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 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.     

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)     

Absent pulmonary valve syndrome with aortic regurgitation in a 50-year-old man

A 50-year-old man was diagnosed as having absent pulmonary valve syndrome with aneurysmal dilatation of the pulmonary arteries. He also had a large subaortic ventricular septal defect and severe aortic regurgitation. He underwent successful intracardiac repair and aortic valve replacement. This case is presented in view of its rarity and controversies in the surgical management.     

Growth of the pulmonary anulus and pulmonary arteries after the Waterston anastomosis.

Twenty-six patients with tetralogy of Fallot underwent angiocardiography before a Waterston shunt and again several years later, prior to complete repair (mean interval 40 +/- 15.6 [SD] months). The ratio between the diameter of the pulmonary valve anulus (PVA) and that of the descending thoracic aorta (Ao) increased by 0.32 +/- 0.233, significantly more than is reported to occur after a Blalock-Taussig anastomosis (p = 0.0001). This increase had some relation to the interval between the two angiographic studies (r = 0.34, p = 0.08) and to the mean pressure in the main pulmonary artery (MPA) at the time of the second study (r = 0.35, p = 0.08). The ratio of diameter of the right (RPA) and left pulmonary arteries (LPA) and that of the Ao also increased by 0.24 +/- 0.226 and 0.20 +/- 0.201, respectively. These data suggest that an initial large aortopulmonary shunt may result in less need for transannular patching at complete repair.     

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 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.     

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.     

Pulmonary homograft implantation for ventricular outflow tract reconstruction: early phase results

The pulmonary valve homograft (PH) has been reported to have potential advantages over the aortic valve homograft, including a larger diameter, a thinner wall, and decreased intrinsic calcification. From January 16, 1986, to July 14, 1987, eight consecutive patients underwent repair of congenital cardiac anomalies using a cryopreserved PH. Patients ranged in age from 18 months to 32 years. Diagnoses included tetralogy of Fallot with pulmonary atresia (3 patients); tetralogy with absent pulmonary valve (1 patient); corrected transposition with pulmonic stenosis (1 patient); transposition of the great arteries, ventricular septal defect, and pulmonic stenosis (2 patients); and double-outlet right ventricle with pulmonic stenosis (1 patient). The PH was implanted orthotopically in the patient with absent pulmonary valve, and in the other 7 it was placed as a valved extracardiac conduit. Two of the tetralogy patients with severe bifurcational pulmonary stenosis and another with nonconfluent pulmonary arteries and origin of the left pulmonary artery from a patent ductus arteriosus had their repairs facilitated using the branching pulmonary arterial portion of the PH. There were no hospital or posthospital deaths. Postrepair right ventricular to left ventricular systolic pressure ratios were a mean of 0.35 at 18 hours postoperatively (range, 0.21-0.61). All patients were studied with Doppler and echocardiography after repair. The mean gradient across the PH was 9 mm Hg (range, 2-27 mm Hg), and no pulmonary valve incompetence was present. One patient (12.5%) required reoperation seven months after repair for conduit revision due to compression by the sternum and is now well.(ABSTRACT TRUNCATED AT 250 WORDS)     

Aortic pulmonary autograft implant: medium-term follow-up with a note on a new right ventricular pulmonary artery conduit

BACKGROUND: The Ross operation has been applied to various aortic valve pathologies, particularly when somatic growth is an issue. However, associated cardiac disease and technical problems may limit its use with regard to associated procedures and issues of right ventricular outflow reconstruction. MATERIALS AND METHODS: From December 1992 to March 1998, 24 patients underwent aortic pulmonary autograft implantation. There were 14 males and 10 females, 15+/-10 years of age (mean +/- SD) (range 1 to 50 years), weighing 42.8+/-20 kg (mean +/- SD) (range 8 to 78 kg). Aortic insufficiency was present in 15 (62.5%) patients, stenosis in 8 (33.3%) patients, and valvar stenosis associated with left ventricular outflow tract obstruction in 1 (4.1%) patient. Etiology was rheumatic in 17 patients and congenital in 7. The Ross procedure was accompanied by a partial-Konno left ventricular outflow enlargement in one patient, and mitral valve annuloplasty, mitral commissurotomy, and tricuspid valve replacement in three other patients, respectively. The right ventricular outflow was reconstructed with a valved pulmonary homograft in 14 patients and with a Shelhigh No-React porcine pulmonary conduit in 10 patients. Evaluation was done by New York Heart Association (NYHA) Class and by echocardiography at a follow-up of 22.8+/-24 months (mean +/- SD) (range 3 to 63 months). RESULTS: There were no operative mortalities and no postoperative arrhythmias. One (4.1%) patient required intra-aortic balloon pump (IABP) support for 3 days, one (4.1%) patient died 2 years later of probable arrhythmia, and one (4.1%) patient required mechanical aortic valve replacement 2 years later for severe autograft insufficiency. Left ventricular ejection fraction was unchanged (preoperative 62.4%+/-30%, postoperative 64.2%+/-30% [mean +/- SD], [p = NS]) and no significant gradient was documented by echocardiographic Doppler in the right and left ventricular outflow tracts. The aortic insufficiency scale decreased from a mean of 3.9+/-0.2 to a mean of 1+/-0 (p < 0.01). NYHA Class decreased to I in all patients, from III (10) and II (14). CONCLUSIONS: The pulmonary autograft in the aortic position is suitable for aortic valve replacement in pediatric and adult patients with good medium-term results and in patients with rheumatic etiology, and it provides a desirable solution in the presence of associated pathologies, such as left ventricular tract obstruction or associated multivalvular disease. The development of new means of right ventricular outflow reconstruction must parallel the progress achieved for the left side.     

