Sudden Cardiac Death Syndrome

Podcast – Sudden Cardiac Death Syndrome

This post accompanies the podcast ‘Sudden Cardiac Death Syndrome with Harshil Dhutia at the Premier Conference 2024‘. This was recorded live at the Hope Church in Winchester as part of the PREMIER conference. We are grateful to the organising team for hosting us and allowing us to use the audio. The PIER and PREMIER websites are full of amazing resources for anyone working in Paediatric Emergency Medicine and we recommend them highly.


Listening Time – 19:20

Sudden Cardiac Death

Introduction

Sudden cardiac death (SCD) in young individuals, particularly athletes, is a tragic and often shocking event. The Premier Conference, held in Winchester in June 2024, provided crucial insights into this phenomenon. Consultant cardiologist from Leicester, Harshaal Gupta, presented an in-depth discussion on the causes, diagnosis, and management of SCD in the young. This blog post summarizes the key points from his talk, offering valuable information for healthcare professionals and families alike.

Defining Sudden Cardiac Death

Sudden cardiac death is defined as an unexpected death from a confirmed or suspected cardiac cause within an hour of symptom onset if witnessed or within 24 hours of last being seen in good health if unwitnessed. This condition predominantly affects young individuals between 12 and 35 years of age. Although rare, SCD is the leading cause of non-accidental death in young people, often striking without prior symptoms.

Recognising Symptoms and Risk Factors

In many cases, the first sign of an underlying cardiac condition is a sudden cardiac arrest. Key symptoms to watch for include unexplained syncope, particularly if it occurs during exercise, without warning, or is associated with injury. Other vague symptoms like chest pain and breathlessness can be misleading and often go unnoticed.

The major symptom that you must take seriously is syncope, particularly in the presence of red flags

  • Syncope without warning
  • Syncope occuring during physical exercise
  • Syncope with injury
  • Syncope associated with any abnormal clinical findings

Other symptoms like palpitations and breathlessness are common, but non specific.

Causes of Sudden Cardiac Death

SCD can result from various conditions, which can be broadly categorized into inherited cardiomyopathies (heart muscle diseases), ion channel diseases (such as long QT syndrome, Brugada syndrome, and CPVT), diseases of the aorta, and acquired conditions like myocarditis. Among these, inherited cardiac conditions are significant contributors to SCD.

Importance of ECG and Genetic Testing

ECG plays a crucial role in diagnosing potential heart conditions that could lead to SCD. Certain ECG patterns are indicative of specific diseases:

  • Long QT Syndrome: Characterized by a prolonged QT interval.
  • Brugada Syndrome: Identified by a coved-shaped ST elevation in leads V1 to V2.
  • CPVT: Exercise-induced bidirectional ventricular tachycardia.

Genetic testing and molecular autopsy are vital for diagnosing and managing these conditions. They help identify the genetic mutations responsible for these diseases, which can then inform family screening and preventive measures.

Family Evaluation and Screening

Following a case of sudden cardiac death, it is mandatory to perform a specialist post-mortem to identify the cause of death. This often involves genetic testing, which can provide closure for the family and guide further evaluations. Family members of the deceased should undergo screening in a dedicated inherited cardiac conditions clinic, which includes genetic counselling and testing if a pathogenic mutation is found.

Treatment and Management

Managing young patients with inherited cardiac conditions involves a combination of lifestyle advice, medication, and in some cases, invasive treatments. Lifestyle changes may include avoiding certain medications or strenuous activities. Medications like beta-blockers can help manage symptoms, while an implantable cardioverter-defibrillator (ICD) might be necessary for those at high risk of SCD.

Exercise Considerations

One of the significant dilemmas is advising young athletes with inherited cardiac conditions on exercise. While exercise has numerous benefits, it can also increase the risk of SCD in those with underlying cardiac abnormalities. Shared decision-making, respecting patient autonomy, and individualized risk assessments are essential. Not all sports carry the same risk, and the type and intensity of exercise should be carefully considered.

