European Prospective Registry on Anomalous Aortic Origin of the Coronary Arteries (EUROAAOCA)

EUROAAOCA Registry

Anomalous aortic origin of a coronary artery (AAOCA) is a group of rare congenital heart defects with various clinical presentations. The lifetime-risk of an individual living with AAOCA is unknown, and data from multicentre registries are urgently needed to adapt current recommendations and guide optimal patient management. The European Registry for AAOCA (EURO-AAOCA) aims to assess differences with regard to AAOCA management between centres.

Study Overview

• Status: Recruiting
• Conditions: Arrhythmia; Heart Defects, Congenital; Congenital Heart Disease; Sudden Cardiac Death; Surgical Procedure, Unspecified; Diagnosis; Adult Children; Coronary Artery Anomaly
• Intervention / Treatment: Procedure: unroofing or other surgery if needed

Detailed Description

EURO AAOCA study database on management for "ANOMALOUS AORTIC ORIGIN OF CORONARY ARTERIES.

This is a simple Excel database in 8 sections (see below). The patient's identification (ID) should be anonymous, and can be decided by each center; the investigators suggest to enclose Centre ID number (which is communicated by the leading center) and a sequential number or date of birth (dd/mm/yy).

If no procedure has been done for the patients , it is required to the "PERIPROCEDURAL, SURGICAL DETAILS" and "POSTPROCEDURAL" data sheets (which are outlined in RED). In the "PERIPROCEDURAL" sheet, the participants may refer either to the surgical or non surgical procedure.

Baseline and long term clinical evaluation sections must be completed for all patients.

In the database, there are lists of multiple different variables which are summarized in tables enclosed in the section, in which each item corresponds to a number.

EURO AAOCA Study DATABASE sections

1. Baseline demo and anatomy: it includes demographic data, and anatomical details for each patient; basic anatomy and course can be selected scrolling an enclosed list; remaining data are requested as yes/no answer
2. Baseline symptoms and indications: it includes symptoms info and indications to surgical/interventional/clinical follow up for each patient; symptoms can be selected scrolling an enclosed list; remaining data are requested as yes/no answer or text; in particular, the participants are required to distinguish between recreational non-competitive sports (2-3/week) and Competitive (>5 times/week), Reason for diagnosis in asymptomatic patient can be selected scrolling a list.
3. Baseline instrumental: it includes all possible diagnostic methodologies used for first diagnosis; the test is considered abnormal when it is gives diagnosis of AAOCA, and in this case , the participants are required to describe the findings as precise as possible. In particular, for non-surgical patients, the participants are required to provide a date of the first time any instrumental test suspected AAOCA. ECG data can be selected scrolling an enclosed list; remaining data are requested as yes/no answer or text
4. Periprocedural: if the patient has gone to surgical or interventional management, the participants are required to fill this section: data can be selected scrolling an enclosed list; remaining data are requested as yes/no answer or text
5. Surgical details: for surgical patients only, the participants are required to fill this section; data can be selected scrolling an enclosed list; remaining data are requested as yes/no answer or text
6. Post procedural: for surgical or interventional patients only, the participants are required to fill this section; data can be selected scrolling an enclosed list; remaining data are requested as yes/no answer or text
7. Follow up 1: this section must be filled for all patients (surgical/interventional /only medical management) and must be updated every year and sent back to the coordinator every June 15th, so as to check yearly the patients. The Investigators suggest an annual follow up for at least 5 years in a row. Follow up data can be selected scrolling an enclosed list; remaining data are requested as yes/no answer or text. T
8. Follow up Instrumental diagnosis: this section must be filled for all patients (surgical/interventional /only medical management) and must be updated every year and sent back to the coordinator every June 15th, so as to check yearly the patients. The investigators suggest to do this for at least 5 years in a row. Follow up data can be selected scrolling an enclosed list; remaining data are requested as yes/no answer or text

• Study Type: Observational
• Enrollment (Estimated): 500

Contacts and Locations

• Study Contact: Name: Massimo Padalino, MD PhD; Phone Number: 00393408922564; Email: massimo.padalino@unipd.it
• Study Contact Backup: Name: Chris Grani, MD PhD; Phone Number: 0041788158858; Email: christoph.graeni@insel.ch

Study Locations

• Italy
  • PD
    • Padova, PD, Italy, 35128
      • Recruiting
      • Massimo Padalino
      • Contact: Massimo Padalino; Phone Number: 3408922564; Email: massimo.padalino@unipd.it
• Switzerland
  • Bern, Switzerland
    • Recruiting
    • Christopher Grani
    • Contact: Christopher Grani, MD PhD; Email: christoph.graeni@insel.ch

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child; Adult; Older Adult
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

all patients undergoing incidental or not diagnosis of AAOCA

Description

Inclusion Criteria:

• All patients with a diagnosis of AAOCA (either referred to surgery or to medical follow-up )

Exclusion Criteria:

• isolated high-coronary take-off (≥ 5mm above sino-tubular junction), anomalous origin of a circumflex from the right coronary artery, anomalous course with a normal origin, and association to major congenital heart disease (i.e. Tetralogy of Fallot, transposition of the great arteries, anomalous origin of a coronary from the pulmonary artery).

Study Plan

How is the study designed?