Postoperative prone position management of tetralogy of fallot with absent pulmonary valve syndrome

Tetralogy of Fallot with absent pulmonary valve syndrome is commonly associated with respiratory failure both before and after surgery. This report describes our experience using prone positioning with bilateral pillows to avoid compression of the anterior chest wall after surgery. In the case here, the patient’s respiratory distress was improved by this positioning. Prone position and avoiding anterior chest compression has an effect on severe respiratory distress of tetralogy of Fallot with absent pulmonary valve syndrome.     

肺动脉瓣缺如综合征的外科治疗

目的总结肺动脉瓣缺如综合征的外科治疗经验。方法1985年1月至2003年9月，纠治肺动脉瓣缺如综合征18例中男13例，女5例。伴法洛四联症17例，伴室间隔缺损1例。全组均在中低温体外循环下行纠治手术，即切除、折叠整形扩张的肺动脉；解除右心室流出道梗阻同时关闭室间隔缺损。7例肺动脉瓣处置单瓣。结果术后死亡1例，死亡率5．6％。术后并发低心排血量综合征3例，Ⅲ度房室传导阻滞和心包积液各1例，经治疗均痊愈。术后随访0．5—5．0年，轻度残余右室流出道梗阻1例、轻度肺动脉瓣反流7例、中度肺动脉瓣反流3例、重度肺动脉瓣反流伴充血性右心衰1例。结论婴幼儿型肺动脉瓣缺如需立即手术，在解除右室流出道梗阻和肺动脉做切除、折叠整形时，须在肺动脉瓣区置入单瓣或带瓣管道。儿童型肺动脉瓣缺如选择性根治，处理原则同普通伴发畸形。     

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) presenting with ventricular fibrillation in an adult: a case report

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly. The usual clinical course is severe left sided heart failure and mitral valve insufficiency presenting during the first months of life. However, in some cases collateral blood supply from the right coronary artery is sufficient and symptoms may be subtle or even absent. Arrhythmias or sudden cardiac death in adult life may be the first clinical presentation in patients with ALCAPA. We report a case, where a 39-year old woman presented with ventricular fibrillation during phycial exertion. Coronary angiography and CT-angiography revealed an anomalous origin of the left coronary artery, and an aortic reimplantation of the left coronary artery was performed followed by ICD implantation. A review of the literature on ALCAPA is presented along with CT images before and after surgery.     

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.     

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.     

A report of two cases of absent pulmonary valve syndrome

Two infants, aged 8 and 18 months, with absent pulmonary valve syndrome unaccompanied by severe pulmonary symptoms or roentgenographic evidence of emphysema underwent a corrective operation consisting of infundibular muscle resection, patch closure for ventricular septal defect (perimembranous outlet type), and right ventricular outflow tract reconstruction with a monocusp patch. The patients tolerated pulmonary regurgitation relatively well, although aneurysmal dilatation of the pulmonary arteries was present preoperatively. Since the postoperative course was uneventful, we think that, in milder cases of this syndrome, a pulmonary valve prosthesis, the insertion of which is advocated by some authors, is not essential, and that right ventricular outflow tract reconstruction with a monocusp patch may suffice in selected cases.     

