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.     

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.     

法乐四联症心肌纤维扫描电镜的观察

为选择法乐四联症手术的适宜年龄，应用扫描电子显微镜对３０例法乐四联症病人的右室流出道心肌纤维进行观察，结果可见多数心肌纤维排列紊乱、增生、肥大；肌纤维表面横纹的宽度粗细不均，有的异常增宽、增高。细胞表面凹凸不平，在肌膜下可见异常大量堆积的线粒体。心肌纤维内的肌原纤维排列方向紊乱，致使横小管不在同一水平。心肌间质明显增生。随着年龄的增长，上述改变愈重。因此法乐四联症病人，在条件允许下，应尽早手术     

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.     

Twenty-to-thirty-seven-year follow-up after repair for Tetralogy of Fallot.

OBJECTIVE: To describe the long-term prognosis after repair of Tetralogy of Fallot with pulmonary stenosis beyond 20 years. METHODS: One hundred and eighty five patients underwent corrective repair of Tetralogy of Fallot at Rigshospitalet in Copenhagen between January 1960 and July 1977. Ninety seven patients had undergone a palliative operation prior to Tetralogy of Fallot repair. All the 125 patients who were discharged from the hospital were traced through the population register and the patients alive July 1997 were contacted by mail and/or telephone and questioned about use of medicine, professional status, family status and ability to perform sport activities. RESULTS: Sixty patients died in hospital and 125 patients, 78 males and 47 females, were discharged alive. Among operative survivors, median age at operation was 12.8 years (range 0.4-41 years). Thirteen patients required a reoperation, the main indication was failed VSD closure. There were 16 late cardiac deaths, out of which seven were sudden and unexpected and three were in immediate relation to reoperations. One hundred and nine patients were alive at follow-up. The mean follow-up time was 25.5 years (range 20-38 years). Sixteen percent used cardiac drugs, 89% were, or had been, working normally (all professions from academics to hard manual labors were represented), 53% (64% of women) had given birth after the repair and 51% performed sport activities regularly. No patients were lost to follow-up. CONCLUSIONS: The vast majority of the patients seemed to live normal lives 20-37 years after Tetralogy of Fallot repair. Late deaths were cardiac in origin, including sudden death from arrhythmias. The number of late reoperation has been low. Considering the natural history of the disease, Fallot repair has proven to be a beneficial procedure even including the very early experience short after introduction of open heart surgery.     

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.     

Intraoperative hypercyanosis in a patient with pulmonary artery band: case report and review of the literature

A case of intraoperative cyanosis in a patient with a common atrioventricular canal palliated with a pulmonary artery (PA) band is presented. The patient's physiology was consistent with cyanosis due to inadequate pulmonary blood flow, and responded quickly to typical interventions used for a hypercyanotic episode in a patient with unrepaired Tetralogy of Fallot. Differences and similarities in the physiology of PA banding compared with Tetralogy of Fallot are presented, including a rationale for treatment options for hemodynamic decompensation occurring in the setting of anesthesia and surgery.     

不同年龄的法乐氏四联症病人心肌细胞核染色体倍性分布特征

对６０例不同年龄法乐氏四联症病人的心肌及３０例正常心肌的细胞核染色体倍性分布进行研究，结果发现：心肌细胞核染色体的二倍体随年龄增长而显著减少，四倍体和八倍体显著增多，且四联症病人与正常的心肌细胞核染色体倍性分布有显著性差异，６岁时已与正常成人相似。本研究结果提示为了防止法乐氏四联症病人心肌的不可逆病理损害，尽早实施心内修复术是必要的。     

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.     

Recent results in the surgical treatment of Fallot's tetralogy.

Tetralogy of Fallot consists essentially of two abnormalities: a large, unrestrictive ventricular septal defect and severe obstruction to the outflow of the right ventricle. A clear understanding of the principles described in this article should result in a mortality of under 5% after complete correction of tetralogy of Fallot.     

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.     

Video Review

Book review in this Article
Pantpis Sakornpant, M.D.: Technique of Correction of Tetralogy of Fallot through the Pulmonary Artery     

Sevoflurane for dental extraction in children with Tetralogy of Fallot.

Two children with Tetralogy of Fallot presented for dental extraction. Anaesthesia was induced rapidly and smoothly by inhalation of sevoflurane. We discussed the advantages of sevoflurane as an induction agent as compared to halothane in these children.     

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.     

Repeat conduit replacement in the pulmonary position without sternal resplitting for the patient with repaired Tetralogy of Fallot and the absent inferior caval vein

Recently, sternal reentry has been performed with low perioperative mortality and morbidity. In some patients, however, there are specific problems leading to life-threatening events during sternal reentry. A 27-year-old woman with repaired Tetralogy of Fallot and the absent inferior caval vein was referred to our department for pulmonary conduit replacement. Preoperative computed tomography disclosed the dominant right coronary artery and the ascending aorta longitudinally running just beneath the midline of the sternum. Therefore, we selected bilateral antero-lateral thoracotomy as the alternative approach to avoid the injury of these vital organs. The left axillar and right femoral artery were used for arterial perfusion sites. Venous drainage could be obtained from the left superior caval vein and the isolated hepatic vein via the left antero-lateral thoracotomy, and the right superior caval vein via the right antero-lateral thoracotomy. We successfully performed the repeat conduit replacement via the right antero-lateral thoracotomy.     

