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.

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.

Correction of Absent Pulmonary Valve Syndrome Using a Pericardial Valved Conduit

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.

Dimensions of the pulmonary arteries in rat fetuses with congenital heart disease

1994 ◽  
Vol 4 (3) ◽  
pp. 291-297
Author(s):  
Kazuo Momma ◽  
Masahiko Ando ◽  
Masaaki Yoshigi
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Congenital Heart ◽  
Pulmonary Arteries ◽  
Common Arterial Trunk ◽  
Arterial Trunk ◽  
Right Pulmonary Artery ◽  
The Right

AbstractSo as to understand better the pathogenesis of enlargement or hypoplasia of pulmonary arteries in congenital heart disease, we studied the dimensions of the pulmonary arteries in 74 fetuses with congenital heart disease induced by administration of bis-diamine to pregnant rats. The congenital malformations induced included 12 with large ventricular septal defect, 17 with tetralogy of Fallot, 15 with tetralogy together with severe valvar pulmonary stenosis and absence of the arterial duct, 17 with tetralogy with absent pulmonary valve syndrome and absence of the arterial duct, and 13 with common arterial trunk with a confluent segment supplying the pulmonary arteries. For comparison, 16 fetuses of the same gestational age with normal hearts were studied. After rapid whole-body freezing on the 21st day of gestation, the fetuses were studied by means of serial cross-sectional photographs of the thorax. The diameter of the right pulmonary artery of the fetus was of comparable dimensions in the normal hearts (480±10 µm) (mean±SEM), those with ventricular septal defects (470±10 µm), common arterial trunk (520±20 µm), and tetralogy of Fallot (500±10 µm). These findings suggest that the commonly observed enlargement of the right pulmonary artery in patients with ventricular septal defect and common arterial trunk, and hypoplasia of the right pulmonary artery in tetralogy of Fallot, occur postnatally in response to abnormal postnatal pulmonary blood flow.

Left hemitruncus with normal right-sided pressures in an adult

2013 ◽  
Vol 24 (5) ◽  
pp. 926-928 ◽  
Author(s):  
Arima Nigam ◽  
Vijay Trehan
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Artery ◽  
Right Ventricle ◽  
Rare Case ◽  
Congenital Heart ◽  
Left Pulmonary Artery ◽  
Ascending Aorta ◽  
Anomalous Origin ◽  
Right Pulmonary Artery ◽  
The Right

AbstractHemitruncus is a rare congenital heart disease. Anomalous origin of the left pulmonary artery is not only rare but also pathogenetically different from anomalous origin of the right pulmonary artery from the ascending aorta. In most cases in isolated hemitrucus pressures in the right ventricle and the normally originating pulmonary artery are systemic or suprasystemic. We present a rare case of anomalous origin of the left pulmonary artery from the ascending aorta diagnosed in an adult with normal pressures in the right ventricle and normally originating pulmonary artery. To the best of our knowledge, this unique haemodynamics has never been reported in the literature.

Sign in / Sign up

Export Citation Format

Share Document