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

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.

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Absent pulmonary valve in tricuspid atresia with left ventricular outflow tract obstruction and patent arterial duct

Cardiology in the Young ◽

10.1017/s1047951100005941 ◽

1997 ◽

Vol 7 (1) ◽

pp. 94-97 ◽

Cited By ~ 1

Author(s):

Robert P. Lemke ◽

Neils G. Giddins ◽

Jonah N.K. Odim

Keyword(s):

Septal Defect ◽

Pulmonary Valve ◽

Ventricular Outflow Tract ◽

Left Ventricular ◽

Tricuspid Atresia ◽

Absent Pulmonary Valve ◽

Arterial Duct ◽

Left Ventricular Outflow ◽

Patent Arterial Duct ◽

Absent Pulmonary Valve Syndrome

AbstractWe describe a neonate with tricuspid atresia, absent pulmonary valve, right ventricular hypoplasia, small venrricular septal defect, left ventricular ourflow tract obstruction and a patent arterial duct. These finding were diagnosed by echocardiography and confirmed by cardiac catherterization and postmortem examination. This is a unique report of a functionally single ventricle variant of absent pulmonary valve syndrome and biventricular outlet obstuction.

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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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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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‘Absent pulmonary valve’ syndrome associated with muscular ventricular septal defect and aneurysmal dilation of the ascending aorta

Cardiology in the Young ◽

10.1017/s1047951100002183 ◽

1994 ◽

Vol 4 (2) ◽

pp. 184-186 ◽

Cited By ~ 1

Author(s):

Dennis Kececioglu ◽

Omar Galal ◽

Fuad Abbag

Keyword(s):

Aortic Valve ◽

Ventricular Septal Defect ◽

Septal Defect ◽

Pulmonary Valve ◽

Ascending Aorta ◽

Absent Pulmonary Valve ◽

Rare Association ◽

Muscular Ventricular Septal Defect ◽

Aneurysmal Dilation ◽

Absent Pulmonary Valve Syndrome

SummaryA rare association of ‘absent pulmonary valve’ syndrome, midmuscular ventricular septal defect and aneurysmatic dilation of the ascending aorta without pathology of the aortic valve is described in a 10-month-old infant. The diagnosis was established by echocardiography and angiography.

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Absent pulmonary valve syndrome with ventricular septal defect: Role of the arterial duct

The American Journal of Cardiology ◽

10.1016/0002-9149(90)90598-u ◽

1990 ◽

Vol 66 (2) ◽

pp. 233-234 ◽

Cited By ~ 37

Author(s):

Jose A. Ettedgui ◽

Gurleen K. Sharland ◽

Sunder K. Chita ◽

Andrew Cook ◽

Nuala Fagg ◽

...

Keyword(s):

Ventricular Septal Defect ◽

Septal Defect ◽

Pulmonary Valve ◽

Absent Pulmonary Valve ◽

Arterial Duct ◽

Absent Pulmonary Valve Syndrome

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Successful palliation by pulmonary artery banding in absent pulmonary valve syndrome with aneurysmal pulmonary arteries

Journal of Thoracic and Cardiovascular Surgery ◽

10.1016/s0022-5223(19)38909-3 ◽

1983 ◽

Vol 85 (1) ◽

pp. 125-128 ◽

Cited By ~ 15

Author(s):

J.C. Opie ◽

G.G.S. Sandor ◽

P.G. Ashmore ◽

M.W.H. Patterson

Keyword(s):

Pulmonary Artery ◽

Pulmonary Valve ◽

Pulmonary Arteries ◽

Pulmonary Artery Banding ◽

Absent Pulmonary Valve ◽

Absent Pulmonary Valve Syndrome

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Clinical presentation, natural history, and outcome of patients with the absent pulmonary valve syndrome

Cardiology in the Young ◽

10.1017/s1047951104004093 ◽

2004 ◽

Vol 14 (4) ◽

pp. 402-408 ◽

Cited By ~ 35

Author(s):

Nili Zucker ◽

Ilya Rozin ◽

Aviva Levitas ◽

Eli Zalzstein

Keyword(s):

Ventricular Septal Defect ◽

Tetralogy Of Fallot ◽

Septal Defect ◽

Clinical Presentation ◽

Pulmonary Valve ◽

Ventricular Septum ◽

Intact Ventricular Septum ◽

Absent Pulmonary Valve ◽

History Of ◽

Absent Pulmonary Valve Syndrome

So-called ‘absent pulmonary valve syndrome’ is a rare cardiac malformation, usually associated with tetralogy of Fallot. Congenital absence of the leaflets of the pulmonary valve is less common when the ventricular septum is intact. Characteristic features of the syndrome include dysplasia or absence of the pulmonary valvar leaflets, permitting severe pulmonary regurgitation, and aneurysmal dilation of the pulmonary arteries. The purpose of our study was to review our experience with patients diagnosed as having the absent pulmonary valve syndrome, and to describe their clinical presentation, natural history, and outcome. We reviewed retrospectively data from 18 patients with absent pulmonary valve syndrome, 10 boys and eight girls, treated between March 1983 and May 2003. We identified two groups of patients, one made up of 11 patients with a ventricular septal defect, in whom the morphology of the subpulmonary outflow tract was phenotypic for tetralogy of Fallot, and another group, with seven patients, having an intact ventricular septum. Family history of congenital heart disease was common only in patients with ventricular septal defect, being found in 73%, all of whom were diagnosed during infancy with variable respiratory distress. Diagnosis was delayed in 43% of the patients with an intact ventricular septum. Cardiac surgery was performed in eight patients with ventricular septal defect (73%), compared to only two patients (28%) with an intact ventricular septum. Overall mortality was 28%, with five patients dying. Although our sample was small, two clinical patterns emerged depending on the presence or absence of a ventricular septal defect. Patients with a ventricular septal defect and phenotypic features of tetralogy of Fallot have a strong family history of congenital cardiac disease, develop respiratory symptoms during infancy and exhibit a variable prognosis, despite cardiac surgery. Patients with an intact ventricular septum are usually asymptomatic, present later in life, and show a relatively benign prognosis.

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Tetralogy of Fallot With Absent Pulmonary Valve and Nonconfluent Pulmonary Arteries: A Management Conundrum

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135118775661 ◽

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.

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