Surgical management of tetralogy of Fallot with absent pulmonary valve

Tetralogy of Fallot and absent pulmonary valve (T-APV) is associated with massively dilated pulmonary arteries that cause tracheobronchial compression in the newborn and heart failure and cyanosis in older patients. Corrective operations have been attended by high mortality rates due to pulmonary insufficiency causing right heart failure (RHF) and pulmonary complications. Pulmonic valve insertion (PVI) with complete repair has resulted in improved survival. During the last 5 years, 152 patients with tetralogy were corrected. Of these, 10 patients (ages 51 days to 34 years) had absent pulmonary valve. One patient (age 51 days) presented with severe RHF and pulmonary insufficiency and 9 patients presented with mild RHF and cyanosis. Chest roentgenograms showed increased cardiothoracic ratio and pulmonary prominence in all. Arteriography revealed massively enlarged pulmonary arteries with a mean ratio of 2:1 for size of pulmonary artery to aorta. Associated pulmonic stenosis and insufficiency was present in all. Seven patients underwent closure of ventricular septal defect (VSD) and PVI. Of these, 3 had PVI (2 tissue and 1 prosthetic) with outflow patch and 4 had right ventricle to pulmonary artery (RV-PA) tissue valved conduits. Two patients had repair without PVI, and 1 had repair with a monocusp pericardial valve patch. Nine patients have done well with no episodes of thromboembolism or infection. Death occurred in a 51-day-old infant who had VSD closure and relief of pulmonic stenosis. Pulmonary valve insertion seems to be indicated in these patients because it lowers peak pulmonary artery pressure and, thus, reduces compression effects on the trachea and bronchi. When PVI was used, RHF did not occur post-operatively.     

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.     

Perioperative management of tetralogy of Fallot with absent pulmonary valve

BACKGROUND: Tetralogy of Fallot with absent pulmonary valve is a rare form of congenital heart disease. The records of patients with this lesion were reviewed over a 6(1/2) year period, and the perioperative management of 13 patients is described. METHODS: We found that our patients could be divided into two groups by age. The younger group failed medical management and was referred for surgery at less than 1 year of age. This group of patients had considerable respiratory disease at the time of surgery, greater ventilatory problems during surgery and prolonged recovery. Most patients in this group have residual respiratory disease. RESULTS: There were significant differences in postoperative outcome between younger versus older patients: days of ventilation 37 +/- 41 versus 1 +/- 0.6 days, length of ICU stay 28 +/- 31 versus 3 +/- 1 days and length of hospital stay 64 +/- 48 versus 9 +/- 5 days.     

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.     

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.     

Congenital quadricuspid aortic valve with tetralogy of Fallot and pulmonary atresia

We report the case of a 4-year-old girl who had quadricuspid aortic valve regurgitation with tetralogy of Fallot and pulmonary atresia. This combination is very uncommon. Aortic valve replacement was performed successfully due to aortic regurgitation which had progressed one year after the total repair. The dilated aortic annulus plus quadricuspid aortic valve may result in progressive aortic regurgitation for a short period.     

Surgical outcomes in the treatment of patients with tetralogy of Fallot and absent pulmonary valve.

OBJECTIVE: Tetralogy of Fallot and absent pulmonary valve (TOF/APV) is associated with significant pulmonary artery dilatation and airway compression. Treatment of infants presenting with respiratory symptoms early in life is associated with high mortality (20-60%). We aim to report our results and identify factors associated with survival and prolonged ventilation. METHODS: We performed a retrospective review of 62 consecutive patients following repair of TOF/APV (1982-2006). Median age at repair was 1.4 years (1 day-35 years). Twenty patients required preoperative intubation. RESULTS: Sixty-one patients underwent complete repair. Thirty-three patients underwent pulmonary artery plication (n=15) or reduction (n=18). The right ventricular outflow tract (RVOT) was reconstructed with valved conduit (n=31), bioprosthetic valve (n=18), monocusp (n=8), or transannular patch (n=4). There were three perioperative and five late deaths. All perioperative deaths were in neonates and before 1995. Five- and ten-year survival was 93+/-4% and 87+/-5%. Mean ventilatory requirements for neonates, infants, and children > or =1 year were 36+/-35, 8+/-8, and 2.6+/-2.4 days (p<0.0001). On multivariable analysis, significant factors associated with prolonged ventilation were neonates (p<0.0001) and preoperative mechanical ventilation (p=0.088). Eight airway reinterventions were needed in seven infants with persistent postoperative airway compromise, pulmonary artery suspension (n=4), innominate artery suspension (n=2), and lobectomy (n=2). Freedom from RVOT reoperation was 89+/-5% and 59+/-9% at 5 and 10 years. There were no significant risk factors for time-related survival or RVOT reoperation on multivariable analysis. CONCLUSIONS: In contrast to children and adults with TOF/APV, neonates and small infants presenting with respiratory symptoms require prolonged ventilation and additional reinterventions for airway compression. Our current surgical approach which includes reduction and suspension of pulmonary arteries, reconstruction of a competent RVOT, and aggressive postoperative ventilatory management to relieve airway obstruction offers satisfactory outcomes.     

