel with an internal conduit. Additionally, right ventricle outflow tract reconstruction was performed by a Dacron patch. The postoperative cathetherization demonstrated no stenotic region in both left and right ventricular outflow tract. The Kawashima intraventricular rerouting can be applied to the patients without the subaortic stenosis if banding of the pulmonary artery have been performed long before.     

Palliative reconstruction of right ventricular outflow tract in tetralogy with hypoplastic pulmonary arteries.

Twenty-five symptomatic patients with tetralogy of Fallot underwent palliative reconstruction of the right ventricular outflow tract without closure of the ventricular septal defect. Their ages ranged from 5 months to 20 years (mean age, 3.5 years). Eight patients had had 13 prior systemic-pulmonary arterial shunts. There were six hospital deaths (24%). Three patients needed a repeat right ventricular outflow reconstruction. There was one late death. The other patients manifested clinical improvement; hematocrit decreased from 0.54 to 0.43 (p less than 0.01) and arterial oxygen saturation increased from 63.0% to 83.2% (p less than 0.01). The size of the pulmonary artery, defined as the ratio of the sum of the diameter of the right and left pulmonary arteries to the diameter of the descending aorta, increased from 0.72 to 2.06 (p less than 0.01). Sixteen patients underwent a corrective operation 2.4 years after palliation. The results of palliative right ventricular outflow tract reconstruction suggest that it may be an optional strategy for the treatment of symptomatic patients who have tetralogy of Fallot with severely hypoplastic pulmonary arteries.     

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.     

Simplified technique for pericardial patch of the right ventricular outflow tract

Pericardial patching to enlarge the right ventricular outflow tract is often required in congenital cardiac surgery in lesions such as tetralogy of Fallot, pulmonary atresia, pulmonary stenosis, and pulmonary artery repair following removal of a palliative pulmonary artery band. A method of simplifying the attachment of the pericardial patch onto the right ventricular outflow tract is described.     

Pulmonary artery balloon counterpulsation for right ventricular failure after right ventriculotomy in the swine

To assess the efficacy of intrapulmonary balloon counterpulsation in the management of right ventricular failure after right ventriculotomy, we undertook an experimental study in a swine model. To mimic the clinical settings more closely, (1) we left the automatic control of the heart intact (2) did not use cardiopulmonary bypass to support the left side of the heart, and (3) induced right ventricular failure by means of a generous surgical incision (50% to 70% of the anterior wall) of the right ventricle. The criteria set for right ventricular failure were (1) 50% increase in right ventricular end-diastolic pressure, (2) 30% decrease in mean arterial pressure, and (3) 30% decrease in cardiac output. Right ventricular failure was attained in all animals studied: A 230% increase in right ventricular end-diastolic pressure, a 43% decrease in cardiac output, and a 34% decrease in mean arterial pressure were evident after the right ventriculotomy. A specially designed intrapulmonary balloon catheter (Datascope Corp., Oakland, N.J.) was placed into the left pulmonary artery through the right ventricular outflow tract. A Datascope console was used for counterpulsation. Effects of counterpulsation for 40 minutes in a 1:1 mode were assessed after surgical induction of right ventricular failure in 14 swine. Each animal served as its own control. The mean hemodynamic changes are outlined: Right ventricular end-diastolic pressure decreased by 48.9% (p = 0.01). Mean arterial pressure increased by 68.8% (p = 0.01) and cardiac output by 44.2% (p = 0.01). Histologic studies disclosed no morphologic damage to the pulmonary artery or valve in the specimens analyzed. In addition, these results were compared with those in a second group of seven swine in which right ventricular failure was induced by right ventriculotomy and a balloon was placed into the left pulmonary artery but not activated. These results of short-term counterpulsation should be evaluated in a longer term model so as to mimic more closely the clinical setting. If the hemodynamic benefits are duplicated, intrapulmonary balloon counterpulsation should be considered as a simple, effective device when right ventricular failure develops after right ventriculotomy. It effectively improves right ventricular function without damaging the pulmonary artery or valve.     

Recurrent pulmonary intimal sarcoma involving the right ventricular outflow tract

Intimal sarcoma of the pulmonary artery is commonly misdiagnosed as chronic pulmonary embolism. Rarely, it can involve the right ventricular outflow tract and the pulmonary valve. We report a patient who was treated surgically for an intimal sarcoma of the pulmonary artery involving the right ventricular outflow tract and the pulmonary valve. The sarcoma recurred in about 8 weeks. It responded favorably to chemoradiation therapy and shows some signs of regression.     

Isolated pulmonary stenosis in an elderly person: Report of a case

We report a rare case of isolated pulmonary stenosis in a 66-year-old woman. The patient underwent successful pulmonary valve replacement with a stentless bioprosthetic valve. The pulmonary valve was exposed with a longitudinal incision from the right ventricular outflow tract to the pulmonary trunk. The proximal posterior part of the bioprosthetic valve was anastomosed to the pulmonary valve annulus and the distal side was anastomosed to the pulmonary artery with an inclusion technique. The defect from the pulmonary artery to the right ventricular outflow tract was closed using an equine pericardium patch. The patient had an uneventful postoperative course.     

Primary repair minimizing the use of conduits in neonates and infants with tetralogy or double-outlet right ventricle and anomalous coronary arteries

OBJECTIVE: The purpose of this study was to review our results with an approach of early primary repair for tetralogy of Fallot or double-outlet right ventricle with anomalous coronary arteries, using several techniques to minimize the use of a conduit. METHODS: Twenty consecutive neonates and infants with anomalous coronary arteries crossing an obstructed right ventricular outflow tract underwent primary repair. Median age was 5.5 months and mean weight 6.22 kg. The anomalous coronary arteries included the left anterior descending from the right coronary artery (n = 10), the right coronary artery from the left anterior descending (n = 1), the left anterior descending from the right sinus (n = 1), and a significant conal branch from the right coronary artery (n = 7) or left anterior descending (n = 1). Two neonates had pulmonary atresia. The right ventricular outflow tract was reconstructed without a conduit in 18 patients, including those with pulmonary atresia. Surgical techniques included main pulmonary artery translocation in 4 patients, transannular repair under a mobilized left anterior descending coronary artery in 2 patients, and displaced ventriculotomy with subcoronary suture lines in 8 patients. In 4 patients the right ventricular outflow tract was repaired via the ventriculotomy and/or pulmonary arteriotomy. A homograft was used as the sole right ventricle-pulmonary artery connection in 1 patient and in another a homograft was added to a hypoplastic native pathway. RESULTS: There have been no early or late deaths. The right ventricular/left ventricular pressure ratio within 48 hours of the operation was 0.47 +/- 0.10. There were 2 reoperations at 8 and 11 years after the operation, during a mean follow-up of 5.2 years (1-11.3 years). CONCLUSIONS: Primary repair of tetralogy of Fallot or double-outlet right ventricle with anomalous coronary arteries can be done in neonates and infants with excellent results. Alternative surgical techniques for right ventricular outflow tract reconstruction, such as main pulmonary artery translocation, can avoid the use of a conduit in most patients.     

Aneurysm of pericardial right ventricular outflow tract patches.

Among 1,022 patients who underwent repair of tetralogy of Fallot, 252 received a pericardial patch of the right ventricular outflow tract; of these, 10 subsequently developed an aneurysm of the right ventricular outflow tract. Cardiac catheterization and angiography revealed moderate pulmonary insufficiency in all patients, a residual pressure gradient in the right ventricular outflow tract in 7, and a residual ventricular septal defect in 2 patients. Reoperation was indicated in 8 patients because of progressive distention of the aneurysm, residual infundibular or pulmonary artery stenosis, and recurrent ventricular septal defect. Reconstruction of the right ventricular outflow tract was accomplished by resection of the aneurysm and insertion of a woven Dacron patch in 5 patients, primary suture of the pulmonary artery in 2, and implantation of a woven Dacron conduit containing a Björk-Shiley cardiac valve prosthesis in 1 patient. There were no early or late deaths. When reconstruction of the right ventricular outflow tract is necessary, we recommend a woven Dacron patch because pericardium may form an aneurysm.     

