Abstract
Background
Bilateral coronary ostial atresia (BCAO) is one of the rarest congenital cardiac abnormalities. Consequently, outcomes in patients with bilateral coronary ostial atresia are poorly characterized.
Case Summary
We report the clinical course of a term girl with prenatally diagnosed ventricular septal defect and normal ventricular function, who rapidly deteriorated at 6 weeks. Catheterization confirmed BCOA with the right coronary artery connecting to the right atrium via a fistula and the left coronary filling from the right ventricular outflow tract.
Discussion
This case highlights the malignant clinical course of BCOA. Early detection is limited by difficulty detecting coronary anatomy on echocardiography. Outcome was poor despite extracorporeal membrane oxygenation support. To our knowledge, this variant of BCOA has not been described.
Take-Home Messages
BCOA is rare and lethal; early stability may mask rapid deterioration. Suspicious coronary imaging warrants urgent angiography. Limited management options include extracorporeal membrane oxygenation or transplantation.
Key words: bilateral coronary ostial atresia, congenital heart disease, coronary anomalies, fistulous coronary supply, infant, right ventricle–dependent coronary circulation, severe left ventricular dysfunction
Visual Summary
Visual Summary.
Hand Injection of Contrast Is Performed in the Aortic Root
This still frame angiographic image of the aortic root shows absence of both the coronary ostia.
History of presentation
A 6-week-old term girl was born via uncomplicated vaginal delivery. She was prenatally diagnosed with a conoventricular ventricular septal defect (VSD) at 22 weeks’ gestation. She underwent serial fetal echocardiographic surveillance and was discharged home on day 2 of life after an unremarkable neonatal course. Outpatient evaluations at 7, 14, and 28 days of life demonstrated appropriate clinical progress without signs of heart failure or ischemia. However, at 42 days of life she developed poor oral intake. An echocardiogram showed moderately depressed left ventricular (LV) systolic function (ejection fraction [EF]: 37%).
Take-Home Messages
-
•
Bilateral coronary ostial atresia is a rare but potentially lethal congenital coronary anomaly in which early clinical stability and normal ventricular function do not exclude impending ischemic ventricular dysfunction and rapid clinical deterioration.
-
•
Management options are limited. Timely placement on advanced support such as ECMO and cardiac transplantation can be lifesaving.
Past medical history
Medical history was notable for prenatal diagnosis of VSD at 22 weeks’ gestation, with an otherwise unremarkable in utero clinical course. The family history was noncontributory.
Differential diagnosis
The differential diagnosis included myocarditis and coronary abnormalities such as anomalous left coronary artery from the pulmonary artery, single coronary artery, coronary artery fistulae, and coronary ostial stenosis or atresia.1
Investigations
A 12-lead electrocardiogram on day 7 of life was normal.
Transthoracic echocardiogram showed a moderate-sized conoventricular VSD, a patent foramen ovale (PFO) with left-to-right shunting, and normal biventricular size and systolic function. No valvar pathology was noted. The coronary origins were poorly visualized. Follow-up evaluations at 14 and 28 days of life showed unchanged echocardiographic findings (Figure 1, Video 1), with coronary origins remaining difficult to delineate.
Figure 1.
Color Comparison Echocardiographic Image (Day 28 of Life) Profiles the Conoventricular Ventricular Septal Defect With Predominantly Left-to-Right Flow
Left atrial and ventricular size appears normal.
At 42 days of life, the patient developed acute-onset poor oral intake and was admitted for further evaluation. Laboratory testing showed an elevated troponin level of 0.25 ng/mL (normal: 0-0.08 ng/mL) and brain type natriuretic peptide level of 4,289 pg/mL (normal: 15-75 pg/mL). A respiratory viral panel was negative. Repeat echocardiography demonstrated moderately depressed LV systolic function (EF: 37%) in the presence of a large conoventricular VSD with a low-velocity left-to-right shunt (Figure 2, Video 2). Coronary anatomy appeared abnormal, characterized by poorly defined ostia, multiple small tortuous vessels, possible coronary fistulae, and dilation of coronary veins—echocardiographic features previously described in congenital coronary ostial atresia and coronary-cameral fistulous connections.1,2
Figure 2.
2-Dimensional Echocardiographic Image (Day 42 of Life) Shows Moderately Dilated Left Ventricle and Mild to Moderately Dilated Lift Atrium
Given the echocardiographic findings she underwent cardiac catheterization with angiography on day 43 of life, which demonstrated bilateral coronary ostial atresia (Video 3). Both left and right coronary arterial systems were supplied via anomalous connections. Selective right ventricular outflow tract (RVOT) angiography confirmed left coronary artery originating from the RVOT and branching into the left anterior descending and left circumflex branches, without aortic connection (Figure 3, Video 4). Right ventricular myocardial injections demonstrated delayed opacification of the right coronary artery (RCA) with fistulous drainage into the right atrium, likely the right atrial appendage (Figure 4, Video 5). An aortic origin of the RCA was not visualized. Multiple right atrial angiograms were performed; however, selective engagement of the fistulous connection to the RCA was unsuccessful.
