Single right coronary artery-R-I subtype: A report of an extremely rare case

doi:10.1016/j.morpho.2008.06.001

Morphologie

Volume 92, Issue 297, August 2008, Pages 90-93

https://doi.org/10.1016/j.morpho.2008.06.001 Get rights and content

Summary

A single coronary ostium with no associated congenital cardiac disease is a rare congenital coronary-artery anomaly. However, a single right coronary artery has a much rarer incidence. We report here the antemortem diagnosis of a case with R-I subtype single coronary artery supplying the entire myocardium. A 36-year-old female with chest pain and dyspnea on exertion was admitted to the hospital, whose coronary angiography revealed a single, large coronary artery originating in the right aortic sinus. No observable change was detected in her electrocardiogram and her angiographic examination did not reveal any significant luminal narrowing although she experienced chest pain and dyspnea on exertion. R-I type of single coronary artery is an anomaly with a very rare incidence, which may cause myocardial ischemia and sudden death and whose recognition might be of use to physicians when diagnosing and treating this anomaly.

Résumé

La présence d’un ostium coronaire unique sans malformation cardiaque congénitale associée est un événement rare. De plus, une artère coronaire droite unique est une découverte encore plus exceptionnelle. Nous rapportons le cas d’une femme de 36 ans, présentant une artère coronaire droite unique de type R-I vascularisant l’ensemble du myocarde et diagnostiqué du vivant de la patiente. Cette patiente fut hospitalisée pour des douleurs thoraciques et une dyspnée à l’effort. La coronarographie montra une artère coronaire unique de grand diamètre issue du sinus aortique droit. Son électrocardiogramme était normal. De plus, aucune sténose vasculaire n’était visible, malgré les douleurs thoraciques et la dyspnée d’effort. L’artère coronaire, unique de type R-I est une anomalie très exceptionnelle qui peut entraîner une ischémie myocardique et une mort subite. Sa mise en évidence est impérative pour les praticiens qui traitent ce type d’anomalie.

Introduction

Single coronary artery (R-I subtype) is an extremely rare congenital anomaly of the coronary arteries in which only one coronary artery arises from the aortic trunk with a single coronary ostium. In a recent review, Shirani and Roberts [1] identified only 97 patients with this anomaly and among them only four had a single right coronary artery supplying the entire myocardium. Of these four cases, three were diagnosed at necropsy, while only one of them was diagnosed antemortem by coronary angiography. Passman et al. [2] diagnosed, antemortem, a second case with a single coronary artery originating from the right part of the coronary sulcus and supplying the entire heart. We report here another antemortem diagnosis of a single right coronary artery.

Section snippets

Case report

A 36-year-old female with chest pain and dyspnea on exertion was admitted to the hospital. Her physical examinations revealed a blood pressure of 120/70 mmHg. Her heart rate was noted to be 84 per minute with a regular rhythm. No observable change was detected in her electrocardiogram. Coronary angiography was then performed using Judging technique in standard projections. Coronary angiography showed a single, large coronary artery originating from the right aortic sinus, passing within the

Discussion

A single coronary artery is described as a rare coronary-artery anomaly since an isolated single coronary artery occurs in approximately 0.04 to 0.4% of the population undergoing coronary arteriography [3], [4], [5], [6]. According to the site of origin and anatomical distribution of the branches, isolated single coronary arteries were classified into three groups in 1979 by Lipton et al. [5] (Fig. 2), a classification that was further modified by Yamanaka and Hobbs [6]. The anomalous coronary

References (10)

J. Shirani et al.
Solitary coronary ostium in the aorta in the absence of other congenital cardiovascular anomalies
JACC
(1993)
R. Barriales Villa et al.
Adult congenital anomalies of the coronary arteries described over 31 years of angiographic studies in the Asturias principality: main angiographic and clinical characteristics
Rev Esp Cardiol
(2001)
J.M. de la Torre Hernandez et al.
Symptomatic ischemia in a patient with a single right coronary artery without lesions
Rev Esp Cardiol
(1998)
A.J. Taylor et al.
Sudden cardiac death associated with isolated congenital coronary artery anomalies
J Am Coll Cardiol
(1992)
R.S. Passman et al.
Single coronary artery: an angiographic and MRI case report
Cathet Cardiovasc Diagn
(1997)

There are more references available in the full text version of this article.

