Surgical treatment of right ventricular outflow tract obstruction after Tetralogy of Fallot {S,D,I} repair

Situs solitus of viscera and atria, D-looped ventricle with atrioventricular concordance, and inverted normally related great arteries with ventriculoarterial concordance characterize the segmental anatomy of an extremely rare {S,D,I} variant of Tetralogy of Fallot. The main pulmonary artery is rightward of the aorta and the right coronary artery crosses over the right ventricular outflow tract from left to right to reach the right atrioventricular groove. Our patient had undergone a neonatal repair due to persistent cyanosis with the implantation of a pericardial infundibular patch after the diagnosis of Tetralogy of Fallot {S,D,I}. He presented with an annular right ventricular outflow tract obstruction at 19 months of age. We describe the surgical approach employing a transannular patch implantation for the relief of the annular right ventricular outflow tract obstruction.     

Complete atrioventricular canal and tetralogy of Fallot with pulmonary atresia

Our patient was diagnosed with complete atrioventricular canal and Tetralogy of Fallot with pulmonary atresia at the age of 1 month. Then he underwent right and left Blalock-Taussig shunts at the ages of 2 months and 5 years, respectively. His cyanosis had increased at 20 years of age. Cardiac catheterization showed occlusion of the left Blalock-Taussig shunt and absence of the left pulmonary artery. Lung perfusion scintigram showed late phase perfusion in the left lung. Chest computed tomographic scan demonstrated the left pulmonary artery. We describe the operative technique of total correction.     

Ebstein's anomaly of the tricuspid valve in combination of tetralogy of Fallot: total correction in infancy

Ebstein's anomaly of the tricuspid valve in combination with Tetralogy of Fallot is extremely rare. We report a patient with these combined entities in a baby presented with cyanosis and right heart failure soon after birth. Total correction toward biventricular repair was successfully conducted when he was 7 months old. For management of a patient with such rare complex anomalies, we recommend Carpentier's technique for Ebstein's anomaly to restore the morphology of the right ventricle, and "one and half ventricle repair" can be a salvage procedure if the right ventricle is not functionally tolerant.     

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.     

Coronary artery obstruction due to membranous ridge of the right sinus valsalva associated with Tetralogy of Fallot: syncope mimics anoxic spell

We report an infant with Tetralogy of Fallot who had suffered from repetitive attacks of syncope without obvious cyanosis. Careful observation by means of echocardiography and angiography revealed that the attacks resulted from acute coronary artery obstruction due to membranous ridge covering the sinus Valsalva. Surgical resection of the abnormal ridge and repair of Tetralogy of Fallot successfully improved the patient's symptoms. Syncope in children should be extensively investigated to exclude obstruction of the coronary arteries.     

Tetralogy of Fallot: T-shaped infundibulotomy for pulmonary valve-sparing procedure

This new and easily reproducible pulmonary valve-sparing technique for the correction of Tetralogy of Fallot is based on a conservative management of the native pulmonary valve to preserve its growth potential. From July 2015 to December 2019, 67 children presenting with a Tetralogy of Fallot were operated consecutively in a single centre using this technique in all cases. A T-shaped infundibulotomy is used to release the anterior pulmonary annulus from any muscular attachment. After myocardial resection and ventricular septal defect closure, an extensive commissurotomy is achieved. Finally, the right ventricular outflow tract remodelling is completed by a shield-shaped bovine patch with an oversized square superior edge, attached directly on the pulmonary valve annulus, with an effect of systolic traction. Sixty patients (89.5%) had a Tetralogy of Fallot repair with preservation of the pulmonary valve. To date, with a median follow-up of 38.2 [14–64] months, no patient has needed a surgical or interventional procedure for pulmonary valve stenosis or regurgitation, with low residual gradients. This procedure could provide a significant increase in native pulmonary valve preservation. Long-term studies are needed to assess pulmonary valve growth and the consequent reduction in surgical or interventional reoperations.     

Tetralogy of fallot: a surgical perspective

Tetralogy of Fallot (TOF) is an index lesion for all paediatric and congenital heart surgeons. In designing an appropriate operation for children with TOF, the predicted postoperative physiology must be taken into account, both for the short and long term. A favourable balance between pulmonary stenosis (PS) and pulmonary insufficiency (PI) may be critical for preservation of biventricular function. A unified repair strategy to limit both residual PS and PI is presented, along with supportive experimental evidence. A strategy for dealing with coronary anomalies and some comments regarding best timing of operation are also included.     