Repair of an aneurysm of the pulmonary trunk in a 65-year-old patient

The case of a 65-year-old patient with asymptomaticaneurysm of the pulmonary trunk associated with severe insufficiency of the pulmonary valve and symptomatic coronary artery disease is presented. The surgical procedure included coronary artery bypass grafting, aneurysmectomy, and pulmonary artery replacement with implantation of a stentless bioprosthesis and lengthening of the root of the bioprosthesis with a reversed vascular Y prosthesis, which was anastomosed to the left and right pulmonary artery.     

Anomalous left coronary artery from pulmonary artery with mitral stenosis

The usual presentation of anomalous left coronary artery from pulmonary artery is severe left-sided heart failure and mitral valve insufficiency presenting during the first months of life. The manifestations of left heart failure may be masked if pulmonary artery pressure remains high. We believe this is a rarest of rare case of anomalous left coronary artery from pulmonary artery with severe mitral stenosis and pulmonary hypertension in which pulmonary hypertension, along with good collateral circulation helped to preserve left ventricular function.     

Preservation of systemic tricuspid valve function by pulmonary conduit banding in a patient with corrected transposition of the great arteries

Systemic tricuspid valve regurgitation increases mortality and morbidity in patients with a corrected transposition of the great arteries. A 17-year old male with a physiologically corrected transposition after the closure of a ventricular septal defect and conduit placement between a morphological left ventricle and pulmonary artery presented with exertional dyspnoea. The transthoracic echocardiography showed a severe conduit stenosis, and cardiac catheterization revealed a pressure gradient of 114 mmHg. The patient underwent conduit re-replacement using a pulmonary heterograft. Intraoperative transoesophageal echocardiography revealed an acute severe tricuspid regurgitation after a conduit re-replacement. Pulmonary conduit banding was performed under transoesophageal echocardiography guidance, during which the left ventricular to right ventricular pressure ratio increased from 0.33 to 0.60 and the degree of tricuspid regurgitation decreased mildly. The patient was discharged uneventfully at postoperative day 16. Conduit banding might be a useful technique to preserve the systemic tricuspid valve function during conduit re-replacement in patients with a corrected transposition.     

Surgically created double-orifice left atrioventricular valve: a valve-sparing repair in selected atrioventricular septal defects

OBJECTIVES: Some features of the left atrioventricular valve (large mural leaflet, dystrophic tissue) represent a challenge for repair of atrioventricular septal defects without postoperative regurgitation. A retrospective study was conducted to evaluate the results of surgically creating a double-orifice left atrioventricular valve in such circumstances. Clinical results were analyzed according to valvular and subvalvular left atrioventricular valve measurements in pathologic specimens with atrioventricular septal defects. METHODS: Among 157 patients operated on for atrioventricular septal defect since October 1989, 10 patients underwent primary repair (n = 8) or reoperation (n = 2) by this procedure. Median age at repair was 3.3 years (0.1-33 years). Anatomic types were complete (n = 3), intermediate (n = 5), and partial (n = 2). Preoperative moderate to severe left atrioventricular valve regurgitation was present in 6 patients. After the repair (two-patch technique in complete atrioventricular septal defect, cleft closed in each case), these 10 patients were found to have moderate to severe residual regurgitation not amenable to repair by annuloplasty. The top edge of the mural leaflet was anchored to the facing free edge of the cleft. RESULTS: No hospital death or morbidity was observed. Left atrioventricular valve regurgitation was absent or trivial (8 patients) and mild (2 patients). Color-coded echocardiography did not show significant left atrioventricular valve stenosis. The mean diastolic pressure gradient across the left atrioventricular valve was 3.2 +/- 1.1 mm Hg (1.4-4.5 mm Hg). At a median follow-up of 72 months (6-91 months), there was 1 late death, unrelated to left atrioventricular valve malfunction, due to pulmonary vascular obstructive disease. Left atrioventricular valve regurgitation did not increase over time, except in 1 patient in whom regurgitation recently progressed from mild to moderate. At rest, the mean diastolic pressure gradient across the left atrioventricular valve was 3.8 +/- 2.9 mm Hg (1.5-11.2 mm Hg). One child had an early moderate stenosis without pulmonary hypertension. Studies on pathologic specimens (n = 34) indicated that long chordal lengths and large mural leaflet size are essential independent anatomic features to assess its feasibility. CONCLUSIONS: Surgical creation of a double-orifice left atrioventricular valve is an effective additional procedure for repair of atypical cases of atrioventricular septal defect. The operation may decrease the need for reoperation or left atrioventricular valve replacement.