Figure 3.
Hand Injection of Contrast Is Performed in the Right Ventricular Outflow Tract
This angiographic clip shows that the left coronary artery system originates from the base of the right ventricular outflow tract via a fistulous connection.
Figure 4.
Hand Injection of Contrast Is Performed in the Right Atrium
This angiographic clip shows that the right coronary artery system originates from the right atrium (likely the right atrial appendage) via a fistulous connection.
Repeat echocardiogram on day 44 of life demonstrated progression to mild right ventricular dysfunction, dilation of the left-sided cardiac chambers, and severely depressed left ventricular function, with a declining EF (EF: 26% and then 19%) (Video 6).
Management
Given the large VSD, the patient was started on furosemide 1 mg/kg every 12 hours on day 7 of life. On day 45 of life, the patient developed cardiogenic shock prompting initiation of extracorporeal membrane oxygenation. Atrial septostomy was performed to optimize left atrial decompression, and afterload-reducing therapy was started. Given the technical complexity and limited success of revascularization in bilateral coronary ostial atresia, transfer to a cardiac transplant center was arranged.2
Discussion
Congenital anomalies of coronary origin are rare, with an estimated prevalence of approximately 0.5% to 1.5% in angiographic series; coronary ostial atresia represents one of the least frequently observed subtypes. Although most coronary anomalies are incidental and benign, others—particularly those affecting coronary perfusion—can have significant hemodynamic consequences.3
Bilateral coronary ostial atresia signifies the complete absence of both coronary ostia at the aortic root, forcing myocardial perfusion to depend on collaterals or fistulous communication. Echocardiographically confirmed neonatal cases with associated complex cardiac lesions (eg, pulmonary atresia) illustrate similar dependence on right ventricular–dependent coronary circulation, often with poor outcomes in early infancy.1,4
Olabiyi et al5 reported a patient in whom the entire coronary system arose from the right ventricle via a fistulous connection and another with bilateral coronary ostial atresia and a single coronary arising from the RVOT. The latter developed progressive ventricular dysfunction by day 13 and died on day 21 despite supportive measures. The patient had normal intracardiac anatomy. The present patient is unique in the sense that right coronary perfusion originated from the right atrium, whereas the left coronary circulation arose from the right ventricle. To our knowledge, this has not been previously reported in patients with bilateral coronary ostial atresia.
The precise incidence of bilateral coronary ostial atresia is unknown because of its rarity. Although bilateral coronary artery atresia has been reported in a handful of patients with pulmonary atresia and intact ventricular septum, reports in patients with other congenital heart disease or structurally normal heart are exceedingly rare.1,2
Wein et al2 reported a newborn with a patent ductus arteriosus and a PFO who had bilateral coronary ostial atresia, who had rapid postnatal deterioration, dying at 36 hours of life. Similarly, another infant was reported with structurally normal heart and bilateral coronary artery ostial stenosis. This infant had a patent ductus arteriosus and a PFO and deteriorated shortly after birth and died at 40 hours of life.6
In contrast, the present patient remained clinically stable until 6 weeks of age, with subsequent deterioration that likely coincided with the normal postnatal drop in pulmonary vascular resistance. Because the left coronary artery originated from fistulous connections with the right ventricle, elevated right ventricular pressures may initially have supported the coronary perfusion, delaying the onset of ischemia.
Early postnatal stability followed by abrupt decline has been described in prior cases of bilateral coronary ostial atresia.2 Electrocardiography findings may be normal in affected neonates, and coronary artery abnormalities often overlap in imaging appearance, making definitive diagnosis challenging without angiography.1,2
Embryologic studies in avian and human models suggest that coronary ostia formation results from penetration of the aortic wall by coronary vasculature surrounding the aorta and pulmonary artery between 6 and 7 weeks’ gestation. Failure of coronary-aortic penetration may result in coronary ostial atresia, with a likely multifactorial cause.2,7,8
Follow-Up and Outcomes
Despite extracorporeal membrane oxygenation support and aggressive medical management, ventricular function continued to deteriorate. The patient was transferred for cardiac transplant evaluation because coronary revascularization options were not feasible in the setting of bilateral ostial atresia. Transplantation has been described as the only potential definitive therapy in select cases; however, survival remains limited.1 After transfer, the patient experienced a stroke and died several days later as a consequence of these complications.
Conclusions
Bilateral coronary ostial atresia with right ventricular–dependent left coronary system and right atrial–dependent right coronary system represents an extremely rare anatomic pattern with a malignant clinical course. Early recognition and invasive evaluation are critical when coronary origins are unclear on echocardiography, especially in neonates with ventricular dysfunction.
Funding Support and Author Disclosures
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Footnotes
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the Author Center.
Appendix
For supplemental videos, please see the online version of this paper.
