False aneurysm of right pulmonary artery. New complication of aorta-right pulmonary artery anastomosis.

The case history of an infant with tetralogy of Fallot who had a Waterston anastomosis performed at the age of 1 year is presented. Subsequent chest roentgenograms revealed a ringlike calcification which was shown at angiography and operation to be due to a false aneurysm of the right pulmonary artery at the site of the Waterston anastomosis. The aneurysm also produced unidirectional obstruction of the right pulmonary artery. A review of the literature suggested that a false aneurysm of the right pulmonary artery after a Waterson anatomosis had not been reported previously.     

False aneurysm of the ascending aorta from an expanded polytetrafluoroethylene (Gore-Tex) aortopulmonary shunt

A false aneurysm of the ascending aorta developed in a 25-month-old male infant with tetralogy of Fallot because of an expanded polytetrafluoroethylene (PTFE) shunt that had been inserted between the ascending aorta and right pulmonary artery when the patient was 3 months of age. Surgical repair of tetralogy of Fallot with ligation of the PTFE graft was performed at 19 months of age. The false aneurysm at the site of the systemic anastomosis to the shunt was discovered 6 months later and was successfully repaired. Because of the potential for graft dehiscence secondary to growth, we recommend that PTFE shunts be removed completely or, at least, ligated and divided at the time of corrective surgical intervention.     

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.     

Thrombosis of the left main pulmonary artery in an infant with tetralogy of Fallot. Treatment by thrombectomy and simultaneous Potts-Smith anastomosis

Thrombosis in the small pulmonary arteries is a common finding in patients dying of severe tetralogy of Fallot. The development of these thrombi are thought to be a result of slow blood flow and polycythemia. A case is described wherein thrombosis of the left main pulmonary artery developed in a six month old infant with tetralogy of Fallot associated with severe hypoxic spells. A successful pulmonary artery thrombectomy was achieved and a Potts-Smith anastomosis performed to the same arteriotomy.     

Repair of tetralogy of Fallot with obstruction of right pulmonary artery; report of a case

Patients with tetralogy of Fallot showing unilateral obstruction of a pulmonary artery, especially the right pulmonary artery, are a high-risk group for pulmonary hypertension after repair. This case of tetralogy of Fallot with the obstruction of the right pulmonary artery received a Blalock-Taussig shunt at 7 months old, and the occluded right pulmonary artery caused empyema after surgery. At 2 years old, a cardiac catheter study showed a pulmonary artery index of 193.6 mm2/m2, so we undertook intracardiac repair. After the repair, she showed a relativery favorable clinical course. Systolic pulmonary artery pressure and right ventricular pressure were about 30 and 50 mmHg, respectively. We considered that tetralogy of Fallot with obstruction of right pulmonary artery could be repaired, as long as the pulmonary artery index was within the limits of indication and the left ventricle was well-developed.     

Surgical correction of tetralogy of Fallot with anomalous origin of left pulmonary artery from the ascending aorta

A surgically corrected case of tetralogy of Fallot with anomalous origin of left pulmonary artery from the ascending aorta is presented. The patient was under-developed 3-year-old girl. She weighed only 1,000 grams at birth and had a systolic heart murmur and slight cyanosis. Echocardiography demonstrated the features of tetralogy of Fallot shortly after birth. Chest roentgenogram showed right ventricular enlargement, but vascular marking of lung was almost normal. Catheterization and cineangiography demonstrated the findings of tetralogy of Fallot. Final diagnosis of anomalous origin of left pulmonary artery from ascending aorta was confirmed by aortography. Surgical correction was accomplished using cardiopulmonary bypass with surface cooling. The left pulmonary artery was detached from the aorta and anastomosed to the main pulmonary artery. Pulmonary valve stenosis and severe infundibular hypertrophy were corrected, and ventricular septal defect was closed with a patch. Postoperative recovery was uneventful. On follow up after surgery, she appears to be in very good condition.     

