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.

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

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.

An unusual case of tetralogy of Fallot with an absent pulmonary valve associated with a retro-aortic innominate vein in a patient with a 16p12.2 microdeletion

2021 ◽  
pp. 1-2
Author(s):  
Niall Linnane ◽  
Andrew Green ◽  
Colin J. McMahon
Keyword(s):  
Aortic Arch ◽  
Tetralogy Of Fallot ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Unusual Case ◽  
Pulmonary Valve ◽  
Heart Defects ◽  
Innominate Vein ◽  
Absent Pulmonary Valve ◽  
Rare Association

Abstract 16p12.2 microdeletion has been associated with congenital heart defects and developmental delay. In this case, we describe the rare association between tetralogy of Fallot with an absent pulmonary valve a right-sided aortic arch and a retro-aortic innominate vein associated with a 16p12.2 microdeletion and epilepsy.

scholarly journals Tetralogy of Fallot with Absent Pulmonary Valve - A Case Report

1970 ◽  
Vol 6 (1) ◽  
pp. 48-50
Author(s):  
Md Mazibur Rahman
Keyword(s):  
Heart Failure ◽  
Tetralogy Of Fallot ◽  
Septal Defect ◽  
Pulmonary Valve ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Absent Pulmonary Valve ◽  
Transannular Patch ◽  
Patch Closure ◽  
Successful Repair

A 25 years male patient of Tetralogy of Fallot (TOF) with congenital absent of pulmonary valve (APV) presented with symptoms of palpitation and exertional respiratory distress without congestive heart failure. He underwent successful repair of intracardiac defects. The procedures consisted of patch closure of ventricular septal defect and right. ventricular outflow tract reconstruction with a monocusp transannular patch. Resection or plication of dilated pulmonary artery was not required. The patient is doing well without any symptoms. Key words: Tetralogy of Fallot; Absent pulmonary valve DOI: 10.3329/uhj.v6i1.7197University Heart Journal Vol.6(1) 2010 pp.48-50

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