Biventricular repair with end-to-side aorta to pulmonary artery central shunt for ventricular septal defect, severe pulmonary stenosis with hypoplastic pulmonary artery

The case was 11-month-old girl diagnosed as tetralogy of Fallot with severe pulmonary valve stenosis and suffering from severe cyanosis. A catheter study demonstrated pulmonary artery (PA) was well arborized, but severely hypoplastic in spite of previous transcatheter pulmonary valve dilatation; PA index was calculated as 69 mm2/m2. A central end-to-side aorta to PA shunt was created. Cyanosis was well improved, but congestive heart failure occurred after 1 month from the operation. Subsequent catheter study demonstrated pulmonary artery growth, 166 mm2/m2 of PA index and major aortopulmonary collateral artery (MAPCA) coil embolization was performed. Patient underwent Rastelli type definitive repair 9 month after palliation. The central end-to-side aorta to PA shunt is reported as useful measure for promoting PA size increase and definitive repair achievement in patient with pulmonary atresia, ventricular septal defect and severely hypoplastic PA. Appropriate consideration of criteria, carefull follow up and treatments are necessary.     

Tetralogy of Fallot with Coronary Artery to Pulmonary Artery Fistula and Unusual Coronary Pattern: Missed Diagnosis

Abstract  Coronary arteries to pulmonary artery fistulas are rare in patients with Tetralogy of Fallot. Even rarer is the occurrence of these fistulas from a solitary coronary system. We report one such patient in whom the preoperative diagnosis was missed. The importance of a high index of suspicion for accurate preoperative diagnosis is discussed.     

A case of tetralogy of Fallot with pulmonary aneurysm

A case of pulmonary artery aneurysm associated with tetralogy of Fallot is presented. A 28-year-old man was admitted for evaluation of cyanosis and cardiac murmur. Preoperative angiography revealed tetralogy of Fallot and an aneurysm of the main pulmonary artery aneurysm. Operation consisted of pulmonary artery aneurysmectomy and total correction of the tetralogy of Fallot. Microscopic examination of the resected aneurysm showed the thickening of intima and fragmented elastic fibers of media. A brief review of the literature emphasizes the necessity of the surgical intervention.     

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.     

Complete Surgical Repair of Tetralogy of Fallot in Adults,Is It Ever Too Late?

A minority of patients with Tetralogy of Fallot (TF) survive into adulthood. These patients have been subjected to a prolong period of cyanosis and hypoxia. The aim of this study is to assess the benefits of total correction of TF in this adult population. From August 1995 to March 2005, fifty one patients underwent total correction of TF. The mean age was 22.2 years (range 16 to 38 years). There were 31 males and 20 females. Twenty two percent of patients were in NYHA functional class III prior to the operation. The mean gradient across the right ventricular outflow tract was 81.7 mmHg (range 30 to 130 mmHg). The operation was performed through the right ventricle in the majority of patients. Transannular patch was used in 33 patients. The mean follow-up period was 42 months ranging from 1 to 116 months. Hospital mortality was 1.9% (1 patient), and one patient died three months after the operation. Post-operatively 87.3% of patients were in NYHA functional class I. During the follow-up period four patients required re-operation; two for residual ventricular septal defect, one for residual pulmonary stenosis and one had pulmonary valve replacement for severe pulmonary regurgitation. Complete repair of TF in adults is feasible with acceptable mortality and morbidity. The main benefit of the operation is functional improvement in this patient population.     

A rare case of tetralogy of fallot with congenital tubercular bronchiectasis

A 15% of children with congenital heart disease also have an extracardiac abnormality.¹ Tetralogy of Fallot is occasionally associated with various well known extracardiac malformation. We report a rare extracardiac associatation of congenital bronchiectasis and Tetralogy of Fallot presented in late childhood with breathlessness on exertion and frequent respiratory infections. This late presentation was probably due to less destruction caused by oligemic lung of Tetralogy of Fallot. She was successfully managed by intracardiac repair and left upper lobectomy in a single setting.     

Surgical treatment of tetralogy of Fallot with unilateral absence of a pulmonary artery

Tetralogy of Fallot with unilateral absence of a pulmonary artery imposes more marked symptomatology and a much shorter life-span in comparison with the usual tetralogy of Fallot. The operative mortality with total correction has been 50%, and the survivors require prolonged hospitalization for control of severe right heart failure.     

Complete repair of Tetralogy of Fallot with absent pulmonary valve including the role of airway stenting