Neonatal repair of tetralogy of Fallot with and without pulmonary atresia

Our experience with the arterial switch operation for transposition of the great arteries has confirmed the attainability of excellent results with elective neonatal surgery. Up to this time, we have repaired tetralogy of Fallot during the neonatal period only when symptoms, either severe persistent cyanosis or cyanotic spells, have been present. This review assesses the results of such nonelective neonatal correction of tetralogy between 1973 and 1988. Twenty-seven neonates with either symptomatic tetralogy of Fallot or symptomatic tetralogy of Fallot with valvar pulmonary atresia underwent repair. Mean age at repair was 8 +/- 8.4 days and mean weight was 3.0 +/- 0.7 kg. Unsatisfactory palliative shunts had previously been placed elsewhere in four patients. Twenty-five transannular patches and two conduits were used for reconstruction of the right ventricular outflow tract. There were five deaths in the hospital, three of which were due to avoidable technical problems. All deaths occurred in patients with pulmonary artery (Nakata) index less than 150 mm2/m2. One premature child weighing 2.3 kg displayed an absent pulmonary valve-like syndrome after repair and died late of respiratory complications caused by aneurysmal branch pulmonary arteries. Actuarial survival at 5 years was 74%. There was a single rapidly declining hazard phase for death, with the hazard approaching zero at 1 1/2 years after repair. Actuarial freedom from need for reoperation was 76% at 5 years. Postoperative catheterization of 15 long-term survivors showed right ventricular pressure less than 70% systemic in 13 cases. All patients are symptomatically well and functioning in sinus rhythm 1 to 15 years after repair (mean, 5 +/- 4 years). This experience with neonates with symptoms suggests that, if mortality is lower in the absence of symptoms, elective repair of tetralogy of Fallot could be reasonably undertaken during the first months of life.     

Late pulmonary valve replacement after repair of tetralogy of Fallot

BACKGROUND: Pulmonary regurgitation appears to be well tolerated early after repair of tetralogy of Fallot; however, it may result in progressive right ventricular dilatation and dysfunction necessitating eventual valve replacement. Our objective was to review our experience with late pulmonary valve replacement after complete repair of tetralogy of Fallot.Methods and results: A total of 42 patients (16 female and 26 male) were operated on between July 1, 1974, and January 1, 1998. Mean age was 22 years (range 2-65 years). The mean interval between tetralogy repair and pulmonary valve replacement was 10.8 years (range 1.6 months-33 years). Mean follow-up was 7.8 +/- 6.0 years (maximum 23 years). Indications for pulmonary valve replacement included decreased exercise tolerance in 58%, right heart failure in 21%, arrhythmia in 14%, syncope in 10%, and progressive isolated right ventricular dilatation in 7%. Heterograft prostheses were used in 33 patients and homografts in 9. Five patients underwent isolated pulmonary valve replacement; concomitant procedures performed in 37 patients included tricuspid valve repair/replacement (n = 18), residual ventricular septal defect repair (n = 12), atrial septal defect closure (n = 4), pulmonary artery patch angioplasty (n = 17), and right ventricular outflow tract enlargement (n = 13). One patient died early (2%) of multiorgan failure. There were 6 late deaths, 3 of which were cardiac related. Survival was 95.1% +/- 3.4% and 76.4% +/- 8.9% at 5 and 10 years, respectively. Functional class of patients was improved significantly; preoperatively, 76% of patients were in New York Heart Association class III-IV, and after pulmonary valve replacement, 97% of surviving patients were in class I-II (P =.0001). Moderate to severe reduction in right ventricular function was noted on preoperative echocardiography in 59% and on late echocardiography in 18% (P =.03). Of the 5 patients who had supraventricular arrhythmias before pulmonary valve replacement, 1 had postoperative recurrence and the arrhythmia is controlled with antiarrhythmic therapy; the other 4 are in normal sinus rhythm at late follow-up. Eight patients subsequently underwent pulmonary valve re-replacement without early mortality at a mean interval of 9.0 +/- 4.2 years (range 3.8-16.8 years). Freedom from pulmonary valve re-replacement was 93.1% +/- 4.7% and 69.8% +/- 10.7% at 5 and 10 years, respectively. The only significant risk factor for re-replacement was young age at the time of the initial pulmonary valve replacement (P =.023). CONCLUSION: Late pulmonary valve replacement after tetralogy repair significantly improves right ventricular function, functional class, and atrial arrhythmias, and it can be performed with low mortality. Subsequent re-replacement may be necessary to maintain functional improvement.     

