Intermediate results with correction of tetralogy of Fallot with absent pulmonary valve using a new approach.

OBJECTIVE: The intermediate results achieved with a new technique for primary repair of tetralogy of Fallot with absent pulmonary valve syndrome are reported. METHOD: Apart from correction of tetralogy of Fallot, this approach includes translocation of the pulmonary artery anterior to the aorta and away from the tracheobronchial tree. Since November 1998 this technique has been employed in three symptomatic newborns and three infants with a diagnosis of tetralogy of Fallot with absent pulmonary valve. The median age at surgery was 37 days (range 14-256 days). The median weight at operation was 3.4 kg (range 2.9-4 kg). All patients had severe respiratory problems and congestive heart failure with cyanosis. RESULTS: There was no early or late death during the follow-up (median 27 months). One patient required redo due to failure to thrive as a consequence of right ventricle volume overload. Valved conduit was inserted to pulmonary position. Respiratory symptoms disappeared or were significantly reduced in all patients. Postoperative computed tomographic scan showed no compression of trachea and main bronchi, pulmonary artery was away from tracheobronchial tree in all patients. Patients are doing well with adequate growth. CONCLUSIONS: The new technique described here has a potential to reduce or eliminate bronchial compression by pulmonary artery. Translocation of pulmonary artery anterior to the aorta takes the dilated pulmonary artery away from the trachea and bronchial tree. This approach was found to be technically feasible and can be useful especially in symptomatic newborns and infants.     

A new approach to correction of tetralogy of Fallot with absent pulmonary valve

A new technique for primary repair of tetralogy of Fallot with absent pulmonary valve syndrome is suggested. In addition to the standard intracardiac portion of tetralogy of Fallot repair, this approach includes translocation of the pulmonary artery anterior to the aorta, shortening of the ascending aorta, and shortening and plication of the pulmonary artery. This new technique should eliminate compression of the tracheobronchial tree caused by impingement by the pulmonary artery.     

Early medical and surgical intervention for tetralogy of Fallot with absence of pulmonic valve

Tetralogy of Fallot with absent pulmonic valve is a rare but life-threatening syndrome. Children with this complex may have aneurysmally dilated pulmonary arteries which compress the tracheobronchial tree and cause airway compromise and death. This paper reviews the course of 10 children with this syndrome who were seen at our institution from 1962 until the present. Two infants with respiratory distress secondary to tetralogy plus absent pulmonary valve were treated with early aggressive medical intervention followed by operation. After closure of the ventricular septal defect and reconstruction of the right ventricular outflow tract, the pulmonary artery aneurysms were resected. Both children are growing and are well more than 1 year after the operations. Our experience with these two patients suggests that such infants may benefit from this form of therapy.     

法乐四联症肺动脉瓣缺如综合征的外科治疗

手术治疗６例法乐四联症肺动脉瓣缺如综合征。除１例伴左肺动脉缺如者于术后１天死亡外，余５例恢复良好，经随访手术效果满意，作者认为对儿童型无呼吸道症状者可在３－４岁纠治，对婴幼型伴严重呼吸道症状必须早期手术治疗。     

Successful staged repair of a symptomatic neonate of tetralogy of Fallot with severe absent pulmonary valve syndrome

Tetralogy of Fallot with absent pulmonary valve has been classified to a few groups. The most severe one is characterized by symptomatic onset immediately after birth. The others are no or slightly symptomatic at least during their neonatal period. In a severely symptomatic 12-day-old neonate of tetralogy of Fallot with absent pulmonary valve who had required intubation immediately after birth, tight pulmonary banding and left side modified Blalock-Taussig shunt were performed on emergency basis. Consequently, prior massive pulmonary regurgitation was decreased significantly. Forty-five days after this first stage operation, he weaned from respiratory management. At 1-year-old, radical repair based on conotruncal repair, which consisted of patch closure of ventricular septal defect preserving the tricuspid septal leaflet function, resection of anterior wall of enlarged left pulmonary artery, and right ventricular outflow tract reconstruction using autologous tissue and a pericardial patch was performed. Bicuspid pulmonary valve, posterior one of procured autologous pulmonary wall and anterior one of polytetrafluoroethylene (PTFE) respectively, was created to minimize deterioration of the pulmonary insufficiency. Although postoperative cardiac function was kept feasible showing his central venous pressure of 7 mmHg in the main, postoperative general course was eventful especially regarding the respiratory function. The patient was weaned from the prolonged ventilator management 5 months after this radical repair eventually. Generally, to diminish the massive pulmonary regurgitation in early lifetime period could reduce a progressive airway obstruction and minimize pulmonary tissue damage. However, even after the total correction in this case, considerable peripheral segmental pulmonary obstructive lesions were persistent according to the perfusion lung scanning with 99mTc macroaggregated albumin and 99mTechnegas ventilation lung scanning studies. This persistent, supposed to be innate, pulmonary obstructive lesions might prevent ordinal recovery after cardiac radical repair for this most severe subtype of absent pulmonary valve syndrome.     