Surgical Treatment of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery Associated with Tetralogy of Fallot

An 8-year-old boy with anomalous origin of the left coronary artery from the pulmonary artery associated with tetralogy of Fallot, which was definitely diagnosed preoperatively, was operated on with success. Direct implantation of the left coronary artery into the aorta following division of the left coronary artery from the pulmonary artery and, concomitantly, total repair for tetralogy of Fallot using an external valved conduit were performed. Postoperative cineangiogram revealed a hemodynamically well-repaired intracardiac condition and anterograde filling of the left coronary artery, compared with retrograde left coronary flow from intercoronary collateral vessels preoperatively.To the best of our knowledge, there is not a previously published report of anomalous origin of the left coronary artery from the pulmonary artery associated with tetralogy of Fallot that was treated surgically with success.     

Repair of tetralogy of Fallot associated with anomalous origin of right pulmonary artery and accessory left anterior descending coronary artery

We present an unusual combination of lesions in an 8-month-old child diagnosed with tetralogy of Fallot, anomalous origin of right pulmonary artery, and anomalous coronary artery crossing the pulmonary annulus. The association of anomalous origin of branch pulmonary artery from aorta and tetralogy of Fallot (TOF) is extremely rare with an incidence of 0.4%. The incidence of anomalous coronary artery in TOF is 10.3%. However, a combination of all three lesions poses challenges to surgical repair and has not been previously reported.     

Tetralogy of Fallot associated with anomalous origin of the left coronary artery from the pulmonary artery, pulmonary artery hypoplasia and atrial septal defect. A case report

A 7-year-old girl had tetralogy of Fallot with anomalous origin of the left coronary artery from the pulmonary artery, pulmonary artery hypoplasia and atrial septal defect. The case is presented and diagnostic and surgical implications are discussed.     

One-stage correction of tetralogy of Fallot associated with anomalous origin of the left pulmonary artery from the aorta and aortopulmonary window

We report a rare case of tetralogy of Fallot associated with anomalous origin of the left pulmonary artery from the aorta and aortopulmonary window. Successful one-stage total correction was performed under cardiopulmonary bypass, including implantation of the left pulmonary artery, repair of aortopulmonary window, and correction of tetralogy of Fallot. The patient experienced an uneventful postoperative course and was asymptomatic at one-year follow-up.     

A case of a patient with the non-confluent main pulmonary artery with the left pulmonary artery after a left Blalock-Taussig shunt for tetralogy of Fallot, which underwent a successful two staged radical repair

We report on a 14-year-old girl with tetralogy of Fallot and the non-confluent main pulmonary artery with the left pulmonary artery after a left Blalock-Taussig shunt. She underwent a left-sided classic Blalock-Taussig shunt at the age of one. However, she showed repeated dyspnea and increasing cyanosis, and cardioangiography showed the non-confluent main pulmonary artery with the left pulmonary artery. Corrective surgery of tetralogy of Fallot was done by a two-staged operation. In the first stage, a EPTFE prosthesis was grafted between the main pulmonary artery and the left pulmonary artery, and in the second stage, intracardiac operation was performed. The late postoperative status and hemodynamic results were satisfactory and we emphasize the rational for this approach.     

Pulmonary origin of left anterior descending coronary artery in tetralogy of Fallot

We report the case of a 4-year-old girl with an anomalous origin of the left anterior descending coronary artery from the pulmonary artery in association with tetralogy of Fallot. Creation of an intrapulmonary tunnel from a newly created aortopulmonary window to the coronary ostium in the pulmonary trunk and total repair of tetralogy of Fallot were successfully carried out. The advantage of intrapulmonary tunnel technique (Takeuchi method) for this very rare combination is stressed.     

