Defective regional myocardial development and vascularization in one variant of tricuspid atresia—clinical and necropsy findings in three cases

1992 ◽  
Vol 2 (1) ◽  
pp. 42-52 ◽  
Author(s):  
William N. O'Connor ◽  
Carol M. Cottrill ◽  
Michael T. Marion ◽  
Jacqueline A. Noonan
Keyword(s):  
Coronary Artery ◽  
Right Ventricle ◽  
Tricuspid Valve ◽  
Right Coronary Artery ◽  
Interventricular Septum ◽  
Ventricular Myocardium ◽  
Tricuspid Atresia ◽  
Subaortic Stenosis ◽  
Myocardial Development ◽  
The Right

SummaryFive previously reported cases have established the combination of a small right ventricle (sometimes with Uhl's anomaly), imperforate tricuspid valve with fibrotic tensor apparatus, congenital absence of the pulmonary valve, and an intact interventricular septum with muscular subaortic stenosis as a rare variant of tricuspid atresia. In this study of three new autopsy cases, we additionally identified Ebstein's malformation of the imperforate tricuspid valve, partial Uhl's anomaly, regional dysplasia of right ventricular myocardium and thinning of the interventricular septum by intramyocardial sinusoids from the right ventricle. All three new cases had epicardial anomalies of the right coronary artery—a fistula to right ventricle, ostial stenosis and proximal arterial hypoplasia. Sinusoidal connections from the right ventricle to the right coronary artery and to the sclerotic left anterior descending artery were identified in serial sections of the right ventricle and septum. Myofiber disarray, with thick walled intramyocardial arteries and sinusoids from left ventricle, involved the bulging subaortic interventricular septum. These studies are consistent with the hypothesis that defective development of the the right ventricle, along with its blood supply and associated atrioventricular and arterial valves, may underlie this unusual form of congenital heart disease.

scholarly journals 478 A rare case of right ventricular ischaemia and ventricular tachycardia in neonate caused by intermittent occlusion of origin of the right coronary artery by an echogenic mass

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
S Padmanabhan
Keyword(s):  
Ventricular Tachycardia ◽  
Coronary Artery ◽  
Early Diagnosis ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Right Coronary Artery ◽  
Perinatal Asphyxia ◽  
Poor Condition ◽  
Post Surgery ◽  
The Right

Abstract OnBehalf Cornwall A term neonate with poor condition at birth was noted to be markedly bradycardic when crying. ECG showed first degree heart block. Echocardiogram demonstrated poor bi-ventricular function. He was treated for Hypoxic ischemic encephalopathy (HIE). In the next few hours he developed short episodes of ventricular tachycardia with left bundle branch block and non-conducted P waves suggesting an origin of tachycardia from the right ventricle. His repeat ECHO suggested that he had right ventricular infarction due to a very rare finding of intermittent occlusion of the origin of the right coronary artery by an echogenic mass. He was transferred for emergency cardiac surgery to remove a clot of 1.6cm, occluding the right coronary artery. Histology findings were in keeping with a thrombus. Thrombophilia screen for both parents and infant were negative. MRI Brain did not show evidence of HIE suggesting his poor condition at birth was secondary to intermittent coronary ischemia. Discussion: Myocardial infarction (MI) in neonates is a rarely encountered and potentially life-threatening condition, with mortality rate as high as 90%. We present one of the first reported cases of successful surgical management of an acute right coronary artery thrombosis after an early diagnosis. The cause of thrombosis remains unclear in our patient. They were born in poor condition with initial pH 6.9 and lactate of 10, but with a structurally normal heart and negative thrombophilia screen. Perinatal asphyxia is a potential cause; however there is doubt that this may be a symptom rather than cause of the right coronary artery occlusion. Early diagnosis was key in management after a high level of clinical suspicion. He made significant recovery with near normal RV function, and is currently on captopril and carvedilol post-surgery. This is one of the first cases to document near full return of cardiac function following ischaemia to the right ventricle Abstract 478 Figure. 5

The Influence of Collateral Flow on the Antegrade and Retrograde Distribution of Cardioplegia in Patients with an Occluded Right Coronary Artery 

1998 ◽  
Vol 89 (5) ◽  
pp. 1099-1107 ◽  
Author(s):  
Solomon Aronson ◽  
Eric Jacobsohn ◽  
Robert Savage ◽  
Mario Albertucci
Keyword(s):  
Coronary Artery ◽  
Right Ventricle ◽  
Right Coronary Artery ◽  
Contrast Echocardiography ◽  
Coronary Artery Occlusion ◽  
Artery Occlusion ◽  
Collateral Flow ◽  
Predictive Values ◽  
Antegrade Cardioplegia ◽  
The Right

