scholarly journals Airway compression management in late-presenting absent pulmonary valve syndrome

2014 ◽  
Vol 25 (2) ◽  
pp. 295-300 ◽  
Author(s):  
Anastasia Martinez-Esteve Melnikova ◽  
Tornike Sologashvili ◽  
Maurice Beghetti ◽  
Cécile Tissot ◽  
Afksendiyos Kalangos ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Chart Review ◽  
Pulmonary Valve ◽  
Pulmonary Arteries ◽  
Retrospective Chart Review ◽  
Absent Pulmonary Valve ◽  
Airway Compression ◽  
Primary Endpoints ◽  
The Mean ◽  
Absent Pulmonary Valve Syndrome

AbstractIntroduction: Patients with absent pulmonary valve syndrome often present early with airway compression from aneurysmal pulmonary arteries. This study reviews our experience in managing absent pulmonary valve syndrome in later presenting children, and techniques used for managing airway compression. Methods: This study is a retrospective chart review of all patients who underwent repair of absent pulmonary valve syndrome from 2000 to 2012 at our institution. The primary endpoints were post-operative bronchoscopic and clinical evidence of persistent airway compression and need for reinterventions on the pulmonary arteries. Results: A total of 19 patients were included during the study period. The mean age at repair was 4.1±3.0 years (range 10 months–11 years). In all, seven patients had pre-operative bronchoscopic evidence of airway compression, which was managed by pulmonary artery reduction plasty in four patients and Lecompte manoeuvre in three patients. There were no peri-operative deaths. In patients with pulmonary artery plasty, two had no post-operative airway compression, one patient had improved compression, and one patient had unchanged compression. In patients managed with a Lecompte manoeuvre, two patients had no or trivial airway compression and one had improved compression. There were six late reinterventions or reoperations on the pulmonary arteries – two out of four in the pulmonary artery plasty group and one out of three in the Lecompte group. Conclusions: Most late-presenting patients with absent pulmonary valve syndrome do not have airway compression. Either pulmonary artery reduction plasty or the Lecompte manoeuvre can relieve proximal airway compression, without a significantly different risk of pulmonary artery reintervention between techniques.

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 Absent Pulmonary Valve Syndrome With Tricuspid Atresia, Ventricular Septal Defect, and Aneurysmal Dilated Pulmonary Artery: A Case Report of Successful Fontan Completion

2016 ◽  
Vol 9 (1) ◽  
pp. 101-104 ◽  
Author(s):  
Ayano Futuki ◽  
Keiichi Fujiwara ◽  
Kosuke Yoshizawa ◽  
Hisanori Sakazaki
Keyword(s):  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Septal Defect ◽  
Pulmonary Valve ◽  
Pulmonary Arteries ◽  
Tricuspid Atresia ◽  
Absent Pulmonary Valve ◽  
Aneurysmal Dilatation ◽  
Central Pulmonary Artery ◽  
Absent Pulmonary Valve Syndrome

Of the associated cardiac defects with absent pulmonary valve, the combination of tricuspid atresia, ventricular septal defect, and aneurysmal dilatation of the pulmonary arteries is very rare. We report the case of a low-birth-weight girl (2,282 g) with this anomaly, which was prenatally diagnosed. Fontan completion was successfully achieved at 16 months of age, following staged palliative procedures including banding of the main pulmonary trunk and plication of the aneurysmal dilated central pulmonary artery.

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.

Lecompte Procedure for Relief of Severe Airway Compression in Children With Congenital Heart Disease

2019 ◽  
Vol 10 (5) ◽  
pp. 558-564
Author(s):  
Christina L. Greene ◽  
Richard D. Mainwaring ◽  
Douglas Sidell ◽  
Michal Palmon ◽  
Frank L. Hanley
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Surgical Procedure ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Heart Defects ◽  
Pulmonary Atresia ◽  
Absent Pulmonary Valve ◽  
Airway Compression ◽  
Absent Pulmonary Valve Syndrome

Purpose: Children with congenital heart disease may present with severe airway compression prior to any surgical procedure or may develop airway compression following their surgical procedure. This combination of congenital heart defect and airway compression poses a significant management challenge. The purpose of this study was to review our experience with the Lecompte procedure for relief of severe airway compression. Methods: This was a retrospective review of ten patients who underwent a Lecompte procedure for relief of severe airway compression over the past nine years (2010-2018). Three patients with absent pulmonary valve syndrome presented with severe symptoms prior to any surgical procedure. Seven patients presented with symptoms of airway compression following repair of their congenital heart defects (one with absent pulmonary valve syndrome, three patients had repair of pulmonary atresia with ventricular septal defect, and three patients had undergone aortic arch surgery). The median age at presentation was two years (range: one day to seven years). Results: The ten patients underwent a Lecompte procedure without any significant complications or operative mortality. The median interval between the surgical procedure and extubation was 9.5 days. No patients have required any further interventions for relief of airway obstruction. Conclusions: The Lecompte procedure is a surgical option for young children who present with severe airway compression. The patients in this series responded well to the Lecompte procedure as evidenced by clinical relief of airway compression.

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.

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.

Anomalous origin of the right pulmonary artery from the ascending aorta accompanied by absent pulmonary valve syndrome and right-sided aortic arch: a rare case in adult congenital heart disease

2014 ◽  
Vol 25 (4) ◽  
pp. 794-796 ◽  
Author(s):  
Zahra Khajali ◽  
Ali Mohammadzadeh ◽  
Marzieh Khayatzadeh
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Artery ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Ascending Aorta ◽  
Absent Pulmonary Valve ◽  
Right Pulmonary Artery ◽  
The Right ◽  
Absent Pulmonary Valve Syndrome

AbstractWe present a rare congenital heart disease in a 20-year-old man with anomalous origin of the right pulmonary artery from the ascending aorta, accompanied by absent pulmonary valve syndrome, and a right-sided aortic arch suspected initially in transthoracic echocardiography and subsequently confirmed by cardiac catheterisation and computed tomography angiography.

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