Repeated Repair of Tetralogy of Fallot: Report of 11 Cases and review of the literature

Eleven patients underwent late repeated correction of tetralogy of Fallot in 1991–1993. The previous operation was repair of simple Fallot's tetralogy in seven cases, repair plus transannular patch in one case and repair of tetralogy and pulmonic atresia in three cases. The indications for reoperation were residual ventricular septal defect, right ventricular outflow tract (R.V.O.T.) obstraction, residual branch pulmonary artery stenosis, aneurysmal dilatation of R.V.O.T. Patch or combination of any of the above. At reoperation these defects were corrected. The post operative course was uneventful in eight patients. Two required mechanical ventilation for 2-3 days, and one underwent another operation for residual branch pulmonary artery stenosis. The functional and haemodynamic results were good in ten patients, and one had residual distal pulmonary artery stenosis. There were no death during 2 years of follow-up. Repeated correction of tetralogy of Fallot thus had low postoperative morbidity and good haemodynamic results. For the relatively few patients initially found to have tetralogy of Fallot and pulmonic atresia, the outcome may be less favorable.     

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.     

Pericardial Outflow Prosthesis of the Right Ventricle

In a small group of 5 cases where pericardial outflow prosthesis of the right ventricle and pulmonary artery was used, a significant dilatation was found at the follow up in three cases. In one of these an aneurys-matic formation of the degenerated and calcified pericardial tissue was found thus necessitating a reoperation. Therefore the dacron arterial grafts are preferred for the use as outflow prosthesis in cases of tetralogy of Fallot.

The use of the patient's own pericardium has been recommended by Gross for outflow prosthesis of the right ventricle and the pulmonary artery. In a small group of 5 cases with Fallot's tetralogy the follow-up, however, showed dilatation of the free pericardial graft in 3 cases. In one of these already after 2 years the degeneration with calcific deposits within the pericardium caused a massive aneurysm necessitating reoperation.     

Transfusion-free complex cardiac surgery with cardiopulmonary bypass in a 3.55-kg Jehovah's Witness neonate

Complex cardiac surgery using cardiopulmonary bypass normally requires the transfusion of autologous blood components, particularly in neonates. This is predominately caused by the relatively high priming volume of the circuit with subsequent extreme hemodilution and the often extended and complex perfusions leading to progressive consumption of platelets and coagulation factors. We report on a strategy to minimize the cardiopulmonary bypass circuit and adjust the perfusion technique that resulted in transfusion-free correction of tetralogy of Fallot with an absent pulmonary valve and an aneurysm of the left pulmonary artery in a 3.55 kg Jehovah's Witness neonate boy.     

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.     

Postoperative Studies in Patients with Tetralogy of Fallot

The present series demonstrates that hemodynamic abnormalities persist following intracardiac repair of tetralogy of Fallot despite clinical improvement. The incidence of residual ventricular communication and residual outflow obstruction is consistent with previous reports. The pulmonary incompetence is well tolerated when it exists as an isolated residual abnormality. The use of Teflon cloth to relieve the outflow obstruction has not led to aneurysm formation. This report calls attention to the occurrence of tricuspid insufficiency in patients with tetralogy of Fallot and the increased postoperative morbidity with this combination. Finally, aortic regurgitation is part of the pathophysiology of tetralogy of Fallot and may well affect the results of the intracardiac repair. The long-term consequences of these abnormalities are left to future consideration.     

Aneurysm of the pulmonary artery. Rare long term complication of central aorto-pulmonary shunts for congenital heart disease. Report of two cases with review of the literature

Aneurysm of the left pulmonary artery developed in two patients with tetralogy of Fallot at the site of a Potts' shunt. Although such a complication is known we could not find any previously reported case of successful surgical treatment in the literature. Cardiac catheterization and angiography provided the definitive diagnosis. One patient refused surgical intervention but later presented with fatal rupture and massive hemoptysis. Closure of the Potts' shunt, angioplastic repair of the pulmonary artery aneurysm and total correction of tetralogy of Fallot were successfully accomplished using cardiopulmonary bypass and cardioplegic arrest. Aneurysm should be suspected whenever a mediastinal mass appears in patients with a previous systemic-pulmonary artery shunt.     

Absent right pulmonary artery complicating Tetralogy of Fallot.

A case of tetralogy of Fallot associated with absence of the right pulmonary artery is presented. The patient underwent successful surgical correction. The embryological and clinical aspects of this patient are discussed.