Scimitar syndrome with absence of the right pulmonary artery: A case with volume-induced, reversible, left-sided pulmonary hypertension

1992 ◽  
Vol 13 (2) ◽  
pp. 119-121 ◽  
Author(s):  
Albrecht Beitzke ◽  
Gerfried Zobel ◽  
Bruno Rigler ◽  
J�rg Ingolf Stein ◽  
Christa Suppan
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Scimitar Syndrome ◽  
Right Pulmonary Artery ◽  
The Right

Scimitar syndrome associated with absence of the right pulmonary artery and a persistent primitive hepatic venous plexus

2005 ◽  
Vol 15 (2) ◽  
pp. 216-218 ◽  
Author(s):  
Gabriella Agnoletti ◽  
Damien Bonnet ◽  
Jacques De Blic
Keyword(s):  
Pulmonary Artery ◽  
Scimitar Syndrome ◽  
Venous Plexus ◽  
Caval Vein ◽  
Inferior Caval Vein ◽  
Right Pulmonary Artery ◽  
Unusual Variant ◽  
The Right

An 18-month-old boy, referred because of an infection of the airways, was found to have a right-sided heart, a hypoplastic right lung, absence of the right pulmonary artery, and persistence of the hepatic venous plexus. The benign association of this unusual variant of the scimitar syndrome and persistence of the hepatic venous plexus needs to be recognised in order to avoid extensive investigations and surgery involving the inferior caval vein.

Systemic arterial supply of the right lung with venous drainage to the pulmonary arterial circuit

2011 ◽  
Vol 21 (6) ◽  
pp. 710-712 ◽  
Author(s):  
Peter Fritsch ◽  
Freyja-Maria Smolle-Juettner ◽  
Andreas Gamillscheg
Keyword(s):  
Pulmonary Artery ◽  
Venous Drainage ◽  
Left Pulmonary Artery ◽  
Scimitar Syndrome ◽  
Arterial Supply ◽  
Respiratory Problems ◽  
Right Pulmonary Artery ◽  
Pulmonary Arterial ◽  
The Right ◽  
Systemic Arterial Supply

AbstractIn a girl suffering from “Scimitar syndrome”, a rerouting of the scimitar vein was performed at the age of 6 years, but no embolisation of the aberrant systemic vessel was done. She presented with recurring respiratory problems 13 years later. An angiography revealed an invert flow from the aberrant systemic vessel via the right pulmonary artery into the left pulmonary artery. After pneumonectomy, she recovered well.

scholarly journals P1488 Anomalous origin of left coronary artery from right pulmonary artery in association with scimitar syndrome

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
S Aly ◽  
R Lizano Santamaria ◽  
S J Yoo
Keyword(s):  
Pulmonary Hypertension ◽  
Coronary Artery ◽  
Pulmonary Artery ◽  
Scimitar Syndrome ◽  
Right Atrial ◽  
Anomalous Origin ◽  
Coronary Perfusion ◽  
Antegrade Flow ◽  
The Right