Two cases of tetralogy of Fallot with absent pulmonary valve repaired by modified Kreutzer’s technique

Two severely symptomatic infants of tetralogy of Fallot with absent pulmonary valve successfully repaired by a modified Kreutzer’s technique are described. In addition to the standard repair of tetralogy of Fallot, our surgical technique consists of extensive pulmonary reduction arterioplasty, which allows tracheobronchial decompression. Both patients tolerated surgery and showed significant relief of airway compression and clinical symptoms, although they required prolonged mechanical ventilatory support. The modified Kreutzer’s technique is relatively simple and could be sufficiently efficacious even for symptomatic infants of tetralogy of Fallot with absent pulmonary valve.     

A case report of compression of right pulmonary artery and bronchus by aneurysmal dilated ascending aorta in tetralogy of Fallot--suspension of ascending aorta

The patient was a 63-days-old boy who was admitted to our hospital because of moderate cyanosis and tachypnea. After admission, severe respiratory distress and emphysematous change of the right lung on the chest X-ray developed progressively. Echocardiogram and angiocardiogram demonstrated that a tetralogy of Fallot associated with right aortic arch and absence of pulmonary valve, and revealed remarkably dilated ascending aorta which compressed the right pulmonary artery and bronchus. Therefore, the emergency operation in that the ascending aorta was suspended to the 2nd rib was performed through a right thoracotomy. After surgery, his respiratory distress and emphysema of the right lung completely disappeared. To our knowledge, this is the 2nd reported case in which suspension of ascending aorta was successfully performed for pulmonary complication in congenital cardiovascular anomalies as this patient.     

Unilateral absence of a pulmonary artery in absent pulmonary valve syndrome: a case report and review of literature.

A six-year-old boy presented to the Cardiology clinic with history of mild cyanosis and dyspnea on exertion from age 1. He had a to-and-fro murmur at the middle left sternal border. Chest examination was normal but chest x-ray showed a small left lung. Echocardiography established the diagnosis of tetralogy of Fallot (TOF) and absent pulmonary valve with severe pulmonary regurgitation and moderate stenosis at the pulmonary valve site. There was severe dilatation of the main and right pulmonary arteries. The left pulmonary artery (LPA) could not be seen. Angiography failed to show a LPA. This case of an absent LPA associated with absent pulmonary valve syndrome is discussed and the literature is reviewed.     

Absent pulmonary valve associated with tetralogy of Fallot and complete atrioventricular septal defect: report of a case.

A male infant with an extremely rare combination of absent pulmonary valve, tetralogy of Fallot and atrioventricular septal defect presented without symptoms of respiratory distress or congestive heart failure. He underwent successful primary repair at the age of 5 months. The procedure consisted of double-patch repair of the atrioventricular septal defect and right ventricular outflow tract reconstruction with a monocusp transannular patch. Resection or plication of a dilated pulmonary artery was not required. The patient is doing well without any symptoms 5 years after repair.     

Anesthetic management for an infant with tetralogy of fallot with absent pulmonary valve

Tetralogy of Fallot with absent pulmonary valve (TOF/APV) is a rare disease, and characterized by massive dilatation of pulmonary artery. Bronchial compression by the aneurysm-like dilated pulmonary artery sometimes results in severe respiratory compromise. Surgery including closure of VSD and right ventricular outflow construction is often needed in infancy. But, even after the surgical correction, patients often suffer from right ventricular failure due to right ventriculotomy and poor development of pulmonary capillaries. Therefore, preventing pulmonary hypertension is important during the perioperative period. We report an infant with TOF/APV. This 3 month-old boy developed respiratory distress suddenly. After the induction of anesthesia with sevoflurane, fentanyl, and vecuronium, continuous infusion of alprostadil and olprinone was started. Cardiopulmonary bypass (CPB) was instituted smoothly. VSD was closed and right ventricular outflow tract was reconstructed. Dilated pulmonary arteries were resected and plicated. Weaning from CPB was easy and postoperative course was uneventful with mild pulmonary hypertension. In the cases predisposed to right ventricular failure like TOF/APV, olprinone may be useful.     

Traumatic papillary muscle rupture--a case report

A previously healthy young man sustained a chest injury. During hospital admission following the accident, he exhibited progressive deterioration in blood gases. The chest X-ray showed pulmonary venous congestion. Positive pressure ventilation was required with a tentative diagnosis of respiratory distress syndrome. However, he remained in NYHA class IV. Trans-esophageal cross-sectional echocardiography showed prolapse of the anterior mitral leaflet with papillary muscle rupture. Mitral valve replacement was performed successfully.     

A case of tetralogy of Fallot with absent pulmonary valve

A 3-month-boy weighing 1,968 gm with absent pulmonary valve associated with tetralogy of Fallot, who suffered from severe respiratory distress, underwent emergency operation. Preoperatively, the patient had atelectasis of left lung, since left main bronchus was compressed by aneurysmal dilatation of the main pulmonary artery. Aneurysmorraphy and suspension of the pulmonary artery with retrosternal fascia was performed successfully. This procedure is a simple and useful method of relief from airway obstruction at the first stage for such critically ill infants.     

Adult respiratory distress syndrome after cardiac surgery

Adult respiratory distress syndrome, characterized by high permeability pulmonary oedema caused by endothelial cell damage, resulting in refractory hypoxemia, has a very high mortality. Cardiopulmonary bypass is said to be responsible for the development of adult respiratory distress syndrome after cardiac surgery. The present study was performed in order to identify predicting and aetiological factors of adult respiratory distress syndrome and multiple organ failure after cardiac surgery. Between January 1984 and December 1993, 3848 patients underwent cardiac surgery with cardiopulmonary bypass in the authors' institution, and were analysed in a retrospective manner. The operations performed were 3444 coronary artery bypass grafts (CABG), 267 valve and 137 combined (CABG + valve) procedures. The incidence of adult respiratory distress syndrome was 1.0% (38 of 3848) with an overall mortality rate of 68.4% (26 patients); 24 of these died from multiple organ failure. Multivariate regression analysis identified hypertension, current smoking, emergency surgery, preoperative New York Heart Association (NYHA) class 3 and 4, low postoperative cardiac output and left ventricular ejection fraction < 40% as significant, independent predictors for adult respiratory distress syndrome. Combined cardiac surgery and diffuse coronary disease were also significant predictors; cardiopulmonary bypass time was not. Thirty-six of the 38 patients that later developed adult respiratory distress syndrome had low postoperative cardiac output, 12 requiring intra-aortic balloon pump support. The remaining two had severe hypotension caused by postoperative bleeding. Twenty-six adult respiratory distress syndrome patients (68%) had confirmed gastrointestinal complications (e.g. intestinal ischaemia). Adult repiratory distress syndrome is a rare complication after cardiac surgery but is associated with a very high mortality. Preoperative predictors were identified. Cardiopulmonary bypass alone was not found to be an important factor. Postoperative low cardiac output leading to splanchnic hypoperfusion may be the most important single factor in developing adult respiratory distress syndrome after cardiac surgery.     

Long-Term Fate Of Valve Cusp Patches For Right Ventricular Outflow Tract Reconstruction

Six patients with tetralogy of Fallot, pulmonary atresia, or absent pulmonary valve syndrome were operated with a RVOT patch containing a single aortic allograft cusp from an adult donor. The method was based on animal work with similar patches implanted in growing puppies and proved that the single cusp functioned as an effective RV outflow valve for several weeks after surgery. Postoperative angiograms in the animals confirmed nearly completely competent valves. Sacrifice of animals at 3 months documented that the allograft tissue was flexible without early deterioration or calcification. Clinical application of this method raised several questions regarding the procedure, its efficacy, and subsequent fate of the allograft valve in children requiring RVOT reconstruction. There were six children age 19 months to 10 years who required division of the pulmonary valve annulus to relieve pulmonary outflow obstruction or with absent pulmonary valve syndrome and compromised airways from pulmonary insufficiency. One patient had a positive culture from the valve and required immediate removal of the infected allograft. Patients had clinically competent pulmonary valves immediately after surgery with decreased right ventricular systolic and end-diastolic pressures when compared with patients in whom nonvalve patches were inserted. All patients had some degree of pulmonary insufficiency, but no symptoms. Follow-up up of five patients available 4 to 6 years after surgery revealed no patients with stenosis of the valve cusps, although two patients have severe, one moderate, and two mild pulmonary insufficiency. We conclude that a patch containing a single adult allograft valve cusp should be considered for all patients requiring reconstruction of the RVOT, particularly if RV overload is expected to compromise the immediate postoperative result.     

Bronchial compression by dilated pulmonary artery. Surgical treatment

Bronchial compression by a dilated left pulmonary artery complicated the postoperative course in two infants: one after a Mustard operation for transposition of the great arteries, restrictive ventricular septal defect, and moderate pulmonary stenosis, the second after correction of tetralogy of Fallot with absent pulmonary valve. In both cases the bronchial compression was successfully treated by division of the left pulmonary artery and its prolongation by interposition of a conduit.     

164例法洛四联症的外科治疗

目的总结164例法洛四联症(tetralogyofFallot,TOF)患者手术治疗的经验,探讨手术时机、危险因素和围术期处理等。方法对164例TOF患者行根治手术,其中单纯右心室流出道漏斗部狭窄37例,漏斗部及肺动脉瓣狭窄14例,主肺动脉及左右肺动脉狭窄113例。合并肺动脉闭锁5例。分别给予自体心包片或自体心包卷成心外管道加宽右心室流出道、同种带瓣血管行右心室-肺动脉连接等处理;合并的心脏畸形作相应的矫正。结果全组手术死亡6例,手术死亡率为3.66%(6/164)。死亡原因严重低心排血量综合征2例,冠状动脉移植后不能脱离体外循环机1例,发生室性心律失常1例,术后发生急性呼吸窘迫综合征2例。术后发生心肺并发症55例,均经治疗后恢复。结论行TOF根治术患者年龄应减小,低心排血量综合征已不是导致术后并发症和死亡的主要原因,术中、术后处理的重点应为预防肺动脉残余梗阻和肺部并发症。     

Anterior translocaton of pulmonary artery without aortic transection in repair of tetralogy of Fallot with absent pulmonary valve.

We developed a technique of translocation of the pulmonary artery anterior to the ascending aorta without transection of the aorta in the repair of tetralogy of Fallot with absent pulmonary valve. Our technique includes detachment of the main pulmonary artery (MPA) from the pulmonary annulus, vertical division of the MPA, anterior translocation of the pulmonary artery with patch augmentation between the vertically divided MPA.     

A case of reoperation 24 years after repair of absent pulmonary valve syndrome with anomalous origin of the left pulmonary artery

We experienced a case of a 38-year-old woman with a persistent cough, 24 years after repair of absent pulmonary valve syndrome with anomalous origin of the left pulmonary artery. The right pulmonary artery was massively dilated, thus it caused the compression of the bronchi, whitch was thought to result in her respiratory symptom. This dilatation of the right pulmonary artery seemed to have progressed because of the following two reasons. The first is the pulmonary hypertension caused by the late reconstruction of the left pulmonary artery. The second is residual pulmonary stenosis and regurgitation after the initial operation without a pulmonary valve insertion. We performed a reoperation consisting of reconstruction of the right ventricular outflow tract using a valved conduit and plication of the right pulmonary artery. Her postoperative course has been without any complications and satisfactory for the past 2 years.     

Innovative technique for repair of Tetralogy of Fallot with absent pulmonary,valve syndrome using autologous pericardial patch with monocusp valve

Background  Conventional surgery for absent pulmonary valve is repair with valve conduit between right ventricle (RV) and Pulmonary artery (PA).We describe a technique of repairing absent pulmonary valve with autologous pericardial patch with monocusp valve, without using valve conduit and its results. Methods  From February 2004 to September 2006, 14 consecutive patients with absent pulmonary valve syndrome (APVS) were repaired using this technique. Age range was 5–168 months (median- 24 months) and weight range was 6–31 kgs (median-10 kgs), 5 were infants. Varying degree of respiratory symptoms were present in all patients including tachypnoea, difficulty in feeding, wheezing, recurrent pneumonia and cyanosis. Repair consisted of ventricular septal defect closure, relief of right ventricular outflow obstruction with autologous pericardial patch with monocusp valve. PA aneurysmorrhaphy and PA reduction arterioplasty were done in selected cases. Result  There was one mortality. The follow up ranged from 12 months to 36 months, respiratory symptoms disappeared or were significantly improved in all patients. Two dimensional (2D) Echo showed trivial to mild pulmonary regurgitation (PR) in 11 patients & moderate in two patients. One patient had minimal right ventricular outflow tract (RVOT) obstruction. None of them required reintervention. Conclusion  New innovative technique of using autologous pericardial patch with monocusp valve in absent pulmonary valve syndrome can be safely performed in infants and children. It avoids conduit related problems like reintervention and is economical. Our early and midterm results are encouraging, however long term results are awaited.     

A case of reoperation 24 years after repair of absent pulmonary valve syndrome with anomalous origin of the left pulmonary artery]

We experienced a case of a 38-year-old woman with a persistent cough, 24 years after repair of absent pulmonary valve syndrome with anomalous origin of the left pulmonary artery. The right pulmonary artery was massively dilated, thus it caused the compression of the bronchi, which was thought to result in her respiratory symptom. This dilatation of the right pulmonary artery seemed to have progressed because of the following two reasons. The first is the pulmonary hypertension caused by the late reconstruction of the left pulmonary artery. The second is residual pulmonary stenosis and regurgitation after the initial operation without a pulmonary valve insertion. We performed a reoperation consisting of reconstruction of the right ventricular outflow tract using a valved conduit and plication of the right pulmonary artery. Her postoperative course has been without any complications and satisfactory for the past 2 years.