Cited by (2)

Right single coronary artery as an incidental finding in Takotsubo syndrome and acute heart failure: Case report and review of the literature
2019, Revista Portuguesa de Cardiologia
Single coronary artery (SCA) with no associated congenital heart disease is a rare congenital anomaly. Most cases are asymptomatic and incidental findings, but SCA can cause ischemia, congestive heart failure, and sudden cardiac death (SCD).
A 44-year-old woman presented with Takotsubo cardiomyopathy and cardiogenic shock. Selective cannulation of the left coronary artery (LCA) was not possible on coronary angiography (CA); an SCA was revealed arising from the right sinus, continuing distally as the circumflex artery and thereafter as the left anterior descending artery. Coronary computed tomography angiography (CCTA) confirmed left main atresia and no coronary stenosis. Cardiac magnetic resonance imaging (MRI) showed diffuse myocardial edema and no perfusion defects. The patient's clinical course was favorable under conservative management.
Our paper describes an incidental finding of right SCA. We report a Lipton type R-I, in which a dominant right SCA supplies the entire myocardium. It is the rarest SCA presentation, with an incidence of 0.0008%; only 15 cases have been reported in the literature, all of which were studied by CA. Of these 15, one had SCD, five angina, one ventricular arrhythmia and one complicated acute coronary syndrome. CCTA confirmed the diagnosis in seven patients, MRI in one and transesophageal echocardiography in another. Nine patients had coronary lesions. Two underwent coronary artery bypass grafting, one percutaneous intervention and 11 conservative treatment.
Right SCA with congenital absence of the LCA is one of the rarest coronary artery anomalies. In a significant percentage of patients it is associated with ischemia and can be life-threatening. CCTA and MRI are the modalities of choice for diagnosis and risk stratification.
Artéria coronária única (ACU) sem cardiopatia congénita associada é uma entidade rara. Maioria dos casos é assintomática e achados acidentais, mas podem causar isquémia, insuficiência cardíaca congestiva e morte súbita (MS).
Mulher, 44 anos, com cardiomiopatia Takotsubo e choque cardiogénico. Canulação seletiva da artéria coronária esquerda (ACE) não possível na coronariografia, tendo-se revelado uma ACU a emergir do seio direito, que se continuava distalmente como artéria circunflexa e, posteriormente, como artéria descendente anterior. Angio-tomografia computorizada coronária (ATCC) confirmou atrésia tronco comum, sem estenoses coronárias. A ressonância magnética cardíaca (RMN) mostrou edema miocárdico difuso, sem defeitos perfusão. Evolução favorável sob tratamento conservador.
Os autores descrevem um achado acidental de uma ACU direita. Reportam um tipo R-I de Lypton, onde a ACU direita dominante irriga todo o miocárdio. É a apresentação de ACU mais rara, com uma incidência de 0,0008%. Apenas 15 casos foram descritos na literatura. Todos realizaram coronariografia. De salientar que um teve MS, cinco angina, um arritmias ventriculares e um síndrome coronária aguda complicada. ATCC confirmou o diagnóstico em sete doentes, RMN em um e ecocardiograma transesofágico noutro. Nove doentes tinham lesões coronárias. Dois foram submetidos a cirurgia de revascularização miocárdica, um a intervenção percutânea e 11 a tratamento conservador.
ACU direita com ausência congénita da ACE é uma das anomalias das artérias coronárias mais raras. Cursa numa percentagem significativa de doentes com isquémia e pode constituir uma ameaça à vida. ATCC e RMN são as modalidades de escolha para diagnóstico e estratificação do risco.
Extremely rare R-I subtype coronary artery anomaly and accompanying incomplete myocardial bridging on the left anterior descending artery in a symptomatic patient: Multidetector computed tomography and coronary angiography findings
2009, Acta Radiologica

View full text

Case reportSingle right coronary artery-R-I subtype: A report of an extremely rare caseArtère coronaire droite unique de type R-I : une découverte très rare

Summary

Résumé

Introduction

Section snippets

Case report

Discussion

JACC

Rev Esp Cardiol

Rev Esp Cardiol

J Am Coll Cardiol

Single coronary artery: an angiographic and MRI case report

Cathet Cardiovasc Diagn

Case report
Single right coronary artery-R-I subtype: A report of an extremely rare caseArtère coronaire droite unique de type R-I : une découverte très rare