Summary
Background
Congenital left coronary artery abnormalities such as ostial stenosis or atresia are extremely rare. Diagnosis in the neonate has not been reported.
Aims
To describe five neonates with left coronary artery orifice abnormalities and discuss pathophysiology, diagnosis and treatment options, with a focus on the importance of autopsy in unexpected neonatal death.
Methods
Retrospective assessment of medical files of neonates with left coronary abnormalities seen during a 12-year period (2000–2012).
Results
Three neonates with anatomical ( n = 2) and functional ( n = 1) left coronary stenosis and two neonates with ostial atresia were identified. The three infants with coronary stenosis died within minutes to days after birth because of cardiac failure refractory to intensive care treatment; at autopsy, left coronary ostial stenosis ( n = 2) and high take-off with acute angle origin and tangential vertical course ( n = 1) were diagnosed. The fourth neonate was in cardiac failure due to critical aortic stenosis; left coronary ostial atresia was diagnosed during an emergency catheter procedure and the infant died after aortic valve dilatation. The fifth infant had a cardiac arrest on the third day of life; she was diagnosed with left coronary ostial atresia by coronary angiography and died during attempted revascularization surgery at 2 weeks of life.
Conclusion
Congenital coronary ostial abnormalities can lead to severe heart failure and unexpected neonatal death. Systematic examination of the coronary arteries should be part of any neonatal autopsy. Coronary angiography remains the diagnostic method of choice despite advances in non-invasive imaging. Revascularization surgery seems indicated in symptomatic children based on small patient series.
Résumé
Contexte
Les anomalies congénitales de la coronaire gauche comme la sténose ou l’atrésie ostiale sont extrêmement rares. Leur diagnostic en période néonatale n’a pas encore été décrit.
Objectifs
Nous rapportons cinq cas néonataux en discutant la physiopathologie, les options diagnostiques et thérapeutiques et l’importance de l’autopsie en cas de décès néonatal.
Méthodes
Analyse rétrospective monocentrique des nouveau-nés avec anomalie congénitale de la coronaire gauche entre 2000 à 2012.
Résultats
Nous avons identifié trois nouveau-nés avec une sténose soit anatomique ( n = 2), soit fonctionnelle ( n = 1) ainsi que deux nouveau-nés avec une atrésie ostiale de la coronaire gauche. Les trois nouveau-nés avec sténose coronaire sont morts entre quelques minutes et quelques jours de vie malgré une réanimation maximale. L’autopsie a montré une sténose ostiale de la coronaire gauche ( n = 2) et une naissance ectopique au-dessus de la jonction sinotubulaire avec un trajet tangentiel vertical ( n = 1). L’atrésie de l’ostium gauche chez le quatrième patient a été diagnostiquée lors d’une dilatation aortique percutanée pour sténose aortique critique. Le patient est décédé après le cathétérisme cardiaque. Le diagnostic d’atrésie pour le cinquième nouveau-né a été fait lors d’un cathétérisme cardiaque fait pour explorer un arrêt cardiorespiratoire. Il est décédé lors de la tentative de revascularisation chirurgicale.
Conclusion
Des anomalies coronaires congénitales peuvent mener à une insuffisance cardiaque néonatale et à un décès néonatal. Toute autopsie néonatale doit comprendre un examen des artères coronaires. La coronarographie reste la méthode diagnostique de choix chez le nouveau-né malgré les avancées en imagerie non invasive. La revascularisation chirurgicale semble indiquée dans les petites séries rapportées.
Background
Congenital abnormalities of the left coronary orifice such as ostial stenosis or atresia are rare cardiac defects, with the exception of those seen in common arterial trunk . Only a few autopsy or case reports and small surgical series of adult and paediatric cases have been reported in the literature . The clinical picture is heterogeneous and depends on age at onset of clinical symptoms. In the infant or small child, cardiac failure due to severe biventricular dysfunction can be the clinical presentation in case of left coronary artery (LCA) atresia or severe ostial stenosis, the latter being extremely rare. Coronary ostial atresia can thus mimic the clinical picture usually seen in case of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), which is far more frequent in this age group . In the older child, adolescent or adult, clinical symptoms are those of chest pain during exercise or at rest, suggestive of coronary abnormality.
