Holt–Oram syndrome: novel TBX5 mutation and associated anomalous right coronary artery

2011 ◽  
Vol 21 (3) ◽  
pp. 351-353 ◽  
Author(s):  
Caio B. Vianna ◽  
Nana Miura ◽  
Alexandre C. Pereira ◽  
Marcelo B. Jatene

AbstractThe Holt–Oram syndrome was confirmed in an asymptomatic 36-year-old man by a novel TBX5-gene mutation (exon 8 acceptor splicing site, c.663-1G greater than A). Computed tomography showed an atrial septal defect and an anomalous right coronary artery crossing between the aorta and pulmonary arteries. Surgery corrected the septal defect and the initial segment of the anomalous vessel was unroofed and enlarged. Anomalous coronary arteries were not previously described in the Holt–Oram syndrome patients and should be added to the list of possible associated cardiac defects.

2021 ◽  
pp. 1-4
Author(s):  
Charlie J. Sang ◽  
Stephen A. Clarkson ◽  
Elizabeth A. Jackson ◽  
Firas Al Solaiman ◽  
Marc G. Cribbs

Abstract Anomalous coronary arteries from the pulmonary artery are uncommon causes of heart failure in the adult population. This case demonstrates the unusual presentation in a patient with anomalous right coronary artery from the pulmonary artery and discusses the complex pathophysiology of this lesion and the role of guideline-directed medical therapy in the management of these patients.


2016 ◽  
Vol 2016 ◽  
pp. 1-4
Author(s):  
Asma Mursleen ◽  
Gregory Hartlage ◽  
Aarti Patel ◽  
Eric E. Harrison ◽  
C. Alberto Morales

Coronary anomalies can be observed in 1–1.2% of all angiograms performed. Majority of coronary anomalies are benign and do not lead to cardiac ischemia; however anomalous coronary arteries from the opposite sinus (ACAOS) are often associated with sudden cardiac deaths, typically in 0.11–0.35% of individuals who participate in vigorous physical activity (Peñalver et al., 2012). Left and right ACAOS have an incidence of 0.15% and 0.92%, respectively. Left ACAOS are often associated with higher incidence of sudden cardiac death; this could be secondary to greater territory of myocardial perfusion by the left coronary artery. ACAOS are often asymptomatic and initially present as sudden death following exertion in young athletes. The management of left ACAOS is clear and surgery is usually indicated. However there is a lack of consensus on the management of certain cases of right ACAOS. In this paper a case of 20 yo M with right coronary artery from left sinus is going to be presented with a discussion on pathophysiology, diagnosis, and management.


2015 ◽  
Vol 4 ◽  
pp. 66
Author(s):  
Gaurav Tyagi ◽  
Ramin Assadi ◽  
Anthony Hilliard

<p>Background: Anomalous coronary arteries pose unique challenges in coronary interventions.</p><p>Case (Method &amp; Results): Here, we report a case of use of the TIGER guiding catheter for percutaneous coronary intervention of an anomalous right coronary artery (RCA) with high anterior takeoff from trans-femoral approach, after multiple failed attempts to cannulate RCA with other catheters.</p><p>Discussion (Conclusions): Unique shape of TIGER guiding catheter allowed coaxial engagement of anomalous RCA, adequate lesion assessment and stent delivery.</p>


2020 ◽  
Vol 23 (5) ◽  
pp. E665-E667
Author(s):  
Hyungdon Kook ◽  
Jin-Ho Choi ◽  
Hyun Jong Lee ◽  
Cheol Woong Yu

Herein, we present a brief case of anomalous coronary arteries mistaken to be chronic total occlusion. Since we first presumed the anomalous coronary arteries to be chronic total occlusion of the distal right coronary artery, percutaneous coronary intervention was attempted for the presumed lesion, but it failed. Before the second attempt of percutaneous coronary intervention, coronary computed tomography angiography revealed the coronary artery from the left anterior descending artery corresponding with the distal part of the right coronary artery without connection to the right coronary artery. Therefore, we recognized the patient had anomalous coronary arteries with no connection of the vascular wall between the main and distal segments of the right coronary artery. This case may give insights to the importance of meticulous examination of coronary computed tomography angiography imaging before chronic total occlusion percutaneous coronary intervention to avoid the unnecessary procedure.


2016 ◽  
Vol 26 (5) ◽  
pp. 987-990
Author(s):  
Elena K. Grant ◽  
Charles I. Berul

AbstractA 16-year-old patient underwent successful ablation of ventricular arrhythmia originating from the aortic sinus of Valsalva following surgical unroofing of an anomalous right coronary artery. This case illustrates the complexity of decision making in the management of patients with anomalous coronary arteries and the importance of keeping an open mind when determining ventricular arrhythmia aetiology and origin.


2021 ◽  
pp. 1-3
Author(s):  
Giuliano Giusti ◽  
Salvatore Caputo ◽  
Marco Pozzi

Abstract We report on the diagnosis of anomalous coronary artery in two brothers. Following the diagnosis of anomalous coronary artery in one sibling, we screened immediate family relatives and found the same anomaly in the older brother. Familiarity in this pathology is extremely rare. We analysed and compared clinical, echocardiographic and radiological findings in the two brothers.


2021 ◽  
pp. 1-3
Author(s):  
Giuliano Giusti ◽  
Mariantonia Villano ◽  
Marco Pozzi

Abstract We report on a patient with bicuspid aortic valve and anomalous right coronary artery from the opposite sinus without evidence of intramural course. Different authors support the universal presence of intramural course in patients with origin of the right coronary artery from the opposite sinus of Valsalva in normal heart. The occurrence of both bicuspid aortic valve and the absence of intramural course may not be accidental. This might suggest a developmental interaction between bicuspid aortic valve and anomalous coronary artery. Large observational study including characterisation by intravascular ultrasonography in patients with bicuspid aortic valve and anomalous coronary is needed.


2019 ◽  
Vol 10 (5) ◽  
pp. 643-644
Author(s):  
Yoshikazu Ono ◽  
Takaya Hoashi ◽  
Kenichi Kurosaki ◽  
Hajime Ichikawa

A patient was born with truncus arteriosus type 2, left aortic arch, anomalous origin of arch vessels, left coronary artery from the right common carotid artery, and multiple extracardiac anomalies. Surgery involving translocation of the left coronary artery to the truncal root, division of branch pulmonary arteries from the truncal artery, and right ventricle-to-branch pulmonary arteries conduit placement was performed at the age of four months. Closure of the ventricular septal defect using a one-way fenestrated patch and conduit upsizing was performed successfully when the patient reached four years of age.


2020 ◽  
Vol 30 (10) ◽  
pp. 1510-1511
Author(s):  
Rachel Rosenthal ◽  
Hannah Obasi ◽  
Daniel D. Im

AbstractMyocarditis and coronary artery anomalies are both potentially life-threatening aetiologies of cardiac chest pain in children. We present a case of a young man presenting with non-exertional chest pain and subsequently found to have an anomalous origin of the right coronary artery from the left coronary sinus with an interarterial course in addition to a diagnosis of myocarditis. The patient subsequently was able to undergo surgical correction of his anomalous coronary to mitigate the risk of sudden cardiac death.


Sign in / Sign up

Export Citation Format

Share Document