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
adult > 30 years; All adult patient > 30 yrs undergoing diagnosis (incidental or not) of anomalous aortic origin of coronary arteries	Procedure: unroofing or other surgery if needed; unroofing of coronary artery; Other Names: clinical follow up
Juvenile < 30 yrs; All young patient <30 yrs undergoing diagnosis (incidental or not) of anomalous aortic origin of coronary arteries	Procedure: unroofing or other surgery if needed; unroofing of coronary artery; Other Names: clinical follow up

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Incidence of Symptoms at diagnosis and after surgery	The study will assess the presence of symptoms (angina, chest pain, distress, sudden cardiac death-SCD, syncope, aborted SCD) at diagnosis, and after eventual surgical repair, to assess incidence of symptoms in patients with this anomaly and effectiveness of surgery in removing symptoms.	5 years
Incidence of postoperative adverse events	The study will assess the incidence of operative death and adverse events after surgery (in those who undergo surgery).	5 years
Incidence of adverse events at follow up	The study will assess the incidence of death and adverse events at clinical follow up either in patients who required a surgical repair or in those who did not undergo surgery because of lack of indications.	5 years

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Incidence of any arrhythmias at Follow up	The study will assess the incidence of any (mostly ventricular) arrhythmias in all patients, either surgical or non surgical	5 years
Rate of return to unrestricted competitive sport	The study will assess the rate of return to unrestricted sport and competitive sport activity in all patients, either surgical or non surgical	5 years

Collaborators and Investigators

• Sponsor: University of Padova
• Collaborators: University Medical Center Groningen; Azienda Ospedaliera Universitaria Integrata Verona; IRCCS Policlinico S. Donato; Guy's and St Thomas' NHS Foundation Trust; University Hospital, Ghent; Azienda Ospedaliero-Universitaria di Parma; Hospital Universitario La Paz; Ospedale Civile Ca' Foncello; Insel Gruppe AG, University Hospital Bern; Mitera Children's Hospital, Athens Heart Surgery Institute, Greece; Cliniques universitaires Saint-Luc, Bruxelles, Belgium
• Investigators: Principal Investigator: Massimo Padalino, MD PhD, University of Padova

Publications and helpful links

General Publications

• Padalino MA, Jegatheeswaran A, Blitzer D, Ricciardi G, Guariento A. Surgery for Anomalous Aortic Origin of Coronary Arteries: Technical Safeguards and Pitfalls. Front Cardiovasc Med. 2021 May 12;8:626108. doi: 10.3389/fcvm.2021.626108. eCollection 2021.
• Bigler MR, Kadner A, Raber L, Ashraf A, Windecker S, Siepe M, Padalino MA, Grani C. Therapeutic Management of Anomalous Coronary Arteries Originating From the Opposite Sinus of Valsalva: Current Evidence, Proposed Approach, and the Unknowing. J Am Heart Assoc. 2022 Oct 18;11(20):e027098. doi: 10.1161/JAHA.122.027098. Epub 2022 Oct 7.
• Ponzoni M, Frigo AC, Padalino MA. Surgery for Anomalous Aortic Origin of a Coronary Artery (AAOCA) in Children and Adolescents: A Meta-Analysis. World J Pediatr Congenit Heart Surg. 2022 Jul;13(4):485-494. doi: 10.1177/21501351221095424.
• Grani C, Padalino MA. Editorial: Coronary Artery Anomalies: A 2020 Review. Front Cardiovasc Med. 2022 Feb 10;9:776951. doi: 10.3389/fcvm.2022.776951. eCollection 2022. No abstract available.
• Thiene G, Frescura C, Padalino M, Basso C, Rizzo S. Coronary Arteries: Normal Anatomy With Historical Notes and Embryology of Main Stems. Front Cardiovasc Med. 2021 May 31;8:649855. doi: 10.3389/fcvm.2021.649855. eCollection 2021.
• Padalino MA, Franchetti N, Hazekamp M, Sojak V, Carrel T, Frigiola A, Lo Rito M, Horer J, Roussin R, Cleuziou J, Meyns B, Fragata J, Telles H, Polimenakos AC, Francois K, Veshti A, Salminen J, Rocafort AG, Nosal M, Vedovelli L, Guariento A, Vida VL, Sarris GE, Boccuzzo G, Stellin G. Surgery for anomalous aortic origin of coronary arteries: a multicentre study from the European Congenital Heart Surgeons Associationdagger. Eur J Cardiothorac Surg. 2019 Oct 1;56(4):696-703. doi: 10.1093/ejcts/ezz080.

Study record dates

Study Major Dates

• Study Start (Actual): January 1, 2019
• Primary Completion (Estimated): January 1, 2025
• Study Completion (Estimated): January 1, 2029

Study Registration Dates

• First Submitted: October 3, 2023
• First Submitted That Met QC Criteria: October 11, 2023
• First Posted (Actual): October 19, 2023

Study Record Updates

• Last Update Posted (Actual): October 19, 2023
• Last Update Submitted That Met QC Criteria: October 11, 2023
• Last Verified: October 1, 2023

More Information

Terms related to this study

• Keywords: coronary; sudden cardiac death; anomalous
• Additional Relevant MeSH Terms: Pathologic Processes; Cardiovascular Diseases; Death, Sudden; Cardiovascular Abnormalities; Heart Arrest; Heart Diseases; Death; Congenital Abnormalities; Heart Defects, Congenital; Death, Sudden, Cardiac
• Other Study ID Numbers: EUROAAOCA group

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO
• IPD Plan Description: The anonymized demographic and clinical data underlying this study can be shared on reasonable request to the principal investigator

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No