Association of Inherited Cardiac Conditions (AICC)
Arrhythmia/Cardiomyopathy Clinical Pathway (Proband)
Arrhythmia/Cardiomyopathy Clinical Pathway (Relative)
.png)
Hypertrophic Cardiomyopathy Clinical Pathway (Proband)
Hypertrophic Cardiomyopathy Clinical Pathway (Relative)
Anticoagulation in Hypertrophic Cardiomyopathy
Patients with HCM have a higher risk of developing atrial fibrillation (AF) (diastolic dysfunction, left atria (LA) enlargement) and have a higher risk of stroke (even in the absence of documented AF (See Guttmann et al) – particularly in patients with a severely dilated LA). Given the relatively young cohort of patients we see in clinic, a stroke or other systemic embolism can have considerable morbidity or mortality. It is important that all such patients are assessed for risk of stroke. Device checks or Holters should be obtained regularly (annually) to look for AF and even short runs of AF (seconds) should prompt consideration of anticoagulation (taking bleeding risk into account). Anticoagulation should be considered even in the absence of AF if there is a severely dilated LA (>50mm). There is also a lower threshold to consider anticoagulation in patients with severely dilated LA in the context of restrictive cardiomyopathy (RCM) or HCM with a restrictive physiology.
**Be aware that the CHA2DS2Vasc score is not applicable in HCM and decisions to defer anticoagulation should not be based on a score of 0. Aim to calculate a HASBLED score to make an informed decision on risks and benefits when discussing anticoagulation with patients.
SADS Families Pathway
LQT Syndrome Pathway
Brugada Syndrome Pathway
CPVT Pathway
COVID-19 Guidance
AICC Recommendations for vaccination for children with inherited cardiac conditions (ICC) September 2021
The joint committee on vaccination and immunisation recently recommended that 12- 15 year olds in specific risk categories should receive two doses of COVID-19 vaccine. This advice is available on
The risk categories included 12-15 year olds with “haemodynamically significant congenital or acquired heart disease or milder heart disease with other co-morbidity”. The 12-15 year olds who should be offered two doses of the Pfizer vaccine has been considered within the AICC and includes
-
Children with cardiomyopathy requiring medication
-
Children on medication to improve heart muscle function
-
Patients who have undergone heart transplantation
-
Children who have an inherited cardiac condition but also have significant coexisting conditions eg chronic kidney or lung disease
-
Individual children who have been specifically advised to receive vaccination by their cardiologist because of their specific clinical situation
The Chief Medical Officers of all nations of the United Kingdom have recommended that 12- 15 year olds without associated heart conditions should be offered vaccination against COVID-19 with a single dose of vaccine (Pfizer). This is based on assessment of balance of medical and educational benefits of preventing severe COVID-19 infection versus rare potential side effects of vaccination. 12 to 15 year olds with ICC outside the risk groups outline above should be offered a single dose of COVID-19 vaccination in the same way as those without associated risk factors as it is believed that the benefits outweigh the risks.
Current evidence suggests that the risk of myocarditis from CoVID-19 is far higher than from the vaccine, and that current evidence suggests that children who do get the very rare complication of myocarditis from vaccination appear to recover quickly and usually completely.
Children with Brugada or potential Brugada syndrome should take paracetamol before and after vaccination (as with other vaccinations) to try and minimise the effect of incurring a temperature following vaccination.
AICC Guidance in advising patients with ICCs about COVID-19 September 2021
The AICC wish to provide some guidance for its members in advising their patients with ICCs about COVID-19. This advice is consistent with that submitted to Specialist Commissioning and expected to be available on-line from NHS England and through 111.
All patients should follow relevant government advice on COVID-19 provided by the 111 website and call line.
Patients who are at increased risk of complications of infection who will require strict self-isolation to reduce the chance of contracting the virus:
-
Dilated, arrhythmogenic and hypertrophic cardiomyopathy patients with LV impairment and/or symptomatic left heart failure.
-
Arrhythmogenic cardiomyopathy patients with RV impairment and/or symptomatic right heart failure.
-
Symptomatic hypertrophic cardiomyopathy with or without significant obstruction.
Patients who may require special instructions:
-
All patients with Brugada syndrome and/or sodium channel disease should self-treat with paracetamol immediately if they develop signs of fever and self-isolate.
-
If patients with Brugada syndrome and/or sodium channel disease without an ICD, especially those with a spontaneous type 1 pattern, develop a high fever (>38.5C) despite paracetamol, they should contact 111 by phone, stating their condition, and may need to attend A+E*. A+E will need to be advised either by 111 or by the patient that they will attend to allow assessment by staff with suitable protective equipment. Assessment should include an ECG** and monitoring for arrhythmia. If an ECG shows the type 1 Brugada ECG pattern, then the patient will need to be observed until fever and/or the ECG pattern resolves. If all ECGs show no sign of the type 1 ECG pattern, then they can go home to self-isolate. Patients with fever who have an ICD can isolate at home and follow guidance provided by 111.
* A+E attendance may be regulated according to the capacity of service and risk of COVID-19 infection.
** ideally three different ECGs with V1 and V2 in the 4th, 3rd and 2nd intercostal spaces should be taken.
Long QT syndrome patients infected with COVID-19 who receive antivirals and/or chloroquine/hydroxychloroquine will require ECG monitoring in case of exacerbation of QT prolongation and increased risk of arrhythmias during therapy.
Guidance for Managing the Peri-operative Period in ICCs
AICC Recommendations for Managing the Peri-operative period in those affected with HCM
The AICC wish to provide some guidance for its members in advising healthcare professionals and their patients with ICCs about managing the peri-operative period in those with Hypertrophic Cardiomyopathy (HCM).
This section will follow shortly.
Guidance for Managing Dental Procedures in those with ICCs
AICC Recommendations for Managing Dental Procedures in those with an ICC
The AICC wish to provide some guidance for its members in advising healthcare professionals and their patients with ICCs about how to managing those undergoing dental procedures.
Please see this guidance document for managing dental procedures in those with LQT Syndrome: