Existence of Hidden Pulmonary Arteries in Tetralogy of Fallot and pulmonary artery hypertension in patients Operated with one Pulmonary Artery (original) (raw)
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2020
Introduction: The absence of a pulmonary artery is a rare congenital anomaly that occurs on its own or with some congenital cardiac disorders, particularly tetralogy of Fallot (TOF), while the hidden pulmonary artery might originate from a closed ductus arteriosus (DA) that can be stented to reach the artery.Material and methods: This prospective study describes cardiac catheterization of our nine TOF patients who had the absence of the left pulmonary artery before the operation. The patients were stratified in three groups: group one, whose closed DA were found and stented successfully to the hidden pulmonary artery; group two, whose hidden pulmonary arteries were found via the pulmonary vein angiography; and group three, for whom we could not find the remnant of the DA, or our attempt to stent the DA to the hidden pulmonary artery was not successful.We also evaluated outcomes of the other surgically-corrected TOF patients who were operated with the absent left pulmonary artery.Res...
Stenting and Reimplanting Disconnected Pulmonary Artery in Tetralogy of Fallot
The Annals of thoracic surgery, 2017
Tetralogy of Fallot with absent pulmonary valve syndrome (TOF/APV) is a rare congenital malformation. Although pulmonary artery (PA) anomalies have been observed in TOF, its association with disconnected PA is extremely rare. We report successful stenting of the disconnected left PA in a 3-year-old boy with TOF/APV followed by surgical reimplantation. The significance of this transcatheter intervention for guidance during surgery and the importance of visualizing a ductal stump on angiography as an indicator of disconnected PA are discussed.
Ductal origin of the left pulmonary artery in severe tetralogy of Fallot: Problems in management
Pediatric Cardiology, 1984
Cardiac catheterization in a neonate demonstrated tetralogy of Fallot and absence of anatomic origin of the left pulmonary artery from the main pulmonary artery. A central aortopulmonary shunt was performed in order to increase pulmonary blood flow. Because of the concern that the left pulmonary artery was actually being supplied by a ductus arteriosus, repeat catheterization was performed and this revealed closure of the ductus arteriosus with obliteration of arterial supply to the left pulmonary artery. A left Blalock-Taussig shunt was subsequently performed and this reestablished blood supply to the left pulmonary artery. This case report describes problems in management of congenital heart disease with absence of anatomic origin of the left pulmonary artery from the main pulmonary artery.
Fallot's tetralogy with absent pulmonary valve and anomalous origin of the left pulmonary artery
International Journal of Cardiology, 1993
A l-day-old asymptomatic neonate with a to and fro precordial murmur was diagnosed by cross sectional echocardiography to have Fallot's tetralogy with absent pulmonary valve, and origin of the left pulmonary artery from the ascending aorta. Moderate stenoses at the origin of the anomalous left pulmonary artery and of the right pulmonary artery were present, allowing definitive surgical correction to be deferred.
Arquivos Brasileiros de Cardiologia, 2002
We describe the case of a 40-day-old female patient with a history of breathlessness since birth who was referred to our hospital for surgical correction of common arterial trunk. The invasive investigation disclosed a Fallot¢s tetralogy anatomy associated with an anomalous origin of the left pulmonary artery from the ascending aorta. Immediately after diagnosis, the patient underwent a successful total surgical correction of the defect, including simultaneous anastomosis of the left pulmonary artery to the pulmonary trunk.
Surgical Treatment of Tetralogy of Fallot with Unilateral Absence of a Pulmonary Artery
The Annals of Thoracic Surgery, 1972
Tetralogy of Fallot with unilateral absence of a pulmonary artery imposes more marked symptomatology and a much shorter lifespan 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. An aorta-to-right pulmonary artery shunt is suggested as a satisfactory palliative measure which may have beneficial effects on the pulmonary vascular bed, permitting a safer total correction at a later date.
Congenital Heart Disease, 2022
Tetralogy of Fallot (TOF) with total anomalous pulmonary vein connections (TAPVC) is a rare type of complex congenital heart disease among all TOF cases. Co-presentation of major aortopulmonary collateral arteries (MAPCAs) compensates for the lack of central pulmonary blood flow and decreases the severity of right-to-left shunting in TOF. We present a case of a 2-year-old child with complex diagnoses of TOF, TAPVC, a large secundum atrial septal defect (ASD), and intraoperatively identified MAPCAs. She underwent surgery to repair the TAPVC, valve-sparing reconstruction of the right ventricular outflow tract, interventricular defect closure, and the creation of patent foramen ovale (PFO). After the operation, hemodynamic instability happened along with sudden blood pressure drop, desaturation, and increased central venous pressure, which subsided after administering inhalational nitric oxide (NO). A postoperative pulmonary hypertension crisis was suggested when the patient experienced recurrent symptoms after the termination of NO. Echocardiographic findings of a D-shaped left ventricle (LV), right-to-left PFO shunt and high tricuspid valve gradient firmly established the diagnosis. It was subsequently managed with continuous NO inhalation and sildenafil, which rendered a satisfactory outcome. Repaired TOF and TAPVC could be at particular risk of developing pulmonary hypertension crisis, especially in the presence of MAPCAs due to possible remodeling of the pulmonary vasculature. Furthermore, a relatively noncompliant LV function and small left atrial size may exacerbate the risk of developing postcapillary pulmonary hypertension after TAPVC repair. A successful postoperative outcome calls for a meticulous preoperative analysis of the anatomical lesions, as well as careful monitoring. KEYWORDS Pulmonary hypertension crisis; major aortopulmonary collateral arteries; tetralogy of Fallot; total anomalous pulmonary vein connection; congenital heart disease; case report This work is licensed under a Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery, 2014
Tetralogy of Fallot (TOF) with hemitruncus (HT) is a rare entity. In this report, we present our experience with this condition over the last 20 years. Between January 1994 and June 2013, 11 patients with HT and TOF underwent surgery at the All India Institute of Medical Sciences, New Delhi, India. All available clinical, radiographic, echocardiographic, cardiac catheterization, operative and follow-up data were reviewed. The mean age was 73 ± 7.1 months (range 7 months to 18 years) and the mean weight was 15.7 ± 1.2 kg. The mean preoperative saturation was 79.3 ± 11.7% (range 62-92%). Six patients had anomalous left pulmonary artery (PA), whereas 5 had an anomalous right PA arising from the aorta. Surgical procedures consisted of complete intracardiac repair of TOF with direct implantation of the anomalous PA into the main PA (n = 7), intracardiac repair of TOF with an interposition saphenous vein graft between the right PA and main PA (n = 1), and reconstruction of the left PA wit...
The American Journal of Cardiology, 1996
T he presence of systemic to pulmonary collateral arteries is well described in tetralogy of Fallot with pulmonary atresia,' but, to our knowledge, has not been described in tetralogy of Fallot and absent pulmonary valve syndrome except as a passing reference.2 We describe 2 patients with tetralogy/absent pulmonary valve syndrome who were found to have hemodynamitally important systemic to pulmonary collateral arteries. These patients were diagnosed from 1992 to 1995 at Rainbow Babies and Childrens Hospital in Cleveland, Ohio, and represent 2 of 5 patients diagnosed with this lesion during that period.