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.     

Current surgical technique to repair Fallot's tetralogy with absent pulmonary valve syndrome

The experience with surgical repair of Fallot's tetralogy and absent pulmonary valve syndrome is reviewed. Twenty-two patients aged 1 day to 8 years were treated between 1982 and 1989 using one surgical technique. This consisted of resection of the main pulmonary artery and large parts of the anterior wall of the right and left pulmonary artery. The ventricular septal defect was closed with a patch, and an aortic or pulmonary homograft (size, 8 to 24 mm) was interposed between the right ventricle and the pulmonary artery. Two of the 8 infants operated on died; there was one late death. All 14 older children survived the operation. All survivors are well up to 7 years follow-up (mean follow-up, 3.6 years). We recommend early treatment of infants; older children can be treated electively. The technique used in our series gives excellent results.     

Compression of the left main coronary artery by a pulmonary artery aneurysm in a patient with tetralogy of Fallot and an absent pulmonary valve

Abstract A case of a 16‐year‐old female with tetralogy of Fallot and absent pulmonary valve is presented, who on coronary angiography and computerized tomography (CT) angiography had severe compression of the left main coronary artery by the dilated main pulmonary artery. The patient was successfully managed by surgical correction of the intracardiac defect, with right ventricular outflow tract reconstruction by the Contegra^® bovine jugular vein conduit. (J Card Surg 2011;26:330‐332)     

Preservation of the pulmonary valve during intracardiac repair of tetralogy of Fallot.

From January 1981 to December 1990, intracardiac repair of tetralogy of Fallot in 148 pediatric patients, with one surgical death, was directed toward preservation of the native pulmonary valve. Using the accepted preoperative angiographic criterion for the pulmonary valve annulus area (PVA) of 1.8 cm2/m2, 85 patients were candidates for transannular right ventricular outflow patch (TAP). However, in 54 patients with a mean PVA of 1.5 cm2/m2 (range 1.06-1.76), the valve was preserved without using TAP because the morphological changes (cusp thickening and annular distensibility) seemed acceptable for preservation in view of its probable hemodynamic efficiency and growth potential. A morphological classification of pulmonary valve changes has evolved. Retrospectively, 24 (77%) of the 31 patients with TAP had moderate to severe cusp thickening and ring rigidity; this incidence was significantly higher (p < 0.001) than that in preserved patients (18 of 54 or 33%). The incidence of morphological changes increased with operative age; that is, 2 of 13 (15%) patients younger than 1 year versus 23 of 40 (58%) patients older than 4 years (p < 0.01). All 54 patients with preserved pulmonary valves were catheterized one month postoperatively. The intraoperative right to left ventricular systolic pressure ratio (RVP/LVP) decreased significantly (p < 0.005) in one month, from a mean of 0.79 (range 0.44-1.36) to 0.57 (range 0.36-0.97). The PVA increased from a mean of 1.5 to 1.9 cm2/m2 (range 1.20-2.65), and the rate of its increase was significantly larger (p < 0.005) as operative age decreased. Pulmonary valve regurgitation of greater than mild degree occurred in 8 of 54 (15%) patients with the valve preserved.(ABSTRACT TRUNCATED AT 250 WORDS)     

Surgical approach to severely symptomatic newborn infants with tetralogy of Fallot and absent pulmonary valve

The surgical management of severely symptomatic newborn infants with tetralogy of Fallot and absent pulmonary valve has been controversial, and the results of a variety of operative approaches have not been satisfactory. We report on a technique for the treatment of these patients, which consists of (1) ligation of the main pulmonary artery to eliminate pulmonary regurgitation, excessive right ventricular stroke output, and secondary pulmonary artery dilation and airway obstruction and (2) insertion of a subclavian-pulmonary artery polytetrafluoroethylene shunt to provide pulmonary blood flow. The procedure was used in four neonates. Two patients operated on at 2 and 3 days of age are doing well 15 and 19 months postoperatively. The other two, operated on at 3 and 4 weeks of age after unsuccessful prolonged medical treatment and positive-pressure ventilation, failed to show long-term improvement and died of sepsis and respiratory failure 3 and 5 months after operation. This experience, though limited, suggests that early surgical intervention to control pulmonary regurgitation prevents progressive pulmonary artery dilatation and secondary bronchial compression, decreases the need for prolonged preoperative and postoperative ventilation, and improves the outcome of these critically ill neonates.