Repair in infancy of tetralogy of Fallot with absence of the leaflets of the pulmonary valve (absent pulmonary valve syndrome) using a valved pulmonary artery homograft

1992 ◽  
Vol 2 (1) ◽  
pp. 25-29 ◽  
Author(s):  
Delores Danilowicz ◽  
Salvatore Presti ◽  
Stephen B. Colvin ◽  
Eugenie F. Doyle
Keyword(s):  
Heart Failure ◽  
Congestive Heart Failure ◽  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Primary Repair ◽  
Absent Pulmonary Valve ◽  
Symptomatic Group ◽  
Pulmonary Arterial ◽  
Absent Pulmonary Valve Syndrome

SummaryTwo infants, aged eight days and four months, with tetralogy of Fallot and absence of the leaflets of the pulmonary valve, presented with acute respiratory distress and congestive heart failure. Primary repair was performed using a cryopreserved pulmonary arterial homograft. Both infants survived and are doing well three and two years later. A review of the surgical procedures attempted in this symptomatic group of patients is presented together with a discussion of the pathophysiology of this defect.

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.

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

Compression of the superior caval vein by an aneurysmal right pulmonary artery in a patient with absent pulmonary valve syndrome

2014 ◽  
Vol 25 (1) ◽  
pp. 191-192 ◽  
Author(s):  
Tomoaki Murakami ◽  
Yuko Saito ◽  
Hiromichi Nakajima
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Absent Pulmonary Valve ◽  
Unique Case ◽  
Caval Vein ◽  
Superior Caval Vein ◽  
Right Pulmonary Artery ◽  
Absent Pulmonary Valve Syndrome

AbstractWe report a unique case of compression of the superior caval vein by an aneurysmal right pulmonary artery in a patient with tetralogy of Fallot with absent pulmonary valve.

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.

scholarly journals Unrepaired Tetralogy of Fallot with Absent Pulmonary Valve in a Mildly Symptomatic 16-Year-Old Boy

2016 ◽  
Vol 43 (6) ◽  
pp. 517-519 ◽  
Author(s):  
Diana E. Drogalis-Kim ◽  
Brian L. Reemtsen ◽  
Leigh Christopher Reardon
Keyword(s):  
Heart Failure ◽  
Tetralogy Of Fallot ◽  
Unusual Case ◽  
Medical Literature ◽  
Pulmonary Valve ◽  
Primary Repair ◽  
Failure To Thrive ◽  
Left Pulmonary Artery ◽  
Absent Pulmonary Valve ◽  
Absent Left Pulmonary Artery

Absent pulmonary valve is a rare and severe variant seen in only 3% to 6% of patients with tetralogy of Fallot. Fetuses with this combined condition who survive through birth typically need intervention in infancy or early childhood because of respiratory distress, heart failure, or failure to thrive. We describe the unusual case of a mildly symptomatic 16-year-old boy with these conditions who underwent successful primary repair. Our search of the medical literature yielded fewer than 5 cases of tetralogy of Fallot with absent pulmonary valve (or variants with an absent left pulmonary artery) and survival without repair into later adolescence or adulthood.

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