Proximal tangential coronary course is recognized as a rather rare congenital coronary abnormality but there are few reports on functional impact, even though secondary myocardial ischaemia as a consequence seems possible. Acute coronary angle take-off has been associated with sudden death in adults .
To our knowledge, neonatal diagnosis of LCA ostial stenosis or atresia has not been previously reported. Here, we describe five neonates with anomalies of the LCA orifice (atresia/stenosis/acute angle take-off with tangential proximal course) whose common particularities are the early onset of symptoms and fatal outcome. This report underlines the importance of a well-conducted neonatal autopsy to elucidate unexpected neonatal death.
Methods
We searched our institutional database for neonates with the diagnosis of congenital LCA abnormalities (ostial atresia/stenosis, abnormal course, ectopic ostium) seen at our centre from January 2000 to October 2012. All medical files, including autopsy protocols and available pictures, were reviewed.
Results
Five neonatal cases of congenital abnormalities of the LCA orifice were finally identified in our institutional database. Two patients had isolated severe LCA ostial stenosis and one patient had a high take-off with acute angle of origin and proximal tangential LCA course. All three anomalies were diagnosed at autopsy. The other two neonates had atresia of the LCA orifice, both diagnosed by coronary angiography; in one case it was an isolated abnormality and in the other it was associated with severe neonatal aortic stenosis.
Case 1
The first neonate was born at 35 weeks’ gestation with a birth weight of 2230 g and an Apgar score of 10-8-5; he became quickly hypoxic with respiratory distress requiring mechanical ventilation and transfer to a neonatal intensive care unit. Emergency echocardiography showed signs of persistent pulmonary hypertension with normal cardiac anatomy (including normal pulmonary veins) but with associated severe biventricular dysfunction. The neonate died after a few hours in refractory hypoxia with multiorgan failure, despite all the intensive care treatment strategies that were undertaken. Autopsy showed an anatomically normal heart except for a pinpoint LCA orifice ( Fig. 1 A and B ). The right coronary artery (RCA) was completely normal. No other congenital extracardiac malformations were visible. There was no macroscopic congestion of liver, kidney or bowels. Ischaemic alterations of the grey matter were seen in both hemispheres. Histological analysis of samples from different sites in the left ventricle did not reveal any myocardial necrosis or ischaemia. The pulmonary parenchyma showed moderate quantities of hyaline membranes in the alveoli without significant obstruction of the airways.
Case 2
This neonate was prematurely born at 33 weeks’ gestation with a birth weight of 1700 g after an uneventful pregnancy without obstetrical complications. Neonatal adaptation was characterized by immediate bradycardia. Usual neonatal resuscitation manoeuvres were unsuccessful and the baby died 30 min after delivery. Autopsy was performed and the heart was anatomically normal except for a slit-like LCA orifice ( Fig. 2 ). The right coronary artery (RCA) was normal in origin and branching pattern. Histopathological analysis of samples systematically taken from different sites in both ventricles did not reveal signs of myocardial necrosis or ischaemia. There were no associated extracardiac congenital malformations.
Case 3
This neonate (twin pregnancy) was delivered by Caesarean section due to maternal metrorrhagia at 31 weeks’ gestation with a birth weight of 1500 g; on the third day of life she brutally deteriorated with a clinical low cardiac output syndrome. Emergency echocardiography showed two dilated and severely hypokinetic ventricles. The neonate died after an hour despite all the resuscitation manoeuvres that were undertaken. Sepsis work-up and metabolic investigations were negative. Autopsy showed an anatomically normal heart with an RCA that was normally situated and permeable. The LCA had a high take-off with a normally sized and shaped orifice situated above the sinotubular junction of the left coronary cusp ( Fig. 3 A ). The coronary thus had an acute angle of origin and a proximal tangential and nearly vertical descending course on the aortic wall ( Fig. 3 B).