Appendix
Color Comparison Echocardiographic Clip (Day 28 of Life) Profiles the Conoventricular Ventricular Septal Defect
Left atrial and left ventricular size appear normal, suggesting a lack of significant shunt across the defect likely due to elevated pulmonary artery pressures.
2-Dimensional 4-Chamber Echocardiographic Clip (Day 42 of Life)
This clip shows moderately diminished left ventricular systolic function (ejection fraction: 37%). The left ventricle appears moderately dilated; the left atrium appears mild to moderately dilated.
Hand Injection of Contrast Is Performed in the Aortic Root
This angiographic clip of the aortic root shows absence of coronary arteries.
Hand Injection of Contrast Is Performed in the Right Ventricular Outflow Tract
This angiographic clip shows that the left coronary artery system originates from the base of the right ventricular outflow tract via a fistulous connection.
Hand Injection of Contrast Is Performed in the Right Atrium
This angiographic clip shows that the right coronary artery system originates from the right atrium (likely right atrial appendage) via a fistulous connection.
2-Dimensional 4-Chamber Echocardiographic Clip With Anterior Angulation (Day 44 of Life)
This clip shows rapid deterioration of left ventricular systolic function (ejection fraction: 19%). The left ventricle appears moderately dilated.
References
- 1.Wald R.M., Juraszek A.L., Pigula F.A., Geva T. Echocardiographic diagnosis and management of bilateral coronary ostial atresia in a patient with pulmonary atresia and intact ventricular septum. J Am Soc Echocardiogr. 2006;19:939.e1–939.e3. doi: 10.1016/j.echo.2006.02.006. [DOI] [PubMed] [Google Scholar]
- 2.Wein C.E., Weinberg P.M., Guleserian K.J. Congenital absence of coronary ostia in a single/common coronary system. Cardiol Young. 2019;29:993–995. doi: 10.1017/S1047951119001100. [DOI] [PubMed] [Google Scholar]
- 3.Aydinlar A., Ciçek D., Sentürk T., et al. Primary congenital anomalies of the coronary arteries: a coronary arteriographic study in Western Turkey. Int Heart J. 2005;46:97–102. doi: 10.1536/ihj.46.97. [DOI] [PubMed] [Google Scholar]
- 4.Kato A., Yoshii K., Nishikawa H., Sakurai H. Bilateral coronary ostial atresia in a patient with pulmonary atresia and ventricular septal defect. Cardiol Young. 2021;31:644–645. doi: 10.1017/S1047951121000779. [DOI] [PubMed] [Google Scholar]
- 5.Olabiyi O., Kearney D., Krishnamurthy R., Morales D., Cabrera A.G. First description of coronary artery ostial atresia with fistulous origin from a normal right ventricle. Pediatr Cardiol. 2013;34:1877–1881. doi: 10.1007/s00246-012-0427-0. [DOI] [PubMed] [Google Scholar]
- 6.Choi N.H., Piccininni J., Pollaro M., Law S., Brachio S., Levey A. Atretic coronary artery ostia in a full-term infant with early postnatal demise. J Pediatr Intensive Care. 2021;10:79–82. doi: 10.1055/s-0040-1709997. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 7.Bogers A.J., Gittenberger-de Groot A.C., Poelmann R.E., Péault B.M., Huysmans H.A. Development of the origin of the coronary arteries, a matter of ingrowth or outgrowth? Anat Embryol (Berl) 1989;180:437–441. doi: 10.1007/BF00305118. [DOI] [PubMed] [Google Scholar]
- 8.Tomanek R.J. Developmental progression of the coronary vasculature in human embryos and fetuses. Anat Rec (Hoboken) 2016;299:25–41. doi: 10.1002/ar.23283. [DOI] [PMC free article] [PubMed] [Google Scholar]
Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Supplementary Materials
Color Comparison Echocardiographic Clip (Day 28 of Life) Profiles the Conoventricular Ventricular Septal Defect
Left atrial and left ventricular size appear normal, suggesting a lack of significant shunt across the defect likely due to elevated pulmonary artery pressures.
2-Dimensional 4-Chamber Echocardiographic Clip (Day 42 of Life)
This clip shows moderately diminished left ventricular systolic function (ejection fraction: 37%). The left ventricle appears moderately dilated; the left atrium appears mild to moderately dilated.
Hand Injection of Contrast Is Performed in the Aortic Root
This angiographic clip of the aortic root shows absence of coronary arteries.
Hand Injection of Contrast Is Performed in the Right Ventricular Outflow Tract
This angiographic clip shows that the left coronary artery system originates from the base of the right ventricular outflow tract via a fistulous connection.
Hand Injection of Contrast Is Performed in the Right Atrium
This angiographic clip shows that the right coronary artery system originates from the right atrium (likely right atrial appendage) via a fistulous connection.
2-Dimensional 4-Chamber Echocardiographic Clip With Anterior Angulation (Day 44 of Life)
This clip shows rapid deterioration of left ventricular systolic function (ejection fraction: 19%). The left ventricle appears moderately dilated.