Palliative right ventricular outflow reconstruction in tetralogy of Fallot with pulmonary atresia and aberrant right subclavian artery; report of a case

A 1-month-old girl weighting 3.1 kg was diagnosed as tetralogy of Fallot (TOF) with pulmonary artery atresia (PA) and aberrant right subclavian artery. Before the operation, pulmonary blood flow from a ducus arteriosus was maintained by lipo prostaglandin E1 (PGE1). The patient underwent palliative right ventricular outflow tract reconstruction (pRVOTR) because the proximal aberrant right subclavian artery was stenotic and the ductus arteriosus and branch of the left pulmonary artery were so close. Postoperative course was uneventful and pulmonary artery showed good growth. The pRVOTR as 1st procedure is a useful method for hypoplastic pulmonary artery to get equal and good growth. Although there are controversies about the size of right ventricular outflow tract (RVOT), 5 or 6 mm diameter of RVOT is recommended for the operative repair of hypoplastic left heart syndrome. We concluded that the pRVOTR should be one of the options as 1st palliative procedure for TOF with PA and diminutive pulmonary artery.     

Management of symptomatic tetralogy of Fallot in the first year of life.

The surgical management of symptomatic tetralogy of Fallot in infants is debatable. The question of total correction versus palliation and the type of palliative procedure remain controversial. During the past 4 years, 30 infants, aged 1 day to 12 months (mean 5.6 months) with symptomatic tetralogy of Fallot underwent either total correction (21 infants) or palliation by relieving the pulmonary stenosis with a right ventricular outflow tract patch (nine infants). The ratio of diameter of the right pulmonary artery to diameter of the ascending aorta (PA/Ao ratio) was calculated from the anteroposterior cineangiogram of all patients. There were three operative deaths in the total correction group; two of these occurred in infants with PA/Ao ratios less than 0.3. One death occurred in the 19 patients undergoing total correction with PA/Ao ratios greater than 0.3 (mortality rate 5.3%). All nine infants undergoing right ventricular outflow tract patching had PA/Ao ratios less than 0.3, and one operative death occurred in this group. Four patients who had right ventricular outflow tract patching have had repeat cardiac catheterization 2 to 15 months postoperatively. All four have shown symmetrical enlargement of the pulmonary arterial tree and significant increases in their PA/Ao ratios.     

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.     

Reoperation after insertion of aortic homograft as a right ventricular outflow tract

Three patients who developed pseudoaneurysm or stenosis of the right ventricular outflow tract after insertion of an aortic homograft between the right ventricle and the pulmonary artery presented the technical problems at reoperation of intimate adherence of the homograft to the sternum. Successful replacement of the defective aortic homograft was accomplished by sequentially employing peripheral venous and arterial cannulation, heart-lung bypass, hypothermia, circulatory arrest, sternotomy, right atrial cannulation, and resumption of bypass.     

Double outlet right ventricle with L-position of the aorta, D-loop, subaortic VSD, and pulmonary stenosis.

We describe a case of double outlet right ventricle with subaortic ventricular septal defect and pulmonary stenosis treated successfully with cardiopulmonary bypass. We consider the clinical history and angiocardiographic and surgical findings of this rare anomaly. We stress the difficulties of reconstruction of the outflow tract of the right ventricle, because of the anomalous pathway of the right coronary artery, the posterior situation of the pulmonary artery, and the abnormal anatomy present in the outflow tract of the right ventricle.     

Coronary artery anomalies in patients with tetralogy of Fallot.

A left anterior descending coronary artery arising from the right coronary artery can be easily injured during performance of a right ventriculotomy for correction of tetralogy of Fallot. This occurred in 2 of the 23 patients in this series, and both patients died from myocardial failure in the early period after operation. Of 19 patients who presented a combination of tetralogy of Fallot and unusual coronary artery distribution, injury to the abnormal coronary artery was avoided by a transverse right ventriculotomy either alone or combined with an upper vertical incision in 17 patients. In 2 patients a Dacron tube graft was inserted between the right ventricular outflow tract and the pulmonary artery. In 2 patients a right ventriculotomy was avoided by closing the ventricular septal defect through a transaortic approach. All 21 patients survived. Before a vertical or longitudinal incision in the right ventricular outflow tract is performed, the coronary artery distribution should always be checked and confirmation made of the normal origin of the left anterior descending branch from the left coronary artery to the left of the pulmonary artery.     

A new operation for ventricular septal defect with aortic incompetence

Two boys, 10 years of age, with ventricular septal defect and severe aortic insufficiency were treated using a new method. An autograft of anterior pulmonary artery wall and the adjacent leaflet and subpulmonary muscle was excised. The excised pulmonary artery segment was used to replace the prolapsed right coronary cusp, and the autograft muscle was used to close the ventricular septal defect. The right ventricular outflow tract and pulmonary valve were repaired using an autologous pericardial patch with an autologous single cusp. The results in both cases were satisfactory.     

