Right aortic arch with mirror image branching accompanied by absent pulmonary valve syndrome and tricuspid stenosis: Prenatal echocardiographic diagnosis of an unusual congenital heart defect

2019 ◽  
Vol 36 (10) ◽  
pp. 1952-1955
Author(s):  
Zexuan Yang ◽  
Liuying Zhou
Keyword(s):  
Aortic Arch ◽  
Congenital Heart Defect ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Mirror Image ◽  
Right Aortic Arch ◽  
Absent Pulmonary Valve ◽  
Heart Defect ◽  
Absent Pulmonary Valve Syndrome

scholarly journals Importance of Absent Ductus Arteriosus in Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

2014 ◽  
Vol 41 (6) ◽  
pp. 664-667 ◽  
Author(s):  
Muhammad Yasir Qureshi ◽  
Harold M. Burkhart ◽  
Paul Julsrud ◽  
Frank Cetta
Keyword(s):  
Aortic Arch ◽  
Tetralogy Of Fallot ◽  
Subclavian Artery ◽  
Pulmonary Valve ◽  
Left Subclavian Artery ◽  
Ductus Arteriosus ◽  
Vascular Ring ◽  
Right Aortic Arch ◽  
Absent Pulmonary Valve ◽  
Absent Pulmonary Valve Syndrome

Tetralogy of Fallot without pulmonary valve syndrome is almost always associated with an absent ductus arteriosus. Patients with right aortic arch and retroesophageal left subclavian artery have a vascular ring if the left ductus arteriosus or its remnant and the Kommerell diverticulum are present. We report the cases of 2 infants in whom the role of an absent ductus arteriosus or its remnant is noteworthy. Both patients had a combination of tetralogy of Fallot with absent pulmonary valve syndrome and right aortic arch with retroesophageal left subclavian artery without a vascular ring. The absence of the ductus arteriosus has a role in the pathogenesis of tetralogy of Fallot with absent pulmonary valve syndrome. The absence of a ductus arteriosus in the right aortic arch with retroesophageal left subclavian artery precludes a vascular ring.

scholarly journals Airway obstruction in children with complex conotruncal cardiac anomalies

2021 ◽  
pp. 1-8
Author(s):  
Courtney E. Wein ◽  
Luisa Cervantes ◽  
Nao Sasaki
Keyword(s):  
Mortality Rate ◽  
Airway Obstruction ◽  
Aortic Arch ◽  
Clinical Data ◽  
Pulmonary Valve ◽  
Right Aortic Arch ◽  
Absent Pulmonary Valve ◽  
Vascular Rings ◽  
Cardiac Anomalies ◽  
Absent Pulmonary Valve Syndrome

Abstract The incidence of airway obstruction in patients with complex CHD other than vascular rings and absent pulmonary valve syndrome is unknown. We reviewed pre-operative CT and clinical data of children with conotruncal abnormalities to assess for airway obstruction. Airway obstruction was common (41% of patients), often moderate to severe, of diverse aetiology, and most commonly associated with a right aortic arch. Patients with airway obstruction showed a trend towards a higher mortality rate. Patients with complex conotruncal abnormalities should be assessed for airway obstruction as it may help predict the need for additional interventions and assist with prognostication.

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.

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.

Absent pulmonary valve syndrome with interrupted aortic arch

1998 ◽  
Vol 66 (1) ◽  
pp. 244-246 ◽  
Author(s):  
Carmelo Mignosa ◽  
Dirk G Wilson ◽  
Andrew Wood ◽  
C.Richard Kirk ◽  
Francesco Musumeci
Keyword(s):  
Aortic Arch ◽  
Pulmonary Valve ◽  
Interrupted Aortic Arch ◽  
Absent Pulmonary Valve ◽  
Absent Pulmonary Valve Syndrome

scholarly journals COMBINED SEVERE AORTIC AND PULMONARY VALVULAR STENOSIS IN A CHILD AND ITS MANAGEMENT: A CASE REPORT

2022 ◽  
Vol 54 (4) ◽  
pp. 377-379
Author(s):  
Usman Rashid ◽  
Rashid Nawaz ◽  
Zile Fatima ◽  
Shaifa Ashraf
Keyword(s):  
Case Report ◽  
Local Anesthesia ◽  
Congenital Heart Defect ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Case Reports ◽  
Semilunar Valves ◽  
Rare Combination ◽  
Heart Defect ◽  
Valvular Stenosis

Combined congenital aortic and pulmonary valvular stenosis is a rare congenital heart defect. Prevalence of severe combined valvular stenosis of aortic and pulmonary valve accounts about 0.01% and also has association with many syndromes.  This combination presents unusual diagnostic as well as management problems. Apart from a few case reports, there is little in the literature on the combined stenosis of both semilunar valves and its management. We present this rare combination in a 9 year old boy which was promptly managed with the balloon valvoplasty without any complications in the same setting under local anesthesia with sedation.

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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