Tetralogy of Fallot With Absent Pulmonary Valve Syndrome, Right Aortic Arch, and Disconnected Left Pulmonary Artery

2013 ◽  
Vol 4 (2) ◽  
pp. 206-209 ◽  
Author(s):  
Ruth Solana-Gracia ◽  
David Crossland ◽  
Leslie Mitchell ◽  
Christopher Wren ◽  
Massimo Griselli
Keyword(s):  
Pulmonary Artery ◽  
Aortic Arch ◽  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Left Pulmonary Artery ◽  
Right Aortic Arch ◽  
Absent Pulmonary Valve ◽  
Absent Pulmonary Valve Syndrome

scholarly journals Importance of Absent Ductus Arteriosus in Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

2014 ◽  
Vol 41 (6) ◽  
pp. 664-667 ◽  
Author(s):  
Muhammad Yasir Qureshi ◽  
Harold M. Burkhart ◽  
Paul Julsrud ◽  
Frank Cetta
Keyword(s):  
Aortic Arch ◽  
Tetralogy Of Fallot ◽  
Subclavian Artery ◽  
Pulmonary Valve ◽  
Left Subclavian Artery ◽  
Ductus Arteriosus ◽  
Vascular Ring ◽  
Right Aortic Arch ◽  
Absent Pulmonary Valve ◽  
Absent Pulmonary Valve Syndrome

Tetralogy of Fallot without pulmonary valve syndrome is almost always associated with an absent ductus arteriosus. Patients with right aortic arch and retroesophageal left subclavian artery have a vascular ring if the left ductus arteriosus or its remnant and the Kommerell diverticulum are present. We report the cases of 2 infants in whom the role of an absent ductus arteriosus or its remnant is noteworthy. Both patients had a combination of tetralogy of Fallot with absent pulmonary valve syndrome and right aortic arch with retroesophageal left subclavian artery without a vascular ring. The absence of the ductus arteriosus has a role in the pathogenesis of tetralogy of Fallot with absent pulmonary valve syndrome. The absence of a ductus arteriosus in the right aortic arch with retroesophageal left subclavian artery precludes a vascular ring.

scholarly journals Tetralogy of Fallot with Absent Pulmonary Valve Syndrome with Absent Left Pulmonary Artery - A Rare Presentation

2020 ◽  
Vol 35 (1) ◽  
pp. 74-77
Author(s):  
Abul Kalam Shamsuddin ◽  
Prodip Kumar Biswas ◽  
Muhammad Ishtiaque Sayeed Al Manzoo ◽  
Md Abul Kalam Azad ◽  
Md Nurul Akhtar Hasan ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Postoperative Mortality ◽  
Left Pulmonary Artery ◽  
Absent Pulmonary Valve ◽  
Congenital Cardiac Anomaly ◽  
Ct Angiogram ◽  
Absent Left Pulmonary Artery ◽  
Absent Pulmonary Valve Syndrome

Absent left pulmonary artery with Tetralogy of Fallot (TOF) with absent pulmonary valve syndrome (APVS), is a rare congenital cardiac anomaly. Here we present such a case of A 2 year 11 month old girl with cyanosis, exertional dyspnoea. Her diagnosis is confirmed by echocardiography and CT angiogram. There are very few cases have been reported till date with high postoperative mortality. Although per operative decision making was challenging regarding pulmonary valve and size of the RPA, we performed ICR with RPA reductionplasty and creation of monocuspid pulmonary valve with success. As it is a rare association and we have overcome the hindrance we came across per operatively, we are reporting this case. Bangladesh Heart Journal 2020; 35(1) : 74-77

Tetralogy of Fallot With Absent Pulmonary Valve and Nonconfluent Pulmonary Arteries: A Management Conundrum

2018 ◽  
Vol 11 (4) ◽  
pp. NP168-NP171
Author(s):  
Sethuraman Swaminathan ◽  
Arpit Agarwal ◽  
Juan C. Infante ◽  
Eliot Rosenkranz
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Ductus Arteriosus ◽  
Pulmonary Arteries ◽  
Left Pulmonary Artery ◽  
Absent Pulmonary Valve ◽  
Rare Form ◽  
Respiratory Management ◽  
Absent Pulmonary Valve Syndrome

Tetralogy of Fallot with absent pulmonary valve syndrome is a rare form of congenital heart disease. Among the different variations with this rare anomaly is nonconfluent pulmonary artery branches with anomalous origin of the left pulmonary artery from the ductus arteriosus. The authors present one such case which was diagnosed prenatally to have tetralogy of Fallot with absent pulmonary valve and identified postnatally to have nonconfluent pulmonary artery branches in addition. We discuss the conundrum of respiratory management in this patient pre- and postoperatively due to a unique ventilation perfusion mismatch problem, which varies between the two lungs.

Unusual association of isolation of right pulmonary artery in absent pulmonary valve syndrome

2021 ◽  
pp. 021849232110063
Author(s):  
Kothandam Sivakumar ◽  
Asish Ranjan Mohakud ◽  
Ravi Agarwal
Keyword(s):  
Pulmonary Artery ◽  
Pulmonary Valve ◽  
Pulmonary Regurgitation ◽  
Left Pulmonary Artery ◽  
Absent Pulmonary Valve ◽  
Etiologic Role ◽  
Right Pulmonary Artery ◽  
Proximal Extension ◽  
Common Association ◽  
Absent Pulmonary Valve Syndrome

Absence of arterial duct, a sixth aortic arch derivative, plays an important etiologic role in Tetralogy of Fallot with absent pulmonary valve syndrome. When fetal ductus is absent, the large right ventricular stroke volume dilates the pulmonary trunk leading to pulmonary regurgitation. A proximal extension of the embryonic insult to the entire left sixth arch causes absence of the left pulmonary artery, a common association of absent pulmonary valve syndrome. On the contrary, absence of right pulmonary artery is not reported in absent pulmonary valve syndrome. A rare combination of tetralogy, absent pulmonary valve syndrome and isolation of a hypoplastic right pulmonary artery offered challenges to diagnosis and management.

Modified Repair of Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

1996 ◽  
Vol 4 (3) ◽  
pp. 178-180
Author(s):  
Jacques AM van Son ◽  
Volkmar Falk ◽  
Friedrich W Mohr
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Main Pulmonary Artery ◽  
Pulmonary Arteries ◽  
Absent Pulmonary Valve ◽  
Modified Technique ◽  
Ventricular Compliance ◽  
Critically Ill Neonates ◽  
Absent Pulmonary Valve Syndrome

A modified technique for the repair of tetralogy of Fallot with absent pulmonary valve syndrome and stenosis at the origin of the lobar or segmental pulmonary artery branches is described. This consists of generous reduction plasty of the redundant anterior and posterior walls of the aneurysmally dilated branches of the pulmonary artery, resection of the main pulmonary artery, pericardial patch augmentation of the stenotic origins of the lobar or segmental pulmonary artery branches, insertion of a valved pulmonary allograft, and closure of the ventricular septal defect. The advantages of this technique, in addition to relief of the left-to-right shunt, consist of complete decompression of the tracheobronchial structures, and restoration of the capacitance of the central pulmonary arteries. This may result in a reduced tendency for postoperative respiratory insufficiency as well as long-term preservation of right ventricular compliance. The technique was successfully performed on 2 critically ill neonates and a 3-month-old infant.

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