Therapeutic considerations in congenital absence of the right pulmonary artery. Use of internal mammary artery as a preparatory shunt

Congenital unilateral absence of a pulmonary artery is rare. When present, it is usually associated with other cardiac malformations. In this communication an infant with coarctation of the aorta, ventricular septal defect and absent right pulmonary artery is reported. After correction of the first two defects, due to persistent respiratory difficulty, the infant had exploration of the right pulmonary hilum in hopes of finding a pulmonary artery remnant and re-establishing blood flow to that lung. A small hilar vessel supplying all three lobes of the right lung was found and as a first stage, to enhance pulmonary arterial growth, a palliative systemic to pulmonary artery shunt was performed utilizing a large internal mammary artery. This is the first report of a case in which the internal mammary artery is used in an infant to establish systemic to pulmonary arterial flow in congenital unilateral absence of a pulmonary artery.     

小儿永存动脉干右室流出道重建

目的 总结小儿永存动脉干(PTA)右室流出道重建治疗经验.方法 2000年1月至2007年12月共行PTA根治手术治疗43例,男26例,女17例.年龄1.5个月～3.8岁;体重3.2～23.0kg.Ⅰ型、Ⅱ型和Ⅲ型PTA分别为26例、11例和6例.18例PTA的肺动脉直接下拖至右室流出道切口上缘相吻合,前壁再用心包补片扩大;8例用Homograft管道、14例用牛颈静脉管道连接远端肺动脉和右心室,重建右室流出道;3例Ⅱ型者,肺动脉后壁用左心耳壁与右室流出道上缘做吻合,前壁再用心包补片扩大.结果 术后所有病儿均生存,5例表现为右心功能不全,2例右肺动脉(RPA)压差37.5～47.3 mm Hg(1mm Hg=0.133kPa),术后17d恢复至35.3mm Hg以下.左心室流出道阶差均小于20.3mm Hg.残余VSD(2mm)1例,乳糜胸1例.5例病儿肺血管阻力高,吸一氧化氮(NO)治疗7d左右后好转.随访3个月～3年,2例病儿RPA残余压差24.0～29.3 mm Hg,均无明显右心室或左心室流出道梗阻.结论 肺总动脉后壁直接与右心室切口作吻合重建PTA的右室流出道,早期和远期效果良好.牛颈静脉的带瓣管道的应用,解决了小尺寸同种带瓣管道来源不足的问题,操作简便.     

Arterial switch operation with a single coronary artery

OBJECTIVE: Our purpose was to evaluate the impact of coronary pattern on survival and reintervention in patients who underwent the arterial switch operation with a single coronary artery. METHODS: We conducted a retrospective analysis of 53 patients with a single coronary artery who underwent the arterial switch operation between 1983 and 2000 at Children's Hospital Boston. Recent follow-up information was obtained for 40 of the 46 long-term survivors (mean follow-up 7.3 +/- 4.5 years). RESULTS: Thirty-five patients had a single right coronary artery, with the left coronary artery posterior to the pulmonary artery in 27. Eighteen patients had a single left coronary artery (16 with the right coronary artery anterior to the aorta). Six of 7 total patients who died had a single right coronary artery; all died before 1992. There were 5 early deaths, all with a single right coronary artery, with 4 deaths due to coronary malperfusion. Survivals for all patients were 91% at 6 months and 87% at 1, 5, and 10 years after the arterial switch operation. Survival figures were lower for patients having a single right ostium with the left main coronary artery posterior to the pulmonary artery compared with all other subtypes (P =.02, log-rank test). Seven patients had reintervention, 4 because of right ventricular outflow tract obstruction, 1 for heart transplantation, 1 for mitral valve repair and 1 for pacemaker implantation. Freedom from reintervention for all patients was 96% at 6 months, 92% at 1 year, 86% at 5 years, and 82% at 10 years after the arterial switch operation, with lower rates for patients having a single left ostium with the right coronary artery anterior to the aorta (P =.0003, log-rank test). CONCLUSIONS: In the current era, the arterial switch operation with a single coronary artery can be performed safely irrespective of the coronary anatomy. Risk of reintervention is higher in patients having a single left ostium with the right coronary artery anterior to the aorta.