Absent Pulmonary Valve with L-Transposition of Great Arteries: A Case report

Absent pulmonary valve syndrome and L-transposition of the great arteries are rare congenital anomalies. To our knowledge, patients with both have not been previously reported. We present a case of surgical success in the patient with absent pulmonary valve, L-transposition of the great arteries, dextrocardia, double outlet left ventricle, hypoplastic anatomical right ventricle, valvular aortic stenosis and tracheomalacia. We performed anatomical left ventricle to pulmonary artery shunt using an 8-mm expanded polytetrafluoroethylene bicuspid-valved conduit, pulmonary artery plication, and tracheostomy. The patient was discharged in a stable condition while waiting for the Glenn procedure until getting her parent’s permission.

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Unilateral Pulmonary Artery Banding for Critically ill Neonate with Absent Pulmonary Valve Syndrome: A Case Report

10.21203/rs.3.rs-38986/v1 ◽

2020 ◽

Author(s):

Junichi Koizumi ◽

Shigeto Tsuji ◽

Tomoyuki Iwase ◽

Azuma Tabayashi ◽

Hajime Kin ◽

...

Keyword(s):

Pulmonary Artery ◽

Critically Ill ◽

Pulmonary Valve ◽

Main Pulmonary Artery ◽

Stable Condition ◽

Bloody Stool ◽

Absent Pulmonary Valve ◽

Palliative Procedure ◽

Open Chest ◽

Absent Pulmonary Valve Syndrome

Abstract Backgound Absent pulmonary valve syndrome (APVS) is a rare congenital heart disease that is challenging to manage when it presents with severe symptoms at an early age. We describe a critically ill neonate with tetralogy of Fallot (TOF) with APVS in whom main pulmonary artery (PA) banding failed, but alternative left PA banding and PA plication and open chest strategy effectively stabilized the patient’s condition and enabled elective cardiac repair 6 days later. Case presentation A 3.2-kg baby girl with prenatal diagnosis of TOF and APVS was electively delivered by cesarean section. She was intubated soon after birth, however her condition deteriorated with respiratory and metabolic acidosis even with maximum ventilator settings and inotropic support. On the second day of life, she developed bloody stool suggesting necrotizing enterocolitis, indicating the need for a palliative procedure without cardiopulmonary bypass. First, we attempted main PA banding but abandoned it due to suspected myocardial ischemia caused by left main coronary artery compression. As an alternative procedure, we performed left PA banding, which improved oxygen saturation and increased blood pressure; the chest was left open. Postoperatively, the patient’s condition improved dramatically within 48 hours. On the eighth day of life, we performed an elective intracardiac repair on her under stable preoperative condition. At the 5-year follow-up, she was in a stable condition at home with home oxygen therapy. Conclusion Unilateral PA banding and open chest strategy may be an alternative treatment of choice for critically ill neonates with TOF with APVS.

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Two-stage repair in a patient with “absent pulmonary valve syndrome”

Cardiology in the Young ◽

10.1017/s1047951100010672 ◽

1993 ◽

Vol 3 (1) ◽

pp. 67-69

Author(s):

Hussam Tamimi ◽

Omar Galal ◽

Zohair Al Halees

Keyword(s):

Respiratory Distress ◽

Pulmonary Valve ◽

Stable Condition ◽

Postoperative Recovery ◽

Pulmonary Trunk ◽

Dramatic Improvement ◽

Absent Pulmonary Valve ◽

Two Stage ◽

Severe Respiratory Distress ◽

Absent Pulmonary Valve Syndrome

SummaryA seven-month-old boy with severe respiratory distress because of absence of the leaflets of the pulmonary valve underwent banding of the pulmonary trunk. Postoperative recovery was uneventful with dramatic improvement of his general and hemodynamic status. Repair was performed successfully almost two years later when the child had grown satisfactorily and was in stable condition.

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

Asian Cardiovascular and Thoracic Annals ◽

10.1177/02184923211006309 ◽

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.

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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

Cardiology in the Young ◽

10.1017/s1047951114001097 ◽

2014 ◽

Vol 25 (4) ◽

pp. 794-796 ◽

Cited By ~ 1

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

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135112470960 ◽

2013 ◽

Vol 4 (2) ◽

pp. 206-209 ◽

Cited By ~ 2

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

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Correction of Absent Pulmonary Valve Syndrome Using a Pericardial Valved Conduit

Asian Cardiovascular and Thoracic Annals ◽

10.1177/021849230201000321 ◽

2002 ◽

Vol 10 (3) ◽

pp. 270-272

Author(s):

Sukasom Attanawanich ◽

Pongsak Khowsathit ◽

Wichaya Withurawanit

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Pulmonary Valve ◽

Pulmonary Regurgitation ◽

Effective Alternative ◽

Absent Pulmonary Valve ◽

Aortic Homograft ◽

Valved Conduit ◽

Absent Pulmonary Valve Syndrome

Absent pulmonary valve syndrome in a 4-month-old infant was successfully corrected using a fresh autologous pericardial trileaflet valved conduit. He recovered from operation with only mild pulmonary regurgitation at 4 months postoperatively. This technique is an effective alternative for infants with congenital heart disease who need tissue valved conduits. It may be more suitable than the aortic homograft by reason of the shortage of small homografts and its lower costs.

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Airway compression management in late-presenting absent pulmonary valve syndrome

Cardiology in the Young ◽

10.1017/s1047951113002151 ◽

2014 ◽

Vol 25 (2) ◽

pp. 295-300 ◽

Cited By ~ 3

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.

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Absence of Left Pulmonary Artery in Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

Journal of Cardiac Surgery ◽

10.1111/jocs.12461 ◽

2014 ◽

Vol 30 (1) ◽

pp. 104-107 ◽

Cited By ~ 1

Author(s):

Mustafa Kemal Avsar ◽

Tolga Demir ◽

Hale Bolgi Demir

Keyword(s):

Pulmonary Artery ◽

Tetralogy Of Fallot ◽

Pulmonary Valve ◽

Left Pulmonary Artery ◽

Absent Pulmonary Valve ◽

Absent Pulmonary Valve Syndrome

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Modified Repair of Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

Asian Cardiovascular and Thoracic Annals ◽

10.1177/021849239600400314 ◽

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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