Tetralogy of Fallot (TOF) with absent pulmonary valve (APV) represents an extreme form of tetralogy where pulmonary insufficiency and mild annular stenosis often results in massive pulmonary arterial (PA) dilatation. The aneurysmal left and right PAs often compress the adjacent trachea and bronchi, leading to airway obstruction and respiratory failure in infancy. Between 1991 and 1997, 11 patients underwent a single stage repair of TOF and APV using a valved (10 patients) or nonvalved (1 patient) homograft conduit and PA reduction arterioplasty. There was one (1/11 [9.1%) perioperative and one (9.1%) late death. Both deaths were related to airway complications. Morbidity associated with postoperative respiratory complications and ventilator-dependency due to underlying tracheobronchomalacia is an important problem. Intermediate follow-up shows a high incidence of reintervention for conduit stenosis and/or insufficiency and tracheobronchial compression. These infants also required multiple hospitalizations for recurrent respiratory infections secondary to their tracheobronchomalacia. Stenting of the right and left main bronchi with balloon expandable metallic stents is a new experimental therapy that has been useful in two recent patients with respiratory failure despite satisfactory intracardiac repair. It may provide an attractive alternative therapy to prolonged mechanical ventilation with positive end expiratory pressure in patients with severe tracheobronchomalacia. Complete repair with a valved homograft conduit and reduction pulmonary arterioplasty in infancy at the time of diagnosis is the procedure of choice for infants with TOF with APV. With this approach the patient outcome is essentially determined by their airway status and airway management.     

A case report of total repair in a 71-year-old patient with tetralogy of Fallot

A 71-year-old male is presented as ever the oldest patient of tetralogy of Fallot who underwent successful radical surgery. Heart murmur was pointed out at the age of 10 years. The patient consulted us because of dyspnea and cough, and was noted to have cyanosis and clubbing fingers. Polycythemia was also detected by hemoglobin of 20.8 g/dl and hematocrit of 58.4%, and a low PaO2 of 48.5 mmHg at room temperature was pointed out. Preoperative echocardiography and cardiac catheterization indicated a ventricular septal defect, overriding of the aorta, and right ventricular outflow tract stenosis with a pressure gradient of 115 mmHg between the right ventricle and the main pulmonary artery. Under cardiopulmonary bypass, the ventricular septal defect was closed with a dacron patch and the right ventricular outflow tract was enlarged by a patch of collagen-coated vascular graft with a commissurotomy of the pulmonary valve. Postoperatively, cyanosis disappeared and the pressure gradient was decreased to 26 mmHg.     

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.     

Pulmonary valve replacement with a mechanical prosthesis. Promising results of 28 procedures in patients with congenital heart disease.

OBJECTIVE: Pulmonary valve replacement is performed increasingly late after correction of Tetralogy of Fallot. Most reports deal with pulmonary allografts as the valvar substitute of choice, although late deterioration and reoperation(s) are the rule. Mechanical valves are scarcely reported and if so only because of complications. Although life-long anticoagulation therapy is indicated for mechanical prostheses, the chance of subsequent re-operations can be expected to be low. We report the results of 28 mechanical valve replacements in the pulmonary position. METHODS: A mechanical valve was implanted in 27 of 79 patients indicated for pulmonary valve replacement. Tetralogy of Fallot was the most common basic lesion. The results and follow-up were reviewed retrospectively, where death and re-operation were primary end points. Routine outpatient follow-up, including trans-thoracic echocardiography, was performed. RESULTS: Twenty-eight pulmonary valve implantations were done in 27 patients. Thirty-day hospital mortality was 1/28 (3.6%), because of a cerebro-vascular accident. One patient died late (2.8 years postoperatively). Median age was 33 years and the median interval between primary repair and insertion of the prosthesis was 26 years. Freedom from re-operation at 1 year was 100%. One valve had to be replaced 14 years after implantation because of malfunction due to ingrowth of endomyocardial fibrosis. No thrombo-embolic events were observed. CONCLUSIONS: Our series do not confirm the bad reputation of mechanical valvar prostheses in the pulmonary position. On the contrary they perform well and result in a much lower re-operation rate than can be expected and in fact is reported after allograft usage. No thrombo-embolic complications were noted. In our experience pulmonary mechanical valve prostheses do well.     

Diagnosis of a left-sided superior vena cava during placement of a pulmonary artery catheter

We report a case of a left sided superior vena cava (SVC) that was diagnosed during placement of a pulmonary artery (PA) catheter. After entering the left internal jugular, the PA catheter passed into the left side of the heart, through the aortic valve, and into the aorta. This was an unusual cause of right-to-left shunting and persistent cyanosis in a patient who had undergone two open cardiac procedures, including repair of an atrial septal defect. Cardiac catherization and echocardiography also failed to reveal the abnormality.

The embryology and physiology of a left sided SVC is reviewed, including an historical perspective. A discussion of the variants of the syndrome is included, as is a review of aberrant placement of central venous catheters.     

Tetralogy of Fallot: anatomic variants and their impact on surgical management

Tetralogy of Fallot is the most common form of cyanotic congenital heart disease. In this condition, episodic worsening of hypoxemia results from dynamic shifts in physiology, so-called "Tet spells." The relative frequency of this lesion and the risks of exacerbating "Tet spells" make anesthetic management of this patient population challenging. The conduct of palliative and reparative cardiac surgery is determined in large part by the anatomic variations within the spectrum of this disorder, most notably the severity of right ventricular outflow tract obstruction. This review will address the impact that the anatomic substrate has on the perioperative management of this interesting patient population.