Pitfalls of multi slice computed tomography

Multi slice computed tomography is increasingly being used as a diagnostic modality in congenital heart disease in place of angiography. We report a six year old child with Tetralogy of Fallot in whom multi slice computed tomography (CT) gave incomplete information. The child subsequently needed angiography and a change in the operative plan.     

Lung perfusion with protective solution relieves lung injury in corrections of Tetralogy of Fallot

BACKGROUND: The aim of this study was to evaluate the protective effect of pulmonary perfusion with hypothermic protective solution on lung function after cardiopulmonary bypass in corrections of Tetralogy of Fallot. METHODS: Sixty-four consecutive children with Tetralogy of Fallot were randomly divided into a control group (n = 30) and a protective group (n = 34). Hypothermic protective solution was infused to the main pulmonary artery in the protective group. Hemodynamics and lung functions were monitored. Concentrations of malondialdehyde, tumor necrosis factor-alpha, von Willebrand factor, and endothelin in plasma were measured. The interleukin-6 and interleukin-8 levels in bronchoalveolar lavage fluid were also determined. Lung biopsy specimens were obtained after weaning from cardiopulmonary bypass. RESULTS: Oxygenation values (oxygen index and alveolar-arterial O(2) gradient) were better preserved in the protective group than in the control group. The time of mechanical ventilation and length of intensive care unit stay were shorter in the protective group compared with the control group. The tumor necrosis factor-alpha and malondialdehyde levels in plasma increased in both groups after operations, and the rising extents were lower in the protective group than in the control group. The von Willebrand factor and endothelin levels in plasma increased more significantly in the control group than in the protective group. The concentrations of interleukin-6 and interleukin-8 in bronchoalveolar lavage fluid were lower in the protective group than in the control group. The examination of histopathology demonstrated capillary hyperemia and hemorrhage, intra-alveolar edema, leukocytes accumulation, mitochondria swelling and vacuolation, and gas-blood barrier broadening in the control group, whereas there were no significant changes in the protective group. The intercellular adhesion molecule-1 expression on lung vascular endothelial cells was stronger in the control group. CONCLUSIONS: Lung perfusion with hypothermic protective solution during cardiopulmonary bypass relieved lung injury in corrections of Tetralogy of Fallot. The inhibition of lung vascular endothelial cell injury may be the major mechanism of relieving cardiopulmonary bypass-induced lung injury.     

Combined primary repair of tetralogy of Fallot and aortic root replacement

Tetralogy of Fallot associated with ascending aortic aneurysm and aortic valve regurgitation is unusual. This combination necessitates a change in operative strategy during complete tetralogy repair. We present a 43-year-old woman who successfully underwent composite graft replacement of the ascending aorta and aortic valve during primary complete tetralogy repair. Histologic examination of the aorta revealed medial degeneration.     

Andreas Vesalius (1514-1567)

ABSTRACT

Vivien Thomas (1910–1985) was an African-American scientist, pioneer, and renowned educator. Thomas's contributions to cardiovascular surgery were unequivocal, and yet it was only after his death that he gained more widespread recognition. Thomas's more notable work involves aiding in the discovery of the cause of traumatic shock, designing and guiding the first operation to treat Tetralogy of Fallot, carrying out the first atrial septectomy, and helping develop the electrical defibrillator.

Thomas struggled amidst the adversities of racism and the Great Depression (1929–1941), armed merely with a high school degree. Nevertheless, his genius and determination eventually led him to receive an honorary doctorate from John Hopkins University. His story inspired the creation of the award winning PBS documentary in 2003 Partners of the Heart and also the 2004 Emmy Award-winning HBO film Something the Lord Made. This article will aim to provide an overview to the more notable events in Thomas's amazing tale, with a particular focus on his work on the Tetralogy of Fallot.     

Surgical management of tetralogy of fallot with idiopathic thrombocytopenic purpura

The association of cyanotic heart disease with Idiopathic Thrombocytopenic Purpura (ITP) may accentuate the hemorrhagic complications of cardiac surgery under cardiopulmonary bypass. A-5-year-old girl with Chronic Steroid Refractory Idiopathic Thrombocytopenic Purpura with Tetralogy of Fallot (TOF) underwent successful intracardiac repair under normothermic cardiopulmonary bypass and warm blood potassium cardioplegia. Presurgery she received immunoglobulins and perioperatively she was administered inj methylprednisolone, inj aprotinin, platelets and fresh frozen plasma.