Abstract Clinical Presentation A full-term neonate was referred to our institution because of respiratory distress. CXR was significant for right lung hypoplasia and mild cardiomegaly. ECG showed normal sinus rhythm, right atrial enlargement, and right ventricular hypertrophy with no signs of ischemia. Imaging Findings The initial echocardiogram demonstrated PAPVD with the right upper pulmonary vein draining into IVC/RA junction with flow acceleration (mean gradient= 7 mmHg), moderate ASD, small muscular VSD with left-right shunting, moderate PDA with bidirectional shunting. Forward flow was seen in the proximal part of left main coronary artery (LMCA). RV systolic pressure was supra-systemic with a qualitatively moderately reduced RV systolic function. The patient was taken to the catheterization lab where MPA angiography revealed an antegrade flow from the RPA into LMCA supplying both the anterior descending and the circumflex arteries. A selective injection within the scimitar vein showed drainage of the right lung into a vertical vein connecting with stenosis to IVC. A follow up echocardiogram to re-examine the coronary origin revealed an anomalous origin of LMCA from proximal RPA; 3 mm distal to branch pulmonary artery bifurcation with mainly antegrade low velocity flow into LMCA and LAD. (Image 1) Role of Imaging in Patient Care - Imaging of the coronary origin in patients with ALCAPA can be challenging especially if the LMCA originates from RPA. Also, the presence of pulmonary hypertension might contribute to maintain coronary perfusion and lead to misinterpretation of the antegrade flow in LMCA and its branches. - In certain situations, cardiac catheterization is essential to make the diagnosis of ALCAPA which prevented a potentially catastrophic outcome. Catheter intervention with a series of balloon dilations of the stenotic scimitar vein was successful in relieving the stenosis. Summary/Discussion Points: - Extensive review of the available literature revealed only three cases of Scimitar syndrome associated with ALCAPA. In all of these cases, the LMCA originated from the posterior sinus of MPA. Our case is the first to report ALCAPA from RPA in association with Scimitar syndrome. This presentation might have led to the initial misinterpretation of the echocardiography images. - The presence of pulmonary hypertension in our patient maintained an adequate antegrade flow across the LMCA preventing significant coronary steal and signs of myocardial ischemia. - The report highlights the challenges in making the diagnosis of ALCAPA with echocardiograms. Moreover, we discuss the role of cross-sectional and invasive imaging to rule out potential coronary arteries anomalies in patients with Scimitar syndrome, as this a rare although a very significant association that may have important implications in their outcomes. Abstract P1488 Figure. ALCAPA origin from RPA

Anterior translocation of the right pulmonary artery for airway obstruction caused by pulmonary hypertension

2002 ◽  
Vol 74 (2) ◽  
pp. 569-571 ◽  
Author(s):  
Masaaki Yamagishi ◽  
Keisuke Shuntoh ◽  
Akiyuki Takahashi ◽  
Takeshi Shinkawa ◽  
Takako Miyazaki ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Airway Obstruction ◽  
Right Pulmonary Artery ◽  
The Right

Unusual right pulmonary artery–to-left atrial communication associated with scimitar syndrome

2021 ◽  
pp. 1-3
Author(s):  
Asish R. Mohakud ◽  
Sangamesh Bawage ◽  
Kothandam Sivakumar
Keyword(s):  
Pulmonary Artery ◽  
Blood Supply ◽  
Left Atrial ◽  
Venous Drainage ◽  
Scimitar Syndrome ◽  
Rare Association ◽  
Different Types ◽  
Right Pulmonary Artery ◽  
The Right

Abstract Right pulmonary artery to left atrial fistula is classified based on the right pulmonary artery branching, individual right pulmonary venous drainage, and presence of an aneurysmal segment. A rare association with scimitar syndrome and right lung devoid of blood supply from right pulmonary artery is described in this report. The anatomical and management differences between the different types are highlighted.

Surgical treatment of isolated unilateral absence of pulmonary artery with pulmonary hypertension

2021 ◽  
pp. 1-2
Author(s):  
Moyu Hasegawa ◽  
Shigemitsu Iwai ◽  
Kazuhiko Ishimaru
Keyword(s):  
Pulmonary Hypertension ◽  
Surgical Treatment ◽  
Pulmonary Artery ◽  
Pulmonary Artery Pressure ◽  
Left Pulmonary Artery ◽  
Single Stage ◽  
Artery Pressure ◽  
Medication Therapy ◽  
Right Pulmonary Artery ◽  
The Right

Abstract A one-and-a-half-month-old patient with isolated unilateral absence of the right proximal pulmonary artery with prominent left pulmonary hypertension was diagnosed using CT. Medication therapy was initiated first. Left pulmonary artery pressure decreased after the initiation of medication therapy, and single-stage reconstruction of the right pulmonary artery was performed. The patient is asymptomatic 14 months postoperatively.

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