Evaluating family members

  • A post mortum is mandatory in the UK to try to identify the cause of death – this will include a molecular post mortum looking for potential causes.
  • If a genetic cause is identified, prompt family screening should take place, ideally in a specialist clinic.
  • There is currently a pilot taking place to standardise the care after sudden unexpected death.
  • Many of the cause of sudden cardiac death are autosomal dominant and so there is a 50% chance of relatives having a genetic predisposition.

Emerging Technologies and Screening Programs

Advances in technology, such as implantable loop recorders, provide better monitoring for at-risk patients. These devices can continuously track heart rhythms and detect arrhythmias that could lead to SCD.

While controversial, screening programs for young athletes have shown promise in countries like Italy, where mandatory ECG screening has reduced the incidence of SCD. However, the cost-effectiveness and feasibility of implementing such programs on a larger scale remain debated.

Should all at risk get an implantable cardiac defibrillator?

These are very difficult decisions, with lifelong implications, and really only suitable for those at highest risk, to balance out the harm and benefit of the procedure

  • If the patient has survived a cardiac arrest and arrythmogenic cause found an ICD is indicated.
  • Those without cardiac arrest, but with high risk features (which vary condition to condition) an ICD will be discussed and ideally a shared decision made.
  • Implantation of an ICD is not indicated purely to keep playing sport.

Challenges and Future Directions

There are several challenges in managing and preventing SCD in young individuals:

  • Heterogeneity of Conditions: Inherited cardiac conditions are highly variable, making diagnosis and management complex.
  • Limited Evidence Base: Current guidelines are often based on expert consensus rather than robust clinical trials.
  • Systemic and Research Limitations: There is a need for large, long-term registries to better understand these conditions and improve care.

Conclusion

  • Arrythmogenic Right Ventricular Cardiomyopathy is a particular risk, compared to other conditions.
  • These decisions are not evidence based and based in expert opinion.
  • The low event rates make evidence hard to gather and large registries and years of follow up are needed.

This talk highlighted the importance of a comprehensive approach to managing sudden cardiac death in the young. This includes early recognition of symptoms, thorough family screening, appropriate use of diagnostic tools, and personalized treatment plans. As we continue to advance our understanding and technology, the goal is to reduce the incidence of SCD and improve the quality of life for those affected by inherited cardiac conditions.


ECGs

Long QT

Life in the Fast Lane – Long QT

Brugada Syndrome

Life in the Fast Lane – Brugada Syndrome

Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)

Life in the Fast Lane – Biventricular Tachycardia (typical of CPVT)

Further Reading


Podcast Transcription


The Speaker – Harshil Dhutia

Harshil Dhutia is a consultant cardiologist at Glenfield Hospital, University Hospitals of Leicester the lead for inerited cardiac conditions service in the region, providing specialist care for patients with genetic heart diseases and their family members. He is a International Board of Heart Rhythm Examiners certified heart rhythm specialist for all aspects of cardiac device implantation and management including pacemakers, defibrillators and cardiac resynchronisation therapy. He is an expert in sports cardiology and has extensive clinical and research interests in the management of competitive and recreational athletes with cardiovascular disease. He is the medical lead for the Joe Humphries Memorial Trust, a Leicestershire based charity that raises awareness of sudden cardiac death in young people and provides cardiovascular resuscitation and AED training in schools, sports clubs and to members of the community.

Harshil Dhutia

Where to listen

You can listen to our podcast in numerous ways, ensuring you never miss an episode no matter where you are or what device you’re using. For the traditionalists, Apple Podcasts and Google Podcasts offer easy access with seamless integration across all your Apple or Android devices. Spotify and Amazon Music are perfect for those who like to mix their tunes with their talks, providing a rich listening experience. If you prefer a more curated approach, platforms like Podchaser and TuneIn specialize in personalising content to your tastes. For those on the go, Overcast and Pocket Casts offer mobile-friendly features that enhance audio quality and manage playlists effortlessly. Lastly, don’t overlook YouTube for those who appreciate a visual element with their audio content. Choose any of these platforms and enjoy our podcast in a way that suits you best!


Cite this article as: Iain Beardsell, "Podcast – Sudden Cardiac Death Syndrome," in St.Emlyn's, June 20, 2024, https://www.stemlynsblog.org/sudden-cardiac-death/.

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