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.

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 Echocardiographic findings in children with Tetralogy of Fallot

2015 ◽  
Vol 12 (2) ◽  
pp. 65-68
Author(s):  
Urmila Shakya ◽  
Poonam Sharma ◽  
Manish Shrestha
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Ductus Arteriosus ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Anatomical Variations ◽  
Left Pulmonary Artery ◽  
Double Outlet Right Ventricle ◽  
Echocardiographic Findings

Background and Aims: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease in children. The aim of this study was to identify various cardiac defects associated with TOF by transthoracic echocardiography.Methods: All children presenting to Shahid Gangalal National Heart Centre, with the diagnosis of TOF from 2014 to 2015 were included in the study. Echocardiograpic reports of all eighty eight children were reviewed.Results: The mean age of presentation was 3.5 } 3.9 years. Fifty six (64%) children were male and thirty two (36%) were female. Pulmonary artery anomaly was found in nine (10.2%) children which included hypoplastic pulmonary arteries, absent left pulmonary artery, post stenotic and aneurysmal dilatation of pulmonary artery. Pulmonary valve anomalies were seen in 13(14.8%) children which include pulmonary atresia, pin-hole opening of valve and bicuspid and absent pulmonary valve. Double outlet right ventricle, patent ductus arteriosus/major aortico-pulmonary collaterals, atrial septal defect and persistent left superior venacava were present in 12 (13.6%), 21 (23.9%), 22 (22.7%) and nine (10.2%) respectively. Right sided aortic arch was seen in 20 (22.7%).Conclusion: TOF is associated with various anatomical variations which need to be identified to avoid surgical complications. These can be diagnosed by echocardiography which has greatly reduced the need for invasive cardiac catheterization for these patients.Nepalese Heart Journal 2015;12(2):65-68

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

Significance of lung anomalies in fetuses affected by tetralogy of Fallot with absent pulmonary valve syndrome

2017 ◽  
Vol 27 (9) ◽  
pp. 1740-1747 ◽  
Author(s):  
Estelle Tenisch ◽  
Marie-Josée Raboisson ◽  
Françoise Rypens ◽  
Julie Déry ◽  
Andrée Grignon ◽  
...  
Keyword(s):  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Pulmonary Arteries ◽  
Fetal Lung ◽  
Bronchial Tree ◽  
Normal Lung ◽  
Absent Pulmonary Valve ◽  
Tree Compression ◽  
Significant Compression ◽  
Absent Pulmonary Valve Syndrome

AbstractObjectivesTetralogy of Fallot with absent pulmonary valve syndrome is a rare form of tetralogy of Fallot with dilatation of large pulmonary arteries. Prognosis is related to the severity of the cardiac malformation and to bronchial tree compression by dilated pulmonary arteries. This study analyses the prenatal echographic lung appearance in fetuses with tetralogy of Fallot with absent pulmonary valve and discusses its significance.MethodsWe carried out a retrospective review of fetal and postnatal files of nine fetuses diagnosed with tetralogy of Fallot with absent pulmonary valve syndrome in our institution. Correlations of prenatal ultrasound and cardiac imaging findings were obtained with outcome.ResultsAbnormal heterogeneous fetal lung echogenicity was detected in eight cases out of nine, always associated with significant lobar arterial dilatation. This aspect was well correlated with postnatal imaging and outcome in the four neonatal cases. The only fetus with normal lung echogenicity also had lower degree of pulmonary artery dilatation in the series.ConclusionsThis study demonstrates that a heterogeneous ultrasound appearance of the fetal lungs can be detected in utero in the most severe cases. This aspect suggests an already significant compression of the fetal bronchial tree by the dilated arteries that may have prognostic implications.

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