We assessed the effect of anomalous aortic origin of a coronary artery on the risk of early and late postoperative events after aortic or mitral valve replacement in adults.

Between 2005 and 2022, 29,579 adults underwent surgical aortic or mitral valve replacement at Cleveland Clinic. Among these, 29 had an unrepaired coronary artery rising anomalously from the aorta that was not intervened upon during valve surgery, 19 (65%) an anomalous circumflex, and 9 (31%) an anomalous right. Operative outcomes were compared between the 29 patients with anomalous coronary arteries and 87 balancing score (1:3) matched patients with normal coronary origin. Median follow-up was 6.5 years.

Among matched groups, major morbidity and mortality 24% (n = 7) in patients with anomalous coronaries and 20% (n = 17) among patients with normal coronary origin (P = .7). Ten-year freedom from coronary reintervention was 83% versus 100% (P[log-rank] = .005), and 10-year survival was 59% versus 53% (P[log-rank] = .8). One patient experienced a coronary injury from valve surgery, in which the incidentally found anomalous retroaortic circumflex was immediately repaired without further complication. There was no coronary reintervention after discharge in the normal coronary origin group and three in the anomalous coronary group; however, only one of these patients required intervention on the anomalous coronary.

Anomalous coronaries were uncommon in surgical valve replacement patients at a high-volume centre. The origin and course of each coronary should be assessed before valve replacement. With careful planning, valve replacement does not result in a significantly higher prevalence of postoperative ischaemia, mortality, or reintervention.

Individuals with childhood-onset coronary artery anomalies are at increased risk of lifelong complications. Although pregnancy is thought to confer additional risk, a few data are available regarding outcomes in this group of women. We sought to define outcomes of pregnancy in this unique population.

We performed a retrospective survey of women with paediatric-onset coronary anomalies and pregnancy in our institution, combined with a systematic review of published cases. We defined paediatric-onset coronary artery anomalies as congenital coronary anomalies and inflammatory arteriopathies of childhood that cause coronary aneurysms. Major cardiovascular events were defined as pulmonary oedema, sustained arrhythmia requiring treatment, stroke, myocardial infarction, cardiac arrest, or death.

A total of 25 surveys were mailed, and 20 were returned (80% response rate). We included 46 articles from the literature, which described cardiovascular outcomes in 82 women (138 pregnancies). These data were amalgamated for a total of 102 women and 194 pregnancies; 59% of women were known to have paediatric-onset coronary artery anomalies before pregnancy. In 23%, the anomaly was unmasked during or shortly after pregnancy. The remainder, 18%, was diagnosed later in life. Major cardiovascular events occurred in 14 women (14%) and included heart failure (n=5, 5%), myocardial infarction (n=7, 7%), maternal death (n=2, 2%), cardiac arrest secondary to ventricular fibrillation (n=1, 1%), and stroke (n=1, 1%). The majority of maternal events (13/14, 93%) occurred in women with no previous diagnosis of coronary disease.

Women with paediatric-onset coronary artery anomalies have a 14% risk of adverse cardiovascular events in pregnancy, indicating the need for careful assessment and close follow-up. Prospective, multicentre studies are required to better define risk and predictors of complications during pregnancy.

Anomalous aortic origin of a coronary artery is a congenital cardiac condition that can be associated with increased risk of sudden death. To date, quality of life and exercise performance have not been evaluated in patients with this condition who do not undergo surgical repair.

We carried out a cross-sectional analysis of patients with unoperated anomalous aortic origin of a coronary artery at our institution from 1 January, 2000 to 31 January, 2016. We prospectively assessed quality of life using standardised questionnaires. Medical records were reviewed for clinical and exercise stress test data. Statistical analyses were performed using Student’s t-tests and Spearman’s correlation coefficients.

In total, 56 families completed the questionnaires. The average age at enrolment was 14.7±6 years. The majority were male (n=44, 78.6%) and had interarterial anomalous right coronary artery (n=38, 67.9%). Patients had normal quality of life on the PedsQL 4.0 Report, Child Health Questionnaire Child Form 87, and SF-36v2. Their parents had normal quality of life on the PedsQL 4.0 Parent Report, but parents of exercise-restricted patients had decreased Physical Functioning, General Health Perception, Emotional Impact on Parent, and Physical Summary scores (p<0.001–0.048) on the Child Health Questionnaire Parent Form 50.

Patients with unoperated anomalous aortic origin of a coronary artery appear to have normal quality of life, but parents of exercise-restricted patients have decreased general health and emotional and physical quality of life scores. Improved counselling of families may be beneficial in this group. Future studies with more patients should evaluate quality of life and exercise performance over time.