Background The predictive value of electrocardiography (ECG) and coronary angiography for cardioplegia distribution in patients with an occluded right coronary artery was evaluated. Methods Coronary angiograms and ECGs were evaluated in 15 patients with right coronary artery occlusion. Prediction of antegrade cardioplegia distribution was based on ECG evidence of infarction and coronary collateral flow determined from the angiogram. Antegrade and retrograde delivery of cardioplegia was directly assessed in all patients by myocardial contrast echocardiography. Intraoperative transesophageal echocardiographic images of the right ventricular free wall, the apex, and the intraventricular septum were recorded while 4 ml of Albunex (Mallinckrodt Medical, St. Louis, MO) was injected into antegrade and retrograde cardioplegic catheters during cardioplegia delivery. The observed (myocardial contrast echocardiography) cardioplegia distribution was compared to the predicted cardioplegia distribution. Sensitivity, specificity, positive predictive values, and negative predictive values were calculated. Results Eighty seven of 90 (97%) segments were analyzed. Angiography and ECG poorly predicted incomplete cardioplegia distribution. Electrocardiography was a better predictor of inadequate cardioplegia distribution to the right ventricle than was angiography. The negative predicted values of cardioplegia distribution ranged from 20 to 50% for the septum and right ventricle, respectively, with ECG criteria and from 0 to 33% for the septum and apex, respectively, with angiographic criteria. Antegrade cardioplegia delivery was distributed to the right ventricle in 31% of patients, despite 100% occlusion of the right coronary artery; whereas retrograde cardioplegia delivery to the right ventricle occurred 20% of the time. Conclusions In the presence of 100% right coronary artery occlusion, retrograde cardioplegia delivery is not often observed and antegrade delivery of cardioplegia to the right ventricle is not easily predicted. The preoperative angiography and ECG are not predictive of coronary collateral circulation and therefore not predictive of cardioplegia distribution to the right ventricle.

Single right coronary artery with right ventricular fistula and congenital absence of left coronary artery: an extremely rare combination

2019 ◽  
Vol 29 (11) ◽  
pp. 1402-1403
Author(s):  
Tamer Yoldaş ◽  
Meryem Beyazal ◽  
Utku A. Örün
Keyword(s):  
Coronary Artery ◽  
Right Ventricle ◽  
Right Coronary Artery ◽  
Left Coronary Artery ◽  
Coronary Artery Fistula ◽  
Left Ventricular ◽  
Congenital Absence ◽  
Collateral Vessels ◽  
The Right ◽  
Single Right Coronary Artery

AbstractWe report an extremely rare case of a 14-month-old girl who was diagnosed with a single right coronary artery with coronary artery fistula communicating with the right ventricle and congenital absence of left coronary artery. Angiography showed a dilated and tortuous single right coronary artery draining into the right ventricle, absence of left coronary system, and left ventricular coronary circulation supplied via collateral vessels.

scholarly journals Anomalous Right Coronary Artery: A Multimodality Hunt for the Origin

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
J. Gilmour ◽  
H. Kafka ◽  
G. Ropchan ◽  
A. M. Johri
Keyword(s):  
Coronary Artery ◽  
Right Coronary Artery ◽  
Cardiac Ct ◽  
Interventricular Septum ◽  
Imaging Modalities ◽  
Diagnostic Tools ◽  
Conventional Angiography ◽  
Multiple Imaging ◽  
The Right ◽  
Ct And Mri

Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital anomaly. Although there have been several cases of ARCAPA reported in the literature, we present a case which highlights the challenges of diagnosing this rare condition and the incremental value of using multiple imaging modalities. A healthy 48 year old female presented with angina and exertional shortness of breath. She had a normal cardiovascular examination, negative cardiac enzymes and an unremarkable chest X-ray. She did, however, have T-wave inversions in leads V1–V3. Transthoracic echocardiography (TTE), as the first imaging investigation, led to an initial provisional diagnosis of a coronary-cameral fistula. It showed unusual colour Doppler signals in the right ventricle and a prominent pattern of diastolic flow within the right ventricular myocardium, especially along the interventricular septum. A subsequent multimodality approach, correlating images from angiography, CT and MRI was instrumental in confirming the diagnosis of ARCAPA and planning for surgical correction. Cardiac CT and MRI are non-invasive, three-dimensional imaging modalities with high diagnostic accuracy for coronary artery anatomic anomalies. If echocardiography and conventional angiography have been inconclusive, cardiac CT and MRI are especially important diagnostic tools.

ASSOCIATION OF TRANSPOSITION OF THE GREAT VESSELS AND RUDIMENTARY RIGHT VENTRICLE, WITH AND WITHOUT TRICUSPID ATRESIA

PEDIATRICS ◽  
1957 ◽  
Vol 19 (5) ◽  
pp. 851-857
Author(s):  
Althea Kessler ◽  
Paul Adams
Keyword(s):  
Right Ventricle ◽  
Clinical Findings ◽  
Left Ventricular ◽  
Tricuspid Atresia ◽  
Subaortic Stenosis ◽  
P Waves ◽  
Congestive Failure ◽  
Great Vessels ◽  
Atrial Shunt ◽  
The Right

A group of eight cases with three different combinations of congenital cardiac defects but similar clinical findings has been presented. The differential diagnosis from other types of congenital heart disease has been discussed. All had transposition of the great vessels and same obstruction to aortic outflow, either subaortic stenosis or a rudimentary right ventricle from which the aorta arose. Six cases had tricuspid atresia and two had rudimentary right ventricle without tricuspid atresia. The salient features were: 1) Early congestive failure. 2) Absent or minimal cyanosis. 3) Large heart and pulmonary congestion roentgenographically. 4) Electrocardiographic findings of peaked P waves and a pattern of left ventricular hypertrophy. 5) Angiocardiographic findings of a right to left atrial shunt, failure to visualize the right ventricle, early opacification of the left ventricle and pulmonary artery, and late and inadequate opacification of a small aorta.

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